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Sample records for spinal artery syndrome

  1. Anterior Spinal Artery Syndrome Following Coronary Artery Bypass Grafting: a Case Report.

    PubMed

    Mirhosseini, Seyed Mohsen; Meghdadi, Soheil; Moghaddam, Ali Sanjari

    2017-01-01

    We present a patient with unstable angina candidate for coronary artery bypass grafting. Saphenous vein graft was used in obtuse marginal and left internal mammary artery to left anterior descending artery properly. After surgery, the patient experienced flaccid paralysis of lower limb and impaired sensation of touch and warmth of knee and below. A computed tomography angiogram of lower limbs and thoracolumbar magnetic resonance imaging showed no abnormality. Based on the symptom, clinical diagnosis of anterior spinal artery syndrome was considered. The artery of Adamkiewicz is an important supplier to the anterior spinal artery. Internal thoracic mammary artery, used in coronary artery bypass grafting, is suspected as a collateral supplier of the artery of Adamkiewicz and has been accused for cause of spinal infarction.

  2. Anterior Spinal Artery Syndrome Following Coronary Artery Bypass Grafting: a Case Report

    PubMed Central

    Mirhosseini, Seyed Mohsen; Meghdadi, Soheil; Moghaddam, Ali Sanjari

    2017-01-01

    We present a patient with unstable angina candidate for coronary artery bypass grafting. Saphenous vein graft was used in obtuse marginal and left internal mammary artery to left anterior descending artery properly. After surgery, the patient experienced flaccid paralysis of lower limb and impaired sensation of touch and warmth of knee and below. A computed tomography angiogram of lower limbs and thoracolumbar magnetic resonance imaging showed no abnormality. Based on the symptom, clinical diagnosis of anterior spinal artery syndrome was considered. The artery of Adamkiewicz is an important supplier to the anterior spinal artery. Internal thoracic mammary artery, used in coronary artery bypass grafting, is suspected as a collateral supplier of the artery of Adamkiewicz and has been accused for cause of spinal infarction. PMID:28492795

  3. Posterior spinal artery syndrome showing marked swelling of the spinal cord: A clinico-pathological study

    PubMed Central

    Matsubayashi, Jun; Tsuchiya, Kuniaki; Shimizu, Soichiro; Kitagawa, Naoyuki; Wakabayashi, Yukari; Kuroda, Masahiko; Sakurai, Michio; Nagao, Toshitaka

    2013-01-01

    Objective To describe a rare autopsy case of posterior spinal artery syndrome with marked swelling of the spinal cord, an unusually subacute onset and short clinical course. Methods Case report. Findings An 84-year-old Japanese woman presented with bilateral muscle weakness of the lower legs and sensory disturbance 1 week after head contusion. Neurological findings worsened gradually. She developed phrenic nerve paralysis and died of respiratory failure 6 weeks after the onset of neurological symptoms. On pathological examination, the spinal cord was markedly swollen in the cervical and upper thoracic segments. Microscopically, there was loss of myelin sheath in the bilateral posterior columns and neuronal loss of the posterior horns in all of the spinal segments. However, findings were unremarkable in the bilateral anterior columns and bilateral anterior horns in most of the spinal segments. Posterior spinal arteries had no stenosis, occlusion, or thrombosis. We considered that pathogenesis was infarction associated with head injury. Conclusion To our knowledge, this is the first report of a case of posterior spinal artery syndrome with a markedly swollen spinal cord and poor prognosis. PMID:23433332

  4. The superior mesenteric artery syndrome in patients with spinal deformity.

    PubMed

    Altiok, Haluk; Lubicky, John P; DeWald, Christopher J; Herman, Jean E

    2005-10-01

    A retrospective review. To determine the incidence of the superior mesenteric artery syndrome (SMAS) after surgical correction for scoliosis and if it is influenced by newer derotation/translation surgical systems. The SMAS is a known complication after surgery. Of 2939 charts reviewed, 17 patients between 1960 and 2002 matched inclusion criteria. Our incidence of the SMAS was 0.5%. Onset of symptoms was 7.2 days. Several scoliosis diagnoses were included in the study group. Instrumentation that was used included: nondistraction systems (n = 14), Harrington rod with body cast (n = 1), Luque rod with sublaminar wires (n = 1), and casted in situ posterior spinal fusion (n = 1). Before surgery, 10 of 17 patients weighed less than the 50th percentile. Mean preoperative BMI was 18.6 kg/cm/cm. Postoperative height gain averaged 3.175 cm, and weight loss at onset of symptoms averaged 4.5 kg. There were 14 patients who required nasogastric suction for an average duration of 10.2 days, 11 required hyperalimentation, and 5 concurrently received hyperalimentation with enteric feeding. The SMAS recurred in 2 patients. Postoperative weight loss appears to be more important for the development of the SMAS than asthenic body type. Newer derotation/translation corrective techniques have not eliminated the SMAS. Gastrointestinal imaging is indicated when nausea and vomiting occur 6-12 days after surgery, associated with early satiety and normal bowel sounds. Decompression and nutritional support remain the mainstays of treatment.

  5. [Anterior spinal artery syndrome due to cervical spondylosis presenting as cervical angina].

    PubMed

    Odaka, Masaaki; Hirata, Koichi

    2004-11-01

    A 54-year-old woman developed acute progressive paraparesis after repeated precordial pain. Neurological examination revealed bilateral four-limb weakness predominant in the distal part of the upper limbs, upper limbs brisk tendon reflexes, superficial sensory impairment below the C8 level, and atonic bladder. T2-weighted cervical MRI disclosed hyperintense lesion with disc herniation in gray matter of spinal cord between C5 and C7. No vertebral artery abnormalities were detected. We hypothesized that she developed anterior spinal artery syndrome after cervical angina caused by cervical spondylosis. We conclude that physicians need to be aware of patients who experience chest pain without evidence of cardiac disease and that they take into consideration spinal cord infarction.

  6. Congestive Myelopathy due to Intradural Spinal AVM Supplied by Artery of Adamkiewicz: Case Report with Brief Literature Review and Analysis of the Foix-Alajouanine Syndrome Definition.

    PubMed

    Sood, Dinesh; Mistry, Kewal A; Khatri, Garvit D; Chadha, Veenal; Garg, Swati; Suthar, Pokhraj P; Patel, Dhruv G; Patel, Ankitkumar

    2015-01-01

    Spinal arteriovenous malformations (AVMs) can lead to development of congestive myelopathy (Foix-Alajouanine syndrome). Spinal AVMs are rare and so is this syndrome. Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome. We report a case of a 47-year-old male patient suffering from this rare syndrome with an AVM arising from the artery of Adamkiewicz, which is another rarity. Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms. We also present a brief review of literature on spinal AVMs and elucidate the evolution of the term Foix-Alajouanine syndrome. Use of the term "Foix-Alajouanine syndrome" should be restricted to patients with progressive subacute to chronic neurological symptoms due to congestive myelopathy caused by intradural spinal AVMs. CT angiography should supplement DSA as preliminary Imaging modality. Patients may be treated with surgery or endovascular procedures.

  7. Congestive Myelopathy due to Intradural Spinal AVM Supplied by Artery of Adamkiewicz: Case Report with Brief Literature Review and Analysis of the Foix-Alajouanine Syndrome Definition

    PubMed Central

    Sood, Dinesh; Mistry, Kewal A.; Khatri, Garvit D.; Chadha, Veenal; Garg, Swati; Suthar, Pokhraj P.; Patel, Dhruv G.; Patel, Ankitkumar

    2015-01-01

    Summary Background Spinal arteriovenous malformations (AVMs) can lead to development of congestive myelopathy (Foix-Alajouanine syndrome). Spinal AVMs are rare and so is this syndrome. Diagnosis is often missed due to its rarity and confusing definitions of the Foix-Alajouanine syndrome. Case Report We report a case of a 47-year-old male patient suffering from this rare syndrome with an AVM arising from the artery of Adamkiewicz, which is another rarity. Our patient was treated by embolization of the lesion with 20% glue, after which he showed mild improvement of symptoms. We also present a brief review of literature on spinal AVMs and elucidate the evolution of the term Foix-Alajouanine syndrome. Conclusions Use of the term “Foix-Alajouanine syndrome” should be restricted to patients with progressive subacute to chronic neurological symptoms due to congestive myelopathy caused by intradural spinal AVMs. CT angiography should supplement DSA as preliminary Imaging modality. Patients may be treated with surgery or endovascular procedures. PMID:26171088

  8. Superior mesenteric artery syndrome

    PubMed Central

    Giedrius, Bernotavičius; Kęstutis, Saniukas; Irena, Karmonaitė; Rimantas, Zagorskis

    2016-01-01

    Background. An obstruction of the distal part of the duodenum can occur because of the superior mesenteric artery syndrome (SMAS) after a surgical correction of scoliosis. It is essential to evaluate the risk factors and diagnose the SMAS in time because complications of this condition are life-threatening and it is associated with a high rate of morbidity. Diagnostics of the SMAS is challenging, because it is rare and its symptoms are non-specific. Therefore, in order to better understand the essence of this pathology and to make diagnosis easier we present a rare clinical case of the superior mesenteric artery syndrome after a surgical correction of neuromuscular scoliosis. The clinical case. A 12-year-old girl with a specific development disorder, sensory neuropathy and progressive kypho-scoliosis was admitted to Vilnius University Children’s Hospital. The patient had right side 50-degree thoracic scoliosis and an 80-degree thoracic kyphosis. She underwent posterior spinal fusion with hooks and screws from Th1 to L2. On the fourth day after the surgery the patient developed nausea and began to vomit each day 1-2 times per day, especially after meals. The SMAS was suspected and a nasogastric tube was inserted, stomach decompression and the correction of electrolytes disbalance were made. After the treatment, the symptoms did not recur and a satisfactory correction and balance of the spine were made in coronal and sagittal planes. Conclusions. It is extremely important to identify the risk factors of the SMAS and begin preoperative diet supplements before surgical correction of scoliosis for patients with a low body mass index. After the first episode of vomiting following the surgery, we recommend to investigate these patients for a gastrointestinal obstruction as soon as possible. Decompression of the stomach, enteral or parenteral nutrition, and fluid therapy are essential in treating the SMAS. PMID:28356803

  9. Currarino syndrome and spinal dysraphism.

    PubMed

    Kole, Matthew J; Fridley, Jared S; Jea, Andrew; Bollo, Robert J

    2014-06-01

    Currarino syndrome is a rare constellation of congenital anomalies characterized by the triad of sacral dysgenesis, presacral mass, and anorectal malformation. It is frequently associated with other congenital anomalies, often including occult spinal dysraphism. Mutations in the MNX1 gene are identified in the majority of cases. The authors report a rare case of Currarino syndrome in an infant with tethered cord syndrome and a dorsal lipomyelomeningocele continuous with a presacral intradural spinal lipoma, in addition to an imperforate anus and a scimitar sacrum. They review the literature to highlight patterns of occult spinal dysraphism in patients with Currarino syndrome and their relationship to tethered cord syndrome. Approximately 60% of the patients with Currarino syndrome reported in the literature have an occult spinal dysraphism. Published studies suggest that the risk of tethered cord syndrome may be higher among patients with a lipoma and lower among those with a teratoma or anterior meningocele.

  10. Spinal anomalies in Pfeiffer syndrome.

    PubMed

    Moore, M H; Lodge, M L; Clark, B E

    1995-05-01

    Review of the spinal radiographs of a consecutive series of 11 patients with Pfeiffer syndrome presenting to the Australian Craniofacial Unit was performed. The prevalence of cervical spine fusions was high, and the pattern of fusion complex. Isolated anomalies were evident at lower levels, including two cases of sacrococcygeal eversion. Spinal anomalies occur more frequently in the more severely involved cases of Pfeiffer syndrome emphasizing the generalized dysostotic nature of this condition.

  11. Tethered Spinal Cord Syndrome

    MedlinePlus

    ... Disparities Neural Interfaces Parkinson's Disease Spinal Cord Injury Stem Cells Traumatic Brain Injury Trans-Agency Activities Interagency Research ... Disparities Neural Interfaces Parkinson's Disease Spinal Cord Injury Stem Cells Traumatic Brain Injury Trans-Agency Activities Interagency Research ...

  12. Congestive myelopathy (Foix-Alajouanine Syndrome) due to intradural arteriovenous fistula of the filum terminale fed by anterior spinal artery: Case report and review of literature.

    PubMed

    Krishnan, Prasad; Banerjee, Tapas Kumar; Saha, Manash

    2013-07-01

    Spinal arteriovenous fistulas are rare entities. They often present with congestive myelopathy but are infrequently diagnosed as the cause of the patients' symptoms. Only one such case has been described previously in Indian literature. We describe one such case who presented to us after a gap of 3 years since symptom onset and following a failed laminectomy where the cause was later diagnosed to be an intradural fistula in the filum terminale fed by the anterior spinal artery and review the available literature.

  13. Spinal-cord syndrome due to non-compressive Paget's disease of bone: a spinal-artery steal phenomenon reversible with calcitonin.

    PubMed

    Herzberg, L; Bayliss, E

    1980-07-05

    A 76-year-old man had progressive low back pain, leg weakness, and sensory loss. Radiology showed changes consistent with wide-spread Paget's disease, but no cord compression or involvement of nerve roots was detected by myelography or computerised axial tomography. His symptoms were relieved within 12 days of starting 100 MRC units of subcutaneous salmon calcitonin and recurred when calcitonin was discontinued for 5 days. The improvement continued on calcitonin treatment for 1 year, with falls in serum alkaline phosphatase and urinary hydroxyproline excretion. It is suggested that calcitonin treatment, in reducing the abnormally high metabolic activity of the diseased bone, and hence its vascular perfusion, allows more blood to reach the spinal cord.

  14. Anterior spinal arteries. Origin and distribution in man.

    PubMed

    Rodríguez-Baeza, A; Muset-Lara, A; Rodríguez-Pazos, M; Domenech-Mateu, J M

    1989-01-01

    This study was designed to extend our present knowledge of the origin and distribution of anterior spinal arteries and was carried our using 31 human cadavers with arterial injections of natural latex. The conclusions reached are that there are always one or two anterior spinal arteries (right or left), which proceed from the intracranial segment of the vertebral arteries. These arteries have a descending course, with distribution into the ventral face of the medulla oblongata and the first cervical segments of the medulla spinalis. In cases where there are two anterior spinal arteries, they anastomose with each other to form the common, unpaired and median anterior spinal artery. The different observed locations of the origins of anterior spinal arteries may be systematised into three types. Each of these types has differential characteristics: bilateral origin (type I; 77.4%), unilateral origin (type II; 9.7%) and origin in an intervertebral transversal anastomosis (type III; 12.9%). We do however subdivide the bilateral origin group, according to the calibre of the arteries, into the subtypes 'balanced' (type Ia; 22.6%), 'right dominated' (type Ib; 32.2%) and 'left dominated' (type Ic; 22.6%). The collaterals of the anterior spinal arteries which distribute into the ventral face of the medulla oblongata are described.

  15. Periconal arterial anastomotic circle and posterior lumbosacral watershed zone of the spinal cord.

    PubMed

    Gailloud, Philippe; Gregg, Lydia; Galan, Peter; Becker, Daniel; Pardo, Carlos

    2015-11-01

    The existence of spinal cord watershed territories was suggested in the 1950s. Segmental infarcts within the junctional territories of adjacent radiculomedullary contributors and isolated spinal gray matter ischemia constitute two well-recognized types of watershed injury. This report describes the existence of another watershed territory related to the particular configuration of the spinal vasculature in the region of the conus medullaris. The anatomical bases underlying the concept of a posterior lumbosacral watershed zone are demonstrated with angiographic images obtained in a 16-year-old child. The clinical importance of this watershed zone is illustrated with MRI and angiographic data of three patients with a conus medullaris infarction. In all three cases of spinal ischemia an intersegmental artery providing a significant radiculomedullary contribution for the lower cord was compromised by a compressive mechanism responsible for decreased spinal cord perfusion (diaphragmatic crus syndrome in two cases, disk herniation in one). The ischemic injury, located at the junction of the anterior and posterior spinal artery territories along the dorsal aspect of the conus medullaris, was consistent with a watershed mechanism. This zone is at risk because of the caudocranial direction of flow within the most caudal segment of the posterior spinal arterial network which, from a functional standpoint, depends on the anterior spinal artery. The posterior thoracolumbar watershed zone of the spinal cord represents an area at increased risk of ischemic injury, particularly in the context of partial flow impairment related to arterial compression mechanisms. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  16. Anorgasmia in anterior spinal cord syndrome.

    PubMed

    Berić, A; Light, J K

    1993-05-01

    Three male and two female patients with anorgasmia and dissociated sensory loss due to an anterior spinal cord syndrome are described. Clinical, neurophysiological and quantitative sensory evaluation revealed preservation of the large fibre dorsal column functions from the lumbosacral segments with concomitant severe dysfunction or absence of the small fibre neospinothalamic mediated functions. These findings indicate a role for the spinothalamic system in orgasm.

  17. Intercostal Artery Reconstruction: The Simple and Effective Technique on Spinal Cord Protection during Thoracoabdominal Aortic Replacement.

    PubMed

    Zhang, Liang; Sun, Xiao-Gang; Yu, Cun-Tao; Chang, Qian; Qian, Xiang-Yang

    2016-07-01

    To retrospectively analyze the role of intercostal artery reconstruction in the spinal cord protection for patients undergoing extensive thoracoabdominal aortic aneurysm repair. From August 2007 to 2014, thoracoabdominal aortas (Crawford II) of 81 consecutive patients with mean age 39.4 ± 10.32 years were repaired. Seventy-three of these patients (90.12%) were diagnosed with aortic dissection in our group, 25 (30.86%) with Stanford type A dissection and 48 (59.26%) with Stanford B aortic dissection. All 25 patients with type A dissection have previously undergone surgical procedures which include Bentall's procedures in 11 cases, ascending aortic replacement in 6 cases, and total aortic arch replacement in 8 cases. All procedures were performed under profound hypothermia with interval cardiac arrest after making a thoracoabdominal incision. Extracorporeal circulation was instituted with 2 arterial cannulae and a single venous cannula in the right atrium. T6-T12 intercostal arteries and L1 and L2 lumbar arteries were formed to a neo-intercostal artery in place and were connected to an 8 mm branch for maintaining spinal cord blood perfusion. Visceral arteries were joined into a patch and anastomosed to the end of the main graft. The left renal artery was anastomosed to an 8 mm branch or joined to the patch. The other 10 mm branches were anastomosed to iliac arteries. With 100% follow-up, early mortality was 7.4%. Six deaths were recorded; 1 patient died of cerebral hemorrhage, 3 of renal failure, 1 of heart failure because of myocardial infarction, and the last one died from the rupture of celiac artery dissection. The rate of postoperative spinal cord deficits was 3.7%, 2 patients with paraplegia and 1 patient with paraparesis. None had bladder or rectum dysfunction. Neo-intercostal arteries were clogged in 12 patients within follow-up period and formed pseudoaneurysm in 2 patients with Marfan syndrome. The mean survival time in this group was 54.22 ± 3.03

  18. [Infarction of the spinal cord in the posterior spinal arterial supply area as a result of intervertebral disc embolism (author's transl)].

    PubMed

    Budka, H; Perneczky, A; Pusch, S

    1979-09-14

    A female patient aged 49 developed an acute transverse lesion of the spinal cord from D11 downwards. Autopsy revealed spinal cord infarcts mainly in the dorsal parts corresponding to the posterior spinal arterial supply area, caused by multiple arterial and venous fibrocartilaginous emboli. This particular cause of spinal vascular syndrome has been reported previously only in 11 patients, all outside Austria. This case report serves to stress the poor clinical delineation of a distinct "posterior spinal syndrome". The source of the emboli is the intervertebral disc, mainly the nucleus pulposus. The spinal cord vessels are probably entered by the following route: extrusion of disc material into the venous bone marrow sinus (probably favoured by trauma or endocrine factors) yield basivertebral veins yield internal vertebral venous plexus; the spread is supported by blood stream changes caused by increased intraabdominal/intrathoracic pressure; there is also the possibility of direct penetration of disc material into the internal vertebral plexus, as demonstrated in dogs yield radicular veins yield meningeal and cord veins; sometimes entry occurs through arteriovenous shunts into cord arteries. The true incidence of this condition is unknown since cases are likely to be overlooked in the absence of extensive histopathological investigation.

  19. Spinal accessory neuropathy, droopy shoulder, and thoracic outlet syndrome.

    PubMed

    Al-Shekhlee, Amer; Katirji, Bashar

    2003-09-01

    Droopy shoulder has been proposed as a cause of thoracic outlet syndrome. Two patients developed manifestations of neurovascular compression upon arm abduction, associated with unilateral droopy shoulder and trapezius muscle weakness caused by iatrogenic spinal accessory neuropathies following cervical lymph node biopsies. The first patient developed a cold, numb hand with complete axillary artery occlusion when his arm was abducted to 90 degrees. The second patient complained of paresthesias in digits 4 and 5 of the right hand, worsened by elevation of the arm, with nerve conduction findings of right lower trunk plexopathy (low ulnar and medial antebrachial cutaneous sensory nerve action potentials). Spinal accessory nerve grafting (in the first patient) coupled with shoulder strengthening physical exercises in both patients resulted in gradual improvement of symptoms in 2 years. These two cases demonstrate that unilateral droopy shoulder secondary to trapezius muscle weakness may cause compression of the thoracic outlet structures.

  20. Anomalous vertebral artery compression of the spinal cord at the cervicomedullary junction

    PubMed Central

    Ball, Bret Gene; Krueger, Bruce R; Piepgras, David G

    2011-01-01

    Background: Myelopathy from ectatic vertebral artery compression of the spinal cord at the cervicomedullary junction is a rare condition. Case Description: A 63-year-old female was originally diagnosed with occult hydrocephalus syndrome after presenting with symptoms of ataxia and urinary incontinence. Ventriculoperitoneal shunting induced an acute worsening of the patient′s symptoms as she immediately developed a sensory myelopathy. An MR scan demonstrated multiple congenital abnormalities including cervicomedullary stenosis with anomalous vertebral artery compression of the dorsal spinal cord at the cervicomedullary junction. The patient was taken to surgery for a suboccipital craniectomy, C1-2 laminectomy, vertebral artery decompression, duraplasty, and shunt ligation. Intraoperative findings confirmed preoperative radiography with ectactic vertebral arteries deforming the dorsal aspect of the spinal cord. There were no procedural complications and at a 6-month follow-up appointment, the patient had experienced a marked improvement in her preoperative signs and symptoms. Conclusion: Myelopathy from ectatic vertebral artery compression at the cervicomedullary junction is a rare disorder amenable to operative neurovascular decompression. PMID:21886876

  1. Anorgasmia in anterior spinal cord syndrome.

    PubMed Central

    Berić, A; Light, J K

    1993-01-01

    Three male and two female patients with anorgasmia and dissociated sensory loss due to an anterior spinal cord syndrome are described. Clinical, neurophysiological and quantitative sensory evaluation revealed preservation of the large fibre dorsal column functions from the lumbosacral segments with concomitant severe dysfunction or absence of the small fibre neospinothalamic mediated functions. These findings indicate a role for the spinothalamic system in orgasm. PMID:8505649

  2. Isolated spinal artery aneurysm: a rare culprit of subarachnoid haemorrhage.

    PubMed

    Sung, Tony H T; Leung, Warren K W; Lai, Bill M H; Khoo, Jennifer L S

    2015-04-01

    Isolated spinal artery aneurysm is a rare lesion which could be accountable for spontaneous spinal subarachnoid haemorrhage. We describe the case of a 74-year-old man presenting with sudden onset of chest pain radiating to the neck and back, with subsequent headache and confusion. Initial computed tomography aortogram revealed incidental finding of subtle acute spinal subarachnoid haemorrhage. A set of computed tomography scans of the brain showed further acute intracranial subarachnoid haemorrhage with posterior predominance, small amount of intraventricular haemorrhage, and absence of intracranial vascular lesions. Subsequent magnetic resonance imaging demonstrated a thrombosed intradural spinal aneurysm with surrounding sentinel clot, which was trapped and excised during surgical exploration. High level of clinical alertness is required in order not to miss this rare but detrimental entity. Its relevant aetiopathological features and implications for clinical management are discussed.

  3. Giant extramedullary arteriovenous fistula supplied by the anterior spinal artery: treatment by detachable balloons.

    PubMed

    Riché, M C; Scialfa, G; Gueguen, B; Merland, J J

    1983-01-01

    A case of a large extramedullary arteriovenous fistula in a young man, which was deemed inoperable, is described. The fistula was mainly supplied by the anterior spinal artery and was responsible for a progressive myeloradicular syndrome. Successful treatment with detachable balloons was carried out. Along with this case report, a review is made of the different types of extramedullary arteriovenous fistulas and their treatment based upon 11 cases seen at Lariboisière Hospital: type 1 is a plain small fistula where embolization is contraindicated; type 2 shows an enlarged anterior spinal artery with dysplastic vein and may be embolized with solid particles; type 3 is a very large multipediculated fistula where the best treatment seems to be detachable balloons. The indications for using detachable balloons for temporary occlusion and for definitive treatment are reviewed. Special emphasis is placed on the possibility of detaching balloons without the need for a coaxial catheter.

  4. Hemi-spinal cord infarction due to vertebral artery dissection in congenital afibrinogenemia.

    PubMed

    Laufs, H; Weidauer, S; Heller, C; Lorenz, M; Neumann-Haefelin, T

    2004-10-26

    Isolated ischemia of the cervical spinal cord is an uncommon but increasingly recognized complication of vertebral artery dissection (VAD). The authors report a young patient with congenital afibrinogenemia, who developed VAD with extensive unilateral spinal cord infarction, probably caused by local compression of spinal radicular feeders at their origin by vertebral artery hematoma.

  5. Coronary artery anomalies in Turner Syndrome.

    PubMed

    Viuff, Mette H; Trolle, Christian; Wen, Jan; Jensen, Jesper M; Nørgaard, Bjarne L; Gutmark, Ephraim J; Gutmark-Little, Iris; Mortensen, Kristian H; Gravholt, Claus Højbjerg; Andersen, Niels H

    Congenital heart disease, primarily involving the left-sided structures, is often seen in patients with Turner Syndrome. Moreover, a few case reports have indicated that coronary anomalies may be more prevalent in Turner Syndrome than in the normal population. We therefore set out to systematically investigate coronary arterial anatomy by computed tomographic coronary angiography (coronary CTA) in Turner Syndrome patients. Fifty consecutive women with Turner Syndrome (mean age 47 years [17-71]) underwent coronary CTA. Patients were compared with 25 gender-matched controls. Coronary anomaly was more frequent in patients with Turner Syndrome than in healthy controls [20% vs. 4% (p = 0.043)]. Nine out of ten abnormal cases had an anomalous left coronary artery anatomy (absent left main trunk, n = 7; circumflex artery originating from the right aortic sinus, n = 2). One case had a tubular origin of the right coronary artery above the aortic sinus. There was no correlation between the presence of coronary arterial anomalies and karyotype, bicuspid aortic valve, or other congenital heart defects. Coronary anomalies are highly prevalent in Turner Syndrome. The left coronary artery is predominantly affected, with an absent left main coronary artery being the most common anomaly. No hemodynamically relevant coronary anomalies were found. Copyright © 2016 Society of Cardiovascular Computed Tomography. All rights reserved.

  6. Genetics Home Reference: arterial tortuosity syndrome

    MedlinePlus

    ... result in massive loss of blood from the circulatory system. Blockage of blood flow to vital organs such ... adulthood. Features of arterial tortuosity syndrome outside the circulatory system are caused by abnormal connective tissue in other ...

  7. Arnold-Chiari malformation type 1 complicated by sudden onset anterior spinal artery thrombosis, tetraparesis and respiratory arrest.

    PubMed

    Wani, Abdul Majid; Zayyani, Najah R; Al Miamini, Wail; Khoujah, Amer M; Alharbi, Zeyad; Diari, Mohd S

    2011-04-15

    Chiari in 1891 described a constellation of anomalies at the base of the brain inherited congenitally, the characteristic of which are (1) extension of a tongue of cerebellar tissue posterior to the medulla and cord that extends into the cervical spinal canal; (2) caudal displacement of the medulla and the inferior part of the fourth ventricle into the cervical canal; and (3) a frequent but not invariable association with syringomyelia or a spinal developmental abnormality. Chiari recognized four types of abnormalities. Presently, the term has come to be restricted to Chiari's types I and II, that is, to cerebellomedullary descent without and with a meningomyelocele, respectively. The association of Arnold-Chairi malformation and high cervical cord infarction is unusual. The most common syndrome, anterior spinal artery syndrome (ASAS), is caused by interruption of blood flow to the anterior spinal artery, producing ischaemia in the anterior two-thirds of the cord, with resulting neurologic deficits. Causes of ASAS include aortic disease, postsurgical, sepsis, hypotension and thromboembolic disorders. The authors present an interesting case of cervical cord infarction due to anterior spinal artery thrombosis in a patient of type 1 Arnold-Chiari malformation without any of the above predisposing factors.

  8. Case misclassification in studies of spinal manipulation and arterial dissection.

    PubMed

    Cai, Xuemei; Razmara, Ali; Paulus, Jessica K; Switkowski, Karen; Fariborz, Pari J; Goryachev, Sergey D; D'Avolio, Leonard; Feldmann, Edward; Thaler, David E

    2014-09-01

    Spinal manipulation has been associated with cervical arterial dissection and stroke but a causal relationship has been questioned by population-based studies. Earlier studies identified cases using International Classification of Diseases Ninth Revision (ICD-9) codes specific to anatomic stroke location rather than stroke etiology. We hypothesize that case misclassification occurred in these previous studies and an underestimation of the strength of the association. We also predicted that case misclassification would differ by patient age. We identified cases in the Veterans Health Administration database using the same strategy as the prior studies. The electronic medical record was then screened for the word "dissection." The presence of atraumatic dissection was determined by medical record review by a neurologist. Of 3690 patients found by ICD-9 codes over a 30-month period, 414 (11.2%) had confirmed cervical artery dissection with a positive predictive value of 10.5% (95% confidence interval [CI] 9.6%-11.5%). The positive predictive value was higher in patients less than 45 years of age vs 45 years of age or older (41% vs 9%, P < .001). We reanalyzed a previous study, which reported no association between spinal manipulation and cervical artery dissection (odds ratio [OR] = 1.12, 95% CI .77-1.63) and recalculated an odds ratio of 2.15 (95% CI .98-4.69). For patients less than 45 years of age, the OR was 6.91 (95% CI 2.59-13.74). Prior studies grossly misclassified cases of cervical dissection and mistakenly dismissed a causal association with manipulation. Our study indicates that the OR for spinal manipulation exposure in cervical artery dissection is higher than previously reported. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  9. Goldenhar Syndrome Associated with Extensive Arterial Malformations

    PubMed Central

    Modica, Renee Frances; Barbeau, L. Daphna Yasova; Co-Vu, Jennifer; Beegle, Richard D.; Williams, Charles A.

    2015-01-01

    Goldenhar Syndrome is characterized by craniofacial, ocular and vertebral defects secondary to abnormal development of the 1st and 2nd branchial arches and vertebrae. Other findings include cardiac and vascular abnormalities. Though these associations are known, the specific anomalies are not well defined. We present a 7-month-old infant with intermittent respiratory distress that did not improve with respiratory interventions. Echocardiogram suggested a double aortic arch. Cardiac CT angiogram confirmed a right arch and aberrant, stenotic left subclavian artery, dilation of the main pulmonary artery, and agenesis of the left thyroid lobe. Repeat echocardiograms were concerning for severely dilated coronary arteries. Given dilation, a rheumatologic workup ensued, only identifying few weakly positive autoantibodies. Further imaging demonstrated narrowing of the aorta below the renal arteries and extending into the common iliac arteries and proximal femoral arteries. Given a physical exam devoid of rheumatologic findings, only weakly positive autoantibodies, normal inflammatory markers, and presence of the coronary artery dilation, the peripheral artery narrowings were not thought to be vasculitic. This case illustrates the need to identify definitive anomalies related to Goldenhar Syndrome. Although this infant's presentation is rare, recognition of specific vascular findings will help differentiate Goldenhar Syndrome from other disease processes. PMID:26688769

  10. Incidental occlusion of anterior spinal artery due to Onyx reflux in embolization of spinal type II arteriovenous malformation.

    PubMed

    Kim, Joohyun; Lee, Jang-Bo; Cho, Tai-Hyoung; Hur, Junseok W

    2017-05-01

    Onyx embolization is one of the standard treatments for brain arteriovenous malformations (AVMs) and is a promising method for spinal AVMs as well. Its advantages have been emphasized, and few complications have been reported with Onyx embolization in spinal AVMs. Here, we report an incidental anterior spinal artery (ASA) occlusion due to Onyx reflux during embolization of a spinal type II AVM. A 15-year-old boy presented with weakness in both upper and lower extremities. Magnetic resonance imaging and spinal angiogram revealed a spinal type II AVM with two feeders including the right vertebral artery (VA) and the right deep cervical artery. Onyx embolization was performed gradually from the VA to the deep cervical artery and an unexpected Onyx reflux to the ASA was observed during the latter stage deep cervical artery embolization. Post-operative quadriplegia and low cranial nerves (CN) dysfunction were observed. Rehabilitation treatment was performed and the patient showed marked improvement of neurologic deterioration at 1-year follow-up. Onyx is an effective treatment choice for spinal AVMs. However, due to the small vasculature of the spine compared to the brain, the nidus is rapidly packed with a small amount of Onyx, which allows Onyx reflux to unexpected vessels. Extreme caution is required and dual-lumen balloon catheter could be considered for Onyx embolization in spinal AVMs treatment.

  11. Posterior spinal artery aneurysm rupture after 'Ecstasy' abuse.

    PubMed

    Johnson, Jeremiah; Patel, Shnehal; Saraf-Lavi, Efrat; Aziz-Sultan, Mohammad Ali; Yavagal, Dileep R

    2015-07-01

    Posterior spinal artery (PSA) aneurysms are a rare cause of subarachnoid hemorrhage (SAH). The commonly abused street drug 3,4-methylenedioxymethamphetamine (MDMA) or 'Ecstasy' has been linked to both systemic and neurological complications. A teenager presented with neck stiffness, headaches and nausea after ingesting 'Ecstasy'. A brain CT was negative for SAH but a CT angiogram suggested cerebral vasculitis. A lumbar puncture showed SAH but a cerebral angiogram was negative. After a spinal MR angiogram identified abnormalities on the dorsal surface of the cervical spinal cord, a spinal angiogram demonstrated a left PSA 2 mm fusiform aneurysm. The patient underwent surgery and the aneurysmal portion of the PSA was excised without postoperative neurological sequelae. 'Ecstasy' can lead to neurovascular inflammation, intracranial hemorrhage, SAH and potentially even de novo aneurysm formation and subsequent rupture. PSA aneurysms may be treated by endovascular proximal vessel occlusion or open surgical excision. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.

  12. [Peripheral artery disease and acute coronary syndrome].

    PubMed

    Martínez-Quintana, Efrén; Rodríguez-González, Fayna

    2015-01-01

    Peripheral arterial disease is a common manifestation of systemic atherosclerosis that is associated with increased cardiovascular risk. When presented in the context of an acute coronary syndrome a differential diagnosis with aorta dissection should be made, because peripheral arterial disease may be asymptomatic despite the absence or asymmetry of femoral pulses. Copyright © 2014 Sociedad Española de Arteriosclerosis. Published by Elsevier España. All rights reserved.

  13. Anterior spinal and bulbar artery supply to the posterior inferior cerebellar artery revealed by a ruptured aneurysm: case report.

    PubMed

    Gabrieli, Joseph; Sourour, Nader-Antoine; Chauvet, Dorian; Di Maria, Federico; Chiras, Jacques; Clarençon, Frédéric

    2017-02-01

    The posterior inferior cerebellar artery (PICA) is a vessel located between the intra- and extracranial circulation. The artery is characterized by a complex embryological development and numerous anatomical variants. The authors present a case of the PICA supplied by both a hypertrophic anterior spinal artery and a hypoplastic bulbar artery. This unusual arrangement somehow completes the list of previously published variants, and the spontaneous rupture of a related aneurysm confirmed the fragility of this network. The authors discuss anatomical and treatment considerations.

  14. Superior Mesenteric Artery Syndrome with Abdominal Compartment Syndrome

    PubMed Central

    Reece, Kevin; Day, Rachel

    2016-01-01

    Superior Mesenteric Artery (SMA) syndrome is a condition in which the duodenum becomes compressed between the SMA and the aorta, resulting in bowel obstruction which subsequently compresses surrounding structures. Pressure on the inferior vena cava (IVC) and aorta decreases cardiac output which compromises distal blood flow, resulting in abdominal compartment syndrome with ischemia and renal failure. A 15-year-old male with SMA syndrome presented with 12 hours of pain, a distended, rigid abdomen, mottled skin below the waist, and decreased motor and sensory function in the lower extremities. Exploratory laparotomy revealed ischemic small bowel and stomach with abdominal compartment syndrome. Despite decompression, the patient arrested from hyperkalemia following reperfusion. PMID:28003918

  15. Reduced artery diameters in Klinefelter syndrome.

    PubMed

    Foresta, C; Caretta, N; Palego, P; Ferlin, A; Zuccarello, D; Lenzi, A; Selice, R

    2012-10-01

    Various epidemiological studies in relatively large cohorts of patients with Klinefelter syndrome (KS) described the increased morbidity and mortality in these subjects. Our aim was to study the structure and function of arteries in different districts to investigate in these subjects possible alterations. A total of 92 patients having non-mosaic KS, diagnosed in Centre for Human Reproduction Pathology at the University of Padova, and 50 age-matched healthy male controls were studied. Klinefelter syndrome subjects and controls evaluation included complete medical history, physical examination, measurement of concentrations of the reproductive hormones, lipidic and glycidic metabolism, AR function and sensitivity, ultrasound examinations (diameters, carotid intima-media thickness and brachial flow-mediated dilation) of brachial, common carotid and common femoral artery and abdominal aorta. Klinefelter syndrome patients showed significantly reduced artery diameters in all districts evaluated. On the contrary no statistically significant difference was found in cIMT and brachial FMD values between KS patients and controls. Furthermore, we found no statistically significant correlation of artery diameters with reproductive hormones, metabolic parameters, anthropometric measures and weighted CAG repeats. To our knowledge, this is the first study finding a reduced artery diameter in several districts in KS patients compared with that of normal male subjects and overlapping to that of female subjects. We have not an explanation for this phenomenon, even if a possible involvement of genes controlling the development of vascular system might be hypothesized, and further research is required to verify this hypothesis.

  16. Physical exercise improves arterial stiffness after spinal cord injury

    PubMed Central

    Hubli, Michèle; Currie, Katharine D.; West, Christopher R.; Gee, Cameron M.; Krassioukov, Andrei V.

    2014-01-01

    Objective/background Aortic pulse wave velocity (PWV), the gold-standard assessment of central arterial stiffness, has prognostic value for cardiovascular disease risk in able-bodied individuals. The aim of this study was to compare aortic PWV in athletes and non-athletes with spinal cord injury (SCI). Design Cross-sectional comparison. Methods Aortic PWV was assessed in 20 individuals with motor-complete, chronic SCI (C2–T5; 18 ± 8 years post-injury) using applanation tonometry at the carotid and femoral arterial sites. Ten elite hand-cyclists were matched for sex to 10 non-athletes; age and time since injury were comparable between the groups. Heart rate and discrete brachial blood pressure measurements were collected throughout testing. Outcome measures Aortic PWV, blood pressure, heart rate. Results Aortic PWV was significantly lower in athletes vs. non-athletes (6.9 ± 1.0 vs. 8.7 ± 2.5 m/second, P = 0.044). There were no significant between-group differences in resting supine mean arterial blood pressure (91 ± 19 vs. 81 ± 10 mmHg) and heart rate (60 ± 10 vs. 58 ± 6 b.p.m.). Conclusion Athletes with SCI exhibited improved central arterial stiffness compared to non-athletes, which is in agreement with the previous able-bodied literature. This finding implies that chronic exercise training may improve arterial health and potentially lower cardiovascular disease risk in the SCI population. PMID:24976366

  17. Aneurysms of medium-sized arteries in Marfan syndrome.

    PubMed

    Awais, Mazen; Williams, David M; Deeb, G Michael; Shea, Michael J

    2013-11-01

    Marfan syndrome is a relatively common connective tissue disorder that causes skin, ocular, skeletal, and cardiovascular abnormalities. High morbidity and mortality occur with aortic aneurysm and dissection. Other large-artery aneurysms, including carotid, subclavian, and iliac artery aneurysms, have also been associated with Marfan syndrome. It is not clear whether small- to medium-sized artery aneurysms are associated with Marfan syndrome. This report describes 4 patients with Marfan syndrome who have associated small- to medium-sized artery aneurysms with several complications. Additional investigations are needed to determine whether Marfan syndrome can cause small- to medium-sized artery aneurysms and how patients with these aneurysms should be treated.

  18. Bone cement implantation syndrome during spinal surgery requiring cardiac surgery.

    PubMed

    Sastre, José A; López, Teresa; Dalmau, María J; Cuello, Rafael E

    2013-12-15

    During a posterior segmental spinal fusion procedure, a 71-year-old woman developed cardiac and pulmonary embolism characterized by nonsustained ventricular tachycardia during cement injection, rapid and severe hypoxemia, and hemodynamic instability. Management included exploratory cardiotomy under cardiopulmonary bypass and removal of the emboli from the pulmonary vessels. Postoperative recovery was successful, and the patient was discharged without sequelae. We discuss the pathophysiology of bone cement implantation syndrome during spinal fusion, possible causative factors, and treatment alternatives.

  19. Family satisfaction following spinal fusion in Rett syndrome.

    PubMed

    Downs, Jenny; Torode, Ian; Ellaway, Carolyn; Jacoby, Peter; Bunting, Catherine; Wong, Kingsley; Christodoulou, John; Leonard, Helen

    2016-01-01

    We evaluated family satisfaction following spinal fusion in girls with Rett syndrome. Families participating in the population-based and longitudinal Australian Rett Syndrome Database whose daughter had undergone spinal fusion provided data on satisfaction overall, care processes and expected changes in health and function. Content analysis of responses to open-ended questions was conducted. Families reported high levels of overall satisfaction and consistently high ratings in relation to surgical and ICU care. Outstanding clinical care and the development of strong partnerships with clinical staff were much appreciated by families, whereas poor information exchange and inconsistent care caused concerns. Family satisfaction is an important outcome within a patient-centred quality of care framework. Our findings suggest strategies to inform the delivery of care in relation to spinal fusion for Rett syndrome and could also inform the hospital care of other children with disability and a high risk of hospitalization.

  20. Paraplegia Due to Spinal Cord Infarction After Coronary Artery Bypass Graft Surgery.

    PubMed

    Sevuk, Utkan; Kaya, Sedat; Ayaz, Firat; Aktas, Ulas

    2016-01-01

    Paraplegia is an extremely rare complication after coronary artery bypass grafting (CABG) and the underlying mechanisms remain poorly understood. We report a patient who developed paraplegia after CABG and review the literature on spinal cord ischemia following CABG surgery.

  1. Pseudoexfoliation syndrome and coronary artery ectasia.

    PubMed

    Akdemir, M O; Sayin, M R; Armut, M; Akpinar, I; Ugurbas, S H

    2014-05-01

    To determine whether pseudoexfoliation syndrome is associated with coronary artery ectasia or not. This cross-sectional study was undertaken at Bulent Ecevit University's Ophthalmology Department with the participation of the Cardiology Department. Eighty consecutive patients who underwent coronary angiography and were classified into two groups-those diagnosed with a normal coronary artery (n=40) and those diagnosed with coronary artery ectasia (n=40)-were included in the study. Pseudoexfoliation was diagnosed if typical exfoliation material was found to be present on the anterior surface of the lens, the iris, or at the pupillary border on slit-lamp examination. Age, sex, presence of pseudoexfoliation material, hypertension, diabetes mellitus, hyperlipidemia rates, cigarette smoking history, and family history of coronary artery disease were compared between the two groups. The continuous variables were compared by using the independent sample t-test or the Mann-Whitney U-test, and the categorical variables were compared using Pearson's χ(2)-test or Fisher's Exact χ(2)-test for the two groups. There was no difference in demographic data of both groups. The presence of pseudoexfoliation material was more common in patients with coronary ectasia compared with controls [(n=21 (52.5%) vs n=8 (20%), P=0.005)]. In all patients, there was a higher glaucoma rate in the patients with pseudoexfoliation syndrome [n=7 (24.1%), 6 CAE (+), 1 CAE (-)] than in the normal patients [n=2 (3.9%), 2 CAE (-)] (P=0.010). In this study, we found a possible association of pseudoexfoliation and coronary artery ectasia. Coronary artery ectasia patients had higher prevalence of pseudoexfoliation.

  2. [Conversion to clinically definite multiple sclerosis after isolated spinal cord syndrome: value of brain and spinal MRI].

    PubMed

    Pericot, I; Tintoré, M; Grivé, E; Briev, L; Rovira, A; Montalbán, X

    2001-02-17

    Isolated spinal cord syndrome might be due to a first episode of multiple sclerosis. The aim of the study was to determine the clinical usefulness and paraclinical characteristics and of spinal and brain MR imaging predicting conversion to clinically definite multiple sclerosis (CDMS) in patients with an isolated spinal cord syndrome. We have evaluate thirty-eight patients with isolated spinal cord syndrome. A clinical protocol, lumbar puncture, evoked potential and brain-spinal cord MRI were performed. Twenty two percent of the patients fulfilling brain MRI Paty criteria (p < 0.01), 54.5% Fazekas (p = 0.007) and 80% of patients fulfilling Barkhof criteria (p = 0.009) presented CDMS. The spinal MR imaging from CDMS patients was always abnormal, showing cervical and marginal location with a diameter < 2 cm. Brain MRI is strongly predictive of the risk of developing CDMS and spinal cord MRI may increase the sensitivity to detect conversion to CDMS.

  3. Microsurgical anatomy of the Adamkiewicz artery-anterior spinal artery junction.

    PubMed

    N'da, Hermann Adonis; Chenin, Louis; Capel, Cyril; Havet, Eric; Le Gars, Daniel; Peltier, Johann

    2016-07-01

    The aim of this study is to describe the anterior spinal artery-Adamkiewicz artery (ASA-AKA) junction and establish a classification allowing defining the neurological risk in either thoracoabdominal aorta aneurysm treatment and in anterior or transforaminal thoracolumbar spine surgery. Fifteen spinal cords of fresh cadavers were dissected. Both lumbar arteries and ASA were injected with strongly diluted red-colored silicon. The dural crossing of AKA was located on the left side in 86 % of cases, between T8 and T10 in 73.33 % of cases and L1-L2 in 26.67 % of cases. The average diameter of the ascending branch of AKA was 1.10 mm (range 0.8-1.9 mm), and its average length was 30.27 mm (range 12.3-60 mm). The AKA's arch average diameter was 11.3 mm (range 9-20 mm) with an open downward angle average of 20.1° (range 11°-30°). The descending branch of AKA which was a continuation of ASA had an average diameter of 1.33 mm (range 0.8-1.86 mm). The ASA at the top of the arch had an average diameter of 0.74 mm (range 0.2-1.77 mm). According to these findings, we have proposed a new classification with two types of junctions. The type I and its variant correlated to high neurological risk were present in 93.33 % of cases. The type II, correlated to medium or low neurological risk, was present in 6.67 % of cases. These anatomical findings allow a planning of the neurological risk before thoracoabdominal aorta aneurysm or thoracolumbar anterior or transforaminal spine surgery.

  4. Complications after spinal anesthesia in adult tethered cord syndrome

    PubMed Central

    Liu, Jing-Jie; Guan, Zheng; Gao, Zhen; Xiang, Li; Zhao, Feng; Huang, Sheng-Li

    2016-01-01

    Abstract Since little has been reported about complications of spinal anesthesia in adult tethered cord syndrome (TCS), we sought to delineate the characteristics of the condition. A total of 4 cases of adult TCS after spinal anesthesia were reviewed. The medical charts of the patients were obtained. Anesthesia, which was combined spinal and epidural anesthesia or spinal anesthesia was performed, and follow-up were carried out in all patients. The most common neurological symptom of adult TCS before surgery was occasional severe pain in back, perineal region, or legs. Frequent micturition, diminished knee and ankle reflexes, and difficulty in bending were exhibited in partial patients. Paraesthesia of perineal region or/and lower extremities existed 2 to 3 days after spinal anesthesia in all the cases. Weakness of lower extremities existed in 1 case. Lumbar magnetic resonance imaging showed the low location of conus medullaris. At follow-up, 3 cases recovered completely within 3 weeks, and 1 case underwent permanent disability. These cases suggest anesthesiologists and surgeons alert to the association of adult TCS and spinal anesthesia. Spinal anesthesia should be prohibited in patients with adult TCS to prevent neurological damages. PMID:27442670

  5. Wilkie's Syndrome or Superior Mesenteric Artery Syndrome: Fact or Fantasy?

    PubMed Central

    Zaraket, Vera; Deeb, Liliane

    2015-01-01

    Superior mesenteric artery (SMA) syndrome (known as Wilkie's syndrome) is a rare cause of upper gastrointestinal obstruction. It is an acquired disorder in which acute angulation of the SMA causes compression of the third part of the duodenum between the SMA and the aorta. This is commonly due to loss of fatty tissue as a result of a variety of debilitating conditions. We report a 17-year-old female who presented with intermittent abdominal pain and intractable vomiting following significant weight loss after hospitalization for pneumonia. Symptoms persisted for 2 years and the patient underwent extensive invasive and non-invasive tests, but to no avail. Thereafter she developed acute high intestinal obstruction, which unraveled her diagnosis. This case emphasizes the challenges in the diagnosis of SMA syndrome and the need for increased awareness of this entity. This will improve early recognition in order to reduce irrelevant tests and unnecessary treatments. PMID:26120301

  6. Spinal MRI Findings of Guillain-Barré Syndrome

    PubMed Central

    Alkan, Ozlem; Yildirim, Tulin; Tokmak, Naime; Tan, Meliha

    2009-01-01

    Guillain-Barré syndrome is a relatively common, acute, and rapidly progressive, inflammatory demyelinating polyneuropathy. The diagnosis is usually established on the basis of symptoms and signs, aided by cerebrospinal fluid findings and electrophysiologic criteria. Previously, radiologic examinations have been used only to rule out other spinal abnormalities. We report a case of systemic lupus erythematosus associated with Guillain-Barré syndrome with marked enhancement of nerve roots of the conus medullaris and cauda equina on MR imaging. These MR observations may help confirm the diagnosis of Guillain-Barré syndrome. PMID:22470650

  7. Horner's syndrome and headache due to carotid artery disease.

    PubMed Central

    West, T E; Davies, R J; Kelly, R E

    1976-01-01

    Horner's syndrome was accompanied by ipsilateral pain in the head, face, or neck in eight patients. In all cases angiography showed narrowing of the internal carotid artery in a pattern compatible with dissection within the arterial wall. In one patient in whom this diagnosis was substantiated by surgical exploration biopsy of the arterial wall showed changes typical of cystic medial necrosis. It is suggested that this clinical presentation constitutes a distinct syndrome. Images FIG 1 FIG 2 PMID:1260349

  8. Multiple spinal cavernous malformations in Klippel-Trenaunay-Weber syndrome.

    PubMed

    Göksu, Ethem; Alpsoy, Erkan; Uçar, Tanju; Tuncer, Recai

    2012-01-01

    Klippel-Trenaunay-Weber syndrome (KTWS) is a rare, congenital vascular disorder characterized by cutaneous haemangiomas, venous varicosities, and hypertrophy of the osseous and soft tissue. Various vascular anomalies of the central nervous system have been described in this syndrome. Two previous associations between KTWS and spinal cord cavernous malformations have been reported in the English literature. In this report, we present a patient in whom multiple cavernous malformations located in the conus medullaris region and cauda equina were associated with KTWS. General physical examination as well as neuroradiological and operative findings are described.

  9. Atypical presentation of popliteal artery entrapment syndrome: involvement of the anterior tibial artery.

    PubMed

    Bou, Steven; Day, Carly

    2014-11-01

    Popliteal artery entrapment syndrome (PAES) is a rare condition that should be suspected in a young patient with exertional lower extremity pain. We report the case of an 18-year-old female volleyball player with bilateral exertional lower extremity pain who had been previously diagnosed with tendinitis and periostitis. Diagnostic studies showed entrapment of the left popliteal artery and the left anterior tibial artery. To our knowledge, there has only been 1 previous report of anterior tibial artery involvement in PAES.

  10. Evidence for greater burden of peripheral arterial disease in lower extremity arteries of spinal cord-injured individuals.

    PubMed

    Bell, Jeffrey W; Chen, David; Bahls, Martin; Newcomer, Sean C

    2011-09-01

    Spinal cord injury leads to increased risk for cardiovascular disease and results in greater risk of death. Subclinical markers of atherosclerosis have been reported in carotid arteries of spinal cord-injured individuals (SCI), but the development of lower extremity peripheral arterial disease (PAD) has not been investigated in this population. The purpose of this study was to determine the effect of spinal cord injury on ankle-brachial index (ABI) and intima-media thickness (IMT) of upper-body and lower-extremity arteries. We hypothesized that the aforementioned measures of lower-extremity PAD would be worsened in SCI compared with controls and that regular participation in endurance exercise would improve these in both groups. To test these hypotheses, ABI and IMT were determined in 105 SCI and compared with 156 able-bodied controls with groups further subdivided into physically active and sedentary. ABIs were significantly lower in SCI versus controls (0.96 ± 0.12 vs. 1.06 ± 0.07, P < 0.001), indicating a greater burden of lower-extremity PAD. Upper-body IMTs were similar for brachial and carotid arteries in controls versus SCI. Lower extremity IMTs revealed similar thicknesses for both superficial femoral and popliteal arteries, but when normalized for artery diameter, individuals with SCI had greater IMT than controls in the superficial femoral (0.094 ± 0.03 vs. 0.073 ± 0.02 mm/mm lumen diameter, P < 0.01) and popliteal (0.117 ± 0.04 vs. 0.091 ± 0.02 mm/mm lumen diameter, P < 0.01) arteries. The ABI and normalized IMT of SCI compared with controls indicate that subclinical measures of lower-extremity PAD are worsened in individuals with SCI. These findings should prompt physicians to consider using the ABI as a screening method to detect lower-extremity PAD in SCI.

  11. Evidence for greater burden of peripheral arterial disease in lower extremity arteries of spinal cord-injured individuals

    PubMed Central

    Bell, Jeffrey W.; Chen, David; Bahls, Martin

    2011-01-01

    Spinal cord injury leads to increased risk for cardiovascular disease and results in greater risk of death. Subclinical markers of atherosclerosis have been reported in carotid arteries of spinal cord-injured individuals (SCI), but the development of lower extremity peripheral arterial disease (PAD) has not been investigated in this population. The purpose of this study was to determine the effect of spinal cord injury on ankle-brachial index (ABI) and intima-media thickness (IMT) of upper-body and lower-extremity arteries. We hypothesized that the aforementioned measures of lower-extremity PAD would be worsened in SCI compared with controls and that regular participation in endurance exercise would improve these in both groups. To test these hypotheses, ABI and IMT were determined in 105 SCI and compared with 156 able-bodied controls with groups further subdivided into physically active and sedentary. ABIs were significantly lower in SCI versus controls (0.96 ± 0.12 vs. 1.06 ± 0.07, P < 0.001), indicating a greater burden of lower-extremity PAD. Upper-body IMTs were similar for brachial and carotid arteries in controls versus SCI. Lower extremity IMTs revealed similar thicknesses for both superficial femoral and popliteal arteries, but when normalized for artery diameter, individuals with SCI had greater IMT than controls in the superficial femoral (0.094 ± 0.03 vs. 0.073 ± 0.02 mm/mm lumen diameter, P < 0.01) and popliteal (0.117 ± 0.04 vs. 0.091 ± 0.02 mm/mm lumen diameter, P < 0.01) arteries. The ABI and normalized IMT of SCI compared with controls indicate that subclinical measures of lower-extremity PAD are worsened in individuals with SCI. These findings should prompt physicians to consider using the ABI as a screening method to detect lower-extremity PAD in SCI. PMID:21742999

  12. Spinal cord injury increases the reactivity of rat tail artery to angiotensin II

    PubMed Central

    Al Dera, Hussain; Brock, James A.

    2015-01-01

    Studies in individuals with spinal cord injury (SCI) suggest the vasculature is hyperreactive to angiotensin II (Ang II). In the present study, the effects of SCI on the reactivity of the rat tail and mesenteric arteries to Ang II have been investigated. In addition, the effects of SCI on the facilitatory action of Ang II on nerve-evoked contractions of these vessels were determined. Isometric contractions of artery segments from T11 (tail artery) or T4 (mesenteric arteries) spinal cord-transected rats and sham-operated rats were compared 6–7 weeks postoperatively. In both tail and mesenteric arteries, SCI increased nerve-evoked contractions. In tail arteries, SCI also greatly increased Ang II-evoked contractions and the facilitatory effect of Ang II on nerve-evoked contractions. By contrast, SCI did not detectably change the responses of mesenteric arteries to Ang II. These findings provide the first direct evidence that SCI increases the reactivity of arterial vessels to Ang II. In addition, in tail artery, the findings indicate that Ang II may contribute to modifying their responses following SCI. PMID:25610365

  13. Unilateral posterior cervical spinal cord infarction due to spontaneous vertebral artery dissection.

    PubMed

    Richard, Sébastien; Abdallah, Chifaou; Chanson, Anne; Foscolo, Sylvain; Baillot, Pierre-Alexandre; Ducrocq, Xavier

    2014-03-01

    Ischemia of the cervical spinal cord is a rare complication of spontaneous vertebral artery dissection (VAD) and usually involves the ventral portion. We describe a less evocative clinical presentation and images of unilateral posterior spinal cord infarction due to spontaneous VAD in order to facilitate early diagnosis. A previously fit 30-year-old man presented with persistent headaches and proximal motor deficit of the right arm. He was diagnosed with spontaneous dissection of both vertebral arteries, with occlusion of the right one, and the right carotid artery. Neurological examination also revealed a right C2-C3 tactile sensory loss, with unilateral proprioceptive deficit below. Brain images revealed small bilateral cerebellar infarcts which could not be responsible for the clinical symptoms. Magnetic resonance imaging of the spinal cord showed a right posterior cervical spinal cord infarction. The patient achieved nearly complete recovery after several weeks of anticoagulation and rehabilitation. Infarction of the caudal portion of the cervical spinal cord, especially unilateral, caused by spontaneous VAD, has rarely been described and is certainly under-diagnosed due to less suggestive symptoms, like unilateral and mainly sensory deficit. Nevertheless, early diagnosis of this condition is important to guide patient management and rehabilitation.

  14. The thoracic anterior spinal cord adhesion syndrome

    PubMed Central

    Taylor, T R; Dineen, R; White, B; Jaspan, T

    2012-01-01

    Objectives This study included a series of middle-aged male and female patients who presented with chronic anterior hemicord dysfunction progressing to paraplegia. Imaging of anterior thoracic cord displacement by either a dural adhesion or a dural defect with associated cord herniation is presented. Methods This is a retrospective review of cases referred to a tertiary neuroscience centre over a 19-year period. Imaging series were classified by two experienced neuroradiologists against several criteria and correlated with clinical examination and/or findings at surgery. Results 16 cases were available for full review. Nine were considered to represent adhesions (four confirmed surgically) and four to represent true herniation (three confirmed surgically). In the three remaining cases the diagnosis was radiologically uncertain. Conclusion The authors propose “thoracic anterior spinal cord adhesion syndrome” as a novel term to describe this patient cohort and suggest appropriate clinicoradiological features for diagnosis. Several possible aetiologies are also suggested, with disc rupture and inflammation followed by disc resorption and dural pocket formation being a possible mechanism predisposing to herniation at the extreme end of a clinicopathological spectrum. PMID:22665931

  15. Sexual asphyxia causing blunt carotid artery injury and Horner's syndrome.

    PubMed

    Muddaiah, A; Banigo, A; Galli, F; Latif, M A

    2012-12-01

    To highlight a rare cause of Horner's syndrome, and to review the management of blunt carotid artery injury. Literature search via PubMed for related articles. Horner's syndrome and blunt carotid artery injury are rare phenomena; sexual asphyxia as a cause has not previously been reported. This case is also the first of its kind to have radiological evidence of injury to the external carotid artery but not the internal carotid artery. In Horner's syndrome, additional symptoms of ipsilateral headache or neck pain, tinnitus, or any cerebral ischaemic symptoms should raise suspicion of blunt carotid injury. Blunt carotid artery injury is a potentially fatal condition and can present without radiological evidence. Early recognition and management with anticoagulants or antiplatelet drugs is crucial to prevent mortality and morbidity.

  16. Coronary artery bypass and superior vena cava syndrome.

    PubMed Central

    Thomas, T V; Masrani, K; Thomas, J L

    1999-01-01

    Superior vena cava syndrome is the obstruction of the superior vena cava or its main tributaries by benign or malignant lesions. The syndrome causes edema and engorgement of the vessels on the face, neck, and arms, nonproductive cough, and dyspnea. We discuss the case of a 48-year-old obese diabetic woman who was admitted with unstable angina. She had previously been diagnosed with superior vena cava syndrome. Urgent coronary artery bypass grafting was necessary Although thousands of coronary artery bypasses are performed every year, there are not many reports on patients with superior vena cava syndrome who successfully undergo cardiopulmonary bypass and coronary artery grafting with an internal mammary artery as the conduit. The results of the case and alternative recommended methods are discussed. Images PMID:10653258

  17. Coronary artery bypass and superior vena cava syndrome.

    PubMed

    Thomas, T V; Masrani, K; Thomas, J L

    1999-01-01

    Superior vena cava syndrome is the obstruction of the superior vena cava or its main tributaries by benign or malignant lesions. The syndrome causes edema and engorgement of the vessels on the face, neck, and arms, nonproductive cough, and dyspnea. We discuss the case of a 48-year-old obese diabetic woman who was admitted with unstable angina. She had previously been diagnosed with superior vena cava syndrome. Urgent coronary artery bypass grafting was necessary Although thousands of coronary artery bypasses are performed every year, there are not many reports on patients with superior vena cava syndrome who successfully undergo cardiopulmonary bypass and coronary artery grafting with an internal mammary artery as the conduit. The results of the case and alternative recommended methods are discussed.

  18. A new diagnostic approach to popliteal artery entrapment syndrome

    SciTech Connect

    Williams, Charles; Kennedy, Dominic; Bastian-Jordan, Matthew; Hislop, Matthew; Cramp, Brendan; Dhupelia, Sanjay

    2015-09-15

    A new method of diagnosing and defining functional popliteal artery entrapment syndrome is described. By combining ultrasonography and magnetic resonance imaging techniques with dynamic plantarflexion of the ankle against resistance, functional entrapment can be demonstrated and the location of the arterial occlusion identified. This combination of imaging modalities will also define muscular anatomy for guiding intervention such as surgery or Botox injection.

  19. Aneurysmal dilatation of medium caliber arteries in Turner syndrome.

    PubMed

    Polkampally, Pritam R; Matta, Jatin R; McAreavey, Dorothea; Bakalov, Vladimir; Bondy, Carolyn A; Gharib, Ahmed M

    2011-01-01

    Turner syndrome is the most common chromosomal abnormality in female subjects, affecting 1 in 2000 live births. The condition is associated with a generalized vasculopathy as well as congenital cardiac and other defects. We report aneurysmal dilation of medium caliber arteries involving the celiac axis and coronary vessels in two women with Turner syndrome.

  20. Superior Mesenteric Artery Syndrome Treated with Percutaneous Radiologic Gastrojejunostomy.

    PubMed

    Choi, Jeong Woo; Lee, Ju Young; Cho, Hyeon Geun

    2016-06-25

    Superior mesenteric artery (SMA) syndrome is a rare condition that must be differentiated from other gastrointestinal diseases manifesting as upper abdominal pain, nausea, or vomiting. The description of SMA syndrome is compression of the third portion of the duodenum by the SMA and the abdominal aorta. SMA syndrome is managed with nasoenteral nutrition or surgical strategies such as laparoscopic duodenojejunostomy. However, SMA syndrome treated using enteral nutrition by percuta-neous radiologic gastrojejunostomy has not been reported. Here, we report our experience of successfully managing a case of SMA syndrome with percutaneous radiologic gastrojejunostomy.

  1. Pulse wave velocity for assessment of arterial stiffness among people with spinal cord injury: a pilot study.

    PubMed

    Miyatani, Masae; Masani, Kei; Oh, Paul I; Miyachi, Motohiko; Popovic, Milos R; Craven, B Cathy

    2009-01-01

    The most significant complication and leading cause of death for people with spinal cord injury (SCI) is coronary artery disease (CAD). It has been confirmed that aortic pulse wave velocity (PWV) is an emerging CAD predictor among able-bodied individuals. No prior study has described PWV values among people with SCI. The objective of this study was to compare aortic (the common carotid to femoral artery) PWV, arm (the brachial to radial artery) PWV, and leg (the femoral to posterior tibial artery) PWV in people with SCI (SCI group) to able-bodied controls (non-SCI group). Participants included 12 men with SCI and 9 non-SCI controls matched for age, sex, height, and weight. Participants with a history of CAD or current metabolic syndrome were excluded. Aortic, arm, and leg PWV was measured using the echo Doppler method. Aortic PWV (mean +/- SD) in the SCI group (1,274 +/- 369 cm/s) was significantly higher (P < 0.05) than in the non-SCI group (948 +/- 110 cm/s). There were no significant between-group differences in mean arm PWV (SCI: 1,152 +/- 193 cm/s, non-SCI: 1,237 +/- 193 cm/s) or mean leg PWV (SCI: 1,096 +/- 173 cm/s, non-SCI: 994 +/- 178 cm/s) values. Aortic PWV was higher among the SCI group compared with the non-SCI group. The higher mean aortic PWV values among the SCI group compared with the non-SCI group indicated a higher risk of CAD among people with SCI in the absence of metabolic syndrome.

  2. The association of breast arterial calcification and metabolic syndrome

    PubMed Central

    Yildiz, Seyma; Toprak, Huseyin; Aydin, Sinem; Bilgin, Mehmet; Oktay, Veysel; Abaci, Okay; Kocas, Cuneyt

    2014-01-01

    OBJECTIVES: We investigated the relationship between metabolic syndrome and breast arterial calcification detected via mammography in a cohort of postmenopausal subjects. METHODS: Among 837 patients referred to our radiology department for mammographic screening, 310 postmenopausal females (105 patients with and 205 patients without breast arterial calcification) aged 40 to 73 (mean 55.9±8.4) years were included in this study. The groups were compared with respect to clinical characteristics and metabolic syndrome criteria. Univariate and multivariate analyses identified the factors related to breast arterial calcification. RESULTS: Age, postmenopausal duration and the frequencies of diabetes mellitus, hypertension and metabolic syndrome were significantly higher in the subjects with breast arterial calcification than in those without (p<0.05). Multivariate analysis indicated that age (OR = 1.3, 95% CI = 1.1–1.6, p = 0.001) and metabolic syndrome (OR = 4.0, 95% CI = 1.5−10.4, p = 0.005) were independent predictors of breast arterial calcification detected via mammography. The independent predictors among the features of metabolic syndrome were low levels of high-density lipoproteins (OR = 8.1, 95% CI = 1.0−64.0, p = 0.047) and high blood pressure (OR = 8.7, 95% CI = 1.5−49.7, p = 0.014). CONCLUSIONS: The likelihood of mammographic detection of breast arterial calcification increases with age and in the presence of hypertension or metabolic syndrome. For patients undergoing screening mammography who present with breast arterial calcification, the possibility of metabolic syndrome should be considered. These patients should be informed of their cardiovascular risk factors and counseled on appropriate lifestyle changes. PMID:25627997

  3. [Myelomalacia caused by embolism of the substantia gelatinosa in the vertebral column and spinal cord arteries].

    PubMed

    Barz, H; Majerowitsch, B

    1986-01-01

    A 56 year old woman developed a transverse lumbar paraplegia within two hours following a jump from an 80 centimeter high window. The patient died 13 days later from a pulmonary embolus. At autopsy an intervertebral disc tissue embolus was found in the arteries to the 12th thoracic vertebral body and the spinal cord. The embolus originated in a Schmorl nodule in the 12th thoracic vertebral body. The findings and the clinical course suggest that dislodged nucleus pulposus tissue was expressed into the vascular system over some time as a result of tears in small vessels. The implications for treatment of patients with similar spinal cord symptoms are considered.

  4. Superior mesenteric artery syndrome in children and adolescents with spine deformities undergoing corrective surgery.

    PubMed

    Tsirikos, Athanasios I; Jeans, Lindsay A

    2005-06-01

    Obstruction of the third part of the duodenum by the superior mesenteric artery (SMA) is associated with spinal manipulation in the surgical or conservative management of scoliosis. The purpose of the present study was to investigate the prevalence of SMA syndrome in a cohort of 165 consecutive pediatric patients who underwent spine deformity surgery and had minimum 2-year follow-up. The study group comprised 85 patients with idiopathic scoliosis, 20 patients with neuromuscular and 18 patients with miscellaneous or syndromic scoliosis, and 42 patients with congenital spine deformities. Posterior spine arthrodesis was performed in 94 patients, combined anteroposterior in 60 patients, and anterior spinal fusion in 11 patients. We identified four patients who developed SMA syndrome postoperatively. These were all markedly underweight adolescent girls; two patients had adolescent idiopathic scoliosis, one had neuromuscular scoliosis, and one congenital scoliosis. Third-generation instrumentation systems with derotational effect were used in three patients. The spine arthrodesis in the patient with neuromuscular scoliosis was performed using bone graft followed by application of a spinal jacket. The symptoms developed at a mean of 3.7 days post surgery and included nausea, vomiting, increased nasogastric aspirates, and abdominal pain and distension. Conservative management with prolonged nasojejunal feedings achieved resolution of the symptoms in all but one patient, who required derotation of the duodenum and jejunum. There was no evidence of recurrence of the condition in any patient. The prevalence of SMA syndrome in our series was 2.4%. This work draws attention to the significance of the prevention of the condition by recognizing patients who are at a higher risk. An early diagnosis of SMA syndrome will allow for application of conservative methods and will increase the chances for a successful outcome.

  5. Transcatheter Splenic Artery Occlusion for Treatment of Splenic Artery Steal Syndrome After Orthotopic Liver Transplantation

    SciTech Connect

    Uflacker, Renan; Selby, J. Bayne; Chavin, Kenneth; Rogers, Jeffrey; Baliga, Prabhakar

    2002-08-15

    Purpose: To review some aspects of the problem of splenic artery steal syndrome as cause of ischemia in transplanted livers and treatment by selective splenic artery occlusion. Materials and Methods: Eleven liver transplant patients from a group of 350 patients, nine men and two women,ranging in age from 40 years to 61 years (mean 52 years), presented with biochemical evidences of liver ischemia and failure, ranging from one to 60 days following orthotopic liver transplantation. Diagnosis of splenic artery steal syndrome was suspected by elevated enzymes, Doppler ultrasound and confirmed by celiac angiogram. Patients with confirmed hepatic artery thrombosis before angiography were excluded from the study. Embolization with Gianturco coils was performed. Results: All patients were treated by splenic artery embolization with Gianturco coils. The 11 patients improved clinically within 24 hours of the procedure with significant change in the biochemical and clinical parameters. Followup ranged from one month to two years. One of the 11 patient initially improved, but developed hepatic artery thrombosis within 24 hours of the embolic treatment,requiring surgical repair. Conclusion: Splenicartery steal syndrome following liver transplantation surgery can be diagnosed by celiac angiography, and effectively treated by splenic artery embolization with coils. Embolization is one of the treatments available, it is minimally invasive, and leads to immediate clinical improvement. Hepatic artery thrombosis is a possible complication of the procedure.

  6. The arterial supply of the human spinal cord: a new approach to the arteria radicularis magna of Adamkiewicz.

    PubMed

    Rodriguez-Baeza, A; Muset-Lara, A; Rodriguez-Pazos, M; Domenech-Mateu, J M

    1991-01-01

    The arteria radicularis magna (Adamkiewicz's artery) was studied in 30 human spinal cords after arterial injection. The artery was present in all cases, between T8 and L2, and was identified by its diameter and position. The arteria radicularis magna was the main blood supply to the lowest region of the spinal cord. In one out of three cases it accompanied the ventral root at T9, and in 80% of the cases studied it was found on the left side. The arteria radicularis magna had a posterior component in 63% of the cases. We did not observe specific radiculo-medullary arteries in the conus medullaris region.

  7. Reversible cerebral vasoconstriction syndrome with concurrent bilateral carotid artery dissection.

    PubMed

    Bayer-Karpinska, Anna; Patzig, Maximilian; Adamczyk, Christopher; Dimitriadis, Konstantinos; Wollenweber, Frank A; Dichgans, Martin; Jahn, Klaus; Opherk, Christian

    2013-05-01

    The pathophysiological basis of reversible cerebral vasoconstriction syndrome is poorly understood but carotid artery dissection has been discussed as a rare possible cause. So far, only single cases of unilateral carotid artery dissection and reversible cerebral vasoconstriction syndrome have been reported. Here, we describe the case of a 54-year old patient presenting to the emergency department with right hemiparesis, hypaesthesia and dysarthria. Furthermore, he reported two episodes of thunderclap headache after autosexual activity. Cerebral imaging showed ischaemic infarcts, slight cortical subarachnoid haemorrhage, bilateral carotid artery dissection and fluctuating intracranial vessel irregularities, compatible with reversible cerebral vasoconstriction syndrome. An extensive diagnostic work-up was normal. No typical trigger factors of reversible cerebral vasoconstriction syndrome could be found. The patient received intravenous heparin and the calcium channel blocker nimodipine. Follow-up imaging revealed no vessel irregularities, the left internal carotid artery was still occluded. This case supports the assumption that carotid artery dissection should be considered as a potential trigger of reversible cerebral vasoconstriction syndrome, possibly by altering sympathetic vascular tone.

  8. Spastic quadriparesis caused by anomalous vertebral artery compression of spinal cord at the cervico-medullary junction.

    PubMed

    Betgeri, Somsharan Shankerappa; Rajesh, S; Adkatalwar, Vijayendra; Shiva, Meyyappan; Agrawal, Nitesh; Ramakrishnan, K G

    2015-02-01

    Vascular compression of medulla or spinal cord at the cervico-medullary junction has been commonly described in the literature and is often attributed to dolichoectasia of the vertebrobasilar arteries. We describe a case of anomalous course of the cervical segments of the bilateral vertebral arteries which were seen entering the spinal canal directly after exiting the transverse foramen of axis and causing significant cord compression at the cervico-medullary region leading to spastic quadriparesis.

  9. Concomitant origin of the anterior or posterior spinal artery with the feeder of a spinal dural arteriovenous fistula (SDAVF).

    PubMed

    Adrianto, Yudhi; Yang, Ku Hyun; Koo, Hae-Won; Park, Wonhyoung; Jung, Sung Chul; Park, Jie Eun; Kim, Kwang-Kuk; Jeon, Sang Ryong; Suh, Dae Chul

    2017-04-01

    The concomitant origin of the anterior spinal artery (ASA) or the posterior spinal artery (PSA) from the feeder of a spinal dural arteriovenous fistula (SDAVF) is rare and the exact incidence is not known. We present our experience with the management of SDAVFs in such cases. In 63 patients with SDAVF between 1993 and 2015, the feeder origin of the SDAVF was evaluated to determine whether it was concomitant with the origin of the ASA or PSA. Embolization was attempted when the patient did not want open surgery and an endovascular approach was regarded as safe and possible. The outcome of the procedure was evaluated as complete, partial, or no obliteration. The clinical outcome was evaluated by Aminoff-Logue (ALS) gait and micturition scale scores. Nine patients (14%) had a concomitant origin of the ASA or PSA with the feeder. There were two cervical, five thoracic, and two lumbar level SDAVFs. A concomitant origin of the feeder was identified with the ASA (n=7) and PSA (n=2). Embolization was performed in four patients and open surgery was performed in five. Embolization resulted in complete obliteration in three patients and partial obliteration in one. Using the ALS gait and micturition scale, the final outcome improved in six while three cases remained in an unchanged condition over 2-148 months. The concomitant origin of the ASA or PSA with the feeder occurs occasionally. Complete obliteration of the fistula can be achieved either by embolization or open surgery. Embolization can be carefully performed in selected patients who are in a poor condition and do not want to undergo open surgery. Published by the BMJ Publishing Group Limited. For permission to use (where not already granted under a licence) please go to http://www.bmj.com/company/products-services/rights-and-licensing/.

  10. Acute cervical cord infarction in anterior spinal artery territory with acute swelling mimicking myelitis.

    PubMed

    Abou Al-Shaar, Hussam; AbouAl-Shaar, Iyad; Al-Kawi, Mohammed Z

    2015-10-01

    Acute infarction of the cervical segment of the spinal cord is extremely uncommon. Patients may present with signs and symptoms mimicking that of acute myelitis. On imaging, both conditions may present as a hyperintense area on T-2 weighted MRI. History of sudden onset is essential in establishing the diagnosis. We report a case of cervical spinal cord infarction in a 40-year-old man who was diagnosed with acute transverse myelitis, and was treated with high dose intravenous corticosteroids followed by 5 sessions of plasma exchange. An MRI of the spine revealed abnormal high T2 signal intensity extending from the C2 to C7 level involving the anterior two-thirds of the cord with more central involvement. The findings were consistent with anterior spinal artery territory cervical cord infarction.

  11. Acute cervical cord infarction in anterior spinal artery territory with acute swelling mimicking myelitis

    PubMed Central

    Al-Shaar, Hussam Abou; AbouAl-Shaar, Iyad; Al-Kawi, Mohammed Z.

    2015-01-01

    Acute infarction of the cervical segment of the spinal cord is extremely uncommon. Patients may present with signs and symptoms mimicking that of acute myelitis. On imaging, both conditions may present as a hyperintense area on T-2 weighted MRI. History of sudden onset is essential in establishing the diagnosis. We report a case of cervical spinal cord infarction in a 40-year-old man who was diagnosed with acute transverse myelitis, and was treated with high dose intravenous corticosteroids followed by 5 sessions of plasma exchange. An MRI of the spine revealed abnormal high T2 signal intensity extending from the C2 to C7 level involving the anterior two-thirds of the cord with more central involvement. The findings were consistent with anterior spinal artery territory cervical cord infarction. PMID:26492118

  12. Spinal Anesthesia Facilitates the Early Recognition of TUR Syndrome

    PubMed Central

    McGowan-Smyth, Sam; Vasdev, Nikhil; Gowrie-Mohan, Shan

    2016-01-01

    Objectives To investigate what clinical features typically present in transurethral resection (TUR) syndrome and to see which classically present first. The purpose of the study was to establish whether or not a particular method of anesthesia is preferred in detecting this syndrome in its early stages. Methods A total of 1,502 transurethral resection of the prostate (TURP) over a 15 year period were reviewed to see which, if any, went on to experience this complication. Of these cases, 48 developed TUR syndrome. The case records were reviewed retrospectively and the presenting clinical features were analysed. All TURPs were routinely performed under spinal anesthesia and followed a standardised set up. The irrigation fluid used in all operations was Glycine 1.5%. Results Forty eight patients displayed clinical features of TUR syndrome giving an incidence of 3.2%. Trainees of varying experience caused all but one case. Median resection time, resection weight and volume of intraoperative glycine irrigation fluid were 55 minutes (range 40-75 minutes), 44 grams (range 24-65 g), and 28 l (24-48 l) respectively. Only 16/48 TURPs had a recorded capsular perforation. Pre- vs. post-operative median hematocrit, hemoglobin and serum sodium were 0.42 vs. 0.33, 14.2 g/dl vs. 10.1 g/dl and 142 mmol/l vs. 121 mmol/l respectively. Patients presented with nausea 44/48, vomiting 28/48, visual disturbance 29/48, apprehension 37/48, disorientation 17/48, breathing difficulties 17/48, and bradycardia 19/21. The earliest observed sign was nausea 21/48, then bradycardia 11/48, apprehension 11/48, and visual disturbance 10/48; after which the procedure was abandoned. None of the patients developed stupor, coma or seizures. Out of the 48 patients, 9 were admitted to high dependency units and all of these were treated with IV furosemide. One patient required a blood transfusion. All patients recovered within 48 hours (range 18-48 hours) and none had any long term complications on follow up

  13. [Spinal cord ischemia].

    PubMed

    Masson, C; Leys, D; Meder, J F; Dousset, V; Pruvo, J P

    2004-01-01

    Traditional data and recent advances in the field of spinal cord ischemia are reviewed, with special attention to clinical and radiological features, as well as underlying etiology, outcome, and pathophysiology. Acute spinal cord ischemia includes arterial and venous infarction and global ischemia resulting from cardiac arrest or severe hypotension. MRI has become the technique of choice for the imaging diagnosis of spinal cord infarction. Correlation of clinical and MRI data has allowed diagnosis of clinical syndromes due to small infarcts in the central or peripheral arterial territory of the spinal cord. Diffusion-weighted MR imaging may increase the sensitivity and specificity for diagnosis of acute spinal cord infarction. Diagnosis of venous spinal cord infarction remains difficult. As for global ischemia, neuropathological studies demonstrated a great sensitivity of spinal cord to ischemia, with selective vulnerability of lumbosacral neurons. Chronic spinal cord ischemia results in a syndrome of progressive myelopathy. The cause is usually an arteriovenous malformation. Most often, diagnosis may be suspected on MRI, leading to diagnostic, and eventually therapeutic, spinal angiography.

  14. Multiple Coronary Artery Microfistulas in a Girl with Kleefstra Syndrome

    PubMed Central

    Vargiami, Euthymia; Ververi, Athina; Al-Mutawa, Hamda; Gioula, Georgia; Gerou, Spyridon; Rouvalis, Fotios; Kambouris, Marios; Zafeiriou, Dimitrios I.

    2016-01-01

    Kleefstra syndrome is characterized by hypotonia, developmental delay, dysmorphic features, congenital heart defects, and so forth. It is caused by 9q34.3 microdeletions or EHMT1 mutations. Herein a 20-month-old girl with Kleefstra syndrome, due to a de novo subterminal deletion, is described. She exhibits a rare and complex cardiopathy, encompassing multiple coronary artery microfistulas, VSD/ASD, and PFO. PMID:27239352

  15. Posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI): A case report.

    PubMed

    Khokhar, Harsh Vardhan; Choudhary, Pradeep; Saxena, Sangeeta; Arif, Mohamed

    2016-01-01

    Posterior reversible encephalopathy syndrome with spinal cord involvement (PRES-SCI) is a recently described entity with a handful of cases reported in literature. We describe a case of PRES in setting of Henoch-Schönlein purpura (HSP) with involvement of brain stem and spinal cord.

  16. Successful nutritional therapy for superior mesenteric artery syndrome.

    PubMed

    Chan, Dedrick Kok Hong; Mak, Kenneth Seck Wai; Cheah, Yee Lee

    2012-11-01

    Superior mesenteric artery (SMA) syndrome is an uncommon cause of duodenal outlet obstruction. Symptoms and signs suggestive of this condition are nonspecific, and a high index of suspicion coupled with appropriate imaging studies are necessary for diagnosis. We present the case of a 70-year-old man who developed SMA syndrome following prolonged hospitalisation for a surgically treated bleeding duodenal ulcer. His SMA syndrome resolved after successful nonoperative management based on accepted guidelines for nutritional therapy, thus avoiding the need for reoperation and its attendant risks in a malnourished patient.

  17. Dilated Basilar Arteries in Patients with Congenital Central Hypoventilation Syndrome

    PubMed Central

    Kumar, Rajesh; Nguyen, Haidang D.; Macey, Paul M.; Woo, Mary A.; Harper, Ronald M.

    2009-01-01

    Congenital central hypoventilation syndrome (CCHS) patients show hypoventilation during sleep and severe autonomic impairments, including aberrant cardiovascular regulation. Abnormal sympathetic patterns, together with increased and variable CO2 levels, lead to the potential for sustained cerebral vasculature changes. We performed high-resolution T1-weighted imaging in 13 CCHS and 31 control subjects using a 3.0-Tesla magnetic resonance imaging scanner, and evaluated resting basilar and bilateral middle cerebral artery cross-sections. Two T1-weighted image series were acquired; images were averaged and reoriented to common space, and regions containing basilar and both middle cerebral arteries were oversampled. Cross-sections of the basilar and middle cerebral arteries were manually outlined to calculate cross-sectional areas, and differences between and within groups were evaluated. Basilar arteries in CCHS were significantly dilated over control subjects, but both middle cerebral artery cross-sections were similar between groups. No significant differences appeared between left and right middle cerebral arteries within either group. Basilar artery dilation may result from differential sensitivity to high CO2 over other vascular beds, damage to serotonergic or other chemosensitive cells accompanying the artery, or enhanced microvascular resistance, and that dilation may impair tissue perfusion, leading to further neural injury in CCHS. PMID:19822189

  18. Pulmonary Arterial Hypertension: The Clinical Syndrome

    PubMed Central

    Lai, Yen-Chun; Potoka, Karin C.; Champion, Hunter C.; Mora, Ana L.; Gladwin, Mark T.

    2014-01-01

    Pulmonary arterial hypertension (PAH) is a progressive disorder in which endothelial dysfunction and vascular remodeling obstruct small pulmonary arteries, resulting in increased pulmonary vascular resistance and pulmonary pressures. This leads to reduced cardiac output, right heart failure, and ultimately death. In this review, we attempt to answer some important questions commonly asked by patients diagnosed with PAH pertaining to the disease, and aim to provide an explanation in terms of classification, diagnosis, pathophysiology, genetic etiologies, demographics, and prognostic factors. Furthermore, important molecular pathways that are central to the pathogenesis of PAH are reviewed, including nitric oxide, prostacyclin, endothelin-1, reactive oxygen species, and endothelial and smooth muscle proliferation. PMID:24951762

  19. [Prophylaxis and treatment of arterial hypotension during caesarean with spinal anaesthesia].

    PubMed

    Arias, J; Lacassie, H J

    2013-11-01

    Caesarean section is one of the most common surgical procedures worldwide. Arterial hypotension is the most prevalent adverse effect after spinal anaesthesia. Various methods have been used to prevent or treat hypotension. Since there is no treatment 100% effective by itself, a multimodal management is required to achieve an optimum balance and avoidance of hemodynamic imbalance. Strategies to avoid this side effect are analyzed on the basis of the best evidence available so far, summarized as mechanical factors, anesthetics, fluids and vasopressors. After spinal anaesthesia for caesarean section, the best strategy available for prevention of hypotension appears to be the combination of crystalloids along with an alpha 1 agonist vasopressor. Copyright © 2012 Sociedad Española de Anestesiología, Reanimación y Terapéutica del Dolor. Published by Elsevier España. All rights reserved.

  20. Posterior spinal artery infarct due to patent foramen ovale: a case report.

    PubMed

    Petruzzellis, Marco; Fraddosio, Angela; Giorelli, Maurizio; Prontera, Mariapia; Tinelli, Angelica; Lucivero, Vincenzo; Federico, Francesco

    2010-03-01

    Case report. To report the first case of posterior spinal artery (PSA) infarct due to patent foramen ovale (PFO). Infarct in the territories of PSA are very rare: till now 38 cases are reported in the literature. Moreover only 1 case of spinal cord infarction was attributed to paradoxical embolism through PFO, but in the anterior spinal artery territory. A 60-year-old woman was hospitalized for sudden numbness of the right leg. Neurologic examination revealed right leg mild paresis and loss of proprioception and dysesthesia at T11. Spine-MRI showed T5-T7 posterolateral cord ischemia. Transesophageal echocardiography disclosed a PFO with severe right-left shunt confirmed by transcranial Doppler. During the hospitalization she was treated with oral-500 mg ticlopidine, because of a mild allergic reaction to acetylsalicylic acid. No steroids were administrated. Physiotherapy was performed daily. Motor and urinary symptoms disappeared in 20 days. At 1-month clinical follow-up only suspended dysesthesia on the right side was present. At 3 months follow-up spine-MRI showed no signal abnormalities within the spinal cord but the patient still complained of dysesthesia. The therapy was changed to oral 75 mg clopidogrel, cause of leucopoenia. At 1-year follow-up dysesthesia was still present, but less complaining and no recurrence and adverse effect due to clopidogrel therapy were reported. This report describe a case of acute nontraumatic myelopathy. At 4 days after onset, PSA infarct was diagnosed on the basis of neurologic findings and MR images. After extensive diagnostic work-up, we were able to identify only PFO, so it was the first case of PSA due to probable paradoxical embolism. The patient was treated with antiplatelet therapy with good recovery and no recurrence at 1-year follow-up.

  1. [Horner syndrome as a manifestation of carotid artery dissection].

    PubMed

    Rohrweck, S; España-Gregori, E; Gené-Sampedro, A; Pascual-Lozano, A M; Aparici-Robles, F; Díaz-Llopis, M

    2011-11-01

    A 42-year-old man presented with ptosis and miosis in his left eye and a history of headache over the last 20 days. An angioresonance showed dissection of internal carotid artery. "Painful Horner's Syndrome" is considered to be a medical emergency due possible onset of an internal carotid artery dissection. We consider that awareness of neuro-ophthalmologic emergencies is very important in the clinical praxis of an ophthalmologist. Multidisciplinary treatment and follow-up of these patients is required. Copyright © 2011 Sociedad Española de Oftalmología. Published by Elsevier Espana. All rights reserved.

  2. Bilateral axillary artery aneurysms after Bentall procedure in Marfan syndrome.

    PubMed

    Haruki, Takashi; Ito, Hiroshi; Sakata, Kensuke; Kobayashi, Yurio

    2015-11-01

    A man with Marfan syndrome underwent a Bentall procedure for annuloaortic ectasia and severe aortic regurgitation at 43 years of age. Twenty-eight years after the Bentall procedure, he developed bilateral axillary artery aneurysms (length × diameter: right: 80 × 39 mm; left: 103 × 45 mm). Aneurysmectomy and reconstruction of the axillary artery were performed using an artificial vascular graft. Histological examination revealed cystic medial necrosis. The postoperative course was uneventful, but long-term follow-up is necessary.

  3. Primary Spinal Epidural Lymphoma As a Cause of Spontaneous Spinal Anterior Syndrome: A Case Report and Literature Review.

    PubMed

    Córdoba-Mosqueda, M E; Guerra-Mora, J R; Sánchez-Silva, M C; Vicuña-González, R M; Torre, A Ibarra-de la

    2017-01-01

    Background Primary spinal epidural lymphoma (PSEL) is one of the rarest categories of tumors. Spinal cord compression is an uncommon primary manifestation and requires to be treated with surgery for the purpose of diagnosis and decompression. Case Presentation A 45-year-old man presented with a new onset thoracic pain and progress to an anterior spinal syndrome with hypoesthesia and loss of thermalgesia. Magnetic resonance image showed a paravertebral mass that produces medullary compression at T3. The patient was taken up to surgery, where the pathology examination showed a diffuse large B-cell lymphoma. Conclusions PSEL is a pathological entity, which must be considered on a middle-aged man who began with radicular compression, and the treatment of choice is decompression and biopsy. The specific management has not been established yet, but the literature suggests chemotherapy and radiotherapy; however, the outcome is unclear.

  4. Activation of intestinal spinal afferent endings by changes in intra-mesenteric arterial pressure

    PubMed Central

    Humenick, A; Chen, B N; Wiklendt, L; Spencer, N J; Zagorodnyuk, V P; Dinning, P G; Costa, M; Brookes, S J H

    2015-01-01

    Spinal sensory neurons innervate many large blood vessels throughout the body. Their activation causes the hallmarks of neurogenic inflammation: vasodilatation through the release of the neuropeptide calcitonin gene-related peptide and plasma extravasation via tachykinins. The same vasodilator afferent neurons show mechanical sensitivity, responding to crushing, compression or axial stretch of blood vessels – responses which activate pain pathways and which can be modified by cell damage and inflammation. In the present study, we tested whether spinal afferent axons ending on branching mesenteric arteries (‘vascular afferents’) are sensitive to increased intravascular pressure. From a holding pressure of 5 mmHg, distension to 20, 40, 60 or 80 mmHg caused graded, slowly adapting increases in firing of vascular afferents. Many of the same afferent units showed responses to axial stretch, which summed with responses evoked by raised pressure. Many vascular afferents were also sensitive to raised temperature, capsaicin and/or local compression with von Frey hairs. However, responses to raised pressure in single, isolated vessels were negligible, suggesting that the adequate stimulus is distortion of the arterial arcade rather than distension per se. Increasing arterial pressure often triggered peristaltic contractions in the neighbouring segment of intestine, an effect that was mimicked by acute exposure to capsaicin (1 μm) and which was reduced after desensitisation to capsaicin. These results indicate that sensory fibres with perivascular endings are sensitive to pressure-induced distortion of branched arteries, in addition to compression and axial stretch, and that they contribute functional inputs to enteric motor circuits. PMID:26010893

  5. Adjuvant spinal cord stimulation improves wound healing of peripheral tissue loss due to steal syndrome of the hand: clinical challenge treating a difficult case.

    PubMed

    De Caridi, Giovanni; Massara, Mafalda; Benedetto, Filippo; Tripodi, Paolo; Spinelli, Francesco; David, Antonio; Grande, Raffaele; Butrico, Lucia; Serra, Raffaele; de Franciscis, Stefano

    2016-02-01

    Hand ischaemia due to arterial steal syndrome is an infrequent, but potentially serious complication of arteriovenous fistula (AVF) for haemodialysis. We present a case of hand ischaemia caused by steal syndrome in a 69-year-old haemodialysis patient, 10 months after a brachiobasilic fistula creation. The patient underwent multiple operations without resolution of hand pain and tissue loss. The implantation of an adjuvant cervical spinal cord stimulator allowed the patient to obtain complete hand pain relief and wound healing. Probably, the diffuse microangiopathy typical of haemodialysis patients could be responsible for the persistence of ischaemic signs and symptoms after a surgical revascularisation. The effect of sympathetic blockade and the subsequent improvement of the arterial blood flow and tissue oxygenation because of spinal cord stimulation (SCS) can be useful to achieve complete ischaemic pain relief in order to enhance wound healing and to limit the tissue loss. In conclusion, the association of cervical spinal cord stimulation and surgical revascularisation could represent a valid option to treat a critical upper limb ischaemia following steal syndrome due to AVF.

  6. Blood flow increase by cervical spinal cord stimulation in middle cerebral and common carotid arteries.

    PubMed

    Robaina, Francisco; Clavo, Bernardino; Catalá, Luis; Caramés, Miguel Á; Morera, Jesús

    2004-01-01

    The effect of spinal cord stimulation (SCS) on cerebral blood flow (CBF) has, in the past, been evaluated by semiquantitative techniques, but has not been used to treat CBF diseases. The aim of this study was to assess the effect of cervical SCS on regional blood flow by both semiquantitative and quantitative methods. Thirty-five patients with cervical SCS-implanted devices were enrolled. The following parameters were measured before and after cervical SCS: systolic and diastolic velocity (cm/s) in the middle cerebral artery (MCA) by transcranial Doppler (TCD) and volume blood flow quantification (ml/min) in the common carotid artery (CCA) by color Doppler. During cervical SCS there was a significant and bilateral increase in systolic (21%) and diastolic (26%) velocity in the MCA and in CCA blood flow (50%). We conclude that cervical SCS increases blood flow in the middle cerebral artery and common carotid artery. The consistent increase supports the potential usefulness of cervical SCS as an adjuvant treatment for cerebral blood flow diseases.

  7. Selective occlusion of lumbar arteries as a spinal cord ischemia model in rabbits.

    PubMed

    Maeda, Tomoyuki; Mori, Koichi; Shiraishi, Yoshimitsu; Tatebayashi, Kyoko; Kawai, Yasuaki

    2003-02-01

    Paraplegia is a devastating complication of operations requiring transient occlusion of the descending thoracic aorta. Many animal models of spinal cord ischemia have been utilized to examine the efficacy of various neuroprotective methods. In this study, we establish a rabbit model of spinal cord ischemia by selective temporary occlusion of lumbar arteries and examine the protective effects of systemic mild hypothermia in this model. Animals were divided into the following four groups: sham group (group A, n = 6); 10 min ischemia, normothermia (39 degrees C) (group B, n = 6); 20 min ischemia, normothermia (group C, n = 6); and 30 min ischemia, mild hypothermia (35 degrees C) (group D, n = 6). After 7 d of reperfusion, three rabbits in group B and five rabbits in group C developed paraplegia (Tarlov's score = 0). In contrast, all rabbits preserved hindlimb motor function (Tarlov's score = 4) in groups A and D. Histological findings indicated that the number of motor neurons in the anterior horns in group C were significantly fewer than in group A. A large number of motor neurons were preserved in group D. Hypothermia is known to be an effective and reliable method of neuroprotection, but the risk of complications rises at deep hypothermia. Our current results confirm that systemic, mild hypothermia is a safe and effective neuroprotective method during ischemia-reperfusion injury of the spinal cord.

  8. [Horner's syndrome revealing a spontaneous carotid artery dissection].

    PubMed

    Verdin, V; Holemans, C; Otto, B; Van Damme, H; Defraigne, J O

    2013-01-01

    We report a case of spontaneous carotid artery dissection suspected by the appearance of Horner's syndrome. Under medical treatment, the intramural hematoma resolved within 3 months. The patient had an uneventful recovery, without any residual neurologic deficit. Spontaneous arterial dissection is responsible for a hematoma in the arterial wall without significant trauma. The pathogenesis remains unknown. Predisposing factors seem to exist. The clinical presentation is variable mainly due to local compression of adjacent structures which can precede a transient or permanent neurological deficit. The diagnosis is confirmed by Doppler US, CT angiography or magnetic resonance angiography, the best optional investigations. The treatment mainly consists of stroke prevention by anticoagulation versus antiplatelet therapy. The role of surgery and/or endovascular techniques has not yet been confirmed.

  9. Hypothalamic germinoma masquerading as superior mesenteric artery (SMA) syndrome.

    PubMed

    Vethakkan, Shireene R; Venugopal, Yogeswari; Tan, Alexander T B; Paramasivam, Sharmila S; Ratnasingam, Jeyakantha; Razak, Rohaya A; Alias, Azmi; Kassim, Fauziah; Choong, Karen

    2013-01-01

    To report a case of superior mesenteric artery (SMA) syndrome secondary to hypothalamic germinoma. We describe the clinical presentation, diagnostic work-up, management, and clinical course of a patient admitted with SMA syndrome who was subsequently found to have a hypothalamic germinoma. An adolescent boy was admitted to the surgical ward with progressive weight loss over a 2 year period and postprandial vomiting. He was diagnosed with SMA syndrome based on evidence of proximal duodenal dilatation, extrinsic compression of the distal duodenum, and a narrowed aortomesenteric angle (16°). Investigations performed to exclude thyrotoxicosis unexpectedly revealed secondary hypothyroidism and further evaluation demonstrated evidence of pan-hypopituitarism. Psychiatric evaluation excluded anorexia nervosa and bulimia. Magnetic resonance imaging (MRI) of the brain revealed a heterogeneously enhancing hypothalamic lesion, but a normal pituitary gland. Hormone replacement with hydrocortisone, desmopressin, testosterone, and thyroxine resulted in weight gain and resolution of gastrointestinal symptoms. A transventricular endoscopic biopsy subsequently confirmed a hypothalamic germinoma and he was referred to an oncologist. SMA syndrome secondary to severe weight loss is an uncommon cause of upper gastrointestinal obstruction. While there have been reports of poorly controlled diabetes mellitus and thyrotoxicosis manifesting as SMA syndrome, there are no published reports to date of SMA syndrome secondary to hypothalamic/pituitary disease. Management of SMA syndrome is conservative, as symptoms of intestinal obstruction resolve with weight gain following treatment of the underlying cause. Awareness of this uncommon presentation of endocrine cachexia/hypothalamic disease will prevent unnecessary laparotomies and a misdiagnosis of an eating disorder.

  10. Successful treatment of SAPHO syndrome with severe spinal disorder using entercept: a case study.

    PubMed

    Zhang, L L; Zhao, J X; Liu, X Y

    2012-07-01

    SAPHO syndrome (synovitis, acne, pustulosis, hyperostosis, and osteitis) is a rare disease. Presently, there is no treatment guideline for this illness. Several studies suggested entercept, a novel biological agent against tumor necrosis factor-alpha, is effective in treating SAPHO syndrome. We report a case in which the clinical conditions of a middle-aged female patient diagnosed with SAPHO syndrome, with noted spinal disorder, improved significantly after receiving entercept treatment. The patient remained stable after 3-month follow-up.

  11. Controlateral cavernous syndrome, brainstem congestion and posterior fossa venous thrombosis with cerebellar hematoma related to a ruptured intracavernous carotid artery aneurysm.

    PubMed

    Aldea, Sorin; Guedin, Pierre; Roccatagliata, Luca; Boulin, Anne; Auliac, Stéphanie; Dupuy, Michel; Cerf, Charles; Gaillard, Stéphan; Rodesch, Georges

    2011-06-01

    Intracavernous carotid artery aneurysms (ICCAs) are rarely associated with life-threatening complications. We describe a 55-year-old woman who, after the rupture of an intracavernous carotid artery aneurysm, presented with a contralateral cavernous sinus syndrome and severe posterior fossa and spinal cord symptoms. Following parent artery occlusion, thrombosis of the posterior fossa and spinal cord veins caused a progressive worsening of the neurological status to a "locked-in" state. The patient fully recovered with anticoagulation therapy. Comprehension of the pathophysiological mechanism associated with the rupture of ICCA and early diagnosis of the related symptoms are essential in order to plan a correct treatment that includes the management of the aneurysm rupture and of possible complications related to venous thrombosis.

  12. Posterior circulation cerebral hyperperfusion syndrome after high flow external carotid artery to middle cerebral artery bypass.

    PubMed

    Quach, Eric T; Gonzalez, Andres A; Shilian, Parastou; Russin, Jonathan J

    2015-09-01

    We present the first report, to our knowledge, in which revascularization of the middle cerebral artery (MCA) with a high flow extracranial-intracranial procedure resulted in symptomatic hyperemia of the posterior circulation. Cerebral hyperperfusion syndrome (CHS) is a poorly understood phenomenon that is classically seen in the distribution of a revascularized artery. A 37-year-old woman presented with a 3 month history of cognitive and speech difficulties, persistent headaches, weakness, numbness, and paresthesia which was worse in the right extremities and face. She was found to have bilateral watershed infarcts worse in the left cerebral hemisphere, severe bilateral stenosis of the supraclinoid internal carotid artery, and a small left superior hypophyseal aneurysm. The patient underwent left cerebral hemisphere revascularization with a high flow external carotid artery to MCA bypass with aneurysm trapping. During skin closure, significant changes were seen in her bilateral upper extremity motor-evoked potentials. The patient's postoperative exam was noted for an intermittent inability to follow commands, bilateral upper extremity weakness, vertical nystagmus, and alogia that all dramatically improved with strict blood pressure control. Postoperative perfusion imaging revealed posterior circulation hyperemia. This patient highlights the potential for hyperemic complications outside the revascularized arterial territory. Strict blood pressure control is recommended in order to prevent and manage hyperemia-associated symptoms. Improving our understanding of CHS may assist in identifying at risk patients and at risk arterial territories in order to optimize CHS prevention and management strategies.

  13. Midline defects in FG syndrome: does tethered spinal cord contribute to the phenotype?

    PubMed

    Wang, Raymond; Visootsak, Jeannie; Danielpour, Moise; Graham, John M

    2005-04-01

    FG syndrome is an X-linked recessive mental retardation syndrome with ano-rectal anomalies, constipation, and occasional urinary incontinence. Because tethered spinal cord syndrome (TCS) has similar symptoms, we evaluated imaging for TCS in patients with FG syndrome. Patients were recruited from the International FG Syndrome Support Group, and an FG Syndrome Consensus Group reviewed clinical histories, medical records, and photographs of each responding patient. Results of cranial and spinal imaging studies were available for 12 patients. Of 12 boys with FG syndrome, 6 had hypoplasia of the corpus callosum, and 3 of these had TCS (all with constipation and urinary symptoms). The other 9 did not have urinary symptoms. After surgical untethering, bowel and bladder symptoms improved. Tethered spinal cord syndrome occurred in 25% of patients with FG syndrome associated with hypoplasia of the corpus callosum and causing bowel and bladder incontinence. A high index of suspicion is necessary for early diagnosis, and timely intervention results in significant improvement in symptomatology.

  14. Junctional disc herniation syndrome in post spinal fusion treated with endoscopic spine surgery.

    PubMed

    Chiu, John C; Clifford, Thomas; Princenthal, Robert; Shaw, Stephen

    2005-01-01

    Fusions of the cervical and lumbar spine are often followed within months or several years by protrusion of discs at the adjacent level or levels. Biomechanical alterations and mobility lost at the fused levels are thought to be transferring the stress to the adjacent segments or discs, which results in accelerated degeneration of the discs and causes disc protrusion. This post-spinal fusion "junctional disc herniation syndrome" (JDHS), or the post-spinal fusion "adjacent segment disease (ASD)" can occur from 15% to 52% of post-spinal fusion, in both superior and/or inferior adjacent levels. The ways in which endoscopic minimally invasive spinal discectomy procedure can be used to treat this JDHS and preserve spinal segmental motion are discussed herein. Also, case illustrations are presented.

  15. Postoperative spinal adhesive arachnoiditis presenting with hydrocephalus and cauda equina syndrome.

    PubMed

    Koerts, Guus; Rooijakkers, Herbert; Abu-Serieh, Basel; Cosnard, Guy; Raftopoulos, Christian

    2008-02-01

    To our knowledge, the association between hydrocephalus and postoperative spinal adhesive arachnoiditis (SAA) has never been reported. Herein we describe an unusual case of a 45-year-old man with spinal adhesive arachnoiditis (SAA) who developed delayed-onset hypertensive hydrocephalus and cauda equina syndrome (CES) after multiple low-back surgeries. The patient's clinical presentation, imaging findings, surgical management, and the possible mechanisms are discussed in the light of the present literature.

  16. Acute spinal epidural hematoma and cranial interdural hematoma due to a rupture of a posterior communicating artery aneurysm: case report.

    PubMed

    Bartoli, Andrea; Kotowski, Marc; Pereira, Vitor Mendes; Schaller, Karl

    2011-10-01

    We describe an unusual presentation of a ruptured aneurysm of the posterior communicating artery with an acute intracranial hematoma between the dural layers associated with an acute spinal epidural hematoma descending to L1. A 35-year-old woman presented 3 hours after ictus with a postcoital headache, neck stiffness, and bilateral abducens cranial nerve palsy. No other neurological deficits were present. Clinically, she had a subarachnoid hemorrhage World Federation of Neurosurgical Societies grade 1. CT scan demonstrates an acute subdural hematoma, extending from the right parasellar region, around the clivus, tentorium, and falx. Angio-CT showed a posterior communicating artery aneurysm and an anterior communicating artery aneurysm and an extension of the hematoma to the cervical spine. This justified a spinal and cerebral MRI that confirmed an extension of the hematoma to the epidural space at the cervical, thoracic, and lumbar levels. Three-dimensional digital subtraction angiography confirmed aneurysms on the right posterior communicating artery and on the anterior communicating artery. Both aneurysms were completely occluded by coiling. With reference to the concept of the cranial subdural compartment described in studies conducted using an electron microscope, this group of hematomas was classified as interdural. Ruptured aneurysm of the posterior communicating artery may cause cranial acute interdural hematoma with a typical subarachnoid hemorrhage clinical presentation, and it rarely can extend to spinal epidural space.

  17. Mechanical properties of the common carotid artery in Williams syndrome

    PubMed Central

    Aggoun, Y; Sidi, D; Levy, B; Lyonnet, S; Kachaner, J; Bonnet, D

    2000-01-01

    OBJECTIVE—To determine whether arterial wall hypertrophy in elastic arteries was associated with alteration in their mechanical properties in young patients with Williams syndrome.
METHODS—Arterial pressure and intima-media thickness, cross sectional compliance, distensibility, circumferential wall stress, and incremental elastic modulus of the common carotid artery were measured non-invasively in 21 Williams patients (mean (SD) age 8.5 (4) years) and 21 children of similar age.
RESULTS—Systolic and diastolic blood pressures were higher in Williams patients (125/66 v 113/60 mm Hg, p < 0.05). The mean (SD) intima-media thickness was increased in Williams patients, at 0.6 (0.07) v 0.5 (0.03) mm (p < 0.001). Normotensive Williams patients had a lower circumferential wall stress (2.1 (0.5) v 3.0 (0.7) mm Hg, p < 0.01), a higher distensibility (1.1 (0.3) v 0.8 (0.3) mm Hg−1.10−2, p < 0.01), similar cross sectional compliance (0.14 (0.04) v 0.15 (0.05) mm2.mm Hg−1, p > 0.05), and lower incremental elastic modulus (7.4 (2.0) v 14.0 (5.0) mm Hg.102; p < 0.001).
CONCLUSIONS—The compliance of the large elastic arteries is not modified in Williams syndrome, even though increased intima-media thickness and lower arterial stiffness are consistent features. Therefore systemic hypertension cannot be attributed to impaired compliance of the arterial tree in this condition.


Keywords: elastin; Williams syndrome; hypertension; compliance PMID:10956293

  18. Reversible Hypertensive Myelopathy-The Spinal Cord Variant of Posterior Reversible Encephalopathy Syndrome.

    PubMed

    Gocmen, Rahsan; Ardicli, Didem; Erarslan, Yasin; Duzova, Ali; Anlar, Banu

    2017-04-01

    The posterior reversible encephalopathy syndrome (PRES) is a well-known clinical and radiologic entity mainly affecting the territory of the posterior cerebral circulation. Spinal cord involvement is extremely rare, and as of yet, only a few cases have been reported in the literature. The present case describes a reversible, longitudinal spinal cord lesion in a patient with high blood pressure. We discuss the differential diagnosis of longitudinal myelopathy and focus on the clinical presentation, diagnosis, and management of the "spinal cord variant of PRES." Georg Thieme Verlag KG Stuttgart · New York.

  19. Treatment of refractory ischemic pain from chemotherapy-induced Raynaud's syndrome with spinal cord stimulation.

    PubMed

    Ting, Joseph C; Fukshansky, Mikhail; Burton, Allen W

    2007-06-01

    We report the successful treatment of refractory ischemic pain from cisplatin-induced Raynaud's syndrome with spinal cord stimulation after failed pharmacologic management and surgical sympathectomy. A 48-year-old man developed ischemic pain of the hands while undergoing cisplatin and gemcitabine chemotherapy for metastatic pancreatic carcinoma. After extensive pharmacologic management and surgical sympathectomy failed to provide adequate analgesia, the patient underwent a percutaneous spinal cord stimulation trial followed by permanent implantation and received significant pain relief prior to succumbing to his illness. Spinal cord stimulation provided effective therapy for refractory ischemic pain, even after failed sympathectomy.

  20. Restless leg syndrome in patients with spinal cord injury.

    PubMed

    Kumru, Hatice; Vidal, Joan; Benito, Jesus; Barrio, Manuela; Portell, Enric; Valles, Margarita; Flores, Cecilia; Santamaria, Joan

    2015-12-01

    The presence of restless leg syndrome (RLS) in patients with spinal cord injury (SCI) is not well established. We studied the frequency and severity of RLS in a population of patients with SCI and the effect of treatment with dopaminergic drugs when clinically indicated. Consecutive patients with SCI admitted to an out-patient clinic of a neurorehabilitation hospital (n:195) were evaluated for the presence of RLS between February 2013 and May 2014. A diagnosis of RLS was made according to the criteria of the International RLS Study Group, and severity was assessed by the RLS rating scale. Information on gender, age, time since SCI, level and severity of SCI, was obtained. The mean age was 54.7 ± 15.6 years (range: 22-81 year); with time since SCI: 16.9 ± 11.4 years (range: 1-50 years). Thirty-five of 195 patients (17.9%) presented RLS. Twenty-two out of 154 (14.3%) patients with cervicothoracic SCI and 13 out of 41 (31.7%) patients with lumbosacral SCI presented RLS. Restless leg symptoms were mild in 2 patients, moderate in 10 patients, severe in 12 patients and very severe in 11. Ten patients received dopaminergic agonist treatment with a significant reduction in RLS severity scale from 29.1 ± 5.9 to 10.2 ± 7.9. RLS occurs frequently in SCI patients and responds to dopaminergic treatment. Physicians have to be aware of this diagnosis to avoid unnecessary suffering in this patient population. Copyright © 2015 Elsevier Ltd. All rights reserved.

  1. Vertebral artery stump syndrome in acute ischemic stroke.

    PubMed

    Kawano, Hiroyuki; Inatomi, Yuichiro; Hirano, Teruyuki; Yonehara, Toshiro

    2013-01-15

    Although the carotid artery stump as an embolic source for ischemic stroke has been well described, there have been few systematic reports of a similar syndrome in the posterior circulation (PC) after vertebral artery (VA) origin occlusion. The aim of this study was to identify the incidence and characteristics of acute ischemic stroke with VA stump syndrome. Of 3463 consecutive patients who were admitted within 7 days after onset, 865 patients with acute ischemic stroke in the PC were enrolled. The diagnostic criteria of VA stump syndrome included: (1) acute ischemic stroke in the posterior circulation; (2) the VA origin occlusion identified on MRA, duplex ultrasound, CT angiography, and/or conventional angiography; (3) presence of distal antegrade flow in the ipsilateral VA; and (4) absence of other causes of ischemic stroke. Of the 865 patients with PC stroke, 12 (1.4%) were diagnosed as having VA stump syndrome. The ischemic lesions included the cerebellum in all patients. Nine patients had multiple ischemic lesions in the brain stem, thalamus, or posterior lobe other than cerebellum. On duplex ultrasound, a to-and-fro flow pattern was observed in the culprit VA in 10 patients. Three patients had recurrences of ischemic stroke in the PC during the acute phase. VA stump syndrome was not a rare mechanism of PC stroke, and there was a high rate of stroke recurrence during the acute phase. Vascular assessment by a multimodality approach can be used to promptly detect VA stump syndrome. Copyright © 2012 Elsevier B.V. All rights reserved.

  2. A Case Report of Locked-in Syndrome Due to Bilateral Vertebral Artery Dissection After Cervical Spine Manipulation Treated by Arterial Embolectomy.

    PubMed

    Ke, Jiang-Qiong; Yin, Bo; Fu, Fang-Wang; Shao, Sheng-Min; Lin, Yan; Dong, Qi-Qiang; Wang, Xiao-Tong; Zheng, Guo-Qing

    2016-02-01

    Cervical spine manipulation (CSM) is a commonly spinal manipulative therapies for the relief of cervical spine-related conditions worldwide, but its use remains controversial. CSM may carry the potential for serious neurovascular complications, primarily due to vertebral artery dissection (VAD) and subsequent vertebrobasilar stroke. Here, we reported a rare case of locked-in syndrome (LIS) due to bilaterial VAD after CSM treated by arterial embolectomy.A 36-year-old right-handed man was admitted to our hospital with numbness and weakness of limbs after treating with CSM for neck for half an hour. Gradually, although the patient remained conscious, he could not speak but could communicate with the surrounding by blinking or moving his eyes, and turned to complete quadriplegia, complete facial and bulbar palsy, dyspnea at 4 hours after admission. He was diagnosed with LIS. Then, the patient was received cervical and brain computed tomography angiography that showed bilateral VAD. Aortocranial digital subtraction angiography showed vertebrobasilar thrombosis, blocking left vertebral artery, and stenosis of right vertebral artery. The patient was treated by using emergency arterial embolectomy and followed by antiplatelet therapy and supportive therapy in the intensive care unit and a general ward. Twenty-seven days later, the patient's physical function gradually improved and discharged but still left neurological deficit with muscle strength grade 3/5 and hyperreflexia of limbs.Our findings suggested that CSM might have potential severe side-effect like LIS due to bilaterial VAD, and arterial embolectomy is an important treatment choice. The practitioner must be aware of this complication and should give the patients informed consent to CSM, although not all stroke cases temporally related to SCM have pre-existing craniocervical artery dissection.

  3. A Case Report of Locked-in Syndrome Due to Bilateral Vertebral Artery Dissection After Cervical Spine Manipulation Treated by Arterial Embolectomy

    PubMed Central

    Ke, Jiang-Qiong; Yin, Bo; Fu, Fang-Wang; Shao, Sheng-Min; Lin, Yan; Dong, Qi-Qiang; Wang, Xiao-Tong; Zheng, Guo-Qing

    2016-01-01

    Abstract Cervical spine manipulation (CSM) is a commonly spinal manipulative therapies for the relief of cervical spine-related conditions worldwide, but its use remains controversial. CSM may carry the potential for serious neurovascular complications, primarily due to vertebral artery dissection (VAD) and subsequent vertebrobasilar stroke. Here, we reported a rare case of locked-in syndrome (LIS) due to bilaterial VAD after CSM treated by arterial embolectomy. A 36-year-old right-handed man was admitted to our hospital with numbness and weakness of limbs after treating with CSM for neck for half an hour. Gradually, although the patient remained conscious, he could not speak but could communicate with the surrounding by blinking or moving his eyes, and turned to complete quadriplegia, complete facial and bulbar palsy, dyspnea at 4 hours after admission. He was diagnosed with LIS. Then, the patient was received cervical and brain computed tomography angiography that showed bilateral VAD. Aortocranial digital subtraction angiography showed vertebrobasilar thrombosis, blocking left vertebral artery, and stenosis of right vertebral artery. The patient was treated by using emergency arterial embolectomy and followed by antiplatelet therapy and supportive therapy in the intensive care unit and a general ward. Twenty-seven days later, the patient's physical function gradually improved and discharged but still left neurological deficit with muscle strength grade 3/5 and hyperreflexia of limbs. Our findings suggested that CSM might have potential severe side-effect like LIS due to bilaterial VAD, and arterial embolectomy is an important treatment choice. The practitioner must be aware of this complication and should give the patients informed consent to CSM, although not all stroke cases temporally related to SCM have pre-existing craniocervical artery dissection. PMID:26844510

  4. Real-time ultrasound: Key factor in identifying celiac artery compression syndrome.

    PubMed

    Tembey, Raina Anil; Bajaj, Aneeta S; Wagle, Prasad K; Ansari, Abdul Samad

    2015-01-01

    The median arcuate ligament syndrome (MALS) or celiac artery compression syndrome (CACS) is a rare entity, presenting clinically with postprandial abdominal pain and weight loss. The diagnosis is made on computed tomography (CT) angiography, which reveals extrinsic compression of the proximal part of the celiac artery by the median arcuate ligament, producing a characteristic hooked appearance. We report a case of the celiac artery compression syndrome, diagnosed by Doppler USG evaluation.

  5. Intracranial arteries in individuals with the elastin gene hemideletion of Williams syndrome.

    PubMed

    Wint, D P; Butman, J A; Masdeu, J C; Meyer-Lindenberg, A; Mervis, C B; Sarpal, D; Morris, C A; Berman, K F

    2014-01-01

    Williams syndrome, a rare genetic disorder with a striking neurobehavioral profile characterized by extreme sociability and impaired visuospatial construction abilities, is caused by a hemideletion that includes the elastin gene, resulting in frequent supravavular aortic stenosis and other stenotic arterial lesions. Strokes have been reported in Williams syndrome. Although the extracranial carotid artery has been studied in a sample of patients with Williams syndrome, proximal intracranial arteries have not. Using MRA, we studied the intracranial vessels in 27 participants: 14 patients with Williams syndrome (age range, 18-44 years; mean age, 27.3 ± 9.1; 43% women) and 13 healthy control participants with similar age and sex distribution (age range, 22-52 years; mean age, 33.4 ± 7.6; 46% women). All participants with Williams syndrome had hemideletions of the elastin gene. Blinded to group allocation or to any other clinical data, a neuroradiologist determined the presence of intracranial vascular changes in the 2 groups. The Williams syndrome group and the healthy control group had similar patency of the proximal intracranial arteries, including the internal carotid and vertebral arteries; basilar artery; and stem and proximal branches of the anterior cerebral artery, MCA, and posterior cerebral arteries. The postcommunicating segment of the anterior cerebral artery was longer in the Williams syndrome group. Despite the elastin haploinsufficiency, the proximal intracranial arteries in Williams syndrome preserve normal patency.

  6. Tarsal tunnel syndrome associated with a pulsating artery: effectiveness of high-resolution ultrasound in diagnosing tarsal tunnel syndrome.

    PubMed

    Kim, Eunkuk; Childers, Martin K

    2010-01-01

    We describe a patient with tarsal tunnel syndrome in whom ultrasound imaging revealed compression of the posterior tibial nerve by a pulsating artery. High-resolution ultrasound showed a round pulsating hypoechoic lesion in contact with the posterior tibial nerve. Ultrasound-guided injection of 0.5% lidocaine temporarily resolved the paresthesia. These findings suggest an arterial etiology of tarsal tunnel syndrome.

  7. Acute Coronary Syndrome in a Puerperal Patient with Coronary Artery Ectasia due to a Coronary Artery Fistula.

    PubMed

    Okada, Taiji; Endo, Akihiro; Ito, Simpei; Nakamura, Taku; Sugamori, Takashi; Takahashi, Nubuyuki; Yoshitomi, Hiroyuki; Tanabe, Kazuaki

    Coronary artery fistulas are rare and the feeding artery is ectatic and tortuous. It is not well-known whether coronary artery ectasia (CAE) is a risk factor of acute coronary syndrome (ACS) in the puerperal periods. A 40-year-old woman with a coronary artery fistula and an ectatic right coronary artery (RCA) had delivered twins. A month later, she had chest pain and coronary angiography revealed thrombogenesis in the RCA. She had no risk factors of cardiovascular disease or thrombogenesis. We should recognize that CAE is a risk factor for ACS in women in the perinatal and puerperal periods.

  8. Outcomes of Brugada Syndrome Patients with Coronary Artery Vasospasm

    PubMed Central

    Kujime, Shingo; Sakurada, Harumizu; Saito, Naoki; Enomoto, Yoshinari; Ito, Naoshi; Nakamura, Keijiro; Fukamizu, Seiji; Tejima, Tamotsu; Yambe, Yuzuru; Nishizaki, Mitsuhiro; Noro, Mahito; Hiraoka, Masayasu; Sugi, Kaoru

    2017-01-01

    Objective To evaluate the outcomes of patients with concomitant Brugada syndrome and coronary artery vasospasm. Methods Patients diagnosed with Brugada syndrome with an implantable cardiac defibrillator were retrospectively investigated, and the coexistence of vasospasm was evaluated. The clinical features and outcomes were evaluated, especially in patients with coexistent vasospasm. A provocation test using acetylcholine was performed in patients confirmed to have no organic stenosis on percutaneous coronary angiography to confirm the presence of vasospasm. Implantable cardiac defibrillator shock status was checked every three months. Statistical comparisons of the groups with and without vasospasm were performed. A univariate analysis was also performed, and the odds ratio for the risk of implantable cardiac defibrillator shock was calculated. Patients Thirty-five patients with Brugada syndrome, of whom six had coexistent vasospasm. Results There were no significant differences in the laboratory data, echocardiogram findings, disease, or the history of taking any drugs between patients with and without vasospasm. There were significant differences in the clinical features of Brugada syndrome, i.e. cardiac events such as resuscitation from ventricular fibrillation or appropriate implantable cardiac defibrillator shock. Four patients with vasospasm had cardiac events such as resuscitation from ventricular fibrillation and/or appropriate defibrillator shock; three of them had no cardiac events with calcium channel blocker therapy to prevent vasospasm. The coexistence of vasospasm was a potential risk factor for an appropriate implantable cardiac defibrillator shock (odds ratio: 13.5, confidence interval: 1.572-115.940, p value: 0.035) on a univariate analysis. Conclusion Coronary artery vasospasm could be a risk factor for cardiac events in patients with Brugada syndrome. PMID:28090040

  9. Location of Radicular Spinal Arteries in the Lumbar Spine from Analysis of CT Angiograms of the Abdomen and Pelvis.

    PubMed

    Simon, Jeremy I; McAuliffe, Matthew; Smoger, David

    2016-01-01

    Reports of catastrophic neurologic injuries following lumbar transforaminal epidural steroid injections are rare but serious potential complications. The traditional method of performing lumbar transforaminal epidural steroid injections is in the "safe triangle" to avoid contact to the spinal nerve. Some authors advocate an alternative approach by placing the needle inferiorly in a region referred to as "Kambin's triangle" to avoid incurring arteries. This study aimed to determine the location of arteries within the L1-L4 intervertebral foramen in vivo, specifically if they lie within or in close proximity to the "safe triangle" or Kambin's triangle using CT angiograms of the abdomen and pelvis. The authors retrospectively evaluated the location in vivo of arterial vessels in the intervertebral foramen from L1 to L4 in patients who underwent abdominopelvic CT angiograms for aortic vascular disease. The data were reanalyzed to confirm inter-rater reliability. Arteries were found in both the safe triangle and Kambin's triangle at a statistically significant rate (P < 0.05). In this group of patients, an artery was found in either the safe triangle or in Kambin's triangle frequently, suggesting the location of these arteries can be quite variable. Physicians performing these procedures should use universal precautions to avoid inadvertent injection into the lumbar spinal arteries and minimize potential complications regardless of the approach. Published by Oxford University Press on behalf of the American Academy of Pain Medicine. 2016. This work is written by US Government employees and is in the public domain in the US.

  10. Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

    PubMed

    Khatibi, Kasra; Heit, Jeremy J; Telischak, Nicholas A; Elbers, Jorina M; Do, Huy M

    2015-06-24

    A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

  11. Cerebral vascular findings in PAPA syndrome: cerebral arterial vasculopathy or vasculitis and a posterior cerebral artery dissecting aneurysm.

    PubMed

    Khatibi, Kasra; Heit, Jeremy J; Telischak, Nicholas A; Elbers, Jorina M; Do, Huy M

    2016-08-01

    A young patient with PAPA (pyogenic arthritis, pyoderma gangrenosum, and acne) syndrome developed an unusual cerebral arterial vasculopathy/vasculitis (CAV) that resulted in subarachnoid hemorrhage from a ruptured dissecting posterior cerebral artery (PCA) aneurysm. This aneurysm was successfully treated by endovascular coil sacrifice of the affected segment of the PCA. The patient made an excellent recovery with no significant residual neurologic deficit.

  12. Pulmonary Arterial Stent Implantation in an Adult with Williams Syndrome

    SciTech Connect

    Reesink, Herre J.; Henneman, Onno D. F.; Delden, Otto M. van; Biervliet, Jules D.; Kloek, Jaap J.; Reekers, Jim A.; Bresser, Paul

    2007-07-15

    We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure.

  13. Pulmonary arterial stent implantation in an adult with Williams syndrome.

    PubMed

    Reesink, Herre J; Henneman, Onno D F; van Delden, Otto M; Biervliet, Jules D; Kloek, Jaap J; Reekers, Jim A; Bresser, Paul

    2007-01-01

    We report a 38-year-old patient who presented with pulmonary hypertension and right ventricular dysfunction due to pulmonary artery stenoses as a manifestation of Williams syndrome, mimicking chronic thromboembolic pulmonary hypertension. The patient was treated with balloon angioplasty and stent implantation. Short-term follow-up showed a good clinical result with excellent patency of the stents but early restenosis of the segments in which only balloon angioplasty was performed. These stenoses were subsequently also treated successfully by stent implantation. Stent patency was observed 3 years after the first procedure.

  14. Superior mesenteric artery syndrome in the modern trauma patient.

    PubMed

    Adams, John B; Hawkins, Michael L; Ferdinand, Coville H; Medeiros, Regina S

    2007-08-01

    In 1861, von Rokitansky described obstruction of the third part of the duodenum by external compression of the duodenum by the superior mesenteric artery (SMA). In 1926, this entity was furthermore described by Wilke in his presentation of 75 patients with "chronic duodenal compression". In 1968, Mansberger used angiography to define anatomical measurements as the diagnostic criteria for this condition. Current modalities of diagnosis of SMA syndrome include esophagogastroduodenoscopy, computerized tomography angiogram, fluoroscopy, transabdominal ultrasound, and endoscopic ultrasound. The SMA syndrome has been associated with prolonged confinement in the supine position, loss of weight, loss of abdominal wall muscle tone, application of a body cast, and severe burns. With current surgical techniques allowing early ambulation, patients are able to avoid prolonged bed rest. The use of parenteral and enteral nutritional support has limited the loss of weight associated with trauma and burn patients, making this syndrome uncommon in this patient population. Recent reports of SMA syndrome focus on the association with corrective surgical procedures for scoliosis and obesity.

  15. Determination of Vascular Reactivity of Middle Cerebral Arteries from Stroke and Spinal Cord Injury Animal Models Using Pressure Myography.

    PubMed

    Anwar, Mohammad A; Eid, Ali H

    2016-01-01

    Stroke and other neurovascular derangements are main causes of global death. They, along with spinal cord injuries, are responsible for being the principal cause of disability due to neurological and cognitive problems. These problems then lead to a burden on scarce financial resources and societal care facilities as well as have a profound effect on patients' families. The mechanism of action in these debilitating diseases is complex and unclear. An important component of these problems arises from derangement of blood vessels, such as blockage due to clotting/embolism, endothelial dysfunction, and overreactivity to contractile agents, as well as alteration in endothelial permeability. Moreover, the cerebro-vasculature (large vessels and arterioles) is involved in regulating blood flow by facilitating auto-regulatory processes. Moreover, the anterior (middle cerebral artery and the surrounding region) and posterior (basilar artery and its immediate locality) regions of the brain play a significant role in triggering the pathological progression of ischemic stroke particularly due to inflammatory activity and oxidative stress. Interestingly, modifiable and non-modifiable cardiovascular risk factors are responsible for driving ischemic and hemorrhagic stroke and spinal cord injury. There are different stroke animal models to examine the pathophysiology of middle cerebral and basilar arteries. In this context, arterial myography offers an opportunity to determine the etiology of vascular dysfunction in these diseases. Herein, we describe the technique of pressure myography to examine the reactivity of cerebral vessels to contractile and vasodilator agents and a prelude to stroke and spinal cord injury.

  16. Stab wound with lodged knife tip causing spinal cord and vertebral artery injuries: case report and literature review.

    PubMed

    Xia, Xinlei; Zhang, Fan; Lu, Feizhou; Jiang, Jianyuan; Wang, Lixun; Ma, Xiaosheng

    2012-07-01

    Case report and literature review. To report the case of a young patient who sustained a penetrating wound with a knife tip retained in his cervical vertebrae and to review the literature. Stab wound with foreign body retained, associated with spinal cord injury and vertebral artery injury, is not commonly reported. The timing and approach of surgical intervention are still controversial. A 17-year-old boy with a wound in the neck presented with diminishing feeling and dysfunction of the left leg and arm. Radiographs demonstrated a foreign body at the C4 level, and possible spinal cord and vertebral artery injuries were detected by computed tomography. Digital subtracted angiography showed a small lateral opening of the injured artery, which was successfully embolized. The knife tip was removed from the original wound without severe cerebrospinal fluid leakage or bleeding, The patient achieved immediate improvement after the operation. Cases of simultaneous spinal cord injury and vertebral artery injury in which the foreign body is retained are uncommonly reported. Digital subtracted angiography is necessary for cervical penetrating wounds and surgical approach should be individualized.

  17. Phlegmonous gastritis secondary to superior mesenteric artery syndrome.

    PubMed

    Nomura, Kosuke; Iizuka, Toshiro; Yamashita, Satoshi; Kuribayashi, Yasutaka; Toba, Takahito; Yamada, Akihiro; Furuhata, Tsukasa; Kikuchi, Daisuke; Matsui, Akira; Mitani, Toshifumi; Ogawa, Osamu; Hoteya, Shu; Inoshita, Naoko; Kaise, Mitsuru

    2015-01-01

    We herein report a case of phlegmonous gastritis secondary to superior mesenteric artery syndrome. An 80-year-old woman visited the hospital emergency department with the chief complaints of epigastric pain and vomiting. She was hospitalized urgently following the diagnosis of superior mesenteric artery syndrome based on abdominal computed tomography findings. Conservative therapy was not effective, and phlegmonous gastritis was diagnosed based on the findings of upper gastrointestinal endoscopy and biopsy performed on the 12th day of the disease. Undernutrition and reduced physical activity were observed on hospital admission, and proactive nutritional therapy with enteral nutrition was started. An upper gastrointestinal series, performed approximately 1 month later, confirmed the persistence of strictures and impaired gastric emptying. Because conservative therapy was unlikely to improve oral food intake, open total gastrectomy was performed on the 94th day of the disease. Examination of surgically resected specimens revealed marked inflammation and fibrosis, especially in the body of the stomach. Following a good postoperative recovery, the patient was able to commence oral intake and left our hospital on foot approximately 1 month after surgery.

  18. Dropped-head syndrome resulting from injury to the central spinal cord at the upper cervical level.

    PubMed

    Rust, C L; Ching, A C; Hart, R A

    2011-04-01

    There are many causes of paraspinal muscle weakness which give rise to the dropped-head syndrome. In the upper cervical spine the central portion of the spinal cord innervates the cervical paraspinal muscles. Dropped-head syndrome resulting from injury to the central spinal cord at this level has not previously been described. We report two patients who were treated acutely for this condition. Both presented with weakness in the upper limbs and paraspinal cervical musculature after a fracture of C2. Despite improvement in the strength of the upper limbs, the paraspinal muscle weakness persisted in both patients. One ultimately underwent cervicothoracic fusion to treat her dropped-head syndrome. While the cause of the dropped-head syndrome cannot be definitively ascribed to the injuries to the spinal cord, this pattern is consistent with the known patho-anatomical mechanisms of both injury to the central spinal cord and dropped-head syndrome.

  19. The effects of spinal anaesthesia for elective caesarean section on uterine and umbilical arterial pulsatility indexes in normotensive and chronic hypertensive pregnant women: a prospective, longitudinal study.

    PubMed

    Guedes-Martins, Luís; Graça, Helena; Saraiva, Joaquim P; Guedes, Luísa; Gaio, Rita; Cerdeira, Ana S; Macedo, Filipe; Almeida, Henrique

    2014-08-28

    Despite the known effects of neuraxial blockade on major vessel function and the rapid decrease in uterine vascular impedance, it is unclear how the blockade affects the utero-placental circulation in the near-term. We hypothesize that among women with chronic hypertension, a loss of sympathetic tonus consequent to spinal block may cause significant changes in the utero-placental haemodynamics than the changes typical in normal pregnant women. Therefore, the main study objective was to analyse the effect of spinal anaesthesia for caesarean section on uterine and umbilical arterial impedance in pregnant women at term diagnosed with stage-1 chronic hypertension. A prospective, longitudinal study was performed in singleton pregnant women (203 low-risk and 33 with hypertension) scheduled to undergo elective caesarean section. The mean arterial blood pressure and pulsatility indexes for the uterine and umbilical arteries were recorded before and after spinal anaesthesia was performed using 8-9 mg hyperbaric bupivacaine (5 mg/mL) and 2-2.5 μg sufentanil (5 μg/mL). Multiple linear regression models with errors capable of correlation or with unequal variances were fitted using the generalized least squares. In normotensive women, the mean arterial blood pressure decreased after administering spinal anaesthesia (p < 0.05). The pulsatility index of the uterine and umbilical arteries did not change after spinal anaesthesia. In the hypertensive women, the mean arterial blood pressure (p < 0.05) and uterine artery pulsatility index (p < 0.05) decreased. In both groups, the umbilical artery pulsatility index did not change after spinal anaesthesia. In stage-1 chronic hypertensive pregnant women at term, spinal anaesthesia for caesarean section reduces uterine artery impedance but not umbilical artery impedance.

  20. Preoperative splenic artery embolization in klippel-Trenaunay syndrome with massive splenomegaly: A case report.

    PubMed

    Zhang, Zishu; Dasika, Narasimham L; Englesbe, Michael J; Owens, Scott R; Vellody, Ranjith; Novelli, Paula M; Shields, James J

    2014-04-01

    The authors describe a case of Klippel-Trenaunay syndrome (KTS) with massive splenomegaly in a 29-year-old woman. Preoperative splenic artery embolization using the "double embolization technique" (a combination of distal selective splenic artery embolization and proximal splenic artery occlusion) facilitated open splenectomy.

  1. Embolization in a Patient with Ruptured Anterior Inferior Pancreaticoduodenal Arterial Aneurysm with Median Arcuate Ligament Syndrome

    SciTech Connect

    Ogino, Hiroyuki; Sato, Yozo; Banno, Tatsuo; Arakawa, Toshinao; Hara, Masaki

    2002-08-15

    In median arcuate ligament syndrome, the root of the celiac artery is compressed and narrowed by the median arcuate ligament of the diaphragm during expiration, causing abdominal angina.Aneurysm may be formed in arteries of the pancreas and duodenum due toa chronic increase in blood flow from the superior mesenteric artery into the celiac arterial region. We report a patient saved by embolization with coils of ruptured aneurysm that developed with markedly dilated anterior inferior pancreaticoduodenal artery due to median arcuate ligament syndrome.

  2. Pulmonary arterial hypertension (ascites syndrome) in broilers: a review.

    PubMed

    Wideman, R F; Rhoads, D D; Erf, G F; Anthony, N B

    2013-01-01

    Pulmonary arterial hypertension (PAH) syndrome in broilers (also known as ascites syndrome and pulmonary hypertension syndrome) can be attributed to imbalances between cardiac output and the anatomical capacity of the pulmonary vasculature to accommodate ever-increasing rates of blood flow, as well as to an inappropriately elevated tone (degree of constriction) maintained by the pulmonary arterioles. Comparisons of PAH-susceptible and PAH-resistant broilers do not consistently reveal differences in cardiac output, but PAH-susceptible broilers consistently have higher pulmonary arterial pressures and pulmonary vascular resistances compared with PAH-resistant broilers. Efforts clarify the causes of excessive pulmonary vascular resistance have focused on evaluating the roles of chemical mediators of vasoconstriction and vasodilation, as well as on pathological (structural) changes occurring within the pulmonary arterioles (e.g., vascular remodeling and pathology) during the pathogenesis of PAH. The objectives of this review are to (1) summarize the pathophysiological progression initiated by the onset of pulmonary hypertension and culminating in terminal ascites; (2) review recent information regarding the factors contributing to excessively elevated resistance to blood flow through the lungs; (3) assess the role of the immune system during the pathogenesis of PAH; and (4) present new insights into the genetic basis of PAH. The cumulative evidence attributes the elevated pulmonary vascular resistance in PAH-susceptible broilers to an anatomically inadequate pulmonary vascular capacity, to excessive vascular tone reflecting the dominance of pulmonary vasoconstrictors over vasodilators, and to vascular pathology elicited by excessive hemodynamic stress. Emerging evidence also demonstrates that the pathogenesis of PAH includes characteristics of an inflammatory/autoimmune disease involving multifactorial genetic, environmental, and immune system components. Pulmonary

  3. Percutaneous Transluminal Angioplasty and Stent Placement for Subclavian and Brachiocephalic Artery Stenosis inAortitis Syndrome

    SciTech Connect

    Nomura, Motohiro; Kida, Sinya; Yamashima, Tetsumori; Yamashita, Junkoh; Yoshikawa, Jun; Matsui, Osamu

    1999-09-15

    A 43-year-old man with progressive right common carotid, subclavian artery, and brachiocephalic artery stenoses due to aortitis syndrome is presented. The patient's right common carotid artery had been treated by percutaneous transluminal angioplasty (PTA) four times previously, but it was finally occluded. The right subclavian artery was treated by PTA once, which resulted in restenosis. The stenosis extended to the brachiocephalic artery. For this patient, PTA followed by stent placement was performed for the right subclavian and brachiocephalic artery stenosis. Because arterial stenosis is progressive in cases of aortitis syndrome, simple PTA alone does not appear to be sufficient for treatment. We suggest that PTA followed by stent placement may be an alternative treatment for recurrent stenosis in aortitis syndrome.

  4. Percutaneous transluminal angioplasty and stent placement for subclavian and brachiocephalic artery stenosis in aortitis syndrome

    SciTech Connect

    Nomura, Motohiro; Kida, Sinya; Yamashima, Tetsumori; Yamashita, Junkoh; Yoshikawa, Jun; Matsui, Osarru

    1999-09-15

    A 43-year-old man with progressive right common carotid, subclavian artery, and brachiocephalic artery stenoses due to aortitis syndrome is presented. The patient's right common carotid artery had been treated by percutaneous transluminal angioplasty (PTA) four times previously, but it was finally occluded. The right subclavian artery was treated by PTA once, which resulted in restenosis. The stenosis extended to the brachiocephalic artery. For this patient, PTA followed by stent placement was performed for the right subclavian and brachiocephalic artery stenosis. Because arterial stenosis is progressive in cases of aortitis syndrome, simple PTA alone does not appear to be sufficient for treatment. We suggest that PTA followed by stent placement may be an alternative treatment for recurrent stenosis in aortitis syndrome.

  5. Non-traumatic adhesive arachnoiditis as a cause of spinal cord syndromes. Investigation of 507 patients.

    PubMed

    Jenik, F; Tekle-Haimanot, R; Hamory, B H

    1981-01-01

    Spinal cord syndromes with a mainly syringomyelic pattern of sensory diorders, radiculopathies, mixed paresis of varying degree (without any history of trauma), have been found in 507 out of 1305 new patients referred to out Clinic from January 1976 till 31 October 1977. In 105 randomised and unselected cases with these syndromes, myelographies have disclosed findings compatible with an adhesive spinal and/or cisternal arachnoiditis. A prospective study of the syndromes for evidence of infectious aetiology has been performed, in which tuberculosis, syphilis and other infections appear to be causative agents. A randomised therapeutic trial on a limited number of cases has been evaluated, as well as the results of specific therapy in a larger number of cases. Results of treatment have not been satisfactory. Operations were performed on only five patients and in no case was an autopsy obtained. Spinal cord syndromes due to non-traumatic adhesive arachnoiditis are discussed. The possible pathogenetic mechanisms the predominantly syringomyelic sensory deficits in those syndromes are briefly mentioned.

  6. Ischemic Bowel Syndrome in Patients with Spinal Cord Injury: A Nationwide Study

    PubMed Central

    Tseng, Chih-Wei; Lin, Cheng-Li; Chen, Yu-Tso; Jeng, Long-Bin

    2017-01-01

    Purpose The aim of this study was to determine whether spinal cord injuries (SCI) is associated with increased risk of ischemic bowel syndrome (IBS) in an Asian population by analyzing data from the National Health Insurance Research Database (NHIRD) in Taiwan. Methods Patients aged ≥20 years in the inpatient database with newly identified SCI from 2000 to 2011 were selected as the SCI cohort. For the non-SCI cohort, patients were selected based on a 1:4 risk-set sampling. Hospitalization with a new diagnosis of IBS during the follow-up was the main outcome measure. We used the standard univariable and multivariable Cox proportional hazard regression models to determine adjusted subhazard ratios (SHR) and 95% confidence interval (CI) in the SCI and non-SCI cohorts. Results Patients with SCI were at significant risk for IBS, with an adjusted SHR (aSHR) of 1.25, 95% CI = 1.04–1.51. Multivariable analysis showed individuals with SCI were associated with a greater risk of IBS than individuals without SCI among males (aSHR = 1.47, 95% CI = 1.16–1.86), all age groups (≤49 y: aSHR = 2.15, 95% CI = 1.24–3.74; 50–65 y: aSHR = 1.82, 95% CI = 1.15–2.88; >65 y: aSHR = 1.39, 95% CI = 1.11–1.74) and those without comorbidities (aSHR = 1.41, 95% CI = 1.04–1.93). Comorbidities including diabetes, hypertension, heart failure, coronary artery disease (CAD), Stroke, and end stage renal disease (ESRD) significantly increased the risk of IBS. Conclusion Patients hospitalized for SCI have increased risks of developing IBS. Though the mechanism that predisposes SCI patients to IBS is unclear, we suggest that physicians promptly identify and treat correctable risk factors. PMID:28056095

  7. Two cases of Brown-Séquard syndrome in penetrating spinal cord injuries.

    PubMed

    Amendola, L; Corghi, A; Cappuccio, M; De Iure, F

    2014-01-01

    Brown-Séquard syndrome due to a stab injuries is uncommon and results from a lesion in one half of the spinal cord. The role of surgery in the treatment of penetrating spinal injury often remain controversial. To discuss the current diagnostic and therapeutic approach for these types of injuries. The Authors describe two rare cases of Brown-Séquard syndrome due to civilian stab injuries differently treated. Mechanism of damage, clinical features and neurological outcome are reported. The recovery of neurological function in the first case indicates that the spinal tracts were injured by a contusion, rather than by a direct injury as in the second case. Moreover, surgery was required in the second patient to remove the weapon and to stabilize the spine, presenting bony and ligamentous instability. The diagnostic and therapeutic management are debated. An overview on clinical research in sperimental medical treatment of spinal cord injury was considered to evaluate future possible approaches to these injuries. As the neurologic improvement depends on the type and severity of the spinal cord damage, the indications for acute surgical management are limited and conservative management should be preferred.

  8. [Chemolysis of intravertebral disk hernia and spinal syndrome].

    PubMed

    Richard, O; Vallée, C; Chevrot, A; Hilliquin, P; Menkès, C J; L'Huillier, F; Rousselin, B; Dupont, A M; Godefroy, D

    1993-04-01

    The authors report a 31 year old patient who underwent L5-S1 chemonucleolysis. The prenucleolysis discogram disclosed intravertebral discal displacement. Severe spinal symptoms and development of geodes in the endplates adjacent to the treated disk occurred after the procedure. This case suggests that intraosseous discal displacement may be a risk factor for postchymopapain nucleolysis discitis and may contraindicate chemonucleolysis.

  9. Spinal cord infarction remote from maximal compression in a patient with Morquio syndrome.

    PubMed

    Tong, Calvin K W; Chen, James C H; Cochrane, D Douglas

    2012-06-01

    Morquio syndrome, or mucopolysaccharidosis type IV, is a rare enzyme deficiency disorder and results in skeletal dysplasia. Odontoid dysplasia is common among affected patients, resulting in atlantoaxial instability and spinal cord compression. Surgical treatments include decompression and prophylactic fusion, during which intraoperative neuromonitoring is important to alert the surgical team to changes in cord function so that they can prevent or mitigate spinal cord injury. This report describes a 16-year-old girl with Morquio syndrome who developed paraplegia due to thoracic spinal cord infarction during foramen magnum and atlantal decompression. This tragic event demonstrates the following: 1) that patients with Morquio syndrome are at risk for ischemic spinal cord injury at levels remote from areas of maximal anatomical compression while under anesthesia in the prone position, possibly due to impaired cardiac output; 2) the significance of absent motor evoked potential responses in the lower limbs with preserved upper-limb responses in an ambulatory patient; 3) the importance of establishing intraoperative neuromonitoring baseline assessments prior to turning patients to the prone position following induction of anesthesia; and 4) the importance of monitoring cardiac output during prone positioning in patients with chest wall deformity.

  10. Reversible hypertensive encephalomyelopathy - the spinal variant of the posterior reversible encephalopathy syndrome.

    PubMed

    Wu, Teddy Y; Wei, Diana Y; Jordan, Anthony; Kenedi, Chris; Smith, Andrew D; Kilfoyle, Dean H

    2015-05-01

    Posterior reversible encephalopathy syndrome (PRES) is characterised clinically by encephalopathy, headache, visual disturbance and/or focal neurological symptoms. Bilateral cerebral oedema on T2 MRI sequences within the posterior cerebral white matter is the radiological hallmark, although involvement of the frontal lobe, basal ganglia and brainstem can occur. PRES with spinal cord involvement has been rarely reported and is under-recognised due to lack of myelopathic features in nearly half of the reported cases. We report a patient with PRES with spinal cord involvement and review the literature.

  11. [Double traumatic cervical spine lesion (odontoid fracture and spinal cord injury) and Klippel-Feil syndrome].

    PubMed

    Graillon, T; Pech-Gourg, G; Adetchessi, T; Metellus, P; Dufour, H; Fuentes, S

    2012-12-01

    Klippel-Feil syndrome (KFS) is defined as a congenital fusion of at least two cervical vertebrae. Patients with KFS are known to be at high risk for spinal cord injury in case of cervical trauma even with weak kinetic. We report the case of a patient with C4-C5 and C6-C7 congenital fusion, harbouring C5-C6 post-traumatic spinal cord injury, associated with an odontoid fracture type 2 of Anderson and D'Alonzo classification following a motorbike accident.

  12. Spontaneous Spinal Epidural Hematoma Coexisting Guillan-Barré Syndrome in a Child: A Case Report.

    PubMed

    Lee, Chi Hyung; Song, Geun Sung; Kim, Young Ha; Son, Dong Wuk; Lee, Sang Weon

    2016-09-01

    Spontaneous spinal epidural hematoma (SSEH) has been reported as a rare cause of spinal cord compression, especially in children. Clinical features are usually nonspecific, although cervicothoracic location of hematoma could be presented with progressive paraplegia. Guillian-Barré syndrome (GBS) is clinically defined as an acute peripheral neuropathy causing progressive limb weakness. Because SSEH and GBS have very similar signs and symptoms, SSEH could be misdiagnosed as GBS. Nevertheless, they can be presented together. We describe a rare case of SSEH coexisting with GBS.

  13. Spontaneous Spinal Epidural Hematoma Coexisting Guillan-Barré Syndrome in a Child: A Case Report

    PubMed Central

    Lee, Chi Hyung; Kim, Young Ha; Son, Dong Wuk; Lee, Sang Weon

    2016-01-01

    Spontaneous spinal epidural hematoma (SSEH) has been reported as a rare cause of spinal cord compression, especially in children. Clinical features are usually nonspecific, although cervicothoracic location of hematoma could be presented with progressive paraplegia. Guillian-Barré syndrome (GBS) is clinically defined as an acute peripheral neuropathy causing progressive limb weakness. Because SSEH and GBS have very similar signs and symptoms, SSEH could be misdiagnosed as GBS. Nevertheless, they can be presented together. We describe a rare case of SSEH coexisting with GBS. PMID:27800000

  14. A 12-year-old girl with absent radial pulse: arterial thoracic outlet syndrome with subclavian artery aneurysm and thrombosis of the brachial artery.

    PubMed

    Schroeder, S; Cannizzaro, E; Kellenberger, C J; Saurenmann, R K

    2012-11-01

    Brachial arterial occlusion is rare in children and adolescents. Once a traumatic cause is excluded, the differential diagnosis consists of a variety of rare conditions. We report the case of a 12-year-old girl whose presenting symptoms--an absent radial pulse and Raynaud's phenomenon of the right hand--could be easily mistaken for a vasculitis. She was found to have arterial thoracic outlet syndrome with right subclavian artery compression and aneurysm formation caused by an anomalous first rib and consecutive thromboembolic occlusion of the brachial artery. The diagnosis and differential diagnosis of this condition are reviewed.

  15. Pramipexole Responsive Neck Numbness: The Therapeutic Role of Dopamine Agonists in the Spinal Cord Indicating to a Common Spinal Pathophysiology with Restless Leg Syndrome (RLS)?

    PubMed

    Yulug, Burak; Hanoglu, Lütfü

    2016-01-01

    There is still speculative data regarding the role of spinal dopaminergic neurotransmission in restless leg syndrome (RLS). We evaluated the therapeutic role of pramipexole in a patient with cervical disc prolapsus who exceptionally presented with neck restlessness. We have found that pramipexole leads to a significant improvement in restlessness symptoms in the neck region. The therapeutic role of pramipexole may not only suggest secondary deficits due to spinal pathologies but also indicate that specialized spinal dopaminergic neurons may play an important role in the pathogenesis of region specific restlessness symptomatology.

  16. Descending aorta-external iliac artery bypass for middle aortic syndrome.

    PubMed

    Okamoto, Yuki; Yamamoto, Kazuo; Sugimoto, Tsutomu; Asami, Fuyuki; Nagasawa, Ayako; Shiraiwa, Satoru; Nakamura, Norihito; Yoshii, Shinpei

    2014-11-01

    We encountered a surgical case of middle aortic syndrome (MAS) in a 56-year-old man who had resistant hypertension. Computed tomography showed severe stenosis of the abdominal aorta from below the superior mesenteric artery to above the inferior mesenteric artery. Although bilateral renal artery stenosis was confirmed, renal function was within normal limits. A 10-mm vascular prosthetic graft was used to perform a descending aorta to left external iliac artery bypass. His hypertension was well controlled without medication. This extra-anatomic bypass may be a simple and useful approach for treating MAS if it is not necessary to reconstruct the renal artery or visceral artery.

  17. Spinal cord injury-induced immune deficiency syndrome enhances infection susceptibility dependent on lesion level.

    PubMed

    Brommer, Benedikt; Engel, Odilo; Kopp, Marcel A; Watzlawick, Ralf; Müller, Susanne; Prüss, Harald; Chen, Yuying; DeVivo, Michael J; Finkenstaedt, Felix W; Dirnagl, Ulrich; Liebscher, Thomas; Meisel, Andreas; Schwab, Jan M

    2016-03-01

    Pneumonia is the leading cause of death after acute spinal cord injury and is associated with poor neurological outcome. In contrast to the current understanding, attributing enhanced infection susceptibility solely to the patient's environment and motor dysfunction, we investigate whether a secondary functional neurogenic immune deficiency (spinal cord injury-induced immune deficiency syndrome, SCI-IDS) may account for the enhanced infection susceptibility. We applied a clinically relevant model of experimental induced pneumonia to investigate whether the systemic SCI-IDS is functional sufficient to cause pneumonia dependent on spinal cord injury lesion level and investigated whether findings are mirrored in a large prospective cohort study after human spinal cord injury. In a mouse model of inducible pneumonia, high thoracic lesions that interrupt sympathetic innervation to major immune organs, but not low thoracic lesions, significantly increased bacterial load in lungs. The ability to clear the bacterial load from the lung remained preserved in sham animals. Propagated immune susceptibility depended on injury of central pre-ganglionic but not peripheral postganglionic sympathetic innervation to the spleen. Thoracic spinal cord injury level was confirmed as an independent increased risk factor of pneumonia in patients after motor complete spinal cord injury (odds ratio = 1.35, P < 0.001) independently from mechanical ventilation and preserved sensory function by multiple regression analysis. We present evidence that spinal cord injury directly causes increased risk for bacterial infection in mice as well as in patients. Besides obvious motor and sensory paralysis, spinal cord injury also induces a functional SCI-IDS ('immune paralysis'), sufficient to propagate clinically relevant infection in an injury level dependent manner.

  18. Spinal cord injury-induced immune deficiency syndrome enhances infection susceptibility dependent on lesion level

    PubMed Central

    Brommer, Benedikt; Engel, Odilo; Kopp, Marcel A.; Watzlawick, Ralf; Müller, Susanne; Prüss, Harald; Chen, Yuying; DeVivo, Michael J.; Finkenstaedt, Felix W.; Dirnagl, Ulrich; Liebscher, Thomas; Meisel, Andreas

    2016-01-01

    Pneumonia is the leading cause of death after acute spinal cord injury and is associated with poor neurological outcome. In contrast to the current understanding, attributing enhanced infection susceptibility solely to the patient’s environment and motor dysfunction, we investigate whether a secondary functional neurogenic immune deficiency (spinal cord injury-induced immune deficiency syndrome, SCI-IDS) may account for the enhanced infection susceptibility. We applied a clinically relevant model of experimental induced pneumonia to investigate whether the systemic SCI-IDS is functional sufficient to cause pneumonia dependent on spinal cord injury lesion level and investigated whether findings are mirrored in a large prospective cohort study after human spinal cord injury. In a mouse model of inducible pneumonia, high thoracic lesions that interrupt sympathetic innervation to major immune organs, but not low thoracic lesions, significantly increased bacterial load in lungs. The ability to clear the bacterial load from the lung remained preserved in sham animals. Propagated immune susceptibility depended on injury of central pre-ganglionic but not peripheral postganglionic sympathetic innervation to the spleen. Thoracic spinal cord injury level was confirmed as an independent increased risk factor of pneumonia in patients after motor complete spinal cord injury (odds ratio = 1.35, P < 0.001) independently from mechanical ventilation and preserved sensory function by multiple regression analysis. We present evidence that spinal cord injury directly causes increased risk for bacterial infection in mice as well as in patients. Besides obvious motor and sensory paralysis, spinal cord injury also induces a functional SCI-IDS (‘immune paralysis’), sufficient to propagate clinically relevant infection in an injury level dependent manner. PMID:26754788

  19. Mini-open spinal column shortening for the treatment of adult tethered cord syndrome.

    PubMed

    Safaee, Michael M; Winkler, Ethan A; Chou, Dean

    2017-10-01

    Tethered cord syndrome (TCS) is a challenging entity characterized by adhesions at the caudal spinal cord that prevent upward movement during growth and result in stretching of the cord with a concomitant constellation of neurologic symptoms. Although growth in height stops in adulthood, some patients still develop progressive symptoms; many underwent detethering as a child or adolescent, resulting in significant scar tissue and re-tethering. Recent strategies have focused on spinal column shortening to reduce tension on the spinal cord without exposing the previous de-tethering site. Mini-open and minimally invasive approaches avoid the large dissection and exposure associated with traditional approaches and are associated with reduced blood loss, shorter hospital stay, and similar outcomes when compared to conventional open approaches. We describe a technique for mini-open spinal column shortening. Using intraoperative navigation pedicle screws were placed at T10, T11, L1, and L2. A mini-open 3-column "egg shell" decancellation osteotomy of T12 was performed through a transpedicular approach with preservation of the superior and inferior endplates. This procedure was performed on a 28year old male with recurrent TCS and neurogenic bladder. Postoperative imaging showed a reduction in spinal column length of 1.5cm and evidence of decreased tension on the spinal cord. At last follow-up he was recovering well with improved urinary function. Spinal column shortening for adult TCS can be safely achieved through a mini-open approach. Future studies should compare the efficacy of this technique to both traditional de-tethering and open spinal column shortening. Copyright © 2017 Elsevier Ltd. All rights reserved.

  20. Identification of the feeding arteries of spinal vascular lesions via phase-contrast MR angiography with three-dimensional acquisition and phase display.

    PubMed

    Mascalchi, M; Quilici, N; Ferrito, G; Mangiafico, S; Scazzeri, F; Torselli, P; Petruzzi, P; Cosottini, M; Tessa, C; Bartolozzi, C

    1997-02-01

    To determine whether identification of the feeding arteries of spinal vascular lesions with phase-contrast MR angiography benefits from the higher spatial resolution of three-dimensional (volume) acquisitions and flow-direction information provided by the phase reconstruction of two-dimensional acquisitions. Fifteen patients with high- or low-flow spinal vascular lesions proved by spinal arteriography underwent MR angiography with phase-contrast techniques. Arteriographic and MR angiographic studies were reviewed to identify the arterial feeders of spinal vascular lesions. On modulus reconstructions of coronal 2-D or 3-D acquisitions, three of four arteries feeding high-flow lesions and three of 14 arteries feeding low-flow lesions were identified as hypertrophic vessels joining the parent intercostal or cervical arteries. Of 11 intradural veins draining dural arteriovenous fistulas, three were identified on coronal 2-D acquisitions and six on coronal 3-D acquisitions as vessels that coursed from a neural foramen to a midline tangle of vessels. Phase reconstruction showed ascending and descending flow patterns in two patients with intramedullary arteriovenous malformations, and diverging flow in perimedullary veins draining a hemangioblastoma. In nine patients with dural arteriovenous fistulas, phase reconstruction provided information as to the level of the arterial feeders. Phase reconstruction in coronal plane acquisitions also provided evidence of centripetal flow. Three-dimensional acquisitions and phase display of 2-D acquisitions improved the visibility of arterial pedicles of spinal vascular lesions at phase-contrast MR angiography.

  1. Skin vasomotor hemiparesis followed by overactivity: characteristic thermography findings in a patient with Horner syndrome due to spinal cord infarction.

    PubMed

    Kobayashi, Makoto

    2016-04-01

    We present a 21-year-old female with Horner syndrome due to spinal cord infarction. In this patient, infrared thermography revealed a hemibody skin temperature increase followed by excessive focal decreases, indicating skin vasomotor hemiparesis and overactivity.

  2. Aging syndrome genes and premature coronary artery disease

    PubMed Central

    Low, Adrian F; O'Donnell, Christopher J; Kathiresan, Sekar; Everett, Brendan; Chae, Claudia U; Shaw, Stanley Y; Ellinor, Patrick T; MacRae, Calum A

    2005-01-01

    Background Vascular disease is a feature of aging, and coronary vascular events are a major source of morbidity and mortality in rare premature aging syndromes. One such syndrome is caused by mutations in the lamin A/C (LMNA) gene, which also has been implicated in familial insulin resistance. A second gene related to premature aging in man and in murine models is the KLOTHO gene, a hypomorphic variant of which (KL-VS) is significantly more common in the first-degree relatives of patients with premature coronary artery disease (CAD). We evaluated whether common variants at the LMNA or KLOTHO genes are associated with rigorously defined premature CAD. Methods We identified 295 patients presenting with premature acute coronary syndromes confirmed by angiography. A control group of 145 patients with no evidence of CAD was recruited from outpatient referral clinics. Comprehensive haplotyping of the entire LMNA gene, including the promoter and untranslated regions, was performed using a combination of TaqMan® probes and direct sequencing of 14 haplotype-tagging single nucleotide polymorphisms (SNPs). The KL-VS variant of the KLOTHO gene was typed using restriction digest of a PCR amplicon. Results Two SNPs that were not in Hardy Weinberg equilibrium were excluded from analysis. We observed no significant differences in allele, genotype or haplotype frequencies at the LMNA or KLOTHO loci between the two groups. In addition, there was no evidence of excess homozygosity at the LMNA locus. Conclusion Our data do not support the hypothesis that premature CAD is associated with common variants in the progeroid syndrome genes LMNA and KLOTHO. PMID:16262891

  3. Pulmonary artery thrombus and subcapsular liver hematoma in a patient with HELLP syndrome: a therapeutic conundrum.

    PubMed

    Calderon, Eduardo G; Khawar, Sarwat; Cunningham, Jennifer A; Russell, Lori D; Alpert, Martin A

    2002-03-01

    HELLP syndrome (hemolysis, elevation of liver enzymes, and low platelet count) occurs during pregnancy. Intrahepatic hemorrhage and subcapsular liver hematoma with or without rupture are reported complications of this syndrome. The patient described in this report developed HELLP syndrome associated with a subcapsular liver hematoma and pulmonary artery thrombus, complications that created a therapeutic conundrum.

  4. Quantitative anatomy of vertebral artery groove on the posterior arch of atlas in relation to spinal surgical procedures.

    PubMed

    Gupta, Tulika

    2008-05-01

    The location of the vertebral artery on a groove on the superior surface of the posterior arch of atlas makes it vulnerable to injury during surgical procedures in this region. Knowledge of the quantitative anatomy of the vertebral artery groove is therefore necessary. In 55 dry adult atlas vertebrae, the distance of the medial edges of the vertebral artery groove were measured from the posterior midline at both the inner and outer cortices of the posterior arch of atlas. In addition, the distance between the vertebral artery grooves on either side as well as the length of the vertebral artery groove was also measured. It was found that a minimum of 1.5 cm of the posterior arch could be safely exposed at both the outer and inner cortices. In addition, with mobilization of the vertebral artery from its groove on both the sides, an additional 1 cm of posterior arch could be exposed on either side. Exposure of the posterior arch of the atlas is an important step in surgical procedures for treatment of diverse conditions of the upper spinal cord and foramen magnum region. Injury to the vertebral artery in its position on the vertebral groove may lead to disastrous complications. The present study reveals that the neurosurgeon can safely expose up to 3.5 cm of the posterior arch of atlas and knowledge of this anatomic fact may help in planning surgical approaches.

  5. Spinal fusion in girls with Rett syndrome: postoperative recovery and family experiences

    PubMed Central

    Marr, Caitlin; Leonard, Helen; Torode, Ian; Downs, Jenny

    2015-01-01

    Background Rett syndrome is a severe neurodevelopmental disorder mainly affecting females and scoliosis is a common comorbidity. Spinal fusion may be recommended if the scoliosis is progressive. This qualitative study investigated recovery of girls with Rett syndrome during the first 12 postoperative months and explored family perspectives and coping around the time of surgery. Method Parents registered with the population-based Australian Rett Syndrome Database were recruited to this study if their daughter had a confirmed pathogenic MECP2 mutation and spinal fusion between 2006 and 2012. Twenty-five interviews were conducted to determine their daughter’s recovery and parental stresses and coping. Themes in the interview data were identified with content analysis and the regaining of gross motor skills over the first 12 postoperative months were described with time-to-event (survival) analysis. Results Pain and energy levels, appetite, mood and coinciding health issues influenced their daughter’s postoperative recovery. The majority of girls recovered preoperative sitting (88%), standing (81%) and walking (80%) by 12 months. The decision to proceed with surgery was associated with feelings of fear, obligation, relief and guilt for families. Development of complications, poor support and feelings of isolation increased their emotional burden whilst adequate information and discharge preparation, confidence in self and staff, and balancing personal needs with their daughter’s care relieved this burden. Interpretation Our study identified clinical practice issues in relation to families whose daughter with Rett syndrome undergoes spinal fusion, issues that are also relevant to other severe disabilities. Return of wellness and gross motor skills following spinal fusion in girls with Rett syndrome occurred within the first 12 postoperative months in most cases. Parents require information and practical support to alleviate their emotional burden. PMID:25752500

  6. Spinal fusion in girls with Rett syndrome: post-operative recovery and family experiences.

    PubMed

    Marr, C; Leonard, H; Torode, I; Downs, J

    2015-11-01

    Rett syndrome is a severe neurodevelopmental disorder mainly affecting females and scoliosis is a common co-morbidity. Spinal fusion may be recommended if the scoliosis is progressive. This qualitative study investigated recovery of girls with Rett syndrome during the first 12 post-operative months and explored family perspectives and coping around the time of surgery. Parents registered with the population-based Australian Rett Syndrome Database were recruited to this study if their daughter had a confirmed pathogenic MECP2 mutation and spinal fusion between 2006 and 2012. Twenty-five interviews were conducted to determine their daughter's recovery and parental stresses and coping. Themes in the interview data were identified with content analysis, and the regaining of gross motor skills over the first 12 post-operative months was described with time-to-event (survival) analysis. Pain and energy levels, appetite, mood and coinciding health issues influenced their daughter's post-operative recovery. The majority of girls recovered preoperative sitting (88%), standing (81%) and walking (80%) by 12 months. The decision to proceed with surgery was associated with feelings of fear, obligation, relief and guilt for families. Development of complications, poor support and feelings of isolation increased their emotional burden whereas adequate information and discharge preparation, confidence in self and staff, and balancing personal needs with their daughter's care relieved this burden. Our study identified clinical practice issues in relation to families whose daughter with Rett syndrome undergoes spinal fusion, issues that are also relevant to other severe disabilities. Return of wellness and gross motor skills following spinal fusion in girls with Rett syndrome occurred within the first 12 post-operative months in most cases. Parents require information and practical support to alleviate their emotional burden. © 2015 John Wiley & Sons Ltd.

  7. Is anterior communicating artery syndrome related to fornix lesions?

    PubMed

    Molino, Ivana; Cavaliere, Carlo; Salvatore, Elena; Quarantelli, Mario; Colucci, Luisa; Fasanaro, Angiola Maria

    2014-01-01

    Anterior communicating artery (ACoA) syndrome, which may occur after rupture of ACoA aneurysms, consists of anterograde memory problems, executive dysfunctions, confabulations, and personality changes. Recently, the employment of diffusion tensor tractography (DTT) has related ACoA to microstructural lesions in the cingulum and the fornix, but an accurate characterization of these subjects should be provided. We report the clinical and neuropsychological findings of a patient who developed a severe and persistent amnesia together with significant behavioral changes, as well as her imaging results, where the sole evidence of brain damage was that of the fornix demonstrated by DTT. The four-year neuropsychological follow-up of the subject allows exclusion of other causes. This case demonstrates that microstructural lesions of fornix may lead to persistent amnesia, executive impairments, and behavioral changes and contributes to the knowledge of its role in cognition.

  8. Spinal anesthesia reduces postoperative delirium in opium dependent patients undergoing coronary artery bypass grafting.

    PubMed

    Tabatabaie, O; Matin, N; Heidari, A; Tabatabaie, A; Hadaegh, A; Yazdanynejad, S; Tabatabaie, K

    2015-01-01

    We investigated the effect of high spinal anesthesia on postoperative delirium in opium dependent patients undergoing coronary artery bypass grafting (CABG). The study was conducted in a tertiary referral university hospital on a population of 60 opium dependent patients undergoing CABG surgery. Patients were divided into two groups based on anesthesia protocol. One group were given general anesthesia (GA Group), the other group additionally received intrathecal morphine and bupivacaine (SGA Group). Postoperative delirium (POD) was defined as the main outcome of interest. Incidence of POD was significantly higher in patients of GA Group as compared with those in SGA Group (47% and 17% for GA and SGA respectively; P-value = 0.01). Time to extubation was on average 2.2 h shorter in SGA than in GA (7.1 h and 9.3 h respectively, P-value < 0.001). Intrathecal morphine and bupivacaine reduced the risk of POD after CABG in a population of opium dependent patients.

  9. Toxoplasmosis of spinal cord in acquired immunodeficiency syndrome patient presenting as paraparesis: a rare entity.

    PubMed

    Agrawal, Sachin R; Singh, Vinita; Ingale, Sheetal; Jain, Ajeet Prasad

    2014-10-01

    Although brain has been the most common site for toxoplasma infection in acquired immunodeficiency syndrome patients, involvement of spinal cord by toxoplasma has been rarely found. Spinal cord toxoplasmosis can present as acute onset weakness in both lower limbs associated with sensory and bladder dysfunction. A presumptive diagnosis can be made in patients with CD4 count <100/mm(3) based on a positive serum Toxoplasma gondii IgG antibodies, no recent prophylaxis against toxoplasmosis, intramedullary ring enhancing lesion in spinal cord supported by similar lesions in brain parenchyma. Institutions of antitoxoplasma treatment in such patients result in prompt clinical response and therefore avoiding the need of unnecessary invasive diagnostic tests. Here, we report a case of toxoplasmic myelitis in immunocompromised patient presenting as myelopathy who showed significant clinical improvement after starting antitoxoplasma treatment. Hence toxoplasmic myelitis should be considered in toxoplasma seropositive immunocompromised patients presenting as myelopathy and imaging studies showing ring enhancing intramedullary lesion.

  10. Mechanism of depression of the spinal pain syndrome by serotonin derivatives

    SciTech Connect

    Kryzhanovskii, G.N.; Lutsenko, N.G.; Grafova, V.N.; Danilova, E.I.; Lutsenko, V.K.; Gordeev, E.N.; Kalacheva, N.A.; Suvorov, N.N.

    1986-09-01

    The authors compare the ability of 5-HT derivatives to stimulate adenylate cyclase in the nervous system with their action on the intensity of the spinal pain syndrome (SPS), induced by the creation of a generator of pathologically enhanced excitation in the lumbar segments of the spinal cord of rats. An SPS was induced in rats by applying the sodium salt of benzylpenicillin to the dorsal surface of the lumbar segments of the spinal cord. The effect of 5-HT derivatives on activity of serotonin-sensitive adenylate cyclase was estimated from the change in cAMP concentration in the synaptosomes. During the experiments, the disintegrated synaptosomes were removed by centrifugation after which the cAMP concentration in the supernatant was determined by means of a kit of reagents for radioligand determination of cAMP. Radioactivity was measured on a scintillation spectrometer.

  11. Brown-Sequard syndrome associated with unusual spinal cord injury by a screwdriver stab wound

    PubMed Central

    Beer-Furlan, André Luiz; Paiva, Wellingson Silva; Tavares, Wagner Malagó; de Andrade, Almir Ferreira; Teixeira, Manoel Jacobsen

    2014-01-01

    Introduction: Stab wounds resulting in spinal cord injuries are very rare. In direct central back stabbings, the layers of muscles and the spinal column tends to deflect blades, rarely causing injuries to the spinal cord. We report an unusual case of traumatic spinal cord injury by a screwdriver stab, presented as Brown-Séquard syndrome and discuss possible pitfalls on the surgical treatment. Case report: A 34 year-old man was brought to the emergency department after a group assault with a single screwdriver stab wound on the back. Neurological examination revealed an incomplete Brown-Sequard syndrome, with grade IV motor deficit on the left leg and contralateral hemihypoalgesia below T9 level. Radiological evaluation showed a retained 9 cm screwdriver that entered and trespassed the spinal canal at T6 level, reaching the posterior mediastinum with close relation to the thoracic aorta. Vascular injury could not be excluded. The joint decision between the neurosurgery and the vascular surgery teams was the surgical removal of the screwdriver under direct visualization. A left mini-thoracotomy was performed. Simultaneously, a careful dissection was done and screwdriver was firmly pulled back on the opposite path of entry under direct visualization of the aorta. The neurological deficit was maintained immediately after the surgical procedure. Follow-up visit after 1 year showed minor motor deficit and good healing. Conclusions: It is important to consider all aspects of secondary injury on the surgical planning of penetrating spinal cord injury. The secondary injury can be minimized with multidisciplinary planning of the surgical procedure. PMID:24482724

  12. Brown-Sequard syndrome associated with unusual spinal cord injury by a screwdriver stab wound.

    PubMed

    Beer-Furlan, André Luiz; Paiva, Wellingson Silva; Tavares, Wagner Malagó; de Andrade, Almir Ferreira; Teixeira, Manoel Jacobsen

    2014-01-01

    Stab wounds resulting in spinal cord injuries are very rare. In direct central back stabbings, the layers of muscles and the spinal column tends to deflect blades, rarely causing injuries to the spinal cord. We report an unusual case of traumatic spinal cord injury by a screwdriver stab, presented as Brown-Séquard syndrome and discuss possible pitfalls on the surgical treatment. A 34 year-old man was brought to the emergency department after a group assault with a single screwdriver stab wound on the back. Neurological examination revealed an incomplete Brown-Sequard syndrome, with grade IV motor deficit on the left leg and contralateral hemihypoalgesia below T9 level. Radiological evaluation showed a retained 9 cm screwdriver that entered and trespassed the spinal canal at T6 level, reaching the posterior mediastinum with close relation to the thoracic aorta. Vascular injury could not be excluded. The joint decision between the neurosurgery and the vascular surgery teams was the surgical removal of the screwdriver under direct visualization. A left mini-thoracotomy was performed. Simultaneously, a careful dissection was done and screwdriver was firmly pulled back on the opposite path of entry under direct visualization of the aorta. The neurological deficit was maintained immediately after the surgical procedure. Follow-up visit after 1 year showed minor motor deficit and good healing. It is important to consider all aspects of secondary injury on the surgical planning of penetrating spinal cord injury. The secondary injury can be minimized with multidisciplinary planning of the surgical procedure.

  13. Horner's syndrome following cervical spinal surgery in the dog.

    PubMed

    Boydell, P

    1995-11-01

    Two dobermanns exhibited Horner's syndrome following the surgical treatment of cervical spondylopathy. Denervation hypersensitivity testing demonstrated a 2nd order lesion in both. Spontaneous resolution occurred within one month.

  14. Spinal column shortening for tethered cord syndrome associated with myelomeningocele, lumbosacral lipoma, and lipomyelomeningocele in children and young adults.

    PubMed

    Aldave, Guillermo; Hansen, Daniel; Hwang, Steven W; Moreno, Amee; Briceño, Valentina; Jea, Andrew

    2017-06-01

    OBJECTIVE Tethered cord syndrome is the clinical manifestation of an abnormal stretch on the spinal cord, presumably causing mechanical injury, a compromised blood supply, and altered spinal cord metabolism. Tethered cord release is the standard treatment for tethered cord syndrome. However, direct untethering of the spinal cord carries potential risks, such as new neurological deficits from spinal cord injury, a CSF leak from opening the dura, and retethering of the spinal cord from normal scar formation after surgery. To avoid these risks, the authors applied spinal column shortening to children and transitional adults with primary and secondary tethered cord syndrome and report treatment outcomes. The authors' aim with this study was to determine the safety and efficacy of spinal column shortening for tethered cord syndrome by analyzing their experience with this surgical technique. METHODS The authors retrospectively reviewed the demographic and procedural data of children and young adults who had undergone spinal column shortening for primary or secondary tethered cord syndrome. RESULTS Seven patients with tethered cord syndrome caused by myelomeningocele, lipomyelomeningocele, and transitional spinal lipoma were treated with spinal column shortening. One patient with less than 24 months of follow-up was excluded from further analysis. There were 3 males and 4 females; the average age at the time was surgery was 16 years (range 8-30 years). Clinical presentations for our patients included pain (in 5 patients), weakness (in 4 patients), and bowel/bladder dysfunction (in 4 patients). Spinal column osteotomy was most commonly performed at the L-1 level, with fusion between T-12 and L-2 using a pedicle screw-rod construct. Pedicle subtraction osteotomy was performed in 6 patients, and vertebral column resection was performed in 1 patient. The average follow-up period was 31 months (range 26-37 months). Computed tomography-based radiographic outcomes showed solid

  15. Spinal subdural hematoma with cauda equina syndrome: A complication of combined spinal epidural anesthesia

    PubMed Central

    Singhal, Neha; Sethi, Priyanka; Jain, Jitesh Kumar; Agarwal, Saurabh

    2015-01-01

    Combined spinal-epidural anesthesia (CSE) is considered safe in lower limb surgeries. We report a case of sudden neurological deterioration in a stable postoperative patient who was given CSE for total knee replacement and low molecular weight heparin in postoperative period. On the 4th postoperative day, she developed sudden onset weakness in left lower limb along with bladder incontinence. Magnetic resonance imaging spine revealed a subdural hematoma at L2-L3 level. Immediate laminectomy along with cord decompression was done and patient recovered well except for a persistent foot drop on left side. PMID:25948911

  16. Reversible stenosis of large cerebral arteries in a patient with combined Sjögren's syndrome and neuromyelitis optica spectrum disorder.

    PubMed

    Ii, Yuichiro; Shindo, Akihiro; Sasaki, Ryogen; Naito, Yutaka; Tanaka, Keiko; Kuzuhara, Shigeki

    2008-10-01

    We report a 49-year-old woman with neuromyelitis optica (NMO) spectrum disorder coexisting with Sjögren's syndrome (SS). She presented with acute brainstem symptoms and transverse myelitis. Brain MRI showed focal high signal intensity lesions in the hypothalamus and the pontine tegmentum on T2-weighted and FLAIR images. MRA revealed stenotic changes of the bilateral middle cerebral artery (MCA), posterior cerebral arteries (PCA) and basilar artery (BA). Spinal MRI revealed hyperintense lesions within the cord extending from the T4 to the T6 level on the T2-weighted image. The patient fulfilled the clinical criteria of primary SS. In addition, anti-AQP4 antibody which is highly specific for NMO was detected in the serum at the acute phase. The patient excellently responded to IVIg while methylprednisolon pulse therapy was not effective. Follow-up MRA displayed complete resolution of the stenosis of the MCA, PCA and BA.

  17. Pulmonary artery sarcoma detected on F-18 FDG PET/CT as origin of multiple spinal metastases.

    PubMed

    Chun, In Kook; Eo, Jae Seon; Paeng, Jin Chul; Kim, Dong Wan; Chung, June-Key; Lee, Dong Soo

    2011-08-01

    A 67-year-old man with back pain was diagnosed as having multiple spinal metastases on MRI. On CT scan, only a filling defect in the right pulmonary artery was observed and suspected as venous thromboembolism. On F-18 fluorodeoxyglucose (FDG) PET/CT, intense hypermetabolism was observed in the right pulmonary artery in addition to the metastatic spine lesions. Biopsy confirmed the lesion as a primary pulmonary artery sarcoma (PAS), and the spine lesions as metastases of PAS. Although PAS is rare and its bone metastasis presenting initial symptom is extremely rare, FDG PET/CT is an effective diagnostic modality for PAS, not only in discrimination from venous thromboembolism, but also in workup of metastatic origin.

  18. Common Iliac Artery Aneurysm and Spontaneous Dissection with Contralateral Iatrogenic Common Iliac Artery Dissection in Classic Ehlers–Danlos Syndrome

    PubMed Central

    Mehta, Sachin; Dhar, Shweta U.; Birnbaum, Yochai

    2012-01-01

    We describe a 43-year-old man who developed a spontaneous dissection of a right iliac artery aneurysm after performing vigorous physical exercise. Additionally, during peripheral intervention, the patient developed iatrogenic dissection of the left iliac artery. The patient had the characteristic physical findings of Ehlers–Danlos syndrome (EDS), classic type. Genetic testing revealed a mutation in the COL5A1 gene associated with EDS, classic type. Vascular aneurysms and dissections are characteristics of EDS vascular type, but not the classic type. Only one previous case with EDS, classic type with spontaneous iliac artery dissection has been described. PMID:23997563

  19. The effects of functional electrical stimulation leg ergometry training on arterial compliance in individuals with spinal cord injury

    PubMed Central

    Zbogar, Dominik; Eng, Janice J.; Krassioukov, Andrei V.; Scott, Jessica M.; Esch, Ben T.A.; Warburton, Darren E.R.

    2011-01-01

    Study design A prospective intervention of functional electrical stimulation leg cycle ergometry (FES-LCE) of four women with spinal cord injury (SCI). Objective To evaluate the effect of FES-LCE training on arterial compliance in individuals with chronic SCI of traumatic origin. Setting Tertiary rehabilitation center in Canada. Methods Large and small artery compliance were measured at the radial artery before and after a 3 month training program using FES-LCE. Results There was no significant change in large artery compliance after FES-LCE (16.0 ± 4.2 to 16.8 ± 6.1 mL/mmHg × 10, p = n.s.). There was a marked (63%) increase in small artery compliance after the FES training program (4.2 ± 1.8 to 6.9 ± 3.2 mL/mmHg × 100, p < 0.05). Conclusion It appears that FES-LCE is effective in improving small artery compliance in females with SCI. PMID:18414425

  20. Straight Back Syndrome: positive response to spinal manipulation and adjunctive therapy – A case report

    PubMed Central

    Gold, Paul M.; Albright, Brianna; Anani, Sabine; Toner, Heather

    2013-01-01

    Straight Back Syndrome (SBS) has been recognized for over 50 years. Not to be confused with flat back syndrome in the lumbar spine, SBS patients present with an obvious loss of the thoracic kyphosis accompanied by apparent heart symptoms. The main purpose of this article is to describe a patient diagnosed with SBS, whose symptoms were successfully managed using spinal manipulative therapy as well as ancillary modalities. The use of diagnostic and laboratory tests are essential to differentially diagnose cardiac disease from SBS. Genesis and incidence of this condition is also discussed as well as roentgenometric analysis. A suggested diagnostic algorithm is presented as well. PMID:23754859

  1. [Boy with Coffin-Lowry syndrome associated with spinal cord injuries].

    PubMed

    Kawana, Yuki; Okamura, Kenta; Kurahashi, Kiyoyasu

    2014-02-01

    A 12-year-old male patient with Coffin-Lowry syndrome was scheduled for posterior cervical decompression and fusion for cervical spinal injuries. The patient had features of Coffin-Lowry syndrome including mental retardation, prominent forehead, a short nose with a wide tip, a wide mouth with full lips, short stature, microcephaly, and kyphoscoliosis. We anticipated major troubles related to anesthesia such as difficult ventilation and intubation, communication difficulty during induction and extubation, and difficulty in using a naso-pharyngeal airway. In addition, we had to stabilize neck alignments during intubation because cervical vertebrae were unstable and spinal cord has already been injured. Therefore, we scheduled slow induction with sevoflurane maintaining spontaneous respiration. As we found the full mouth opening of the patient after the induction, we inserted an intubating laryngeal mask, through which ventilation was successfully maintained. A tracheal tube was inserted through the intubating laryngeal mask. When the surgery was completed, we extubated using a tube introducer in the trachea. As we found that the patient's airway was open, we removed the introducer. In conclusion, with a thorough planning of the anesthetic management, we successfully managed anesthesia for cervical spinal surgery in a patient with Coffin-Lowry syndrome.

  2. Coronary artery bypass surgery in a patient with Kartagener syndrome: a case report and literature review.

    PubMed

    Bougioukas, Ioannis; Mikroulis, Dimitrios; Danner, Bernhard; Lawal, Lukman; Eleftheriadis, Savvas; Bougioukas, George; Didilis, Vassilios

    2010-08-26

    Kartagener syndrome consists of congenital bronchiectasis, sinusitis, and total situs inversus in half of the patients. A patient diagnosed with Kartagener syndrome was referred to our department due to 3-vessel coronary disease. An off-pump coronary artery bypass operation was performed using both internal thoracic arteries and a saphenous vein graft. We performed a literature review for cases with Kartagener syndrome, coronary surgery and dextrocardia. Although a few cases of dextrocardia were found in the literature, no case of Kartagener syndrome was mentioned.

  3. The effects of changes in the metabolic syndrome detection status on arterial stiffening: a prospective study.

    PubMed

    Tomiyama, Hirofumi; Hirayama, Yoji; Hashimoto, Hideki; Yambe, Minoru; Yamada, Jiko; Koji, Yutaka; Motobe, Kohki; Shiina, Kazuki; Yamamoto, Yoshio; Yamashinai, Akira

    2006-09-01

    We conducted a prospective study to examine the effects of alterations of the metabolic syndrome detection status on the rate of progression of arterial stiffness, which is recognized as a marker of arterial damage and an indicator of cardiovascular risk. Brachial-ankle pulse wave velocity as an index of arterial stiffening was recorded twice over a 3-year period in 2080 Japanese men (age, 42 +/- 9 years). At the start of the prospective study, pulse wave velocity was higher in the subjects with metabolic syndrome (n=125) than in those without metabolic syndrome (n=1,955) even after adjusting for mean blood pressure. The annual rate of increase of the pulse wave velocity was higher in the group with persistent metabolic syndrome (27 +/- 51 cm/s/year, n=71) than in the group with regression of metabolic syndrome (6 +/- 39 cm/s/year, n=54) or the group in which metabolic syndrome was absent (13 +/- 37 cm/s/year, n=1843; p < 0.05) after adjustment for changes in blood pressure. In conclusion, the changes in the metabolic syndrome detection status of the subjects during the study period affected the annual rate of progression of arterial stiffening, and persistent metabolic syndrome during the study period was associated with acceleration of arterial stiffening in middle-aged Japanese men. On the other hand, resolution of metabolic syndrome may be associated with attenuation of the progression of arterial damage. Therefore, the increased cardiovascular risk associated with the presence of metabolic syndrome may be at least partly mediated by acceleration of the progression of arterial stiffening.

  4. Diagnostic evaluation, monitoring, and perioperative management of spinal cord compression in patients with Morquio syndrome.

    PubMed

    Charrow, Joel; Alden, Tord D; Breathnach, Catherine Ann R; Frawley, Geoffrey P; Hendriksz, Christian J; Link, Bianca; Mackenzie, William G; Manara, Renzo; Offiah, Amaka C; Solano, Martha L; Theroux, Mary

    2015-01-01

    Mucopolysaccharidosis IVA is an autosomal recessive condition caused by mutations in the GALNS gene, which encodes N-acetylgalactosamine-6-sulfatase, also called galactosamine-6-sulfatase (GALNS). A reduction in or absence of effective GALNS leads to faulty catabolism of keratan sulfate and chondroitin-6-sulfate within the lysosome; their accumulation causes cell, tissue, and organ dysfunction. The connective tissue, cartilage, ligaments, and bone of patients with Morquio A syndrome are particularly affected. Patients with Morquio A syndrome are at high risk of neurological complications because of their skeletal abnormalities; many patients are in danger of cervical myelopathy due to odontoid hypoplasia and ligamentous laxity leading to atlantoaxial subluxation. The multisystemic involvement of patients with Morquio A syndrome requires treatment by multidisciplinary teams; not all members of these teams may be aware of the potential for subluxation and quadriparesis. A multinational, multidisciplinary panel of 10 skeletal dysplasia or Morquio A syndrome specialists convened in Miami, FL on December 7 and 8, 2012 to develop consensus recommendations for early identification and effective management of spinal cord compression, for anesthesia and surgical best practices, and for effectual cardiac and respiratory management in patients with Morquio A syndrome. The target audience for these recommendations includes any physician who may encounter a patient with Morquio A syndrome, however doctors who do not have access to the full spectrum of specialists and resources needed to support patients with Morquio A syndrome should attempt to refer patients to a center that does. Physicians who manage Morquio A syndrome or comorbid conditions within specialty centers should review these expert panel recommendations and fully understand the implications of spinal cord instability for their own practices.

  5. The SAPHO syndrome revisited with an emphasis on spinal manifestations.

    PubMed

    Leone, Antonio; Cassar-Pullicino, Victor N; Casale, Roberto; Magarelli, Nicola; Semprini, Alessia; Colosimo, Cesare

    2015-01-01

    The synovitis, acne, pustulosis, hyperostosis, and osteitis (SAPHO) syndrome includes a group of chronic, relapsing, inflammatory musculoskeletal disorders with similar manifestations, in particular synovitis, hyperostosis, and osteitis, which may or may not be associated with neutrophilic skin eruptions such as palmoplantar pustulosis and acne conglobata. The syndrome occurs at any age, can involve any skeletal site, and its imaging appearances are variable, depending on the stage/age of the lesion and imaging method. The diagnosis is difficult if there is no skin disease. Awareness of the imaging appearances, especially in the spine, may help the radiologist in avoiding misdiagnosis (e.g., infection, tumor) and unnecessary invasive procedures, while facilitating early diagnosis and selection of an effective treatment. In this article, we provide an overview of the radiological appearances of SAPHO syndrome, focusing on the magnetic resonance imaging findings of vertebral involvement, and present relevant clinical and pathological features that assist early diagnosis.

  6. Subclavian artery aneurysm in a patient with vascular Ehlers-Danlos syndrome.

    PubMed

    Yasuda, Shota; Imoto, Kiyotaka; Uchida, Keiji; Uranaka, Yasuko; Kurosawa, Kenji; Masuda, Munetaka

    2016-02-01

    We describe our experience of surgical treatment in a 28-year-old woman with vascular Ehlers-Danlos syndrome. A right subclavian artery aneurysm was detected. The right vertebral artery arose from the aneurysm. Digital subtraction angiography showed interruption of the left vertebral artery. The aneurysm was excised and the right vertebral artery was anastomosed end-to-side to the right common carotid artery under deep hypothermia and circulatory arrest. The patient remained very well 4 years after surgery, with no late vascular complication.

  7. Posterior Reversible Encephalopathy Syndrome Occurring After Uterine Artery Embolization for Uterine Myoma

    SciTech Connect

    Suzuki, Satoshi Tanigawa, Noboru; Kariya, Syuji; Komemushi, Atsushi; Kojima, Hiroyuki; Tokuda, Takanori; Kishimoto, Masanobu; Tomino, Atsutoshi; Fujioka, Masayuki; Kitazawa, Yasuhide; Sawada, Satoshi

    2011-02-15

    This case report describes posterior reversible encephalopathy syndrome (PRES) occurring after uterine artery embolization (UAE) for uterine myoma. This is the first report of PRES occurring after uterine vascular radiologic intervention. The mechanism by which UAE induced PRES is unclear.

  8. Budd-chiari syndrome and renal arterial neurysms due to behcet disease: a rare association

    PubMed Central

    Batur, Abdussamet; Dorum, Meltem; Yüksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm. PMID:26491527

  9. Budd-chiari syndrome and renal arterial neurysms due to Behcet disease: a rare association.

    PubMed

    Batur, Abdussamet; Dorum, Meltem; Yüksekkaya, Hasan Ali; Koc, Osman

    2015-01-01

    Behcet's disease is a multisystemic vasculitis of unknown etiology with a chronic relapsing course. Vasculitis in Behcet's disease with predominant vascular involvement is the only vasculitis that affects both arteries and veins of any size. Involvement of the renal artery and inferior vena cava is rare among the arteries and veins, respectively. When disease affect the veins, it is in the form of thrombosis. Arterial complications include aneurysms, stenosis and occlusions. Both rupture of arterial aneurysm and occlusion of suprahepatic veins, causing Budd-Chiari syndrome, are associated with a high mortality rate. Vascular involvement is more common in male patients than in female patients. Men and patients with a younger age of onset present with a more severe prognosis. In this case report, we describe a very rare cause of intrarenal arterial aneurysm's rupture with previous Budd-Chiari syndrome due to Behcet's disease and successful angiographic embolization of actively bleeding aneurysm.

  10. Acute changes in arterial stiffness following exercise in people with metabolic syndrome.

    PubMed

    Radhakrishnan, Jeyasundar; Swaminathan, Narasimman; Pereira, Natasha M; Henderson, Keiran; Brodie, David A

    2016-08-22

    This study aims to examine the changes in arterial stiffness immediately following sub-maximal exercise in people with metabolic syndrome. Ninety-four adult participants (19-80 years) with metabolic syndrome gave written consent and were measured for arterial stiffness using a SphygmoCor (SCOR-PVx, Version 8.0, Atcor Medical Private Ltd, USA) immediately before and within 5-10min after an incremental shuttle walk test. The arterial stiffness measures used were pulse wave velocity (PWV), aortic pulse pressure (PP), augmentation pressure, augmentation index (AI), subendocardial viability ratio (SEVR) and ejection duration (ED). There was a significant increase (p<0.05) in most of the arterial stiffness variables following exercise. Exercise capacity had a strong inverse correlation with arterial stiffness and age (p<0.01). Age influences arterial stiffness. Exercise capacity is inversely related to arterial stiffness and age in people with metabolic syndrome. Exercise induced changes in arterial stiffness measured using pulse wave analysis is an important tool that provides further evidence in studying cardiovascular risk in metabolic syndrome. Copyright © 2016 Diabetes India. Published by Elsevier Ltd. All rights reserved.

  11. Stent dislodgement induced by a vasodilator used for severe coronary artery spasm caused by Kounis syndrome.

    PubMed

    Nishi, Masahiro; Zen, Kan; Kambayashi, Daisuke; Asada, Satoshi; Yamaguchi, Shinichiro; Tatsukawa, Hirotaka

    2016-01-01

    Coronary stent dislodgement is a rare but critical complication of percutaneous coronary intervention. It can potentially result in serious consequences, such as stent embolization and emergent coronary artery bypass graft surgery. Here, we describe the successful retrieval of an extracoronary dislodged stent, where dislodgement was induced by a vasodilator used for severe coronary artery spasm caused by Kounis syndrome.

  12. Aerobic exercise training reduces arterial stiffness in metabolic syndrome.

    PubMed

    Donley, David A; Fournier, Sara B; Reger, Brian L; DeVallance, Evan; Bonner, Daniel E; Olfert, I Mark; Frisbee, Jefferson C; Chantler, Paul D

    2014-06-01

    The metabolic syndrome (MetS) is associated with a threefold increase risk of cardiovascular disease (CVD) mortality partly due to increased arterial stiffening. We compared the effects of aerobic exercise training on arterial stiffening/mechanics in MetS subjects without overt CVD or type 2 diabetes. MetS and healthy control (Con) subjects underwent 8 wk of exercise training (ExT; 11 MetS and 11 Con) or remained inactive (11 MetS and 10 Con). The following measures were performed pre- and postintervention: radial pulse wave analysis (applanation tonometry) was used to measure augmentation pressure and index, central pressures, and an estimate of myocardial efficiency; arterial stiffness was assessed from carotid-femoral pulse-wave velocity (cfPWV, applanation tonometry); carotid thickness was assessed from B-mode ultrasound; and peak aerobic capacity (gas exchange) was performed in the seated position. Plasma matrix metalloproteinases (MMP) and CVD risk (Framingham risk score) were also assessed. cfPWV was reduced (P < 0.05) in MetS-ExT subjects (7.9 ± 0.6 to 7.2 ± 0.4 m/s) and Con-ExT (6.6 ± 1.8 to 5.6 ± 1.6 m/s). Exercise training reduced (P < 0.05) central systolic pressure (116 ± 5 to 110 ± 4 mmHg), augmentation pressure (9 ± 1 to 7 ± 1 mmHg), augmentation index (19 ± 3 to 15 ± 4%), and improved myocardial efficiency (155 ± 8 to 168 ± 9), but only in the MetS group. Aerobic capacity increased (P < 0.05) in MetS-ExT (16.6 ± 1.0 to 19.9 ± 1.0) and Con-ExT subjects (23.8 ± 1.6 to 26.3 ± 1.6). MMP-1 and -7 were correlated with cfPWV, and both MMP-1 and -7 were reduced post-ExT in MetS subjects. These findings suggest that some of the pathophysiological changes associated with MetS can be improved after aerobic exercise training, thereby lowering their cardiovascular risk. Copyright © 2014 the American Physiological Society.

  13. Aerobic exercise training reduces arterial stiffness in metabolic syndrome

    PubMed Central

    Donley, David A.; Fournier, Sara B.; Reger, Brian L.; DeVallance, Evan; Bonner, Daniel E.; Olfert, I. Mark; Frisbee, Jefferson C.

    2014-01-01

    The metabolic syndrome (MetS) is associated with a threefold increase risk of cardiovascular disease (CVD) mortality partly due to increased arterial stiffening. We compared the effects of aerobic exercise training on arterial stiffening/mechanics in MetS subjects without overt CVD or type 2 diabetes. MetS and healthy control (Con) subjects underwent 8 wk of exercise training (ExT; 11 MetS and 11 Con) or remained inactive (11 MetS and 10 Con). The following measures were performed pre- and postintervention: radial pulse wave analysis (applanation tonometry) was used to measure augmentation pressure and index, central pressures, and an estimate of myocardial efficiency; arterial stiffness was assessed from carotid-femoral pulse-wave velocity (cfPWV, applanation tonometry); carotid thickness was assessed from B-mode ultrasound; and peak aerobic capacity (gas exchange) was performed in the seated position. Plasma matrix metalloproteinases (MMP) and CVD risk (Framingham risk score) were also assessed. cfPWV was reduced (P < 0.05) in MetS-ExT subjects (7.9 ± 0.6 to 7.2 ± 0.4 m/s) and Con-ExT (6.6 ± 1.8 to 5.6 ± 1.6 m/s). Exercise training reduced (P < 0.05) central systolic pressure (116 ± 5 to 110 ± 4 mmHg), augmentation pressure (9 ± 1 to 7 ± 1 mmHg), augmentation index (19 ± 3 to 15 ± 4%), and improved myocardial efficiency (155 ± 8 to 168 ± 9), but only in the MetS group. Aerobic capacity increased (P < 0.05) in MetS-ExT (16.6 ± 1.0 to 19.9 ± 1.0) and Con-ExT subjects (23.8 ± 1.6 to 26.3 ± 1.6). MMP-1 and -7 were correlated with cfPWV, and both MMP-1 and -7 were reduced post-ExT in MetS subjects. These findings suggest that some of the pathophysiological changes associated with MetS can be improved after aerobic exercise training, thereby lowering their cardiovascular risk. PMID:24744384

  14. Association between chromosomal aberration of COX8C and tethered spinal cord syndrome: array-based comparative genomic hybridization analysis

    PubMed Central

    Zhao, Qiu-jiong; Bai, Shao-cong; Cheng, Cheng; Tao, Ben-zhang; Wang, Le-kai; Liang, Shuang; Yin, Ling; Hang, Xing-yi; Shang, Ai-jia

    2016-01-01

    Copy number variations have been found in patients with neural tube abnormalities. In this study, we performed genome-wide screening using high-resolution array-based comparative genomic hybridization in three children with tethered spinal cord syndrome and two healthy parents. Of eight copy number variations, four were non-polymorphic. These non-polymorphic copy number variations were associated with Angelman and Prader-Willi syndromes, and microcephaly. Gene function enrichment analysis revealed that COX8C, a gene associated with metabolic disorders of the nervous system, was located in the copy number variation region of Patient 1. Our results indicate that array-based comparative genomic hybridization can be used to diagnose tethered spinal cord syndrome. Our results may help determine the pathogenesis of tethered spinal cord syndrome and prevent occurrence of this disease. PMID:27651783

  15. Electroencephalographic evoked pain response is suppressed by spinal cord stimulation in complex regional pain syndrome: a case report.

    PubMed

    Hylands-White, Nicholas; Duarte, Rui V; Beeson, Paul; Mayhew, Stephen D; Raphael, Jon H

    2016-12-01

    Pain is a subjective response that limits assessment. The purpose of this case report was to explore how the objectivity of the electroencephalographic response to thermal stimuli would be affected by concurrent spinal cord stimulation. A patient had been implanted with a spinal cord stimulator for the management of complex regional pain syndrome of both hands for 8 years. Following ethical approval and written informed consent we induced thermal stimuli using the Medoc PATHWAY Pain & Sensory Evaluation System on the right hand of the patient with the spinal cord stimulator switched off and with the spinal cord stimulator switched on. The patient reported a clinically significant reduction in thermal induced pain using the numerical rating scale (71.4 % reduction) with spinal cord stimulator switched on. Analysis of electroencephalogram recordings indicated the occurrence of contact heat evoked potentials (N2-P2) with spinal cord stimulator off, but not with spinal cord stimulator on. This case report suggests that thermal pain can be reduced in complex regional pain syndrome patients with the use of spinal cord stimulation and offers objective validation of the reported outcomes with this treatment.

  16. The arterial supply of the cervical and thoracic spinal muscles and overlying skin: Anatomical study with implications for surgical wound complications.

    PubMed

    Yue, Brian Yin Ting; le Roux, Cara Michelle; Corlett, Russell; De La Harpe, David; Richardson, Martin; Ashton, Mark

    2013-07-01

    Postoperative spinal wound dehiscence is a significant complication following the posterior midline approach. It is postulated that this approach disrupts the vasculature supplying the paraspinal muscles and overlying skin. Although the spinal vasculature has been investigated previously, the smaller arterioles have not been described in the context of the posterior midline approach. Eight cadaveric neck and posterior torso specimens were dissected after injection with a radio-opaque lead oxide mixture and subsequent radiographs taken were analyzed. The deep cervical, vertebral, superficial cervical, and occipital arteries consistently supplied the cervical paraspinal muscles. The latter two arteries also vascularized the overlying skin. The deep cervical arteries were found to be located lateral to the C3 to C6 vertebrae, vulnerable to damage with the posterior approach. In the thoracic region, the superior and posterior intercostal arteries consistently supplied the spinal muscles. In all specimens, two small anastomotic vessels posterior to the laminae were found connecting the intercostal artery perforators. Both the arterial perforators and their anastomotic channels were situated in the surgical field and susceptible to damage with the posterior approach. It is likely that the disruption in spinal vasculature contributes to the multifactorial problem of wound dehiscence with the posterior midline approach. Copyright © 2012 Wiley Periodicals, Inc.

  17. Assessment of arterial stiffness in patients with metabolic syndrome in Ecuador: A cross-sectional study.

    PubMed

    Peñaherrera, Carlos A; Peñaherrera, Ruben; Duarte, Maria C; Peñaherrera, Ernesto

    Metabolic syndrome increases cardiovascular risk, and causes damage to the vascular wall. Through this mechanism, it might increase arterial rigidity, leading to further complications and heart strain. The use of a device that measures arterial rigidity can help determine if metabolic syndrome is related to a higher vascular stiffness and changes in estimated arterial age. Cross-sectional study at Luis Vernaza hospital of Guayaquil, Ecuador. Inpatients and outpatients from the Department of Cardiology with a full blood panel and echocardiogram were included. We used the IDF criteria to diagnose metabolic syndrome and measured arterial rigidity parameters in all of them, including augmentation indexes, central blood pressure, pulse wave velocity and arterial age. 95 patients were included for analysis, 44.2% were females and 55.8% were males. Mean age was 61.7 years (±13.1). Metabolic syndrome was diagnosed in 49.5% of our patients and it was significantly more prevalent in women. We found significant differences in PWV, augmentation indexes, and peripheral and central blood pressure between metabolic and non-metabolic syndrome patients. There was a non-significant relationship between estimated arterial age and metabolic syndrome (p=0.32). Patients with metabolic syndrome have an increased arterial rigidity. This, added to the high prevalence of the disease we found in our sample, shows the high cardiovascular risk these patients are at. A multidisciplinary approach to management is needed, along with patient collaboration. The introduction of these devices to measure arterial stiffness in developing countries can improve diagnosis and therapy of patients with cardio-metabolic conditions. Copyright © 2016 Diabetes India. Published by Elsevier Ltd. All rights reserved.

  18. The Prevalence of Metabolic Syndrome in Coronary Artery Disease Patients

    PubMed Central

    Montazerifar, Farzaneh; Bolouri, Ahmad; Mahmoudi Mozaffar, Milad; Karajibani, Mansour

    2016-01-01

    Background Metabolic syndrome (MetS) is a worldwide health problem, which is growing in Iranian adults. MetS is associated with risk of type 2 diabetes and coronary artery disease (CAD). In this study, we aimed to investigate the prevalence of MetS and its individual components in CAD patients. Methods This cross-sectional study was performed on 200 CAD patients who had undergone elective coronary angiography at the cardiology department. Anthropometric indices including waist circumference (WC) and body mass index were measured. Blood samples were obtained to determine glucose and lipid profile. MetS components were defined according to the modified Adult Treatment Panel III (ATP III) criteria. Results The prevalence of MetS among patients was 49.5% (women: 55.9%; men: 40.2%; P < 0.05). The prevalence increased with age. The low high-density lipoprotein-cholesterol (low HDL-C) (84.8%), high fasting blood glucose (high FBG) (77.8%) and high WC (75.8%) were the most prevalent risk factors in CAD patients with MetS. Conclusions Recent data indicate that the dyslipidemia, hyperglycemia and abdominal obesity are crucial predictors of MetS in CAD patients. Further prospective studies are recommended for more clarification. PMID:28197293

  19. Spinal involvement in mucopolysaccharidosis IVA (Morquio-Brailsford or Morquio A syndrome): presentation, diagnosis and management.

    PubMed

    Solanki, Guirish A; Martin, Kenneth W; Theroux, Mary C; Lampe, Christina; White, Klane K; Shediac, Renée; Lampe, Christian G; Beck, Michael; Mackenzie, William G; Hendriksz, Christian J; Harmatz, Paul R

    2013-03-01

    Mucopolysaccharidosis IVA (MPS IVA), also known as Morquio-Brailsford or Morquio A syndrome, is a lysosomal storage disorder caused by a deficiency of the enzyme N-acetyl-galactosamine-6-sulphate sulphatase (GALNS). MPS IVA is multisystemic but manifests primarily as a progressive skeletal dysplasia. Spinal involvement is a major cause of morbidity and mortality in MPS IVA. Early diagnosis and timely treatment of problems involving the spine are critical in preventing or arresting neurological deterioration and loss of function. This review details the spinal manifestations of MPS IVA and describes the tools used to diagnose and monitor spinal involvement. The relative utility of radiography, computed tomography (CT) and magnetic resonance imaging (MRI) for the evaluation of cervical spine instability, stenosis, and cord compression is discussed. Surgical interventions, anaesthetic considerations, and the use of neurophysiological monitoring during procedures performed under general anaesthesia are reviewed. Recommendations for regular radiological imaging and neurologic assessments are presented, and the need for a more standardized approach for evaluating and managing spinal involvement in MPS IVA is addressed.

  20. Reversible cerebral vasoconstriction syndrome and bilateral vertebral artery dissection presenting in a patient after cesarean section

    PubMed Central

    Mitchell, Lex A; Santarelli, Justin G; Singh, Inder Paul; Do, Huy M

    2013-01-01

    Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset thunderclap headache and focal neurologic deficits. Once thought to be a rare syndrome, more advanced non-invasive imaging has led to an increase in RCVS diagnosis. Unilateral vertebral artery dissection has been described in fewer than 40% of cases of RCVS. Bilateral vertebral artery dissection has rarely been reported. We describe the case of a patient with RCVS and bilateral vertebral artery dissection presenting with an intramedullary infarct treated successfully with medical management and careful close follow-up. This rare coexistence should be recognized as the treatment differs. PMID:23354867

  1. Reversible cerebral vasoconstriction syndrome and bilateral vertebral artery dissection presenting in a patient after cesarean section.

    PubMed

    Mitchell, Lex A; Santarelli, Justin G; Singh, Inder Paul; Do, Huy M

    2013-01-24

    Reversible cerebral vasoconstriction syndrome (RCVS) is characterized by sudden-onset thunderclap headache and focal neurologic deficits. Once thought to be a rare syndrome, more advanced non-invasive imaging has led to an increase in RCVS diagnosis. Unilateral vertebral artery dissection has been described in fewer than 40% of cases of RCVS. Bilateral vertebral artery dissection has rarely been reported. We describe the case of a patient with RCVS and bilateral vertebral artery dissection presenting with an intramedullary infarct treated successfully with medical management and careful close follow-up. This rare coexistence should be recognized as the treatment differs.

  2. Severe spinal cord ischemic injury secondary to device embolization after transcatheter closure of a patent arterial duct.

    PubMed

    Tang, Liang; Zhou, Shenghua; Shen, Xiangqian

    2014-02-01

    Percutaneous closure of patent arterial ducts with the Amplatzer Ductal Occluder has become an effective and widely accepted alternative to surgical management. Although rarely, the occluder can be dislodged after an initially successful deployment, and with catastrophic consequences. We describe such a case in a 12-month-old girl who underwent transcatheter closure of a patent arterial duct. After device deployment, the occluder embolized in the patient's descending thoracic aorta, and severe spinal cord ischemic injury resulted. To our knowledge, ours is the first report of this complication after the deployment of an Amplatzer Ductal Occluder. We discuss pathophysiologic mechanisms that could expose patients to the risk of device dislodgment, and we review the relevant medical literature.

  3. Vertebral artery dissection after neck extension in an adult patient with Klippel-Feil syndrome.

    PubMed

    Dornbos, David; Ikeda, Daniel S; Slivka, Andrew; Powers, Ciaran

    2014-04-01

    The association between Klippel-Feil syndrome and vertebral artery dissection is quite rare. We report an adult patient with vertebral artery dissection and Klippel-Feil syndrome, to our knowledge only the third reported case of its kind. A 45-year-old woman with a known history of Klippel-Feil syndrome presented with occipital head and neck pain following forced neck extension. Diagnostic cerebral angiography revealed a high grade vertebral artery stenosis, consistent with vertebral artery dissection. Following 6 months of medical management, a repeat diagnostic angiogram revealed complete healing of the vessel. While cervical fusion, as seen in Klippel-Feil syndrome, has previously been shown to cause neurologic injury secondary to hypermobility, the association with vertebral artery dissection is incredibly rare. We hypothesize that this hypermobility places abnormal shear force on the vessel, causing intimal injury and dissection. Patients with seemingly spontaneous vertebral artery dissection may benefit from cervical spine radiography, and this predisposition to cerebrovascular injury strongly suggests further evaluation of vascular injury following trauma in patients with Klippel-Feil syndrome or other cervical fusion as clinically warranted.

  4. Isolated toxoplasmosis of the thoracic spinal cord in a patient with acquired immunodeficiency syndrome. Case report.

    PubMed

    Resnick, D K; Comey, C H; Welch, W C; Martinez, A J; Hoover, W W; Jacobs, G B

    1995-03-01

    Toxoplasmosis and lymphoma are the two most common causes of intraparenchymal cerebral mass lesions in patients with acquired immunodeficiency syndrome (AIDS). The clinical and radiographic features of the intracranial lesions have been well described. Because of the high frequency of toxoplasmosis in the AIDS population, common therapy for patients presenting with intracranial mass lesions consists of an empirical trial of anti-Toxoplasma chemotherapy, with biopsy reserved for cases demonstrating features considered to be more consistent with lymphoma, or for lesions that do not improve despite adequate anti-Toxoplasma treatment. A similar treatment algorithm does not exist for intramedullary lesions of the spinal cord. The authors describe a patient who presented with paraparesis resulting from an isolated thoracic intramedullary lesion. An open biopsy of the lesion revealed characteristic structures containing Toxoplasma tachyzoites. The clinical and radiographic presentation of the lesion is discussed, the available literature is reviewed, and a treatment strategy for spinal cord lesions in AIDS patients is proposed.

  5. Spinal cord toxoplasmosis in human immunodeficiency virus infection/acquired immunodeficiency syndrome.

    PubMed

    García-García, Concepción; Castillo-Álvarez, Federico; Azcona-Gutiérrez, José M; Herraiz, María J; Ibarra, Valvanera; Oteo, José A

    2015-05-01

    Neurological complications in patients with human immunodeficiency virus infection/acquired immunodeficiency syndrome (HIV/AIDS) are still common, even in the era of highly active antiretroviral therapy. Opportunistic infections, immune reconstitution, the virus itself, antiretroviral drugs and neurocognitive disorders have to be considered when establishing the differential diagnosis. Toxoplasmic encephalitis remains the major cause of space-occupying lesions in the brain of patients with HIV/AIDS; however, spinal cord involvement has been reported infrequently. Here, we review spinal cord toxoplasmosis in HIV infection and illustrate the condition with a recent case from our hospital. We suggest that most patients with HIV/AIDS and myelitis with enhanced spine lesions, multiple brain lesions and positive serology for Toxoplasma gondii should receive immediate empirical treatment for toxoplasmosis, and a biopsy should be performed in those cases without clinical improvement or with deterioration.

  6. A Case of Superior Mesenteric Artery Syndrome in a Healthy Active Duty Marine.

    PubMed

    Thota, Darshan; Portouw, Steven J; Bruner, David I

    2015-10-01

    Superior mesenteric artery (SMA) syndrome is an uncommon disorder that can lead to small bowel obstructions or perforations. Typical populations include young females with anorexia. However, there have been a few reports of healthy males with acute vomiting reported to have SMA syndrome. Our case report highlights an active duty Marine who developed SMA syndrome and the importance of recognizing this disease given the severity in delay of diagnosis in population of young healthy active duty members.

  7. Oral opioid analgesics vs. spinal steroid injections in the treatment of low back pain syndromes.

    PubMed

    Nampiaparampil, Devi E; Nampiaparampil, Geetha M; Nampiaparampil, Robert G

    2012-02-01

    The aim of this study was to examine the outcomes related to analgesia, function, mortality, and adverse effects of oral opioid analgesics and spinal steroid injections on low back pain syndromes. Databases including Medline, EMBASE, PubMed, and Cochrane Library were searched in September 2009 using combinations of terms related to spinal pain and its treatment. A systematic review was performed of randomized controlled trials that enrolled patients with low back pain syndromes and that evaluated patient outcomes after intervention using either oral opioids or spinal steroid injections. Eight high-quality and ten moderate-quality randomized controlled trials were identified. One high-quality study on oral opioid therapy showed significant improvements in pain relief and patient function. Those on spinal steroid injections had a decreased Visual Analog Scale pain score by 7.18 (95% confidence interval, 2.21-12.1) points more than the control group at 1 mo or less and by 0.429 (95% confidence interval, -4.41 to 5.27) points at 1-3 mos. At more than 6 mos, there was no significant benefit: 0.930 (95% confidence interval, -5.03 to 6.89). Spinal steroids decreased the Oswestry Disability Index by 3.53 (95% confidence interval, 0.480-6.57) at 1 mo or less, by -0.281 (95% confidence interval, -3.18 to 2.62) at 1-3 mos, by -11.0 (95% confidence interval, -14.8 to -7.16) at 3-6 mos, and by -0.205 (95% confidence interval, -3.50 to 3.09) compared with the control group at 6 mos or more, suggesting that there was improvement in function. All-cause mortality was low in our analysis of patients attending specialty clinics. It was difficult to assess the adverse effects of opioid therapy because they influenced up to 28% of patients to withdraw from the original studies. In terms of spinal steroid injections, headache appeared to be the most common adverse effect. However, there was no significantly increased risk of headaches associated with spinal steroids compared with

  8. Left subclavian artery stenting: an option for the treatment of the coronary-subclavian steal syndrome

    PubMed Central

    de Almeida, Bruno Lorenção; Kambara, Antonio Massamitsu; Rossi, Fabio Henrique; Moreira, Samuel Martins; de Oliveira, Eduardo Silva Jordao; Linhares Filho, Frederico Augusto de Carvalho; Metzger, Patrick Bastos; Passalacqua, Aldo Zampieri

    2014-01-01

    Introduction The subclavian steal syndrome is characterized by the vertebral artery flow inversion, due to a stenotic lesion in the origin of the subclavian artery. The Coronary-subclavian Steal Syndrome is a variation of the Subclavian Steal Syndrome and is characterized by inversion of flow in the Internal Thracic artery that has been used as conduct in a myocardial revascularization. Its diagnosis must be suspected in patients with difference in pulse and arterial pressure in the upper limbs, that present with angina pectoris and that have done a myocardial revascularization. Its treatment must be a surgical bypass or a transluminal angioplasty. Objective The objective is to show the left subclavian artery stenting as a safe and effective method to treat the coronary-subclavian steal syndrome. Methods Historical prospective, non-randomized trial, through revision of the hospital records of the patients treated with the stenting of the left subclavian artery, from January 2006 to September 2012. Results In the mentioned period, 4.291 miocardial revascularizations were performed with the use of the left mammary artery, and 16 patients were identified to have the Coronary-subclavian steal syndrome. All of them were submitted to endovascular treatment. The success rate was 100%; two patients experienced minor complications; none of them presented with major complications. Eleven of the 16 patients had ultrassonographic documentation of patent stent for at least one year; two patients lost follow up and other two died. Conclusion The stenting of the left subclavian artery is a good option for the treatment of the Coronary-subclavian Steal Syndrome, with high level of technical and clinical success. PMID:25140474

  9. Klippel-Feil Syndrome With Spinal Dysraphism: Diastematomyelia on 18F-NaF Bone PET, CT, and MRI Imaging.

    PubMed

    Hudson, Emmanuel W; Makis, William

    2016-05-01

    A 49-year-old woman with known Klippel-Feil syndrome had a CT scan for staging of stage IIIA invasive carcinoma of the left breast. She was found to have cervicothoracic spinal dysraphism with diastematomyelia. An F-NaF bone PET done as part of the metastatic workup showed the characteristic sagittal bone spur, and MRI demonstrated a complete split of the cervical and upper thoracic spinal cord, previously unknown to the patient.

  10. [Pulmonary artery sarcoma mimicking a Hughes Stovin syndrome. Report of one case].

    PubMed

    Erlij, Daniel; Michalland, Susana; Neira, Óscar; Fernández, Cristina; Wolff, Verónica

    2014-11-01

    Pulmonary artery sarcoma is an uncommon entity with high mortality. Its clinical presentation is usually indistinguishable from pulmonary embolism, which leads to a significant delay in diagnosis. Hughes-Stovin syndrome is characterized by venous thrombosis and aneurysms of the pulmonary or bronchial artery. We report a 59 year-old female with a history of recurrent pulmonary embolism. In the last thromboembolic episode a pulmonary artery aneurysm was found on a CT scan. The patient was operated performing a left inferior lobectomy. The patient died five days after surgery and the pathological examination of the surgical piece revealed a pulmonary artery sarcoma.

  11. Young patient with arterial thrombosis and skin changes as the onset manifestations: POEMS syndrome

    PubMed Central

    Han, Ting-Ting; Zheng, Shuang; Chen, Zeng-Ai; Liu, Wei; Hu, Yao-Min

    2016-01-01

    POEMS syndrome is a rare multi-systemic disease characterized by polyneuropathy, organomegaly, endocrinopathy, monoclonal protein and skin changes. Arterial thrombosis is a distinctively unusual feature in patients with POEMS syndrome. We report a 33-year-old man with intermittent amaurosis of left eye and skin changes as the onset manifestations, who was finally confirmed as having POEMS syndrome. Most notably, this was a young man without high risk factors of arterial thrombosis and no monoclonal protein was detected until the repeated measurement later. This case evokes the need to consider the diagnosis of POEMS syndrome for young patients with symptoms of arterial thrombosis but no high risk factors of thrombosis. PMID:27738309

  12. Klippel-Trénaunay syndrome: treatment of lower extremity pain with a spinal cord stimulator.

    PubMed

    Franz, Randall W; Prok, Aleksey

    2009-01-01

    A young adult man with Klippel-Trénaunay syndrome presented to our pain management service with complaints of severe lower extremity neuropathic pain (pain scale 8 of 10 on the left and 4 of 10 on the right). The pain in his left leg was so severe that he wanted to undergo a left lower extremity amputation. The patient declined chronic use of narcotic medications for pain relief, believing that this would interfere with his educational and lifestyle pursuits. After a complete evaluation for possible sources of pain, we performed a trial placement of a spinal cord stimulator at the T9 level, which relieved his pain. We then placed a stimulator at the T10 level. At 1 year postimplantation, he was pain free (pain scale 1 of 10 bilaterally) and was able to function normally without narcotic support. We believe this to be the first use of a spinal cord stimulator for lower extremity pain resulting from Klippel-Trénaunay syndrome. We also discuss the clinical evaluation and treatment of a Klippel-Trénaunay syndrome patient with chronic pain.

  13. [Case of cerebellar and spinal cord infarction presenting with acute brachial diplegia due to right vertebral artery occlusion].

    PubMed

    Fujii, Takayuki; Santa, Yo; Akutagawa, Noriko; Nagano, Sukehisa; Yoshimura, Takeo

    2012-01-01

    A 73-year-old man was admitted for evaluation of sudden onset of dizziness, bilateral shoulder pain, and brachial diplegia. Neurological examination revealed severe bilateral weakness of the triceps brachii, wrist flexor, and wrist extensor muscles. There was no paresis of the lower limbs. His gait was ataxic. Pinprick and temperature sensations were diminished at the bilateral C6-C8 dermatomes. Vibration and position senses were intact. An MRI of the head revealed a right cerebellar infarction and occlusion of the right vertebral artery. An MRI of the cervical spine on T₂ weighted imaging (T₂WI) showed cord compression at the C3/4-C5/6 level secondary to spondylotic degeneration without any intramedullary signal changes of the cord. On the following day, however, high-signal lesions on T₂WI appeared in the C5-C6 spinal cord, suggesting cord infarction. Unilateral vertebral artery occlusion does not usually result in cervical cord infarction because of anastomosis of arteries. Because of the long-term mechanical compression in our case, it was likely that cervical cord ischemia was present before the onset of symptoms. On the basis of chronic cord compression, our case suggests that occlusion of a unilateral vertebral artery could cause cervical cord infarction.

  14. Migratory low velocity intradural lumbosacral spinal bullet causing cauda equina syndrome: report of a case and review of literature.

    PubMed

    Baldawa, Sachin; Shivpuje, Vijay

    2017-05-01

    Migration of the bullet within the spinal subarachnoid space has long been recognized as unusual complication of spinal gunshot injury. We report a case of migratory low velocity intradural lumbosacral spinal bullet causing cauda equina syndrome. The relevant literature is reviewed and all cases of migratory spinal bullet are summarised, and management strategies are discussed. Literature review. A 32-year-old male suffered abdominal gunshot injury for which emergency laparotomy and repair of colonic perforation were performed. The bullet was seen lodged within the sacral spinal canal behind the S1 vertebral body. The probable entry point was at L2-L3 level. Caudal migration of the bullet within the spinal subarachnoid space leads to the appearance of cauda equina syndrome. Bullet was retrieved following upper sacral and lower lumbar laminectomy. Prone positioning of the patient had lead to cranial migration of the bullet at L4 level which was confirmed on fluoroscopy. Laminectomy had to be extended upwards with the patient in reverse Trendelenburg position for bullet removal. Caudal migration of the bullet within the lumbosacral subarachnoid space results in cauda equina syndrome. Surgical retrieval of the bullet ensures the early recovery of neurological symptoms. Prone patient positioning can influence bullet location. Intraoperative fluoroscopy prior to skin incision is essential in addition to preoperative imaging to locate the bullet and thus avoid incorrect lower level laminectomy. Trapping the bullet after durotomy using suction and dissector in reverse Trendelenburg position is a useful aid in bullet removal.

  15. Is pseudoexfoliation syndrome associated with coronary artery disease?

    PubMed Central

    Emiroglu, Mehmet Yunus; Coskun, Erol; Karapinar, Hekim; Capkın, Musa; Kaya, Zekeriya; Kaya, Hasan; Akcakoyun, Mustafa; Kargin, Ramazan; Simsek, Zeki; Acar, Göksel; Aung, Soe Moe; Pala, Selcuk; Özdemir, Burak; Esen, Ali Metin; Kırma, Cevat

    2010-01-01

    Background: Pseudoexfoliation syndrome (PEX) is recognised by chronic deposition of abnormal pseudoexfoliation material on anterior segment structures of the eye, especially the anterior lens capsule. In recent years, several studies have shown the presence of vascular, cardiac and other organ pseudoexfoliative material in patients with ocular pseudoexfoliation. Aims: The purpose of this study is to determine whether an association exists between ocular pseudoexfoliation and coronary artery disease, aortic aneurysms and peripheric vascular disease. Patients and Methods: 490 patients who underwent coronary angiography (CAG) at Kosuyolu Cardiovascula Research and Training Hospital were included in the study. Patients were evaluated for conventional risk factors such as age, sex, family history, hypertension, diabetes, dislipidemia and smoking. Detailed eye examinations including evaluation of lens were done in all patients. The presence of PEX material in the anterior segment was best appreciated by slit lamp after pupillary dilation. The patients were divided into two groups according to the presence of PEX, and compared for the presence of CAD and other risk factors. Results: CAD was present in 387 patients. 103 patients had normal coronary angiography. 20 (5.2 %) of CAD patients and 4 (3.9%) of normal CAG patients were found to have PEX (p>0.05). There was no significant relationship between CAD and the presence of PEX (p>0.05). When patients were grouped according to the presence of PEX, only age was significantly different between the two groups (r: 0.25, p<0.001). Conclusion: There is no significant relationship between the presence of PEX and CAD. Further studies in larger scales with elderly population may be more valuable. PMID:22558552

  16. Metabolic syndrome and peripheral artery disease: Two related conditions.

    PubMed

    Oriol Torón, Pilar Ángeles; Badía Farré, Teresa; Romaguera Lliso, Amparo; Roda Diestro, Jovita

    2016-01-01

    To ascertain the prevalence of metabolic syndrome (MS) in patients with peripheral artery disease (PAD) at the Martorell primary care (PC) center. To analyze the differences in comorbidities and cardiovascular risk factors between patients with PAD with and without MS. A cross-sectional, descriptive study on patients diagnosed with PAD according to computerized clinical records of the Martorell PC center. Variables collected included age, sex, high blood pressure (HBP), dyslipidemia (DLP), diabetes (DM), smoking, obesity, cardiovascular disease (CVD), erectile dysfunction (ED), renal failure (RF), and oligoalbuminuria. An analysis comparing patients with and without MS was performed. There were 131 patients diagnosed with PAD, 104 (79%) of whom were male. Sixty-three (48.1%) also had MS. Patients with both PAD and MS had, as compared to those with PAD only, a higher prevalence of HBP (87.3 vs. 60.3%, P: 0.001), DLP (77.8 vs. 60.3%, P: 0.03), DM (69.8 vs. 30.9%, P<.001), obesity (25.4 vs. 10.3%, P: 0.03), CVD (42.9 vs. 19.1%); P: 0.004), ED (81.3 vs. 54.3%, P: 0.02), and RF (40.3 vs. 17.9%, P: 0.006). Patients with both PAD and MS had a higher prevalence of HBP, DLP, DM, and obesity. They also had more cardiovascular events and were significantly associated with pathological conditions highly relevant for cardiovascular prognosis such as erectile dysfunction and chronic kidney disease. Copyright © 2016 SEEN. Published by Elsevier España, S.L.U. All rights reserved.

  17. Metabolic syndrome in patients with peripheral arterial disease.

    PubMed

    Estirado, E; Lahoz, C; Laguna, F; García-Iglesias, F; González-Alegre, M T; Mostaza, J M

    2014-11-01

    The prevalence of metabolic syndrome (MS) in patients with peripheral arterial disease (PAD) and coronary or cerebrovascular disease is increasing, but it is not known whether this association also exists in patients with isolated PAD. The aim of the current study was to assess the prevalence of MS in patients with PAD who had no coronary or cerebrovascular disease, the prescription rate of evidence-based cardiovascular therapies and the attainment of therapeutic goals in patients with PAD and with and without MS. Multicenter, cross-sectional study of 3.934 patients aged ≥ 45 years with isolated PAD who were treated in primary care and specialized outpatient clinics during 2009. A diagnosis of PAD was reached for ankle brachial indices <0.9, a previous history of amputation or revascularization. In the overall population, the mean age was 67.6 years, 73.8% were males and 63% had MS (95% CI 61.5-64.3%). Patients with MS had a higher prevalence of cardiovascular risk factors and comorbidities, more severe PAD and higher prescription rate of evidence-based cardiovascular therapies. After adjusting for risk factors and comorbidity, there was a more frequent use of renin-angiotensin system blockers, beta-blockers, diuretics and statins among the patients with MS. A lower percentage of patients with MS achieved the therapeutic goals for blood pressure (22% vs. 41.5%, p<0.001). Similarly, a lower percentage of patients with diabetes achieved the glycated hemoglobin goals (44% vs. 53.1%, p<0.001), with no differences in LDL-cholesterol levels (29.8% vs. 39.1%, p=0.265). Patients with PAD have a high prevalence of MS. Patients with MS do not attain therapeutic goals as frequently as those without, despite taking more cardiovascular drugs. Copyright © 2014 Elsevier España, S.L.U. All rights reserved.

  18. [A case of DiGeorge syndrome with left internal carotid artery absence probably causing one-and-a-half syndrome].

    PubMed

    Maruyama, Shigeru; Suda, Masashi; Kobayashi, Takehiro

    2012-09-01

    We experienced a case of DiGeorge syndrome with left internal carotid artery absence probably causing one-and-a-half syndrome. MR angiogram demonstrated the apparent absence of the left internal carotid artery and consequently abnormal blood supply to the left middle cerebral artery, which was derived from the basilar artery via the left posterior communicating artery. The patient alsoshowed both an extremely narrow carotid canal on the left side and a very fine vessel extending to the terminal of the left internal carotid artery. Therefore, we regarded this abnormality as severe hypoplasia of left internal carotid artery and supposed that this hypoplasia had originated in maldevelopment of the third aortic arch based on the coexisting lower bifurcation of the right common carotid artery. Since the lesion of one-and-a-half syndrome is restricted to the pontine tegmentum, we speculated that it had resulted from ischemia of the basilar artery area during the embryonic period associated with the absence of the internal carotid artery. To our knowledge, DiGeorge syndrome has never been reported as a complication of internal carotid artery absence. The patient did not demonstrate either chromosome 22q11.2 deletion or TBX1 gene mutation, which is considered the gene responsible for 22q11.2 deletion syndrome. Therefore, the etiology of DiGeorge syndrome in this case remains unclear.

  19. [Hemothorax complicated with celiac artery compression syndrome (CACS); report of a case].

    PubMed

    Uga, Naoko; Adachi, Katsutoshi; Tarukawa, Tomohito; Okuda, Yasuyuki; Tanigawa, Kanji; Nakaya, Hitoshi; Sato, Tomoaki; Hioki, Iwao

    2014-05-01

    We report a case of hemothorax complicated with celiac artery compression syndrome (CACS). A 43-year-old man presented with a sudden onset left back pain. Computed tomography (CT) showed its hemothorax, esophageal artery aneurysm and severe stenosis of the celiac truncus with its anterior compression by median accurate ligament, and a diagnosis of CACS associated with rupture of the aneurysm was made. Emergent transcatheter arterial embolization of the aneurysm resulted in a technical failure, although the patient's condition was stable and performed esophageal artery ligation through video-assisted thoracoscopic surgery on day 5 after onset. After surgery, the patient recovered without significant incidents. A cause of this aneurysmal development was supposed to be a significantly increased esophageal arterial blood flow with its luminal dilation to compensate a decreased celiac blood flow. Segmental arterial mediolysis could not be excluded as another cause.

  20. Changes of blood flow, oxygen tension, action potential and vascular permeability induced by arterial ischemia or venous congestion on the spinal cord in canine model.

    PubMed

    Kobayashi, Shigeru; Yoshizawa, Hidezo; Shimada, Seiichiro; Guerrero, Alexander Rodríguez; Miyachi, Masaya

    2013-01-01

    It is generally considered that the genesis of myelopathy associated with the degenerative conditions of the spine may result from both mechanical compression and circulatory disturbance. Many references about spinal cord tissue ischemic damage can be found in the literature, but not detailed studies about spinal cord microvasculature damage related to congestion or blood permeability. This study investigates the effect of ischemia and congestion on the spinal cord using an in vivo model. The aorta was clamped as an ischemia model of the spinal cord and the inferior vena cava was clamped as a congestion model at the 6th costal level for 30 min using forceps transpleurally. Measurements of blood flow, partial oxygen pressure, and conduction velocity in the spinal cord were repeated over a period of 1 h after release of clamping. Finally, we examined the status of blood-spinal cord barrier under fluorescence and transmission electron microscope. Immediately after clamping of the inferior vena cava, the central venous pressure increased by about four times. Blood flow, oxygen tension and action potential were more severely affected by the aorta clamping; but this ischemic model did not show any changes of blood permeability in the spinal cord. The intramedullar edema was more easily produced by venous congestion than by arterial ischemia. In conclusions, venous congestion may be a preceding and essential factor of circulatory disturbance in the compressed spinal cord inducing myelopathy.

  1. Spinal cord stimulation for a woman with complex regional pain syndrome who wished to get pregnant.

    PubMed

    Ito, Shoji; Sugiura, Takeshi; Azami, Takafumi; Sasano, Hiroshi; Sobue, Kazuya

    2013-02-01

    A woman with complex regional pain syndrome (CRPS) in the right lower extremity who wished to discontinue medications to get pregnant underwent implantation of a spinal cord stimulation system (SCS). An electrode lead was placed at Th(10-11) in the epidural space, accessed via the L(2-3) interspace with a paramedian approach, and a pulse generator was implanted in the left buttock. She kept the SCS on 24 h a day. After she had experienced several chemical abortions, finally she got pregnant via artificial insemination. She had an uneventful delivery of a healthy baby by cesarean resection under spinal anesthesia. In a patient with CRPS who has an implanted SCS system and wishes to get pregnant, the electrode lead into the low thoracic epidural space should be accessed via the high lumbar intervertebral space in consideration of a future requirement for spinal or epidural anesthesia for cesarean section. The generator should be placed in the buttock to prevent impairment of the SCS system being caused by the enlarged abdomen during pregnancy. Although we were apprehensive of adverse effects owing to the electromagnetic field force and change of blood flow in the pelvic viscera, our patient had a successful delivery. SCS is a favorable option for patients with CRPS who wish to get pregnant.

  2. Spinal sagittal balance substantially influences locomotive syndrome and physical performance in community-living middle-aged and elderly women.

    PubMed

    Muramoto, Akio; Imagama, Shiro; Ito, Zenya; Hirano, Kenichi; Ishiguro, Naoki; Hasegawa, Yukiharu

    2016-03-01

    Spinal sagittal imbalance has been well known risk factor of decreased quality of life in the field of adult spinal deformity. However, the impact of spinal sagittal balance on locomotive syndrome and physical performance in community-living elderly has not yet been clarified. The present study investigated the influence of spinal sagittal alignment on locomotive syndrome (LS) and physical performance in community-living middle-aged and elderly women. A total of 125 women between the age of 40-88 years (mean 66.2 ± 9.7 years) who completed the questionnaires, spinal mouse test, physical examination and physical performance tests in Yakumo study were enrolled in this study. Participants answered the 25-Question Geriatric Locomotive Function Scale (GLFS-25), the visual analog scale (VAS) for low back pain (LBP), knee pain. LS was defined as having a score of >16 points on the GLFS-25. Using spinal mouse, spinal inclination angle (SIA), thoracic kyphosis angle (TKA), lumbar lordosis angle (LLA), sacral slope angle (SSA), thoracic spinal range of motion (TSROM), lumbar spinal range of motion (LSROM) were measured. Timed-up-and-go test (TUG), one-leg standing time with eyes open (OLS), and maximum stride, back muscle strength were also measured. The relationship between spinal sagittal parameters and GLFS-25, VAS and physical performance tests were analyzed. 26 people were diagnosed as LS and 99 were diagnosed as non-LS. LBP and knee pain were greater, physical performance tests were poorer, SIA were greater, LLA were smaller in LS group compared to non-LS group even after adjustment by age. SIA significantly correlated with GLFS-25, TUG, OLS and maximum stride even after adjustment by age. The cutoff value of SIA for locomotive syndrome was 6°. People with a SIA of 6° or greater were grouped as "Inclined" and people with a SIA of less than 6° were grouped as "Non-inclined". 21 people were "Inclined" and 104 were "Non-inclined". Odds ratio to fall in LS of

  3. Computer navigation-assisted spinal fusion with segmental pedicle screw instrumentation for scoliosis with Rett syndrome: a case report.

    PubMed

    Tanaka, Masato; Nakanishi, Kazuo; Sugimoto, Yoshihisa; Misawa, Haruo; Takigawa, Tomoyuki; Nishida, Keiichiro; Ozaki, Toshifumi

    2009-12-01

    Scoliosis is a common clinical manifestation of Rett syndrome, a neurodevelopmental disorder that almost exclusively affects females. The spinal curve in patients with Rett syndrome is typically a long C curve of a neuromuscular type. As the onset of the scoliosis is very early and shows rapid progression, early surgical intervention has been recommended to prevent a life-threatening collapsing spine syndrome. However, there are high perioperative risks in Rett syndrome patients who undergo spinal surgery, such as neurological compromise and respiratory dysfunction due to rigid spinal curve. We herein report the surgical result of treating severe rapid progressive thoracic scoliosis in a 16-year-old girl with Rett syndrome. Posterior segmental pedicle screw fixation was performed from T1 to L3 using a computer-assisted technique. Post-operative radiography demonstrated a good correction of the curve in both the sagittal and coronal alignment. There were no postoperative complications such as neurological compromise. The patient had maintained satisfactory spinal balance as of the 3-year follow-up examination.

  4. Isolated cervical spinal canal stenosis at C-1 in the pediatric population and in Williams syndrome.

    PubMed

    Desai, Sohum K; Vadivelu, Sudhakar; Patel, Akash J; Brayton, Alison; Jea, Andrew

    2013-06-01

    Isolated cervical canal stenosis at the level of the atlas (C-1) is a rare cause of cervical myelopathy in the pediatric population. It has been associated with several genetic disorders including spondyloepiphysial dysplasia congenita, Down syndrome, and Klippel-Feil syndrome. The purpose of this study is to highlight the authors' experience with 4 additional pediatric cases, review the literature, and report a new association of this disease with Williams syndrome. The medical records and radiological imaging studies of 4 patients treated at Texas Children's Hospital for symptomatic hypoplasia of the atlas were retrospectively reviewed. Pertinent patient demographic data, clinical presentation, imaging findings, and outcomes after surgery were recorded. A thorough literature review was performed, allowing the authors to compare and contrast their 4 cases to surgical cases already published in the literature. There were 11 boys and 1 girl in the aggregate series. The average age of the patients was 7 years (range 13 months-14 years), and the duration of symptoms prior to presentation was 6 months (range 0-36 months). The mean sagittal diameter of the spinal canal at the level of the atlas measured from the posterior aspect of the dens to the anterior aspect of the arch of C-1 was 11.9 mm (range 8.3-16 mm) in the aggregate series. In 2 new pediatric patients with hypoplasia of the atlas the disease was associated with Williams syndrome, which has not been previously described. Patients in the aggregate series were followed for an average of 18 months (range 3-50 months). Laminectomy of C-1 provided neurological improvement in all patients who presented. Isolated cervical spinal canal stenosis at the level of the atlas is a rare cause of cervical myelopathy. The authors hope that this report will prompt clinicians to consider it when searching for the origin of signs and symptoms of cervical myelopathy, especially in children.

  5. Superior mesenteric artery (SMA) syndrome: an unusual cause of intestinal obstruction in palliative care.

    PubMed

    So, Chun-Yan; Chan, Kwok-Ying; Au, Ho-Yan; Chan, Man-Lui; Lai, Theresa

    2017-01-01

    Superior mesenteric artery (SMA) syndrome is an uncommon cause of intestinal obstruction and seldom been mentioned in palliative care. Hereby, we reported a case of SMA syndrome who presented with symptoms of upper intestinal obstruction in a 68-year-old patient; subsequent CT findings were classical of SMA syndrome. The patient's history of poliomyelitis and recent significant weight loss were the predisposing factors for SMA syndrome. It also highlights the importance of monitoring signs and symptoms of intestinal obstruction in such patients before considering switching to oral feeding.

  6. Effects of Dry Needling on Spinal Mobility and Trigger Points in Patients with Fibromyalgia Syndrome.

    PubMed

    Castro-Sanchez, Adelaida Maria; Garcia-Lopez, Hector; Mataran-Penarrocha, Guillermo A; Fernandez-Sanchez, Manuel; Fernandez-Sola, Cayetano; Granero-Molina, Jose; Aguilar-Ferrandiz, Maria Encarnacion

    2017-02-01

    The etiology of fibromyalgia syndrome (FMS) is inconclusive, but central mechanisms are well accepted for this pain condition. Myofascial pain syndrome (MPS) is one of the most common musculoskeletal pain diseases and is characterized by myofascial trigger points (MTrPs). It has been suggest that MTrPs have an important factor in the genesis of FMS. The purpose of the current randomized clinical trial was to compare the effectiveness of dry needling versus cross tape on spinal mobility and MTrPs in spinal muscles in patients with FMS. A single-blind randomized controlled trial was conducted on patients with FMS. Clinical setting. Sixty-four patients with FMS were randomly assigned to an experimental group receiving dry needling therapy or to a control group for cross tape therapy in the MTrPs in the latissimus dorsi, iliocostalis, multifidus, and quadratus lumbourum muscles. Spinal mobility measures and MTrPs algometry were recorded at baseline and after 5 weeks of treatment. The repeated measures analysis of variance (ANOVA) demonstrated that significant differences between groups were achieved for the MTrPs in latissimus dorsi muscle (right axillary portion: F = 9.80, P = 0.003); multifidus muscle (right L2 level: F = 11.80, P = 0.001); quadratus lumborum (right lateral superficial upper: F = 6.67, P = 0.012; and right lateral superficial lower: F = 5.38, P = 0.024). In addition, the ANOVA repeated measures test showed significant differences between groups for the segmental amplitude thoracic spine in the standing erect position (F = 7.33, P = 0.009), and segmental amplitude of lumbar spine (F = 11.60, P = 0.001) in the sitting erect position. The outcomes were not collected from a long-term follow-up period. Dry needling therapy or cross tape were used alone when in reality physical therapists usually treat patients with FMS using a multi-modal approach. A non-treatment control group was not included. This study has demonstrated that dry needling therapy

  7. Laparoscopic Toupet fundoplication with duodenojejunostomy for the management of superior mesenteric artery syndrome with reflux symptoms

    PubMed Central

    Yan, Chao; Hu, Zhi-Wei; Wu, Ji-Min; Zhang, Chao; Yan, Liang; Wang, Zhong-Gao

    2017-01-01

    Abstract Rationale: The patient had symptoms of GERD and the reflux even caused the symptom of cough. Gaining weight is a risk factor for the treatment of reflux as it could exacerbated symptoms of reflux and the drug treatment is not effective. Surgical intervention becomes necessary when there is failure following conservative medical therapy or the patient. Patient concerns: The patient was not satisfied with the drug treatment. Diagnoses: Superior mesenteric artery syndrome, gastroesophageal reflux disease. Interventions: Laparoscopic Toupet fundoplication with duodenojejunostomy. Outcomes: The patient discharged from hospital 10 days after surgery without any postoperative complication. The patient achieved complete relief of symptoms and discontinuation of drug. Lessons subsections: Superior mesenteric artery (SMA) syndrome may manifest the symptoms of GERD such as heartburn, acid reflux and cough. It is necessary to complete examination to exclude superior mesenteric artery syndrome for these patients. Laparoscopic fundoplication with duodenojejunostomy provided an effective treatment for patients who failed drug treatment. PMID:28099334

  8. [Spinal cord stimulation and failed back surgery syndrome. Clinical results with laminectomy electrodes].

    PubMed

    García March, Guillermo; Bordes, Vicente; Roldán, Pedro; Real, Luis; González Darder, José Manuel

    2015-01-01

    Spinal cord stimulation is a widely-accepted technique in the treatment of back pain resulting from failed back surgery. Classically, stimulation has been carried out with percutaneous electrodes implanted under local anaesthesia and sedation. However, the ease of migration and the difficulty of reproducing electrical paresthesias in large areas with such electrodes has led to increasing use of surgical plate leads, which have the disadvantage of the need for general anaesthesia and a laminectomy for implantation. Our objective was to report the clinical results, technical details, advantages and benefits of laminectomy lead placement under epidural anaesthesia in failed back surgery syndrome cases. Spinal cord stimulation was performed in a total of 119 patients (52 men and 67 women), aged between 31 and 73 years (average, 47.3). Epidural anaesthesia was induced with ropivacaine. In all cases we inserted the octapolar or 16-polar lead in the epidural space through a small laminectomy. The final position of the leads was the vertebral level that provided coverage of the patient's pain. The electrodes were connected at dual-channel or rechargeable pulse generators. After a mean follow-up of 4.7 years, the results in terms of improvement of the previous painful situation was satisfactory, with an analgesia level of 58% of axial pain and 60% of radicular pain in more than 70% of cases. None of the patients said that the surgery stage was painful or unpleasant. No serious complications were included in the group, but in 6 cases the system had to be explanted because of ineffectiveness or intolerance of long-term neurostimulation. This study, with a significant number of patients, used epidural anaesthesia for spinal cord stimulation of lead implants by laminectomy in failed back surgery syndromes. The technique seems to be safe and effective. Copyright © 2014 Sociedad Española de Neurocirugía. Published by Elsevier España. All rights reserved.

  9. Peripheral arterial chemoreceptors and sudden infant death syndrome.

    PubMed

    Gauda, Estelle B; Cristofalo, Elizabeth; Nunez, Jeanne

    2007-07-01

    Sudden infant death syndrome (SIDS) is the major cause of death in infants between 1 month and 1 year of age. Two particular concerns are that (1) premature or low birth weight (<2500-g) infants have a 2- to 40-fold greater risk of dying of SIDS (depending on the sleep position) than infants born at term and of normal birth weight, and that (2) the proportion of premature infants dying of SIDS has increased from 12 to 34% between 1988 and 2003. Hypo- and hypersensitivity of peripheral arterial chemoreceptors (PACs) may be one biological mechanism that could help to explain the epidemiological association between the increased incidence of SIDS in formerly premature infants. Because premature infants are often exposed to the extremes of oxygen stress during early postnatal development, they are more likely to have a maladaptive response of PACs later in their lives. As the first line of defense that mediates an increase in ventilation to a hypoxic challenge during wakefulness and sleep, PACs also mediate arousal responses during sleep in response to an asphyxial event that is often associated with upper airway obstruction. In most mammalian species, PACs are not fully developed at birth and thus are vulnerable to plasticity-induced changes mediated by environmental exposures such as the extremes of oxygen tension. Hypoxic or hyperoxic exposure during early postnatal development can lead to hyposensitive or hypersensitive PAC responses later in life. Although baseline chemoreceptor activity may not be the cause of an initial hypoxic or asphyxial event, the level of peripheral chemoreceptor drive does modulate the (1) time to arousal, (2) resumption of airflow during airway obstruction, (3) escape behaviors during rebreathing, and (4) cardiorespiratory responses that result from activation of the laryngeal chemoreflex. The laryngeal chemoreflex can be stimulated by reflux of gastric contents above the upper esophageal sphincter, or an increase in nasopharyngeal

  10. Dural ectasia associated with Mounier-Kuhn syndrome: a novel association in the context of spinal stenosis.

    PubMed

    Sheridan, Gerard Anthony; Godkin, Owen; Devitt, Aiden

    2017-08-30

    We present the case of a patient undergoing lumbar spine decompression for stenosis with a history of Mounier-Kuhn syndrome. The patient presented with axial lumbar spine pain over 6 months with progressive radicular pain to the left L3 dermatome. MRI confirmed spinal stenosis at L3/4 level with associated dural ectasia. The patient had an uneventful spinal decompression with resolution of radicular symptoms and axial spine pain. Dural ectasia poses a significant risk when operating on the lumbosacral spine. Larger cerebrospinal fluid volumes and a capacious dural canal can result in anaesthetic and orthopaedic complications such as inadequate spinal anaesthesia, complicated epidural analgesia, intraoperative dural tears and difficult pedicle screw insertion due to narrow pedicles. This is the first case in the literature detailing the association between dural ectasia and Mounier-Kuhn syndrome. We recommend adequate spinal imaging in patients with Mounier-Kuhn syndrome to exclude dural ectasia prior to undergoing lumbosacral spinal procedures. © BMJ Publishing Group Ltd (unless otherwise stated in the text of the article) 2017. All rights reserved. No commercial use is permitted unless otherwise expressly granted.

  11. Minimizing shoulder syndrome with intra-operative spinal accessory nerve monitoring for neck dissection.

    PubMed

    Lee, C-H; Huang, N-C; Chen, H-C; Chen, M-K

    2013-04-01

    The objective of this study was to analyze the safety and results of intra-operative SAN (spinal accessary nerve) monitoring during selective neck dissection, with emphasis on shoulder syndrome. Twenty-five consecutive patients with head and neck cancer were studied. Selective neck dissection was performed by a single clinical fellow under the supervision of the department chief using an intra-operative SAN monitor. Electrophysiological data were recorded after initial identification of the SAN and continued until just before closure. Electromyographic evaluation was carried out to assess SAN function one month postoperatively. Shoulder disability was also evaluated at this time using a questionnaire for shoulder syndrome (shrug, flexion, abduction, winging, and pain). No patients had postoperative shoulder syndrome involving shrug, flexion, abduction, or winging. Twenty-two of the 25 (88%) patients had shoulder pain, but the average pain score was low (2.3 ± 1.3). No patients had neck recurrence during at least 1 year of follow up. By using nerve monitoring during selective neck dissection, no patient developed significant "shoulder syndrome", with the exception of slight pain.

  12. Modulation of somatosensory profiles by spinal cord stimulation in primary Raynaud's syndrome.

    PubMed

    Münster, Tino; Tiebel, Nils; Seyer, Hendrikus; Maihöfner, Christian

    2012-07-01

    Spinal cord stimulation (SCS) is an effective antinociceptive treatment for various neuropathic pain syndromes. Apart from antinociceptive action, it may modulate overall somatosensory perception. This case report targets the question of whether SCS may alter quantitative sensory testing (QST) in a patient with primary Raynaud's syndrome.  We report on a 44-year-old female patient with primary Raynaud's syndrome who had SCS via cervical and lumbar electrodes. QST was performed in a standardized manner assessing cold detection threshold (CDT) and warm detection threshold (WDT), cold pain threshold (CPT) and heat pain threshold (HPT), mechanical detection threshold (MDT) and mechanical pain threshold (MPT) thresholds, and vibration detection threshold (VDT) and pressure pain thresholds (PPT). We tested at the dorsum of the right/left hand of the patient with engaged and disengaged SCS. Test results were compared with a control group of 80 subjects.  Without SCS, the patient showed a sensory decrease in CDT, MDT, MPT, and VDT. SCS influenced the perception of cold, warm, and tactile detection thresholds, whereby CDT, WDT, and VDT were impaired and MDT was improved.  SCS significantly modulated the somatosensory profile in a patient with primary Raynaud's syndrome. These effects were pronounced in qualities involving Aβ, C, and A∂ nerve fibers. Further investigations may help to understand the mechanisms of action of SCS. © 2012 The Authors. Pain Practice © 2012 World Institute of Pain.

  13. Atypical moyamoya syndrome with brain calcification and stenosis of abdominal aorta and renal arteries.

    PubMed

    Uchikawa, Hideki; Fujii, Katsunori; Fujita, Mayuko; Okunushi, Tomoko; Shimojo, Naoki

    2017-09-01

    Moyamoya syndrome is a progressive cerebrovascular disease that is characterized by stenosis of the terminal portion of the internal carotid artery and its main branches, in combination with an accompanying disease. We herein describe an 8-year-old boy exhibiting transient loss of consciousness, who had recurrent seizures in infancy with progressive brain calcification. On admission, he was alert but magnetic resonance angiography showed bilateral stenosis of the whole internal carotid artery and proliferation of vascular collaterals, and brain CT revealed calcification on bilateral putamen. Given that this fulfilled diagnostic criteria, we finally diagnosed him as having moyamoya syndrome, though the etiology was unclear. Interestingly, a whole vessel survey revealed vascular stenosis of abdominal aorta and renal arteries, in which the former has not been reported in moyamoya syndrome. We considered that brain calcification was gradually formed by decreased cerebral vascular flow from infancy, and stenosis of abdominal aorta was possibly extended from renal arteries. This is, moyamoya syndrome with brain calcification and stenosis of abdominal aorta, suggesting that morphological screening of whole vessels containing cerebral and abdominal arteries should be considered in cases of slowly progressive brain calcification. Copyright © 2017 The Japanese Society of Child Neurology. Published by Elsevier B.V. All rights reserved.

  14. Rapidly progressive coronary artery disease as the first manifestation of antiphospholipid syndrome.

    PubMed

    Abdullah, Abdullah Sayied; Yagoub, Hatim; Kiernan, Thomas J; Daly, Caroline

    2014-04-08

    Antiphospholipid syndrome (APS) is an autoimmune multisystem disorder characterised by high incidence of arterial and venous thrombosis. Cardiovascular manifestations also include valvular heart disease, ventricular thrombi and higher risk for coronary artery disease (CAD). In this case report, we describe a 61-year-old woman who had no significant risk factors for CAD, and presented with aggressive disease in native and graft vessels that required multiple coronary interventions. The extent of her aggressive CAD could not be explained by her risk factors profile. Therefore autoantibodies screening was carried out and showed a strongly positive anticardiolipin and β2 glycoprotein-I antibody, and hence a diagnosis of antiphospholipid syndrome was made.

  15. Reversible vasoconstriction syndrome involving the basilar artery in an adolescent: imaging and clinical features.

    PubMed

    Guerriero, Réjean M; Rivkin, Michael J

    2015-06-01

    Reversible cerebral vasoconstriction syndrome is characterized by recurrent episodes of "thunderclap headache" and by transient, multifocal vasoconstriction of cerebral vasculature. Here we present an adolescent boy whose clinical features fit the diagnostic criteria and whose neurovascular imaging revealed reversible vasoconstriction of the basilar artery alone. A previously healthy 14-year-old boy presented with repeated severe sudden thunderclap headaches following exercise. These symptoms were accompanied by isolated basilar artery stenosis. Reversible cerebral vasoconstriction syndrome is a condition with several clinical triggers. Its pathophysiology is poorly understood. This patient adds to a broadening spectrum of clinical features of this disorder. Copyright © 2015 Elsevier Inc. All rights reserved.

  16. Tarsal tunnel syndrome associated with a perforating branch from posterior tibial artery: a case report.

    PubMed

    Kosiyatrakul, Arkaphat; Luenam, Suriya; Phisitkul, Phinit

    2015-03-01

    Tarsal tunnel syndrome, a compressive neuropathy of the tibial nerve or its branches with in the tarsal tunnel, is an uncommon condition. Various etiologies of the syndrome have been described. We report a rare case of tarsal tunnel syndrome associated with a perforating branch from the posterior tibial artery. A 56-year-old woman presented with 1-year history of paresthesia and hypoesthesia in the medial and lateral plantar area of the left foot. Tinel's sign was elicited at the tarsal tunnel. Electrodiagnostic studies confirmed the diagnosis of left tarsal tunnel syndrome. Intraoperatively, the perforating branch from posterior tibial artery which traveled through a split in the tibial nerve was encountered. The patient's symptom improved significantly at 2 years after tarsal tunnel release and vascular ligation. Only a minor degree of paresthesia remains in the forefoot.

  17. Bilateral internal carotid artery occlusion associated with the antiphospholipid antibody syndrome.

    PubMed

    Anand, Pria; Mann, Sharan K; Fischbein, Nancy J; Lansberg, Maarten G

    2014-01-01

    A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies.

  18. Bilateral Internal Carotid Artery Occlusion Associated with the Antiphospholipid Antibody Syndrome

    PubMed Central

    Anand, Pria; Mann, Sharan K.; Fischbein, Nancy J.; Lansberg, Maarten G.

    2014-01-01

    A 39-year-old woman presented with a right-hemispheric stroke 1 year after she had suffered a left-hemispheric stroke. Her diagnostic workup was notable for bilateral occlusions of the internal carotid arteries at their origins and a positive lupus anticoagulant antibody test. There was no evidence of carotid dissection or another identifiable cause for her carotid occlusions. These findings suggest that the antiphospholipid antibody syndrome may be implicated in the pathological changes that resulted in occlusions of the extracranial internal carotid arteries. Young stroke patients who present with unexplained internal carotid artery occlusions may benefit from testing for the presence of antiphospholipid antibodies. PMID:24707268

  19. Popliteal Artery Entrapment Syndrome: Diagnosis and Management, with Report of Three Cases

    PubMed Central

    Radonić, Vedran; Koplić, Stevan; Giunio, Lovel; Boz̆ić, Ivo; Mas̆ković, Josip; Buća, Ante

    2000-01-01

    Popliteal artery entrapment syndrome is an important albeit infrequent cause of serious disability among young adults and athletes with anomalous anatomic relationships between the popliteal artery and surrounding musculotendinous structures. We report our experience with 3 patients, in whom we used duplex ultrasonography, computed tomography, digital subtraction angiography, and conventional arteriography to diagnose popliteal artery entrapment and to grade the severity of dynamic circulatory insufficiency and arterial damage. We used a posterior surgical approach to give the best view of the anatomic structures compressing the popliteal artery. In 2 patients, in whom compression had not yet damaged the arterial wall, operative decompression of the artery by resection of the aberrant muscle was sufficient. In the 3rd patient, operative reconstruction of an occluded segment with autologous vein graft was necessary, in addition to decompression of the vessel and resection of aberrant muscle. The result in each case was complete recovery, with absence of symptoms and with patency verified by Doppler examination. We conclude that clinicians who encounter young patients with progressive lower-limb arterial insufficiency should be aware of the possibility of popliteal artery entrapment. Early diagnosis through a combined approach (careful physical examination and history-taking, duplex ultrasonography, computerized tomography, and angiography) is necessary for exact diagnosis. The treatment of choice is the surgical creation of normal anatomy within the popliteal fossa. PMID:10830621

  20. Stiffness of the large arteries in individuals with and without Down syndrome.

    PubMed

    Rodrigues, Anabel N; Coelho, Luan Cesar; Goncalves, Washington L S; Gouvea, Sonia Alves; Vasconcellos, Maria José Rossi; Cunha, Roberto S; Abreu, Glaucia R

    2011-01-01

    Down syndrome is known to cause premature aging in several organ systems. However, it remains unclear whether this aging effect also affects the structure and function of the large arterial trunks. In this controlled study, the possibility of changes in the large arteries due to aging was evaluated in patients with Down syndrome. Eighty-two subjects of both genders were selected. The Down syndrome group had 41 active subjects consisting of 19 males and 22 females (mean age 21 ± 1, range 13-42 years) without cardiovascular complications and who did not use vasoactive drugs. The control group consisted of 41 healthy individuals without trisomy 21 of the same gender and age as the Down syndrome group and who did not use vasoactive medication. Carotid-femoral pulse wave velocity was obtained as an index of aortic stiffness using an automatic noninvasive method. Individuals with Down syndrome had significantly lower blood pressure than those in the control group. Systolic blood pressure for the Down syndrome group and control group was 106 ± 2 mmHg vs 117 ± 2 mmHg (P < 0.001), respectively; diastolic blood pressure was 66 ± 2 mmHg vs 77 ± 2 mmHg (P < 0.001); and mean arterial pressure was 80 ± 1 mmHg vs 90 ± 1 mmHg (P < 0.001). Only age and systolic blood pressure were shown to correlate significantly with pulse wave velocity, but the slopes of the linear regression curves of these two variables showed no significant difference between the two study groups. Pulse wave velocity, which was initially significantly lower in the Down syndrome group (7.51 ± 0.14 m/s vs 7.84 ± 0.12 m/s; P <0.05), was similar between the groups after systolic blood pressure adjustment (7.62 ± 0.13 m/s vs 7.73 ± 0.13 m/s). Despite evidence in the literature that patients with Down syndrome undergo early aging, this process does not seem to affect the large arterial trunks, given that values of carotid-femoral pulse wave velocity were similar in individuals with or without trisomy 21

  1. Stiffness of the large arteries in individuals with and without Down syndrome

    PubMed Central

    Rodrigues, Anabel N; Coelho, Luan Cesar; Goncalves, Washington LS; Gouvea, Sonia Alves; Vasconcellos, Maria José Rossi; Cunha, Roberto S; Abreu, Glaucia R

    2011-01-01

    Background: Down syndrome is known to cause premature aging in several organ systems. However, it remains unclear whether this aging effect also affects the structure and function of the large arterial trunks. In this controlled study, the possibility of changes in the large arteries due to aging was evaluated in patients with Down syndrome. Methods: Eighty-two subjects of both genders were selected. The Down syndrome group had 41 active subjects consisting of 19 males and 22 females (mean age 21 ± 1, range 13–42 years) without cardiovascular complications and who did not use vasoactive drugs. The control group consisted of 41 healthy individuals without trisomy 21 of the same gender and age as the Down syndrome group and who did not use vasoactive medication. Carotid–femoral pulse wave velocity was obtained as an index of aortic stiffness using an automatic noninvasive method. Results: Individuals with Down syndrome had significantly lower blood pressure than those in the control group. Systolic blood pressure for the Down syndrome group and control group was 106 ± 2 mmHg vs 117 ± 2 mmHg (P < 0.001), respectively; diastolic blood pressure was 66 ± 2 mmHg vs 77 ± 2 mmHg (P < 0.001); and mean arterial pressure was 80 ± 1 mmHg vs 90 ± 1 mmHg (P < 0.001). Only age and systolic blood pressure were shown to correlate significantly with pulse wave velocity, but the slopes of the linear regression curves of these two variables showed no significant difference between the two study groups. Pulse wave velocity, which was initially significantly lower in the Down syndrome group (7.51 ± 0.14 m/s vs 7.84 ± 0.12 m/s; P <0.05), was similar between the groups after systolic blood pressure adjustment (7.62 ± 0.13 m/s vs 7.73 ± 0.13 m/s). Conclusion: Despite evidence in the literature that patients with Down syndrome undergo early aging, this process does not seem to affect the large arterial trunks, given that values of carotid-femoral pulse wave velocity were

  2. Aortic arch aneurysm, pseudocoarctation, and coronary artery disease in a patient with Behçet's syndrome.

    PubMed

    Bardakci, Hasmet; Kervan, Umit; Boysan, Emre; Birincioglu, Levent; Cobanoglu, Adnan

    2007-01-01

    Aortic arch aneurysm, pseudocoarctation, and coronary artery stenosis are extremely rare in Behçet's syndrome. We present the case of a 25-year-old man with Behçet's syndrome who underwent coronary artery bypass grafting for severe stenosis in the proximal left anterior descending coronary artery and concomitant surgical correction of a saccular aneurysm that was causing pseudocoarctation of the aortic arch. The surgery was successful.

  3. Galactorrhoea amenorrhoea syndrome due to internal carotid artery aneurysm.

    PubMed Central

    Garg, S. K.; Dash, R. J.

    1985-01-01

    A 32 year old female with hyperprolactinaemia-galactorrhoea-amenorrhoea due to a right internal carotid artery aneurysm just before its bifurcation is described. She had two episodes of subarachnoid haemorrhage necessitating an emergency internal carotid artery ligation. She responded to bromocriptine treatment with restoration of her menses, normalization of circulating prolactin and disappearance of galactorrhoea. Images Figure 1 Figure 2 Figure 3 PMID:4040632

  4. Abnormal cutaneous flexor reflex activity during controlled isometric plantarflexion in human spinal cord injury spasticity syndrome.

    PubMed

    Gómez-Soriano, J; Bravo-Esteban, E; Pérez-Rizo, E; Ávila-Martín, G; Galán-Arriero, I; Simón-Martinez, C; Taylor, J

    2016-09-01

    Although abnormal cutaneous reflex (CR) activity has been identified during gait after incomplete spinal cord injury (SCI), this activity has not been directly compared in subjects with and without the spasticity syndrome. Characterisation of CR activity during controlled rest and 'ramp and hold' phases of controlled plantarflexion in subjects with and without the SCI spasticity syndrome. Transverse descriptive study with non-parametric group analysis. SCI rehabilitation hospital. Tibialis Anterior (TA) reflexes were evoked by innocuous cutaneous plantar sole stimulation during rest and ramp and hold phases of plantarflexion torque in non-injured subjects (n=10) and after SCI with (n=9) and without (n=10) hypertonia and/or involuntary spasm activity. Integrated TA reflex responses were analysed as total (50-300 ms) or short (50-200 ms) and long-latency (200-300 ms) activity. Total and long-latency TA activity was inhibited in non-injured subjects and the SCI group without the spasticity syndrome during plantarflexion torque but not in the SCI spasticity group. Furthermore, loss of TA reflex inhibition during plantarflexion correlated with time after SCI (ρ=0.79, P=0.009). Moreover, TA reflex activity inversely correlated with maximum plantarflexion torque in the spasticity group (ρ=-0.75, P=0.02), despite similar non-reflex TA electromyographic activity during plantarflexion after SCI in subjects with (0.11, 0.08-0.13 mV) or without the spasticity syndrome (0.09, 0.07-0.12 mV). This reflex testing procedure supports previously published evidence for abnormal CR activity after SCI and may characterise the progressive disinhibition of TA reflex activity during controlled plantarflexion in subjects diagnosed with the spasticity syndrome.

  5. The influence of the relative timing of arterial and subarachnoid space pulse waves on spinal perivascular cerebrospinal fluid flow as a possible factor in syrinx development.

    PubMed

    Bilston, Lynne E; Stoodley, Marcus A; Fletcher, David F

    2010-04-01

    The mechanisms of syringomyelia have long puzzled neurosurgeons and researchers alike due to difficulties in identifying the driving forces behind fluid flow into a syrinx, apparently against a pressure gradient between the spinal cord and the subarachnoid space (SAS). Recently, the synchronization between CSF flow and the cardiac cycle has been postulated to affect fluid flow in the spinal cord. This study aims to determine the effect of changes in the timing of SAS pressure on perivascular flow into the spinal cord. This study uses a computational fluid dynamics model to investigate whether the relative timing of a spinal artery cardiovascular pulse wave and fluid pressure in the spinal SAS can influence CSF flow in the perivascular spaces. The results show that the mass flow rate of CSF through a model periarterial space is strongly influenced by the relative timing of the arterial pulse wave and the SAS pressure. These findings suggest that factors that might alter the timing of the pulse wave or the fluid flow in the SAS could potentially affect fluid flow into a syrinx.

  6. Does This Older Adult With Lower Extremity Pain Have the Clinical Syndrome of Lumbar Spinal Stenosis?

    PubMed Central

    Suri, Pradeep; Rainville, James; Kalichman, Leonid; Katz, Jeffrey N.

    2012-01-01

    Context The clinical syndrome of lumbar spinal stenosis (LSS) is a common diagnosis in older adults presenting with lower extremity pain. Objective To systematically review the accuracy of the clinical examination for the diagnosis of the clinical syndrome of LSS. Data Sources MEDLINE, EMBASE, and CINAHL searches of articles published from January 1966 to September 2010. Study Selection Studies were included if they contained adequate data on the accuracy of the history and physical examination for diagnosing the clinical syndrome of LSS, using a reference standard of expert opinion with radiographic or anatomic confirmation. Data Extraction Two authors independently reviewed each study to determine eligibility, extract data, and appraise levels of evidence. Data Synthesis Four studies evaluating 741 patients were identified. Among patients with lower extremity pain, the likelihood of the clinical syndrome of LSS was increased for individuals older than 70 years (likelihood ratio [LR], 2.0; 95% confidence interval [CI], 1.6–2.5), and was decreased for those younger than 60 years (LR, 0.40; 95% CI, 0.29–0.57). The most useful symptoms for increasing the likelihood of the clinical syndrome of LSS were having no pain when seated (LR, 7.4; 95% CI, 1.9–30), improvement of symptoms when bending forward (LR, 6.4; 95% CI, 4.1–9.9), the presence of bilateral buttock or leg pain (LR, 6.3; 95% CI, 3.1–13), and neurogenic claudication (LR, 3.7; 95% CI, 2.9–4.8). Absence of neurogenic claudication (LR, 0.23; 95% CI, 0.17–0.31) decreased the likelihood of the diagnosis. A wide-based gait (LR, 13; 95% CI, 1.9–95) and abnormal Romberg test result (LR, 4.2; 95% CI, 1.4–13) increased the likelihood of the clinical syndrome of LSS. A score of 7 or higher on a diagnostic support tool including history and examination findings increased the likelihood of the clinical syndrome of LSS (LR, 3.3; 95% CI, 2.7–4.0), while a score lower than 7 made the diagnosis much less

  7. Metachronous Bilateral Posterior Tibial Artery Aneurysms in Ehlers-Danlos Syndrome Type IV

    SciTech Connect

    Hagspiel, Klaus D.; Bonatti, Hugo; Sabri, Saher; Arslan, Bulent; Harthun, Nancy L.

    2011-04-15

    Ehlers-Danlos syndrome type IV is a life-threatening genetic connective tissue disorder. We report a 24-year-old woman with EDS-IV who presented with metachronous bilateral aneurysms/pseudoaneurysms of the posterior tibial arteries 15 months apart. Both were treated successfully with transarterial coil embolization from a distal posterior tibial approach.

  8. Branch retinal artery occlusion with visual field and multifocal erg in Susac syndrome: a case report.

    PubMed

    van Winden, Marianne; Salu, Paul

    2010-12-01

    A 19-year-old woman presented with subacute encephalopathy and subsequently developed hearing loss and occlusions of branches of the central retinal artery. The triad of microangiopathy of the brain, retina and cochlea is typical for Susac syndrome. The etiology of this syndrome is still unknown, but the prognosis is good in most cases. Spontaneous resolution usually occurs, but early treatment minimizes the risk of sequelae. Multifocal ERG may be useful to assess subclinical retinal dysfunction after recovery of subjective symptoms of BRAO in Susac syndrome.

  9. Spontaneous coronary artery dissection causing acute coronary syndrome in a young patient without risk factors

    PubMed Central

    Chevli, Parag; Kelash, Fnu; Gadhvi, Pragnesh; Grandhi, Sreeram; Syed, Amer

    2014-01-01

    Spontaneous coronary artery dissection (SCAD) is a rare cause of acute myocardial infarction that is more common in younger patients (under age 50) and in women. Although the etiology is not known, some predisposing conditions to SCAD are well known and include Marfan syndrome, pregnancy and peripartum state, drug abuse, and some anatomical abnormalities of the coronary arteries such as aneurysms and severe kinking. We describe a case of SCAD in a young woman who presented with sudden onset of chest pain and was admitted for the treatment of acute coronary syndrome. The coronary angiography showed dissection of the left anterior descending artery. The patient underwent successful percutaneous transluminal coronary angioplasty and stent placement. PMID:25317268

  10. Aortic and pulmonary artery calcification: An unusual manifestation of twin-to-twin transfusion syndrome

    PubMed Central

    Venkatesh, Sumitra; Sanyukta, J; Jain, S; Prabhu, SS; Kulkarni, S

    2017-01-01

    Twin-to-twin transfusion syndrome (TTTS) at times complicates monochorionic twin gestations, resulting in conditions ranging from discordant sizes to fetal demise of one baby. Various types of cardiac defects have been described in the recipient twin of this syndrome. Isolated great artery calcification, i.e. aortic and pulmonary artery calcification is one such uncommon condition associated with TTTS. Calcification of the walls of great vessels may be due to chronic vascular injury sustained as a result of circulatory volume overload in the recipient twin. It may also cause severe systemic hypertension and cardiomyopathy. An accurate diagnosis is important for an optimal follow-up and appropriate genetic counseling. We report a case of aortic and pulmonary artery calcification in association with TTTS. PMID:28163434

  11. Aortic and pulmonary artery calcification: An unusual manifestation of twin-to-twin transfusion syndrome.

    PubMed

    Venkatesh, Sumitra; Sanyukta, J; Jain, S; Prabhu, S S; Kulkarni, S

    2017-01-01

    Twin-to-twin transfusion syndrome (TTTS) at times complicates monochorionic twin gestations, resulting in conditions ranging from discordant sizes to fetal demise of one baby. Various types of cardiac defects have been described in the recipient twin of this syndrome. Isolated great artery calcification, i.e. aortic and pulmonary artery calcification is one such uncommon condition associated with TTTS. Calcification of the walls of great vessels may be due to chronic vascular injury sustained as a result of circulatory volume overload in the recipient twin. It may also cause severe systemic hypertension and cardiomyopathy. An accurate diagnosis is important for an optimal follow-up and appropriate genetic counseling. We report a case of aortic and pulmonary artery calcification in association with TTTS.

  12. Transcriptome Analysis and Gene Identification in the Pulmonary Artery of Broilers with Ascites Syndrome

    PubMed Central

    Xiao, Qingyang; Guo, Xiaoquan; Zhuang, Yu; Zhang, Caiying; Wang, Tiancheng; Lin, Huayuan; Song, Yalu; Hu, Guoliang; Liu, Ping

    2016-01-01

    Background Pulmonary arterial hypertension, also known as Ascites syndrome (AS), remains a clinically challenging disease with a large impact on both humans and broiler chickens. Pulmonary arterial remodeling presents a key step in the development of AS. The precise molecular mechanism of pulmonary artery remodeling regulating AS progression remains unclear. Methodology/Principal Findings We obtained pulmonary arteries from two positive AS and two normal broilers for RNA sequencing (RNA-seq) analysis and pathological observation. RNA-seq analysis revealed a total of 895 significantly differentially expressed genes (DEGs) with 437 up-regulated and 458 down-regulated genes, which were significantly enriched to 12 GO (Gene Ontology) terms and 4 KEGG (Kyoto Encyclopedia of Genes and Genomes) pathways (Padj<0.05) regulating pulmonary artery remodeling and consequently occurrence of AS. These GO terms and pathways include ribosome, Jak-STAT and NOD-like receptor signaling pathways which regulate pulmonary artery remodeling through vascular smooth cell proliferation, inflammation and vascular smooth cell proliferation together. Some notable DEGs within these pathways included downregulation of genes like RPL 5, 7, 8, 9, 14; upregulation of genes such as IL-6, K60, STAT3, STAT5 Pim1 and SOCS3; IKKα, IkB, P38, five cytokines IL-6, IL8, IL-1β, IL-18, and MIP-1β. Six important regulators of pulmonary artery vascular remodeling and construction like CYP1B1, ALDH7A1, MYLK, CAMK4, BMP7 and INOS were upregulated in the pulmonary artery of AS broilers. The pathology results showed that the pulmonary artery had remodeled and become thicker in the disease group. Conclusions/Significance Our present data suggested some specific components of the complex molecular circuitry regulating pulmonary arterial remodeling underlying AS progression in broilers. We revealed some valuable candidate genes and pathways that involved in pulmonary artery remodeling further contributing to the AS

  13. Acute Onset of Guillain-Barré Syndrome After Elective Spinal Surgery.

    PubMed

    Boghani, Zain; Livingston, Andrew D; Simpson, Ericka P; Holman, Paul J; Grossman, Robert G

    2015-08-01

    Guillain-Barré syndrome (GBS) is an acute peripheral neuropathy caused by an autoimmune response against myelin of peripheral nerves. GBS has been reported after surgery, in general, and after spinal surgery, in particular. In most cases, GBS developed 1-3 weeks after surgery. Report of 2 cases of GBS after elective spine surgery that developed in the immediate postoperative period. Within 1 and 3 hours after surgery, respectively, both patients developed ascending loss of motor and sensory function. They were taken back urgently to the operating room for wound exploration to ensure that an epidural hematoma had not developed. Cerebrospinal fluid studies and electromyography/nerve conduction velocity were then rapidly obtained and were compatible with acute inflammatory demyelinating polyradiculoneuropathy. Therapy was initiated with administration of intravenous immunoglobulin and plasmapheresis. Both patients made substantial motor recovery during the course of 1-2 years but have residual sensory abnormalities. GBS developing acutely after spinal surgery is a rare occurrence but should be considered in the differential diagnosis of neurological deterioration after surgery. Rapid diagnosis and treatment are essential for recovery of neurological function. Copyright © 2015 Elsevier Inc. All rights reserved.

  14. Persistent cauda equina syndrome after caudal epidural injection under severe spinal stenosis: a case report.

    PubMed

    Seo, Young Tak; Kong, Hyun Ho; Lee, Goo Joo; Bang, Heui Je

    2017-01-01

    Caudal epidural injection (CEI) is one of the most common treatments for low-back pain with sciatica. CEI rarely leads to neurologic complications. We report a case of persistent cauda equina syndrome after CEI. A 44-year-old male patient with severe L4 and L5 spinal ste-nosis underwent CEI for low-back pain and sciatica. The CEI solution consisted of bupivacaine, hyaluronidase, triamcinolone acetonide, and normal saline. He experienced motor weakness and sensory loss in both lower extremities and neurogenic bladder for more than 1 year after the procedure. His ankle dorsiflexors, big-toe extensors, and ankle plantar flexors on both sides were checked and categorized as motor-power Medical Research Council grade 0. His bilateral ankle-jerk reflection was absent. An electrophysiological study showed lumbosacral polyradiculopathy affecting both sides of the L5 and S1 nerve roots. A urodynamic study revealed hypoactive neurogenic bladder affecting both sacral roots.

  15. Spinal muscular atrophy with respiratory distress syndrome (SMARD1): Case report and review of literature

    PubMed Central

    Lingappa, Lokesh; Shah, Nikit; Motepalli, Ananth Sagar; Shaik, Farhan

    2016-01-01

    Spinal muscular atrophy with respiratory distress syndrome (SMARD1) is a rare cause of early infantile respiratory failure and death. No cases have been currently described from India. Two low-birth-weight infants presented prior to 6 months of age with recurrent apnea and respiratory distress. Both required prolonged ventilation, and had distal arthrogryposis and diaphragmatic eventration. Nerve conduction study revealed motor sensory axonopathy. Genetic testing confirmed mutations in immunoglobulin mu binding protein (IGHMBP2). These two cases establish presence of SMARD1 in our population. Both infants died on discontinuation of ventilation. Antenatal diagnoses done in one pregnancy. Though rare, high index of suspicion is essential in view of poor outcome and aid antenatal counseling. PMID:27570397

  16. Guillain-Barre syndrome: A possibility in a spinal cord injured patient.

    PubMed

    Jagatsinh, Yogendrasinh

    2007-07-01

    A 28-year-old male had paraplegia as a result of fracture dislocation of T12/L1 six years ago. He was functioning independently until four weeks ago, when he started complaining of trunkal paraesthesia which later progressed to include the upper extremities. The initial diagnosis was that of posttraumatic syringomyelia (PTS). While awaiting the MRI scan he developed weakness of upper limbs. The weakness restricted his self-care activities including transfers. The MRI did not show any evidence of syringomyelia. Neurological consultation and assessment yielded provisional diagnosis of Guillain-Barre syndrome (GBS). The patient was treated with immunoglobulins and regained 90% of his previous neurological status. This case is reported to raise awareness among clinicians to include the possibility of the GBS in the differential diagnosis of progressive neurological loss on top of existing neurological deficiency in spinal cord injured patients.

  17. [Throat infection, neck spinal disease, chest pain and cardiac response: a new clinical syndrome?].

    PubMed

    Zhou, Chang-Qing; Yan, Jiang-Tao; Fan, Qiao; Li, Zhou-Ya; Cianflone, Katherine; Wang, Dao-Wen

    2010-02-01

    To analyze the characteristics of a new clinical syndrome, including throat infection, neck spinal disease, chest pain and cardiac response. A total of 165 patients with above mentioned symptoms admitted to Tongji hospital from 2003 to 2005 were included in this study and underwent further medical history inquiry, physical examination and laboratory tests. Eighty-five healthy subjects served as controls. Serum myocardial auto-antibodies against beta(1)-adrenoceptor, alpha-myosin heavy chain, M(2)-muscarinic receptor and adenine-nucleotide translocator were detected, inflammatory cytokines, high sensitivity C-reaction protein, serum antibodies against Coxsackie virus-B, cytomegalovirus, Mycoplasma pneumoniae and Chlamydia pneumoniae were determined and lymphocyte subclasses were assayed by flow cytometry. All patients had a complex of four symptoms or tetralogy: (1) persistent throat or upper respiratory tract infection; (2) neck pain; (3) chest pain; (4) chest depression or dyspnea, some of them with anxiety. Anti-myocardial auto-antibodies (AMCA) were present in all patients vs. 8% in controls. TNF-alpha, IL-1 and IL-6 were significantly higher in patients than controls (P < 0.01). CD3(+) and CD4(-)CD8(+) lymphocytes were significantly higher and CD56(+) lymphocytes lower in patients than those in controls (P < 0.01). The ratios of serum pathogen antibodies positive against Coxsackie virus-B, cytomegalovirus, Mycoplasma pneumoniae and Chlamydia pneumoniae were all significantly higher in patients than in controls. These data led to identification of a persistent respiratory infection-related clinical syndrome, including persistent throat infection, neck spinal lesion, rib cartilage inflammation, symptoms of cardiac depression and dyspnea with or without anxiety.

  18. Agenesis of internal carotid artery in a child with ipsilateral Horner's syndrome.

    PubMed

    Fons, Carmen; Vasconcelos, Monica; Vidal, Mariona; Puy, Ramón; Capdevila, Antonio; Sanchez, Laia; Campistol, Jaime

    2009-01-01

    Horner's syndrome is characterized by a classic triad of ipsilateral pupillary miosis, partial eyelid ptosis, and facial anhydrosis. This case study reports a 7-year-old boy with right miosis, mild blepharoptosis, and iris hypopigmentation detected in a routine pediatric follow-up without ipsilateral facial anhydrosis, flushing, or pain. There was no history of birth trauma and test with cocaine provoked no response of the right pupil, suggesting right Horner's syndrome. Mediastinal tumor was ruled out and brain magnetic resonance imaging incidentally showed absence of flow in the right internal carotid artery. Subsequent magnetic resonance angiography demonstrated agenesis of the right internal carotid artery without other vascular-associated malformations. The final diagnosis was right, congenital Horner's syndrome due to ipsilateral internal carotid agenesis. We describe in detail the radiological findings and pathophysiological mechanisms of this unusual association.

  19. Pathophysiology of carotid artery disease and related clinical syndromes.

    PubMed

    De Reuck, J L

    2004-02-01

    The severity of internal carotid artery lesion per se is a poor indicator of the cerebral haemodynamic status of the brain. Positron emission tomography (PET) of the brain allows to study the different pathophysiological changes related to carotid artery disease. Several stages of impairment of cerebral blood flow and metabolism can be shown such as the compensatory phases by the haemodynamic and the metabolic reserve, the transition to reversible (penumbra) and irreversible ischaemia and the phase of luxury perfusion. Distinct PET patterns correlate with certain clinical outcomes. PET studies found evidence for an embolic origin rather than for a low-flow status as most frequent cause of stroke in severe carotid artery disease. Except for deep borderzone infarcts in the centrum semi-ovale, most cortical borderzone infarcts are not due to chronic misery perfusion. Recent silent and recurrent infarcts can be demonstrated with Cobalt-55 PET, which is of importance for the therapeutical decision. The PET studies argue that carotid endarterectomy is successful probably by removing the source of emboli rather than by restoring the cerebral perfusion in severe carotid artery stenosis.

  20. Congenital Spinal Malformation and Stroke: Aneurysmal Dilatations and Bilateral Rotational Vertebral Artery Occlusion.

    PubMed

    de la Riva, Patricia; Martínez-Zabaleta, Maria Teresa; Pardo, Edurne; Samprón, Nicolás; Mondragón-Rezola, Elisabet; Arruti González, Maialen; Larrea, Jose Ángel; Martí-Massó, José Félix

    2016-03-01

    A 30-year-old woman suffered from acute vertebrobasilar stroke. Cranial tomography (CT) scans showed multiple vertebral abnormalities suggestive of congenital spine malformation, and angiographic CT revealed aneurysmal dilatations (ADs) at segment V2 of both vertebral arteries (VAs). Dynamic neuroimaging tests including angiography and angio-CT were performed and showed occlusion of both VAs at the point of the ADs with contralateral rotation of the neck. The presence of a bony structure causing the artery compression was excluded and embolic phenomena originating at the AD was proposed as the likely source of stroke. Even if infrequent, the presence of craniocervical anomalies should be considered in vertebrobasilar stroke of indeterminate etiology.

  1. Endovascular treatment of hepatic artery aneurysm in a patient with Ehlers-Danlos syndrome. Case report.

    PubMed

    Casana, R; Nano, G; Dalainas, I; Tealdi, D G

    2004-09-01

    Ehlers-Danlos syndrome (EDS) is a rare, variably inherited disorder affecting connective tissue. Patients with EDS often develop aneurysms of the thoracic and abdominal aorta as well as the visceral arteries. We report our experience with an elective endovascular exclusion of an hepatic artery aneurysm in a 26-year-old female patient with EDS type IV. A balloon-expandable 4x26 mm stent-graft was placed in the common hepatic artery, excluding the aneurysm. The follow-up at the 2, 6 and 18 months showed the patency of the common hepatic artery, the absence of endo-leak and the right location of the stent-graft. In EDS patients, the endovascular treatment could be preferable to open surgery because of the minimum trauma.

  2. Reversible cerebral vasoconstriction syndrome with multivessel cervical artery dissections and a double aortic arch.

    PubMed

    Nouh, Amre; Ruland, Sean; Schneck, Michael J; Pasquale, David; Biller, José

    2014-02-01

    Reversible cerebral vasoconstriction syndrome (RCVS) has been associated with exposure to vasoactive substances and few reports with cervical arterial dissections (CADs). We evaluated a 32-year-old woman with history of depression, migraines without aura, and cannabis use who presented with a thunderclap headache unresponsive to triptans. She was found to have bilateral occipital infarcts, bilateral extracranial vertebral artery dissections, bilateral internal carotid artery dissecting aneurysms, and extensive distal multifocal segmental narrowing of the anterior and posterior intracranial circulation with a "sausage on a string-like appearance" suggestive of RCVS. Subsequently, she was found to have a distal thrombus of the basilar artery, was anticoagulated, and discharged home with no residual deficits. We highlight the potential association of CADs and RCVS. The association of RCVS and a double aortic arch has not been previously reported. Copyright © 2014 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  3. Usefulness of spinal magnetic resonance imaging in patients with myelodysplastic syndromes

    PubMed Central

    Kwiatkowska-Pamuła, Anna; Ziółko, Ewa; Muc-Wierzgoń, Małgorzata; Nowakowska-Zajdel, Ewa; Podwińska, Ewa; Adamczyk, Tomasz

    2013-01-01

    Summary Background: Myelodysplastic syndrome is a rare, chronic hematological disease characterized by heterogeneous clinical presentations. Subtypes of myelodysplastic syndrome are characterized by different survival times and ability to transform into acute myeloid leukemia. Objectives: The objective of the study included the assessment of the relationship between the images obtained by magnetic resonance scans of lumbar spine and the clinical symptoms of the disease in patients diagnosed with myelodysplastic syndrome, as well as the assessment of the correlation of the images with the phase of transformation into acute myeloid leukemia. Material/Methods: The study-related tests were carried out in Specialist Hospital No. 1 in Bytom between 2006 and 2011 and involved 53 patients aged 55÷77, divided into groups according to the diagnosed subtype of myelodysplastic syndrome. The study also included the prognosis of overall survival and time to transformation into AML on the basis of valid classifications. The spinal magnetic resonance scans were obtained from medical documentation. The analysis included images obtained using T1- and T2-weighted sequences in sagittal, transverse and frontal planes in all patients, images obtained using the STIR sequence from 21 patients as well as 40 images obtained after contrast administration. The statistical analysis of the results was carried out using STATISTICA software. Conclusions: The obtained results demonstrated that the magnetic resonance scans revealed statistically significant changes in the images of bone marrow in vertebral body scans; with a decrease in the intensity of MRI signals correlated with the RAEB subtype, particularly with transformation into acute myeloid leukemia as well as with the high IPSS risk score with regard to the time of survival and transformation into acute myeloid leukemia. The research-related test results indicate the importance of magnetic resonance imaging in diagnostics and the

  4. Motor deficits and recovery in rats with unilateral spinal cord hemisection mimic the Brown-Sequard syndrome.

    PubMed

    Filli, Linard; Zörner, Björn; Weinmann, Oliver; Schwab, Martin E

    2011-08-01

    Cervical incomplete spinal cord injuries often lead to severe and persistent impairments of sensorimotor functions and are clinically the most frequent type of spinal cord injury. Understanding the motor impairments and the possible functional recovery of upper and lower extremities is of great importance. Animal models investigating motor dysfunction following cervical spinal cord injury are rare. We analysed the differential spontaneous recovery of fore- and hindlimb locomotion by detailed kinematic analysis in adult rats with unilateral C4/C5 hemisection, a lesion that leads to the Brown-Séquard syndrome in humans. The results showed disproportionately better performance of hindlimb compared with forelimb locomotion; hindlimb locomotion showed substantial recovery, whereas the ipsilesional forelimb remained in a very poor functional state. Such a differential motor recovery pattern is also known to occur in monkeys and in humans after similar spinal cord lesions. On the lesioned side, cortico-, rubro-, vestibulo- and reticulospinal tracts and the important modulatory serotonergic, dopaminergic and noradrenergic fibre systems were interrupted by the lesion. In an attempt to facilitate locomotion, different monoaminergic agonists were injected intrathecally. Injections of specific serotonergic and noradrenergic agonists in the chronic phase after the spinal cord lesion revealed remarkable, although mostly functionally negative, modulations of particular parameters of hindlimb locomotion. In contrast, forelimb locomotion was mostly unresponsive to these agonists. These results, therefore, show fundamental differences between fore- and hindlimb spinal motor circuitries and their functional dependence on remaining descending inputs and exogenous spinal excitation. Understanding these differences may help to develop future therapeutic strategies to improve upper and lower limb function in patients with incomplete cervical spinal cord injuries.

  5. Post-traumatic pseudoaneurysm of the medial plantar artery combined with tarsal tunnel syndrome: two case reports.

    PubMed

    Park, Sang-Eun; Kim, Ji-Chang; Ji, Jong-Hun; Kim, Young-Yul; Lee, Hwan-Hee; Jeong, Jae-Jung

    2013-03-01

    Pseudoaneurysms in the foot are more often reported in the lateral plantar artery than the medial plantar artery, most likely because of its more superficial location. There are no reports of pseudoaneurysm of the medial plantar artery after trauma. We present two cases of pseudoaneurysm of the medial plantar artery after blunt foot trauma and foot laceration. This pseudoaneurysm compressed a posterior tibial nerve, resulting in tarsal tunnel syndrome. The patients were treated successfully using transcatheter embolization without the need for surgical intervention. The tarsal tunnel syndrome also subsided. Here, the authors report these cases and provide a review of literature.

  6. Anomalous Origin of the Left Common Carotid Artery from the Main Pulmonary Artery: A Rare Association in an Infant with CHARGE Syndrome

    PubMed Central

    Jones, Blaise; Hirsch, Russel

    2016-01-01

    Case Report. Isolated carotid artery originating from the pulmonary trunk is an exceedingly rare anomalous origin of head and neck vessels. We present this finding, along with a persistent embryonic trigeminal artery, in a male infant with multiple cardiac defects and other congenital anomalies associated with CHARGE syndrome. After extensive investigations, cardiac catheterization revealed the anomalous left common carotid artery arising from the cranial aspect of the main pulmonary artery. There was retrograde flow in this vessel, resulting from the lower pulmonary pressure, essentially stealing arterial supply from the left anterior cerebral circulation. The persistent left-sided trigeminal artery provided collateral flow from the posterior circulation to the left internal carotid artery territory, allowing for safe ligation of the anomalous origin of the left common carotid artery, thereby reversing the steal of arterial blood flow into the pulmonary circulation and resulting in a net improvement of cerebral perfusion. Conclusion. The possibility of this vascular anomaly should be considered in all infants with CHARGE syndrome. Surgical repair or ligation should be tailored to the specific patient circumstances, following a careful delineation of all sources of cerebral perfusion. PMID:27974985

  7. [Emergency coronary artery bypass grafting for acute coronary syndrome].

    PubMed

    Yamaguchi, Atsushi; Murayama, Takanori

    2012-09-01

    Since the drug eluting stents appeared in Japan, the indication for percutaneous coronary intervention has become wide-spread for the treatment of coronary artery disease. In the past decade, 216 patients underwent emergency/urgent coronary artery bypass grafting (CABG) in our institution, while the annual numbers of both emergency and elective CABG cases have declined. On the contrary to the decreasing number, emergency CABG patients were significantly getting older with multiple co-morbidities. Thus, off-pump CABG is likely to be beneficial for preventing postoperative complications, leading to the decreased postoperative mortality. When emergency CABG patients developed refractory myocardial ischemia and unstable hemodynamics, a percutaneous cardiopulmonary support system was quickly applicable and useful for on-pump beating CABG achieving complete surgical revascularization. For keeping satisfactory hemodynamics during CABG, communication between cardiovascular surgeons, anesthesiologists, and perfusionists is most important.

  8. Common Carotid Artery Stump Syndrome Due to Mobile Thrombus Detected by Carotid Duplex Ultrasonography.

    PubMed

    Omoto, Shusaku; Hasegawa, Yuki; Sakai, Kenichiro; Matsuno, Hiromasa; Arai, Ayumi; Terasawa, Yuka; Mitsumura, Hidetaka; Iguchi, Yasuyuki

    2016-10-01

    Carotid stump syndrome is a cause of recurrent embolic stroke following occlusion of the ipsilateral internal carotid artery. The present report describes a case of recurrent cerebral embolism ipsilateral to a chronically occluded left common carotid artery (CCA), i.e., "CCA stump syndrome." Doppler color flow imaging showed anterograde flow in the left internal and external carotid arteries, which were supplied by collateral flow from the superior thyroid artery inflowing just proximal to the left carotid bifurcation. According to carotid duplex ultrasonography (CDU), a low-echoic mobile thrombus was noted at the distal stump of the occluded CCA, which presumably caused distal embolism. The low-echoic mobile thrombus dramatically changed to a homogenously high-echoic thrombus, and there was no recurrence of stroke after antiplatelet and anticoagulant therapy. This is the first report to demonstrate a CDU-verified temporal change in the thrombus at the stump in CCA stump syndrome. CDU is a noninvasive and useful technique to characterize hemodynamics, thrombus morphology, and the response to therapy.

  9. Superior Mesenteric Artery Syndrome in a Young Military Basic Trainee

    DTIC Science & Technology

    2013-03-01

    computed tomography angiogram of the abdomen was an abnormally narrow takeoff angle of the superior mesenteric artery (SMA) from the aorta near the...loss of retroperitoneal fat , resulting in obstruction at the level of the third portion of duodenum as it is distended by food.3 It most commonly occurs...discomfort out of proportion to exam was concerning for intestinal angina. We obtained a computed tomography (CT) angiogram of the abdomen and surgical

  10. Spinal Cord Stimulation in Failed Back Surgery Syndrome: Review of Clinical Use, Quality of Life and Cost-Effectiveness

    PubMed Central

    Modrić, Marta; Paturej, Agnieszka; Malyshev, Stanislav M.; Przygocka, Agnieszka; Garnier, Hanna; Szmuda, Tomasz

    2016-01-01

    Failed back surgery syndrome (FBSS) is complex and recurrent chronic pain after spinal surgery. Several important patient and surgery related risk factors play roles in development of FBSS. Inadequate selection of the candidates for the spinal surgeries is one of the most crucial causes. The guidelines suggest that conservative management featuring pharmacologic approaches and rehabilitation should be introduced first. For therapy-refractory FBSS, spinal cord stimulation (SCS) is recommended in selected patients. Treatment efficacy for FBSS has increased over the years with the majority of patients experiencing pain relief and reduced medicinal load. Improved quality of life can also be achieved using SCS. Cost-effectiveness of SCS still remains unclear. However evidence for SCS role in FBSS is controversial, SCS can be beneficial for carefully classified patients. PMID:27994797

  11. Anaesthetic management of a child with Freeman-sheldon syndrome undergoing spinal surgery.

    PubMed

    Richa, F C; Yazbeck, P H

    2008-03-01

    Freeman-Sheldon syndrome, or distal arthrogryposis type 2A, is a rare congenital myopathy and dysplasia characterised by multiple contractures, abnormalities of the head and face, defective development of the hands and feet and skeletal malformations. The facial muscle contracture produces the typical 'whistling face' appearance. Anaesthetic issues include difficult intravenous access, difficult airway and postoperative pulmonary complications. Although an association with malignant hyperthermia has been suggested, this has not been confirmed. We report the management of a seven-year-old girl with Freeman-Sheldon syndrome undergoing anterior and posterior spinal surgery and describe a successful anaesthetic regimen based on a total intravenous anaesthesia technique with remifentanil and propofol without neuromuscular blocking agents. The child had an uneventful anaesthetic and postoperative course. We believe the presence of the myopathy warranted the use of a 'non-triggering' anaesthetic, as suxamethonium and volatile agents may be associated with significant complications such as muscle rigidity and rhabdomyolysis in myopathic patients, even in the absence of malignant hyperthermia.

  12. Central retinal artery occlusion following laser treatment for ocular ischemic aortic arch syndrome

    PubMed Central

    Shah, Payal J.; Ellis, Brian; DiGiovine, Lauren R.; Hogg, Jeffery P.; Leys, Monique J.

    2015-01-01

    Objective: Ocular ischemic syndrome is a rare blinding condition generally caused by disease of the carotid artery. We describe a 69-year-old female with a 50 pack-year smoking history with aortic arch syndrome causing bilateral ocular ischemic syndrome. Methods: The patient presented with progressive visual loss and temple pain. Slit lamp biomicroscopy revealed bilateral iris neovascularization. This finding prompted a cardiovascular work up. Panretinal photocoagulation with retrobulbar block was performed in the right eye. Results: A temporal artery biopsy was negative. The carotid duplex ultrasound showed only a 1–39% stenosis. MRA revealed a more proximal occlusion of the aortic branch for which she underwent subclavian carotid bypass surgery. At the one month follow up, the right eye suffered profound vision loss secondary to a central retinal artery occlusion. Conclusion: Ocular neovascularization may be one of the clinical manifestations of aortic arch syndrome. This case also illustrates the limitations of relying solely on carotid duplex ultrasound testing. We caution against overly aggressive panretinal photocoagulation utilizing retrobulbar anesthesia. PMID:27625958

  13. Botulinum toxin as a treatment for functional popliteal artery entrapment syndrome.

    PubMed

    Isner-Horobeti, Marie-Eve; Muff, Guillaume; Masat, Julien; Daussin, Jean-Luc; Dufour, Stephane P; Lecocq, Jehan

    2015-06-01

    Functional popliteal artery entrapment syndrome is responsible for exercise-induced muscle leg pain. This syndrome is caused, in most of the cases, by the excessive size of the gastrocnemius muscles. Currently, its treatment is based only on surgery with variable results. We report the case of a young professional soldier in a combat unit with bilateral functional popliteal artery entrapment syndrome that was confirmed by dynamic arteriography, magnetic resonance angiography, and ultrasonography and did not improve after bilateral popliteal arteriolysis without resection of the gastrocnemius medial head. Treatment by injecting botulinum toxin in the proximal part of the gastrocnemius muscles was proposed and carried out. Regular follow-up (from 1 month to 3 yr after botulinum toxin treatment) showed the disappearance of exercise-induced pain and the improvement of the patient's physical and sports performance. Results of follow-up ultrasonography during dynamic maneuvers at 2.5 months and 2 yr after botulinum toxin injection were normal. Neither adverse effects nor motor deficit of the gastrocnemius muscles was reported. This case report suggests that botulinum toxin treatment could be an alternative to surgery for patients with functional popliteal artery entrapment syndrome. Botulinum toxin could reduce functional compression and, consequently, exercise-induced pain by decreasing the volume of the gastrocnemius muscle.

  14. Vascular anatomy of the spinal cord

    SciTech Connect

    Thron, A.K.

    1988-01-01

    The book summarizes the anatomic guidelines of external blood supply to the spinal cord. The basic principles of arterial supply and venous drainage are illustrated by explicit schemes for quick orientation. In the first part of the book, systematic radiologic-anatomic investigations of the superficial and deep vessels of all segments of the spinal cord are introduced. The microvascular morphology is portrayed by numerous microradiographic sections in all three dimensions without overshadowing. The three-dimensional representation of the vascular architecture illustrates elementary outlines and details of arterial territories, anastomotic cross-linking as well as the capillary system, particularly the hitherto unknown structure of the medullary venous system with its functionally important anastomoses and varying regional structures. These often now radiologic-anatomic findings are discussed as to their functional and pathophysiologic impact and constitute the basic on which to improve one's understanding of vascular syndromes of the spinal cord.

  15. Superior Mesenteric Artery Syndrome: A Case Report of Two Surgical Options, Duodenal Derotation and Duodenojejunostomy

    PubMed Central

    2016-01-01

    Superior mesenteric artery (SMA) syndrome is a rare cause of duodenal obstruction and its management is usually conservative with nasojejunal feeding. The pathophysiology entails the loss of the fat pad between the superior mesenteric artery and the abdominal aorta. This reduces the angle between the two vessels to less than 20 degrees with the resultant compression of the third part of the duodenum. The surgical management is usually a laparoscopic duodenojejunostomy. The two cases in our series had two different surgical procedures with good outcomes in both patients. The surgical management of each patient should be determined on its own merits irrespective of the standard of care. PMID:28101395

  16. Superior Mesenteric Artery Syndrome: A Case Report of Two Surgical Options, Duodenal Derotation and Duodenojejunostomy.

    PubMed

    Pillay, Yagan

    2016-01-01

    Superior mesenteric artery (SMA) syndrome is a rare cause of duodenal obstruction and its management is usually conservative with nasojejunal feeding. The pathophysiology entails the loss of the fat pad between the superior mesenteric artery and the abdominal aorta. This reduces the angle between the two vessels to less than 20 degrees with the resultant compression of the third part of the duodenum. The surgical management is usually a laparoscopic duodenojejunostomy. The two cases in our series had two different surgical procedures with good outcomes in both patients. The surgical management of each patient should be determined on its own merits irrespective of the standard of care.

  17. Fontan Operation in a Patient with Severe Hypoplastic Right Pulmonary Artery, Single Ventricle, and Heterotaxy Syndrome

    PubMed Central

    Pan, Jun-Yen; Lin, Chu-Chuan; Chang, Jen-Ping

    2016-01-01

    Assessment of the pulmonary circulation status including pressure, resistance, size, and absence of anatomical distortion, is crucial to the successful Fontan operation. Most patients are found to have acceptable pulmonary arteries after previous palliation, although some degree of distortion is not uncommon. However, in rare instances, some patients have only one functioning lung with another pulmonary artery seriously hypoplastic or atretic. For theses patients, completion of a Fontan operation will be challenging. We reported a 17-year-old girl with a single ventricle and heterotaxy syndrome and only her left lung functioning, who underwent one-lung Fontan operation with a satisfactory result. PMID:27713611

  18. Extracranial internal carotid artery stenting in Moya-Moya syndrome: a case report.

    PubMed

    Casana, R; Tolva, V; Guy Bianchi, P; Dalainas, I

    2012-12-01

    A 58-year-old Caucasian lady presented for severe left internal carotid artery (ICA) stenosis. Two months before she was operated for right carotid endarterectomy (CEA) in another Institution, complicated with internal carotid artery thrombosis and development of transient hemiplegia and aphasia. Postoperative selective DSA showed the development of an abnormal basal meshwork of collateral vessels with typical evidence for moyamoya disease. Preoperative workup in our Institution included cerebral MRI witch showed two ischemic right frontal and parietal lesions. The patient underwent successful stenting of the left ICA. This is the first report of extracranial ICA stenting in a patient with moyamoya syndrome.

  19. Indications, algorithms, and outcomes for coronary artery bypass surgery in patients with acute coronary syndromes

    PubMed Central

    Yerokun, Babatunde A.; Williams, Judson B.; Gaca, Jeffrey; Smith, Peter K.; Roe, Matthew T.

    2016-01-01

    For patients with a non-ST-segment elevation acute coronary syndrome (NSTE-ACS), guideline recommendations and treatment pathways focus on revascularization for definitive treatment if the patient is an appropriate candidate. Despite the widespread use of revascularization for NSTE-ACS, most patients undergo a percutaneous coronary intervention, whereas a minority of patients undergo coronary artery bypass grafting. Focusing specifically on the USA, the contemporary utilization, preoperative and perioperative considerations, and outcomes of NSTE-ACS patients undergoing coronary artery bypass grafting have not been comprehensively reviewed. PMID:26945187

  20. Spontaneous coronary artery dissection in a young woman with polycystic ovarian syndrome.

    PubMed

    Mirra, Marco; Kola, Nertil; Mattiello, Giacomo; Morisco, Carmine; Spinelli, Letizia

    2016-12-16

    Polycystic ovarian syndrome (PCOS) affects 4% to 12% of women in reproductive age, representing a clinical condition that could predispose to cardiovascular diseases. We report a case of a 34-year-old woman with PCOS, presenting with chest pain, onset two days before, and ST segment-elevation myocardial infarction. She was not pregnant or in a postpartum state. Subsequent cardiac angiography revealed spontaneous left anterior descending coronary artery dissections, managed by conservative approach. The patient was discharged in medical therapy after 5days. This is the first observation of spontaneous coronary artery dissection occurring in a PCOS patient.

  1. Traumatic lumbar artery rupture after lumbar spinal fracture dislocation causing hypovolemic shock: An endovascular treatment.

    PubMed

    Eun, Jong-Pil; Oh, Young-Min

    2015-01-01

    Recently, we observed a case of lumbar artery injury after trauma, which was treated by endovascular embolization. A 67-year-old woman who was injured in a traffic accident was brought to the emergency room. She was conscious and her hemodynamic condition was stable, but she had paraplegia below L1 dermatome. Contrast-enhanced computed tomography scan of abdomen and pelvis revealed fracture dislocation of L3/4 along with retroperitoneal hematomas. However, there was no evidence of traumatic injury in both thoracic and abdominal cavity. At that time, her blood pressure suddenly decreased to 60/40 mmHg and her mental status deteriorated. Also, her hemoglobin level was 5.4 g/dl. While her hemodynamic condition stabilized with massive fluid resuscitation including blood transfusion, an angiography was immediately performed to look for and embolize site of retroperitoneal hemorrhage. On the angiographic images, there was an active extravasation from ruptured left 3rd lumbar artery, and we performed complete embolization with GELFOAM and coil. Lumbar artery injury after trauma is rare and endovascular treatment is useful in case of hemodynamic instability.

  2. Cost Utility Analysis of Percutaneous Adhesiolysis in Managing Pain of Post-lumbar Surgery Syndrome and Lumbar Central Spinal Stenosis.

    PubMed

    Manchikanti, Laxmaiah; Helm, Standiford; Pampati, Vidyasagar; Racz, Gabor B

    2015-06-01

    The increase in the number of interventions for the management of chronic pain and associated escalation of healthcare costs has captured the attention of health policymakers, in no small part due to the lack of documentation of efficacy, cost-effectiveness, or cost utility analysis. A recent cost utility analysis of caudal epidural injections in managing chronic low back pain of various pathologies showed a high cost utility with improvement in quality of life years, competitive with various other modalities of treatments. However, there are no analyses derived from high-quality controlled studies related to the cost utility of percutaneous adhesiolysis in the treatment of post-lumbar surgery syndrome or lumbar central spinal stenosis. This analysis is based on 2 previously published controlled studies. To assess the cost utility of percutaneous adhesiolysis procedures in managing chronic low back and lower extremity pain secondary to post-lumbar surgery syndrome and lumbar central spinal stenosis. A private, specialty referral interventional pain management center in the United States. Two controlled studies were conducted assessing the clinical effectiveness of percutaneous adhesiolysis for post-lumbar surgery syndrome and lumbar central spinal stenosis in an interventional pain management setting utilizing contemporary interventional pain management practices. A cost utility analysis was performed with direct payment data for a total of 130 patients in treatment groups over a 2-year period. Various outcome measures were included with significant improvement, defined as at least 50% improvement with reduction in pain and disability status. The results of 2 controlled studies of low back pain with 60 and 70 patients and a 2-year follow-up with the actual reimbursement data showed cost utility for 1 year of quality-adjusted life year (QALY) of USD $2,652 for post-lumbar surgery syndrome and USD $2,649 for lumbar central spinal stenosis. The results of this

  3. Reversible cerebral vasoconstriction syndrome and cervical artery dissection in 20 patients.

    PubMed

    Mawet, Jérôme; Boukobza, Monique; Franc, Julie; Sarov, Mariana; Arnold, Marcel; Bousser, Marie-Germaine; Ducros, Anne

    2013-08-27

    To describe clinical-radiologic characteristics in a prospective series of patients having both confirmed reversible cerebral vasoconstriction syndrome (RCVS) and cervical artery dissection (CeAD). From January 2004 to December 2011, from our prospective cohorts of RCVS and CeAD, we studied patients with both conditions. Of 173 RCVS cases and 285 CeAD cases, 20 patients (18 women, 2 men; mean age 41 years) had both RCVS and CeAD. Main associated conditions were migraine (12/20) and postpartum (5/18). Clinical features included severe headache in all patients, neck pain in 15, focal neurologic deficit in 9, and seizures in 4. Pain was the only symptom in 10 patients. All patients had multifocal cerebral vasoconstriction. There were brain lesions in 12 patients, cortical subarachnoid hemorrhage in 11, posterior reversible encephalopathy syndrome in 4, intracerebral hemorrhage in 3, and infarcts in 4. CeAD involved one artery in 13 patients and multiple arteries in 7. CeAD mostly affected vertebral arteries (25 of 30 CeAD). Only one vertebral CeAD was associated with a related symptomatic infarct. At 3 months, 18 patients had fully recovered, all patients showed reversal of cerebral vasoconstriction, and 21 dissected arteries had normalized, whereas 9 arteries showed residual stenosis (7) and/or aneurysm (3). The association of RCVS and CeAD was found in 12% of our patients with RCVS and 7% of our patients with CeAD. Underlying mechanisms are unknown. In practice, our results point to the need for a systematic study of both cervical and intracranial arteries in the 2 conditions.

  4. Klippel-Feil syndrome and levo-looped transposition of the great arteries.

    PubMed

    Martínez-Quintana, Efrén; Rodríguez-González, Fayna

    2015-03-01

    Klippel-Feil syndrome is a skeletal disorder characterised by low hairline and a short neck due to abnormal fusion of two or more cervical vertebrae. Although congenital heart and lung defects are infrequent, some abnormalities such as cor triatriatum, coarctation of the aorta, total anomalous pulmonary venous connection, or lung agenesis have been reported. The challenge of recognising Klippel-Feil syndrome lies in the fact that there is an association of this syndrome with other significant conditions such as skeletal, genitourinary, neurological, ear, and some cardiac defects. We report a Klippel-Feil syndrome type III 14-year-old patient with a levo-looped transposition of the great arteries. In addition, the patient had agenesis of the left upper-lung lobe.

  5. Charcot-Marie-Tooth syndrome and neurofibromatosis type 1 with multiple neurofibromas of the entire spinal nerve roots

    PubMed Central

    Onu, David O; Hunn, Andrew W; Peters-Willke, Jens

    2013-01-01

    The coexistence of polyneuropathy which has the definite clinical and electromyographical findings consistent with Charcot-Marie-Tooth (CMT) syndrome and neurofibromatosis type 1 (NF1) has infrequently been reported. We describe a patient with both CMT and NF1, who had multiple neurofibromas involving the entire spinal neural axis. In addition, he had multiple neurofibromas distributed within the ileopsoas and gluteus muscles and subcutaneous tissues. These lesions were detected readily by MRI and the patient underwent successful surgical resection of the largest tumours compressing bilateral C2 nerve roots. To our knowledge, this is the first reported case of CMT syndrome coexisting with NF1 in which multiple neurofibromas involved the entire spinal nerve roots. We discuss the diagnostic and therapeutic challenges, emphasising the role of MRI and electrophysiology in such cases and provide a literature review. PMID:23853192

  6. Charcot-Marie-Tooth syndrome and neurofibromatosis type 1 with multiple neurofibromas of the entire spinal nerve roots.

    PubMed

    Onu, David O; Hunn, Andrew W; Peters-Willke, Jens

    2013-07-13

    The coexistence of polyneuropathy which has the definite clinical and electromyographical findings consistent with Charcot-Marie-Tooth (CMT) syndrome and neurofibromatosis type 1 (NF1) has infrequently been reported. We describe a patient with both CMT and NF1, who had multiple neurofibromas involving the entire spinal neural axis. In addition, he had multiple neurofibromas distributed within the ileopsoas and gluteus muscles and subcutaneous tissues. These lesions were detected readily by MRI and the patient underwent successful surgical resection of the largest tumours compressing bilateral C2 nerve roots. To our knowledge, this is the first reported case of CMT syndrome coexisting with NF1 in which multiple neurofibromas involved the entire spinal nerve roots. We discuss the diagnostic and therapeutic challenges, emphasising the role of MRI and electrophysiology in such cases and provide a literature review.

  7. [Popliteal artery entrapment syndrome in 3 young athletes].

    PubMed

    Delgado Daza, R; Moga Donadeu, L L; Muncunill Gil, J; Mañosa Bonamich, J; Vidal Conde, V

    1993-01-01

    Three cases of entrapment of the popliteal arteries from three young patients are reported. In all cases, the symptomatology was erroneously attributed to several muscular injuries produced in practice of a sport: Basketball, Football and Roller Hockey, respectively. In all cases, symptoms were progressing for 6 months as a minimum. The roads followed by these patients until they were visited in a Vascular Surgery Department is described. Clinical picture, the results from the different examinations practiced and the applied treatments to each patients are described.

  8. Higher prevalence of restless legs syndrome/Willis-Ekbom disease in multiple sclerosis patients is related to spinal cord lesions.

    PubMed

    Minár, M; Petrleničová, D; Valkovič, P

    2017-02-01

    Multiple sclerosis (MS) is connected with higher prevalence of secondary restless legs syndrome/Willis-Ekbom disease (RLS/WED). Aim of this study was to determine risk factor for developing symptoms of RLS in MS patients. In cross-sectional study we examined 200 random MS patients. After obtaining informed consents, patients undervent a structured interview based on RLS and MS symptoms and characteristics, demographic, and health-related data. Than we collected results of brain/spinal cord magnetic resonance imaging (MRI). Blood samples were examined for blood count and biochemistry. From all 200 subjects, 26% were RLS-positives (95% CI: 20-32%). From positive patients, 44% had negative family history for RLS, and developed secondary RLS after onset of MS. Compared to RLS-negatives, the positives had significantly higher prevalence of spinal cord lesions (p=0.01). Presence of spinal pathology was connected with higher risk of RLS development (OR=3.846, 95%CI:1.304-11.346). There were no statistically significant differences in the levels of red blood cells, iron metabolism parameters, or levels of B or D vitamins. Risk of RLS/WED in MS increases with presence of lesions in spinal cord. The role of decreased dopamine delivery to lower spinal regions as the pathological background must be proved by more detailed research. Copyright © 2017 Elsevier B.V. All rights reserved.

  9. Systemic vascular resistance is increased and associated with accelerated arterial stiffening change in patients with chronic cervical spinal cord injury.

    PubMed

    Huang, S C; May-Kuen Wong, A; Lien, H Y; Fuk-Tan Tang, S; Fu, T C; Lin, Y; Wang, J S

    2013-02-01

    Despite of stiffening change of conduit arteries, how total peripheral resistance (TPR) is adapted to chronic spinal cord injury (SCI) remains unclear. To investigate how chronic cervical SCI influences hemodynamic characteristics Cross-sectional, case-control study. Rehabilitation department in the tertiary medical center. Twenty-one male patients with traumatic SCI resulting from cervical spine fracture were recruited. The injury occurred three to 289 months (46 months in average) previously. Twenty-one healthy male participants with matched age and body mass index were enrolled as control group. The subjects were asked to maintain supine rest (SR) and then head-up tilt (HUT) at 60 degree for five minutes, respectively. A novel noninvasive bio-reactance device was employed to measure cardiac hemodynamics, whereas heart rate variability was used to determine cardiac autonomic activity. Additionally, the digital volume pulse analysis was applied to calculate arterial stiffness index (SI) and arteriolar reflection index (RI). SCI patients revealed less stroke volume and cardiac output (CO), as well as, greater total peripheral resistance (TPR) and SI during SR than normal subjects did. Moreover, the positive correlation between TPR and SI was observed in SCI patients rather than normal subjects. In SCI patients, HUT (1) markedly decreased TPR while CO and cardio-acceleration responses remained intact and (2) decreased HF power value but failed to change LF/HF ratio. Furthermore, the degree of orthostatic hypotension was correlated with the TPRHUT/TPRSR ratio but not the COHUT/COSR ratio. Chronic cervical SCI leads to a progressively accelerated increase in vascular stiffness, which is associated with increase in systemic vascular resistance. Furthermore, the cervical SCI-related orthostatic hypotension lies in the impairment of vasoconstriction without cardiac dysfunction. Clinical Rehabilitation Impact. SI, rather than blood pressure, reflects not only

  10. [Arterial hypertension and sleep apnea hypopnea syndrome in primary care].

    PubMed

    Bayó Llibre, J; Riel Cabrera, R; Mellado Breña, E; Filomena Paci, J; Priego Artero, M; García Alfaro, F J; Grau Granero, J M; Vázquez González, D; López Solana, J; Fernández San Martín, M I

    2015-01-01

    Sleep apnea hypopnea syndrome (SAHS) is frequent in hypertensive patients and plays a role in a greater incidence of cardiovascular morbidity-mortality. This study aims to know the clinical profile of hypertensive patients with SAHS compared to hypertensive patients without SAHS to know which variables should be used to orient their screening from primary care. An observational, descriptive, retrospective study of cases (hypertensive patients with SAHS) and controls (hypertensive patients without) was performed in an urban health care center. Based on a computerized registry of the site, patients diagnosed of SAHS and hypertension over 30 years of age were selected. For each case, one control case of hypertensive patients without SAHS paired by age and gender was randomly obtained. A total of 64 cases and 64 controls were selected. Standing out in the bivariate analysis were greater BMI (34.3±12.8 vs. 28.6±3.6), predominance of obesity (70.3 vs. 35.9%), metabolic syndrome (77.3 vs. 42.2%), consumption of psychopharmaceuticals (19.7 vs. 7.8%) and anithypertensive drugs (26.5 vs. 14.0%), ischemic heart disease (20.3 vs. 9.4%) in the case group versus control group (P<.05 for all the variables). The multivariate analysis showed that only the presence of metabolic syndrome was related with the presence of SAHS in hypertensive patients (OR 4.65; 95% CI: 2.03-10.64; P<.001). Screening for SAHS should be performed in hypertensive patients seen in primary care if they have metabolic syndrome criteria. Copyright © 2014 SEHLELHA. Published by Elsevier Espana. All rights reserved.

  11. Delayed Diagnosis of Cauda Eqina Syndrome with Perineural Cyst after Combined Spinal-Epidural Anesthesia in Hemodialysis Patient

    PubMed Central

    Akeda, Koji; Tsujii, Masaya; Sudo, Akihiro

    2013-01-01

    Symptomatic Tarlov (perineural cysts) are uncommon. In the following hemodialysis case, cauda equina syndrome was not detected after combined spinal-epidural anesthesia untilthe patient reported a lack of sensation in the perianal area 14 days postoperatively. She had normal motor function of her extremities. A laminectomy and cyst irrigation was performed. After the operation, her sphincter disturbance subsided gradually and her symptoms had disappeared. PMID:24066221

  12. Spinal cord stimulation, conception, pregnancy, and labor: case study in a complex regional pain syndrome patient.

    PubMed

    Segal, R

    1999-01-01

    Introduction. Interventional modalities for pain treatment are reserved for patients failing multidisciplinary pain management, including psychological, physical, pharmacological, and anesthetic techniques. Objective. Medications for intractable pain may be unacceptable because the risk of teratogenic effects. The purpose of this study is to find out whether spinal cord stimulation may be safe during conception, pregnancy, and delivery. Materials and Methods. We report a 30-year old, female, neonatal nurse who developed left hand burning pain, swelling, coldness, and weakness following a mild brachial plexus injury in a motor vehicle accident. The patient responded well to a combination of Neurontin, Trazadone, Ultram, and Vicodin. A year later, the patient married and wanted to become pregnant but was afraid of possible teratogenic effects of the medications. Therefore, she requested an interventional modality for control of her symptoms. We recommended spinal cord stimulation (SCS) based on our excellent experience with this modality in the management of complex regional pain syndromes (CRPS). However, we did inform the patient that no data had been published regarding the safety of this modality in pregnancy and labor. Results. Cervical SCS resulted in excellent pain control and discontinuation of the medications. Thirteen months later, she delivered a healthy five pound baby girl. Mother and baby were discharged home in two days. The SCS was not turned off at any time during the labor and delivery. Conclusion. SCS was safe for implantation in our case study of a pregnant woman. This may constitute a new indication for SCS in patients otherwise successfully managed with non-interventional modalities for pain control.

  13. Metabolic risk factors and arterial stiffness in Indian children of parents with metabolic syndrome.

    PubMed

    Khadilkar, Anuradha V; Chiplonkar, Shashi A; Pandit, Deepa S; Kinare, Arun S; Khadilkar, Vaman V

    2012-02-01

    To investigate the possible association between metabolic syndrome (MS) and arterial stiffness in Indian children with parental MS status. A cross-sectional study was conducted in 140 overweight/obese and 60 normal-weight Indian children (mean age, 11.4 ± 2.8 years) along with one of their parents during 2008-2009. Data on weight, height, blood pressure, serum lipids, zinc, insulin, and glucose were collected. Intima media thickness (CIMT) and stiffness parameters were assessed in the right carotid artery. Physical activity and diet were assessed using structured questionnaires. Body composition was measured using dual-energy x-ray absorptiometry. A gradual increase in the percentage of MS children with an increasing number of MS components in parents was observed. Mean values for arterial stiffness, pulse wave velocity, and elastic modulus were significantly higher in MS children of MS parents than in MS children of normal parents (p < 0.05). A significant correlation was observed for lifestyle, metabolic, and arterial parameters among child-parent pairs (p < 0.05). Multiple logistic regression revealed that children's CIMT and arterial stiffness were significantly associated (p < 0.01) with their serum levels of triglycerides, high-density lipoprotein, and zinc, as well as with parental MS-CIMT. Parental MS status and lifestyle factors increase the risk of MS and arterial abnormalities in children.

  14. Artery of Percheron Infarction as an Unusual Cause of Korsakoff's Syndrome

    PubMed Central

    Zhou, Yongxing; Fox, Derrick; Anand, Abhishek; Elhaj, Amal; Kapoor, Arushi; Najibi, Faranak; Kim, Han; Weir, Roger; Jayam-Trouth, Annapurni

    2015-01-01

    The Korsakoff syndrome is defined as “an abnormal mental state in which memory and learning are affected out of all proportion to other cognitive functions in an otherwise alert and responsive patient.” Confabulation refers to false or erroneous memories arising, not deliberately, in the context of a neurological amnesia and is often thought of as pathognomonic of the Korsakoff syndrome. Although the exact pathophysiology is unknown, various studies have identified brain lesions in the thalami, mammillary bodies, and frontal cortex. We report a case of a 68-year-old male presenting with acute altered mental status on July 16, 2015. The neuropsychological dysfunctions included prominent Korsakoff's syndrome, which became apparent when the altered mental status resolved. Amnesia was accompanied by prominent confabulation, disorientation, and lack of insight into his own disability. Neuroradiological data indicated that the intralaminar and dorsomedial nuclei in bilateral thalami were infarcted by occlusion of the artery of Percheron. We believe that ours is one of few reported cases of Korsakoff syndrome in a patient with infarction involving the territory of the artery of Percheron. We conclude that bilateral thalamic lesions could cause Korsakoff's syndrome and the intralaminar and dorsomedial nuclei might be important structures in the pathogenesis of confabulation. PMID:26688763

  15. Congenital Horner Syndrome with Heterochromia Iridis Associated with Ipsilateral Internal Carotid Artery Hypoplasia

    PubMed Central

    Coulier, Julie; Rommel, Denis; Boschi, Antonella

    2015-01-01

    Background Horner syndrome (HS), also known as Claude-Bernard-Horner syndrome or oculosympathetic palsy, comprises ipsilateral ptosis, miosis, and facial anhidrosis. Case Report We report herein the case of a 67-year-old man who presented with congenital HS associated with ipsilateral hypoplasia of the internal carotid artery (ICA), as revealed by heterochromia iridis and confirmed by computed tomography (CT). Conclusions CT evaluation of the skull base is essential to establish this diagnosis and distinguish aplasia from agenesis/hypoplasia (by the absence or hypoplasia of the carotid canal) or from acquired ICA obstruction as demonstrated by angiographic CT. PMID:25749818

  16. Fibromuscular dysplasia of the vertebral artery presenting with lateral medullary syndrome: a case report.

    PubMed

    Chen, Yuan-Liang; Hsu, Yaw-Don; Lee, Jiunn-Tay

    2004-12-01

    We have recently encountered a rare case of fibromuscular dysplasia (FMD) of the vertebral artery (VA) presenting as lateral medullary syndrome. A 39-year-old male was admitted to our hospital due to vertigo, dysarthria and numbness of the left face and the right limbs. A magnetic resonance imaging (MRI) and magnetic resonance angiography (MRA) of brain revealed lateral medullary infarction in the territory of the left posterior inferior cerebellar artery (PICA). The angiography of the VA revealed tubular stenosis of the left extracranial VA and a focal vascular kinking as well as web in the right extracranial VA, confirming the diagnosis of FMD. We present this rare case to emphasize that FMD could be one of the risk factors causing lateral medullary syndrome in young people.

  17. Mean platelet volume in patients with metabolic syndrome and its relationship with coronary artery disease.

    PubMed

    Tavil, Yusuf; Sen, Nihat; Yazici, Hüseyin Uğur; Hizal, Fatma; Abaci, Adnan; Cengel, Atiye

    2007-01-01

    Mean platelet volume (MPV) is an indicator of platelet activation which is a central process in the pathophysiology of coronary heart disease. The metabolic syndrome (MS) is characterized as the clustering of closely associated and interdependent atherosclerotic risk factors. MS has also been shown to be strongly associated with poor outcome in patients with coronary artery disease (CAD). The present study was designed to investigate MPV values in patients MS and to interrogate the association with CAD. We measured MPV in 205 consecutive patients with metabolic syndrome (mean age, 53+/-7 years) and 140 control subjects without metabolic syndrome (mean age, 52+/-6 years). All patients were selected from individuals who underwent coronary angiography in our hospital with a suspicion of coronary artery disease. To evaluate the severity of coronary artery disease, the patients with MS were subdivided depending upon the coexistence of coronary artery disease: normal coronary arteries, having coronary stenotic lesions of <50%, and having coronary stenotic lesions of >50%. The MPV was significantly higher in patients with MS than in the control group (10.19+/-1.49 fl vs 8.21+/-1.02 fl, p<0.001). According to the CAD severity, there were no statistically significant differences in MPV among these subgroups. We have shown for the first time that patients with MS have higher MPV compared to control subjects with normal coronary angiograms and to be associated with CAD. Hence MPV can be used as a follow up marker in patients with MS in the point of CAD.

  18. Mechanical assessment of elastin integrity in fibrillin-1-deficient carotid arteries: implications for Marfan syndrome

    PubMed Central

    Ferruzzi, Jacopo; Collins, Melissa J.; Yeh, Alvin T.; Humphrey, Jay D.

    2011-01-01

    Aims Elastin is the primary component of elastic fibres in arteries, which contribute significantly to the structural integrity of the wall. Fibrillin-1 is a microfibrillar glycoprotein that appears to stabilize elastic fibres mechanically and thereby to delay a fatigue-induced loss of function due to long-term repetitive loading. Whereas prior studies have addressed some aspects of ageing-related changes in the overall mechanical properties of arteries in mouse models of Marfan syndrome, we sought to assess for the first time the load-carrying capability of the elastic fibres early in maturity, prior to the development of ageing-related effects, dilatation, or dissection. Methods and results We used elastase to degrade elastin in common carotid arteries excised, at 7–9 weeks of age, from a mouse model (mgR/mgR) of Marfan syndrome that expresses fibrillin-1 at 15–25% of normal levels. In vitro biaxial mechanical tests performed before and after exposure to elastase suggested that the elastic fibres exhibited a nearly normal load-bearing capability. Observations from nonlinear optical microscopy suggested further that competent elastic fibres not only contribute to load-bearing, they also increase the undulation of collagen fibres, which endows the normal arterial wall with a more compliant response to pressurization. Conclusion These findings support the hypothesis that it is an accelerated fatigue-induced damage to or protease-related degradation of initially competent elastic fibres that render arteries in Marfan syndrome increasingly susceptible to dilatation, dissection, and rupture. PMID:21730037

  19. Right pulmonary artery agenesis with patent ductus arteriosus and Eisenmenger syndrome: a rare case diagnosed during the postpartum period.

    PubMed

    Beker-Acay, Mehtap; Ozkececi, Gulay; Unlu, Ebru; Hocaoglu, Elif; Kacar, Emre; Onrat, Ersel

    2014-01-01

    Unilateral absence of a pulmonary artery a very rare congenital disorder. We here present a case of a 22-year-old female patient with agenesis of the right pulmonary artery accompanying patent ductus arteriosus and Eisenmenger syndrome, diagnosed by chest X-ray and multidetector computed tomography 5 days after giving birth.

  20. Popliteal artery thrombosis in a patient with Cogan syndrome: Treatment with thrombolysis and percutaneous transluminal angioplasty

    SciTech Connect

    Bastug, Demir E.; Dominic, Anthony; Ortiz, Orlando; DiBartolomeo, Anthony G.; Kotzan, Jeffrey M.; Abraham, F. Matthew

    1997-01-15

    A 31-year-old woman with Cogan syndrome (a rare form of systemic vasculitis) was evaluated for a cold, painful left foot with diminished pulses. Arteriography demonstrated thrombosis of the left popliteal artery with evidence of vasculitis. Thrombolytic therapy was begun with initial success but eventual rethrombosis. After reinitiating thrombolytic therapy combined with intraarterial vasodilator therapy, successful angioplasty was performed with sustained results, at 6-month follow-up.

  1. Hypereosinophilic syndrome accompanying gangrene of the toes with peripheral arterial occlusion--a case report.

    PubMed

    Funahashi, Satoru; Masaki, Ichiro; Furuyama, Tadashi

    2006-01-01

    The authors herein report the case of a teenage boy who presented with peripheral arterial occlusion of both upper and lower extremities associated with hypereosinophilia. During a 10-year follow-up, corticosteroid therapy was continued for the treatment of hypereosinophilia. The patient underwent bilateral lumbar sympathectomies because of severe ischemia of the bilateral lower extremities with gangrene of the toes. Based on the progress of his disease over the past 10 years, he was suspected to have idiopathic hypereosinophilic syndrome (HES) accompanied by peripheral arterial obstruction. Idiopathic HES is a disease characterized by unexpected hypereosinophilia, which may lead to organ damage. This is a very rare case of peripheral arterial occlusion associated with idiopathic HIS.

  2. Insulin Enhances Endothelial Function Throughout the Arterial Tree in Healthy But Not Metabolic Syndrome Subjects

    PubMed Central

    Jahn, Linda A.; Hartline, Lee; Rao, Nagashree; Logan, Brent; Kim, Justin J.; Aylor, Kevin; Gan, Li-Ming; Westergren, Helena U.

    2016-01-01

    Context: Insulin reportedly impairs endothelial function in conduit arteries but improves it in resistance and microvascular arterioles in healthy humans. No studies have assessed endothelial function at three arterial levels in healthy or metabolic syndrome (METSYN) subjects. Objective: The objective of the study was to compare endothelial responsiveness of conduit arteries, resistance, and microvascular arterioles to insulin in healthy and METSYN subjects. Design: We assessed conduit, resistance, and microvascular arterial function in the postabsorptive and postprandial states and during euglycemic hyperinsulinemia (insulin clamp). Setting: The study was conducted at a clinical research unit. Participants: Age-matched healthy and METSYN subjects participated in the study. Interventions: We used brachial flow-mediated dilation, forearm postischemic flow velocity, and contrast-enhanced ultrasound to assess the conduit artery, resistance arteriole, and microvascular arteriolar endothelial function, respectively. We also assessed the conduit artery stiffness (pulse wave velocity and augmentation index) and measured the plasma concentrations of 92 cardiovascular disease biomarkers at baseline and after the clamp. Results: Postabsorptive and postprandial endothelial function was similar in controls and METSYN in all tested vessels. METSYN subjects were metabolically insulin resistant (P < .005). In controls, but not METSYN subjects, during euglycemic hyperinsulinemia, endothelial function improved at each level of arterial vasculature (P < .05 or less for each). Conduit vessel stiffness (pulse wave velocity) was increased in the METSYN group. Twelve of 92 biomarkers differed at baseline (P < .001) and remained different at the end of the insulin clamp. Conclusions: We conclude that insulin enhances arterial endothelial function in health but not in METSYN, and this vascular insulin resistance may underlie its increased cardiovascular disease risk. PMID:26756115

  3. Lower limb conduit artery endothelial responses to acute upper limb exercise in spinal cord injured and able-bodied men

    PubMed Central

    Totosy de Zepetnek, Julia O; Au, Jason S; Ditor, David S; MacDonald, Maureen J

    2015-01-01

    Vascular improvements in the nonactive regions during exercise are likely primarily mediated by increased shear rate (SR). Individuals with spinal cord injury (SCI) experience sublesional vascular deconditioning and could potentially benefit from upper body exercise-induced increases in lower body SR. The present study utilized a single bout of incremental arm-crank exercise to generate exercise-induced SR changes in the superficial femoral artery in an effort to evaluate the acute postexercise impact on superficial femoral artery endothelial function via flow-mediated dilation (FMD), and determine regulatory factors in the nonactive legs of individuals with and without SCI. Eight individuals with SCI and eight age, sex, and waist-circumference-matched able-bodied (AB) controls participated. Nine minutes of incremental arm-crank exercise increased superficial femoral artery anterograde SR (P = 0.02 and P < 0.01), retrograde SR (P < 0.01 and P < 0.01), and oscillatory shear index (OSI) (P < 0.001 and P < 0.001) in both SCI and AB, respectively. However, these SR alterations resulted in acute postexercise increases in FMD in the AB group only (SCI 6.0 ± 1.2% to 6.3 ± 2.7%, P = 0.74; AB 7.5 ± 1.4% to 11.2 ± 1.4%, P = 0.03). While arm exercise has many cardiovascular benefits and results in changes in SR patterns in the nonactive legs, these changes are not sufficient to induce acute changes in FMD among individuals with SCI, and therefore are less likely to stimulate exercise training-associated improvements in nonactive limb endothelial function. Understanding the role of SR patterns on FMD brings us closer to designing effective strategies to combat impaired vascular function in both healthy and clinical populations. PMID:25847920

  4. Markers of calcium and phosphate metabolism and osteopenic syndrome in patients with coronary artery disease.

    PubMed

    Golovkin, Alexey S; Kokov, Alexander N; Masenko, Vladislava; Khryachkova, Oksana N; Malyuta, Еlena; Barbarash, Olga L

    2016-12-01

    The aim of the present study is to assess the level of specific markers of calcium and phosphate metabolism in the development of coronary atherosclerosis in patients with stable coronary artery disease, depending on the severity of osteopenic syndrome. METHODSː In the study 112 male patients aged from 49 to 73 years with verified coronary artery disease were included in the study. Calcium Score was measured using the Agatston Score. Besides, all of them were tested on the serum level of parathyroid hormone, calcitonin, osteocalcin, bone-specific alkaline phosphatase, osteoprotegerin, osteopontin, cathepsin K, estradiol and testosterone. RESULTSː The distribution of patients according to the severity of coronary atherosclerosis using the Syntax Score suggested that the levels of the studied markers did not differ significantly among the patients, despite significant differences in the severity of coronary artery disease. The levels of osteoprotegerin in patients with mild, moderate and severe calcification were significantly lower compared to patients with a zero calcium score. There were no clinical manifestations of osteopenic syndrome in all patients. However, they underwent osteodensitometry with measurement of bone mineral density at the lumbar spine and femoral neck to determine pre-clinical symptoms of bone destruction. Significant increase (P=0.03) in serum levels of osteocalcin was found in patients with radiological evidence of osteoporosis while the other markers did no differ significantly. CONCLUSIONSː We suppose that there is a reciprocal interaction of regulatory vectors with increased calcium deposition in the arterial wall and resorption of bone tissue.

  5. Cervical artery dissection expands the cardiovascular phenotype in FBN1-related Weill-Marchesani syndrome.

    PubMed

    Newell, Kelsey; Smith, Wendy; Ghoshhajra, Brian; Isselbacher, Eric; Lin, Angela; Lindsay, Mark E

    2017-09-01

    Weill-Marchesani syndrome (WMS) is a rare form of acromelic dysplasia that is characterized by distinctive skeletal, ocular, and cardiovascular abnormalities. Previously described cardiac manifestations of WMS include aortic and pulmonary valve stenosis, mitral valve prolapse, mitral stenosis, and QTc prolongation. Autosomal dominant forms of WMS result from heterozygous pathogenic variants in FBN1, a gene with a well characterized role in the pathogenesis of thoracic aortic aneurysm (TAA) in the context of Marfan syndrome. In contrast, only one patient has been reported with aortic disease in WMS. Although the risk of aortic dissection from preceding TAA remains the leading cause of morbidity for individuals with Marfan syndrome, rare reports of arterial dissection in the peripheral vasculature have been described. Peripheral artery dissection has not been previously reported in other FBN1-related diseases. We describe a three generation family with FBN1-related WMS whose cardiovascular manifestations include TAA and cervical artery dissection, thus expanding the cardiovascular phenotype of WMS. Further research is required to quantify these risks and establish appropriate recommendations for cardiovascular imaging, medical management, and prophylactic surgical intervention in individuals with FBN1--related acromelic dysplasia. © 2017 Wiley Periodicals, Inc.

  6. Lemierre's Syndrome Associated Mycotic Aneurysm of the External Carotid Artery with Primary Internal Carotid Artery Occlusion in a Previously Healthy 18-Year-Old Female.

    PubMed

    Chamseddin, Khalil H; Kirkwood, Melissa L

    2016-10-01

    Lemierre's syndrome is a rare life-threatening condition characterized by internal jugular vein thrombosis and is typically associated with a gram-negative infection with septic metastasis secondary to a retropharyngeal abscess that involves the vasculature of the head and neck. We report a case of Lemierre's syndrome in an 18-year-old female adolescent who developed an internal carotid artery occlusion and ipsilateral external carotid artery (ECA) mycotic aneurysm complicated by fulminant pseudomonal sepsis. The patient was managed with open ligation of the ECA with essentially complete recovery. Copyright © 2016 Elsevier Inc. All rights reserved.

  7. Repeated Transient Wallenberg's Syndrome: Probable Association with Ipsilateral Vertebral Artery Hypoplasia and Aortic Valve Disease.

    PubMed

    Liu, Feng-Di; Zhao, Rong; Wang, Shuo; Li, Wen-Ting; Tao, Xiao-Xiao; Sun, Ting; Shen, Xiao-Lei; Liu, Jian-Ren

    2015-08-01

    Here we report a rare case of repeated transient Wallenberg's syndrome and discuss its mechanism. Case report and literature review. A 57-year-old man was admitted for 1.5-month repeated transient Wallenberg's syndrome, including right-sided Horner's syndrome, lower limb weakness, and paresthesia on the right side of the body and face. His symptom appeared mostly during physical activity. Symptoms occurred nearly everyday and lasted from 5 minutes to 30 minutes. His cranial magnetic resonance imaging (MRI) including diffusion-weighted MRI imaging was normal, and his cervical contrast-enhanced magnetic resonance angiography reflected right vertebral artery hypoplasia. Twenty-four-hour electrocardiogram and electroencephalography showed no abnormalities. Echocardiography showed aortic valve calcification with moderate aortic stenosis, moderate aortic insufficiency, and dilated aorta. Dual-antiplatelets or warfarin (international normalized ratio reached 2.07) were not effective to reduce the attacks. Hemodynamic instability due to valve disease combined with right vertebral artery hypoplasia could lead to transient Wallenberg's syndrome. Antithrombotics are often ineffective for this kind of patients and the best therapy for them could be to cure their valve disease. Repeated transient Wallenberg's syndrome is rare and that caused by ipsilateral vertebral artery hypoplasia and severe valve disease has not been reported up till now to our knowledge, so it will widen the knowledge on etiologies of transient ischemic attacks and provide information and reference to cardiologists and neurologists in diagnosis and treatment for patients with similar clinical manifestations. Copyright © 2015 National Stroke Association. Published by Elsevier Inc. All rights reserved.

  8. Spinal cord stimulation in pain management: a review.

    PubMed

    Jeon, Young Hoon

    2012-07-01

    Spinal cord stimulation has become a widely used and efficient alternative for the management of refractory chronic pain that is unresponsive to conservative therapies. Technological improvements have been considerable and the current neuromodulation devices are both extremely sophisticated and reliable in obtaining good results for various clinical situations of chronic pain, such as failed back surgery syndrome, complex regional pain syndrome, ischemic and coronary artery disease. This technique is likely to possess a savings in costs compared with alternative therapy strategies despite its high initial cost. Spinal cord stimulation continues to be a valuable tool in the treatment of chronic disabling pain.

  9. Morning Glory Syndrome with Carotid and Middle Cerebral Artery Vasculopathy.

    PubMed

    Nezzar, Hachemi; Mbekeani, Joyce N; Dalens, Helen

    2015-12-01

    To report a case of incidental asymptomatic atypical morning glory syndrome (MGS) with concomitant ipsilateral carotid and middle cerebral dysgenesis. A 6-year-old child was discovered to have incidental findings of MGS, with atypia. All visual functions were normal including vision and stereopsis. Neuroimaging revealed ipsilateral carotid and middle cerebral vascular narrowing without associated collateral vessels or cerebral ischemia commonly seen in Moyamoya disease. Subsequent annual examinations have been stable, without signs of progression. This case demonstrates disparity between structural aberrations and final visual and neurological function and reinforces the association between MGS and intracranial vascular disruption. Full ancillary ophthalmic and neuroimaging studies should be performed in all patients with MGS with interval reassessments, even when the patient is asymptomatic and functionally intact.

  10. Increasing Rates of Imaging in Failed Back Surgery Syndrome Patients: Implications for Spinal Cord Stimulation.

    PubMed

    Farber, S Harrison; Han, Jing L; Petraglia Iii, Frank W; Gramer, Robert; Yang, Siyun; Pagadala, Promila; Parente, Beth; Xie, Jichun; Petrella, Jeffrey R; Lad, Shivanand P

    2017-09-01

    Failed back surgery syndrome (FBSS) has a high incidence following spinal surgery, is notoriously refractory to treatment, and results in high health care utilization. Spinal cord stimulation (SCS) is a well-accepted modality for pain relief in this population; however, until recently magnetic resonance imaging (MRI) was prohibited due to risk of heat conduction through the device. We examined trends in imaging use over the past decade in patients with FBSS to determine its impact on health care utilization and implications for patients receiving SCS. Retrospective. Inpatient and outpatient sample. We identified patients from 2000 to 2012 using the Truven MarketScan database. Annual imaging rates (episodes per 1000 patient months) were determined for MRI, computed tomography (CT) scan, x-ray, and ultrasound. A multivariate Poisson regression model was used to determine imaging trends over time, and to compare imaging in SCS and non-SCS populations. A total of 311,730 patients with FBSS were identified, of which 5.17% underwent SCS implantation (n = 16,118). The median (IQR) age was 58.0 (49.0 - 67.0) years. Significant increases in imaging rate ratios were found in all years for each of the modalities. Increases were seen in the use of CT scans (rate ratio [RR] = 3.03; 95% confidence interval [CI]: 2.79 - 3.29; P < 0.0001), MRI (RR = 1.73; 95% CI: 1.61 - 1.85; P < 0.0001), ultrasound (RR = 2.00; 95% CI: 1.84 - 2.18; P < 0.0001), and x-ray (RR = 1.10; 95% CI: 1.05 - 1.15; P < 0.0001). Despite rates of MRI in SCS patients being half that in the non-SCS group, these patients underwent 19% more imaging procedures overall (P < 0.0001). SCS patients had increased rates of x-ray (RR = 1.27; 95% CI: 1.25 - 1.29), CT scans (RR = 1.32; 95% CI: 1.30 - 1.35), and ultrasound (RR = 1.10; 95% CI: 1.07 - 1.13) (all P < 0.0001). This study is limited by a lack of clinical and historical variables including the complexity of prior surgeries and pain symptomatology. Miscoding cannot

  11. Anterior Tarsal Tunnel Syndrome With Thrombosed Dorsalis Pedis Artery: A Case Report

    PubMed Central

    Gani, Naseemul; Khan, Hayat Ahmad; Kamal, Younis; Farooq, Munir; Jeelani, Hina; Shah, Adil Bashir

    2015-01-01

    Introduction: The aanterior tarsal tunnel syndrome denotes the entrapment of the deep peroneal nerve under the inferior extensor retinaculum. Although various etiological factors have been reported to cause anterior tarsal syndrome, its occurrence with thrombosed dorsalis pedis artery has not been reported in the English literature. Case Presentation: A 40 -year-old male patient was presented with the history of persistent pain along the dorsal surface of right foot, which was aggravated with the activities. Conservative management was tried without much relief. Diagnosis of anterior tarsal tunnel syndrome was made and the patient was planned for surgery. Thrombosed dorsalis pedis artery was found along with two adjacent collateral vessels. Retinaculum was released and nerve was mobilized. Tight compartment got released. Postoperative period was uneventful. No recurrence was seen on follow-up. Conclusion: The anterior tarsal tunnel syndrome is a known disease. A high index of clinical suspicion is required while dealing with the chronic cases. A detailed history to rule out any traumatic event is necessary too. Timely investigations and surgical release give dramatic relief. PMID:26064866

  12. Patients' Expectations on Spinal Cord Stimulation for Failed Back Surgery Syndrome: A Qualitative Exploration.

    PubMed

    Henssen, Dylan J H A; Scheepers, Nicole; Kurt, Erkan; Arnts, Inge; Steegers, Monique; Vissers, Kris; van Dongen, Robert; Engels, Yvonne

    2017-07-25

    Spinal cord stimulation (SCS) is an effective therapy to reduce pain in patients that suffer from failed back surgery syndrome (FBSS). In order to inform patients optimally prior to this therapy, knowing their expectations is crucial. Thirteen patients suffering from FBSS and scheduled for SCS were interviewed, using a semi-structured protocol. Patients were interviewed either at home or at their treating hospital. Data from these interviews were analyzed using directed content analysis. In addition to the qualitative interviews, an adjusted Brief Pain Inventory (BPI-) questionnaire was used to quantify expectations. The expectations of patients with regard to SCS could be subdivided into 13 categories, which could be grouped into six general themes: 1) physical well-being, 2) social well-being, 3) material well-being, 4) emotional well-being, 5) development and activity and 6) constraints of the procedure of SCS. These findings confirm patients' expectations about the improvement of their quality of life by SCS for FBSS. This indicates that assessing pain relief is not enough to adequately evaluate the effects of SCS. The small diversity within the studied population and the lack of patient-to-patient education are two possible limitations of this study. To improve education for patients prior to SCS surgery and to evaluate the effects of SCS, a multidimensional approach needs to be implemented. Possible disadvantages of SCS need to be discussed prior to the treatment. This article is protected by copyright. All rights reserved. This article is protected by copyright. All rights reserved.

  13. Dopaminergic treatment of restless legs syndrome in spinal cord injury patients with neuropathic pain

    PubMed Central

    Kumru, Hatice; Albu, Sergiu; Vidal, Joan; Barrio, Manuela; Santamaria, Joan

    2016-01-01

    Recent studies report high incidence of restless legs syndrome (RLS) in patients with spinal cord injury (SCI), who may also present pain and sensory disturbances. In the present manuscript, we examine and discuss diagnostic and treatment challenges of comorbid RLS and neuropathic pain (NP) in SCI. We evaluated seven men with a mean age of 55.6 (s.d.=14.0) years, with chronic complete or incomplete SCI at the thoracic or lumbar level, for complaints of sensory disturbances in the legs, which initially were attributed to drug-resistant NP. Because overlapped RLS was suspected, clinical evaluation of NP and RLS, serum ferritin and iron level assessment, and video polysomnographic (VPSG) studies were conducted. Pramipexole (0.18 mg q.d.−1) was added to treat RLS, and a follow-up was performed at 2 months. We found that in six subjects the RLS was comorbid with NP and in one subject the symptoms of RLS were misdiagnosed as NP. VPSG revealed periodic limb movements (PLMs) in all patients, including PLMs of the legs, arms or both. Serum ferritin was <50 ng ml−1 in two patients. RLS improved significantly after 2 months with pramipexole. On the basis of current findings, we recommend physicians to be aware of the comorbidity between RLS and NP secondary to SCI to include suitable diagnostic procedures and effective treatments. PMID:28053765

  14. Dopaminergic treatment of restless legs syndrome in spinal cord injury patients with neuropathic pain.

    PubMed

    Kumru, Hatice; Albu, Sergiu; Vidal, Joan; Barrio, Manuela; Santamaria, Joan

    2016-01-01

    Recent studies report high incidence of restless legs syndrome (RLS) in patients with spinal cord injury (SCI), who may also present pain and sensory disturbances. In the present manuscript, we examine and discuss diagnostic and treatment challenges of comorbid RLS and neuropathic pain (NP) in SCI. We evaluated seven men with a mean age of 55.6 (s.d.=14.0) years, with chronic complete or incomplete SCI at the thoracic or lumbar level, for complaints of sensory disturbances in the legs, which initially were attributed to drug-resistant NP. Because overlapped RLS was suspected, clinical evaluation of NP and RLS, serum ferritin and iron level assessment, and video polysomnographic (VPSG) studies were conducted. Pramipexole (0.18 mg q.d.(-1)) was added to treat RLS, and a follow-up was performed at 2 months. We found that in six subjects the RLS was comorbid with NP and in one subject the symptoms of RLS were misdiagnosed as NP. VPSG revealed periodic limb movements (PLMs) in all patients, including PLMs of the legs, arms or both. Serum ferritin was <50 ng ml(-1) in two patients. RLS improved significantly after 2 months with pramipexole. On the basis of current findings, we recommend physicians to be aware of the comorbidity between RLS and NP secondary to SCI to include suitable diagnostic procedures and effective treatments.

  15. [Hypervascularization syndrome of the penis following arterialization of the dorsal penile vein for erectile impotence].

    PubMed

    Fritsch, P; Grubauer, G; Hilty, N; Biedermann, H

    1989-07-01

    A hypervascularization syndrome following arterialization of the deep dorsal vein of the penis to amend venous erectile impotence is a rare cause of penile ulcers that has not previously been described in the dermatological literature. Arterialization is performed by installing a shunt from the inferior epigastric artery or a venous bypass from the femoral artery to the deep dorsal vein of the penis, resulting in a blockage of venous outflow and in retrograde inflow into the corpora cavernosa. Complications arise as a result of persistently elevated blood pressure in the deep venous system and the erectile tissue in 10-20% of cases and are most often linked to dilatation of the shunt: the consequences are enlargement and induration of the glans, hazard of phimosis and paraphimosis, pulsation of the penis, micturation difficulties and, ultimately, ulceration of the glans. Surgical reduction of the arterial inflow ("banding" of the shunt) is the only therapeutic procedure that reduces hypervascularization without compromising the newly gained erectile function.

  16. Vertebral artery dissection presented as lateral medullary syndrome in a patient with migraine: a case report.

    PubMed

    Yen, Ju-Chun; Chan, Lung; Lai, Yen-Jun

    2010-12-01

    Migraine and artery dissection are both rare causes of ischemic stroke. The mechanism of migraine-related intracranial artery dissection is still unknown. It is proposed that the repeated attack of migraine would make the involved artery more vulnerable to tearing and lead to dissection. We describe a 42-year-old female suffering from basilar-type migraine for more than 20 years. The patient complained severe dizziness with hyperventilation while watching television. Initially anxiety and migraine attack were impressed in the emergency room, but dizziness accompanied with dysarthria and dysphagia was noted later. After admission, lateral medullary syndrome was suspected after a detailed neurological examination, and a brain magnetic resonance image (MRI) revealed an acute infarction on the left lateral medulla oblongata, confirming the clinical diagnosis Furthermore, cerebral angiography revealed the left distal vertebral artery dissection. The patient was reluctant to use an anticoagulant; therefore aspirin was given for secondary stroke prevention and topiramate for migraine prophylaxis. The exact mechanism of migraine-related intracranial artery dissection has yet to be proven, we propose that this may be caused by vessel wall edematous changes with repeated migraine attacks resulting in sudden or unusual stretching.

  17. Embolization of Life-Threatening Arterial Rupture in Patients with Vascular Ehlers–Danlos Syndrome

    SciTech Connect

    Okada, Takuya; Frank, Michael; Pellerin, Olivier Primio, Massimiliano Di Angelopoulos, Georgios; Boughenou, Marie-Fazia; Pagny, Jean-Yves; Messas, Emmanuel; Sapoval, Marc

    2013-05-09

    PurposeTo evaluate the safety and efficacy of transarterial embolization of life-threatening arterial rupture in patients with vascular Ehlers–Danlos syndrome (vEDS) in a single tertiary referral center.MethodsWe retrospectively analyzed transarterial embolization for vEDS performed at our institution from 2000 to 2012. The indication of embolization was spontaneous arterial rupture or pseudoaneurysm with acute bleeding. All interventions used a percutaneous approach through a 5F or less introducer sheath. Embolic agents were microcoils and glue in 3 procedures, glue alone in 2, and microcoils alone in 2.ResultsFive consecutive vEDS patients were treated by 7 embolization procedures (4 women, mean age 29.8 years). All procedures were successfully performed. Two patients required a second procedure for newly arterial lesions at a different site from the first procedure. Four of the five patients were still alive after a mean follow-up of 19.4 (range 1–74.7) months. One patient died of multiple organ failure 2 days after procedure. Minor procedural complications were observed in 3 procedures (43 %), all directly managed during the same session. Remote arterial lesions occurred after 3 procedures (43 %); one underwent a second embolization, and the other 2 were observed conservatively. Puncture site complication was observed in only one procedure (14 %).ConclusionEmbolization for vEDS is a safe and effective method to manage life-threatening arterial rupture.

  18. Guillain-Barré Syndrome after Coronary Artery Bypass Graft Surgery: a Case Report.

    PubMed

    Hekmat, Manouchehr; Ghaderi, Hamid; Foroughi, Mahnoosh; Mirjafari, S Adeleh

    2016-01-01

    Guillain-Barre syndrome is a neurologic disorder that may appear after infection or major surgery. Guillain-Barré syndrome following cardiac surgery is rare and only based on case reports, and we review all of the published cases. A 52-year-old man after 5 months suffering from chest pain was referred to our hospital and underwent coronary artery bypass graft for 3 vessel disease. The patient was discharged without complication on the 5th postoperative day. He presented Guillain-Barré syndrome after 12 months. He has not completely recovered weakness of upper extremities grade 4/5 with atrophy of both upper extremities remains after 18 months. This disorder is similar to classic GBS. It is important to be alert to de novo autoimmune neurological disorders after cardiac surgery. These disorders are similar to classic autoimmune disease and treated with standard therapies.

  19. The effects of resistance exercise training on arterial stiffness in metabolic syndrome.

    PubMed

    DeVallance, E; Fournier, S; Lemaster, K; Moore, C; Asano, S; Bonner, D; Donley, D; Olfert, I M; Chantler, P D

    2016-05-01

    Arterial stiffness is a strong independent risk factor for cardiovascular disease and is elevated in individuals with metabolic syndrome (MetS). Resistance training is a popular form of exercise that has beneficial effects on muscle mass, strength, balance and glucose control. However, it is unknown whether resistance exercise training (RT) can lower arterial stiffness in patients with MetS. Thus, the aim of this study was to examine whether a progressive RT program would improve arterial stiffness in MetS. A total of 57 subjects (28 healthy sedentary subjects; 29 MetS) were evaluated for arterial structure and function, including pulse wave velocity (cfPWV: arterial stiffness), before and after an 8-week period of RT or continuation of sedentary lifestyle. We found that 8 weeks of progressive RT increased skeletal muscle strength in both Con and MetS, but did not change arterial stiffness in either MetS (cfPWV; Pre 7.9 ± 0.4 m/s vs. Post 7.7 ± 0.4 m/s) or healthy controls (cfPWV; Pre 6.9 ± 0.3 m/s vs. Post 7.0 ± 0.3 m/s). However, when cfPWV is considered as a continuous variable, high baseline measures of cfPWV tended to show a decrease in cfPWV following RT. Eight weeks of progressive RT did not decrease the group mean values of arterial stiffness in individuals with MetS or healthy controls.

  20. Splenic Artery Syndrome After Orthotopic Liver Transplantation: Treatment With the Amplatzer Vascular Plug

    SciTech Connect

    Maurer, M. H.; Mogl, M. T.; Podrabsky, P.; Denecke, T.; Grieser, C.; Froeling, V.; Scheurig-Muenkler, C.; Guckelberger, O.; Kroencke, T. J.

    2011-12-15

    Purpose: To evaluate the safety and efficacy of the Amplatzer vascular plug (AVP) for embolization of the splenic artery in patients with hepatic hypoperfusion after orthotopic liver transplantation (OLT). Materials and Methods: Thirteen patients (9 men and 4 women) with a mean age of 56 years (range 22-70) who developed splenic artery syndrome after OLT with decreased liver perfusion and clinically relevant impairment of liver function (increased transaminase or serum bilirubin levels, thrombocytopenia, and/or therapy-refractory ascites) were treated by embolization of the proximal third of the splenic artery using the AVP. The plugs ranged in diameter from 6 to 16 mm, and they were introduced through femoral (n = 9), axillary (n = 3), or brachial (n = 1) access using a 5F or 8F guiding catheter. Results: The plugs were successfully placed, and complete occlusion of the splenic artery was achieved in all patients. Placement of two plugs was necessary for complete occlusion in 3 of the 13 patients. Occlusion took on average 10 min (range 4-35). There was no nontarget embolization or plug migration into more distal segments of the splenic artery. All patients showed improved arterial perfusion, including the liver periphery, on postinterventional angiogram. After embolization, liver function parameters (transaminase and bilirubin levels) improved with normalization of concomitant thrombocytopenia and a decrease in ascites volume. Conclusion: Our initial experience in a small patient population with SAS suggests that the AVP enables precise embolization of the proximal splenic artery, thus providing safe and effective treatment for poor liver perfusion after OLT due to SAS.

  1. Phenotypic variability and diffuse arterial lesions in a family with Loeys-Dietz syndrome type 4.

    PubMed

    Mazzella, J-M; Frank, M; Collignon, P; Langeois, M; Legrand, A; Jeunemaitre, X; Albuisson, J

    2017-03-01

    Syndromic thoracic aortic aneurysm and dissection (TAAD) can suggest Marfan, vascular Ehlers-Danlos or Loeys-Dietz (LDS) syndromes. Several of the TGFβ-pathway-related genes predispose to different types of LDS. Heterozygous loss-of-function variations in TGFβ2 have been shown to be responsible for a novel form of syndromic TAAD associated with an impairment of the mitral valve and cerebrovascular disease called Loeys-Dietz syndrome type 4 (LDS4). We report the clinical characterization of a LDS4 French family with sudden deaths and diffuse vascular lesions, caused by a frameshift mutation in TGFβ2 gene: c.[995del]; p.(Leu332TrpfsTer27). Clinical characteristics include aneurysm of aortic sinus, skeletal and cutaneous features compatible with a syndromic form of TAAD (joint hypermobility, scoliosis, and easy bruises), intracranial aneurysms and rare mitral valve involvement. Iliac aneurysms, systemic medium caliber arteries dissections, and mild developmental delay were present in the family, and have not been described in LDS4. Phenotypic variability was also an important finding, including absence of clinical vascular events at advanced age in one case. Our data expand the phenotype of LDS4: we confirm that TGFβ2 mutations are responsible for true LDS syndrome with non-specific features of connective tissue disorders and diffuse vascular lesions. Adapted vascular follow up and prevention has to be proposed for these patients.

  2. Arterial hypertension in Turner syndrome: a review of the literature and a practical approach for diagnosis and treatment.

    PubMed

    De Groote, Katya; Demulier, Laurent; De Backer, Julie; De Wolf, Daniel; De Schepper, Jean; Tʼsjoen, Guy; De Backer, Tine

    2015-07-01

    Turner syndrome is a rare chromosomal disorder with complete or partial absence of one X chromosome that only occurs in women. Clinical presentation is variable, but congenital and acquired cardiovascular diseases are frequently associated diseases that add significantly to the increased morbidity and mortality in Turner syndrome patients. Arterial hypertension is reported in 13-58% of adult Turner syndrome patients and confers an increased risk for stroke and aortic dissection. Hypertension can be present from childhood on and is reported in one-quarter of the paediatric Turner syndrome patients. This article reviews the prevalence and cause of arterial hypertension in Turner syndrome and describes the relationship between blood pressure, aortic dilation and increased cardiovascular risk. We compare current treatment strategies and also propose an integrated practical approach for the diagnosis and treatment of hypertension in Turner syndrome applicable in daily practice.

  3. Bilateral thalamic infarct caused by spontaneous vertebral artery dissection in pre-eclampsia with HELLP syndrome: a previously unreported association.

    PubMed

    Borelli, Paolo; Baldacci, Filippo; Vergallo, Andrea; Del Dotto, Paolo; Lucetti, Claudio; Nuti, Angelo; Bonuccelli, Ubaldo

    2012-11-01

    Cerebrovascular accidents are not rare during pregnancy and the postpartum period. Pre-eclampsia is a common condition that is characterized by proteinuria and de novo hypertension that may be complicated by hemolysis, elevated liver enzymes, and low platelet count (HELLP) syndrome. Spontaneous cervical artery dissection has been rarely reported in the postpartum period but never in association with HELLP syndrome. We describe a case of pre-eclampsia and HELLP syndrome complicated in the postpartum period by bilateral thalamic infarct as result of left vertebral artery dissection. We speculated about the possible common etiopathologic mechanisms involved in this previously unreported association.

  4. Arterial stiffness and influences of the metabolic syndrome: a cross-countries study.

    PubMed

    Scuteri, Angelo; Cunha, Pedro G; Rosei, E Agabiti; Badariere, Jolita; Bekaert, Sofie; Cockcroft, John R; Cotter, Jorge; Cucca, Francesco; De Buyzere, Marc L; De Meyer, Tim; Ferrucci, Luigi; Franco, Osca; Gale, Nichols; Gillebert, Thierry C; Hofman, A; Langlois, Michel; Laucevicius, Aleksandras; Laurent, Stephane; Mattace Raso, Francesco U S; Morrell, Cristopher H; Muiesan, Maria Lorenza; Munnery, Margaret M; Navickas, Rokas; Oliveira, Pedro; Orru', Marco; Pilia, Maria Grazia; Rietzschel, Ernst R; Ryliskyte, Ligita; Salvetti, Massimo; Schlessinger, David; Sousa, Nuno; Stefanadis, Christodoulos; Strait, James; Van daele, Caroline; Villa, Isabel; Vlachopoulos, Charalambos; Witteman, Jacqueline; Xaplanteris, Panagiotis; Nilsson, Peter; Lakatta, Edward G

    2014-04-01

    Specific clusters of metabolic syndrome (MetS) components impact differentially on arterial stiffness, indexed as pulse wave velocity (PWV). Of note, in several population-based studies participating in the MARE (Metabolic syndrome and Arteries REsearch) Consortium the occurrence of specific clusters of MetS differed markedly across Europe and the US. The aim of the present study was to investigate whether specific clusters of MetS are consistently associated with stiffer arteries in different populations. We studied 20,570 subjects from 9 cohorts representing 8 different European countries and the US participating in the MARE Consortium. MetS was defined in accordance with NCEP ATPIII criteria as the simultaneous alteration in ≥3 of the 5 components: abdominal obesity (W), high triglycerides (T), low HDL cholesterol (H), elevated blood pressure (B), and elevated fasting glucose (G). PWV measured in each cohort was "normalized" to account for different acquisition methods. MetS had an overall prevalence of 24.2% (4985 subjects). MetS accelerated the age-associated increase in PWV levels at any age, and similarly in men and women. MetS clusters TBW, GBW, and GTBW are consistently associated with significantly stiffer arteries to an extent similar or greater than observed in subjects with alteration in all the five MetS components--even after controlling for age, sex, smoking, cholesterol levels, and diabetes mellitus--in all the MARE cohorts. In conclusion, different component clusters of MetS showed varying associations with arterial stiffness (PWV). Copyright © 2014 Elsevier Ireland Ltd. All rights reserved.

  5. Hypertrophic remodeling of subcutaneous small resistance arteries in patients with Cushing's syndrome.

    PubMed

    Rizzoni, Damiano; Porteri, Enzo; De Ciuceis, Carolina; Rodella, Luigi F; Paiardi, Silvia; Rizzardi, Nicola; Platto, Caterina; Boari, Gianluca E M; Pilu, Annamaria; Tiberio, Guido A M; Giulini, Stefano M; Favero, Gaia; Rezzani, Rita; Rosei, Claudia Agabiti; Bulgari, Giuseppe; Avanzi, Daniele; Rosei, Enrico Agabiti

    2009-12-01

    Structural alterations of small resistance arteries in essential hypertensive patients (EH) are mostly characterized by inward eutrophic remodeling. However, we observed hypertrophic remodeling in patients with renovascular hypertension, in those with acromegaly, as well as in patients with non-insulin-dependent diabetes mellitus, suggesting a relevant effect of humoral growth factors on vascular structure, even independent from the hemodynamic load. Cortisol may stimulate the renin-angiotensin system and may induce cardiac hypertrophy. However, presently no data are available about small artery structure in patients with Cushing's syndrome. We have investigated the structure of sc small resistance arteries in 12 normotensive subjects (NT), in 12 EH subjects, and in eight patients with Cushing's syndrome (CS). Small arteries from sc fat were dissected and mounted on a micromyograph. The normalized internal diameter, media thickness, media to lumen ratio, and the media cross-sectional area were measured, as well as indices of oxidative stress. Demographic variables were similar in the three groups, except for clinic blood pressure. The media to lumen ratio was significantly greater in EH and CS, compared with NT; no difference was observed between EH and CS. The media cross-sectional area was significantly greater in CS compared with EH and with NT. An increased vascular oxidative stress was present in CS, as demonstrated by increased levels of superoxide anions, cyclooxygenase-1 and endothelial nitric oxide synthase in the microvessels. Our results suggest the presence of hypertrophic remodeling in sc small resistance arteries of CS, probably as a consequence of growth-promoting properties of circulating cortisol and/or increased vascular oxidative stress.

  6. Spinal cord stimulation for Raynaud's syndrome: long-term alleviation of bilateral pain with a single cervical lead.

    PubMed

    Wolter, Tilman; Kieselbach, Kristin

    2011-01-01

    Spinal cord stimulation (SCS) has been described in a variety of neuropathic and vasospastic pain conditions including Raynaud's syndrome.   We report here the outcome of single lead SCS in the case of a 49-year-old woman with severe Raynaud's syndrome, which had failed to respond to medical therapy.   With a single quadripolar cervical lead in midline position at the C2/C3 level sustained pain relief of the bilateral pain was accomplished. Pain scores sank from 7/10 to 2-3/10 on the nominal analog scale and remained stable more than nearly four years by now.   Treatment of bilateral pain in Raynaud's syndrome with SCS in a single technique is feasible. Advantages and disadvantages as compared with stimulation with bilateral leads are discussed. © 2011 International Neuromodulation Society.

  7. Arginine methylation dysfunction increased risk of acute coronary syndrome in coronary artery disease population

    PubMed Central

    Zhang, Shengyu; Zhang, Shuyang; Wang, Hongyun; Wu, Wei; Ye, Yicong

    2017-01-01

    Abstract The plasma levels of asymmetric dimethylarginine (ADMA) had been proved to be an independent cardiovascular risk factor. Few studies involved the entire arginine methylation dysfunction. This study was designed to investigate whether arginine methylation dysfunction is associated with acute coronary syndrome risk in coronary artery disease population. In total 298 patients undergoing coronary angiography because of chest pain with the diagnosis of stable angina pectoris or acute coronary syndrome from February 2013 to June 2014 were included. Plasma levels of free arginine, citrulline, ornithine, and the methylated form of arginine, ADMA, and symmetric dimethylarginine (SDMA) were measured with high-performance liquid chromatography coupled with tandem mass spectrometry. We examined the relationship between arginine metabolism-related amino acids or arginine methylation index (AMI, defined as ratio of [arginine + citrulline + ornithine]/[ADMA + SDMA]) and acute coronary events. We found that plasma ADMA levels were similar in the stable angina pectoris group and the acute coronary syndrome group (P = 0.88); the AMI differed significantly between 2 groups (P < 0.001). Multivariate logistic regression demonstrated that AMI was an independent risk factor of acute coronary events in patients with coronary artery disease (OR = 0.975, 95% confidence interval 0.956–0.993; P = 0.008). Our study suggested that ADMA levels were very similar in the stable angina and acute coronary syndrome patients; AMI might be an independent risk factor of acute coronary events in coronary artery disease population. PMID:28207514

  8. Thrombosis of the persistent median artery as a cause of carpal tunnel syndrome - case study.

    PubMed

    Rzepecka-Wejs, Ludomira; Multan, Aleksandra; Konarzewska, Aleksandra

    2012-12-01

    Carpal tunnel syndrome is the most frequent neuropathy of the upper extremity, that mainly occurs in manual workers and individuals, whose wrist is overloaded by performing repetitive precise tasks. In the past it was common among of typists, seamstresses and mechanics, but nowadays it is often caused by long hours of computer keyboard use. The patient usually complains of pain, hypersensitivity and paresthesia of his hand and fingers in the median nerve distribution. The symptoms often increase at night. In further course of the disease atrophy of thenar muscles is observed. In the past the diagnosis was usually confirmed in nerve conduction studies. Nowadays a magnetic resonance scan or an ultrasound scan can be used to differentiate the cause of the symptoms. The carpal tunnel syndrome is usually caused by compression of the median nerve passing under the flexor retinaculum due to the presence of structures reducing carpal tunnel area, such as an effusion in the flexor tendons sheaths (due to overload or in the course of rheumatoid diseases), bony anomalies, muscle and tendon variants, ganglion cysts or tumors. In some cases diseases of upper extremity vessels including abnormalities of the persistent median artery may also result in carpal tunnel syndrome. We present a case of symptomatic carpal tunnel syndrome caused by thrombosis of the persistent median artery which was diagnosed in ultrasound examination. The ultrasound scan enabled for differential diagnosis and resulted in an immediate referral to clinician, who recommended instant commencement on anticoagulant treatment. The follow-up observation revealed nearly complete remission of clinical symptoms and partial recanalization of the persistent median artery.

  9. Recurrent arterial aneurysm rupture of the upper extremity in a patient with vascular-type Ehlers-Danlos syndrome.

    PubMed

    Nakanishi, Koji; Tajiri, Nobuhisa; Nakai, Mikizo; Shimizu, Shuji

    2014-10-01

    Arterial aneurysm rupture is one of the most critical complications in patients with vascular-type Ehlers-Danlos syndrome (vEDS). Here, we report a case of recurrent aneurysm rupture successfully treated by endovascular embolization. A 38-year old woman who underwent brachial artery ligation for a ruptured aneurysm was diagnosed postoperatively with vEDS. Impending rupture of a collateral artery aneurysm was encountered 5 months after the initial open surgery. Endovascular embolization with a liquid embolic agent was successfully performed. Given that arterial rupture can occur repeatedly in patients with vEDS, careful life-long follow-up is necessary.

  10. [Spontaneous rupture of common iliac artery: a case of Ehlers-Danlos syndrome and review of the literature].

    PubMed

    Bronzino, P; Abbo, L; Bagnasco, F; Barisone, P; Dezzani, C; Genovese, A M; Iannucci, P; Ippoliti, M; Sacchi, M; Aimo, I

    2006-01-01

    Authors report an uncommon case of spontaneous rupture of common iliac artery in a man 43 years old with Ehlers-Danlos syndrome. The clinical presentation was devious in the beginning and catastrophic after few hours with a haemo-peritoneum and haemorragic shock. The Ehlers-Danlos syndrome is a rare affection of the connective tissue with an incidence of 1/5000, representing one of the most common disorders of the connective tissue. This disease is characterized by the fragility of arteries, intestine and uterus. Its presentation is often catastrophic, with rupture of a big artery, rupture of uterus during pregnancy or bowel perforation. The mean age of death in subjects with Ehlers-Danlos syndrome is 45 years. This syndrome is inherited in most cases in an autosomal dominant manner; 50% of the cases are due to new mutations. A minority of cases, due to deficit of tenascina X, is inherited in an autosomal recessive manner.

  11. Metabolic Syndrome and 30-Day Outcomes in Elective Lumbar Spinal Fusion.

    PubMed

    Chung, Andrew S; Campbell, David; Waldrop, Robert; Crandall, Dennis

    2017-08-29

    Retrospective cohort study OBJECTIVE.: To evaluate the effect of MetS on 30-day morbidity and mortality following elective lumbar spinal fusion SUMMARY OF BACKGROUND DATA.: Metabolic syndrome (MetS) is a variable combination of hypertension, obesity, elevated fasting plasma glucose, and dyslipidemia.MetS has been associated with an increased risk of post-operative morbidity and mortality in multiple surgical settings. To our knowledge, the effect of MetS on 30-day outcomes following elective lumbar spinal fusion has not been well studied. An analysis of ACS-NSQIP data was performed between 2006-2013. Patients undergoing elective posterior lumbar fusion were identified. Emergency procedures, infections, tumor cases, and revision surgeries were excluded. Patients were defined as having MetS if they had a history of hypertension requiring medication, diabetes, and a BMI ≥ 30. 1,590 (10.2%) patients with MetS were identified. A mild increase in major (p = 0.040) and minor complications (p = 0.003) in patients with MetS was noted. MetS was associated with increased rates of pulmonary complications (1.9% compared to 1.0%; p = 0.001), sepsis (1.7% compared to 0.9%; p = 0.005), and acute post-op renal failure (0.4% compared to 0%; p < 0.001). Multivariate analysis confirmed MetS to be an independent predictor of pulmonary complications (OR 1.51; 95% CI 1.00 - 2.27; p = 0.048), sepsis (OR 1.56; 95% CI 1.01 to 2.42; p = 0.039), and acute post-operative renal failure (OR 6.95; 95% CI 2.23 to 21.67; p = 0.001). MetS status was associated with a mild increase in total hospital length of stay (4.38 days compared to 3.81 days; p < 0.001). While MetS is a predictor of post-operative acute renal failure, it only slightly increases the risk of overall complications and is not associated with increased rates of 30-day re-operations or re-admissions following elective lumbar fusion. 3.

  12. Clinical effect of traditional Chinese spinal orthopedic manipulation in treatment of Functional Abdominal Pain Syndrome.

    PubMed

    Xing, Liyang; Qu, Liuxin; Chen, Hong; Gao, Song

    2017-06-01

    To evaluate the clinical effect of Traditional Chinese Spinal Orthopedic Manipulation (TCSOM) in treating Functional Abdominal Pain Syndrome (FAPS) in comparison with Pinaverium Bromide (Dicetel, PBD), and to assess a possible cause for FAPS. 60 cases of FAPS patients were randomly assigned to the TCSOM group and PBD group according to the random number table method. The TCSOM group was treated with thumb pressing manipulation, every other day in the first week, and once every three days in the second week, for 5 times treatments. Patients in the PBD group were instructed to take 50mg 3 times a day, consistently for 2 weeks. The symptoms of pre-treatment and post-treatment were assessed on a visual analog scale (VAS) pain score. A symptom improvement rating (SIR) was implemented in order to evaluate the effects of the treatments, and to statistically compare the two groups. The symptoms of 21 patients of the TCSOM group were resolved soon after the first spinal manipulation treatment and 4 cases were significantly improved. The VAS pain scores in the TCSOM group were significantly lower than those in the PBD group after 2 weeks treatment. According to the SIR based on VAS, the TCSOM research group included 20 cases with excellent results, 8 cases with good, and 2 cases with poor. There were no side effects in the TCSOM group after treatment. Based on VAS, the PBD research group reported 6 cases with excellent results, 8 cases with good and 16 cases with poor. All cases were statistically analyzed, revealing a significant difference (P<0.001) between the two groups. TCSOM group performed much better than PBD group for relief of the symptoms of FAPS. Thumb pressing manipulation on the thoracic and/or lumbar region can correct the displacement of inter-vertebral discs and/or vertebra, resolving the stimuli caused by pressure exerting on the nerves and vessels around the spine. with thumb pressing manipulation on the Back-Shu acupoints, the Jiaji (EX-B2) and the

  13. Spinal Cauda Equina Stimulation for Alternative Location of Spinal Cord Stimulation in Intractable Phantom Limb Pain Syndrome: A Case Report.

    PubMed

    Lee, Pil Moo; So, Yun; Park, Jung Min; Park, Chul Min; Kim, Hae Kyoung; Kim, Jae Hun

    2016-04-01

    Phantom limb pain is a phenomenon in which patients experience pain in a part of the body that no longer exists. In several treatment modalities, spinal cord stimulation (SCS) has been introduced for the management of intractable post-amputation pain. A 46-year-old male patient complained of severe ankle and foot pain, following above-the-knee amputation surgery on the right side amputation surgery three years earlier. Despite undergoing treatment with multiple modalities for pain management involving numerous oral and intravenous medications, nerve blocks, and pulsed radiofrequency (RF) treatment, the effect duration was temporary and the decreases in the patient's pain score were not acceptable. Even the use of SCS did not provide completely satisfactory pain management. However, the trial lead positioning in the cauda equina was able to stimulate the site of the severe pain, and the patient's pain score was dramatically decreased. We report a case of successful pain management with spinal cauda equina stimulation following the failure of SCS in the treatment of intractable phantom limb pain.

  14. Evaluation of clinical characteristics of Kawasaki syndrome and risk factors for coronary artery abnormalities among children in Denmark.

    PubMed

    Patel, Amy; Holman, Robert C; Callinan, Laura S; Sreenivasan, Nandini; Schonberger, Lawrence B; Fischer, Thea K; Belay, Ermias D

    2013-04-01

    To examine clinical characteristics, treatment and outcome of Kawasaki syndrome patients in Denmark. A retrospective chart review of hospitalization records for children <15 years of age with a Kawasaki syndrome discharge diagnosis identified through the Danish National Patient Registry during 1994 through June 2008 was conducted. A total of 284 cases <15 years of age were identified as Kawasaki syndrome (n = 279) and atypical Kawasaki syndrome (n = 5); 70.4% were <5 years of age and 64.4% were male. Most patients (91.5%; 258/282) were treated with intravenous immunoglobulin and 74.6% of these patients (191/256) received intravenous immunoglobulin before the 10th day of illness. A total of 37 (13.3%) Kawasaki syndrome patients were diagnosed with coronary artery abnormalities. Not receiving intravenous immunoglobulin treatment before the 10th day of illness, young age and male sex were significantly associated with the development of coronary artery abnormalities. In Denmark, more than one in 10 children with Kawasaki syndrome develop coronary artery abnormalities. Physicians should increase their index of suspicion for early diagnosis and treatment of Kawasaki syndrome among patients susceptible to increased risk of coronary artery abnormalities, particularly in infants who may have a more atypical presentation of the illness. ©2012 The Author(s)/Acta Paediatrica ©2013 Foundation Acta Paediatrica.

  15. The relationship between the metabolic syndrome and arterial wall thickness: A mosaic still to be interpreted.

    PubMed

    Scuteri, Angelo; Franco, Oscar H; Majiid, AlGhatrif; Jolita, Badariene; Sergey, Boytsov; Cheng, Hao-Min; Chen, Chen-Huan; Choi, Seong-Woo; Francesco, Cucca; De Buyzere, Marc L; Alessandro, Delitala; Marcus, Dörr; Gunnar, Engstrom; Albert, Hofman; Seul-Ki, Jeong; Kweon, Sun-Seog; Michel, Langlois; Lee, Young-Hoon; Mattace Raso, Francesco; Olle, Melander; Morrell, Cristopher H; Park, Kyeong-Soo; Rietzschel, Ernst R; Kristina, Ryliskiene; Ryliskyte, Ligita; Ulf, Schminke; David, Schlessinger; Shin, Min-Ho; Irina, Strazhesko; Shih-Hsien, Sung; Olga, Tkacheva; Völzke, Henry; Lakatta, Edward G; Nilsson, Peter

    2016-12-01

    We aimed to identify clusters of metabolic syndrome (MetS) components, risky for extremely high intima-media thickness. We studied 41,513 volunteers (men and women) from eleven cohorts worldwide, participating in the MARE (Metabolic syndrome and Artery REsearch) Consortium. Specific clusters of MetS components - high triglycerides-high blood pressure-abdominal obesity (TBW), low HDL cholesterol-high blood pressure-abdominal obesity (HBW), high glucose-high blood pressure-abdominal obesity (GBW) - were accompanied by a 50-90% significantly greater likelihood of presenting extremely high intima-media thickness (via ultrasound of carotid artery, CCA IMT), after controlling for age, sex, smoking, non-HDL cholesterol, and presence of diabetes mellitus. This likelihood is comparable to the effect of being 7-8 years older or of being a cigarette smoker or of having non-HDL cholesterol 50 mg/dl higher. The consistent association of specific clusters of MetS components with extremely thick (older) large artery cross-culturally suggests that identification of those clusters in clinical practice will facilitate a personalized health care and a better - i.e. more healthy and cost-effective - prevention of major cardiovascular (CV) events. Copyright © 2016 Elsevier Ireland Ltd. All rights reserved.

  16. Posterior reversible encephalopathy syndrome in an untreated hypertensive patient after spinal surgery under general anesthesia -A case report-

    PubMed Central

    Yi, Ji Hwan; Ha, Sang Hee; Kim, Yong Kook

    2011-01-01

    Posterior reversible encephalopathy syndrome (PRES) is an unfamiliar term to anesthesiologists, and this is characterized by neurologic symptoms that include mental change, headache, seizure and visual disturbance and also abnormal neuroimaging finding. A 71-year-old female patient was operated on for posterior decompression and total laminectomy under general anesthesia for the spinal stenosis. After the operation, she developed generalized tonic-clonic seizure and a stuporous mentality in the recovery room. The magnetic resonance imaging (MRI) revealed swelling and increased signal intensity at the deep gray nuclei, cerebral cortex and cerebellum. After one week, she returned to an alert mentality and then she was diagnosed with PRES. She was discharged without any neurologic deficit on postoperative day 20. This report describes our experience with PRES after spinal surgery was performed under general anesthesia on a suspected untreated hypertensive patient. PMID:21716568

  17. Molecular diagnosis in Vascular Ehlers-Danlos Syndrome Predicts Pattern of Arterial Involvement and Outcomes

    PubMed Central

    Shalhub, Sherene; Black, James H; Cecchi, Alana C.; Xu, Zhi; Griswold, Ben F; Safi, Hazim J; Milewicz, Dianna M.; McDonnell, Nazli B.

    2015-01-01

    OBJECTIVES The management of arterial pathology in individuals with vascular Ehlers-Danlos syndrome (vEDS) remains a challenge. Here we describe the correlation between COL3A1 gene mutation type and the clinical phenotype in individuals with vEDS. METHODS Individuals with confirmed molecular diagnoses of vEDS were enrolled in a multi institutional natural history study. Data collected included demographics, clinical and family histories, arterial pathology (aneurysm, dissection, and rupture), operative details, and autopsy reports. Individuals were classified into two cohorts based on the type of COL3A1 mutations and their effect on the amount of normal collagen produced: MIN group had mutations that lead to minimal (10–15%) production of normal type III collagen and HI group had haploinsufficiency mutations that lead to production of half the normal type III collagen. RESULTS A cohort of 68 (72%) individuals from 56 families had arterial pathology (44% male, 13% HI). The HI group was older at the time of their first vascular event (mean 42 years, range 26–58 vs. 33 years, range 8–62, P = .016). The HI group had a higher incidence of aortic pathology compared to the MIN group (56% vs. 21%. P = .025). Visceral arterial pathology was seen in 43 arteries in 23 individuals in the MIN group versus only a single artery in 5 individuals in the HI group. Emergent surgical procedures were more likely to be undertaken when vEDS diagnosis was not known (81% vs. 41%, P = .005) and the majority of these procedures were open surgical repair compared to endovascular repair (81% vs. 19%, P = .019). In the elective setting, there was equal use of open and endovascular repair. Post-operative complications were highest when the diagnosis of vEDS was not known (62% vs. 14%, P < .001) and when procedures were undertaken in an emergency setting (5% vs. 55% P < .001). There was no mortality due to arterial complications in the HI cohort and 21% in the MIN cohort (P = .132

  18. Increased carotid artery intima-media thickness may be associated with stroke in primary antiphospholipid syndrome

    PubMed Central

    Medina, G; Casaos, D; Jara, L; Vera-Lastra, O; Fuentes, M; Barile, L; Salas, M

    2003-01-01

    Objective: To investigate the prevalence and clinical significance of carotid artery intima-media thickness (IMT) in patients with primary antiphospholipid syndrome (APS). Methods: 28 patients with primary APS with at least a five year follow up, and 28 healthy subjects, matched by age and sex, were included in the study. Colour Doppler with high resolution B mode carotid ultrasonography and spectral analysis were performed in patients and controls. Information on cardiovascular risk factors and the clinical course were collected. Results: The mean (SD) age of patients and controls (12 male, 16 female in each group) was 40 (8.5) years; the mean (SD) disease duration 7.7 (3) years. Carotid artery IMT was found in 23/28 patients (2.6 (1.14) mm) and 7/28 controls (1.2 (0.44)) (p=0.0001). A decrease in the lumen diameter was also found in 11/28 patients with primary APS without carotid atherosclerotic plaque, and 2/28 controls (p=0.004). Hyperlipidaemia, diabetes, smoking, obesity, and hypertension were not associated with carotid artery IMT. Patients with carotid artery IMT had arterial vascular disease more often than patients without: 9/23 v 0/5 (p<0.009). These patients had stroke (seven patients), myocardial infarction (one), and mesenteric thrombosis (one). Subjects with IMT had a threefold higher risk for stroke than those without IMT (95% CI 0.78 to 14.3). Conclusions: Patients with primary APS have a high prevalence of carotid artery IMT and a decreased lumen diameter. IMT in primary APS may be associated with stroke. Patients with primary APS with IMT must be considered as carriers of atherosclerosis. PMID:12810420

  19. Arterial insulin resistance in Yucatan micropigs with diet-induced obesity and metabolic syndrome

    PubMed Central

    Low Wang, Cecilia C.; Lu, Li; Leitner, J. Wayne; Sarraf, Mohammad; Gianani, Roberto; Draznin, Boris; Greyson, Clifford R.; Reusch, Jane E. B.; Schwartz, Gregory G.

    2013-01-01

    Aim Metabolic syndrome affects a large proportion of the population and increases cardiovascular disease risk. Because metabolic syndrome often co-exists clinically with atherosclerosis, it is difficult to distinguish their respective contributions to vascular abnormalities. Accordingly, we utilized a porcine dietary model of metabolic syndrome without atherosclerosis to investigate early abnormalities of vascular function and signaling. Methods Thirty-two Yucatan micropigs were fed either a high fat, high simple sugar, high calorie (HFHS) or standard chow diet (STD) for 6 months. Neither diet contained added cholesterol. Blood pressure and flow-mediated vasodilatation were assessed at baseline and 6 months. Aortas were harvested at 6 months to assess histology, insulin signaling, and endothelial nitric oxide (eNOS) phosphorylation. Results HFHS pigs developed characteristic of metabolic syndrome including obesity, dyslipidemia, and insulin resistance, but without histological evidence of atherosclerosis. Although arterial intima-media thickness did not differ between groups, vascular dysfunction in HFHS was manifest by increased blood pressure and impaired flow-mediated vasodilation of the femoral artery. Compared with STD, aortas from HFHS exhibited increased p85α expression and Ser307 IRS-1 phosphorylation, and blunted insulin-stimulated IRS-1-associated phosphatidylinositol (PI) 3-kinase activity. In the absence of insulin stimulation, aortic Akt Ser473-phosphorylation was greater in HFHS than in STD. With insulin stimulation, Akt phosphorylation increased in STD, but not HFHS. Insulin-induced Ser1177-phosphorylation of eNOS was decreased in HFHS, compared with STD. Conclusions Pigs with metabolic syndrome develop early vascular dysfunction and aortic insulin signaling abnormalities, and could be a useful model for early human vascular abnormalities in this condition. PMID:23558108

  20. Cervical spinal cord dimensions and clinical outcomes in adults with klippel-feil syndrome: a comparison with matched controls.

    PubMed

    Cho, Woojin; Lee, Dong-Ho; Auerbach, Joshua D; Sehn, Jennifer K; Nabb, Colin E; Riew, K Daniel

    2014-12-01

    Study Design Retrospective case-control study. Objectives To confirm the fact that spinal cord dimensions are smaller in adults with Klippel-Feil syndrome (KFS) than in pediatric patients with KFS and to compare the clinical characteristics and outcomes of neurologic complications in patients with KFS with matched controls. Methods We performed an independent 1:2 case-control retrospective radiographic and chart review of a consecutive series of adults with KFS who underwent surgical intervention. The control group consisted of consecutive non-KFS surgical patients. Patients were matched in 1:2 case-control manner. Their charts were reviewed and the clinical characteristics were compared. Axial T2-weighted magnetic resonance imaging (MRI) was used to measure the anteroposterior and mediolateral axial spinal cord and spinal canal at the operative levels and measurements were compared. Results A total of 22 patients with KFS and 44 controls were identified. The KFS group had a tendency of more myeloradiculopathy, and the control group had a tendency toward more radiculopathy. Both tendencies, however, were not significantly different. MRIs of 10 patients from the KFS group and 22 controls were available. There was no difference in the area of both spinal cord and canal at the operative levels. Conclusion Contrary to the finding in previous reports on pediatric patients, there were no differences between KFS and well-matched control groups in terms of age of onset, presentation, revision rate, complication rate, surgical outcome, and cross-sectional spinal cord and canal dimensions at the operative level.

  1. Venous compression syndrome of internal jugular veins prevalence in patients with multiple sclerosis and chronic cerebro-spinal venous insufficiency.

    PubMed

    Mandolesi, Sandro; Niglio, Tarcisio; Orsini, Augusto; De Sio, Simone; d'Alessandro, Alessandro; Mandolesi, Dimitri; Fedele, Francesco; d'Alessandro, Aldo

    2016-01-01

    Analysis of the incidence of Venous Compression Syndrome (VCS) with full block of the flow of the internal jugular veins (IJVs) in patients with Multiple Sclerosis and Chronic cerebro-spinal venous insufficiency. We included 769 patients with MS and CCSVI (299 males, 470 females) and 210 controls without ms and ccsvi (92 males, 118 females). each subject was investigated by echo-color-doppler (ecd). morphological and hemodynamic ecd data were recorded by a computerized mem-net maps of epidemiological national observatory on ccsvi and they were analyzed by mem-net clinical analysis programs. VCS of IJVs occurs in 240 subjects affected by CCSVI and MS (31% of total) and in 12 controls (6% of total). The differences between the two groups are statistical significant (X² = 36.64, p<0.0001). Up to day there are no longitudinal studies that allow us to identify the WC of jugular and/or vertebral veins as etiology of a chronic neurodegenerative disease, but we note that Venous Compression Syndrome of IJVs is strongly associated with MS and CCSVI. Chronic Cerebro-Spinal Venous Insufficiency, Multiple Sclerosis, Venous Compression Syndrome.

  2. Is arterial stiffness predicted by continuous metabolic syndrome score in obese children?

    PubMed

    Prochotska, Katarina; Kovacs, Laszlo; Vitariusova, Eva; Feber, Janusz

    2016-01-01

    The aim of the article was to evaluate arterial stiffness, an early marker of increased cardiovascular risk, in relation to obesity. The continuous metabolic syndrome (cMetS) score was calculated as sum of Z score of mean arterial pressure, body mass index, serum glucose, triglyceride, and high-density lipoprotein cholesterol in 144 obese patients and 66 nonobese controls. Ambulatory arterial stiffness index (AASI) was calculated as 1 minus regression slope of diastolic on systolic blood pressure from ambulatory blood pressure measurements. The mean AASI increased progressively with severity of obesity. The receiver operator curve analysis of body mass index and AASI showed area under the curve of 0.64 ± 0.06; cMetS area under the curve was 0.72 ± 0.05 suggesting a better predictive power of the cMetS for an increased AASI (>0.3). Patients with obesity have significantly higher arterial stiffness. A composite score such as cMetS seems to be better predictor of an increased stiffness than individual risk factors.

  3. Dynamic Computed Tomography Angiography: Role in the Evaluation of Popliteal Artery Entrapment Syndrome

    SciTech Connect

    Anil, Gopinathan Tay, Kiang-Hiong; Howe, Tse-Chiang; Tan, Bien-Soo

    2011-04-15

    This study reviews our experience with dynamic computed tomographic angiography (CTA) as an imaging modality in the evaluation of popliteal artery entrapment syndrome (PAES). Eight patients with surgically proven PAES were included in this study. Dynamic CTA studies performed with the feet in neutral and plantar flexed positions were reviewed for the detailed anatomy of the region and to define the location and extent of the stenosis, occlusions and collateral circulation. These findings were compared with intraoperative observations. CTA provided adequate angiographic and anatomic information required to arrive at the diagnosis and make a surgical decision. Thirteen limbs were affected in eight patients. There was popliteal artery occlusion in four limbs, stenosis at rest that was accentuated on stress imaging in two limbs, and patent popliteal artery with marked stenosis on stress imaging in seven limbs. Long-segment stenosis was seen in functional entrapment compared to short-segment stenosis in anatomic PAES. Anteroposterior compression of the popliteal artery in anatomic PAES unlike the side-to-side compression in functional PAES was a unique observation in this study. The CTA and surgical characterisation and classification of PAES matched in all the patients, except for misinterpretation of compressing fibrous bands as accessory slips of muscles in three limbs. In conclusion, dynamic CTA is a robust diagnostic tool that provides clinically relevant information and serves as a rapidly performed and easily available 'one-stop-shop' imaging modality in the management of PAES.

  4. [Early diagnosis importance for a correct surgical treatment of PAES (popliteal artery entrapment syndrome)].

    PubMed

    Catani, M; De Milito, R; Clementi, I; Romagnoli, F; Simonelli, L; Vardas, P N; Manili, G; Capitano, S

    2004-01-01

    Popliteal artery entrapment syndrome (PAES) is an uncommon pathological entity, caused by segmental popliteal artery compression by the surrounding myofascial structures. Clinical symptoms may appear acutely, with temporary ischaemic attacks, or chronically, with concerned calf claudicatio intermittens and for 30% are bilateral. Diagnosis, besides being based on clinical objectivity (acute and deep pain to the struck limb, mainly during active plantar hyperextension) and history-taking (subject-age and lack of atherosclerosis), is based on ultrasonographic (eco-color Doppler of the aortic-iliac-femural-popliteal trunks, tensiometric Doppler), angio-RM, angio-CT scan and dynamic angiographic exams. Treatment, essentially, is surgical by simple freeing of the popliteal artery from surrounding myofascial structures or by autologous vein (saphenous v.) interposition grafting and patching, or bypass without vessel resection. About clinical case reported by the authors, 44-years female with left calf acute pain symptoms, cold skin by the thermo-touch, hypo-paraesthesia with fifth toe cyanosis and walking inability, surgical treatment, because of precox diagnosis, consisted of simple cut of myofibrous shoot starting from medial head of the left gastrocnemious muscle and compressing popliteal artery, with clinical chart complete resolution.

  5. Spontaneous Dissection of the Renal Artery in Vascular Ehlers-Danlos Syndrome

    PubMed Central

    Pereira, Filipa; Cardoso, Teresa; Sá, Paula

    2015-01-01

    Ehlers-Danlos syndrome (EDS) is a rare heterogeneous group of connective tissue disorders. The vascular type (vEDS) is an autosomal dominant disorder caused by heterozygous mutations in the COL3A1 gene predisposing to premature arterial, intestinal, or uterine rupture. We report a case of a 38-year-old woman with a recent diagnosis of vEDS admitted in the Emergency Department with a suspicion of a pyelonephritis that evolved to a cardiopulmonary arrest. A fatal retroperitoneal hematoma related with a haemorrhagic dissection of the right renal artery was found after emergency surgery. This case highlights the need to be aware of the particular characteristics of vEDS, such as a severe vascular complication that can lead to a fatal outcome. PMID:26175915

  6. Capsular warning syndrome and crescendo lacunar strokes after atherosclerotic stenosis of the recurrent artery of Heubner.

    PubMed

    Cohen, José E; Rabinstein, Alejandro; Gomori, John M; Leker, Ronen R

    2012-12-01

    The stereotype of repetitive transient cerebral ischemia causing unilateral motor, sensory, or sensorimotor deficits that simultaneously affect the face, arm, and leg, clinically localized to the internal capsule, fits with the description of capsular warning syndrome (CWS). A high proportion of individuals with these symptoms develop subsequent capsular stroke, despite various proposed preventative measures. It has been postulated that the mechanism for such strokes is that of small-vessel single-penetrator disease. We present a patient with repetitive CWS intermingled with crescendo capsular strokes secondary to recurrent artery of Heubner disease. This report causally links CWS-crescendo lacunar strokes and Heubner artery atherosclerotic disease (intracranial branch atheromatous disease). Copyright © 2012 Elsevier Ltd. All rights reserved.

  7. Horner's syndrome: a complication of experimental carotid artery surgery in rats.

    PubMed

    Aalbers, M W; Rijkers, K; van Winden, L A A P; Hoogland, G; Vles, J S H; Majoie, H J M

    2009-05-11

    To report on the occurrence of iatrogenic Horner's syndrome (HS) in epileptic rats after implantation of an electrode for vagus nerve stimulation and to describe the possible consequences of this new complication of carotid artery surgery in rats. A bipolar circular electrode was placed around the left carotid artery and vagus nerve of 31 rats. The incidence of HS was evaluated by visual inspection within 24 h after surgery. 68% of rats suffered from HS immediately after surgery. This complication did not affect epileptogenesis. The occurrence of HS in the rat is a frequent complication of vagus nerve electrode implantation, which does not affect epileptogenesis in this study. However, rats affected by HS may suffer from damage to the sympathetic innervation of the gut, due to rat-specific neuroanatomy. Therefore, caution towards other research questions is warranted.

  8. Transient total mesencephalic locked-in syndrome after bilateral ptosis due to basilar artery thrombosis.

    PubMed

    Fujimoto, Yasunori; Ohnishi, Yu-ichiro; Wakayama, Akatsuki; Yoshimine, Toshiki

    2012-11-01

    Locked-in syndrome (LIS) usually occurs as a result of pontine lesions and has been classified into various categories on the basis of neurologic conditions, of which transient total mesencephalic LIS is extremely rare. A 53-year-old man presented with bilateral ptosis followed by a total locked-in state. In the clinical course, the patient successfully recovered with only left slight hemiparesis and skew deviation remaining. Magnetic resonance imaging revealed multiple ischemic lesions caused by thrombosis at the top of basilar artery, including the bilateral cerebral peduncles, tegmentum of the midbrain, and the right cerebellar hemisphere. Antecedent bilateral ptosis before the locked-in state may be related to ischemia in the central caudal nucleus of the oculomotor nuclei. We should pay attention to this easily missed condition during the treatment of ischemic stroke involving the basilar artery.

  9. Call-fleming syndrome (reversible cerebral artery vasoconstriction) and aneurysm associated with multiple recreational drug use.

    PubMed

    Drazin, Doniel; Alexander, Michael J

    2013-01-01

    Drug abuse represents a significant health issue. Evidence suggests that recreational drug use has a direct effect on the cerebral vasculature and is of greater concern in those with undiagnosed aneurysms or vascular malformations. The authors report a case of thunderclap headache with a negative head CT and equivocal lumbar puncture after a drug-fueled weekend. The patient underwent diagnostic cerebral angiogram which demonstrated multisegmental, distal areas of focal narrowing of the middle, anterior, posterior, and posterior inferior cerebral artery and an incidental aneurysm. It is often difficult to determine the exact origin of symptoms; thus we were left with a bit of a chicken or the egg debate, trying to decipher which part came first. Either the aneurysm ruptured with associated concomitant vasospasm or it is a case of Call-Fleming syndrome (reversible cerebral artery vasoconstriction) with an incidental aneurysm. The authors proposed their management and rationale of this complex case.

  10. A reversible bilateral renal artery stenosis in association with antiphospholipid syndrome.

    PubMed

    Remondino, G I; Mysler, E; Pissano, M N; Furattini, M C; Basta, M C; Presas, J L; Allievi, A

    2000-01-01

    We describe a 26-year-old white female with a history of Raynaud phenomenon, erythema nodosum, polyarthralgias, migraine, vertigo, seizures, transient ischemic attacks, one fetal loss, and false positive VDRL, who developed milk hypertension without overt lupus nephritis. She had positive antinuclear antibodies (ANA) and double-stranded deoxyribonucleic acid (dsDNA) antibodies. The lupus anticoagulant test (LAC) and cardiolipins antibodies (aCL) were positive. She was diagnosed as having a Systemic Lupus Erythematosus-like illness (SLE-like) with 'secondary' antiphospholipid syndrome (APS). Renal spiral computed tomography (CT) with intravenous (IV) contrast showed bilateral renal artery stenosis. Anticoagulation with acenocumarol was started. She became normotensive without antihypertensive drugs five months later. A follow-up renal spiral CT showed complete recanalization of both renal arteries, making thrombosis the more likely culprit pathology in the stenosis. After two years follow up the patient is normotensive. She remains on acenocumarol.

  11. Spinal cord stimulation in adolescents with complex regional pain syndrome type I (CRPS-I).

    PubMed

    Olsson, Gunnar L; Meyerson, Björn A; Linderoth, Bengt

    2008-01-01

    Complex regional pain syndrome type I (CRPS-I) is not uncommon in children, particularly in adolescent girls. Most often, the condition involves a foot and is characterized by spontaneous pain, tactile allodynia and dysautonomic signs. There is usually a history of a minor, local trauma but sometimes no reasonable cause can be identified, and there are no signs of persistent tissue injury giving rise to ongoing nociception. Common analgesics are generally of no benefit, and the standard treatment includes sociopsychological support, physiotherapy, tricyclic antidepressants and antiepileptic drugs, sympathetic blocks (SB), and cognitive-behavioural therapy. For a minority of patients who prove to be resistant to such therapies, spinal cord stimulation (SCS) may be tried. The present study comprises seven girls, 11-14 years of age, presenting with severe, incapacitating and therapy-resistant CRPS-I, who were subjected to SCS. In two of them, percutaneous electrode implantation had to be performed in general anaesthesia. Trial stimulation was performed in all, but one. In two cases, it was not possible to produce paraesthesias that entirely covered the pain area. A pain relieving effect of SCS was usually not reported until after 1-2 weeks of trial stimulation. After another 2-6 weeks, pain alleviation was complete in five of the seven patients, one to eight years after the intervention. In one case, a local infection necessitated the removal of the electrode; nevertheless a few days of trial stimulation produced substantial pain relief that still persists. In four patients, the SCS use was gradually diminished and eventually the device could be removed. The favourable outcome in all seven cases with no or minor remaining symptoms and without severe recurrences illustrates that SCS may also be an efficient treatment in paediatric cases with exceptionally therapy resistant forms of CRPS I.

  12. Guyon's canal syndrome due to tortuous ulnar artery with DeQuervain stenosing tenosynovitis, ligamentous injuries and dorsal intercalated segmental instability syndrome, a rare presentation: a case report

    PubMed Central

    2009-01-01

    The Guyon's canal syndrome is a well known clinical entity and may have significant impact on patient's quality of life. We report a case of 43-year-old male who presented with complaints of pain and numbness in right hand and difficulty in writing for past one month. On imaging diagnosis of Guyon's canal syndrome because of tortuous ulnar artery was made with additional findings of DeQuervain's stenosing tenosynovitis and dorsal intercalated segmental instability syndrome with ligamentous injury and subsequently these were confirmed on surgery. Although it is a rare syndrome, early diagnosis and treatment prevents permanent neurological deficits and improve patient's quality of life. PMID:20076781

  13. Insulin resistance in penile arteries from a rat model of metabolic syndrome

    PubMed Central

    Contreras, Cristina; Sánchez, Ana; Martínez, Pilar; Raposo, Rafaela; Climent, Belén; García-Sacristán, Albino; Benedito, Sara; Prieto, Dolores

    2010-01-01

    BACKGROUND AND PURPOSE Metabolic and cardiovascular abnormalities accompanying metabolic syndrome, such as obesity, insulin resistance and hypertension, are all associated with endothelial dysfunction and are independent risk factors for erectile dysfunction. The purpose of the present study was to investigate the vascular effects of insulin in penile arteries and whether these effects are impaired in a rat model of insulin resistance and metabolic syndrome. EXPERIMENTAL APPROACH Penile arteries from obese Zucker rats (OZR) and their counterpart, lean Zucker rats (LZR), were mounted on microvascular myographs and the effects of insulin were assessed in the absence and presence of endothelium and of specific inhibitors of nitric oxide (NO) synthesis, phosphatidylinositol 3-kinase (PI3K) and mitogen-activated protein kinase (MAPK). Insulin-induced changes in intracellular Ca2+ concentration [Ca2+]i were also examined. KEY RESULTS OZR exhibited mild hyperglycaemia, hypercholesterolemia, hypertryglyceridemia and hyperinsulinemia. Insulin induced endothelium- and NO-dependent relaxations in LZR that were impaired in OZR. Inhibition of PI3K reduced relaxation induced by insulin and by the β-adrenoceptor agonist isoprenaline, mainly in arteries from LZR. Antagonism of endothelin 1 (ET-1) receptors did not alter insulin-induced relaxation in either LZR or OZR, but MAPK blockade increased the responses in OZR. Insulin decreased [Ca2+]i, a response impaired in OZR. CONCLUSIONS AND IMPLICATIONS Insulin-induced relaxation was impaired in penile arteries of OZR due to altered NO release through the PI3K pathway and unmasking of a MAPK-mediated vasoconstriction. This vascular insulin resistance is likely to contribute to the endothelial dysfunction and erectile dysfunction associated with insulin resistant states. PMID:20735420

  14. Erectile dysfunction, metabolic syndrome and arterial disease. Clinical-pathological relation by carotid ultrasonography.

    PubMed

    Arrabal-Polo, M A; Vera-Arroyo, B; Lahoz-García, C; Valderrama-Illana, P; Cámara-Ortega, M; Arrabal-Martín, M; Zuluaga-Gomez, A; Lopez-Carmona Pintado, F

    2014-04-01

    Different studies have shown the relationship between erectile dysfunction, metabolic syndrome and cardiovascular disease. The objective of this study was to evaluate the presence of arteriopathy performing carotid ultrasound in patients with and without erectile dysfunction. We conducted a case-control study with 44 patients consulting for erectile dysfunction and 20 controls. All subjects completed the IIEF-5 test and we studied the criteria for metabolic syndrome, and a carotid ultrasound to study the intima-media thickness and the presence of atherosclerotic plaques was performed. Mean intima-media thickness was .71mm±.21 for the right and of .71±.17 for the left carotid in patients with erectile dysfunction. In the control group, the means were .54±0.11 and 0.59±0.15mm respectively, statistically significant differences (P=.02 and P=.05 respectively). No plaque was found in any control, but in 25% of both carotid arteries of patients with erectile dysfunction (P=.01). As metabolic syndrome, according to the American Heart Association, were diagnosed 52.8% of patients with erectile dysfunction, and 16.7% of controls, and according to the International Diabetes Federation, 52.3% of patients with erectile dysfunction and 25% of controls met diagnostic criteria. In both cases there were significant differences (P<.01 and P=.02 respectively). We found a positive linear correlation between waist circumference and the intima-media thickness in both carotid (P<.05). Patients with erectile dysfunction may be at increased risk of cardiovascular disease, as determined by the presence of arterial disease in the carotid arteries, which indicates that we should made a more thorough and comprehensive study of patients with erectile dysfunction. Copyright © 2013 AEU. Published by Elsevier Espana. All rights reserved.

  15. The effect from different numbers of segmental arteries ligation to the spinal cord in the clinical practice of posterior vertebral column resection correction.

    PubMed

    Zhao, Zhi; Xie, Jingming; Wang, Yingsong; Bi, Ni; Li, Tao; Zhang, Ying; Shi, Zhiyue

    2017-07-01

    In using posterior vertebral column resection (PVCR) to treat severe kyphoscoliosis, it is unavoidable to ligate and cut off several segmental arteries (SAs) of the spinal cord for exposure and hemostasis, but which would raise the neurological risks. The aim of this study is to explore the changes of intraoperative spinal cord monitoring (IOM) following ligating different numbers of SAs in PVCR. Twenty-one consecutive patients with severe kyphoscoliosis were included and treated by PVCR correction. In operation, according to ligate different numbers of SAs, the IOM changes were recorded, respectively. Examinations of the covariance between different numbers of SAs ligations and IOM changes were performed to reveal the effect to the spinal cord by SAs ligations. In all the 21 cases, averaging 1.9 pairs of SAs were ligated. With the increased numbers of ligations, SSEP amplitudes and latencies were changed more obviously: from 1 to 3 pairs ligations, the mean decreased percentages of amplitudes were from 53.20 to 78.15%, the mean increased percentages of latency were from 1.23 to 1.40%, and the mean durations of decreased SSEP amplitudes were from 3.23 to 5.2 min; but without abnormal MEP changes. None occurred postoperative or delayed neurological deficit. Correlation analysis identified significant correlations between the number of SAs ligation and decreased percentage of SSEP amplitude (r = 0.945, P < 0.0001), and between the number of SAs being ligated and the duration of SSEP change (r = 0.945, P = 0.0002). Following the increased number of SAs ligation, the amplitude of SSEP is decreased more obviously with a much longer duration of recovery and the risk to spinal cord will be increased greatly. In the PVCR correction on the basis of spinal shortening, the numbers of SAs ligations should be as less as possible for neurological safety.

  16. High Spinal Anesthesia Enhances Anti-Inflammatory Responses in Patients Undergoing Coronary Artery Bypass Graft Surgery and Aortic Valve Replacement: Randomized Pilot Study.

    PubMed

    Lee, Trevor W R; Kowalski, Stephen; Falk, Kelsey; Maguire, Doug; Freed, Darren H; HayGlass, Kent T

    2016-01-01

    Cardiac surgery induces many physiologic changes including major inflammatory and sympathetic nervous system responses. Here, we conducted a single-centre pilot study to generate hypotheses on the potential immune impact of adding high spinal anaesthesia to general anaesthesia during cardiac surgery in adults. We hypothesized that this strategy, previously shown to blunt the sympathetic response and improve pain management, could reduce the undesirable systemic inflammatory responses caused by cardiac surgery. This prospective randomized unblinded pilot study was conducted on 14 patients undergoing cardiac surgery for coronary artery bypass grafting and/or aortic valve replacement secondary to severe aortic stenosis. The primary outcome measures examined longitudinally were serum pro-inflammatory (IL-6, IL-1b, CCL2), anti-inflammatory (IL-10, TNF-RII, IL-1Ra), acute phase protein (CRP, PTX3) and cardiovascular risk (sST2) biomarkers. The kinetics of pro- and anti-inflammatory biomarker was determined following surgery. All pro-inflammatory and acute phase reactant biomarker responses induced by surgical stress were indistinguishable in intensity and duration between control groups and those who also received high spinal anaesthesia. Conversely, IL-10 levels were markedly elevated in both intensity and duration in the group receiving high spinal anesthesia (p = 0.005). This hypothesis generating pilot study suggests that high spinal anesthesia can alter the net inflammatory response that results from cardiac surgery. In appropriately selected populations, this may add incremental benefit by dampening the net systemic inflammatory response during the week following surgery. Larger population studies, powered to assess immune, physiologic and clinical outcomes in both acute and longer term settings, will be required to better assess potential benefits of incorporating high spinal anesthesia. ClinicalTrials.gov NCT00348920.

  17. High Spinal Anesthesia Enhances Anti-Inflammatory Responses in Patients Undergoing Coronary Artery Bypass Graft Surgery and Aortic Valve Replacement: Randomized Pilot Study

    PubMed Central

    Lee, Trevor W. R.; Kowalski, Stephen; Falk, Kelsey; Maguire, Doug; Freed, Darren H.; HayGlass, Kent T.

    2016-01-01

    Background Cardiac surgery induces many physiologic changes including major inflammatory and sympathetic nervous system responses. Here, we conducted a single-centre pilot study to generate hypotheses on the potential immune impact of adding high spinal anaesthesia to general anaesthesia during cardiac surgery in adults. We hypothesized that this strategy, previously shown to blunt the sympathetic response and improve pain management, could reduce the undesirable systemic inflammatory responses caused by cardiac surgery. Methods This prospective randomized unblinded pilot study was conducted on 14 patients undergoing cardiac surgery for coronary artery bypass grafting and/or aortic valve replacement secondary to severe aortic stenosis. The primary outcome measures examined longitudinally were serum pro-inflammatory (IL-6, IL-1b, CCL2), anti-inflammatory (IL-10, TNF-RII, IL-1Ra), acute phase protein (CRP, PTX3) and cardiovascular risk (sST2) biomarkers. Results The kinetics of pro- and anti-inflammatory biomarker was determined following surgery. All pro-inflammatory and acute phase reactant biomarker responses induced by surgical stress were indistinguishable in intensity and duration between control groups and those who also received high spinal anaesthesia. Conversely, IL-10 levels were markedly elevated in both intensity and duration in the group receiving high spinal anesthesia (p = 0.005). Conclusions This hypothesis generating pilot study suggests that high spinal anesthesia can alter the net inflammatory response that results from cardiac surgery. In appropriately selected populations, this may add incremental benefit by dampening the net systemic inflammatory response during the week following surgery. Larger population studies, powered to assess immune, physiologic and clinical outcomes in both acute and longer term settings, will be required to better assess potential benefits of incorporating high spinal anesthesia. Trial Registration Clinical

  18. A histopathological analysis of the human cervical spinal cord in patients with acute traumatic central cord syndrome.

    PubMed

    Jimenez, O; Marcillo, A; Levi, A D

    2000-09-01

    We have applied conventional histochemical and morphometric techniques to study the changes within the human spinal 'hand' motor neuron pool after spinal cord injury in patients who presented with acute traumatic central cord syndrome (ATCCS). To determine whether a reduction of large alpha motor neurons at the C7, C8 and T1 spinal cord levels underlies the mechanism which causes hand dysfunction seen in patients with (ATCCS). The etiology of upper extremity weakness in ATCCS is debated and injury and/or degeneration of motor neurons within the central gray matter of the cervical enlargement has been advanced as one potential etiology of hand weakness. The spinal cords of five individuals with documented clinical evidence of ATCCS and three age-matched controls were obtained. The ATCCS spinal cords were divided into acute/sub-acute (two cases) and chronic (three cases) groups depending on the time to death after their injury; the chronic group was further subdivided according to the epicenter of injury. We counted the motor neurons using light microscopy in 10 randomly selected axial sections at the C7, C8 and T1 spinal cord levels for each group. We also analyzed the lateral and ventral corticospinal tracts (CST) in all groups for evidence of Wallerian degeneration and compared them to controls. A primary injury to the lateral CST was present in each case of ATCCS with evidence of Wallerian degeneration distal to the epicenter of injury. There was minimal Wallerian degeneration within the ventral corticospinal tracts. In the chronic low cervical injury group, there was a decrease in motor neurons supplying hand musculature relative to the other injury groups where the motor neurons sampled at the time of death were not reduced in number when compared to the control group. We hypothesize that hand dysfunction in ATCCS can be observed after spinal cord injury without any apparent loss in the number of motor neurons supplying the hand musculature as seen in our acute

  19. Concomitant idiopathic hypertrophic spinal pachymeningitis and Guillain-Barré syndrome in a patient: coincidence or a triggering mechanism?

    PubMed

    Olubajo, Farouk; Yermakova, Tatyana; Highley, J Robin; Arzoglou, Vasileios

    2017-09-01

    Idiopathic hypertrophic spinal pachymeningitis (IHSP), a rare diffuse inflammatory thickening of the dura mater, and Guillain-Barré syndrome (GBS) are known entities but they have never been reported as concomitant diagnoses. To their knowledge, the authors present the first reported case in the international literature with supportive evidence for both IHSP (based on MRI, intraoperative, and histological findings) and GBS (based on history, clinical examination, and electrophysiological findings). They review the literature on IHSP and the diagnostic criteria for GBS, with the view of identifying a possible causative connection.

  20. [New criteria for the classification of the popliteal artery entrapment syndrome. Our experience with 14 extremities].

    PubMed

    Fernández Valenzuela, V; Matas, M; Maeso, J; Díaz, J; Juan, J; de Sobregrau, R C

    1991-01-01

    Authors explain their experiences with eight patients (14 affected limbs) with a popliteal artery entrapment syndrome. Classification, diagnosis and treatment were reviewed. Six limbs, with any malformation and presenting as a unique sign an important hypertrophy of their intern gastrocnemius muscle, couldn't be classified. As a result, a new classification of this pathology is presented being based on the anatomical and arteriographic aspects as well as oh the surgical indication. The important correlation between anomaly, physical complexion typus athletic and sports is noted.

  1. Styloid-carotid artery syndrome treated surgically with Piezosurgery: a case report and literature review.

    PubMed

    Hoffmann, Eike; Räder, Christian; Fuhrmann, Hendrik; Maurer, Peter

    2013-03-01

    Styloid-carotid artery syndrome was first described by Eagle and is associated with cervical and facial pain caused by head movement resulting from mechanical compression of the carotid nerve plexus due to an elongated styloid process. The case of a 49-year-old man with persistent cervical pain, neurological symptoms and an elongated styloid process of 7.5 cm is reported here; this patient was successfully treated using Piezosurgery. In addition, a literature review is included. Copyright © 2012 European Association for Cranio-Maxillo-Facial Surgery. Published by Elsevier Ltd. All rights reserved.

  2. Case report of atypical hemolytic uremic syndrome with retinal arterial and venous occlusion treated with eculizumab

    PubMed Central

    Greenwood, Gregory T

    2015-01-01

    Atypical hemolytic uremic syndrome (aHUS) is a rare disease caused by chronic, uncontrolled activation of the alternative complement pathway, leading to thrombotic microangiopathy. Renal impairment and progression to end-stage renal disease are common in untreated patients with aHUS, and extrarenal manifestations are being increasingly characterized in the literature. Ocular involvement remains rare in aHUS. This report describes a patient with aHUS with bilateral central retinal artery and vein occlusion, vitreous hemorrhage, and blindness in addition to renal impairment. The patient’s hematologic and renal parameters and ocular manifestation improved following initiation of eculizumab therapy. PMID:26508891

  3. Coronary artery ectasia in an adult Noonan syndrome detected on coronary CT angiography.

    PubMed

    Hakim, Fayaz A; Gruden, James F; Panse, Prasad M; Alegria, Jorge R

    2013-12-01

    Coronary ectasia is rare in patients with Noonan syndrome. When suspected during echocardiography more common causes including Kawasaki disease in children and atherosclerosis coronary artery disease in adults should be ruled out. Coronary CT angiogram, a non-invasive imaging tool may be preferred over conventional coronary angiogram in the initial diagnosis and monitoring the progression of coronary ectasia in such patients. Aspirin may be considered to prevent coronary thrombosis. Copyright © 2013 Australian and New Zealand Society of Cardiac and Thoracic Surgeons (ANZSCTS) and the Cardiac Society of Australia and New Zealand (CSANZ). Published by Elsevier B.V. All rights reserved.

  4. Peripheral Arterial Disease and Digital Gangrene: A Rare Presentation of Diabetic Hand Syndrome

    PubMed Central

    Singh, Santokh; Chand, Gian; Charan, Shiv; Arora, Sahil; Singh, Parampreet

    2013-01-01

    Digital gangrene in upper limbs may be due to systemic sclerosis, trauma, connective tissue disorders, vasculitic disorders and various myeloproliferative disorders or as a part of tropical diabetes hand syndrome which follows trauma. Peripheral arterial disease in diabetics commonly involves lower limbs. The present case, 45-year-old diabetic, presented with dry gangrene in fingertips of both hands for last two weeks without any history of trauma or lower limb gangrene. On examination and workup of the patient was found to have bilateral upper limb arterio-occlusive disease involving ulnar vessels as a macrovascular complication of diabetes mellitus. This presentation of diabetic hand syndrome is very, very rare, hence being reported. PMID:24298503

  5. Horner's Syndrome due to a Spontaneous Internal Carotid Artery Dissection after Deep Sea Scuba Diving.

    PubMed

    Alonso Formento, Jose Enrique; Fernández Reyes, Jose Luis; Envid Lázaro, Blanca Mar; Fernández Letamendi, Teresa; Yeste Martín, Ryth; Jódar Morente, Francisco José

    2016-01-01

    Internal carotid artery dissection (ICAD) is a rare entity that either results from traumatic injury or can be spontaneously preceded or not by a minor trauma such as sporting activities. It represents a major cause of stroke in young patients. The diagnosis should be suspected with the combination of Horner's syndrome, headache or neck pain, and retinal or cerebral ischaemia. The confirmation is frequently made with a magnetic resonance angiography (MRA). Although anticoagulation with heparin followed by vitamin-K-antagonists is the most common treatment, there is no difference in efficacy of antiplatelet and anticoagulant drugs at preventing stroke and death in patients with symptomatic carotid dissection. We describe a patient with ICAD following deep sea scuba diving, who presented with Horner's syndrome and neck pain and was successfully treated with anticoagulants.

  6. [A case of true neurogenic thoracic outlet syndrome accompanied by an aberrant right subclavian artery].

    PubMed

    Sekiguchi, Kenji; Saito, Takanori; Yokota, Ichiro; Kowa, Hisatomo; Kanda, Fumio; Toda, Tatsushi

    2015-01-01

    A 65-year-old woman experienced progressive intrinsic muscle wasting on the right hand over a period of 7 years. The distribution of muscular atrophy and weakness was consistent with the area innervated by the right C8 and Th1 nerve roots. Neurophysiological examination suggested a right lower trunk lesion. An elongated right transverse process of the C7 vertebra and an aberrant subclavian artery were detected on computed tomography images, and the right lower trunk of the brachial plexus appeared to be lifted upward on magnetic resonance images. The patient was diagnosed with true neurogenic thoracic outlet syndrome. A fibrous band extending from the elongated transverse process was found during surgery, and symptoms did not progress further after resection of the band. True neurogenic thoracic outlet syndrome can cause monomelic amyotrophy, and localized neuroimaging and detailed neurophysiological examination were useful for diagnosis.

  7. Horner's Syndrome due to a Spontaneous Internal Carotid Artery Dissection after Deep Sea Scuba Diving

    PubMed Central

    Fernández Reyes, Jose Luis; Envid Lázaro, Blanca Mar; Fernández Letamendi, Teresa; Yeste Martín, Ryth; Jódar Morente, Francisco José

    2016-01-01

    Internal carotid artery dissection (ICAD) is a rare entity that either results from traumatic injury or can be spontaneously preceded or not by a minor trauma such as sporting activities. It represents a major cause of stroke in young patients. The diagnosis should be suspected with the combination of Horner's syndrome, headache or neck pain, and retinal or cerebral ischaemia. The confirmation is frequently made with a magnetic resonance angiography (MRA). Although anticoagulation with heparin followed by vitamin-K-antagonists is the most common treatment, there is no difference in efficacy of antiplatelet and anticoagulant drugs at preventing stroke and death in patients with symptomatic carotid dissection. We describe a patient with ICAD following deep sea scuba diving, who presented with Horner's syndrome and neck pain and was successfully treated with anticoagulants. PMID:27525139

  8. [Long term effect of spinal cord stimulation in a patient with complex regional pain syndrome and phantom pain syndrome type 1 after amputation].

    PubMed

    Enggaard, Thomas Peter; Scherer, Christian; Nikolajsen, Lone; Andersen, Claus

    2008-02-04

    The development of stump and phantom pain after limb amputation in patients with complex regional pain syndrome (CRPS) is very frequent. Stump pain is typically recurred CRPS and the possibilities for effective pharmacological pain relief are often limited. Spinal cord stimulation (SCS) has a well-documented pain relieving effect in patients with CRPS. This case story summarises the long term effect of SCS in a patient with CRPS after two amputations of the right leg. Pharmacological pain therapies as well as Guanethidine blockade were found to be ineffective.

  9. The location and function of respiratory fibres in the second cervical spinal cord segment: respiratory dysfunction syndrome after cervical cordotomy.

    PubMed Central

    Lahuerta, J; Buxton, P; Lipton, S; Bowsher, D

    1992-01-01

    After high cervical percutaneous cordotomy for pain in malignant disease, 12 patients died during sleep at postoperative intervals between 1 and 8 days. Nine died after a first cordotomy and three after a second (contralateral) procedure. All except one had known pulmonary disease before operation. The operated segment of the spinal cord (C2) was studied histologically after death. Superposition of lesion outlines made it possible to determine those parts of the lesioned areas common to all unilateral and bilateral cases respectively. All cases dying of presumed respiratory dysfunction syndrome had lesions involving the region of the anterolateral funiculus in the C2 segment containing "pain" fibres activated from the second to fifth thoracic dermatomes. The fibres whose destruction appeared to be responsible for respiratory dysfunction syndrome were completely intermingled with ascending "pain" fibres. The possibility of these fibres being afferent in function is discussed. Images PMID:1479392

  10. Posterior Branches of Lumbar Spinal Nerves - part II: Lumbar Facet Syndrome - Pathomechanism, Symptomatology and Diagnostic Work-up.

    PubMed

    Kozera, Katarzyna; Ciszek, Bogdan; Szaro, Paweł

    2017-04-12

    Posterior branches of the lumbar spinal nerves are the anatomic substrate of pain in the lower back, sacrum and the gluteal area. Such pain may be associated with various pathologies which cause pain in the posterior branches of the lumbar spinal nerves due to entrapment, mechanical irritation or inflammatory reaction and/or degeneration. The posterior branches are of significant functional importance, which is related to the function of the structures they supply, including facet joints, which are the basic biomechanical units of the spine. Low back pain caused by facet joint pathology may be triggered e.g. by simple activities, such as body rotations, unnatural positions, lifting heavy weights or excessive bending as well as chronic overloading with spinal hyperextension. Pain usually presents at the level of the lumbosacral junction (L 5 -S 1 ) and in the lower lumbar spine (L 4-5 , L 3-4 ). In the absence of specific diagnostic criteria, it is only possible to conclude that patients display tenderness at the level of the affected facet joint and that the pain is triggered by extension. Differential diagnosis for low back pain is difficult, since the pain may originate from various structures. The most reliable method of identifying Lumbar Facet Syndrome has been found to be a positive response to an analgesic procedure in the form of a block of the medial branch or intraarticular injection. There appear to be good grounds for conducting further studies and developing unequivocal diagnostic tests.

  11. A retrospective review to assess whether spinal fusion and scoliosis correction improved activity and participation for children with Angelman syndrome.

    PubMed

    Sewell, Mathew David; Wallace, Charles; Gibson, Alex; Noordeen, Hilali; Tucker, Stewart; Molloy, Sean; Lehovsky, Jan

    2016-10-01

    This study investigates outcome of scoliosis treatment for 11 children with Angelman syndrome (AS), with particular focus on activity, participation and the musculoskeletal factors that may affect these outcomes. Retrospective review of medical records, radiographs and questionnaires administered to caregivers of 11 children (8M:3F) with AS and scoliosis. Six underwent observational treatment during childhood and five underwent spinal fusion. The Activities Scale for Kids (ASKp) questionnaire was used to measure activity and participation. Questionnaire and radiographic data were recorded over a 2 year period. In the observational group, scoliosis increased from 31° to 46°. Mean ASKp decreased from 13.8 to 11.9 (p = 0.06). In the operative group, scoliosis decreased from 68° to 29°. Mean ASKp increased from 11.4 to 15.9 (p < 0.01). There was also a reduction in spinal-related pain and mean number of hospital admissions for chest infection. However, there was a 60% major complication rate. There was no difference in mobility, GMFCS level, feeding or communication in either group before or after treatment. In children with significant scoliosis and AS, spinal fusion was associated with a small improvement in activity and participation, reduction in pain and a decrease in frequency of severe chest infections. Non-operative treatment resulted in progression of scoliosis during childhood and decrease in activity.

  12. [Anti-amphiphysin antibody-positive paraneoplastic neurological syndrome with a longitudinally extensive spinal cord lesion of the dorsal column].

    PubMed

    Neshige, Shuichiro; Hara, Naoyuki; Takeshima, Shinichi; Iwaki, Hirotaka; Shimoe, Yutaka; Takamatsu, Kazuhiro; Kuriyama, Masaru

    2014-01-01

    A 53-year-old woman was admitted to our hospital because of gait disturbance and paresthesia of the lower extremities. She also had marked deep sense impairment in her lower limbs. Cervical MRI showed a longitudinally extensive spinal cord lesion of the dorsal column at levels C1-T11. The findings of cerebrospinal fluid examination, including the IgG index (0.65), were normal. Serum anti-AQP4 antibody was negative, but anti-amphiphysin antibody was positive. Electrophysiological examinations suggested the presence of lesions in the dorsal column of the spinal cord and dorsal root ganglion (DRG). Enlargement of and fluorodeoxyglucose accumulation in her left parasternal lymph node was observed on contrast-enhanced CT and PET-CT, respectively. The lymph node biopsy was underwent by using thoracoscopy. The metastasis of carcinoma was pathologically confirmed. Although the primary tumor was not detected on PET-CT re-examination, immunostaining of the biopsied lymph node specimen was positive for both the progesterone receptor and estrogen receptor. On the basis of these findings, the patient was diagnosed with paraneoplastic neurological syndrome due to potential breast cancer. The disorder is an immunological subacute sensory neuropathy with a longitudinally extensive spinal cord lesion of the dorsal column and a DRG lesion.

  13. The assessment of pelvic statics in patients with spinal overload syndrome treated in whole-body cryotherapy.

    PubMed

    Sliwiński, Zbigniew; Kufel, Wojciech; Michalak, Beata; Halat, Bartłomiej; Kiebzak, Wojciech; Wilk, Magdalena; Jonak, Robert

    2005-04-30

    Background. In pain syndromes involving the lumbo-sacral region, the pelvis, and the lower extremities, the mobility of the hip joint is disrupted by structural and functional changes in tissues, which also cause irritation of the ligaments and muscles of the pelvis. Dislocation of the pelvis with incorrect alignment of the sacroiliac bone leads to an oblique load on the lumbar vertebrae and muscle tension. In recent years whole-body cryotherapy has come to be more and more often applied in the comprehensive treatment of spinal overload syndrome, to reduce pain, relax skeletal muscles, and increase joint mobility. Material and method. The research was conducted in the SP ZOZ outpatient rehabilitation clinic in Zgorzelec, Poland, from December 2004 to March 2005. The study group consisted of 20 persons, 13 women (65%) and 7 men (35%), ranging in age from 23 to 77 years (mean age 47). Each of the subjects received whole-body cryotherapy in a 20-day cycle, once a day for 3 minutes at a temperature of -130 degrees C. The length of the pelvic muscles implicated in overload syndrome, the pain pressure of the pelvic ligaments, the Pidelou test, and Patrick's symptom were assessed before therapy was commenced and after its completion. Immediately after each session the patients received kinesitherapy under supervision of a physiotherapist, Magine exercises, post-isometric relaxation using Mitchel's method for the muscles and the intraspinal, lumbo-sacral and ilio-lumbar ligaments, active of the lumbar spine in the non-painful direction, and neuromobilization using Butler's method. This was supplemented by exercises on the ergometer in horizontal position. Results. Whole-body cryotherapy applied together with kinesitherapy was effective in reducing pressure pain in the ligaments (average 20%) and tension in significant pelvic muscles (average 30%). Conclusions. The cryotherapy and kinesitherapy combination applied to the treatment of spinal overload syndrome gives

  14. Behcet's disease presenting with sudden-onset paraplegia due to anterior spinal artery involvement: 1-year follow-up of rehabilitation in conjunction with medication.

    PubMed

    Duman, Iltekin; Guzelkucuk, Umut; Tezel, Kutay; Aydemir, Koray; Yılmaz, Bilge

    2013-06-01

    A 26-year-old male patient with sudden-onset paraplegia was presented. Clinical and imaging evaluation revealed isolated spinal cord lesions at thoracal levels and anterior spinal arterial involvement. Diagnosis of Behcet's disease was established with associating clinical findings with medical history. Vigorous medication and rehabilitation program were performed. Through the 1-year rehabilitation period in conjunction with medication, strength and functions improved gradually. A satisfactory functional gain as a rehabilitative goal in independence in activities of daily living and long-distance ambulation achieved around 4 months. The patient reached full independence after 1-year. As conclusion, Behcet's disease can present with sudden-onset paraplegia. In case of no evident etiology for paraplegia in young male, neuro-Behcet's disease also should be kept in mind. Contrary to assumption, early aggressive treatment and continuous rehabilitation in conjunction with medication might provide good prognosis with excellent clinical outcome in spinal cord involvement. Satisfactory functional recovery should be expected only after 3-4 months, and complete independence can be achieved after 1 year.

  15. Coronary artery aneurysms in acute coronary syndrome: case series, review, and proposed management strategy.

    PubMed

    Boyer, Nathan; Gupta, Rajesh; Schevchuck, Alex; Hindnavis, Vindhya; Maliske, Seth; Sheldon, Mark; Drachman, Douglas; Yeghiazarians, Yerem

    2014-06-01

    Coronary artery aneurysm (CAA) is an uncommon clinical finding, with an incidence varying from 1.5%-4.9% in adults, and is usually considered a variant of coronary artery disease (CAD). CAA identified in the context of acute coronary syndrome (ACS) represents a unique management challenge, particularly if the morphology of the CAA is suspected to have provoked the acute clinical syndrome. CAA is associated with thrombus formation due to abnormal laminar flow, as well as abnormal platelet and endothelial-derived pathophysiologic factors within the CAA. Once formed, mural thrombus may potentiate the deposition of additional thrombus within aneurysmal segments. Percutaneous revascularization of CAA has been associated with complications including distal embolization of thrombus, no-reflow phenomenon, stent malapposition, dissection, and rupture. Presently, there are no formal guidelines to direct the management of CAA in patients presenting with ACS; controversies exist whether conservative, surgical, or catheter-based management should be pursued. In this manuscript, we present an extensive review of the existing literature and associated clinical guidelines, and propose a management algorithm for patients with this complex clinical scenario. Armed with this perspective, therapeutic decisions may be tailored to synthesize patient factors and preferences, individualized clinical assessment, and existing American Heart Association/American College of Cardiology guidelines for management of ACS.

  16. Role of histamine in posttraumatic spinal cord hyperemia and the luxury perfusion syndrome.

    PubMed

    Kobrine, A I; Doyle, T F

    1976-01-01

    The authors studied the effect of pretreatment of monkeys with antihistamines on hyperemia observed in the lateral funiculus of the spinal cord after severe experimental spinal cord trauma. After administration of Chlorpheniramine and Metiamide, the spinal cords were traumatized with a 600 gm-cm injury. Blood flow in the lateral funiculus at the injury site was then determined hourly for 6 hours. The blood flow at this site remained in the normal range at all times in all animals. Neither a hyperemia nor an ischemia could be demonstrated. This finding reaffirms the authors' previous observation that ischemia does not exist in the lateral funiculus after severe experimental spinal cord trauma, and explains the previous observation of hyperemia as a histamine-related phenomenon, easily blocked by the administration of Chlorpheniramine and Metiamide, potent antihistamines which together block both the H1 and H2 receptor sites.

  17. Anterolateral myocardial infarction induced by coronary-subclavian-vertebral steal syndrome successfully treated with stenting of the subclavian artery.

    PubMed

    Pappy, Reji; Kalapura, Tomachan; Hennebry, Thomas A

    2007-08-01

    A female patient with graft-dependent coronary circulation presented with vertebrobasilar insufficiency and NSTEMI (Non-ST-Elevation Myocardial Infarction) related to a 100 percent stenosis of the left subclavian artery. Our review of the medical literature indicates that this is the first reported case in which a patient presented with an anterolateral NSTEMI and dizziness with subsequent angiographic evidence of both coronary subclavian and vertebral subclavian steal syndromes successfully treated with angioplasty and stenting of the left subclavian artery without any intervention in the coronary arterial tree.

  18. Endovascular repair of a ruptured subclavian artery aneurysm in a patient with Ehlers-Danlos syndrome using a sandwich technique.

    PubMed

    Desai, Sapan S; Codreanu, Maria; Charlton-Ouw, Kristofer M; Safi, Hazim; Azizzadeh, Ali

    2014-10-01

    We present the case of a type IV Ehlers-Danlos syndrome patient with a ruptured right subclavian artery aneurysm and associated arteriovenous fistula who underwent successful endovascular repair requiring simultaneous stent graft repair of the innominate artery using a sandwich technique. A 17-year-old man with known type IV Ehlers-Danlos syndrome developed right neck and shoulder swelling. CTA study demonstrated a 17 × 13-cm ruptured subclavian artery aneurysm with an associated internal jugular vein arteriovenous fistula. In the hybrid suite, a 7 mm × 15-cm stent graft (Viabahn, WL Gore & Associates, Flagstaff, AZ) was advanced from the right brachial approach into the innominate artery. A separate wire was placed into the right carotid artery via the right femoral approach (7 Fr), and a 7 mm × 10-cm stent graft (Viabahn) was advanced into the innominate artery. An additional 8 mm × 10-cm stent graft (Viabahn) was placed from the right brachial approach to obtain a distal-landing zone in the axillary artery. Complex vascular anatomy, in which graft seal creation may be challenging, does not exclude endovascular approaches as the sandwich technique can be utilized as a suitable alternative to open repair.

  19. Clinical import of Horner syndrome in internal carotid and vertebral artery dissection.

    PubMed

    Lyrer, Philippe A; Brandt, Tobias; Metso, Tiina M; Metso, Antti J; Kloss, Manja; Debette, Stephanie; Leys, Didier; Caso, Valeria; Pezzini, Alessandro; Bonati, Leo H; Thijs, Vincent; Bersano, Anna; Touzé, Emmanuel; Gensicke, Henrik; Martin, Juan J; Lichy, Christoph; Tatlisumak, Turgut; Engelter, Stefan T; Grond-Ginsbach, Caspar

    2014-05-06

    To study the prognostic importance of Horner syndrome (HS) in patients with internal carotid artery dissection (ICAD) or vertebral artery dissection (VAD). In this observational study, characteristics and outcome of patients with ICAD or VAD from the CADISP (Cervical Artery Dissection and Ischemic Stroke Patients) database were analyzed. The presence of HS was systematically assessed using a standardized questionnaire. Patients with HS (HS+) were compared with HS- patients. Crude odds ratios (ORs) with 95% confidence intervals and ORs adjusted for age, sex, center, arterial occlusion, bilateral dissection, stroke severity, and type of antithrombotic treatment were calculated. We analyzed 765 patients (n = 496 with ICAD, n = 269 with VAD, n = 303 prospective, n = 462 retrospective). HS was present in 191 (38.5%) of the patients with ICAD and 36 (13.4%) of the patients with VAD (p < 0.001). HS+ ICAD patients presented less often with stroke or TIA (p < 0.001), less often had bilateral (p = 0.019) or occlusive (p = 0.001) dissections, and had fewer severe strokes (p = 0.041) than HS- ICAD patients. HS+ ICAD patients had a better functional 3-month outcome than those without HS (ORcrude = 4.0 [2.4-6.7]), and also after adjustment for outcome-relevant covariates (ORadjusted = 2.0 [1.1-4.0]). HS+ ICAD patients were less likely to have new strokes than HS- ICAD patients (p = 0.039). HS+ VAD patients more often had vessel occlusion (p = 0.014) than HS- patients but did not differ in any of the other aforementioned variables. In patients with ICAD, HS is an easily assessable marker that might indicate a more benign clinical course. HS had no prognostic meaning in patients with VAD.

  20. Prognosis after acute coronary syndrome in relation with ventricular-arterial coupling and left ventricular strain.

    PubMed

    Milewska, Agata; Minczykowski, Andrzej; Krauze, Tomasz; Piskorski, Jarosław; Heathers, James; Szczepanik, Adam; Banaszak, Agnieszka; Guzik, Przemyslaw; Wykretowicz, Andrzej

    2016-10-01

    The value of modern non-invasive indices of the left ventricle (LV) and arterial system function, and their interaction for determining prognosis in contemporarily treated patients with acute coronary syndrome (ACS) is not well established. The study aimed to determine the association of ventricular-arterial (VA) coupling, LV global longitudinal peak systolic strain (GLPSS), global strain rate (GSR) and end-diastolic volume at end-diastolic pressure 30mmHg (V30) with long-term clinical outcomes in patients with ACS. Echocardiography was applied in 569 ACS patients followed up for >12months after hospitalization. Univariate Cox proportional hazard regression models adjusted to various clinical factors, including reduced LV ejection fraction <40%, were used to compare patients between the first and third tertiles of various indices of LV and arterial systems function and their interaction for the prediction of a combined end-point (defined as either stroke, myocardial infarction or death). Results are presented as hazard ratio (HR) with 95% confidence interval (CI). There were 57 clinical outcomes during a median follow-up of 625days. Increased VA coupling >1.68 (HR 2.4; 95% CI: 1.04-5.6); V30>107mL (HR 4.5; 95% CI: 1.9-10.6), GLPSS > -12.8% (HR 2.4; 95% CI: 1.02-5.7), GSR > -0.96 1/s (HR 3.8; 95% CI: 1.6-9.1) were robustly associated with increased hazard. With a sample of contemporarily treated ACS patients, abnormal values of non-invasive indices of LV function and their interaction with arterial system, predict adverse clinical outcomes, independently of LV ejection fraction. Copyright © 2016. Published by Elsevier Ireland Ltd.

  1. Platelet abnormalities in adults with severe pulmonary arterial hypertension related to congenital heart defects (Eisenmenger syndrome).

    PubMed

    Remková, Anna; Šimková, Iveta; Valkovičová, Tatiana; Kaldarárová, Monika

    2016-12-01

    Patients with severe pulmonary arterial hypertension suffer from life-threatening thrombotic and bleeding complications. The aim of this study was to compare selected platelet, endothelial, and coagulation parameters in healthy volunteers and patients with severe pulmonary arterial hypertension because of congenital heart defects. The study included healthy volunteers (n = 50) and patients with cyanotic congenital heart defects classified as Eisenmenger syndrome (n = 41). We investigated platelet count, mean platelet volume, and platelet aggregation - spontaneous and induced by various concentrations of five agonists. Von Willebrand factor (vWF), fibrinogen, factor VIII and XII, plasminogen activator inhibitor, antithrombin, D-dimer, and antiphospholipid antibodies were also investigated. We found a decreased platelet count [190 (147-225) vs. 248 (205-295) 10 l, P < 0.0001], higher mean platelet volume [10.9 (10.1-12.0) vs. 10.2 (9.4-10.4) fl, P < 0.0001], and significantly decreased platelet aggregation (induced by five agonists, in various concentrations) in patients with Eisenmenger syndrome compared with controls. These changes were accompanied by an increase of plasma vWF antigen [141.6 (108.9-179.1) vs. 117.4 (9.2-140.7) IU/dl, P = 0.022] and serum anti-β2-glycoprotein [2.07 (0.71-3.41) vs. 0.47 (0.18-0.99) U/ml, P < 0.0001]. Eisenmenger syndrome is accompanied by platelet abnormalities. Thrombocytopenia with increased platelet size is probably due to a higher platelet turnover associated with platelet activation. Impaired platelet aggregation can reflect specific platelet behaviour in patients with Eisenmenger syndrome. These changes can be related both to bleeding and to thrombotic events. A higher vWF antigen may be a consequence of endothelial damage in Eisenmenger syndrome, but the cause for an increase of anti-β2-glycoprotein is unknown.

  2. Severe arterial hypertension: a possible complication of McCune-Albright syndrome.

    PubMed

    Ohata, Yasuhisa; Yamamoto, Takehisa; Mori, Ikuko; Kikuchi, Toru; Michigami, Toshimi; Imanishi, Yasuo; Satomura, Kenichi; Ida, Shinobu; Ozono, Keiichi

    2009-07-01

    McCune-Albright syndrome is characterized by café-au-lait spot, multiple endocrine hyperfunction, and polyostotic fibrous dysplasia. A somatic point mutation of Gsalpha protein leads to an increase in the Gsalpha-associated hormone activity in McCune-Albright syndrome. Because cyclic adenosine 3',5'-monophosphate stimulates the dopamine beta hydroxylase gene, an activating mutation of the Gsalpha protein may cause the hyperproduction of norepinephrine via dopamine. We report on a 9-year-old girl with McCune-Albright syndrome complicated by severe arterial hypertension. The urinary excretion of norepinephrine was 5- to 10-fold higher than in age-matched controls. Meta-iodobenzylguanidine scintigraphy and positron emission tomography/computed tomography (PET/CT) revealed no hot spots. These findings suggest that severe hypertension might be due to an activating mutation of Gsalpha protein in sympathetic ganglia. Because of the reported association of GNAS1 gene polymorphism with hypertension, our patient provides further evidence for a role of Gsalpha protein in hypertension.

  3. Endovascular Treatment of Bilateral Pulmonary Artery Stenoses and Superior Vena Cava Syndrome in a Patient with Advanced Mediastinal Fibrosis

    PubMed Central

    Kuban, Joshua D.; Ramanathan, Rohit; Whigham, Cliff J.

    2016-01-01

    Vascular stenosis is a relatively uncommon and often fatal sequela of mediastinal fibrosis. There are very few reports in the medical literature of endovascular treatment for concomitant bilateral pulmonary artery stenoses and superior vena cava syndrome. We report the endovascular treatment of these conditions in a 54-year-old man, and the long-term outcome. PMID:27303243

  4. Effects of aerobic exercise on the resting heart rate, physical fitness, and arterial stiffness of female patients with metabolic syndrome

    PubMed Central

    Kang, Seol-Jung; Kim,, Eon-ho; Ko, Kwang-Jun

    2016-01-01

    [Purpose] The purpose of this study was to investigate the effects of aerobic exercise on the resting heart rate, physical fitness, and arterial stiffness or female patients with metabolic syndrome. [Subjects and Methods] Subjects were randomly assigned to an exercise group (n=12) or a control group (n=11). Subjects in the exercise group performed aerobic exercise at 60–80% of maximum heart rate for 40 min 5 times a week for 12 weeks. The changes in metabolic syndrome risk factors, resting heart rate, physical fitness, and arterial stiffness were measured and analyzed before and after initiation of the exercise program to determine the effect of exercise. Arterial stiffness was assessed based on brachial-ankle pulse wave velocity (ba-PWV). [Results] Compared to the control group; The metabolic syndrome risk factors (weight, % body fat, waist circumference, systolic blood pressure, diastolic blood pressure, and HDL-Cholesterol) were significantly improved in the exercise: resting heart rate was significantly decreased; VO2max, muscle strength and muscle endurance were significantly increased; and ba-PWV was significantly decreased. [Conclusion] Aerobic exercise had beneficial effects on the resting heart rate, physical fitness, and arterial stiffness of patients with metabolic syndrome. PMID:27390411

  5. Effects of aerobic exercise on the resting heart rate, physical fitness, and arterial stiffness of female patients with metabolic syndrome.

    PubMed

    Kang, Seol-Jung; Kim, Eon-Ho; Ko, Kwang-Jun

    2016-06-01

    [Purpose] The purpose of this study was to investigate the effects of aerobic exercise on the resting heart rate, physical fitness, and arterial stiffness or female patients with metabolic syndrome. [Subjects and Methods] Subjects were randomly assigned to an exercise group (n=12) or a control group (n=11). Subjects in the exercise group performed aerobic exercise at 60-80% of maximum heart rate for 40 min 5 times a week for 12 weeks. The changes in metabolic syndrome risk factors, resting heart rate, physical fitness, and arterial stiffness were measured and analyzed before and after initiation of the exercise program to determine the effect of exercise. Arterial stiffness was assessed based on brachial-ankle pulse wave velocity (ba-PWV). [Results] Compared to the control group; The metabolic syndrome risk factors (weight, % body fat, waist circumference, systolic blood pressure, diastolic blood pressure, and HDL-Cholesterol) were significantly improved in the exercise: resting heart rate was significantly decreased; VO2max, muscle strength and muscle endurance were significantly increased; and ba-PWV was significantly decreased. [Conclusion] Aerobic exercise had beneficial effects on the resting heart rate, physical fitness, and arterial stiffness of patients with metabolic syndrome.

  6. [Foix-Chavany-Marie syndrome: anarthria and severe dyphagia after sequential bilateral infarction of the middle cerebral artery].

    PubMed

    Guhra, M; Poppenborg, M; Hagemeister, C

    2008-02-01

    Bilateral lesions of the opercula frontoparietalia are uncommon and cause a symptom cluster including anarthria, severe dysphagia, inability to chew and sometimes facial paresis. At the same time there is an automatic-voluntary dissociation, meaning that the affected muscles are functional within the scope of involuntary movements. This syndrome is known as Foix-Chavany-Marie syndrome (FCMS), (bilateral) anterior operculum syndrome or facio-pharyngo-glosso-masticatory diplegia. We report the case of a patient who suffered from FCMS after having infarctions in the territory of the middle cerebral artery on each side 4 years apart.

  7. A comparative study of transulnar and transradial artery access for percutaneous coronary intervention in patients with acute coronary syndrome.

    PubMed

    Liu, Jun; Fu, Xiang-Hua; Xue, Ling; Wu, Wei-Li; Gu, Xin-Shun; Li, Shi-Qiang

    2014-10-01

    Transradial access has become commonly used for elective evaluation of patients with coronary artery disease, but it has some disadvantages and has had limited use in the acute coronary syndrome (ACS). Because the diameter of the ulnar artery is usually larger than that of the radial artery, we hypothesized that the ulnar artery could be used as an access for percutaneous coronary intervention (PCI). The present study compares the feasibility, safety, and outcome of transulnar artery and transradial artery access for PCI in patients with ACS. We reviewed 636 patients who had PCI for ACS from May 2006 to May 2009. The patients were randomly assigned to transulnar intervention (TUI; 317) or transradial intervention (TRI; 319). Several outcomes were similar in the TUI and TRI groups: success rate of first puncture, duration of guiding catheter engagement, puncture-to-balloon inflation time, final thrombolysis in myocardial grade 3 flow, complications at the vascular access site, and postprocedure complications. The incidence of severe arterial spasm and forearm hematoma in the TUI groups was significantly less than that in the TRI group. At 1-year follow-up, the level of blood oxygen saturation at the middle finger and Doppler ultrasonographic characteristics of the ulnar artery did not significantly change from pre-PCI values for these criteria in either group. The TUI approach has results and access complications similar to the TRI approach and is a safe and feasible alternative for ACS patients. © 2014, Wiley Periodicals, Inc.

  8. Spinal cord dimensions in children with Klippel-Feil syndrome: a controlled, blinded radiographic analysis with implications for neurologic outcomes.

    PubMed

    Auerbach, Joshua D; Hosalkar, Harish S; Kusuma, Sharat K; Wills, Brian P D; Dormans, John P; Drummond, Denis S

    2008-05-20

    Retrospective case series. To evaluate the axial cross-sectional dimensions of the spinal cord and cerebrospinal fluid (CSF) column in children with Klippel-Feil Syndrome (KFS) versus an age-matched cohort of control subjects. Neurologic sequelae and chronic pain are known to occur in KFS and factors implicated include spinal cord compression from canal stenosis, segmental instability, vascular disruption, and central nervous system abnormalities including tethered cord, syrinx, and diastomyelia. No study to date, however, has examined the role of spinal cord size as a contributing factor to neurologic sequelae in KFS. We retrospectively reviewed the plain radiographic, magnetic resonance imaging (MRI), and clinical records of 12 consecutive patients between 2 and 18 years with KFS (average age 9.5 +/- 5.3 years) and 14 age-matched controls (average age 8.3 +/- 5.1 year). For each patient, plain film radiography was reviewed to compare Torg ratios at each cervical level, and axial T1-weighted MRI was used to compare spinal cord and CSF column cross-sectional area calculations. The Torg-Pavlov ratios were identical between the 2 groups (0.77 +/- 0.15 vs. 0.77 +/- 0.19). Analysis of axial T1-weighted MRI cross-sectional spinal cord area revealed that the cord was smaller in KFS patients at each level from C2-C7 compared with controls. These differences were statistically significant at C4 (P = 0.016), C5 (P = 0.035), and C6 (P = 0.032). Subset analysis of abnormal (fused) levels compared with controls revealed the same findings, although these differences were not significant due to the limited numbers available at each level. Analysis of the CSF column, however, revealed that overall the canal was slightly larger in KFS patients compared with controls, although this difference was not statistically significant. Four of the 12 KFS patients presented with neurologic symptoms, all of which improved after posterior cervical stabilization. In our cohort of patients we

  9. Ability of non-invasive intermittent blood pressure monitoring and a continuous non-invasive arterial pressure monitor (CNAP™) to provide new readings in each 1-min interval during elective caesarean section under spinal anaesthesia.

    PubMed

    McCarthy, T; Telec, N; Dennis, A; Griffiths, J; Buettner, A

    2012-03-01

    We compared the ability of automated non-invasive intermittent oscillometric blood pressure monitoring with a new device, CNAP(TM) (continuous non-invasive arterial pressure) to provide a new blood pressure reading in each 1-min interval between spinal anaesthesia and delivery during caesarean section. We also compared the accuracy of continuous non-invasive arterial pressure readings with non-invasive blood pressure measurements before spinal anaesthesia. Fifty-nine women participated. The non-invasive and continuous non-invasive monitors displayed new blood pressure readings in a mean of 82% (11%) and 83% (13%) (p = 0.97) of the one-minute intervals between spinal anaesthesia and delivery, respectively. Continuous non-invasive arterial pressure was more likely to fail on two or more consecutive minutes (p=0.001). From the pre-spinal readings, the mean bias, defined as non-invasive-continuous non-invasive arterial pressure, and limits of agreement (±2SD mean bias) for systolic, diastolic and mean blood pressure respectively were +1.3 (±26.0), -2.9 (±21.8) and +2.6 (±20.4) mmHg. The new monitor has disadvantages compared with conventional non-invasive intermittent blood pressure monitoring. Anaesthesia © 2012 The Association of Anaesthetists of Great Britain and Ireland.

  10. A rare presentation of spontaneous internal carotid artery dissection with Horner's syndrome, VIIth, Xth and XIIth nerve palsies.

    PubMed

    Majeed, Azer; Ribeiro, Nuno Pedro Lobato; Ali, Asem; Hijazi, Mohsen; Farook, Hina

    2016-10-01

    Spontaneous internal carotid artery dissection (sICAD) is an uncommon cause of isolated cranial nerve palsies. Commonly patients present with stroke, headache, facial pain and Horner's syndrome, with upto 16% having cranial nerve palsies. We present the case of a 55-year-old man who presented with hoarseness, dysphagia and tongue swelling, mimicking a tongue base tumor. He was found to have unilateral VIIth, Xth and XIIth nerve palsies with Horner's syndrome. Magnetic resonance imaging showed high signal changes and loss of signal void in right internal carotid artery, later confirmed by Angiography as a dissection with pseudo-aneurysm. He was started on anticoagulation and made a good recovery on discharge. This case presents a unique combination of cranial nerve palsies due to internal carotid artery dissection (ICAD) and to our knowledge is the first reported case in the literature. Early recognition and institution of appropriate therapy is critical to prevention of ischemic stroke.

  11. Repair of Multiple Subclavian and Axillary Artery Aneurysms in a 58-Year-Old Man with Marfan Syndrome

    PubMed Central

    Dolapoglu, Ahmet; Preventza, Ourania; Coselli, Joseph S.

    2016-01-01

    Dilation of the ascending aorta and aortic dissections are often seen in Marfan syndrome; however, true aneurysms of the subclavian and axillary arteries rarely seem to develop in patients who have this disease. We present the case of a 58-year-old man with Marfan syndrome who had undergone a Bentall procedure and thoracoabdominal aortic repair for an aortic dissection and who later developed multiple aneurysmal dilations of his right subclavian and axillary arteries. The aneurysms were successfully repaired by means of a surgical bypass technique in which a Dacron graft was placed between the carotid and brachial arteries. We also discuss our strategy for determining the optimal surgical approach in these patients. PMID:27777529

  12. Repair of Multiple Subclavian and Axillary Artery Aneurysms in a 58-Year-Old Man with Marfan Syndrome.

    PubMed

    Dolapoglu, Ahmet; de la Cruz, Kim I; Preventza, Ourania; Coselli, Joseph S

    2016-10-01

    Dilation of the ascending aorta and aortic dissections are often seen in Marfan syndrome; however, true aneurysms of the subclavian and axillary arteries rarely seem to develop in patients who have this disease. We present the case of a 58-year-old man with Marfan syndrome who had undergone a Bentall procedure and thoracoabdominal aortic repair for an aortic dissection and who later developed multiple aneurysmal dilations of his right subclavian and axillary arteries. The aneurysms were successfully repaired by means of a surgical bypass technique in which a Dacron graft was placed between the carotid and brachial arteries. We also discuss our strategy for determining the optimal surgical approach in these patients.

  13. A rare presentation of spontaneous internal carotid artery dissection with Horner's syndrome, VIIth, Xth and XIIth nerve palsies

    PubMed Central

    Majeed, Azer; Ribeiro, Nuno Pedro Lobato; Ali, Asem; Hijazi, Mohsen; Farook, Hina

    2016-01-01

    Spontaneous internal carotid artery dissection (sICAD) is an uncommon cause of isolated cranial nerve palsies. Commonly patients present with stroke, headache, facial pain and Horner's syndrome, with upto 16% having cranial nerve palsies. We present the case of a 55-year-old man who presented with hoarseness, dysphagia and tongue swelling, mimicking a tongue base tumor. He was found to have unilateral VIIth, Xth and XIIth nerve palsies with Horner's syndrome. Magnetic resonance imaging showed high signal changes and loss of signal void in right internal carotid artery, later confirmed by Angiography as a dissection with pseudo-aneurysm. He was started on anticoagulation and made a good recovery on discharge. This case presents a unique combination of cranial nerve palsies due to internal carotid artery dissection (ICAD) and to our knowledge is the first reported case in the literature. Early recognition and institution of appropriate therapy is critical to prevention of ischemic stroke. PMID:27699055

  14. A Longitudinal Relationship Between Depressive Symptoms and Development of Metabolic Syndrome: The Coronary Artery Risk Development in Young Adults Study.

    PubMed

    Womack, Veronica Y; De Chavez, Peter John; Albrecht, Sandra S; Durant, Nefertiti; Loucks, Eric B; Puterman, Eli; Redmond, Nicole; Siddique, Juned; Williams, David R; Carnethon, Mercedes R

    2016-09-01

    Despite variability in the burden of elevated depressive symptoms by sex and race and differences in the incidence of metabolic syndrome, few prior studies describe the longitudinal association of depressive symptoms with metabolic syndrome in a diverse cohort. We tested whether baseline and time-varying depressive symptoms were associated with metabolic syndrome incidence in black and white men and women from the Coronary Artery Risk Development in Young Adults study. Participants reported depressive symptoms using the Center for Epidemiologic Studies Depression Scale at four examinations between 1995 and 2010. At those same examinations, metabolic syndrome was determined. Cox proportional hazards models were used to examine the associations of depressive symptoms on the development of metabolic syndrome in 3208 participants without metabolic syndrome at baseline. For 15 years, the incidence rate of metabolic syndrome (per 10,000 person-years) varied by race and sex, with the highest rate in black women (279.2), followed by white men (241.9), black men (204.4), and white women (125.3). Depressive symptoms (per standard deviation higher) were associated with incident metabolic syndrome in white men (hazard ratio = 1.25, 95% confidence interval = 1.08-1.45) and white women (hazard ratio = 1.17, 95% confidence interval = 1.00-1.37) after adjustment for demographic characteristics and health behaviors. There was no significant association between depression and metabolic syndrome among black men or black women. Higher depressive symptoms contribute modestly to the onset of metabolic syndrome among white adults.

  15. Denervation of the renal arteries in metabolic syndrome: the DREAMS-study.

    PubMed

    Verloop, Willemien L; Spiering, Wilko; Vink, Eva E; Beeftink, Martine M A; Blankestijn, Peter J; Doevendans, Pieter A; Voskuil, Michiel

    2015-04-01

    Chronic elevation of sympathetic nervous system is a key factor in metabolic syndrome. Because renal denervation (RDN) is thought to modulate sympathetic activity, we performed the Denervation of the Renal Arteries in Metabolic Syndrome (DREAMS)-study to investigate the effects of RDN on insulin sensitivity and blood pressure (BP) in patients with metabolic syndrome. Twenty-nine patients fulfilling the criteria for metabolic syndrome and who used a maximum of 1 antihypertensive or 1 antidiabetic drug or 1 of both gave informed consent and were treated by RDN. Glucose tolerance tests and 24-hour ambulatory BP measurements were performed at baseline, at 6 and 12 months of follow-up. Moreover, we performed self-monitored BP measurements at home every month. To assess sympathetic activity, we performed muscle sympathetic nerve activity and heart rate variability measurements at baseline and follow-up. The majority of the included patients was men (57%), mean body mass index was 31±5 kg/m(2). Median insulin sensitivity as assessed by the Simple Index assessing Insulin Sensitivity oral glucose tolerance test did not change at 6- and 12-month follow-up (P=0.60 and P=0.77, respectively). Mean 24-hour BP decreased by 6±12/5±7 mm Hg 12 months after RDN (P=0.04/0.01). However, self-monitored BP measurements data showed no reduction over time. Measurements of sympathetic activity showed no reduction in systemic sympathetic activity. In conclusion, RDN did not lead to a significant improvement of insulin sensitivity ≤12 months after treatment. Although a significant reduction in ambulatory BP was observed in this nearly drug-naïve population, the self-monitored BP measurements data suggest that this may be explained by regression to the mean. Moreover, no effect in systemic sympathetic activity was observed. © 2015 American Heart Association, Inc.

  16. Thoracic Aortic Injury: Embolization of the Tenth Intercostal Artery and Endovascular Treatment in a Young Woman after Posterior Spinal Instrumentation.

    PubMed

    Lagios, Konstantinos; Karaolanis, Georgios; Perdikides, Theodossios; Bazinas, Theodoros; Kouris, Nikolaos; Sfikas, Spiros; Paxinos, Odysseas

    2015-01-01

    Iatrogenic aortic injuries are rare and well-recognized complications of a variety of procedures, including spinal surgery. The placement of pedicle screws is sometimes associated with devastating consequences. Aortic perforation with rapid hematoma formation and delayed aortic trauma leading to pseudoaneurysm formation have been described in the literature. A case describing a significant time interval between iatrogenic aortic injury and diagnosis in the absence of pseudoaneurysm formation is described in this paper and, according to our knowledge, is unique in the literature. The aortic injury was successfully treated, selecting the appropriate graft and, as a consequence, normal spinal cord blood flow was achieved.

  17. The Effect of Prophylactic Phenylephrine and Ephedrine Infusions on Umbilical Artery Blood pH in Women With Preeclampsia Undergoing Cesarean Delivery With Spinal Anesthesia: A Randomized, Double-Blind Trial.

    PubMed

    Higgins, Nicole; Fitzgerald, Paul C; van Dyk, Dominique; Dyer, Robert A; Rodriguez, Natalie; McCarthy, Robert J; Wong, Cynthia A

    2017-09-25

    Spinal anesthesia for cesarean delivery is associated with a high incidence of hypotension. Phenylephrine results in higher umbilical artery pH than ephedrine when used to prevent or treat hypotension in healthy women. We hypothesized that phenylephrine compared to ephedrine would result in higher umbilical artery pH in women with preeclampsia undergoing cesarean delivery with spinal anesthesia. This study was a randomized double-blind clinical trial. Nonlaboring women with preeclampsia scheduled for cesarean delivery with spinal anesthesia at Prentice Women's Hospital of Northwestern Medicine were randomized to receive prophylactic infusions of phenylephrine or ephedrine titrated to maintain systolic blood pressure >80% of baseline. Spinal anesthesia consisted of hyperbaric 0.75% bupivacaine 12 mg, fentanyl 15 µg, and morphine 150 µg. The primary outcome was umbilical arterial blood pH and the secondary outcome was umbilical artery base excess. One hundred ten women were enrolled in the study and 54 per group were included in the analysis. There were 74 and 72 infants delivered in the ephedrine and phenylephrine groups, respectively. The phenylephrine:ephedrine ratio for umbilical artery pH was 1.002 (95% confidence interval [CI], 0.997-1.007). Mean [standard deviation] umbilical artery pH was not different between the ephedrine 7.20 [0.10] and phenylephrine 7.22 [0.07] groups (mean difference -0.02, 95% CI of the difference -0.06 to 0.07; P = .38). Median (first, third quartiles) umbilical artery base excess was -3.4 mEq/L (-5.7 to -2.0 mEq/L) in the ephedrine group and -2.8 mEq/L (-4.6 to -2.2mEq/L) in the phenylephrine group (difference -0.6 mEq/L, 95% CI of the difference -1.6 to 0.3 mEq/L; P = .10). When adjusted for gestational age and infant gender, umbilical artery pH did not differ between groups. There were also no differences in the umbilical artery pH stratified by magnesium therapy or by the severity of preeclampsia. We were unable to demonstrate a

  18. Reversible Cerebral Vasoconstriction Syndrome With Involvement of External Carotid Artery Branches

    PubMed Central

    Shaik, S.; Chhetri, S. K.; Roberts, G.; Wuppalapati, S.

    2014-01-01

    A 44-year-old woman presented with recurrent episodes of thunderclap headache. Neurological examination and computed tomography brain imaging were unremarkable. Cerebrospinal fluid findings were consistent with subarachnoid hemorrhage. Computed tomography angiography of the circle of Willis showed multiple areas of segmental vasoconstriction. This finding was confirmed on cerebral catheter angiography, with segmental vasoconstriction involving bilateral internal carotid, posterior cerebral, and external carotid branches. No aneurysm or other vascular abnormality was identified. She received treatment with nimodipine. A selective serotonin reuptake inhibitor, started 4 weeks earlier, was discontinued. Follow-up angiography after 3 months demonstrated complete resolution of the segmental vasoconstriction, confirming the diagnosis of reversible cerebral vasoconstriction syndrome (RCVS). She remained headache free at follow-up. To our knowledge, external carotid artery branch involvement in RCVS has been described only in one previous occasion. PMID:24982719

  19. Cerebral hyperperfusion syndrome after intracranial stenting of the middle cerebral artery

    PubMed Central

    Maramattom, Boby Varkey

    2016-01-01

    Cerebral hyperperfusion syndrome (CHS) is a rare complication following cerebral revascularization. It presents with ipsilateral headache, seizures, and intracerebral hemorrhage. It has mostly been described following extracranial carotid endarterectomy and stenting and it is very unusual after intracranial stenting. A 71-year-old man with a stuttering stroke was taken up for a cerebral angiogram (digital subtraction angiography), which showed a dissection of the distal left middle cerebral artery. This was recanalized with a solitaire AB stent. After 12 h, the patient developed a right hemiplegia and aphasia. Computed tomography brain showed two discrete intracerebral hematomas in the left hemisphere. This is the first reported case of CHS following intracranial stenting from India. PMID:27829722

  20. Nutritional status and intrarenal arterial stiffness in cardiorenal syndrome: a pilot study.

    PubMed

    Gigante, A; Di Mario, F; Barbano, B; Rosato, E; Di Lazzaro Giraldi, G; Pofi, R; Gasperini, M L; Amoroso, D; Cianci, R; Laviano, A

    2017-01-01

    Cardio-Renal Syndrome (CRS) is a condition, which is more frequently observed in clinical practice. The aim of this study is to explore nutritional status and intrarenal arterial stiffness in patients affected by CRS. 14 consecutive CRS patients, screened for anthropometry, biochemistry, nutritional and metabolic status underwent renal Doppler ultrasound and whole-body bioimpedance spectroscopy (BIS). We found a positive correlation between phase angle (PA) and CKD-EPI and MDRD (p=0.011 and p=0.007), and between body mass index and renal resistive index (RRI) (p=0.002). Finally, we found a negative correlation between fat-free mass and RRI (p=0.024). Body composition assessment may improve the care of patients with chronic kidney disease (CKD). Also, BIS may help identify changes in hydration status in CKD patients resulting as a significant predictor of mortality.

  1. Atypical Unilateral Posterior Reversible Encephalopathy Syndrome Mimicking a Middle Cerebral Artery Infarction.

    PubMed

    Çamlıdağ, İlkay; Cho, Yang-Je; Park, Mina; Lee, Seung Koo

    2015-01-01

    Posterior reversible encephalopathy syndrome (PRES) is usually a reversible clinical and radiological entity associated with typical features on brain MR or CT imaging. However, the not-so-uncommon atypical radiological presentations of the condition are also present and they may go unrecognised as they are confused with other conditions. Here, we report a very rare case of atypical, unilateral PRES in a 49-year-old uremic, post-transplant female patient who presented with seizures. Initial MRI showed high-grade occlusion of the left middle cerebral artery (MCA) and lesions suggestive of subacute infarction in the ipsilateral frontotemporoparietal lobe. Patient symptoms had resolved a day after the onset without any specific treatment but early follow-up CT findings suggested hemorrhagic transformation. Follow-up MRI performed 2 years later showed complete disappearence of the lesions and persisting MCA occlusion.

  2. Derivation of Multivariate Syndromic Outcome Metrics for Consistent Testing across Multiple Models of Cervical Spinal Cord Injury in Rats

    PubMed Central

    Ferguson, Adam R.; Irvine, Karen-Amanda; Gensel, John C.; Nielson, Jessica L.; Lin, Amity; Ly, Johnathan; Segal, Mark R.; Ratan, Rajiv R.; Bresnahan, Jacqueline C.; Beattie, Michael S.

    2013-01-01

    Spinal cord injury (SCI) and other neurological disorders involve complex biological and functional changes. Well-characterized preclinical models provide a powerful tool for understanding mechanisms of disease; however managing information produced by experimental models represents a significant challenge for translating findings across research projects and presents a substantial hurdle for translation of novel therapies to humans. In the present work we demonstrate a novel ‘syndromic’ information-processing approach for capitalizing on heterogeneous data from diverse preclinical models of SCI to discover translational outcomes for therapeutic testing. We first built a large, detailed repository of preclinical outcome data from 10 years of basic research on cervical SCI in rats, and then applied multivariate pattern detection techniques to extract features that are conserved across different injury models. We then applied this translational knowledge to derive a data-driven multivariate metric that provides a common ‘ruler’ for comparisons of outcomes across different types of injury (NYU/MASCIS weight drop injuries, Infinite Horizons (IH) injuries, and hemisection injuries). The findings revealed that each individual endpoint provides a different view of the SCI syndrome, and that considering any single outcome measure in isolation provides a misleading, incomplete view of the SCI syndrome. This limitation was overcome by taking a novel multivariate integrative approach for leveraging complex data from preclinical models of neurological disease to identify therapies that target multiple outcomes. We suggest that applying this syndromic approach provides a roadmap for translating therapies for SCI and other complex neurological diseases. PMID:23544088

  3. Impaired microvascular reactivity and endothelial function in patients with Cushing's syndrome: influence of arterial hypertension.

    PubMed

    Prázný, M; Jezková, J; Horová, E; Lazárová, V; Hána, V; Kvasnicka, J; Pecen, L; Marek, J; Skrha, J; Krsek, M

    2008-01-01

    The aim of the study was to evaluate skin microvascular reactivity (MVR) and possible influencing factors (fibrinolysis, oxidative stress, and endothelial function) in patients with Cushing's syndrome. Twenty-nine patients with active Cushing's syndrome (ten of them also examined after a successful operation) and 16 control subjects were studied. Skin MVR was measured by laser Doppler flowmetry during post-occlusive (PORH) and thermal hyperemia (TH). Malondialdehyde and Cu,Zn-superoxide dismutase were used as markers of oxidative stress. Fibrinolysis was estimated by tissue plasminogen activator (tPA) and its inhibitor (PAI-1). N-acetyl-beta-glucosaminidase, E-selectin, P-selectin, and ICAM-1 were used as markers of endothelial function. Oxidative stress and endothelial dysfunction was present in patients with hypercortisolism, however, increased concentration of ICAM-1 was also found in patients after the operation as compared to controls (290.8+/-74.2 vs. 210.9+/-56.3 ng.ml(-1), p<0.05). Maximal perfusion was significantly lower in patients with arterial hypertension during PORH and TH (36.3+/-13.0 vs. 63.3+/-32.4 PU, p<0.01, and 90.4+/-36.6 vs. 159.2+/-95.3 PU, p<0.05, respectively) and similarly the velocity of perfusion increase during PORH and TH was lower (3.2+/-1.5 vs. 5.2+/-3.4 PU.s(-1), p<0.05, and 0.95+/-0.6 vs. 1.8+/-1.1 PU.s(-1), p<0.05, respectively). The most pronounced impairment of microvascular reactivity was present in patients with combination of arterial hypertension and diabetes mellitus.

  4. An Unusual Aneurysm of the Main Pulmonary Artery Presenting as Acute Coronary Syndrome

    SciTech Connect

    Kholeif, Mona A.; El Tahir, Mohamed Kholeif, Yasser A.; El Watidy, Ahmed

    2006-10-15

    A 70-year old man presented with retrosternal chest pain. His electrocardiogram showed nonspecific T wave changes. Cardiac-specific troponin I (cTnI) was elevated. His condition was managed as acute coronary syndrome, following which he had two minor episodes of hemoptysis. A CT pulmonary angiogram showed no evidence of pulmonary embolism, but a large mass lesion was seen in the mediastinum. Echocardiography and cardiac MRI demonstrated a large solid mass, arising from the right ventricular outflow tract and causing compression of the main pulmonary artery (MPA). The differential diagnosis included pericardial and myocardial tumors and clotted aneurysm of the MPA. At surgery, a clotted aneurysmal sac was identified originating from the MPA and the defect was healed. Aneurysms of the MPA are rare. They most commonly present with dyspnea and chest pain. Compression of surrounding structures produces protean manifestations. A high index of suspicion coupled with imaging modalities establishes the diagnosis. Blunt trauma to the chest, at the time of an accident 4 years previously, may explain this aneurysm. The patient's presentation with chest pain was probably due to compression and/or stretching of surrounding structures. Coronary artery compression simulating acute coronary syndrome has been documented in the literature. The rise in cTnI may have been due to right ventricular strain, as a result of right ventricular outflow obstruction by the aneurysm. This has not been reported previously in the literature. The saccular morphology and narrow neck of the aneurysm predisposed to stagnation leading to clotting of the lumen and healing of the tear, which caused the diagnostic difficulty.

  5. Coronary Subclavian Steal Syndrome Causing Acute Myocardial Infarction in a Patient Undergoing Coronary-Artery Bypass Grafting

    PubMed Central

    Mandak, Jiri; Lojik, Miroslav; Tuna, Martin; Chek, James Lago

    2012-01-01

    Coronary subclavian steal syndrome with retrograde blood flow in the left internal mammary-coronary bypass graft is a rare but severe complication of cardiac surgery. The authors present a case of a 68-year-old man after coronary-artery bypass grafting using an internal mammary artery. He had been suffering from angina pectoris for the last several years before surgery. The patient was resuscitated at home by emergency medical service because of primary ventricular fibrillation due to an acute myocardial infarction 5 years after surgery. An occlusion of the left subclavian artery with the retrograde blood flow in the left internal mammary coronary bypass was found. This could have been the cause of insufficiency in coronary blood flow and ischemia of the myocardial muscle. The subclavian artery occlusion was successfully treated with percutaneous transluminal angioplasty and implantation of 2 stents. The patient remained free of any symptoms 2 years after this procedure. PMID:22969810

  6. A Case Series of Laparoscopic Duodenojejunostomy for the Treatment of Pediatric Superior Mesenteric Artery Syndrome

    PubMed Central

    Bohanon, FJ; Nunez Lopez, O; Graham, BM; Griffin, LW; Radhakrishnan, RS

    2016-01-01

    Superior mesenteric artery syndrome (SMAS) is a rare, debilitating clinical condition caused by compression of the third portion of the duodenum by the superior mesenteric artery. Common symptoms include intermittent postprandial abdominal pain, nausea, weight loss, and bilious vomiting. Here we present a case series of three patients with SMAS who were treated with laparoscopic duodenojejunostomy. Patients were females between 12-17 years old. All patients underwent a successful laparoscopic duodenojejunostomy after diagnosis. Mean time to feedings after surgery was 4.00±1.15 days (mean ± SD) and length of stay was 8.6±2.7 days. SMAS remains a complex disease to diagnose and treat. Here we demonstrate that laparoscopic treatment of SMAS is a safe surgical treatment option, and is associated with earlier initiation of enteral feeds and a shorter hospital stay after surgery when compared to medical treatment. This is a safe, effective, and relatively simple procedure for the experienced minimally invasive surgeon [1]. PMID:27747293

  7. Impact of Metabolic Syndrome on Mortality and Morbidity After Coronary Artery Bypass Grafting Surgery

    PubMed Central

    Ardeshiri, Maryam; Faritus, Zahra; Ojaghi-Haghighi, Zahra; Bakhshandeh, Hooman; Kargar, Faranak; Aghili, Rokhsareh

    2014-01-01

    Background: The prevalence of Metabolic syndrome (MetS) has been increased in Asian countries. It represents a cluster of cardiovascular risk factors including obesity, insulin resistance, lipid abnormality and hypertension. Objectives: The purpose of this study was to assess the association between MetS and outcome in patients undergoing coronary artery bypass grafting surgery (CABG). Patients and Methods: This prospective study was performed on patients scheduled for coronary artery bypass grafting surgery (CABG). All the patients were followed up in hospital and three months afterward. Patients were excluded if they were younger than 18 years or had severe comorbidities, a history of valvular heart disease, and low ejection fraction. Results: A total of 235 patients (135 women) with a mean age of 59 ± 9.3 years were included. MetS was more prevalent in women (P < 0.001). The most prevalent complications were bleeding [20 (8.5%)] and dysrhythmia [18 (7.7%)]. At three months follow-up, the frequency rates of readmission [24 (10.2%)] and mediastinitis [9 (3.8%)] were higher than other complications. Diabetes and MetS were risk factors for a long ICU stay (> 5 days) and atelectasia (P < 0.05). Significant associations were observed between diabetes and pulmonary embolism (P = 0.025) and mediastinitis (P = 0.051). Conclusions: Identification of MetS before CABG can predict the surgery outcome. Patients with MetS have increased risks for longer ICU stay and atelectasia. PMID:25478548

  8. Impact of metabolic syndrome on mortality and morbidity after coronary artery bypass grafting surgery.

    PubMed

    Ardeshiri, Maryam; Faritus, Zahra; Ojaghi-Haghighi, Zahra; Bakhshandeh, Hooman; Kargar, Faranak; Aghili, Rokhsareh

    2014-08-01

    The prevalence of Metabolic syndrome (MetS) has been increased in Asian countries. It represents a cluster of cardiovascular risk factors including obesity, insulin resistance, lipid abnormality and hypertension. The purpose of this study was to assess the association between MetS and outcome in patients undergoing coronary artery bypass grafting surgery (CABG). This prospective study was performed on patients scheduled for coronary artery bypass grafting surgery (CABG). All the patients were followed up in hospital and three months afterward. Patients were excluded if they were younger than 18 years or had severe comorbidities, a history of valvular heart disease, and low ejection fraction. A total of 235 patients (135 women) with a mean age of 59 ± 9.3 years were included. MetS was more prevalent in women (P < 0.001). The most prevalent complications were bleeding [20 (8.5%)] and dysrhythmia [18 (7.7%)]. At three months follow-up, the frequency rates of readmission [24 (10.2%)] and mediastinitis [9 (3.8%)] were higher than other complications. Diabetes and MetS were risk factors for a long ICU stay (> 5 days) and atelectasia (P < 0.05). Significant associations were observed between diabetes and pulmonary embolism (P = 0.025) and mediastinitis (P = 0.051). Identification of MetS before CABG can predict the surgery outcome. Patients with MetS have increased risks for longer ICU stay and atelectasia.

  9. Metabolic syndrome impairs reactivity and wall mechanics of cerebral resistance arteries in obese Zucker rats

    PubMed Central

    Brooks, Steven D.; DeVallance, Evan; d'Audiffret, Alexandre C.; Tabone, Lawrence E.; Shrader, Carl D.; Frisbee, Jefferson C.; Chantler, Paul D.

    2015-01-01

    The metabolic syndrome (MetS) is highly prevalent in the North American population and is associated with increased risk for development of cerebrovascular disease. This study determined the structural and functional changes in the middle cerebral arteries (MCA) during the progression of MetS and the effects of chronic pharmacological interventions on mitigating vascular alterations in obese Zucker rats (OZR), a translationally relevant model of MetS. The reactivity and wall mechanics of ex vivo pressurized MCA from lean Zucker rats (LZR) and OZR were determined at 7–8, 12–13, and 16–17 wk of age under control conditions and following chronic treatment with pharmacological agents targeting specific systemic pathologies. With increasing age, control OZR demonstrated reduced nitric oxide bioavailability, impaired dilator (acetylcholine) reactivity, elevated myogenic properties, structural narrowing, and wall stiffening compared with LZR. Antihypertensive therapy (e.g., captopril or hydralazine) starting at 7–8 wk of age blunted the progression of arterial stiffening compared with OZR controls, while treatments that reduced inflammation and oxidative stress (e.g., atorvastatin, rosiglitazone, and captopril) improved NO bioavailability and vascular reactivity compared with OZR controls and had mixed effects on structural remodeling. These data identify specific functional and structural cerebral adaptations that limit cerebrovascular blood flow in MetS patients, contributing to increased risk of cognitive decline, cerebral hypoperfusion, and ischemic stroke; however, these pathological adaptations could potentially be blunted if treated early in the progression of MetS. PMID:26475592

  10. Brachial Artery Conductance During Reactive Hyperemia is Increased in Women with Polycystic Ovary Syndrome

    PubMed Central

    Raja-Khan, Nazia; Shuja, Showieb A.; Kunselman, Allen R.; Hogeman, Cynthia S.; Demers, Laurence M.; Gnatuk, Carol L.; Legro, Richard S.

    2010-01-01

    Objective To examine changes in brachial artery conductance (BAC) during reactive hyperemia in women with polycystic ovary syndrome (PCOS) compared to controls. Study Design This is a pilot case-control study performed at a single academic medical center. Changes in BAC during reactive hyperemia were evaluated in 31 women with PCOS and 11 healthy control women. Fasting glucose, insulin, lipids and androgen levels were also determined. A mixed-effects model was used to compare the PCOS curve to the control curve for change in BAC from baseline during reactive hyperemia. Results Body mass index (BMI) and testosterone levels were significantly increased in the PCOS group compared to controls (P < 0.05). In addition, the PCOS group had higher total and LDL cholesterol levels (P = 0.05 and 0.09, respectively). Change in BAC from baseline during reactive hyperemia was significantly increased in the PCOS group compared to controls even after adjusting for age, BMI and LDL cholesterol levels (P < 0.0001). There were no significant differences between the two groups in age, blood pressure, or fasting glucose or insulin levels. Conclusions Brachial artery conductance during reactive hyperemia is significantly increased in women with PCOS compared to controls and may be a novel early indicator of increased cardiovascular risk in women with PCOS. PMID:21112136

  11. Characteristics of harmonic indexes of the arterial blood pressure waveform in polycystic ovary syndrome.

    PubMed

    Hsu, Chia-Liang; Hsiu, Hsin; Hsu, Wei-Chen; Chen, Chih-Yu

    2014-08-01

    Patients with polycystic ovary syndrome (PCOS; which is often associated with increased cardiovascular risk factors) may present hemodynamic changes in the cardiovascular system. The aim of the present study was to verify whether harmonic indexes of the arterial blood pressure waveform (BPWs) can be used to discriminate between PCOS patients and healthy individuals. Twenty-minute bilateral radial BPW signals were obtained in 20 PCOS patients and 20 healthy individuals. Amplitude proportions (Cn values) and their coefficients of variation (CVn values) were calculated for harmonics 1-10 of the BPW. C1 was significantly larger whereas C4, CV2, and CV6 were significantly smaller in PCOS patients compared with healthy individuals. The results of the present study suggest that harmonic-analysis indexes of the BPWs could provide information about the arterial pulse transmission (by monitoring Cn) and cardiovascular regulatory activities (by monitoring CVn). These findings could aid the development of an easy-to-perform, noninvasive, and continuous monitoring technique to improve the detection of PCOS-induced hemodynamic changes.

  12. Metabolic syndrome impairs reactivity and wall mechanics of cerebral resistance arteries in obese Zucker rats.

    PubMed

    Brooks, Steven D; DeVallance, Evan; d'Audiffret, Alexandre C; Frisbee, Stephanie J; Tabone, Lawrence E; Shrader, Carl D; Frisbee, Jefferson C; Chantler, Paul D

    2015-12-01

    The metabolic syndrome (MetS) is highly prevalent in the North American population and is associated with increased risk for development of cerebrovascular disease. This study determined the structural and functional changes in the middle cerebral arteries (MCA) during the progression of MetS and the effects of chronic pharmacological interventions on mitigating vascular alterations in obese Zucker rats (OZR), a translationally relevant model of MetS. The reactivity and wall mechanics of ex vivo pressurized MCA from lean Zucker rats (LZR) and OZR were determined at 7-8, 12-13, and 16-17 wk of age under control conditions and following chronic treatment with pharmacological agents targeting specific systemic pathologies. With increasing age, control OZR demonstrated reduced nitric oxide bioavailability, impaired dilator (acetylcholine) reactivity, elevated myogenic properties, structural narrowing, and wall stiffening compared with LZR. Antihypertensive therapy (e.g., captopril or hydralazine) starting at 7-8 wk of age blunted the progression of arterial stiffening compared with OZR controls, while treatments that reduced inflammation and oxidative stress (e.g., atorvastatin, rosiglitazone, and captopril) improved NO bioavailability and vascular reactivity compared with OZR controls and had mixed effects on structural remodeling. These data identify specific functional and structural cerebral adaptations that limit cerebrovascular blood flow in MetS patients, contributing to increased risk of cognitive decline, cerebral hypoperfusion, and ischemic stroke; however, these pathological adaptations could potentially be blunted if treated early in the progression of MetS.

  13. Improved arterial-ventricular coupling in metabolic syndrome after exercise training: a pilot study.

    PubMed

    Fournier, Sara B; Donley, David A; Bonner, Daniel E; Devallance, Evan; Olfert, I Mark; Chantler, Paul D

    2015-01-01

    The metabolic syndrome (MetS) is associated with threefold increased risk of cardiovascular (CV) morbidity and mortality, which is partly due to a blunted CV reserve capacity, reflected by a reduced peak exercise left ventricular (LV) contractility and aerobic capacity and a blunted peak arterial-ventricular coupling. To date, no study has examined whether aerobic exercise training in MetS can reverse peak exercise CV dysfunction. Furthermore, examining how exercise training alters CV function in a group of individuals with MetS before the development of diabetes and/or overt CV disease can provide insights into whether some of the pathophysiological CV changes can be delayed/reversed, lowering their CV risk. The objective of this study was to examine the effects of 8 wk of aerobic exercise training in individuals with MetS on resting and peak exercise CV function. Twenty participants with MetS underwent either 8 wk of aerobic exercise training (MetS-ExT, n = 10) or remained sedentary (MetS-NonT, n = 10) during this period. Resting and peak exercise CV function was characterized using Doppler echocardiography and gas exchange. Exercise training did not alter resting LV diastolic or systolic function and arterial-ventricular coupling in MetS. In contrast, at peak exercise, an increase in LV contractility (40%, P < 0.01), cardiac output (28%, P < 0.05), and aerobic capacity (20%, P < 0.01), but a reduction in vascular resistance (30%, P < 0.05) and arterial-ventricular coupling (27%, P < 0.01), were noted in the MetS-ExT but not in the MetS-NonT group. Furthermore, an improvement in lifetime risk score was also noted in the MetS-ExT group. These findings have clinical importance because they provide insight that some of the pathophysiological changes associated with MetS can be improved and can lower the risk of CV disease.

  14. Patent haemostasis prevents radial artery occlusion in patients with an acute coronary syndrome.

    PubMed

    Wilson, Simon J; Mitchell, Andrew; Gray, Timothy J M; Loh, Hoe Jun; Cruden, Nick L

    2017-08-01

    A haemostatic technique that maintains radial artery flow ("patent haemostasis") following transradial catheterization reduces rates of radial artery occlusion (RAO) in patients with stable coronary disease. It is unclear whether this benefit extends to patients with an acute coronary syndrome (ACS). Patients undergoing inpatient transradial catheterization for an ACS were prospectively enrolled in a consecutive cohort study (n=300). Radial haemostasis was obtained using standard radial compression (cohort 1; n=150) or patent haemostasis (cohort 2; n=150). An end-of-case activated clotting time (ACT) was recorded and radial artery patency assessed within 24 hours of sheath removal by reverse Barbeau's test. The incidence of RAO was 16.0% following standard radial compression and 5.3% following patent haemostasis (p=0.003). Univariate predictors of RAO were patent haemostasis (OR 0.30; [0.13-0.68], p=0.004), hyperlipidaemia (OR 0.46; [0.21-0.98], p=0.04), history of current smoking (OR 2.86; [1.3-6.0], p=0.015) and longer procedure times (OR 1.03/additional minute; [1.01-1.05], p=0.003). There was no association between the end-of-case ACT and RAO (OR 1.00; [0.9-1.01] p=1.00). After adjusting for covariates, patent haemostasis reduced the risk of RAO by 70% compared to standard compression (OR 0.30; [0.12-0.77], p=0.12). The c-statistic for model discrimination was 0.79 (95% CI [0.71-0.86], p<0.001). Inverse probability treatment weighted analysis also confirmed patent haemostasis as an independent predictor of reduced RAO (OR 0.38 [0.15-0.95], p=0.039). Patent haemostasis is highly effective in preventing early RAO in patients with ACS. Copyright © 2017 Elsevier B.V. All rights reserved.

  15. Effect of High-Calcium Diet on Coronary Artery Disease in Ossabaw Miniature Swine With Metabolic Syndrome.

    PubMed

    Phillips-Eakley, Alyssa K; McKenney-Drake, Mikaela L; Bahls, Martin; Newcomer, Sean C; Radcliffe, John S; Wastney, Meryl E; Van Alstine, William G; Jackson, George; Alloosh, Mouhamad; Martin, Berdine R; Sturek, Michael; Weaver, Connie M

    2015-08-13

    Calcium is a shortfall essential nutrient that has been a mainstay of osteoporosis management. Recent and limited findings have prompted concern about the contribution of calcium supplementation to cardiovascular risk. A proposed mechanism is through the acceleration of coronary artery calcification. Determining causality between calcium intake and coronary artery calcification has been hindered by a lack of sensitive methodology to monitor early vascular calcium accumulation. The primary study aim was to assess the impact of high calcium intake on coronary artery calcification using innovative calcium tracer kinetic modeling in Ossabaw swine with diet-induced metabolic syndrome. Secondary end points (in vitro wire myography, histopathology, intravascular ultrasound) assessed coronary disease. Pigs (n=24; aged ≈15 months) were fed an atherogenic diet with adequate calcium (0.33% by weight) or high calcium (1.90% from calcium carbonate or dairy) for 6 months. Following 5 months of feeding, all pigs were dosed intravenously with (41)Ca, a rare isotope that can be measured in serum and tissues at a sensitivity of 10(-18) mol/L by accelerator mass spectrometry. Kinetic modeling evaluated early coronary artery calcification using (41)Ca values measured in serial blood samples (collected over 27 days) and coronary artery samples obtained at sacrifice. Serum disappearance of (41)Ca and total coronary artery (41)Ca accumulation did not differ among groups. Secondary end points demonstrated no treatment differences in coronary artery disease or function. There was no detectable effect of high calcium diets (from dairy or calcium carbonate) on coronary artery calcium deposition in metabolic syndrome swine. © 2015 The Authors. Published on behalf of the American Heart Association, Inc., by Wiley Blackwell.

  16. Effect of High-Calcium Diet on Coronary Artery Disease in Ossabaw Miniature Swine With Metabolic Syndrome

    PubMed Central

    Phillips-Eakley, Alyssa K; McKenney-Drake, Mikaela L; Bahls, Martin; Newcomer, Sean C; Radcliffe, John S; Wastney, Meryl E; Van Alstine, William G; Jackson, George; Alloosh, Mouhamad; Martin, Berdine R; Sturek, Michael; Weaver, Connie M

    2015-01-01

    Background Calcium is a shortfall essential nutrient that has been a mainstay of osteoporosis management. Recent and limited findings have prompted concern about the contribution of calcium supplementation to cardiovascular risk. A proposed mechanism is through the acceleration of coronary artery calcification. Determining causality between calcium intake and coronary artery calcification has been hindered by a lack of sensitive methodology to monitor early vascular calcium accumulation. The primary study aim was to assess the impact of high calcium intake on coronary artery calcification using innovative calcium tracer kinetic modeling in Ossabaw swine with diet-induced metabolic syndrome. Secondary end points (in vitro wire myography, histopathology, intravascular ultrasound) assessed coronary disease. Methods and Results Pigs (n =24; aged ≈15 months) were fed an atherogenic diet with adequate calcium (0.33% by weight) or high calcium (1.90% from calcium carbonate or dairy) for 6 months. Following 5 months of feeding, all pigs were dosed intravenously with 41Ca, a rare isotope that can be measured in serum and tissues at a sensitivity of 10−18 mol/L by accelerator mass spectrometry. Kinetic modeling evaluated early coronary artery calcification using 41Ca values measured in serial blood samples (collected over 27 days) and coronary artery samples obtained at sacrifice. Serum disappearance of 41Ca and total coronary artery 41Ca accumulation did not differ among groups. Secondary end points demonstrated no treatment differences in coronary artery disease or function. Conclusion There was no detectable effect of high calcium diets (from dairy or calcium carbonate) on coronary artery calcium deposition in metabolic syndrome swine. PMID:26272654

  17. Nonthyroidal illness syndrome in off-pump coronary artery bypass surgery

    PubMed Central

    Caluk, Selma; Caluk, Jasmin; Osmanovic, Enes

    2015-01-01

    Objective: It is well known that coronary artery bypass grafting (CABG) is often the cause of non-thyroidal illness syndrome (NTIS). Nonthyroidal illness syndrome (NTIS) is a state characterized by low levels of tri-iodo-thyronine (T3) and high levels of reverse T3 (rT3), with normal or low levels of thyroxin (T4) and normal, low-normal, or low levels of thyroid-stimulating hormone (TSH). Today, there are two main techniques of CABG: CABG with the use of cardiopulmonary bypass (on-pump coronary artery bypass - ONCAB) and CABG without the use of cardiopulmonary bypass (off-pump coronary artery bypass OPCAB), or ’beating-heart surgery.’ The OPCAB technique is considered to be less invasive. We prospectively investigated the influence of these surgical techniques on the occurrence of NTIS. Methods: Serum levels of free fractions of thyroid hormones (FT3 and FT4) and TSH were analyzed in 70 consecutive patients subjected to CABG surgery, using the ONCAB technique in 36 patients and OPCAB technique in 34 patients. The measurements of hormone levels were performed prior to surgery and 12 hours and 14 days after surgery. Results: The basic, the early, and the late postoperative serum levels of FT3 (p=0.458, p=0.632, p=0.869, respectively), FT4 (p=0.664, p=0.301, p=0.417, respectively), and TSH (p=0.249, p=0.058, p=0.324, respectively) were similar in both groups. The levels of FT3 and TSH were significantly lower 12 hours after surgery (p<0.0001, p<0.0001, respectively), and the FT4 levels rose at the same time (p<0.0001). The third measurement showed the return of all investigated parameters back to physiological levels, although they were still not precisely within the initial values. Conclusion: NTIS occurs significantly in patients subjected to CABG. Although the OPCAB technique is considered to be less invasive, its impact on the occurrence of NTIS does not differ significantly from the ONCAB technique. PMID:25592105

  18. Nonthyroidal illness syndrome in off-pump coronary artery bypass surger.

    PubMed

    Caluk, Selma; Caluk, Jasmin; Osmanovic, Enes

    2015-10-01

    It is well known that coronary artery bypass grafting (CABG) is often the cause of non-thyroidal illness syndrome (NTIS). Non-thyroidal illness syndrome (NTIS) is a state characterized by low levels of tri-iodo-thyronine (T3) and high levels of reverse T3 (rT3), with normal or low levels of thyroxin (T4) and normal, low-normal, or low levels of thyroid-stimulating hormone (TSH). Today, there are two main techniques of CABG: CABG with the use of cardiopulmonary bypass (on-pump coronary artery bypass - ONCAB) and CABG without the use of cardiopulmonary bypass (off-pump coronary artery bypass OPCAB), or 'beating-heart surgery.' The OPCAB technique is considered to be less invasive. We prospectively investigated the influence of these surgical techniques on the occurrence of NTIS. Serum levels of free fractions of thyroid hormones (FT3 and FT4) and TSH were analyzed in 70 consecutive patients subjected to CABG surgery, using the ONCAB technique in 36 patients and OPCAB technique in 34 patients. The measurements of hormone levels were performed prior to surgery and 12 hours and 14 days after surgery. The basic, the early, and the late postoperative serum levels of FT3 (p=0.458, p=0.632, p=0.869, respectively), FT4 (p=0.664, p=0.301, p=0.417, respectively), and TSH (p=0.249, p=0.058, p=0.324, respectively) were similar in both groups. The levels of FT3 and TSH were significantly lower 12 hours after surgery (p<0.0001, p<0.0001, respectively), and the FT4 levels rose at the same time (p<0.0001). The third measurement showed the return of all investigated parameters back to physiological levels, although they were still not precisely within the initial values. NTIS occurs significantly in patients subjected to CABG. Although the OPCAB technique is considered to be less invasive, its impact on the occurrence of NTIS does not differ significantly from the ONCAB technique.

  19. Relationship between coronary artery ectasia, cocaine abuse and acute coronary syndromes

    PubMed Central

    Dendramis, Gregory; Paleologo, Claudia; Piraino, Davide; Assennato, Pasquale

    2016-01-01

    Coronary artery ectasia (CAE) often represents a coronary angiography finding casually detected or following the occurrence of an acute coronary syndrome. The pathogenetic role of cocaine abuse in the genesis of CAE is still little known and very few data are available in literature. We describe a case of a 31-year-old male cocaine user admitted to our department for typical acute chest pain. Coronary angiography showed diffuse coronary ectasia with slow flows and without hemodynamically significant stenosis. An increasing of matrix metalloproteinases values and a reduction of their tissue inhibitors was showed both during hospitalization and at one month after discharge. This case report emphasizes the close relationship between cocaine abuse, CAE and acute coronary syndromes in patients without hemodynamically significant coronary stenosis. As reported by Satran et al, cocaine abuse should be considered an important risk factor for CAE and these patients appear to be at increased risk of angina and acute myocardial infarct. Further studies that can strengthen this hypothesis would be useful to deepen and better analyze this interesting association. PMID:27231522

  20. Superior mesenteric artery syndrome following correction of kyphosis in an adult.

    PubMed

    Derincek, Alihan; Wood, Kirkham B; Muench, Clinton A

    2004-12-01

    Superior mesenteric artery (SMA) syndrome is well described in the pediatric literature; however, to date, it has not been described in an adult individual undergoing correction of thoracic kyphosis. Medical history, physical findings, and the results of imaging studies were analyzed to describe the patient's onset of gastrointestinal obstruction following surgical correction of thoracic kyphosis. The postoperative course was marked by the onset of nausea and vomiting on postoperative day 24. The patient was admitted to the hospital, and the small bowel follow-through study showed blockage of the third portion of the duodenum at the level of the SMA. Oral intake was restricted, and total parenteral nutrition was instituted with complete resolution of her symptoms. At 1 year postoperatively, the patient was symptom-free. Although vascular compression of the duodenum with subsequent nausea and vomiting and electrolyte imbalance, caused by the relative lengthening of the spine, is most commonly associated with surgical correction of coronal plane deformities (ie, scoliosis), it may occur in patients undergoing correction of thoracic kyphosis as well. SMA syndrome is a well known potential complication in adolescents, and it should also be taken into consideration in adults undergoing deformity correction. Early institution of general medical measures of nasogastric suction, oral intake restriction, and intravenous alimentation can be successful.

  1. [Surgical anatomy of spinal cord tumors].

    PubMed

    Peltier, J; Chenin, L; Hannequin, P; Page, C; Havet, É; Foulon, P; Le Gars, D

    2015-08-03

    In this article, we respectively describe the morphology of the spinal cord, spinal meningeal layers, main fiber tracts, and both arterial and venous distribution in order to explain signs of spinal cord compression. We will then describe a surgical technique for spinal cord tumor removal.

  2. Compression of the Inferior Vena Cava by the Right Iliac Artery: A Rare Variant of May-Thurner Syndrome

    SciTech Connect

    Fretz, V.; Binkert, C. A.

    2010-10-15

    May-Thurner syndrome is known as compression of the left common iliac vein by the right common iliac artery. We describe a case of an atypical compression of the inferior vena cava by the right common iliac artery secondary to a high aortic bifurcation. Despite an extensive collateral network, there was a significant venous gradient between the iliac veins and the inferior vena cava above the compression. After stenting the venous pressure gradient disappeared. Follow-up 4 months later revealed a good clinical response with a patent stent.

  3. Spontaneous ruptured dissection of the right common iliac artery in a patient with classic Ehlers-Danlos syndrome phenotype.

    PubMed

    Gaines, Rick; Tinkle, Brad T; Halandras, Pegge M; Al-Nouri, Omar; Crisostomo, Paul; Cho, Jae S

    2015-04-01

    Unlike vascular Ehlers-Danlos syndrome (EDS), classic EDS is rarely associated with vascular manifestation. We report the case of a 39-year-old man who presented with acute abdominal pain. At the time of presentation, the patient was in hypovolemic shock, and computed tomography angiogram demonstrated common iliac artery dissection with rupture. He underwent an attempted endovascular repair that was converted to an open repair of a ruptured right common iliac artery dissection. Subsequent genetic testing revealed a substitution of arginine for cysteine in type I collagen, COL1A1 exon 14 c.934C>T mutation, consistent with a rare variant of classic EDS.

  4. [The role of spinal motoneurons in the mechanisms of hypogravitational motor syndrome development].

    PubMed

    Islamov, R R; Tiapkina, O V; Nikol'skiĭ, E E; Kozlovskaia, I B; Grigor'ev, A I

    2013-03-01

    Studies results of gravity unloading influence on spinal control system of muscle structure and functions are summarized. It was shown that demyelization of axons due to reduction of genes expression responsible for myelin proteins synthesis, decrease in one of the key enzymes of cholinergic system--cholineacetyltransferase activity, alteration of normal kinetics of quantal and non-quantal neurotransmitter secretion, impaired autoregulation of acetylcholine secretion from motor nerve endings through presynaptic cholinergic receptors, slowing of axonal transport of substances in motor neurons that innervate postural muscles played the important role in the development of hypogravitational motor symptoms. At the same time, the evidences of neuroprotective mechanisms enclosing (increase in heat shock proteins Hsp25 and Hsp70 expression), that hinder apoptosis development in motor neurons and glial cells in the spinal cord under conditions of model hypogravity, were revealed.

  5. Transient unilateral brachial plexopathy and partial Horner's syndrome following spinal anesthesia for cesarean section.

    PubMed

    Anson, Jonathan A; McQuillan, Patrick M

    2014-04-01

    A healthy 21-year-old primigravida presented for elective cesarean section. At 45 min after intrathecal (IT) injection of bupivacaine, morphine and fentanyl she developed dysphagia, right sided facial droop, ptosis and ulnar nerve weakness. This constellation of signs and symptoms resolved 2 h later. Based on the time course and laterality of her symptoms, as well as the pharmacologic properties of spinal opioids, we believe her symptoms can be attributed to the IT administration of fentanyl.

  6. Vascular dysfunctions following spinal cord injury

    PubMed Central

    Popa, F; Grigorean, VT; Onose, G; Sandu, AM; Popescu, M; Burnei, G; Strambu, V; Sinescu, C

    2010-01-01

    The aim of this article is to analyze the vascular dysfunctions occurring after spinal cord injury (SCI). Vascular dysfunctions are common complications of SCI. Cardiovascular disturbances are the leading causes of morbidity and mortality in both acute and chronic stages of SCI. Neuroanatomy and physiology of autonomic nervous system, sympathetic and parasympathetic, is reviewed. SCI implies disruption of descendent pathways from central centers to spinal sympathetic neurons, originating in intermediolateral nuclei of T1–L2 cord segments. Loss of supraspinal control over sympathetic nervous system results in reduced overall sympathetic activity below the level of injury and unopposed parasympathetic outflow through intact vagal nerve. SCI associates significant vascular dysfunction. Spinal shock occurs during the acute phase following SCI and it is a transitory suspension of function and reflexes below the level of the injury. Neurogenic shock, part of spinal shock, consists of severe arterial hypotension and bradycardia. Autonomic dysreflexia appears during the chronic phase, after spinal shock resolution, and it is a life–threatening syndrome of massive imbalanced reflex sympathetic discharge occurring in patients with SCI above the splanchnic sympathetic outflow (T5–T6). Arterial hypotension with orthostatic hypotension occurs in both acute and chronic phases. The etiology is multifactorial. We described a few factors influencing the orthostatic hypotension occurrence in SCI: sympathetic nervous system dysfunction, low plasma catecholamine levels, rennin–angiotensin–aldosterone activity, peripheral alpha–adrenoceptor hyperresponsiveness, impaired function of baroreceptors, hyponatremia and low plasmatic volume, cardiovascular deconditioning, morphologic changes in sympathetic neurons, plasticity within spinal circuits, and motor deficit leading to loss of skeletal muscle pumping activity. Additional associated cardiovascular concerns in SCI, such as

  7. Vascular dysfunctions following spinal cord injury.

    PubMed

    Popa, Constantin; Popa, Florian; Grigorean, Valentin Titus; Onose, Gelu; Sandu, Aurelia Mihaela; Popescu, Mihai; Burnei, Gheorghe; Strambu, Victor; Sinescu, Crina

    2010-01-01

    The aim of this article is to analyze the vascular dysfunctions occurring after spinal cord injury (SCI). Vascular dysfunctions are common complications of SCI. Cardiovascular disturbances are the leading causes of morbidity and mortality in both acute and chronic stages of SCI. Neuroanatomy and physiology of autonomic nervous system, sympathetic and parasympathetic, is reviewed. SCI implies disruption of descendent pathways from central centers to spinal sympathetic neurons, originating in intermediolateral nuclei of T1-L2 cord segments. Loss of supraspinal control over sympathetic nervous system results in reduced overall sympathetic activity below the level of injury and unopposed parasympathetic outflow through intact vagal nerve. SCI associates significant vascular dysfunction. Spinal shock occurs during the acute phase following SCI and it is a transitory suspension of function and reflexes below the level of the injury. Neurogenic shock, part of spinal shock, consists of severe arterial hypotension and bradycardia. Autonomic dysreflexia appears during the chronic phase, after spinal shock resolution, and it is a life-threatening syndrome of massive imbalanced reflex sympathetic discharge occurring in patients with SCI above the splanchnic sympathetic outflow (T5-T6). Arterial hypotension with orthostatic hypotension occurs in both acute and chronic phases. The etiology is multifactorial. We described a few factors influencing the orthostatic hypotension occurrence in SCI: sympathetic nervous system dysfunction, low plasma catecholamine levels, rennin-angiotensin-aldosterone activity, peripheral alpha-adrenoceptor hyperresponsiveness, impaired function of baroreceptors, hyponatremia and low plasmatic volume, cardiovascular deconditioning, morphologic changes in sympathetic neurons, plasticity within spinal circuits, and motor deficit leading to loss of skeletal muscle pumping activity. Additional associated cardiovascular concerns in SCI, such as deep vein

  8. Lower limb compartment syndrome by reperfusion injury after treatment of arterial thrombosis post-laparoscopic radical hysterectomy and pelvic lymph node dissection for cervical cancer

    PubMed Central

    Yeon, Jihee; Jung, Ye Won; Yang, Shin Seok; Kang, Byung Hun; Lee, Mina; Ko, Young Bok; Yang, Jung Bo; Lee, Ki Hwan

    2017-01-01

    Compartment syndrome is a clinical condition associated with decreased blood circulation that can lead to swelling of tissue in limited space. Several factors including lithotomy position, prolonged surgery, intermittent pneumatic compressor, and reperfusion after treatment of arterial thrombosis may contribute to compartment syndrome. However, compartment syndrome rarely occurs after gynecologic surgery. In this case, the patient was diagnosed as compartment syndrome due to reperfusion injury after treatment of arterial thrombosis, which occurred after laparoscopic radical hysterectomy and pelvic lymph node dissection for cervical cancer. Despite its rarity, prevention and identifying the risk factors of complication should be performed perioperatively; furthermore, gynecologist should be aware of the possibility of complications. PMID:28344966

  9. Lower limb compartment syndrome by reperfusion injury after treatment of arterial thrombosis post-laparoscopic radical hysterectomy and pelvic lymph node dissection for cervical cancer.

    PubMed

    Yeon, Jihee; Jung, Ye Won; Yang, Shin Seok; Kang, Byung Hun; Lee, Mina; Ko, Young Bok; Yang, Jung Bo; Lee, Ki Hwan; Yoo, Heon Jong

    2017-03-01

    Compartment syndrome is a clinical condition associated with decreased blood circulation that can lead to swelling of tissue in limited space. Several factors including lithotomy position, prolonged surgery, intermittent pneumatic compressor, and reperfusion after treatment of arterial thrombosis may contribute to compartment syndrome. However, compartment syndrome rarely occurs after gynecologic surgery. In this case, the patient was diagnosed as compartment syndrome due to reperfusion injury after treatment of arterial thrombosis, which occurred after laparoscopic radical hysterectomy and pelvic lymph node dissection for cervical cancer. Despite its rarity, prevention and identifying the risk factors of complication should be performed perioperatively; furthermore, gynecologist should be aware of the possibility of complications.

  10. Aberrant right subclavian artery in fetuses with Down syndrome: a systematic review and meta-analysis.

    PubMed

    Scala, C; Leone Roberti Maggiore, U; Candiani, M; Venturini, P L; Ferrero, S; Greco, T; Cavoretto, P

    2015-09-01

    The primary objective was to estimate the prevalence of aberrant right subclavian artery (ARSA) in fetuses with Down syndrome. Secondary objectives were to assess the prevalence of ARSA in euploid fetuses, the feasibility of ultrasound evaluation of the right subclavian artery (RSA) in the first and second trimesters of pregnancy, the performance of ARSA in screening for trisomy 21 and its association with other abnormalities. Web-based databases (PubMed, EMBASE and MEDLINE) were searched up to July 2014. The STROBE, PRISMA and QUIPS instruments were used to assess all included studies and for reporting of methodology, results and conclusions. Original studies that reported prenatal ultrasound evaluation of ARSA, assessment of its prevalence in Down-syndrome and euploid fetuses, feasibility of ultrasound evaluation of the RSA in the first and second trimesters of pregnancy and correlation of ARSA with other abnormalities were included, excluding duplications and case reports. Collected data were summarized to estimate prevalence and feasibility. A meta-analysis was performed pooling the study-specific positive and negative likelihood ratios (LR+ and LR-), detection rates and false-positive rates for trisomy 21. Prevalence of ARSA in Down-syndrome fetuses was 23.6% (95% CI, 19.4-27.9%), whereas in euploid fetuses it was 1.02% (95% CI, 0.86-1.10%). Ultrasound evaluation of the RSA course and origin in the first and second trimesters of pregnancy was feasible in 85% and 98% of cases (first and second trimester, respectively) and it was directly related to sonographic experience and fetal crown-rump length and inversely related to maternal body mass index. In more than 20% of fetuses with ARSA there was an association with other abnormalities but ARSA seemed to be an independent marker of trisomy 21. The meta-analysis showed that ARSA is a significant risk factor for Down syndrome (pooled LR+ = 26.93, 95% CI, 19.36-37.47, P for effect < 0.001, P for Q = 0.3, I(2

  11. Therapeutic effect of co-transplantation of neuregulin-1-transfected Schwann cells and bone marrow stromal cells on spinal cord hemisection syndrome.

    PubMed

    Zhang, Ji Fei; Zhao, Fu Sheng; Wu, Geng; Kong, Qing Fei; Sun, Bo; Cao, Jingyan; Zhang, Yao; Wang, Jing Hua; Zhang, Jing; Jin, Xu Dong; Li, Hu Lun

    2011-06-22

    The aim of this present study is to evaluate the therapeutic effect of co-transplantation of neuregulin-1-transfected Schwann cells (SCs) and bone marrow stromal cells (BMSCs) on a rat model of spinal cord hemi-section injuries (Brown-Séquard syndrome), which is relevant to human clinical spinal cord injury. Both in vivo and in vitro data we received demonstrated that co-transplantation BMSCs with NRG1-transfected SCs reduced the size of cystic cavities, promoted axonal regeneration and hind limb functional recovery in comparison with SCs or BMSCs transplantation alone or together, and this treatment could provide important insights into potential therapies of spinal cord hemi-section injuries.

  12. Onyx embolization of an intraosseous pseudoaneurysm of the middle meningeal artery in a patient with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome.

    PubMed

    Settecase, Fabio; Nicholson, Andrew D; Amans, Matthew R; Higashida, Randall T; Halbach, Van V; Cooke, Daniel L; Dowd, Christopher F; Hetts, Steven W

    2016-03-01

    A 13-year-old boy with meningiomatosis, McCune-Albright syndrome, and gray platelet syndrome presented with an enlarging "lump" on his right forehead. A head CT scan revealed a polyostotic fibrous dysplasia involving the entire skull. A 3.4-cm right frontal osseous cavity and an overlying right forehead subcutaneous soft-tissue mass were seen, measuring 5.2 cm in diameter and 1.6 cm thick. Ultrasound of the cavity and overlying mass showed swirling of blood and an arterialized waveform. MRI revealed an en plaque meningioma underlying the cavity. An intraosseous pseudoaneurysm fed by 3 distal anterior division branches of the right middle meningeal artery (MMA) with contrast extravasation was found on angiography. Two MMA feeders were embolized with Onyx, with anterograde filling of the intraosseous cavity with Onyx. A small pocket of residual intracavity contrast filling postembolization from a smaller third MMA feeder eventually thrombosed and the forehead lump regressed.

  13. Disruption of AP1S1, Causing a Novel Neurocutaneous Syndrome, Perturbs Development of the Skin and Spinal Cord

    PubMed Central

    Drouin, Christian A.; Lapointe, Line; Boudreau, Michèle; Meloche, Caroline; Drouin, Régen; Hudson, Thomas J.; Drapeau, Pierre; Cossette, Patrick

    2008-01-01

    Adaptor protein (AP) complexes regulate clathrin-coated vesicle assembly, protein cargo sorting, and vesicular trafficking between organelles in eukaryotic cells. Because disruption of the various subunits of the AP complexes is embryonic lethal in the majority of cases, characterization of their function in vivo is still lacking. Here, we describe the first mutation in the human AP1S1 gene, encoding the small subunit σ1A of the AP-1 complex. This founder splice mutation, which leads to a premature stop codon, was found in four families with a unique syndrome characterized by mental retardation, enteropathy, deafness, peripheral neuropathy, ichthyosis, and keratodermia (MEDNIK). To validate the pathogenic effect of the mutation, we knocked down Ap1s1 expression in zebrafish using selective antisens morpholino oligonucleotides (AMO). The knockdown phenotype consisted of perturbation in skin formation, reduced pigmentation, and severe motility deficits due to impaired neural network development. Both neural and skin defects were rescued by co-injection of AMO with wild-type (WT) human AP1S1 mRNA, but not by co-injecting the truncated form of AP1S1, consistent with a loss-of-function effect of this mutation. Together, these results confirm AP1S1 as the gene responsible for MEDNIK syndrome and demonstrate a critical role of AP1S1 in development of the skin and spinal cord. PMID:19057675

  14. Fully Endoscopic Interlaminar Detethering of Spinal Cord in Tethered Cord Syndrome: A Case Report and Technical Description

    PubMed Central

    Aydín, Salih; Bozdoğan, Deniz

    2015-01-01

    A 19-year-old man presented with long lasting significant back and bilateral leg pain, and hypoesthesia on the lateral side of both his thighs for which he had undergone several courses of medication and bouts of physical therapy treatment. His urodynamic parameters were normal and lumbar magnetic resonance imaging (MRI) revealed a low-lying conus at the L2-3 level with a thickened fatty filum, and he was diagnosed as having tethered cord syndrome (TCS). The patient underwent a fully endoscopic detethering through an interlaminar approach with intraoperative neurophysiological monitoring. The thickened filum terminale was located and then the filum was coagulated and cut. The patient showed a significant improvement in his preoperative symptoms, and reported no problems at 2-year follow-up. Detethering of the spinal cord in tethered cord syndrome using a fully endoscopic interlaminar approach provides the advantages of minimal damage to tissues, less postoperative discomfort, early postoperative recovery, and a shorter hospitalization. PMID:26834820

  15. Evaluation of the radial artery applanation tonometry technology for continuous noninvasive blood pressure monitoring compared with central aortic blood pressure measurements in patients with multiple organ dysfunction syndrome.

    PubMed

    Meidert, Agnes S; Huber, Wolfgang; Hapfelmeier, Alexander; Schöfthaler, Miriam; Müller, Johannes N; Langwieser, Nicolas; Wagner, Julia Y; Schmid, Roland M; Saugel, Bernd

    2013-12-01

    We compared blood pressure (BP) measurements obtained using radial artery applanation tonometry with invasive BP measurements using a catheter placed in the abdominal aorta through the femoral artery in patients with multiple organ dysfunction syndrome (MODS). In 23 intensive care unit patients with MODS, we simultaneously assessed BP values for 15 minutes per patient using radial artery applanation tonometry (T-Line TL-200 pro device; Tensys Medical Inc, San Diego, Calif) and the arterial catheter (standard-criterion technique). A total of 2879 averaged 10-beat epochs were compared using Bland-Altman plots. The mean difference ± SD (with corresponding 95% limits of agreement) between radial artery applanation tonometry-derived BP and invasively assessed BP was +1.0 ± 5.5 mm Hg (-9.9 to +11.8 mm Hg) for mean arterial pressure, -3.3 ± 11.2 mm Hg (-25.3 to +18.6 mm Hg) for systolic arterial pressure, and +4.9 ± 7.0 mm Hg (-8.8 to +18.6 mm Hg) for diastolic arterial pressure, respectively. In intensive care unit patients with MODS, mean arterial pressure and diastolic arterial pressure can be determined accurately and precisely using radial artery applanation tonometry compared with central aortic values obtained using a catheter placed in the abdominal aorta through the femoral artery. Although systolic arterial pressure could also be derived accurately, wider 95% limits of agreement suggest lower precision for determination of systolic arterial pressure. © 2013.

  16. Proliferation of pulmonary artery smooth muscle cells in the development of ascites syndrome in broilers induced by low ambient temperature.

    PubMed

    Wang, J; Qiao, J; Zhao, L H; Li, K; Wang, H; Xu, T; Tian, Y; Gao, M; Wang, X

    2007-12-01

    Pulmonary vascular remodelling, mainly characterized by arterial medial thickening, is an important pathological feature of broiler ascites syndrome (AS). Since vascular smooth muscle cells (VSMC) form the major cellular component of arterial medial layer, we speculate that VSMC proliferation is one of the causes of pulmonary arterial medial thickening in ascitic broilers. Hence, the present study was designed to investigate the role of VSMC proliferation in pulmonary vascular remodelling in development of AS induced by low ambient temperature. Broilers in control group (22 +/- 1.5 degrees C) and low temperature group (11 +/- 2 degrees C) were sampled every week at 15-50 days of age. Proliferative indexes of VSMC in pulmonary arteries were assessed with proliferating cell nuclear antigen, and the relative medial thickness (RMT) and relative wall area (RWA), as indexes of pulmonary vascular remodelling, were examined by computer-image analysing system. The results showed that the high incidence (18.75%) of AS was induced by low temperature, and a significantly increased VSMC proliferation was observed in pulmonary arteries in the low temperature group at 22-50 days of age (P < 0.05). In addition, RMT and RWA in pulmonary arteries were significantly elevated in the low temperature group from 36 days of age (P < 0.05), indicating that pulmonary vascular remodelling occurred following VSMC proliferation in AS. Our data suggest that proliferation of VSMC may facilitate pulmonary vascular remodelling and have a pivotal role in AS induced by low ambient temperature.

  17. The SCIentinel study--prospective multicenter study to define the spinal cord injury-induced immune depression syndrome (SCI-IDS)--study protocol and interim feasibility data.

    PubMed

    Kopp, Marcel A; Druschel, Claudia; Meisel, Christian; Liebscher, Thomas; Prilipp, Erik; Watzlawick, Ralf; Cinelli, Paolo; Niedeggen, Andreas; Schaser, Klaus-Dieter; Wanner, Guido A; Curt, Armin; Lindemann, Gertraut; Nugaeva, Natalia; Fehlings, Michael G; Vajkoczy, Peter; Cabraja, Mario; Dengler, Julius; Ertel, Wolfgang; Ekkernkamp, Axel; Martus, Peter; Volk, Hans-Dieter; Unterwalder, Nadine; Kölsch, Uwe; Brommer, Benedikt; Hellmann, Rick C; Saidy, Ramin R Ossami; Laginha, Ines; Prüss, Harald; Failli, Vieri; Dirnagl, Ulrich; Schwab, Jan M

    2013-11-09

    Infections are the leading cause of death in the acute phase following spinal cord injury and qualify as independent risk factor for poor neurological outcome ("disease modifying factor"). The enhanced susceptibility for infections is not stringently explained by the increased risk of aspiration in tetraplegic patients, neurogenic bladder dysfunction, or by high-dose methylprednisolone treatment. Experimental and clinical pilot data suggest that spinal cord injury disrupts the balanced interplay between the central nervous system and the immune system. The primary hypothesis is that the Spinal Cord Injury-induced Immune Depression Syndrome (SCI-IDS) is 'neurogenic' including deactivation of adaptive and innate immunity with decreased HLA-DR expression on monocytes as a key surrogate parameter. Secondary hypotheses are that the Immune Depression Syndrome is i) injury level- and ii) severity-dependent, iii) triggers transient lymphopenia, and iv) causes qualitative functional leukocyte deficits, which may endure the post-acute phase after spinal cord injury. SCIentinel is a prospective, international, multicenter study aiming to recruit about 118 patients with acute spinal cord injury or control patients with acute vertebral fracture without neurological deficits scheduled for spinal surgery. The assessment points are: i) <31 hours, ii) 31-55 hours, iii) 7 days, iv) 14 days, and v) 10 weeks post-trauma. Assessment includes infections, concomitant injury, medication and neurological classification using American Spinal Injury Association impairment scale (AIS) and neurological level. Laboratory analyses comprise haematological profiling, immunophenotyping, including HLA-DR expression on monocytes, cytokines and gene expression of immune modulators. We provide an administrative interim analysis of the recruitment schedule of the trial. The objectives are to characterize the dysfunction of the innate and adaptive immune system after spinal cord injury and to explore its

  18. The SCIentinel study - prospective multicenter study to define the spinal cord injury-induced immune depression syndrome (SCI-IDS) - study protocol and interim feasibility data

    PubMed Central

    2013-01-01

    Background Infections are the leading cause of death in the acute phase following spinal cord injury and qualify as independent risk factor for poor neurological outcome (“disease modifying factor”). The enhanced susceptibility for infections is not stringently explained by the increased risk of aspiration in tetraplegic patients, neurogenic bladder dysfunction, or by high-dose methylprednisolone treatment. Experimental and clinical pilot data suggest that spinal cord injury disrupts the balanced interplay between the central nervous system and the immune system. The primary hypothesis is that the Spinal Cord Injury-induced Immune Depression Syndrome (SCI-IDS) is 'neurogenic’ including deactivation of adaptive and innate immunity with decreased HLA-DR expression on monocytes as a key surrogate parameter. Secondary hypotheses are that the Immune Depression Syndrome is i) injury level- and ii) severity-dependent, iii) triggers transient lymphopenia, and iv) causes qualitative functional leukocyte deficits, which may endure the post-acute phase after spinal cord injury. Methods/Design SCIentinel is a prospective, international, multicenter study aiming to recruit about 118 patients with acute spinal cord injury or control patients with acute vertebral fracture without neurological deficits scheduled for spinal surgery. The assessment points are: i) <31 hours, ii) 31–55 hours, iii) 7 days, iv) 14 days, and v) 10 weeks post-trauma. Assessment includes infections, concomitant injury, medication and neurological classification using American Spinal Injury Association impairment scale (AIS) and neurological level. Laboratory analyses comprise haematological profiling, immunophenotyping, including HLA-DR expression on monocytes, cytokines and gene expression of immune modulators. We provide an administrative interim analysis of the recruitment schedule of the trial. Discussion The objectives are to characterize the dysfunction of the innate and adaptive immune

  19. NADPH oxidase 4 attenuates cerebral artery changes during the progression of Marfan syndrome.

    PubMed

    Onetti, Yara; Meirelles, Thayna; Dantas, Ana P; Schröder, Katrin; Vila, Elisabet; Egea, Gustavo; Jiménez-Altayó, Francesc

    2016-05-01

    Marfan syndrome (MFS) is a connective tissue disorder that is often associated with the fibrillin-1 (Fbn1) gene mutation and characterized by cardiovascular alterations, predominantly ascending aortic aneurysms. Although neurovascular complications are uncommon in MFS, the improvement in Marfan patients' life expectancy is revealing other secondary alterations, potentially including neurovascular disorders. However, little is known about small-vessel pathophysiology in MFS. MFS is associated with hyperactivated transforming growth factor (TGF)-β signaling, which among numerous other downstream effectors, induces the NADPH oxidase 4 (Nox4) isoform of NADPH oxidase, a strong enzymatic source of H2O2 We hypothesized that MFS induces middle cerebral artery (MCA) alterations and that Nox4 contributes to them. MCA properties from 3-, 6-, or 9-mo-old Marfan (Fbn1(C1039G/+)) mice were compared with those from age/sex-matched wild-type littermates. At 6 mo, Marfan compared with wild-type mice developed higher MCA wall/lumen (wild-type: 0.081 ± 0.004; Marfan: 0.093 ± 0.002; 60 mmHg; P < 0.05), coupled with increased reactive oxygen species production, TGF-β, and Nox4 expression. However, wall stiffness and myogenic autoregulation did not change. To investigate the influence of Nox4 on cerebrovascular properties, we generated Marfan mice with Nox4 deficiency (Nox4(-/-)). Strikingly, Nox4 deletion in Marfan mice aggravated MCA wall thickening (cross-sectional area; Marfan: 6,660 ± 363 μm(2); Marfan Nox4(-/-): 8,795 ± 824 μm(2); 60 mmHg; P < 0.05), accompanied by decreased TGF-β expression and increased collagen deposition and Nox1 expression. These findings provide the first evidence that Nox4 mitigates cerebral artery structural changes in a murine model of MFS.

  20. Obesity hypoventilation syndrome in Japan and independent determinants of arterial carbon dioxide levels.

    PubMed

    Harada, Yuka; Chihara, Yuichi; Azuma, Masanori; Murase, Kimihiko; Toyama, Yoshiro; Yoshimura, Chikara; Oga, Toru; Nakamura, Hiroshi; Mishima, Michiaki; Chin, Kazuo

    2014-11-01

    Obesity hypoventilation syndrome (OHS) prevalence was previously estimated at 9% in patients with obstructive sleep apnoea (OSA) in Japan. However, the definition of OSA in that study was based on an apnoea-hypopnoea index (AHI) of ≥ 20/h rather than ≥ 5/h. Therefore, the prevalence of OHS in OSA was not measured in the same way as for Western countries. Our study objectives were to investigate the characteristics of Japanese patients with OHS. Nine hundred eighty-one consecutive patients investigated for suspected OSA were enrolled. At least 90% of them were from urban areas, including 162 with obese OSA (body mass index (BMI) ≥ 30 kg/m(2) and AHI ≥ 5/h). The prevalence of OHS (BMI 36.7 ± 4.9 kg/m(2) ) in OSA and that in obese OSA were 2.3% and 12.3%, respectively. Multiple regression analysis revealed that independent of age and BMI, arterial oxygen pressure (contribution rate (R(2) ) = 7.7%), 4% oxygen desaturation index (R(2)  = 8.9%), carbon monoxide diffusing capacity/alveolar volume (R(2)  = 8.3%), haemoglobin concentration (R(2)  = 4.9%) and waist circumference (R(2)  = 4.9%) were independently associated with arterial carbon dioxide pressure. After 12.3 ± 4.6 months of CPAP treatment, more than 60% of OHS patients no longer had hypercapnia. The prevalence of OHS in OSA in Japan was 2.3%. The mean BMI of patients with OHS in Japan was lower than that in Western countries (36.7 kg/m(2) vs 44.0 kg/m(2) ). © 2014 Asian Pacific Society of Respirology.

  1. Nonthyroidal illness syndrome in off-pump coronary artery bypass grafting.

    PubMed

    Cerillo, Alfredo Giuseppe; Sabatino, Laura; Bevilacqua, Stefano; Farneti, Pier Andrea; Scarlattini, Maria; Forini, Francesca; Glauber, Mattia

    2003-01-01

    Cardiopulmonary bypass (CPB) is an established cause of nonthyroidal illness syndrome (NTIS). Off-pump coronary artery bypass (OPCAB) has been reported to be less invasive than coronary artery bypass grafting (CABG) with CPB. We prospectively evaluated thyroid metabolism in OPCAB patients. We analyzed free thyroid hormones (FT3 and FT4), thyroid-stimulating hormone (TSH), and reverse T3 (rT3) in 20 consecutive patients undergoing CABG surgery. Nine patients underwent CABG with CPB, and 11 underwent OPCAB. Blood samples were taken on admission, on the day of surgery (7:30 AM), after sternotomy, at the end of the operation, and at 2, 6, 12, 24, 36, 48, 72, 96, 120, and 144 hours postoperatively. The concentrations of FT3, FT4, and TSH were determined on each sample. Reverse T3 concentration was measured in 10 patients up to 48 hours and at 144 hours postoperatively. Baseline, operative, and postoperative variables were similar in the two groups. FT3 concentration dropped significantly (p < 0.0001), reaching its lowest value 12 hours postoperatively. There were no significant differences between CPB and OPCAB patients. FT4 varied significantly in both groups (p < 0.0001), but remained in the normal range. TSH variation was not significant. rT3 concentration rose significantly (p = 0.0002) in both groups, peaking 24 hours after surgery. CONCLUSIONS. OPCAB induces a NTIS similar to that observed after CPB, probably due to the inhibition of T4 conversion to T3. This finding suggests that NTIS is a nonspecific response to stress. CPB should not be considered as the sole trigger of NTIS in cardiac surgical patients.

  2. Carotid artery intima-media thickness and erectile dysfunction in patients with metabolic syndrome

    PubMed Central

    Unal, Mustafa; Aksoy, Duygu Yazgan; Aydın, Yusuf; Tanriover, Mine Durusu; Berker, Dilek; Karakaya, Jale; Guler, Serdar

    2014-01-01

    Background Metabolic syndrome (MS) has become a pandemic in Turkey, as is the case globally. Increase in carotid artery intima-media thickness (CIMT) and erectile dysfunction (ED) may be evident before the clinical signs of cardiovascular disease appear. We aimed to investigate the prevalence of increased CIMT and ED as markers of atherosclerotic disease in patients with MS. Material/Methods Thirty-two patients with MS and 29 healthy controls were included. Anthropometric and biochemical parameters, along with total testosterone (TT), high sensitive C-reactive protein (hs-CRP), were recorded. Carotid artery intima-media thickness was measured. Erectile dysfunction was assessed with International Index of Erectile Function. Results Patients with MS had higher BMI, fasting plasma glucose, post-prandial plasma glucose, insulin, HOMA-IR, total cholesterol, triglycerides, hs-CRP, and CIMT, whereas TT levels were lower (p<0.0001). The prevalence and severity of erectile dysfunction were higher in patients with MS (p<0.0001). Erectile dysfunction scores correlated inversely with CIMT. MS patients with ED were older and had higher CIMT compared to those without ED. Increase in age and HOMA and decrease in TT increased the risk of ED. When KIMT exceeding the 95th percentile of healthy controls was accepted as a risk factor for CVD, presence of ED was the only determinant for this increase. Conclusions Erectile dysfunction was more prevalent and severe in patients with MS and correlated with subclinical endothelial dysfunction. Total testosterone deficiency was prominent among MS patients. Presence of ED points to an increased risk of cardiovascular disease when MS is present. PMID:24869934

  3. Central Hypoventilation Syndrome Complicated with Lateral Medullary Infarction after Endovascular Treatment of the Vertebral Artery Dissecting Aneurysm: A Case Report.

    PubMed

    Tanaka, Katsuhiro; Kanamaru, Hideki; Morikawa, Atsunori; Kawaguchi, Kenji

    2016-10-01

    Lateral medullary infarction rarely leads to central hypoventilation syndrome (CHS). CHS is a life-threatening disorder characterized by hypoventilation during sleep. We report the first case of CHS as a complication of lateral medullary infarction after endovascular treatment. A 65-year-old man presented twice with severe headache. Computed tomography revealed subarachnoid hemorrhage and cerebral angiography showed a right vertebral dissecting aneurysm involving the posterior inferior cerebellar artery. After emergent endovascular patent artery occlusion, he developed Wallenberg syndrome and experienced apnea and a conscious disturbance episode due to CHS on postoperative days 6 and 16. Intensive respiratory care including intubation, tracheostomy, mechanical ventilation, and rehabilitation prevented subsequent recurrence of apnea and the CHS resolved completely. CHS after unilateral medullary infarction involving respiratory centers tends to occur in the acute and subacute phase and may be lethal without careful respiratory management.

  4. Ambulatory (24 h) blood pressure and arterial stiffness measurement in Marfan syndrome patients: a case control feasibility and pilot study.

    PubMed

    Hillebrand, Matthias; Nouri, Ghazaleh; Hametner, Bernhard; Parragh, Stephanie; Köster, Jelena; Mortensen, Kai; Schwarz, Achim; von Kodolitsch, Yskert; Wassertheurer, Siegfried

    2016-05-06

    The aim of this work is the investigation of measures of ambulatory brachial and aortic blood pressure and indices of arterial stiffness and aortic wave reflection in Marfan patients. A case-control study was conducted including patients with diagnosed Marfan syndrome following Ghent2 nosology and healthy controls matched for sex, age and daytime brachial systolic blood pressure. For each subject a 24 h ambulatory blood pressure and 24 h pulse wave analysis measurement was performed. All parameters showed a circadian pattern whereby pressure dipping was more pronounced in Marfan patients. During daytime only Marfan patients with aortic root surgery showed increased pulse wave velocity. In contrast, various nighttime measurements, wave reflection determinants and circadian patterns showed a significant difference. The findings of our study provide evidence that ambulatory measurement of arterial stiffness parameters is feasible and that these determinants are significantly different in Marfan syndrome patients compared to controls in particular at nighttime. Further investigation is therefore indicated.

  5. Gender differences in patients with metabolic syndrome in coronary artery interventions.

    PubMed

    Rami Helvaci, Mehmet; Kaya, Hasan; Gundogdu, Mehmet

    2013-02-01

    We tried to understand whether or not there was a gender difference in coronary artery interventions in coronary heart disease (CHD) cases in the present study. The study was performed in two phases. The first phase was performed at the Internal Medicine Polyclinic of the Dumlupinar University between August 2005 and March 2007. CHD was diagnosed either angiographically or with history of coronary artery stenting (CAS) and/or coronary artery bypass graft (CABG) surgery. The second phase was performed at the Internal Medicine Polyclinic of the Mustafa Kemal University between March 2007 and April 2012. During the second phase, the CHD patients with CAS and/or CABG surgery were detected and divided into two groups according to the gender. Mean age and prevalence of CHD were similar in both genders (p>0.05 for both) in the first phase. Smoking was higher in males with CHD, in 30 cases (54.5%) of males versus six (9.6%) cases of females (p < 0.001), as well as chronic obstructive pulmonary disease (COPD), in ten (18.1%) cases of males versus four (6.4%) cases of females (p < 0.05). Although the body mass index (BMI) and white coat hypertension (WCH) were insignificantly higher (p>0.05 for both), low density lipoprotein cholesterol (LDL-C) and triglyceride (TG) were significantly higher in females with CHD (p= 0.008 and p= 0.002, respectively). Hypertension (HT) and diabetes mellitus (DM) were higher in females with CHD, too (p < 0.001 and p < 0.05, respectively). On the other hand, CAS and/or CABG surgery were significantly higher in male CHD cases (21.8% versus 1.6%, p less 0.001). Parallel to the first phase cases, majority of CAS and/or CABG surgery cases were males in the second phase cases too (90.2% versus 9.7%, p less 0.001). As some components of the metabolic syndrome, smoking and COPD were higher in males whereas BMI, WCH, LDL-C, TG, HT and DM were higher in females. Despite similar prevalences of CHD in both sexes, CAS and/or CABG surgery were significantly

  6. Tibioperoneal Trunk Aneurysm Resulting in Compartment Syndrome with Associated Aneurysms of the Popliteal and Dorsalis Pedis Arteries.

    PubMed

    Ventarola, Daniel J; Labropoulos, Nicos N; Landau, David S; Tassiopoulos, Apostolos K; Loh, Shang A

    2016-08-01

    True aneurysms of the tibioperoneal trunk are rare. Given the scarcity of reports, the clinical presentation and treatment is not well defined. This is a case report of a 50-year-old male patient presenting with severe lower extremity swelling and compartment syndrome with neurological compromise secondary to a tibioperoneal trunk aneurysm. He was also noted to have discrete ipsilateral popliteal and dorsalis pedis artery aneurysms. Given the location and size of the aneurysm, the severe leg swelling, and venous hypertension, aneurysmorrhaphy or aneurysm sac excision with arterial reconstruction was prohibitively dangerous. Thus, following fasciotomies, a hybrid repair utilizing a saphenous vein superficial femoral to anterior tibial artery bypass along with coil embolization of the aneurysm sac was performed. The patient recovered full function of his leg and follow-up computed tomography angiogram demonstrated thrombosis and regression of the aneurysm sac with a patent bypass.

  7. Molecular and functional characterization of Kv 7 channels in penile arteries and corpus cavernosum of healthy and metabolic syndrome rats.

    PubMed

    Jepps, T A; Olesen, S P; Greenwood, I A; Dalsgaard, T

    2016-05-01

    KCNQ-encoded voltage-dependent potassium channels (Kv 7) are involved in the regulation of vascular tone. In this study we evaluated the influence of Kv 7 channel activation on smooth muscle relaxation in rat penile arteries and corpus cavernosum from normal and spontaneously hypertensive, heart failure-prone (SHHF) rats - a rat model of human metabolic syndrome. Quantitative PCR and immunohistochemistry were used to determine the expression of KCNQ isoforms in penile tissue. Isometric tension was measured in intracavernous arterial rings and corpus cavernosum strips isolated from normal and SHHF rats. Transcripts for KCNQ3, KCNQ4 and KCNQ5 were detected in penile arteries and corpus cavernosum. KCNQ1 was only found in corpus cavernosum. Immunofluorescence signals to Kv 7.4 and Kv 7.5 were found in penile arteries, penile veins and corpus cavernosum. The Kv 7.2-7.5 activators, ML213 and BMS204352, relaxed pre-contracted penile arteries and corpus cavernosum independently of nitric oxide synthase or endothelium-derived hyperpolarization. Relaxations to sildenafil, a PDE5 inhibitor, and sodium nitroprusside (SNP), an nitric oxide donor, were reduced by blocking Kv 7 channels with linopirdine in penile arteries and corpus cavernosum. In SHHF rat penile arteries and corpus cavernosum, relaxations to ML213 and BMS204352 were attenuated, and the blocking effect of linopirdine on sildenafil-induced and SNP-induced relaxations reduced. KCNQ3, KCNQ4 and KCNQ5 were down-regulated, and KCNQ1 was up-regulated in corpus cavernosum from SHHF rats. KCNQ1-5 transcripts remained unchanged in penile arteries from SHHF rats. These data suggest that Kv 7 channels play a role in erectile function and contribute to the pathophysiology of erectile dysfunction, an early indicator of cardiovascular disease. © 2016 The British Pharmacological Society.

  8. Molecular and functional characterization of Kv7 channels in penile arteries and corpus cavernosum of healthy and metabolic syndrome rats

    PubMed Central

    Jepps, T A; Olesen, S P; Greenwood, I A

    2016-01-01

    Background and Purpose KCNQ‐encoded voltage‐dependent potassium channels (Kv7) are involved in the regulation of vascular tone. In this study we evaluated the influence of Kv7 channel activation on smooth muscle relaxation in rat penile arteries and corpus cavernosum from normal and spontaneously hypertensive, heart failure‐prone (SHHF) rats – a rat model of human metabolic syndrome. Experimental Approach Quantitative PCR and immunohistochemistry were used to determine the expression of KCNQ isoforms in penile tissue. Isometric tension was measured in intracavernous arterial rings and corpus cavernosum strips isolated from normal and SHHF rats. Key Results Transcripts for KCNQ3, KCNQ4 and KCNQ5 were detected in penile arteries and corpus cavernosum. KCNQ1 was only found in corpus cavernosum. Immunofluorescence signals to Kv7.4 and Kv7.5 were found in penile arteries, penile veins and corpus cavernosum. The Kv7.2–7.5 activators, ML213 and BMS204352, relaxed pre‐contracted penile arteries and corpus cavernosum independently of nitric oxide synthase or endothelium‐derived hyperpolarization. Relaxations to sildenafil, a PDE5 inhibitor, and sodium nitroprusside (SNP), an nitric oxide donor, were reduced by blocking Kv7 channels with linopirdine in penile arteries and corpus cavernosum. In SHHF rat penile arteries and corpus cavernosum, relaxations to ML213 and BMS204352 were attenuated, and the blocking effect of linopirdine on sildenafil‐induced and SNP‐induced relaxations reduced. KCNQ3, KCNQ4 and KCNQ5 were down‐regulated, and KCNQ1 was up‐regulated in corpus cavernosum from SHHF rats. KCNQ1–5 transcripts remained unchanged in penile arteries from SHHF rats. Conclusions and Implications These data suggest that Kv7 channels play a role in erectile function and contribute to the pathophysiology of erectile dysfunction, an early indicator of cardiovascular disease. PMID:26802314

  9. Mild moxibustion decreases the expression of prokineticin 2 and prokineticin receptor 2 in the colon and spinal cord of rats with irritable bowel syndrome.

    PubMed

    Zhou, Cili; Zhao, Jimeng; Wu, Luyi; Huang, Renjia; Shi, Yin; Wang, Xiaomei; Liao, Wen; Hong, Jue; Liu, Shimin; Wu, Huangan

    2014-01-01

    It has been proven that prokineticin 2 (PK2) and its receptor PKR2 play an important role in hyperalgesia, while mild moxibustion can relieve visceral hypersensitivity in a rat model of irritable bowel syndrome (IBS). The goal of the present study was to determine the effects of mild moxibustion on the expression of PK2 and PKR2 in colon and spinal cord in IBS rat model, which was induced by colorectal distension using inflatable balloons. After mild moxibustion treatment, abdominal withdrawal reflex (AWR) scores were assessed by colorectal distension; protein and mRNA expression of PK2 and PKR2 in rat colon and spinal cord was determined by immunohistochemistry and fluorescence quantitative PCR. Compared with normal rats, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly increased in the model group; compared with the model group, the AWR scores of rats and the expressions of PK2/PKR2 proteins and mRNAs in colon and spinal cord tissue were significantly decreased in the mild moxibustion group. These findings suggest that the analgesia effect of mild moxibustion may be associated with the reduction of the abnormally increased expression of the PK2/PKR2 proteins and mRNAs in the colon and spinal cord.

  10. Propranolol Use in PHACE Syndrome with Cervical and Intracranial Arterial Anomalies: Collective Experience in 32 Infants

    PubMed Central

    Metry, Denise; Frieden, Ilona J.; Hess, Christopher; Siegel, Dawn; Maheshwari, Mohit; Baselga, Eulalia; Chamlin, Sarah; Garzon, Maria; Mancini, Anthony J.; Powell, Julie; Drolet, Beth A.

    2016-01-01

    1. Objective Combine collective clinical experience using oral propranolol therapy in PHACE syndrome infants with cerebrovascular anomalies. 2. Design Retrospective study of patients evaluated between July 2008 and October 2011. 3. Setting Seven pediatric dermatology centers. 4. Patients 32 infants with definite PHACE syndrome and cervical and/or intracranial arterial anomalies. 5. Intervention Oral propranolol: average dose of 1.8 mg/kg/day divided t.i.d. or b.i.d., for an average duration of 12.3 months. 6. Main Outcome Measure Adverse neurologic events. 7. Results 7/32 (22%) patients were categorized as higher-risk for stroke, defined on MRA as severe, long-segment narrowing or non-visualization of major cerebral or cervical vessels without anatomic evidence for collateral circulation, often in the presence of concomitant cardiovascular comorbidities. Only 1 patient developed a change in neurologic status during propranolol treatment: a mild right hemiparesis that remained static and improved while propranolol was continued. An additional 3 patients had worsening hemangioma ulceration and/or tissue necrosis during therapy. 8. Conclusions This is the largest report thus far of PHACE patients treated with propranolol. While no catastrophic neurologic events occurred, serious complications, particularly severe ulcerations were seen in a minority of patients, and given the sample size we cannot negate the possibility that propranolol could augment the risk of stroke in this population. We continue to advise caution in using systemic beta-blockers, particularly for children with vascular anomalies at higher risk for stroke. Use of the lowest possible dosage, slow dosage titration, and t.i.d. dosing in order to minimize abrupt changes in blood pressure and close follow-up, including neurologic consultation as needed, are recommended. PMID:22994362

  11. DIVERGENCE BETWEEN ARTERIAL PERFUSION AND FATIGUE RESISTANCE IN SKELETAL MUSCLE IN THE METABOLIC SYNDROME

    PubMed Central

    Frisbee, Jefferson C.; Goodwill, Adam G.; Butcher, Joshua T.; Olfert, I. Mark

    2010-01-01

    The metabolic syndrome is associated with elevated peripheral vascular disease risk, characterized by mismatched blood flow delivery/distribution and local metabolism. The obese Zucker rat (OZR) model of the metabolic syndrome exhibits myriad vascular impairments, although their integrated impact on functional hyperemia remains unclear. In this study, arterial pressor responses and skeletal muscle perfusion were assessed in lean Zucker rats (LZR) and OZR during adrenergic stimulation (phenylephrine), challenge with thromboxane (U46619) and endothelium-dependent dilation (methacholine). OZR were hypertensive versus LZR, but this was abolished by adrenoreceptor blockade (phentolamine); pressor responses to U46619 were similar between strains and were abolished by blockade with the PGH2/TxA2 receptor antagonist, SQ-29548. Depressor reactivity to methacholine was impaired in OZR, but was improved by antioxidant treatment (TEMPOL). Across levels of metabolic demand, blood flow to in situ gastrocnemius muscle was restrained by adrenergic constriction in OZR, although this diminished with increased demand. O2 extraction, reduced in OZR vs. LZR across levels of metabolic demand, was improved by TEMPOL or SQ-29548; treatment with phentolamine did not impact extraction and neither TEMPOL nor SQ-29548 improved muscle blood flow in OZR. While VO2 and muscle performance were consistently reduced in OZR vs. LZR, treatment with all three agents improved outcomes, while treatment with individual agents was less effective. These results suggest that contributions of vascular dysfunction to perfusion, VO2 and muscle performance are spatially distinct, with adrenergic constriction impacting proximal resistance and endothelial dysfunction impacting distal microvessel-tissue exchange. Further, these data suggest that increasing skeletal muscle blood flow in OZR is not sufficient to improve performance, unless distal perfusion inhomogeneities are rectified. PMID:21123363

  12. Control of hypereosinophilic syndrome-associated recalcitrant coronary artery spasm by combined treatment with prednisone, imatinib mesylate and hydroxyurea

    PubMed Central

    Butterfield, Joseph H; Sharkey, Scott W

    2006-01-01

    Uncontrolled hypereosinophilic syndrome is frequently associated with cardiovascular consequences that cause significant morbidity and mortality. The present article reports on a patient with hypereosinophilic syndrome in whom recurrent, recalcitrant coronary artery spasm and associated cardiac arrest were the predominant cardiac manifestations. No valvular abnormalities, evidence of mural thrombi or other cardiac findings commonly associated with hypereosinophilic syndrome were detected, and cardiac function remained normal. The serum tryptase level was normal, cysteine-rich hydrophobic domain 2 (CHIC2) deletion analysis of bone marrow cells was negative and no evidence of mastocytosis or other hematological disorder was found in the bone marrow. To allow for the reduction of prednisone, interferon-alpha-2b was added to the patient’s program, but caused aggravation of chest pain and was discontinued. However, a combination of reduced prednisone dosage, imatinib mesylate and hydroxyurea successfully controlled the eosinophilia, and thereafter, episodes of coronary artery spasm did not recur. The clinical features of the present case suggest that, in some patients, hypereosinophilia may manifest as resistant coronary artery spasm and that aggressive control of eosinophilia is necessary. PMID:18651015

  13. Functional magnetic resonance imaging of cerebral activation during spinal cord stimulation in failed back surgery syndrome patients.

    PubMed

    Stancák, Andrej; Kozák, Jirí; Vrba, Ivan; Tintera, Jaroslav; Vrána, Jirí; Polácek, Hubert; Stancák, Marián

    2008-02-01

    Spinal cord stimulation (SCS) consisting of electrical stimulation of the dorsal spinal cord using epidural electrodes has been shown to relieve chronic neuropathic pain. To analyze the cerebral activation patterns related to SCS, and to evaluate the effects of SCS on the processing of acute experimental pain, we performed functional magnetic resonance imaging (fMRI) on eight patients suffering from failed back surgery syndrome who were also being treated with SCS for severe pain in their legs and lower back. Three types of stimulation were used, each lasting 36s: (i) SCS, (ii) heat pain (HP) applied to the leg affected by neuropathic pain, and (iii) simultaneous HP and SCS. During SCS, we found increased activation of the medial primary sensorimotor cortex somatotopically corresponding to the foot and/or perineal region, contralateral posterior insula, and the ipsilateral secondary somatosensory cortex (S2). Decreased activation was seen in the bilateral primary motor cortices and the ipsilateral primary somatosensory cortex corresponding to the shoulder, elbow and hand. Compared to separately presented HP and SCS, simultaneous HP and SCS showed statistically significant activation of the bilateral inferior temporal cortex and the ipsilateral cerebellar cortex. The activation of the primary motor cortex, insula and S2 during SCS may directly interfere with the processing of neuropathic pain. When SCS is associated with heat pain, the paralimbic association cortex and cerebellum show activation exceeding the sum of activations resulting from separate SCS and heat pain stimulation. The explanation of this could possibly rest with the continuous comparisons of simultaneous pain and somatosensory sensations occurring in a single dermatome.

  14. PUF60 variants cause a syndrome of ID, short stature, microcephaly, coloboma, craniofacial, cardiac, renal and spinal features.

    PubMed

    Low, Karen J; Ansari, Morad; Abou Jamra, Rami; Clarke, Angus; El Chehadeh, Salima; FitzPatrick, David R; Greenslade, Mark; Henderson, Alex; Hurst, Jane; Keller, Kory; Kuentz, Paul; Prescott, Trine; Roessler, Franziska; Selmer, Kaja K; Schneider, Michael C; Stewart, Fiona; Tatton-Brown, Katrina; Thevenon, Julien; Vigeland, Magnus D; Vogt, Julie; Willems, Marjolaine; Zonana, Jonathan; Study, D D D; Smithson, Sarah F

    2017-03-22

    PUF60 encodes a nucleic acid-binding protein, a component of multimeric complexes regulating RNA splicing and transcription. In 2013, patients with microdeletions of chromosome 8q24.3 including PUF60 were found to have developmental delay, microcephaly, craniofacial, renal and cardiac defects. Very similar phenotypes have been described in six patients with variants in PUF60, suggesting that it underlies the syndrome. We report 12 additional patients with PUF60 variants who were ascertained using exome sequencing: six through the Deciphering Developmental Disorders Study and six through similar projects. Detailed phenotypic analysis of all patients was undertaken. All 12 patients had de novo heterozygous PUF60 variants on exome analysis, each confirmed by Sanger sequencing: four frameshift variants resulting in premature stop codons, three missense variants that clustered within the RNA recognition motif of PUF60 and five essential splice-site (ESS) variant. Analysis of cDNA from a fibroblast cell line derived from one of the patients with an ESS variants revealed aberrant splicing. The consistent feature was developmental delay and most patients had short stature. The phenotypic variability was striking; however, we observed similarities including spinal segmentation anomalies, congenital heart disease, ocular colobomata, hand anomalies and (in two patients) unilateral renal agenesis/horseshoe kidney. Characteristic facial features included micrognathia, a thin upper lip and long philtrum, narrow almond-shaped palpebral fissures, synophrys, flared eyebrows and facial hypertrichosis. Heterozygote loss-of-function variants in PUF60 cause a phenotype comprising growth/developmental delay and craniofacial, cardiac, renal, ocular and spinal anomalies, adding to disorders of human development resulting from aberrant RNA processing/spliceosomal function.European Journal of Human Genetics advance online publication, 22 March 2017; doi:10.1038/ejhg.2017.27.

  15. Spinal Fusion for Scoliosis in Rett Syndrome With an Emphasis on Respiratory Failure and Opioid Usage.

    PubMed

    Rumbak, Dania M; Mowrey, Wenzhu; W Schwartz, Skai; Sarwahi, Vishal; Djukic, Aleksandra; Killinger, James S; Katyal, Chhavi

    2016-02-01

    Our objective was to characterize our experience with 8 patients with Rett syndrome undergoing scoliosis surgery in regard to rates of respiratory failure and rates of ventilator-acquired pneumonia in comparison to patients with neurologic scoliosis and adolescent idiopathic scoliosis. This study was a retrospective chart review of patients undergoing scoliosis surgery at a tertiary children's hospital. Patients were divided into 3 groups: (1) adolescent idiopathic scoliosis, (2) neurologic scoliosis, and (3) Rett syndrome. There were 133 patients with adolescent idiopathic scoliosis, 48 patients with neurologic scoliosis, and 8 patients with Rett syndrome. We found that patients with Rett syndrome undergoing scoliosis surgery have higher rates of respiratory failure and longer ventilation times in the postoperative period when compared with both adolescent idiopathic scoliosis and neurologic scoliosis patients. There is insufficient evidence to suggest a difference in the incidence of ventilator-acquired pneumonia between the Rett syndrome and the neurologic scoliosis group. We believe our findings are the first in the literature to show a statistically significant difference between these 3 groups in regard to incidence of respiratory failure.

  16. Pulmonary Arterial Hypertension Associated with Congenital Heart Disease and Eisenmenger Syndrome: Current Practice in Pediatrics

    PubMed Central

    Frank, David B.; Hanna, Brian D.

    2015-01-01

    Pulmonary arterial hypertension (PAH) is an uncommon but serious disease characterized by severe pulmonary vascular disease and significant morbidity and mortality. PAH associated with congenital heart disease (APAH-CHD) is one etiology of PAH that has innate characteristics delineating it from other forms of PAH. The patient with APAH-CHD presents with unique challenges consisting of not only pulmonary vascular disease but also the complexity of the cardiac lesion. Eisenmenger syndrome (ES) represents the severe end of the spectrum for disease in APAH-CHD. Over time, systemic-to-pulmonary shunting through cardiac defects increases pulmonary vascular resistance to levels significant enough to reverse shunting across the defect. Historically, ES patients have been reported to have better outcomes than IPAH despite similarities in pulmonary vascular disease. However, recent studies are challenging this notion. Nonetheless, APAH-CHD survival has improved with the advent of modern PAH targeted therapies. New therapeutic options have allowed us to reconsider the dogma of inoperability in APAH-CHD patients with unrepaired defects. Certainly advances have been made, however, investigators must continue to advance the field through controlled clinical trials in both adult and pediatric APAH-CHD patients. PMID:25604592

  17. Peripheral Arterial Disease in Patients Presenting with Acute Coronary Syndrome in Six Middle Eastern Countries

    PubMed Central

    Al-Thani, Hassan A.; El-Menyar, Ayman; Zubaid, Mohammad; Rashed, Wafa A.; Ridha, Mustafa; Almahmeed, Wael; Sulaiman, Kadhim; Al-Motarreb, Ahmed; Amin, Haitham; Al Suwaidi, Jassim

    2011-01-01

    To describe prevalence and impact of peripheral arterial disease (PAD) in patients with acute coronary syndrome (ACS), data were collected over 5 months from 6 Middle Eastern countries. Patients were divided into 2 groups (with and without PAD). Out of 6705 consecutive ACS patients, PAD was reported in 177 patients. In comparison to non-PAD, PAD patients were older and more likely to have cardiovascular risk factors. They were more likely to have high Killip class, high GRACE risk score, and non-ST elevation ACS (NSTEACS) at presentation. Thrombolytics, antiplatelet use, and coronary intervention were comparable in both groups. When presented with ST-elevation myocardial infarction (STEMI), patients with PAD had worse outcomes, while in NSTEACS; PAD was associated with higher rate of heart failure in comparison to non-PAD patients. In diabetics, PAD was associated with 2-fold increase in mortality when compared to non-PAD (P = 0.028). After adjustment, PAD was associated with high mortality in STEMI (adjusted OR 2.6; 95% CI 1.23–5.65, P = 0.01). Prevalence of PAD in ACS in the Gulf region is low. Patients with PAD and ACS constitute a high risk group and require more attention. PAD in patients with STEMI is an independent predictor of in-hospital death. PMID:22220279

  18. Arterial hypertension during treatment with triptorelin in a child with Williams-Beuren syndrome.

    PubMed

    Siomou, Ekaterini; Kosmeri, Chrysoula; Pavlou, Maria; Vlahos, Antonios P; Argyropoulou, Maria I; Siamopoulou, Antigoni

    2014-09-01

    Arterial hypertension (AHT) is a common finding in children with Williams-Beuren syndrome (WBS). Although cardiovascular and renal abnormalities can explain the AHT in some patients with WBS, its etiology is not fully understood and most cases are considered idiopathic. The case is reported of a 10-year-old girl with WBS who developed severe AHT during treatment with triptorelin, a long-lasting gonadotropin-releasing hormone (GnRH) analog, administered because of early normal puberty. Comprehensive diagnostic studies ruled out other known causes of AHT associated with WBS. After discontinuation of triptorelin, the blood pressure remained within the normal range for her age and height with no antihypertensive treatment on long-term follow-up. To the best of the authors' knowledge, this is the first report of AHT associated with triptorelin administration in a child with WBS. Clinicians should be aware of the possibility, although rare, of AHT developing during triptorelin administration in childhood, specifically in patients at increased risk of AHT, such as those with WBS.

  19. Anxiety Level and Cardiac Autonomic Modulations in Coronary Artery Disease and Cardiac Syndrome X Patients.

    PubMed

    Lutfi, Mohamed Faisal

    2017-01-01

    Anxiety and cardiac autonomic modulations (CAM) were thoroughly investigated in coronary artery disease (CAD) and cardiac syndrome X (CSX) patients worldwide, but not among Sudanese with similar pathology. To compare levels of anxiety and CAM between Sudanese patients with CSX and CAD. Anxiety was evaluated in 51 CAD and 26 CSX patients using Taylor Manifest anxiety score (TMAS) questionnaire while heart rate variability derived indices were used to assess CAM, namely natural logarithm of low frequency (LnLF), high frequency (LnHF) and LF/HF ratio (LnLF/HF). Low anxiety levels were achieved by 6 (23.1%) and 9 (17.6%) patients with CSX and CAD respectively. High anxiety level was achieved by only one (3.8%) patient, who was suffering from CSX. TMAS was significantly higher in CSX (31.27 (21.97)) compared to CAD (21.86 (12.97), P = 0.021). However, abnormally increased anxiety was not associated with higher risk of CSX. LnLF, LnHF and LnLF/HF were comparable in CAD and CSX patients. CSX and CAD patients showed comparable CAM. Although anxiety levels were higher in CSX compared to CAD, TMAS ≥ 35 failed to show significant association with CSX.

  20. Bow hunter's syndrome unmasked after contralateral vertebral artery sacrifice for aneurysmal subarachnoid hemorrhage.

    PubMed

    Ikeda, Daniel S; Villelli, Nicolas; Shaw, Andrew; Powers, Ciarán

    2014-06-01

    Bow hunter's syndrome (BHS), or positional vertebrobasilar insufficiency, is a rare disorder characterized by positional stenosis or occlusion of the dominant vertebral artery (VA) with concordant signs and symptoms of brainstem ischemia. Many etiologies have been described, with the most common level of obstruction occurring at the craniocervical junction. The authors report acquired BHS after the patient's contralateral VA was sacrificed for ruptured aneurysm. A 44-year-old man was referred to our institution for years of continued positional tinnitus, vertigo, and nausea. This symptomatology began 5 years prior, immediately after the patient's right VA was endovascularly sacrificed to treat a ruptured VA aneurysm. From the time of treatment, right head turning caused instantaneous symptoms consistent with brainstem ischemia. Evaluations performed during the 5 year interim before referral included computed tomography angiography, MRI, and diagnostic cerebral angiography (DCA). All failed to identify a causal etiology. A diagnosis of BHS was made with dynamic DCA. With the patient's head turned to the right, angiography of the left VA demonstrated a positional block. The patient underwent left VA decompression. Intraoperative dynamic DCA was utilized to demonstrate adequacy of decompression. The patient tolerated the procedure without complication. Symptomatology immediately completely subsided and remained absent at his 3 month follow-up. An acquired BHS should be recognized as a possible complication if VA sacrifice is considered for an unclippable or uncoilable aneurysm.

  1. Incident and recurrent major depressive disorder and coronary artery disease severity in acute coronary syndrome patients.

    PubMed

    Goodman, Jeanne; Shimbo, Daichi; Haas, Donald C; Davidson, Karina W; Rieckmann, Nina

    2008-07-01

    There is recent evidence that acute coronary syndrome (ACS) patients with first time incident major depressive disorder (MDD) and those with recurrent MDD represent different subtypes among individuals with ACS and comorbid depression. However, few studies have examined whether or not these subtypes differ in coronary artery disease (CAD) severity. We assessed whether those with incident MDD (in-hospital MDD and negative for history of MDD) or recurrent MDD (in-hospital MDD and a positive history of MDD) differ in angiographically documented CAD severity. Within 1 week of admission for ACS, 88 patients completed a clinical interview to assess current and past diagnosis of MDD. CAD severity was assessed in all patients by coronary angiography. A hierarchical regression analysis showed that neither in-hospital MDD status, nor history of MDD were significant predictors of CAD severity, but the interaction term between in-hospital MDD status and history of MDD was a significant predictor of CAD severity, after controlling for age, sex and ethnicity. Follow-up analyses showed that patients with first time, incident MDD had significantly more severe CAD compared to patients with recurrent MDD (p=0.043). To conclude, our study adds to the growing evidence that patients with incident MDD should be considered as a clinically distinct subtype from those with recurrent MDD. Possible mechanisms for differing CAD severity by angiogram between these two subtypes are proposed and implications for prognosis and treatment are discussed.

  2. Impact of Classical Risk Factors for Arterial or Venous Thrombosis in Patients With Antiphospholipid Syndrome.

    PubMed

    Navarro-Carpentieri, Daniel; Castillo-Hernandez, Maria Del Carmen; Majluf-Cruz, Karim; Espejo-Godinez, Guillermo; Carmona-Olvera, Paola; Moreno-Hernandez, Manuel; Lugo-García, Yolanda; Hernandez-Juarez, Jesús; Loarca-Piña, Luis; Isordia-Salas, Irma; Majluf-Cruz, Abraham

    2017-01-01

    There are classical risk factors associated with arterial thrombosis (AT) or venous thromboembolic disease (VTD). However, less is known about these risk factors and AT or VTD episodes in patients with antiphospholipid syndrome (APS). Our aim was to elucidate whether APS-related thrombotic episodes are associated with the same risk factors as the non-APS population. We gathered demographics, medical history, complications, and causes of death associated with the risk factors for AT or VTD in patients with APS. We analyzed 677 thrombotic events in 386 patients. Type 2 diabetes mellitus and grade 3 obesity were associated with VTD instead of AT. There were no significant differences between the groups for almost all laboratory tests analyzed, although lupus anticoagulant was significantly higher in the VTD group. We suggest that thrombosis in APS is due to the APS itself and that the risks factors for AT or VTD do not have a main role. Our findings may have an ethnical background. Therefore, it may be difficult to elaborate predictive thrombotic clinical scores applicable to patients with different ethnical background.

  3. Anterior ethmoidal artery emerging anterior to bulla ethmoidalis: An abnormal anatomical variation in Waardenburg's syndrome

    PubMed Central

    Wong, Danny K. C.; Shao, Angus; Campbell, Raewyn

    2014-01-01

    In endoscopic sinus surgery, the anterior ethmoidal artery (AEA) is usually identified as it traverses obliquely across the fovea ethmoidalis, posterior to the bulla ethmoidalis and anterior to or within the ground lamella's attachment to the skull base. Injury to the AEA may result in hemorrhage, retraction of the AEA into the orbit, and a retrobulbar hematoma. The resulting increase in intraorbital pressure may threaten vision. Waardenburg's syndrome (WS) is a rare congenital, autosomal dominantly inherited disorder, distinguished by characteristic facial features, pigmentation abnormalities, and profound, congenital, sensorineural hearing loss. We present a case of AEAs located anterior to the bulla ethmoidalis in a 36-year-old male with WS and chronic rhinosinusitis. The anatomic abnormality was not obvious on a preoperative computed tomography scan and was discovered intraoperatively when the left AEA was injured, resulting in a retrobulbar hematoma. The hematoma was immediately identified and decompressed endoscopically without lasting complications. The AEA on the right was identified intraoperatively and preserved. The characteristic craniofacial features in WS were probably associated with the abnormal vascular anatomy. Endoscopic sinus surgeons should be aware of these potential anatomic anomalies in patients with abnormal craniofacial development. PMID:25565054

  4. Thrombin generation in acute coronary syndrome and stable coronary artery disease: dependence on plasma factor composition.

    PubMed

    Brummel-Ziedins, K; Undas, A; Orfeo, T; Gissel, M; Butenas, S; Zmudka, K; Mann, K G

    2008-01-01

    Acute coronary syndrome (ACS) is associated with thrombin formation, triggered by ruptured or eroded coronary atheroma. We investigated whether thrombin generation based on circulating coagulation protein levels, could distinguish between acute and stable coronary artery disease (CAD). Plasma coagulation factor (F) compositions from 28 patients with ACS were obtained after onset of chest pain. Similar data were obtained from 25 age- and sex-matched patients with stable CAD. All individuals took aspirin. Patients on anticoagulant therapy were excluded. The groups were similar in demographic characteristics, comorbidities and concomitant treatment. Using each individual's coagulation protein composition, tissue factor (TF) initiated thrombin generation was assessed both computationally and empirically. TF pathway inhibitor (TFPI), antithrombin (AT), factor II (FII) and FVIII differed significantly (P < 0.01) between the groups, with levels of FII, FVIII and TFPI higher and AT lower in ACS patients. When thrombin generation profiles from individuals in each group were compared, simulated maximum thrombin levels (P < 0.01) and rates (P < 0.01) were 50% higher with ACS while the initiation phases of thrombin generation were shorter. Empirical reconstructions of the populations reproduced the thrombin generation profiles generated by the computational model. The differences between the thrombin generation profiles for each population were primarily dependent upon the collective contribution of AT, FII and FVIII. Simulations of thrombin formation based on plasma composition can discriminate between acute and stable CAD.

  5. Anxiety Level and Cardiac Autonomic Modulations in Coronary Artery Disease and Cardiac Syndrome X Patients

    PubMed Central

    2017-01-01

    Background Anxiety and cardiac autonomic modulations (CAM) were thoroughly investigated in coronary artery disease (CAD) and cardiac syndrome X (CSX) patients worldwide, but not among Sudanese with similar pathology. Aims To compare levels of anxiety and CAM between Sudanese patients with CSX and CAD. Materials and Methods Anxiety was evaluated in 51 CAD and 26 CSX patients using Taylor Manifest anxiety score (TMAS) questionnaire while heart rate variability derived indices were used to assess CAM, namely natural logarithm of low frequency (LnLF), high frequency (LnHF) and LF/HF ratio (LnLF/HF). Results Low anxiety levels were achieved by 6 (23.1%) and 9 (17.6%) patients with CSX and CAD respectively. High anxiety level was achieved by only one (3.8%) patient, who was suffering from CSX. TMAS was significantly higher in CSX (31.27 (21.97)) compared to CAD (21.86 (12.97), P = 0.021). However, abnormally increased anxiety was not associated with higher risk of CSX. LnLF, LnHF and LnLF/HF were comparable in CAD and CSX patients. Conclusion CSX and CAD patients showed comparable CAM. Although anxiety levels were higher in CSX compared to CAD, TMAS ≥ 35 failed to show significant association with CSX. PMID:28068419

  6. Randomized comparison of closed-loop feedback computer-controlled with manual-controlled infusion of phenylephrine for maintaining arterial pressure during spinal anaesthesia for caesarean delivery.

    PubMed

    Ngan Kee, W D; Khaw, K S; Ng, F F; Tam, Y H

    2013-01-01

    Closed-loop feedback computer-controlled infusion has not been described for administering phenylephrine to maintain arterial pressure (AP) during spinal anaesthesia for caesarean delivery. We aimed to compare AP control using this automated system with a previously described manual infusion system. We randomly allocated 222 healthy subjects having spinal anaesthesia for scheduled caesarean delivery to have systolic AP maintained near baseline with phenylephrine (100 µg ml(-1)) by computer-controlled infusion utilizing a proportional algorithm or manual-controlled infusion utilizing an on-off algorithm. AP control was assessed by comparing the proportion of systolic AP measurements within ±20% of baseline and by performance error (PE) calculations. A total of 212 subjects finished the study. In the computer-control group, 97% of systolic AP recordings fell within ±20% of baseline compared with 95% in the manual-control group (P=0.0004). For computer-control compared with manual-control, wobble was smaller [median 3.5 (inter-quartile range 2.5-4.8)% vs 4.2 (3.3-5.9)%, P=0.003], but there was no difference in the median PE [2.9 (0.3-4.7)% vs 1.9 (0-4.2)%], median absolute PE [4.7 (3.5-5.6)% vs 4.7 (3.8-6.7)%], or divergence [-0.01 (-0.03-0)% vs -0.06 (-0.26-0.08)%]. Fewer interventions per subject for controlling AP were required in the computer-control group [2 (2-2) vs 10 (8-13), P<0.001]. There were no differences in measured clinical outcomes. Within the constraints of the studied algorithms, closed-loop feedback computer-controlled phenylephrine infusion provided better AP control with fewer interventions required compared with manual-controlled infusion.

  7. Locked-in syndrome after basilary artery thrombosis by mucormycosis masquerading as meningoencephalitis in a lymphoma patient.

    PubMed

    Maffini, Fausto; Cocorocchio, Emilia; Pruneri, Giancarlo; Bonomo, Guido; Peccatori, Fedro; Chiapparini, Laura; Vincenzo, Silvia Di; Martinelli, Giovanni; Viale, Giuseppe

    2013-01-01

    Locked-in syndrome is a rare clinical syndrome due to basilary artery thrombosis generally associated with trauma, vascular, or cardiac malformation. It can present as various types of clinical evolution and occasionally masquerades as other pathological conditions, such as infective meningoencephalitis. These complications are the cause of diagnostic delay, if not promptly recognised, followed by patient death. We report the case of a 42-year-old female with a systemic B and cutaneous T-cell non-Hodgkin's lymphoma, with a severe neutropenia lasting over a year, who eventually developed a rapid and fatal fungal mucormycosis sepsis following a skin infection on her right arm, associated with locked-in syndrome and meningoencephalitis.

  8. Dysregulated expression of microRNAs and mRNAs in pulmonary artery remodeling in ascites syndrome in broiler chickens.

    PubMed

    Liu, Ping; Yang, Fei; Zhuang, Yu; Xiao, Qingyang; Cao, Huabin; Zhang, Caiying; Wang, Tiancheng; Lin, Huayuan; Guo, Xiaoquan; Hu, Guoliang

    2017-01-10

    Ascites syndrome (AS), also known as pulmonary artery hypertension, remains a challenging disease that severely affects both humans and broiler chickens. Pulmonary artery remodeling presents a key step in the development of AS. In this study, we obtained pulmonary artery tissues from broilers with and without AS to perform miRNA sequencing analysis, miRNA-mRNA association analysis and pathological examinations. 29 significantly differentially expressed miRNAs were found both in known and novel miRNAs with 18 up-regulated and 11 down-regulated miRNAs. Their predicted potential targets were involved in a wide range of functional clusters as indicated via GO (Gene Ontology) and KEGG (Kyoto Encyclopedia of Genes and Genomes) analyses. The upregulation of miR-155, miR-23b-3p, miR-146b-5p and miR-146b-3p were found closely associated with the pathogenesis of pulmonary artery remodeling in AS progression. The association analysis for the miRNAs-mRNAs showed that these 29 significantly differentially expressed miRNAs regulate 162 differentially expressed target genes. Among them, 20 miRNAs correlated with 18 predicted target genes that appear to be involved in pulmonary artery remodeling, mainly in three broad physiological processes: the hypoxia sensing response (HIF1α, NHE1, STAT5 and STAT3), endothelial permeability dysfunction (CD44, TRAF2, CDK2AP1, LZTFL1, JAZF1, PEBP1, LRP1B, RPS14 and THBS2) and inflammation (MEOX2, STAT5, STAT3, IRF8, MAP3K8, IL-1BETA and TNFRSF1B). Pathological pulmonary artery remodeling in the AS broilers was consistently observed in the present study. Taken together, the current analysis further illuminates the molecular mechanism of pulmonary artery remodeling underlying AS progression.

  9. [The non-etherifying and free fatty acids of blood plasma. Pathogenesis of arterial hypertension and symptoms of syndrome of overeating-metabolic syndrome: a lecture].

    PubMed

    Titov, V N

    2013-12-01

    From point of view of physiology, the metabolic syndrome is a syndrome of overeating when an optimal by the content of fatty acids in food is too much a physologically. This condition forms an omental variant of increase of body mass. The oleic triglycerides cumulate in fatty cells of omentum and after activation of lypolisis at the level of paracrinically regulating associations of cells and organs release into blood many non-etherifying fatty acids. The albumin has no possibilities to bind them all. The polar fatty acids-free fatty acids which are not bind by albumin form in blood direct heterogeneous micelles which spontaneously incorporate into plasmatic membrane of monolayer of endothelium. At that, the hydrophilic lipid pores are formed through which Ca2+, Na+ and water get into cytosol and K+ gets out. The hydration of cytosol and hypercalcinemia increase dimensions, thickness of monolayer of epithelium, narrow lumen of arterioles of muscular type and increase resistance to blood flow in distal section of arterial channel. The hydrodynamic pressure increases compensatory in proximal section of arterial channel along with the development of arterial hypertension. The late in phylogenesis insulin has no possibilities to block lipolysis in fatty cells of omentum hence these cells have no receptors to this insulin. While in blood plasma the concentration of non-etherifying acids is increased the cell will not absorb and oxidize glucose. The non-etherifying form the resistance too late in phylogenesis insulin, hyperglycemia and hyperinsulinemia. The concentration of oleic triglycerides increases in blood. The increase in omentum of number of fatty cells of loose connective tissue forms biological reaction of inflammation right up to destruction of overloaded oleic triglycerides cells on the type of apoptosis. This occurrence increases the concentration of C-reactive protein in blood plasma. All symptoms of syndrome of overeating (metabolic syndrome) are formed in

  10. Spinal cord stimulation for the treatment of progressive systemic sclerosis and Raynaud's syndrome.

    PubMed

    Francaviglia, N; Silvestro, C; Maiello, M; Bragazzi, R; Bernucci, C

    1994-01-01

    Progressive Systemic Scleroderma (PSS) is a generalized disease of connective tissue involving the skin, as well as other internal organs. The cutaneous signs are characterized by a progressive sclerosis and loss of function or dexterity in the hands. Between 1987 and 1992, 15 patients affected by scleroderma were treated by means of spinal cord stimulation (SCS) in order to reduce signs and symptoms related to vascular damage. The follow-up ranged from 12 months to 6 years. The study confirms that SCS is an effective therapy in patients with PSS and Raynaud's phenomenon because of its beneficial effects on the Raynaud episodes, ulcers, pain, vascular sclerosis and hand function. This method may have a primary role in the treatment of this chronic disorder because of the high probability of failure of other medical or surgical therapy.

  11. An unusual combination of congenital anomalies in an adult patient: patent ductus arteriosus, Kommerell's diverticulum with aberrant right subclavian artery, and heterotaxy syndrome.

    PubMed

    Celik, Murat; Celik, Turgay; Iyisoy, Atila; Guler, Adem

    2011-01-01

    The heterotaxy syndrome is a rare and sporadic disorder. This syndrome presents with situs ambiguus, splenic malformations such as asplenia or polysplenia, and congenital heart disease. Congenital heart diseases associated with this syndrome include a broad variety of manifestations. Patent ductus arteriosus is one of them and percutaneous transcatheter closure can be challenging in the setting of this syndrome. Kommerell's diverticulum is a saccular aneurysmal dilation at the origin of an aberrant subclavian artery, and can be related with other congenital anomalies. However, there is no previous report of Kommerell's diverticulum being found together with patent ductus arteriosus and heterotaxy syndrome.

  12. Central Pain Syndrome

    MedlinePlus

    ... cord. This syndrome can be caused by stroke, multiple sclerosis, tumors, epilepsy, brain or spinal cord trauma, or ... cord. This syndrome can be caused by stroke, multiple sclerosis, tumors, epilepsy, brain or spinal cord trauma, or ...

  13. Type IV spinal arteriovenous malformation in association with familial pulmonary vascular malformations: case report.

    PubMed

    Rosenow, J; Rawanduzy, A; Weitzner, I; Couldwell, W T

    2000-05-01

    Type IVc arteriovenous malformations (AVMs) of the spinal cord consist of multiple high-flow feeding vessels, and they often present a challenging management situation. Their location is intradural and extramedullary, and they are rare malformations that are difficult to treat owing to the risk of thrombosis of the anterior spinal artery. The authors report a case of Type IVc spinal AVM in a patient with a family history of three siblings with pulmonary AVMs. Spinal AVMs have been reported to be associated with inherited syndromes such as familial cutaneous hemangiomas and Kartagener's syndrome, but an association with pulmonary AVMs has not previously been described. A 27-year-old man presented with sudden onset of occipital headache with cervical radiation while weightlifting. Results of computed tomography of the brain were normal, but lumbar puncture revealed a subarachnoid hemorrhage. The patient had a 1-year history of a neurogenic bladder and exhibited marked left calf muscle wasting. The patient underwent spinal magnetic resonance imaging, which revealed the AVM in the conus region. Selective spinal angiography was performed for diagnostic purposes. A laminectomy was performed, and the vessels feeding the AVM were clipped, as was the fistula. The patient remained neurologically stable, and angiography confirmed obliteration of the AVM. This is the first case report of a patient with a spinal AVM who had multiple siblings with pulmonary malformations or AVMs.

  14. Evidence-based and heuristic approaches for customization of care in cardiometabolic syndrome after spinal cord injury

    PubMed Central

    Nash, Mark S.; Cowan, Rachel E.; Kressler, Jochen

    2012-01-01

    Component and coalesced health risks of the cardiometabolic syndrome (CMS) are commonly reported in persons with spinal cord injuries (SCIs). These CMS hazards are also co-morbid with physical deconditioning and elevated pro-atherogenic inflammatory cytokines, both of which are common after SCI and worsen the prognosis for all-cause cardiovascular disease. This article describes a systematic procedure for individualized CMS risk assessment after SCI, and emphasizes evidence-based and intuition-centered countermeasures to disease. A unified approach will propose therapeutic lifestyle intervention as a routine plan for aggressive primary prevention in this risk-susceptible population. Customization of dietary and exercise plans then follow, identifying shortfalls in diet and activity patterns, and ways in which these healthy lifestyles can be more substantially embraced by both stakeholders with SCI and their health care providers. In cases where lifestyle intervention utilizing diet and exercise is unsuccessful in countering risks, available pharmacotherapies and a preferred therapeutic agent are proposed according to authoritative standards. The over-arching purpose of the monograph is to create an operational framework in which existing evidence-based approaches or heuristic modeling becomes best practice. In this way persons with SCI can lead more active and healthy lives. PMID:23031165

  15. Radiographic and intraoperative imaging of a hemisection of the spinal cord resulting in a pure Brown-Séquard syndrome: case report and review of the literature.

    PubMed

    Dlouhy, B J; Dahdaleh, N S; Howard, M A

    2013-03-01

    Brown-Séquard plus syndrome (BSPS) or incomplete spinal cord injuries from stab injury have been widely reported. However, only four detailed cases of pure Brown-Séquard syndrome (BSS) from stab injury have been previously reported. Here we present the case of an 18-year-old man who sustained a penetrating knife stab injury to the right side of his back resulting in a pure Brown-Séquard syndrome with left lower extremity hemiplegia. Imaging revealed right-sided soft tissue and ligamentous damage traveling in a right-to-left fashion as well as left-sided T2-weighted MRI cord signal change at the level of T9. Given concern for a cerebrospinal fluid leak (CSF) leak, the patient was taken for wound exploration, irrigation, laminectomy, dural closure and lumbar drain placement. At three years follow up, the patient was almost full strength. This is the first case in the literature demonstrating radiographic and correlative intraoperative imaging of a hemisection of the spinal cord resulting in a pure Brown-Séquard syndrome.

  16. Embolization of the Systemic Arterial Supply via a Detachable Silicon Balloon in a Child with Scimitar Syndrome

    SciTech Connect

    Sahin, Sinan Celebi, Ahmet; Yalcin, Yalim; Saritas, Mustafa; Bilal, Mehmet S.; Celik, Levent

    2005-04-15

    Scimitar syndrome is a rare congenital disorder. It is characterized by partial or total abnormal venous drainage of the right lung into the inferior vena cava, which is often associated with anomalous systemic arterial supply to the right lung, congenital cardiac anomalies, hypoplasia of the right lung and bronchial anomalies. Symptoms depend on the degree of the shunt and severity of the associated anomalies, which determine the treatment. We present a 6-year-old boy who was diagnosed as having the adult form of scimitar syndrome during evaluation for recurrent pulmonary infections, and underwent embolization with a detachable silicon balloon of the anomalous systemic arterial supply from the abdominal aorta to the right lower lung lobe. Successful elective surgery was performed 6 months later, in which right pulmonary veins were directed to the left atrium using a Gore-Tex patch by creating an intra-atrial tunnel. The patient has been symptom-free period during 6 months of follow-up, which supports the idea that recurrent pulmonary infections can be eliminated by embolization of the anomalous arterial supply.

  17. Association of lower urinary tract syndrome with peripheral arterial occlusive disease

    PubMed Central

    Lin, Wei-Yu; Andersson, Karl-Erik; Lin, Cheng-Li; Kao, Chia-Hung; Wu, Hsi-Chin

    2017-01-01

    Purpose To describe atherosclerosis may lead to chronic bladder ischemia, eventually resulting in lower urinary tract syndrome (LUTS), and peripheral arterial occlusive disease (PAOD). We investigated the association of LUTS with PAOD. Methods This nationwide population-based cohort study was based on data from the Taiwan National Health Insurance Database from 2000 to 2010; follow-up lasted until the end of 2011. We identified patients with newly diagnosed LUTS by using International Classification of Diseases, Ninth Revision, Clinical Modification codes. Results In total, 36,042 and 36,042 patients were enrolled in LUTS and non-LUTS cohorts, respectively. After adjustment for age, sex, and comorbidities, the risk of subsequent PAOD was 1.36-fold higher [95% confidence interval (CI) = 1.26–1.46] in the LUTS cohort than in the non-LUTS cohort. The adjusted risk of PAOD was the highest in patients with LUTS without any comorbidity [adjusted hazard ratio (aHR) = 1.93, 95% CI = 1.54–2.41]. The age-specific relative risk of PAOD was significantly higher in all age groups, particularly in those aged <49 years (aHR = 1.80, 95% CI = 1.39–2.34], in the LUTS cohort than in the non-LUTS cohort. Conclusion LUTS is a risk factor for PAOD. Physicians should consider the possibility of underlying PAOD in patients with LUTS aged <49 years and without cardiovascular comorbidities. Additional studies developing strategies for decreasing the risk of PAOD are warranted. PMID:28301517

  18. TBX4 mutations (small patella syndrome) are associated with childhood-onset pulmonary arterial hypertension.

    PubMed

    Kerstjens-Frederikse, Wilhelmina S; Bongers, Ernie M H F; Roofthooft, Marcus T R; Leter, Edward M; Douwes, J Menno; Van Dijk, Arie; Vonk-Noordegraaf, Anton; Dijk-Bos, Krista K; Hoefsloot, Lies H; Hoendermis, Elke S; Gille, Johan J P; Sikkema-Raddatz, Birgit; Hofstra, Robert M W; Berger, Rolf M F

    2013-08-01

    Childhood-onset pulmonary arterial hypertension (PAH) is rare and differs from adult-onset disease in clinical presentation, with often unexplained mental retardation and dysmorphic features (MR/DF). Mutations in the major PAH gene, BMPR2, were reported to cause PAH in only 10-16% of childhood-onset patients. We aimed to identify more genes associated with childhood-onset PAH. We studied 20 consecutive cases with idiopathic or heritable PAH. In patients with accompanying MR/DF (n=6) array-comparative genomic hybridisation analysis was performed, with the aim of finding common deletion regions containing candidate genes for PAH. Three patients had overlapping deletions of 17q23.2. TBX2 and TBX4 were selected from this area as candidate genes and sequenced in all 20 children. After identifying TBX4 mutations in these children, we subsequently sequenced TBX4 in a cohort of 49 adults with PAH. Because TBX4 mutations are known to cause small patella syndrome (SPS), all patients with newly detected TBX4 mutations were screened for features of SPS. We also screened a third cohort of 23 patients with SPS for PAH. TBX4 mutations (n=3) or TBX4-containing deletions (n=3) were detected in 6 out of 20 children with PAH (30%). All living patients and two parents with TBX4 mutations appeared to have previously unrecognised SPS. In the adult PAH-cohort, one TBX4 mutation (2%) was detected. Screening in the cohort of (predominantly adult) SPS patients revealed no PAH. These data indicate that TBX4 mutations are associated with childhood-onset PAH, but that the prevalence of PAH in adult TBX4 mutation carriers is low.

  19. QT Interval Derived Measurements in Patients with Cardiac Syndrome X Compared to Coronary Artery Disease.

    PubMed

    Lutfi, Mohamed F

    2016-01-01

    Previous studies assessing effect of ischemia on ventricular repolarization are mostly directed toward patients with coronary artery disease (CAD); however, similar reports on cardiac syndrome X (CSX) are scarce. Whether microvascular dysfunction of CSX and ischemia induced by CAD produce comparable effect on ventricular repolarization is unclear and deserve further studies. In the present study, ECG measures of ventricular repolarization were compared between CAD and CSX patients (40 subjects in each group). Following evaluation of sociodemographic characteristics, medical and past medical history, a resting ECG was used to assess measurements of ventricular repolarization in each patient, namely, QT interval (QT), corrected QT interval (QTc), QT dispersion (QTd), corrected QT dispersion (QTcd), adjacent QT dispersion (AdQTd), QT dispersion ratio (QTdR), JT dispersion (JTd), and Corrected JT dispersion (JTcd). Results showed comparable QT intervals and QTd in CAD and CSX patients even after adjustment for the possible variations in gender, age and body mass index of the studied groups. Although JTd was increased in CSX subjects (26.6 ± 7.2 ms) compared with CAD patients (22.7 ± 6.5 ms, p = 0.019), statistical significance disappeared after correcting JT for variations in heart rate. QT and QTc were significantly below 440 ms in CAD as well as CSX patients (p < 0.001). In contrast, maximum QTd, maximum QTcd and AdQTd of CAD and CSX patients were significantly above 440 ms (p < 0.001). The means of JTd and JTcd were significantly above 22 ms and 24 ms respectively (p < 0.001, p = 0.001) in CSX but not CAD patients (p = 0.529, p = 0.281). The present findings clearly demonstrate comparable measures of ventricular repolarization in CAD and CSX patients and consequently an equal risk of cardiac events in both groups.

  20. Treatment of right ventricle to pulmonary artery conduit stenosis in infants with hypoplastic left heart syndrome.

    PubMed

    Münsterer, Andrea; Kasnar-Samprec, Jelena; Hörer, Jürgen; Cleuziou, Julie; Eicken, Andreas; Malcic, Ivan; Lange, Rüdiger; Schreiber, Christian

    2013-09-01

    To determine the incidence of right ventricle-to-pulmonary artery (RV-PA) conduit stenosis after the Norwood I operation in patients with hypoplastic left heart syndrome (HLHS), and to determine whether the treatment strategy of RV-PA conduit stenosis has an influence on interstage and overall survival. Ninety-six patients had a Norwood operation with RV-PA conduit between 2002 and 2011. Details of reoperations/interventions due to conduit obstruction prior to bidirectional superior cavopulmonary anastomosis (BSCPA) were collected. Overall pre-BSCPA mortality was 17%, early mortality after Norwood, 6%. Early angiography was performed in 34 patients due to desaturation at a median of 8 days after the Norwood operation. Fifteen patients (16%) were diagnosed with RV-PA conduit stenosis that required treatment. The location of the conduit stenosis was significantly different in the patients with non-ringed (proximal) and the patients with ring-enforced conduit (distal), P = 0.004. In 6 patients, a surgical revision of the conduit was performed; 3 of them died prior to BSCPA. Another 6 patients had a stent implantation and 3 were treated with balloon dilatation followed by a BSCPA in the subsequent 2 weeks. All patients who were treated interventionally for RV-PA conduit obstruction had a successful BSCPA. Patients who received a surgical RV-PA conduit revision had a significantly higher interstage (P = 0.044) and overall mortality (P = 0.011) than those who received a stent or balloon dilatation of the stenosis followed by an early BSCPA. RV-PA conduit obstruction after Norwood I procedure in patients with HLHS can be safely and effectively treated by stent implantation, balloon dilatation and early BSCPA. Surgical revision of the RV-PA conduit can be reserved for patients in whom an interventional approach fails, and an early BSCPA is not an option.

  1. CT Diagnosis of Fitz-Hugh and Curtis Syndrome: Value of the Arterial Phase Scan

    PubMed Central

    Joo, Seung Ho; Lim, Joon Seok; Kim, Joo Hee; Kim, Ki Whang

    2007-01-01

    Objective We wanted to evaluate the role of the arterial phase (AP) together with the portal venous phase (PP) scans in the diagnosis of Fitz-Hugh-Curtis syndrome (FHCS) with using computed tomography (CT). Materials and Methods Twenty-five patients with FHCS and 25 women presenting with non-specifically diagnosed acute abdominal pain and who underwent biphasic CT examinations were evaluated. The AP scan included the upper abdomen, and the PP scan included the whole abdomen. Two radiologists blindly and retrospectively reviewed the PP scans first and then they reviewed the AP plus PP scans. The diagnostic accuracy of FHCS on each image set was compared for each reader by analyzing the area under the receiver operating characteristic curve (Az). Weighted kappa (wk) statistics were used to measure the interobserver agreement for the presence of CT signs of the pelvic inflammatory disease (PID) on the PP images and FHCS as the diagnosis based on the increased perihepatic enhancement on both sets of images. Results The individual diagnostic accuracy of FHCS was higher on the biphasic images (Az = 0.905 and 0.942 for reader 1 and 2, respectively) than on the PP images alone (Az = 0.806 and 0.706, respectively). The interobserver agreement for the presence of PID on the PP images was moderate (wk = 0.530). The interobserver agreement for FHCS as the diagnosis was moderate on only the PP images (wk = 0.413), but it was substantial on the biphasic images (wk = 0.719). Conclusion Inclusion of the AP scan is helpful to depict the increased perihepatic enhancement, and it improves the diagnostic accuracy of FHCS on CT. PMID:17277562

  2. Ca2+ regulatory mechanisms of exercise protection against coronary artery disease in metabolic syndrome and diabetes

    PubMed Central

    2011-01-01

    Chronic exercise attenuates coronary artery disease (CAD) in humans largely independent of reductions in risk factors; thus major protective mechanisms of exercise are directly within the coronary vasculature. Further, tight control of diabetes, e.g., blood glucose, can be detrimental. Accordingly, knowledge of mechanisms by which exercise attenuates diabetic CAD could catalyze development of molecular therapies. Exercise attenuates CAD (atherosclerosis) and restenosis in miniature swine models, which enable precise control of exercise parameters (intensity, duration, and frequency) and characterization of the metabolic syndrome (MetS) and diabetic milieu. Intracellular Ca2+ is a pivotal second messenger for coronary smooth muscle (CSM) excitation-contraction and excitation-transcription coupling that modulates CSM proliferation, migration, and calcification. CSM of diabetic dyslipidemic Yucatan swine have impaired Ca2+ extrusion via the plasmalemma Ca2+ ATPase (PMCA), downregulation of L-type voltage-gated Ca2+ channels (VGCC), increased Ca2+ sequestration by the sarcoplasmic reticulum (SR) Ca2+ ATPase (SERCA), increased nuclear Ca2+ localization, and greater activation of K channels by Ca2+ release from the SR. Endurance exercise training prevents Ca2+ transport changes with virtually no effect on the diabetic milieu (glucose, lipids). In MetS Ossabaw swine transient receptor potential canonical (TRPC) channels are upregulated and exercise training reverses expression and TRPC-mediated Ca2+ influx with almost no change in the MetS milieu. Overall, exercise effects on Ca2+ signaling modulate CSM phenotype. Future studies should 1) selectively target key Ca2+ transporters to determine definitively their causal role in atherosclerosis and 2) combine mechanistic studies with clinical outcomes, e.g., reduction of myocardial infarction. PMID:21596923

  3. Indocyanine green kinetics with near-infrared spectroscopy predicts cerebral hyperperfusion syndrome after carotid artery stenting.

    PubMed

    Nakagawa, Ichiro; Park, Hun Soo; Yokoyama, Shohei; Yamada, Shuichi; Motoyama, Yasushi; Park, Young Su; Wada, Takeshi; Kichikawa, Kimihiko; Nakase, Hiroyuki

    2017-01-01

    Cerebral hyperperfusion syndrome (HPS) is a potentially life-threatening complication following carotid artery stenting (CAS) and carotid endoarterectomy (CEA). Early prediction and treatment of patients at risk for HPS are required in patients undergoing CAS because HPS occurs significantly earlier after CAS than CEA. Near-infrared spectroscopy (NIRS) is often used for monitoring, and indocyanine green (ICG) kinetics by NIRS (ICG-NIRS) can detect reductions in cerebral perfusion in patients with acute stroke. However, whether ICG-NIRS can predict postoperative hyperperfusion phenomenon (HP) after carotid revascularization is unclear. Here, we evaluated whether the blood flow index (BFI) ratio calculated from a time-intensity curve from ICG-NIRS monitoring can predict HPS after CAS. The BFI ratio was prospectively monitored using ICG-NIRS in 135 patients undergoing CAS. Preoperative cerebrovascular reactivity (CVR) and the postoperative asymmetry index (AI) were also assessed with single-photon emission computed tomography before and after CAS, and the correlation was evaluated. In addition, patients were divided into two groups, a non-HP group (n = 113) and an HP group (n = 22), and we evaluated the correlation with hemodynamic impairment in the ipsilateral hemisphere and clinical results. Twenty-two cases (16%) showed HP, and four (3%) showed HPS after CAS. The BFI ratio calculated from ICG-NIRS showed a significant linear correlation with preoperative CVR and postoperative AI (r = -0.568, 0.538, P < 0.001, <0.001, respectively). The degree of stenosis, the rate of no cross flow, preoperative CVR, and the incidence of HPS were significantly different between the groups. Measurement of ICG kinetics by NIRS is useful for detection of HPS in patients who underwent CAS.

  4. Resveratrol modifies risk factors for coronary artery disease in swine with metabolic syndrome and myocardial ischemia.

    PubMed

    Robich, Michael P; Osipov, Robert M; Chu, Louis M; Han, Yuchi; Feng, Jun; Nezafat, Reza; Clements, Richard T; Manning, Warren J; Sellke, Frank W

    2011-08-16

    Resveratrol has been purported to modify risk factors for obesity and cardiovascular disease. We sought to examine the effects of resveratrol in a porcine model of metabolic syndrome and chronic myocardial ischemia. Yorkshire swine were fed either a normal diet (control), a high cholesterol diet (HCD), or a high cholesterol diet with supplemental resveratrol (HCD-R; 100mg/kg/day) for 11 weeks. After 4 weeks of diet modification a baseline cardiovascular MRI was performed and an ameroid constrictor was placed on the left circumflex coronary artery of each animal to induce chronic myocardial ischemia. At 7 weeks, a second cardiovascular MRI was performed and swine were sacrificed and myocardial tissue harvested. Resveratrol supplementation resulted in lower body mass indices, serum cholesterol, and C-reactive protein levels, improved glucose tolerance and endothelial function, and favorably augmented signaling pathways associated with myocardial metabolism. Interestingly, serum tumor necrosis factor-α levels were not influenced by resveratrol treatment. Immunoblotting for markers of metabolism demonstrated that insulin receptor substrate-1, glucose transporters 1 and 4, and phospho-AMPK were increased in the HCD-R group. Peroxisome proliferator-activated receptor γ and retinol binding protein 4 were downregulated in the HCD-R group as compared to the HCD group. Myocardial perfusion and function at rest as assessed with magnetic resonance imaging were not different between groups. By favorably influencing risk factors, resveratrol may decrease the burden of chronic metabolic disease and improve cardiovascular health. Copyright © 2011 Elsevier B.V. All rights reserved.

  5. Repeated electro-acupuncture attenuates chronic visceral hypersensitivity and spinal cord NMDA receptor phosphorylation in a rat irritable bowel syndrome model.

    PubMed

    Tian, Shun-Lian; Wang, Xiao-Yan; Ding, Guang-Hong

    2008-08-29

    Acupuncture has been used in clinical trials for the treatment of abdominal pain in patients with irritable bowel syndrome (IBS). However, scientific evidence is still lacking and the underlying mechanism remains largely unexplored. The aim of this study was to examine the effects of repeated administration of electro-acupuncture (EA) on chronic visceral hypersensitivity and on the phosphorylation of spinal cord N-methyl-D-aspartic acid (NMDA) receptors in a rat model of IBS. The results showed that repeated administration of EA at bilateral points of Zu-san-li (ST-36) and Shang-ju-xu (ST-37) significantly attenuated chronic visceral hypersensitivity induced in young adult rats by neonatal colon irritation. Such an effect was not seen in either of the two controls: sham-EA at ST-36 and ST-37 without electrical stimulation and EA at control points (BL-62 and tail). Furthermore, rats with chronic visceral hypersensitivity exhibited high-level expression of phosphorylated NMDA receptor subunit 1 (pNR1) in the spinal cord (L4-L5 segments), which was markedly attenuated by EA treatment. In addition, EA at ST-36 and ST-37 neither altered the pain threshold of normal rats nor affected the expression of pNR1 in the lumbosacral spinal cord. Altogether, these data indicate that the EA-mediated attenuation of chronic visceral hypersensitivity is correlated with the down-regulation of NMDA receptors phosphorylation at the spinal level.

  6. Aortic coarctation and carotid artery aneurysm in a patient with Hardikar syndrome: Cardiovascular implications for affected individuals.

    PubMed

    Ryan, Kaitlin M; Ellis, Alexander R; Raafat, Reem; Bhoj, Elizabeth J; Hakonarson, Hakon; Li, Dong; Schrier Vergano, Samantha

    2016-02-01

    Hardikar syndrome is a multiple congenital anomaly syndrome first characterized in 1992 by Hardikar et al. to describe two individuals with cholestasis, cleft lip/palate, retinal pigmentation, intestinal abnormalities, and genitourinary anomalies. Between 1992 and 2002, four individuals with Hardikar syndrome were reported in the literature. The fourth individual [Maluf et al. (2002), Transplantation 74:1058-1061; Poley and Proud (2008) Am J Med Genet Part A 146A:2473-2479], who had progressive cholestatic liver disease ultimately requiring liver transplantation, has continued to be followed at our institution. Recently, at the age of 14 years, during an evaluation for refractory hypertension, she was found to have developed coarctation of the aorta that was treated with aortic angioplasty and stenting, dramatically improving her hypertension. Further vascular investigation also revealed a small aneurysm of her carotid artery requiring neurosurgical evaluation and anticoagulant therapy. To our knowledge, these vascular anomalies have not been reported in Hardikar syndrome and the high association of congenital heart disease in the individuals with Hardikar syndrome has not been further addressed. Herein, we discuss this additional clinical information, speculate briefly on possible molecular etiologies, and discuss potential cardiac surveillance recommendations. We hope that broadening the known phenotype of this very rare disorder will further aid clinicians in their management and surveillance for these individuals.

  7. Reduced arterial circulation to the legs in spinal cord injury as a cause of skin breakdown lesions.

    PubMed

    Deitrick, George; Charalel, Joseph; Bauman, William; Tuckman, John

    2007-01-01

    Skin breakdown lesions (SBLs) of the legs are common in spinal cord injury (SCI). It is assumed that the cause is deficient sensitivity and immobility of the limbs, which result in areas subjected to prolonged pressures. However, poor circulation may also be a significant factor. Indeed, strong reasons suggest that small vessel circulation is decreased in SCI because these patients have increased arteriosclerotic risk factors. Patients in the SCI population have advanced age, are sedentary, often have abnormal carbohydrate and lipid metabolism, and many use tobacco products. Total blood flow (TBF) to the legs and skin blood flows (SBFs) to 4 areas of the feet were measured simultaneously by duplex Doppler sonography and laser Doppler flowmetry in 10 healthy control and 10 chronic subjects with SCI when supine and during 30 minutes in a wheelchair. The average supine control TBF was 540 mL/minute, but greatly reduced between 24-76 mL/minute in 4 of the subjects. During sitting, the average TBF fell by 41% in the controls and increased by 6% in SCI. Nonetheless, in all control and SCI subjects the average sitting SBFs were severely decreased in all areas between 53-75%, similar to results found by others elsewhere in the foreleg. Ischemia of the skin and underlying muscles is important as a cause for the poor healing of SBLs in persons with SCI who daily spend many hours in a wheelchair.

  8. Dorsal Scapular Artery Variations and Relationship to the Brachial Plexus, and a Related Thoracic Outlet Syndrome Case

    PubMed Central

    Verenna, Anne-Marie A.; Alexandru, Daniela; Karimi, Afshin; Brown, Justin M.; Bove, Geoffrey M.; Daly, Frank J.; Pastore, Anthony M.; Pearson, Helen E.; Barbe, Mary F.

    2016-01-01

    Rationale Knowledge of the relationship of the dorsal scapular artery (DSA) with the brachial plexus is limited. Objective We report a case of a variant DSA path, and revisit DSA origins and under-investigated relationship with the plexus in cadavers. Methods The DSA was examined in a male patient and 106 cadavers. Results In the case, we observed an unusual DSA compressing the lower plexus trunk, that resulted in intermittent radiating pain and paresthesia. In the cadavers, the DSA originated most commonly from the subclavian artery (71%), with 35% from the thyrocervical trunk. Nine sides of eight cadavers (seven females) had two DSA branches per side, with one branch from each origin. The most typical DSA path was a subclavian artery origin before passing between upper and middle brachial plexus trunks (40% of DSAs), versus between middle and lower trunks (23%), or inferior (4%) or superior to the plexus (1%). Following a thyrocervical trunk origin, the DSA passed most frequently superior to the plexus (23%), versus between middle and lower trunks (6%) or upper and middle trunks (4%). Bilateral symmetry in origin and path through the brachial plexus was observed in 13 of 35 females (37%) and 6 of 17 males (35%), with the most common bilateral finding of a subclavian artery origin and a path between upper and middle trunks (17%). Conclusion Variability in the relationship between DSA and trunks of the brachial plexus has surgical and clinical implications, such as diagnosis of thoracic outlet syndrome. PMID:28077957

  9. Spinal Arteriovenous Fistula with Progressive Paraplegia after Spinal Anaesthesia

    PubMed Central

    Argyrakis, Nikolaos; Matis, Georgios K.; Mpata-Tshibemba, Stephanie

    2014-01-01

    A case of an iatrogenic spinal arteriovenous fistula with progressive paraplegia in a young woman is reported. The fistula was eventually created after repetitive lumbar punctures performed in the process of spinal anaesthesia. Her symptoms were progressed to paraplegia over a period of 2 years. The digital subtraction angiography demonstrated a single-hole fistula, involving the anterior spinal artery and vein. The lesion was occluded by embolization with immediate improvement. The potential mechanism is discussed. PMID:24653807

  10. Long-term follow-up of functioning after spinal surgery in patients with Rett syndrome.

    PubMed

    Larsson, Eva-Lena; Aaro, Stig; Ahlinder, Peter; Normelli, Helena; Tropp, Hans; Oberg, Birgitta

    2009-04-01

    In a prospective study, 23 consecutive girls with Rett syndrome and neuromuscular scoliosis were evaluated for functioning at a long-term follow-up. The patients had mostly improved, which was confirmed by their parents. Rett syndrome is associated with neuromuscular scoliosis and has a typically long C-shaped thoracolumbar kyphoscoliosis. Prospective long-term follow-up studies related to these patients' total situation are sparse. Most studies focus on the Cobb angle of the scoliosis, whereas parents are mainly concerned about the girls' continued functioning. Twenty-three patients with Rett syndrome and neuromuscular scoliosis were evaluated preoperatively from 1993 to 2002. At follow-up, 19 patients remained in the study. Three patients died (not due to surgery), and one patient could not participate because it was too far to travel. Mean follow-up time was 74 months (range 49-99 months). The assessments comprised the sitting balance, seating supports in wheelchair, weight distribution, time used for rest, care given, and angle of scoliosis. Follow-up questionnaires and two-open-ended questions about the positive and negative effects of surgery were sent to parents. Sitting balance, number of seating supports in wheelchair, weight distribution, time used for rest, and the Cobb angle had all improved after surgery. The parents assessed improvement in seating position, daily activities, time used for rest, and cosmetic appearance. We can conclude that the stabilized spine resulted in sufficient strength to keep the body upright with the possibility of looking around at the surroundings more easily. The girls got better seating position with less need for seating adaptations in the wheelchair and with reduced time needed for resting during the day. Finally we can conclude that the indication for surgery is to get a better posture which lead to less risk of pressure sores, and that un upright position lead to better possibility to easily breath with fewer episodes

  11. Trifocal Monomyelomeric Spinal Cord Arteriovenous Fistulae in a Seven-Year-Old Boy

    PubMed Central

    Piske, R.; Sampaio, M.; Campos, C.; Nunes, J.A.; Lima, S.S.

    2001-01-01

    Summary We describe a rare case of multiple arteriovenous fistulae of the spinal cord (SCΛVF) in the same myelomer in a five-year-old boy. This case report consists of a trifocal SCAVF at the Thl2 myelomeric level without communication between the three different fistulae. Two AVF were located posteriorly`, bilateraly, in the spinal cord, fed by left and right posterior radiculopial arteries and one anteriorly in the anterior spinal axis. The venous drainage was independent for each lesion. The patient presents associated lesions characterized by cutaneous stain and inferior limb asymmetry. A metameric distribution is the explanation for the multiplicity of these lesions in a syndromic association related to Cobb syndrome. The patient was treated by transarterial embolization using glue with occlusion of the three different fistulae. The patient achieved a good improvement in neurological status. PMID:20663337

  12. Unilateral right pulmonary artery agenesis and congenital cystic adenomatoid malformation of the right lung with Ortner's syndrome.

    PubMed

    David, Jane Jackie; Mohanlal, Smilu; Sankhe, Punam; Ghildiyal, Radha

    2016-01-01

    We report a 2.5-year-old girl who presented with hoarseness of voice since 3 months of age and failure to thrive. Chest X-ray showed cardiomegaly with a deviation of the trachea and mediastinum to the right side. Two-dimensional echocardiography showed decreased flow across the right pulmonary artery, a small atrial septal defect (ASD) with a right-to-left shunt, and a dilated right atrium and right ventricle with severe tricuspid regurgitation suggestive of severe pulmonary hypertension. A silent large patent ductus arteriosus was also seen. Multiple detector computerized tomography aortogram confirmed the findings of absent right pulmonary artery and hypoplastic right lung with small cystic lesions suggestive of congenital cystic adenomatoid malformation in the right lower lobe. Hoarseness of voice was due to the left vocal cord palsy probably secondary to severe pulmonary hypertension (Ortner's syndrome).

  13. Arterial tortuosity and aneurysm in a case of Loeys-Dietz syndrome type IB with a mutation p.R537P in the TGFBR2 gene.

    PubMed

    Kiliç, Esra; Alanay, Yasemin; Utine, Eda; Ozgen-Mocan, Burçe; Robinson, Peter N; Boduroğlu, Koray

    2012-01-01

    We report a 13-year-old girl with Loeys-Dietz syndrome (LDS) caused by a known transforming growth factor beta receptor II (TGFBR2) gene mutation, who developed aortic root dilatation and saccular aneurysm of the internal carotid artery. LDS is a rare, autosomal dominant aortic aneurysm syndrome with multisystem involvement. The disease is typically characterized by the triad of arterial tortuosity and aneurysms, hypertelorism, and bifid uvula/cleft palate. The characteristic LDS symptoms observed in the reported case included craniofacial dysmorphism (hypertelorism, cleft palate, blue sclerae, malar hypoplasia, retrognathia), skeletal deformities (scoliosis, talipes equinovarus, pectus deformity, arachnodactyly), congenital heart defects (patent ductus arteriosus, PDA), and arterial tortuosity and aneurysms. Molecular genetic testing revealed a heterozygous mutation (c.1610 G>C, p.R528C) in the serine-threonine kinase domain of the TGFBR2 gene. Magnetic resonance (MR) angiography showed aortic dilatation, tortuosity of bilateral supraaortic arteries, and saccular aneurysm on the right cervical internal carotid artery. LDS resembles Marfan-related disorders (Marfan, Shprintzen-Goldberg and vascular Ehlers-Danlos syndrome), but arterial tortuosity and aneurysms are characteristic for LDS, so a timely diagnosis of LDS is important for early diagnosis and intervention of aneurysms to prevent vascular events. Here, we describe a LDS patient who presented with arterial tortuosity and saccular aneurysm.

  14. A Rare Case of Anomalous Left Coronary Artery From the Pulmonary Artery (Bland-White-Garland Syndrome) in a 68-Year-Old Woman.

    PubMed

    Roberts, S Michael; Banbury, Trey; Mehta, Anand

    2016-07-09

    Anomalous left coronary artery from the pulmonary artery (ALCAPA), or Bland-White-Garland syndrome, is a rare congenital coronary anomaly that results in altered myocardial perfusion and a left to right shunt. It occurs in 1:300000 live births and represents 0.24% to 0.46% of all congenital cardiac diseases. Despite its rarity, it is one of the most common causes of ischemia and infarction in children. Ninety percent of these patients will die within the first year of life if untreated and diagnosing this abnormality in adulthood is extremely rare. Of those patients who survive to adulthood, the average age of sudden cardiac death is 35 years. The initial symptoms of the adult presentation vary widely from progressive dyspnea to sudden cardiac death; therefore, immediate surgical correction is highly recommended upon diagnosis. Understanding the pathophysiology and nature of collateral coronary flow in this congenital anomaly is paramount to the safe anesthetic management of adults with ALCAPA. Here we describe the intraoperative management and echocardiographic findings in a 68-year-old with with recently diagnosed ALCAPA undergoing surgical repair.

  15. Intraoperative dexmedetomidine and postoperative cerebral hyperperfusion syndrome in patients who underwent superficial temporal artery-middle cerebral artery anastomosis for moyamoya disease: A retrospective observational study.

    PubMed

    Seo, Hyungseok; Ryu, Ho-Geol; Son, Je Do; Kim, Jeong-Soo; Ha, Eun Jin; Kim, Jeong-Eun; Park, Hee-Pyoung

    2016-12-01

    Dexmedetomidine, a selective α2-agonist, reduces cerebral blood flow and has neuroprotective effects against cerebral ischemia/reperfusion injury in experimental animals. We examined whether intraoperative dexmedetomidine would reduce the incidence of postoperative cerebral hyperperfusion syndrome (CHS) after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in patients with moyamoya disease.The electronic medical records of 117 moyamoya patients who underwent STA-MCA anastomosis were reviewed retrospectively. The patients were divided into 2 groups: 48 patients received intraoperative dexmedetomidine (Group D), while 69 patients did not (Group ND). The incidence (primary outcome), onset, and duration of postoperative CHS were noted.The incidence of postoperative CHS was 45.8% and 40.6% in groups D and ND, respectively (P = 0.708). The duration of postoperative CHS was shorter in group D than in group ND (median [Q1-Q3], 5 [3-7] vs 8 [5-10] days, P = 0.021). There was no significant difference in the onset of CHS between group D and group ND (0 [0-2] vs 1 [0-3] days, P = 0.226).In conclusion, intraoperative dexmedetomidine did not reduce the incidence of postoperative CHS, although it reduced the duration of CHS, in patients who had undergone direct revascularization surgery for moyamoya disease.

  16. Intraoperative dexmedetomidine and postoperative cerebral hyperperfusion syndrome in patients who underwent superficial temporal artery-middle cerebral artery anastomosis for moyamoya disease

    PubMed Central

    Seo, Hyungseok; Ryu, Ho-Geol; Son, Je Do; Kim, Jeong-Soo; Ha, Eun Jin; Kim, Jeong-Eun; Park, Hee-Pyoung

    2016-01-01

    Abstract Dexmedetomidine, a selective α2-agonist, reduces cerebral blood flow and has neuroprotective effects against cerebral ischemia/reperfusion injury in experimental animals. We examined whether intraoperative dexmedetomidine would reduce the incidence of postoperative cerebral hyperperfusion syndrome (CHS) after superficial temporal artery-middle cerebral artery (STA-MCA) anastomosis in patients with moyamoya disease. The electronic medical records of 117 moyamoya patients who underwent STA-MCA anastomosis were reviewed retrospectively. The patients were divided into 2 groups: 48 patients received intraoperative dexmedetomidine (Group D), while 69 patients did not (Group ND). The incidence (primary outcome), onset, and duration of postoperative CHS were noted. The incidence of postoperative CHS was 45.8% and 40.6% in groups D and ND, respectively (P = 0.708). The duration of postoperative CHS was shorter in group D than in group ND (median [Q1–Q3], 5 [3–7] vs 8 [5–10] days, P = 0.021). There was no significant difference in the onset of CHS between group D and group ND (0 [0–2] vs 1 [0–3] days, P = 0.226). In conclusion, intraoperative dexmedetomidine did not reduce the incidence of postoperative CHS, although it reduced the duration of CHS, in patients who had undergone direct revascularization surgery for moyamoya disease. PMID:28033272

  17. Predicting Cerebral Hyperperfusion Syndrome Following Superficial Temporal Artery to Middle Cerebral Artery Bypass based on Intraoperative Perfusion-Weighted Magnetic Resonance Imaging.

    PubMed

    Wang, Defeng; Zhu, Fengping; Fung, Ka Ming; Zhu, Wei; Luo, Yishan; Chu, Winnie Chiu Wing; Mok, Vincent Chung Tong; Wu, Jinsong; Shi, Lin; Ahuja, Anil T; Mao, Ying

    2015-09-14

    Moyamoya disease leads to the formation of stenosis in the cerebrovasculature. A superficial temporal artery to middle cerebral artery (STA-MCA) bypass is an effective treatment for the disease, yet it is usually associated with postoperative cerebral hyperperfusion syndrome (CHS). This study aimed to evaluate cerebral hemodynamic changes immediately after surgery and assess whether a semiquantitative analysis of an intraoperative magnetic resonance perfusion-weighted image (PWI) is useful for predicting postoperative CHS. Fourteen patients who underwent the STA-MCA bypass surgery were included in this study. An atlas-based registration method was employed for studying hemodynamics in different cerebral regions. Pre- versus intraoperative and group-wise comparisons were conducted to evaluate the hemodynamic changes. A postoperative increase in relative cerebral blood flow (CBF) at the terminal MCA territory (P = 0.035) and drop in relative mean-time-transit at the central MCA territory (P = 0.012) were observed in all patients. However, a significant raise in the increasing ratio of relative-CBF at the terminal MCA territory was only found in CHS patients (P = 0.023). The cerebrovascular changes of the patients after revascularization treatment were confirmed. Intraoperative PWI might be helpful in predicting the change in relative-CBF at MCA terminal territory which might indicate a risk of CHS.

  18. Panax notoginseng saponins ameliorate impaired arterial vasodilation in SHRSP.Z-Lepr(fa) /lzmDmcr rats with metabolic syndrome.

    PubMed

    Wu, Ting; Sun, Jianning; Kagota, Satomi; Maruyama, Kana; Wakuda, Hirokazu; Shinozuka, Kazumasa

    2016-04-01

    Panax notoginseng saponins (PNS) are major components of Panax notoginseng, a herb with established clinical efficacy against vascular diseases. SHRSP.Z-Lepr(fa) /IzmDmcr (SHRSP.ZF) rats, a new animal model for metabolic syndrome, display an impaired vasorelaxation response in aortas and mesenteric arteries that is mediated by nitric oxide (NO). This study investigated whether PNS and its components can ameliorate this vascular dysfunction in SHRSP.ZF rats. In an in vitro study, in the presence or absence of PNS and its components, vasodilation in response to nitroprusside was determined from myographs under isometric tension conditions in aortas and mesenteric arteries from male SHRSP.ZF rats at 18-20 weeks of age. In an in vivo study, PNS (30 mg/kg per day) was orally administered to SHRSP.ZF rats from 8 to 20 weeks of age. In vitro treatment with PNS and Ginsenoside Rb1 increased nitroprusside-induced relaxation of aortas and mesenteric arteries in SHRSP.ZF rats. The PNS-induced increase was not affected by a nitric oxide (NO) synthase inhibitor or endothelium denudation. Relaxation in response to a cell-permeable cGMP analogue was increased by PNS, but cGMP accumulation by nitroprusside was not altered. In vivo treatment with PNS in SHRSP.ZF rats lowered blood pressure and increased relaxation and the expression of soluble guanylyl cyclase protein in arteries, without affecting metabolic abnormalities. These results indicate that PNS causes an increase in vasodilation in response to NO and a decrease in blood pressure, resulting in protection against vascular dysfunction in SHRSP.ZF rats. PNS might be beneficial in alleviating impaired vasodilation in metabolic syndrome.

  19. A Case of Anomalous Origin and Course of Vertebral Artery in a Patient with Klippel Feil Syndrome.

    PubMed

    Ulusoy, Onur Levent; Sasani, Hadi; Barlas, Sezgi Burçin; Mutlu, Ayhan; Sasani, Mehdi

    2016-01-01

    Patients with Klippel-Feil syndrome (KFS) have an increased incidence of vascular anomalies as well as vertebral artery (VA) anomalies. In this article, we presented imaging findings of a 15-year-old female patient with KFS with a rare association of extraforaminal cranially ascending right VA that originated from the ipsilateral carotid bulb. Trifurcation of the carotid bulb with VA is a very unusual variation and to the best of our knowledge, right-sided one has not been reported in the literature.

  20. A Case of Anomalous Origin and Course of Vertebral Artery in a Patient with Klippel Feil Syndrome

    PubMed Central

    Sasani, Hadi; Barlas, Sezgi Burçin; Mutlu, Ayhan; Sasani, Mehdi

    2016-01-01

    Patients with Klippel-Feil syndrome (KFS) have an increased incidence of vascular anomalies as well as vertebral artery (VA) anomalies. In this article, we presented imaging findings of a 15-year-old female patient with KFS with a rare association of extraforaminal cranially ascending right VA that originated from the ipsilateral carotid bulb. Trifurcation of the carotid bulb with VA is a very unusual variation and to the best of our knowledge, right-sided one has not been reported in the literature. PMID:27390547