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Sample records for bilateral wyburn-mason syndrome

  1. Serous retinal detachment and cystoid macular edema in a patient with Wyburn-Mason syndrome.

    PubMed

    Onder, Halil Ibrahim; Alisan, Sibel; Tunc, Murat

    2015-03-01

    Wyburn-Mason syndrome is a rare phacomatosis characterized by unilateral arteriovenous malformations (AVMs) involving the brain, retina, and (rarely) the skin. The diagnosis is concluded with dilated fundus examination and markedly dilated tortuous vascular loops with arteriovenous communications on fluorescent angiography. We present a 14-year-old male patient with Wyburn-Mason syndrome who developed serous macular neuroretinal detachment, cystoid macular edema (CME), and consequent visual deterioration in the left eye. To the best of our knowledge, this is the first report of a patient with Wyburn-Mason syndrome who developed serous retinal detachment and CME. PMID:24171831

  2. [Bilateral operculum syndrome].

    PubMed

    Lerman-Sagie, T; Porat-Alkabetz, E; Meir, J J; Harel, S

    1996-09-01

    The bilateral operculum syndrome, is a unique developmental syndrome. It is characterized by spastic paralysis of the muscles of the face, pharynx, and of mastication, as well as by epilepsy and mental retardation. Imaging studies show bilateral, structural abnormalities in the frontal, perisylvian region consistent with polymicrogyria. These children are usually diagnosed as suffering from cerebral palsy, but in the bilateral operculum syndrome, intelligence is relatively preserved despite the severe motor involvement. Misdiagnosis may lead to improper estimation of rehabilitation potential preventing appropriate therapy, especially in the field of alternative communication. We present a 3-year-old boy, apparently the first case of this syndrome to be described in Israel. PMID:8940497

  3. PHACE(S) syndrome.

    PubMed

    Heyer, Geoffrey L

    2015-01-01

    PHACE(S) syndrome is a neurocutaneous disorder of unknown etiology. The acronym refers to the commonest features of PHACE: posterior fossa malformations, large facial hemangiomas, cerebral arterial anomalies, cardiovascular anomalies, and eye anomalies. When ventral developmental defects such as sternal clefting or supraumbilical raphe occur, the PHACES acronym may be used. The hallmark feature of PHACE is the presence of one or more large facial infantile hemangiomas that occupy at least one facial segment. Infantile hemangiomas differ from the capillary malformation (port wine stain) of Sturge-Weber syndrome, and the arteriovenous malformation of Wyburn-Mason syndrome, distinguishing PHACE syndrome from other neurocutaneous disorders with red birthmarks. The true incidence of PHACE has not yet been established. Girls are more commonly affected than boys. Cerebral vascular anomalies are probably the most common extracutaneous feature. Given that several organ systems are involved, a multidisciplinary approach to disease surveillance and treatment is advised. PMID:26564079

  4. [Bilateral acute depigmentation of the iris syndrome].

    PubMed

    Portmann, A; Gueudry, J; Siahmed, K; Muraine, M

    2011-05-01

    Bilateral acute depigmentation of the iris syndrome (BADI syndrome) is a new clinical entity. Young females from 20 to 45 years of age are most commonly affected. It is characterized by bilateral nontransilluminating depigmentation of the iris stroma. During the acute phase, this clinical entity also combines with red painful eye, pigmentation of the trabecular meshwork, anterior chamber flare, circulating pigment, and pigmented deposit on the endothelium cornea. At the acute stage, the symptoms are controlled with topical corticosteroid treatment. The prognosis is good. We report a 41-year-old woman presenting with BADI syndrome. PMID:21531477

  5. Congenital bilateral perisylvian syndrome: a case study.

    PubMed

    Donders, Jacobus; Mullarkey, Sr Kathryn; Allchin, Joel

    2009-02-01

    We report the case of a 14-year-old boy with bilateral congenital perisylvian syndrome as evidenced by polymicrogyria in the left and right frontotemporal regions, pseudobulbar paresis, seizures, and spastic diplegia. This is the first description of comprehensive neuropsychological assessment in a child with this syndrome. The results reflect significant impairments in auditory processing and independent learning and retention, but relatively preserved cognitive performance on interactive tasks involving frequent feedback and redirection. The findings are interpreted in the context of the neuropathology of this syndrome, with an emphasis on pragmatic implications for rehabilitation. PMID:18618376

  6. Gorlin syndrome and bilateral ovarian fibroma

    PubMed Central

    Pirschner, Fernanda; Bastos, Pollyana Marçal; Contarato, George Luiz; Bimbato, Anna Carolina Bon Lima; Filho, Antônio Chambô

    2012-01-01

    INTRODUCTION Gorlin syndrome (GS), also known as nevoid basal cell carcinoma syndrome (NBCCS), is a rare hereditary, autosomal dominant disease that affects various systems. Its prevalence is estimated at 1/57,000 to 1/256,000 of the population. It is characterized by basal cell carcinomas, multiple odontogenic keratocysts, skeletal abnormalities and ovarian fibroma, among other disorders. PRESENTATION OF CASE To report the case of a young patient with Gorlin syndrome and bilateral ovarian fibroma. DISCUSSION A 20-year old patient with Gorlin syndrome presented with facial asymmetry, broad nasal root, dental abnormalities, micrognathism, convergent strabismus, multiple pigmented lesions on the trunk and face, pectus excavatum, kyphoscoliosis and a palpable mass in the abdomen occupying the entire pelvic region. CONCLUSION Gorlin–Goltz syndrome is a hereditary pathology that includes numerous clinical manifestations. Diagnosis is clinical and genetic confirmation is unnecessary. PMID:22771908

  7. Bilateral Parotid Swelling in Polycystic Ovarian Syndrome.

    PubMed

    Yakubov, Yakov; Mandel, Louis

    2016-05-01

    Polycystic ovarian syndrome (PCOS) is recognized by the presence of polycystic ovaries, irregular menstruation, and increased androgen levels. Many patients have insulin resistance or impaired glucose tolerance and an associated development of type 2 diabetes mellitus. A patient with PCOS is presented whose cosmetic concerns centered on the prolonged existence of substantial bilateral parotid swelling. The pathophysiology, diagnosis, and therapy of sialosis are discussed. PMID:26657398

  8. Acute bilateral spontaneous forearm compartment syndrome.

    PubMed

    Dalton, David M; Munigangaiah, Sudarshan; Subramaniam, Tava; McCabe, John P

    2014-01-01

    Acute spontaneous compartment syndrome of the forearm is rarely reported in the literature. It is typically associated with trauma or thromboembolism in the acute setting and repetitive exertional stress in the chronic setting. However it is rare for it to present bilaterally with no apparent underlying cause. We report the case of a young 31-year-old lady who presented to our Emergency Department with bilateral compartment syndrome of the forearm. Her presenting complaints included acute severe pain and swelling of the forearms bilaterally, with a decreased range of movement of the wrist and fingers. She also complained of numbness in all fingers. She had no history of recent trauma and ultrasound scans showed no evidence of vascular compromise. Past medical history was notable only for idiopathic hypertension and coeliac disease. The patient was taken to theatre urgently where flexor and extensor compartments and carpal tunnel were decompressed. Pronator Teres was found to be dusky initially but turned pink after decompression. All other muscles were normal. An interesting fact of this case was that combination of the high compartment pressures and anaesthetic related hypotension caused the forearm pulses to become impalpable at induction, these returned intra-operatively. The patient has been seen in the outpatient department following discharge. She is well apart from some mildly reduced grip strength in her right hand likely due to carpal tunnel decompression. No cause was found for the scenario after extensive medical investigation. PMID:24641749

  9. [Bilateral jugular thrombosis in Lemierre syndrome].

    PubMed

    Benhayoun, M; Llor, J; Van-Den-Abbeele, T; Elmaleh, M; Mariani, P; Beaufils, F; Dauger, S

    2003-12-01

    An adolescent presented with a rhinosinusitis complicated with bilateral jugular veins and left superior ophthalmic vein thrombosis and respiratory distress with pulmonary hypertension. Blood culture was positive for Haemophilus influenzae and sinus puncture for Streptococcus constellatus. Evolution was under control after 1 week of appropriate antibiotherapy, antithrombotic and anti-inflammatory treatment. He had no neurologic, respiratory or ophthalmologic sequelae 6 months later. Despite lack of pharyngitis or isolation of anaerobic species on blood cultures, the picture was considered compatible with Lemierre syndrome. The risk for such a complication should be considered in cases of severe otorhinolaryngologic infection in young adults. PMID:14643536

  10. Sturge-Weber syndrome with bilateral intracranial calcification.

    PubMed Central

    Boltshauser, E; Wilson, J; Hoare, R D

    1976-01-01

    Four children affected by Sturge-Weber syndrome and demonstrating bilateral intracranial calcification are described, bringing up to 21 the number of similar reported cases. The frequency of bilateral hemisphere involvement in this syndrome is not known, but it might be as high as 15%. If present, neurosurgical intervention is, in our opinion, contraindicated. Images PMID:932761

  11. Anton's Syndrome due to Bilateral Ischemic Occipital Lobe Strokes

    PubMed Central

    Zukić, Sanela; Sinanović, Osman; Hodžić, Renata; Mujagić, Svjetlana; Smajlović, Edina

    2014-01-01

    We present a case of a patient with Anton's syndrome (i.e., visual anosognosia with confabulations), who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton's syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries. PMID:25530893

  12. Anton's Syndrome due to Bilateral Ischemic Occipital Lobe Strokes.

    PubMed

    Zukić, Sanela; Sinanović, Osman; Zonić, Lejla; Hodžić, Renata; Mujagić, Svjetlana; Smajlović, Edina

    2014-01-01

    We present a case of a patient with Anton's syndrome (i.e., visual anosognosia with confabulations), who developed bilateral occipital lobe infarct. Bilateral occipital brain damage results in blindness, and patients start to confabulate to fill in the missing sensory input. In addition, the patient occasionally becomes agitated and talks to himself, which indicates that, besides Anton's syndrome, he might have had Charles Bonnet syndrome, characterized by both visual loss and hallucinations. Anton syndrome, is not so frequent condition and is most commonly caused by ischemic stroke. In this particular case, the patient had successive bilateral occipital ischemia as a result of massive stenoses of head and neck arteries. PMID:25530893

  13. Leg Weakness Caused by Bilateral Piriformis Syndrome: A Case Report

    PubMed Central

    Moon, Hee Bong; Kwon, Bum Sun; Park, Jin Woo; Ryu, Gi Hyeong; Lee, Ho Jun; Kim, Chang Jae

    2015-01-01

    Piriformis syndrome (PS) is an uncommon neuromuscular disorder caused by the piriformis muscle (PM) compressing the sciatic nerve (SN). The main symptom of PS is sciatica, which worsens with certain triggering conditions. Because the pathophysiology is poorly understood, there are no definite diagnostic and therapeutic choices for PS. This case report presents a young woman who mainly complained of bilateral leg weakness. Electromyography revealed bilateral sciatic neuropathy and magnetic resonance imaging confirmed structural lesions causing entrapment of the bilateral SNs. After a laborious diagnosis of bilateral PS, she underwent PM releasing surgery. Few PS cases present with bilateral symptoms and leg weakness. Therefore, in such cases, a high level of suspicion is necessary for accurate and prompt diagnosis and treatment. PMID:26798622

  14. Bilateral Horner's syndrome in cluster type headaches.

    PubMed

    Khurana, R K

    1993-09-01

    A patient with cluster type headaches demonstrated bilateral and alternating ocular sympathetic dysfunction during a spontaneous as well as a nitroglycerin-induced attack. Biochemical evaluation revealed postganglionic pupillary dysfunction on the symptomatic side and preganglionic pupillary dysfunction contralaterally. These findings defy a simple explanation regarding a central or peripheral origin of the oculocephalic sympathetic dysfunction. PMID:8262788

  15. Progressive "vascular" corticobasal syndrome due to bilateral ischemic hemispheric lesions.

    PubMed

    Kim, Young-Do; Kim, Joong-Seok; Lee, Eek-Sung; Yang, Dong-Won; Lee, Kwang-Soo; Kim, Yeong-In

    2009-01-01

    We report a patient that presented with the corticobasal syndrome (CBS), including progressive dementia, asymmetric parkinsonism associated with constructional and ideomotor apraxia, action myoclonus and focal hand dystonia. Magnetic resonance imaging of the brain revealed extensive ischemic lesions in both fronto-temporo-parieto-occipital lobes and steno-occlusion of the middle cerebral arteries, bilaterally. This case illustrates that extensive cortical vascular-ischemic lesions may present with symptoms mimicking the corticobasal syndrome. PMID:19755778

  16. Bilateral renal dysplasia, nephroblastomatosis, and bronchial stenosis. A new syndrome?

    PubMed

    Rodriguez, Maria Matilde; Correa-Medina, Mayrin; Whittington, Elizabeth E

    2015-06-01

    Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith-Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB. PMID:25871299

  17. Bilateral Renal Dysplasia, Nephroblastomatosis, and Bronchial Stenosis. A New Syndrome?

    PubMed Central

    Rodriguez, Maria Matilde; Correa-Medina, Mayrin; Whittington, Elizabeth E.

    2015-01-01

    Bilateral nephroblastomatosis (NB) is an uncommon renal anomaly characterized by multiple confluent nephrogenic rests scattered through both kidneys, with only a limited number of cases reported in the medical literature. Some of these children may have associated either Perlman or Beckwith–Wiedemann syndrome and others do not demonstrate syndromic features. We report a full-term boy with anteverted nose, bilateral bronchial stenosis due to lack of cartilage, bilateral obstructive renal dysplasia and NB with glomeruloid features. The infant had visceromegaly, but neither gigantism nor hemihypertrophy. Immunohistochemistry for PAX2 (Paired box gene-2) and WT-1 (Wilms Tumor 1) were strongly positive in the areas of NB. GLEPP-1 (Glomerular Epithelial Protein) did not stain the areas of NB with a glomeruloid appearance, but was positive in the renal glomeruli as expected. We found neither associated bronchial stenosis nor the histology of NB resembling giant glomeruli in any of the reported cases of NB. PMID:25871299

  18. Ehlers-Danlos Syndrome Type IV with Bilateral Pneumothorax.

    PubMed

    Nakagawa, Hiroaki; Wada, Hiroshi; Hajiro, Takashi; Nagao, Taishi; Ogawa, Emiko; Hatamochi, Atsushi; Tanaka, Toshihiro; Nakano, Yasutaka

    2015-01-01

    A 17-year-old teen was hospitalized with bilateral pneumothorax. After the bilateral lungs were expanded using catheter tubes, he fully recovered and he was discharged from our hospital. He had a history of colon perforation. Ehlers-Danlos syndrome (EDS) was suspected due to the combination of colon perforation and pneumothorax, and EDS type IV was confirmed after a genetic study identified a c.1511g>a mutation in the COL3A1 gene. This is the first report of bilateral pneumothorax caused by EDS type IV. Clinicians should consider EDS type IV in the differential diagnosis for bilateral pneumothorax in conjunction with distinct previous histories and radiological findings. PMID:26666608

  19. Mounier-Kuhn syndrome and bilateral vocal cord paralysis.

    PubMed

    Dincer, H Erhan; Holweger, Joshua D

    2012-07-01

    Mounier-Kuhn syndrome is a rare disorder of unknown cause that is characterized by atrophy of the elastic and smooth muscle of the tracheobronchial tree leading to tracheobronchomegaly and bronchiectasis. The syndrome is likely underdiagnosed, because the patients usually present with common respiratory symptoms such as productive cough and usually labeled as chronic obstructive pulmonary disease. Diagnosis is established on the basis of radiologic findings. Association with bilateral vocal cord paralysis has not been described. Treatment is mainly supportive. Symptomatic patients may require endobronchial stenting if airway collapse is encountered. Here, we described a patient who presented with hoarseness and pneumonia. Further studies confirmed the diagnosis of Mounier-Kuhn syndrome with bilateral vocal cord paralysis. PMID:23207474

  20. Bow hunter's syndrome secondary to bilateral dynamic vertebral artery compression.

    PubMed

    Healy, Andrew T; Lee, Bryan S; Walsh, Kevin; Bain, Mark D; Krishnaney, Ajit A

    2015-01-01

    Bow hunter's syndrome is a condition in which vertebrobasilar insufficiency is resultant from head rotation, clinically manifested by presyncopal sensation, syncope, dizziness, and nausea. It is usually diagnosed clinically, with supporting vascular imaging demonstrating an occluded or at the very least compromised unilateral vertebral artery, while the dominant vertebral artery remains patent in the neutral position. Dynamic imaging is utilized to confirm the rotational compression of the dominant vertebral artery. We present the rare case of a patient with typical Bow hunter's symptoms, bilaterally patent vertebral arteries on neutral imaging, and bilateral compromise with head rotation. Our patient underwent posterior decompression of the culprit atlanto-axial transverse foramen and subaxial cervical fusion, with resolution of his symptoms. Our patient exemplifies the possibility of bilateral dynamic vertebral artery occlusion. We show that Bow hunter's syndrome cannot be ruled out in the setting of bilaterally patent vertebral arteries on neutral imaging and that severe cervical spondylosis should impart further clinical suspicion of this unusual phenomenon. PMID:25070633

  1. Bilateral Ramsay Hunt syndrome in a diabetic patient.

    PubMed

    Syal, Rajan; Tyagi, Isha; Goyal, Amit

    2004-12-01

    BACKGROUND: Herpes zoster oticus accounts for about 10% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. Bilateral herpes zoster oticus can sometime occur in immunocompromised patients, though incidence is very rare. CASE PRESENTATION: Diabetic male, 57 year old presented to us with bilateral facial palsy due to herpes zoster oticus. Patient was having bilateral mild to moderate sensorineural hearing loss. Patient was treated with appropriate metabolic control, anti-inflammatory drugs and intravenous acyclovir. Due to uncontrolled diabetes, glucocorticoids were not used in this patient. Significant improvement in hearing status and facial nerve functions were seen in this patient. CONCLUSIONS: Herpes zoster causes severe infections in diabetic patients and can be a cause of bilateral facial palsy and bilateral Ramsay Hunt syndrome. Herpes zoster in diabetic patients should be treated with appropriate metabolic control, NSAIDS and intravenous acyclovir, which we feel should be started at the earliest. Glucocorticoids should be avoided in diabetic patients. PMID:15575957

  2. Bilateral Ramsay Hunt syndrome in a diabetic patient

    PubMed Central

    Syal, Rajan; Tyagi, Isha; Goyal, Amit

    2004-01-01

    Background Herpes zoster oticus accounts for about 10% cases of facial palsy, which is usually unilateral and complete and full recovery occurs in only about 20% of untreated patients. Bilateral herpes zoster oticus can sometime occur in immunocompromised patients, though incidence is very rare. Case presentation Diabetic male, 57 year old presented to us with bilateral facial palsy due to herpes zoster oticus. Patient was having bilateral mild to moderate sensorineural hearing loss. Patient was treated with appropriate metabolic control, anti-inflammatory drugs and intravenous acyclovir. Due to uncontrolled diabetes, glucocorticoids were not used in this patient. Significant improvement in hearing status and facial nerve functions were seen in this patient. Conclusions Herpes zoster causes severe infections in diabetic patients and can be a cause of bilateral facial palsy and bilateral Ramsay Hunt syndrome. Herpes zoster in diabetic patients should be treated with appropriate metabolic control, NSAIDS and intravenous acyclovir, which we feel should be started at the earliest. Glucocorticoids should be avoided in diabetic patients. PMID:15575957

  3. Bilateral adrenalectomy for Cushing's syndrome: Pros and cons

    PubMed Central

    Prajapati, O. P.; Verma, A. K.; Mishra, A.; Agarwal, G.; Agarwal, A.; Mishra, S. K.

    2015-01-01

    Aim: To assess the outcome of patients undergoing bilateral adrenalectomy for Cushing's syndrome (CS). Methods: All patients who underwent bilateral adrenalectomy for CS at the Department of Endocrine Surgery, Sanjay Gandhi Postgraduate Institute of Medical Sciences hospital between 1991 and 2013 were included. Medical records were reviewed to obtain patient characteristics and follow-up data. Results: Twenty-seven patients were studied. Mean age was 28.74 ± 12.95 years (range 9–60), male:female ratio was 1.7:1. About half that is, 48.19% were of Cushing's disease (failed trans-sphenoidal surgery [TSS]), 37.04% were of ectopic CS (ECS), and 14.81% were of CS due to bilateral adrenal pathology. Median follow-up period was 80.5 months. Before surgery, 74.1% patients had body mass index > which after surgery declined to <25 in 75% of them. Hypertension was present in 85.2% and after surgery resolved in 40%. Diabetes mellitus was present in 44.4% and after surgery resolved in 33% of them. Hirsutism and proximal muscle weakness were present in 55.6% and 70.4% patients, respectively, and after surgery improved markedly in all patients. Adrenal crisis developed in 36.3% and Nelson's syndrome in 41.7% patients during follow-up. Three patients died in perioperative period while three succumbed to the disease during follow-up. Two patients developed recurrence of endogenous cortisol production during the follow-up period. Conclusions: Bilateral adrenalectomy is a valid treatment option for palliating severe symptoms in Pituitary Cushing's with failed TSS and unlocalized ECS but the procedure is curative for CS due to bilateral adrenal disease. Overall morbidity and mortality is higher than other endocrine operations. Co-morbidities tend to be more severe and are a risk factor for mortality during the time patient survives. PMID:26693437

  4. Bilateral pial synangiosis in a child with PHACE syndrome.

    PubMed

    Jack, Andrew S; Chow, Michael M; Fiorillo, Loretta; Chibuk, Thea; Yager, Jerome Y; Mehta, Vivek

    2016-01-01

    The acronym PHACE has been used to denote a constellation of abnormalities: posterior fossa anomalies, facial hemangiomas, arterial anomalies, cardiac anomalies, and eye abnormalities. Approximately 30% of patients with large facial hemangiomas have PHACE syndrome, with the vast majority having intracranial arteriopathy. Few reports characterize neurological deterioration from this intracranial arteriopathy, and even fewer report successful treatment thereof. The authors report on a case of a child with PHACE syndrome who presented with an ischemic stroke from a progressive intracranial arteriopathy and describe her successful treatment with bilateral pial synangiosis. An 8-month old girl diagnosed with PHACE syndrome was found to have bilateral internal carotid artery stenosis. Although initially asymptomatic, a few months after diagnosis she suffered a right frontal and parietal stroke. MRI and cerebral angiography investigations demonstrated progressive intracranial arterial stenosis and occlusion. The patient then underwent indirect cerebral revascularization surgery. At 2-year follow-up, she exhibited clinical improvement with persistent speech and motor developmental delay. Follow-up MRI and cerebral angiography showed no new ischemic events and robust extensive vascular collateralization from surgery. PHACE syndrome is an uncommon disease, and affected patients often have cerebral arteriopathy. Although the underlying natural history of cerebral arteriopathy in PHACE remains unclear, cerebral revascularization may represent a potential therapy for symptomatic patients. PMID:26405843

  5. Bilateral Anterior Opercular Syndrome With Partial Kluver–Bucy Syndrome in a Stroke Patient: A Case Report

    PubMed Central

    2016-01-01

    Bilateral anterior opercular syndrome and partial Kluver–Bucy syndrome are associated with bilateral middle cerebral artery lesions. The combination of these two syndromes has only been reported in a child with limbic encephalitis. In this case, a 44-year-old woman with bilateral middle cerebral artery infarction, which occurred 2 years prior, could walk independently. However, she showed automatic-voluntary dissociation and anarthria with preserved writing skills. She also presented hypersexuality, hypermetamorphosis, and memory disturbances. Here, we report a case of an adult stroke patient who suffered from bilateral anterior opercular syndrome accompanied by partial Kluver–Bucy syndrome. PMID:27446793

  6. Bilateral Anterior Opercular Syndrome With Partial Kluver-Bucy Syndrome in a Stroke Patient: A Case Report.

    PubMed

    Cho, Ah-Ra; Lim, Young-Ho; Chung, Sae-Hoon; Choi, Eun-Hi; Lim, Jong Youb

    2016-06-01

    Bilateral anterior opercular syndrome and partial Kluver-Bucy syndrome are associated with bilateral middle cerebral artery lesions. The combination of these two syndromes has only been reported in a child with limbic encephalitis. In this case, a 44-year-old woman with bilateral middle cerebral artery infarction, which occurred 2 years prior, could walk independently. However, she showed automatic-voluntary dissociation and anarthria with preserved writing skills. She also presented hypersexuality, hypermetamorphosis, and memory disturbances. Here, we report a case of an adult stroke patient who suffered from bilateral anterior opercular syndrome accompanied by partial Kluver-Bucy syndrome. PMID:27446793

  7. Potocki-Lupski syndrome in conjunction with bilateral clubfoot.

    PubMed

    Dhanaraj, Dinesh; Chu, Alice; Pappas, John G; Moran, Ellen; Lehman, Wallace B

    2015-07-01

    Potocki-Lupski syndrome (PTLS) is a rare chromosomal microduplication syndrome resulting in multiple congenital abnormalities including developmental delays, autistic features, and certain structural anomalies, with cardiovascular being the most common. The phenotype of this contiguous gene duplication syndrome is quite variable and may include musculoskeletal abnormalities. Given the infrequency and novelty of this disorder, full phenotypic characterization of PTLS has not yet been fully elucidated. We present a case of severe bilateral clubfoot in a patient with PTLS. Diagnosis was made by array-based comparative genomic hybridization and confirmed by fluorescence in-situ hybridization. Because clubfoot was also present in an apparently unaffected brother, the presence of PTLS may have acted as a modifier of the phenotype. This report highlights the complex interaction of chromosomal and familial factors that contribute to musculoskeletal birth defects. PMID:25768679

  8. Bilateral agenesis of arcuate fasciculus demonstrated by fiber tractography in congenital bilateral perisylvian syndrome.

    PubMed

    Kilinc, Ozden; Ekinci, Gazanfer; Demirkol, Ezgi; Agan, Kadriye

    2015-03-01

    Congenital bilateral perisylvian syndrome (CBPS) is a type of cortical developmental abnormality associated with distinctive clinical and imaging features. Clinical spectrum of this syndrome is quite heterogeneous, with different degrees of neurological impairment in affected individuals. High-definition magnetic resonance imaging (MRI) has a great importance in revealing the presence of CBPS, but is limited in elucidating the heterogeneous clinical spectrum. The arcuate fasciculus (AF) is a prominent language tract in the perisylvian region interconnecting Broca and Wernicke areas, and has a high probability of being affected developmentally in CBPS. Herein, we report a case of CBPS with investigation of AF using diffusion tensor imaging (DTI) and fiber tractography in relation to clinical findings. We postulated that proven absence of AF on DTI and fiber tractography would correlate with a severe phenotype of CBPS. PMID:24852949

  9. Bilateral oculomotor nerve palsy in Guillain-Barre syndrome.

    PubMed

    Burina, Adnan; Sinanović, Osman; Smajlović, Dzevdet; Vidović, Mirjana

    2008-01-01

    Guillain-Barre syndrome (GBS) is an acquired immune-mediated inflammatory disorder of the peripheral nervous system. GBS is also called acute idiopathic polyradiculoneuritis. Cranial nerves are affected in over 50% of all cases, with the facial nerves being affected the most. Otherwise, oculomotor nerves affection is rare and might occur in about 10% of cases. In this case report we present 61 years old female with GBS (acute motor and sensory axonal neuropathy subtype) associated with bilateral oculomotor nerve palsy. At the admittance in the neurological status were flaccid paraplegia, tendon reflexes absent at legs and reduced at arms, sensory disturbances in a distal (stocking-glove) distribution and bilateral ptosis. The disease was diagnosed on clinical features, nerve conduction velocity test (NCV), electromyogram (EMG) and cerebrospinal fluid (CSF) tests. After treatment with intravenous immunoglobulins and physical treatment the patient improved. She was able to walk by her own, mild semiptosis remained and she had no paresthesia. PMID:18669237

  10. Bilateral medial medullary syndrome secondary to Takayasu arteritis.

    PubMed

    Deshpande, Anirudda; Chandran, Vijay; Pai, Aparna; Rao, Suryanarayana; Shetty, Ranjan

    2013-01-01

    Medial medullary syndrome (MMS) is a rare type of stroke which results due to occlusion of the anterior spinal artery or vertebral artery or its branches. In this case report we present a patient who developed MMS secondary to Takayasu arteritis (TA). TA is a chronic inflammatory arteritis primarily involving the arch of aorta and its branches, which in our patient resulted in occlusion of subclavian arteries as well as infarction of the medial medulla bilaterally. To our knowledge this is the first time that MMS has been found to occur secondary to TA. PMID:23943806

  11. [Bilateral tibial compartment syndrome with crush symptoms after cannabis abuse].

    PubMed

    Huber, F X; Eckstein, H H; Allenberg, J R

    1997-08-01

    Acute compartmental syndrome (CS) is a surgical emergency. Different conditions in which high non-physiological pressure appears within a closed fascial space reduce the necessary blood perfusion. CS is caused by trauma, burns, bleeding in patients with coagulopathies arterial injuries, nephrotic syndrome or unusual physical exercise with secondary compartmental swelling. When decompression occurs too late, permanent loss of function and limb contracture may result. In the following paper we report on a case of four-compartmental syndrome in the lower legs of a patient with drug intoxication. After cannabis consumption, the patient fell asleep sitting cross-legged. During our first examination several hours later, the signs of compartmental syndrome with spontaneous pain, turgid swelling and paresis were present. Bilateral skin incision technique was used to gain entrance into the four compartments in both lower legs. Immediately after the operation, the patient showed crush syndrome with high serum creatine kinase activity 140.501 U/l and acute renal failure caused by rhabdomyolysis. Within 2 weeks of haemofiltration and dialysis, a full recovery to a normal serum creatinine level of 0.7 mg/dl was achieved. After emergency treatment and rehabilitation, the patient showed neither vascular nor neural defects. PMID:9377997

  12. Bilaterally Symmetrical Lower Extremity Compartment Syndrome following Massive Transfusion

    PubMed Central

    Karaoren, Gulsah; Bakan, Nurten; Tomruk, Senay Goksu; Topaç, Zelin; Kurtulmuş, Tuhan; Irkören, Saime

    2016-01-01

    Compartment syndrome is a serious condition characterized by raised intracompartmental pressure, which develops following trauma. Well leg compartment syndrome (WLCS) is a term reserved for compartment syndrome in a nontraumatic setting, usually resulting from prolonged lithotomy position during surgery. In literature, 8 cases have been reported regarding well leg compartment syndrome in a supine position and bilateral symmetrical involvement was observed in only 2 cases. In WLCS etiology, lengthy surgery, lengthy hypotension, and extremity malpositioning have been held responsible but one of the factors with a role in the etiology may have been the tissue oedema and impaired microcirculation formed from the effect of vasoactive mediators expressed into the circulation associated with the massive blood transfusion. The case is presented here regarding symmetrical lower extremity compartment syndrome after surgery in which massive transfusion was made for gross haemorrhage from an abdominal injury. In conclusion, blood transfusion applied at the required time is life-saving but potential risks must always be considered. PMID:26885421

  13. Spontaneous bilateral bacterial empyema in a patient with nephrotic syndrome.

    PubMed

    Chen, Wan-Chin; Huang, Jenq-Wen; Chen, Kuan-Yu; Hsueh, Po-Ren; Yang, Pan-Chyr

    2006-09-01

    Spontaneous bacterial empyema (SBEM), a rare infectious complication among liver cirrhosis patients, is characterized by infection in the presence of pleural effusion without evidence of pre-existing pneumonia. The prevalence of SBEM in cirrhotic patients with hydrothorax is about 13%. However, it has previously not been reported in medical literature in patients with nephrotic syndrome. The most common microorganism identified is Escherichia coli, followed by Streptococcus species, Enterococcus species, Klebsiella pneumoniae, and Pseudomonas stutzeri. We present a patient with a history of nephrotic syndrome caused by membranous nephropathy, who received steroids and cyclophosphamide. He developed bilateral SBEM due to Aeromonas hydrophila and E. coli, which were isolated from the left- and right-side pleural fluid, respectively. The detailed clinical course, treatment, and outcome are described. PMID:16457891

  14. Severe ovarian hyperstimulation syndrome coexisting with a bilateral ectopic pregnancy.

    PubMed

    Shiau, Chii-Shinn; Chang, Ming-Yang; Chiang, Chi-Hsin; Hsieh, Ching-Chang; Hsieh, Tsang-Tang

    2004-02-01

    Management of severe ovarian hyperstimulation syndrome (OHSS) includes hospitalization for fluid and electrolyte management. Abdominal paracentesis is also used as minimally invasive form of management in selected cases of severe OHSS following ovulation induction. However, if pregnancy ensues, the syndrome persists for a longer period, and the clinical manifestations of severe OHSS could mask the picture of a bleeding gestational sac. It could be easily overlooked unless the possibility of an ectopic pregnancy is kept in mind in cases of severe OHSS exacerbated by early pregnancy with or without a previous ectopic pregnancy history. We report a case of severe OHSS with simultaneous bilateral tubal pregnancy following intrauterine insemination (IUI). A 31-year-old woman with polycystic ovarian disease developed severe OHSS during the therapeutic course of IUI. An emergent exploratory laparotomy was performed 14 days after admission, and the operative findings showed persistent profuse bleeding from the bilateral fimbrial ends with marked enlargement of the ampullary portions. A linear salpingotomy was performed by a longitudinal incision along the area of maximal distension of the dilated fallopian tubes to preserve her fertility. We recommend that in cases of severe OHSS exacerbated by early pregnancy, serial serum beta-hCG and transvaginal ultrasound follow-up may be necessary due to the potential association of severe OHSS in pregnancy with an ectopic pregnancy. PMID:15095961

  15. Sneddon's syndrome presenting with severe disabling bilateral headache.

    PubMed

    Cavestro, Cinzia; Richetta, Luca; Pedemonte, Enrico; Asteggiano, Giovanni

    2009-06-01

    Sneddon's syndrome is a rare vascular disease affecting mainly skin and brain arterioles leading to their occlusion due to excessive endothelial proliferation. The two main features of this syndrome are livedo reticularis and lacunar subcortical infarcts. Here, we describe the case of a 64-year-old woman presenting with a 4-year history of a throbbing, bilateral, parieto-occipital headache associated with facial pain, but without any other accompanying symptom. The pain, initially misdiagnosed as atypical trigeminal neuralgia, worsened up to chronic daily and such severely disabling headache that she was constrained to bed. She presented with reduced cognitive functions, diffuse and severe livedo reticularis, severe myalgias and mild stiffness. All diagnostic test for different diseases were performed and other diseases excluded except for Sneddon's syndrome. Her symptoms were reduced firstly using acetylsalicylic acid, then ticlopidine 250 mg bid was begun and then Pentoxyphillin, resulting in a significant improvement of symptoms with the disappearance of headache. Her worsening in the first year was characterized by obsessive-compulsive behaviours, body-image misperceptions and panic attacks, improved for a period using olanzapine. Considering this case, we remark the importance of using headache classification to avoid diagnostic errors, secondly, we describe an atypical manifestation of Sneddon's syndrome and therapeutic efficacy of using ticlopidine and pentoxyphillin. PMID:19288055

  16. A Traumatic Bilateral Anterolateral Compartment Syndrome and Subsequent small Bowel Obstruction in a Patient with Schizophrenia

    PubMed Central

    Kapur, Benjamin Pal; Grant, Michael; Ramakrishnan, Muthukrishnan

    2015-01-01

    Introduction: A traumatic bilateral compartment syndrome is not widely reported. There is usually a precipitating event to cause compartment syndrome for example open and closed fractures, plaster of Paris application, burns and post-ischaemia reperfusion injury. This case confirms the need for a high index of suspicion for compartment syndrome in a patient presenting with bilateral leg pain, swelling and erythema as early diagnosis and urgent decompression by fasciotomy is of vital importance to preserve limb function and avoid complications. Case Report: We wish to report the case of atraumatic bilateral anterolateral compartment syndrome in a 58-year-old Caucasian man with a medical history of schizophrenia. He presented to Accident and Emergency with bilateral leg pain, swelling and erythema with no preceding history of trauma. Initially he was treated for bilateral lower leg cellulitis with a late diagnosis of compartment syndrome. Conclusion: This case illustrates the need for a broad differential diagnosis. PMID:27299031

  17. Posterior Reversible Encephalopathy Syndrome with Bilateral Independent Epileptic Foci Precipitated By Guillain-Barrè Syndrome.

    PubMed

    Rossi, Rosario; Saddi, Maria Valeria; Mela, Alessandro; Ticca, Anna

    2016-01-01

    We report the case of a 56-year-old woman who developed status epilepticus (SE) related to independent occipital foci as clinical manifestation of posterior reversible encephalopathy syndrome (PRES) in the background of Guillain-Barrè syndrome (GBS). SE resulted from a series of focal seizures clinically characterized by left- and rightward deviations of the head and consequent oculoclonic movements. Electroencephalography recorded independent seizure activity in both occipital regions with alternate involvement of the two cerebral hemispheres. The epileptic foci corresponded topographically to parenchymal abnormalities of PRES in the occipital lobes. The manifestation of bilateral, independent occipital seizures with alternate deviations of the head and oculoclonic movements, previously not reported in patients with PRES, highlights the acute epileptogenicity of the cerebral lesions in this syndrome. Despite the variable clinical expression of seizures due to occipital damage in PRES, the development of independent seizure activity in both occipital lobes might represent a distinctive epileptic phenomenon of this encephalopathy. PMID:27403359

  18. Posterior Reversible Encephalopathy Syndrome with Bilateral Independent Epileptic Foci Precipitated By Guillain-Barrè Syndrome

    PubMed Central

    Rossi, Rosario; Saddi, Maria Valeria; Mela, Alessandro; Ticca, Anna

    2016-01-01

    We report the case of a 56-year-old woman who developed status epilepticus (SE) related to independent occipital foci as clinical manifestation of posterior reversible encephalopathy syndrome (PRES) in the background of Guillain-Barrè syndrome (GBS). SE resulted from a series of focal seizures clinically characterized by left- and rightward deviations of the head and consequent oculoclonic movements. Electroencephalography recorded independent seizure activity in both occipital regions with alternate involvement of the two cerebral hemispheres. The epileptic foci corresponded topographically to parenchymal abnormalities of PRES in the occipital lobes. The manifestation of bilateral, independent occipital seizures with alternate deviations of the head and oculoclonic movements, previously not reported in patients with PRES, highlights the acute epileptogenicity of the cerebral lesions in this syndrome. Despite the variable clinical expression of seizures due to occipital damage in PRES, the development of independent seizure activity in both occipital lobes might represent a distinctive epileptic phenomenon of this encephalopathy. PMID:27403359

  19. Bilateral coracoclavicular joints as a rare cause of bilateral thoracic outlet syndrome and shoulder pain treated successfully by conservative means.

    PubMed

    Singh, V K; Singh, P K; Balakrishnan, S K

    2009-06-01

    The coracoclavicular joint (CCJ) is a rare anomalous joint. Symptomatic CCJ, being an exceptional rarity, makes it difficult to formulate a standard set of practice or guidelines. We report a 50-year-old Indian man, a machine operator by profession, who experienced bilateral shoulder pain and arm paraesthesia for two years, and was diagnosed with bilateral CCJ. The symptoms gradually increased, affecting his daily activities. Dynamic magnetic resonance imaging revealed the compression of the brachial plexus in extreme shoulder abduction. After a thorough search of the literature, we retrieved four similar cases, all of them treated with individualised protocols. None of the cases was bilateral. The lack of clear evidence in any particular direction and the patient's medical condition prompted us to give a conservative trial, before embarking on more invasive methods. He showed rapid response to the conservative treatment with remission of all symptoms. To the best of our knowledge, this is the first reported case of bilateral symptomatic CCJ with bilateral thoracic outlet syndrome, that was managed conservatively. PMID:19551300

  20. Bilateral Laparoscopic Gonadectomy in a Patient With Complete Androgen Insensitivity Syndrome and Bilateral Sertoli-Leydig Cell Tumor: A Case Report and Brief Review of the Literature

    PubMed Central

    Asl Zare, Mohammad; Kalantari, Mahmood Reza; Asadpour, Amir Abbas; Kamalati, Ali

    2014-01-01

    Introduction: Complete androgen insensitivity syndrome (previously called testicular feminization) is specified by a 46 XY karyotype and negative sex chromatin, bilateral undescended testes, female genitalia appearance, and lack of mullerian derivatives. Case Presentation: A 28-year-old woman with complete (severe) androgen resistance underwent prophylactic laparoscopic bilateral gonadectomy because of the eventually increased risk of gonadal malignancy. Although the gonads appeared grossly normal, microscopic examination revealed bilateral well differentiated sertoli–leydig cell tumor (SLCT). Discussion: Our Medline search revealed that this is the first reported case of bilateral sertoli–leydig cell tumor (SLCT) in androgen insensitivity syndrome. PMID:25032133

  1. Bilateral Subclavian Vein Occlusion in a SAPHO Syndrome Patient Who Needed an Implantable Cardioverter Defibrillator.

    PubMed

    Ishizuka, Masato; Yamamoto, Yuko; Yamada, Shintaro; Maemura, Sonoko; Nakata, Ryo; Motozawa, Yoshihiro; Yamamoto, Keisuke; Takizawa, Masataka; Uozumi, Hiroki; Ikenouchi, Hiroshi

    2016-05-25

    A 79-year-old Asian man was hospitalized because of progressive exertional dyspnea with decreasing left ventricular ejection fraction and frequent non-sustained ventricular tachycardia. Pre-procedure venography for implantable cardioverter defibrillator (ICD) implantation showed occlusion of the bilateral subclavian veins. In consideration of subcutaneous humps in the sterno-clavicular area and palmoplantar pustulosis, we diagnosed him as having synovitis, acne, pustulosis, hyperostosis, osteitis (SAPHO) syndrome and speculated that it induced peri-osteal chronic inflammation in the sterno-clavicular area, resulting in occlusion of the adjacent bilateral subclavian veins. An automatic external defibrillator (AED) was installed in the patient's house and total subcutaneous ICD was considered. Venous thrombosis in SAPHO syndrome is not frequent but has been reported. To the best of our knowledge, this is the first case of bilateral subclavian vein occlusion in a SAPHO syndrome patient who needs ICD implantation. PMID:27181039

  2. Multiple Ocular and Systemic Disorders in Association with Bilateral Duane's Retraction Syndrome

    PubMed Central

    Zare, Mohammad Ali; Akbari, Mohammad Reza; Kiarudi, Mohammad Yaser; Mehrjardi, Hadi Zare

    2016-01-01

    Duane's retraction syndrome (DRS) is characterized by limitations in horizontal eye movements, globe retraction, and palpebral fissure narrowing on attempted adduction. This disorder is caused by a disturbance in innervation originating in the brain stem and represents <1% of all cases of strabismus. It is postulated that this syndrome is due to an insult during the early weeks (8–10 weeks) of pregnancy and is 10–20 times more frequently associated with other systemic congenital anomalies. This case report of bilateral DRS included bilateral iris-retinal coloboma and congenital heart disease, sensory hearing loss, and inguinal hernia. PMID:27555711

  3. A case of adrenal Cushing’s syndrome with bilateral adrenal masses

    PubMed Central

    Guo, Ya-Wun; Hwu, Chii-Min; Won, Justin Ging-Shing; Chu, Chia-Huei

    2016-01-01

    Summary A functional lesion in corticotrophin (ACTH)-independent Cushing’s syndrome is difficult to distinguish from lesions of bilateral adrenal masses. Methods for distinguishing these lesions include adrenal venous sampling and 131I-6β-iodomethyl-19-norcholesterol (131I-NP-59) scintigraphy. We present a case of a 29-year-old Han Chinese female patient with a history of hypercholesterolaemia and polycystic ovary syndrome. She presented with a 6month history of an 8kg body weight gain and gradual rounding of the face. Serial examinations revealed loss of circadian rhythm of cortisol, elevated urinary free-cortisol level and undetectable ACTH level (<5pg/mL). No suppression was observed in both the low- and high-dose dexamethasone suppression tests. Adrenal computed tomography revealed bilateral adrenal masses. Adrenal venous sampling was performed, and the right-to-left lateralisation ratio was 14.29. The finding from adrenal scintigraphy with NP-59 was consistent with right adrenal adenoma. The patient underwent laparoscopic right adrenalectomy, and the pathology report showed adrenocortical adenoma. Her postoperative cortisol level was 3.2μg/dL, and her Cushingoid appearance improved. In sum, both adrenal venous sampling and 131I-NP-59 scintigraphy are good diagnostic methods for Cushing’s syndrome presenting with bilateral adrenal masses. Learning points The clinical presentation of Cushing’ syndrome includes symptoms and signs of fat redistribution and protein-wasting features. The diagnosis of patients with ACTH-independent Cushing’s syndrome with bilateral adrenal masses is challenging for localisation of the lesion. Both adrenal venous sampling and 131I-NP-59 scintigraphy are good methods to use in these patients with Cushing’s syndrome presenting with bilateral adrenal masses. PMID:27252858

  4. Bilateral retinopathy, aplastic anaemia, and central nervous system abnormalities: a new syndrome?

    PubMed Central

    Revesz, T; Fletcher, S; al-Gazali, L I; DeBuse, P

    1992-01-01

    A male infant was found to have bilateral exudative retinopathy at 6 months of age. A month later severe aplastic anaemia was diagnosed, eventually leading to the infant's death. Additional features of this seemingly new syndrome were intrauterine growth retardation, fine sparse hair, fine reticulate skin pigmentation, ataxia because of cerebellar hypoplasia, cerebral calcifications, extensor hypertonia, and progressive psychomotor retardation. Images PMID:1404302

  5. 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.

  6. Cerebellar Ataxia with Bilateral Vestibulopathy: Description of a Syndrome and Its Characteristic Clinical Sign

    ERIC Educational Resources Information Center

    Migliaccio, Americo A.; Halmagyi, G. Michael; McGarvie, Leigh A.; Cremer, Phillip D.

    2004-01-01

    We report four patients with the syndrome of cerebellar ataxia with bilateral vestibulopathy (CABV) and, using search coil oculography, we validate its characteristic clinical sign, namely impairment of the visually enhanced vestibulo-ocular reflex (VVOR) or doll's head reflex. In our four patients, CABV began in the sixth decade of life; they are…

  7. Bilateral multifocal posterior pole lesions in Reiter syndrome

    PubMed Central

    Mansour, Ahmad M; Jaroudi, Mahmoud O; Medawar, Walid A; Tabbarah, Zuhayr A

    2013-01-01

    Reactive arthritis is associated with conjunctivitis or iritis. Rarely reactive arthritis is accompanied by permanent visual loss from macular infarction or foveal scarring. We present the case of a rheumatologist who had a sudden onset of skin lesions, arthritis of several joints and bilateral visual loss. Most of these manifestations resolved after a course of oral corticosteroids. However he was left with decreased vision in the left eye and multiple lesions in the fovea over a follow-up of 2 years. PMID:23576664

  8. Bilateral Giant Coronary Artery Aneurysms Complicated by Acute Coronary Syndrome and Cardiogenic Shock.

    PubMed

    Chiu, Peter; Lynch, Donald; Jahanayar, Jama; Rogers, Ian S; Tremmel, Jennifer; Boyd, Jack

    2016-04-01

    Giant coronary aneurysms are rare. We present a 25-year-old woman with a known history of non-Kawasaki/nonatherosclerotic bilateral coronary aneurysms. She was transferred to our facility with acute coronary syndrome complicated by cardiogenic shock. Angiography demonstrated giant bilateral coronary aneurysms and complete occlusion of the left anterior descending (LAD) artery. Emergent coronary artery bypass grafting was performed. Coronary artery bypass grafting is the preferred approach for addressing giant coronary aneurysms. Intervention on the aneurysm varies in the literature. Aggressive revascularization is recommended in the non-Kawasaki/nonatherosclerotic aneurysm patient, and ligation should be performed in patients with thromboembolic phenomena. PMID:27000621

  9. [Bilateral diaphragmatic paralysis due to Parsonage-Turner syndrome].

    PubMed

    Tissier-Ducamp, D; Martinez, S; Alagha, K; Charpin, D; Chanez, P; Palot, A

    2015-09-01

    We report the case of a 49-years-old patient who presented to the accident and emergency department with sudden onset dyspnea associated with acute shoulder pain. He was breathless at rest with supine hypoxemia. He had an amyotrophic left shoulder with localized paresis of the shoulder. Both hemi-diaphragms were elevated on chest X-rays. Pulmonary function tests showed a restrictive pattern and both phrenic nerve conduction velocities were decreased. At night, alveolar hypoventilation was evidenced by elevated mean capnography (PtcCO2: 57mmHg). Neuralgic amyotrophy, Parsonage-Turner syndrome was the final diagnosis. This syndrome is a brachial plexus neuritis with a predilection for the suprascapular and axillary nerves. Phrenic nerve involvement is rare but where present can be the most prominent clinical feature as in our case report. PMID:25534571

  10. Simultaneous Bilateral Spontaneous Pneumothorax Revealed Birt-Hogg-Dubè Syndrome

    PubMed Central

    Tamburrini, Alessandro; Sellitri, Francesco; Tacconi, Federico; Brancati, Francesco; Mineo, Tommaso Claudio

    2015-01-01

    Simultaneous bilateral spontaneous pneumothorax is a very rare clinical event, comprising approximately 1% of all spontaneous pneumothoraces. Clinical signs and symptoms may vary from mild chest pain and dyspnea to severe respiratory failure; nevertheless immediate treatment is mandatory as this condition can deteriorate and progress to tension pneumothorax. An underlying lung disease has been commonly described; in most istances primary or secondary tumors, interstitial diseases, and infectious diseases. Birt-Hogg-Dubè syndrome is a rare inherited disorder clinically characterized by multiple fibrofolliculomas, renal tumors, lung cysts, and, in ~24% of the patients, occurrence of spontaneous pneumothorax. In this case, we firstly report the concurrence of these rare conditions, as a patient presenting a simultaneous bilateral spontaneous pneumothorax was diagnosed with Birt-Hogg-Dubè syndrome based on the typical radiological findings and genetic testing of the folliculin gene located on chromosome 17. PMID:26346436

  11. Bilateral de quervain syndrome after aromatase inhibitor administration: a case report and review of the literature.

    PubMed

    Papadimitriou, Konstantinos; Kountourakis, Panteleimon; Morakis, Emmanouil; Vassiliou, Vassilios; Barbounis, Vasileios; Ardavanis, Alexandros

    2012-01-01

    Aromatase inhibitors are widely used as one of the main treatment options of both early and advanced hormone receptor-positive breast cancer in postmenopausal women. Unfortunately, musculoskeletal symptoms are often presented in patients treated with aromatase inhibitors (AIs), and, although the pathogenesis is unknown, postulated mechanisms have been described. Herein, to our knowledge, we present the first report of bilateral De Quervain syndrome related with AIs therapy with a review of the relevant literature. PMID:22567020

  12. Bilateral De Quervain Syndrome after Aromatase Inhibitor Administration: A Case Report and Review of the Literature

    PubMed Central

    Papadimitriou, Konstantinos; Kountourakis, Panteleimon; Morakis, Emmanouil; Vassiliou, Vassilios; Barbounis, Vasileios; Ardavanis, Alexandros

    2012-01-01

    Aromatase inhibitors are widely used as one of the main treatment options of both early and advanced hormone receptor-positive breast cancer in postmenopausal women. Unfortunately, musculoskeletal symptoms are often presented in patients treated with aromatase inhibitors (AIs), and, although the pathogenesis is unknown, postulated mechanisms have been described. Herein, to our knowledge, we present the first report of bilateral De Quervain syndrome related with AIs therapy with a review of the relevant literature. PMID:22567020

  13. Development of bilateral coronary artery aneurysms in a child with Noonan syndrome.

    PubMed

    Mauro, David M; Flors, Lucia; Hoyer, Andrew W; Norton, Patrick T; Hagspiel, Klaus D

    2016-03-01

    Noonan syndrome is a constellation of congenital malformations including heart defects, facial anomalies and short stature. The cardiovascular defects are variable and extensive, with the most common being pulmonary stenosis and hypertrophic cardiomyopathy. Coronary artery anomalies have only been reported in a few cases. We report a child with Noonan syndrome status post pulmonary stenosis and atrial septal defect repair, who developed bilateral coronary artery aneurysms. The aneurysms were diagnosed with both cardiac magnetic resonance imaging and coronary computed tomography angiography. There had been no evidence of them on a cardiac MR exam 5 years previously. PMID:26515448

  14. Bilateral Renal Tumour as Indicator for Birt-Hogg-Dubé Syndrome

    PubMed Central

    Johannesma, P. C.; van Moorselaar, R. J. A.; Horenblas, S.; van der Kolk, L. E.; Thunnissen, E.; van Waesberghe, J. H. T. M.; Menko, F. H.; Postmus, P. E.

    2014-01-01

    Birt-Hogg-Dubé (BHD) syndrome is a cancer disorder caused by a pathogenic FLCN mutation characterized by fibrofolliculomas, lung cysts, pneumothorax, benign renal cyst, and renal cell carcinoma (RCC). In this case we describe a patient with bilateral renal tumour and a positive familial history for pneumothorax and renal cancer. Based on this clinical presentation, the patient was suspected for BHD syndrome, which was confirmed after molecular testing. We discuss the importance of recognizing this autosomal dominant cancer disorder when a patient is presented at the urologist with a positive family history of chromophobe renal cell cancer or a positive familial history for renal cell cancer and pneumothorax. PMID:24772173

  15. A case of Cushing's syndrome due to bilateral cortisol-secreting adenomas with unilateral DHEAS oversecretion.

    PubMed

    Monno, Satoshi; Tachikawa, Takamitsu; Maekawa, Takashi; Sasano, Hironobu

    2015-01-01

    A 39-year-old Japanese woman presented with typical clinical symptoms of Cushing's syndrome, including amenorrhea and hirsutism, for 2 years. The results of her initial endocrine evaluation were consistent with ACTH-independent Cushing's syndrome due to bilateral adrenal masses (diameters of 3.1 cm and 2.4 cm on the left and right, respectively). Serum dehydroepiandrosterone levels were 6,901 ng/mL (normal range 230-2,660 ng/mL). Bilateral laparoscopic adrenalectomies were performed (left adrenalectomy first and right adrenalectomy 3 months later). Following the left adrenalectomy, the results of the endocrine evaluations were still consistent with a diagnosis of ACTH-independent Cushing's syndrome. Serum dehydroepiandrosterone sulphate levels, however, were below the normal range (143 ng/mL). Unexpectedly, the patient's menstruation resumed 2.5 months after the left adrenalectomy. Pathological examination of the resected glands showed bilateral adrenocortical adenomas, one on the left with a diameter of 3 cm, and two on the right with diameters of 0.7 cm and 1.3 cm. Immunohistochemical analysis revealed side chain cleavage, 17α-hydroxylase, 3β-hydroxysteroid dehydrogenase, and 21-hydroxylase immunoreactivity in each adenoma. Dehydroepiandrosterone-sulfotransferase immunoreactivity was pronounced in the left adenoma, less pronounced in one of the right adenoma and weak in the other right adenoma. These results were consistent with clinical endocrine findings. Ours is the first case of a patient with bilateral cortisol-secreting adenomas with unilateral oversecretion of dehydroepiandrosterone sulphate. Resumption of menstruation after the correction of the dehydroepiandrosterone-sulphate excess, despite persistent cortisol excess, indicates the importance of adrenal androgens for the regulation of the menstrual cycle. PMID:25736067

  16. Non-haemorrhagic, bilateral adrenal infarction in a patient with antiphospholipid syndrome along with lupus myocarditis.

    PubMed

    Batt, Nicholas Marinus; Malik, Dean; Harvie, Miranda; Sheth, Hemant

    2016-01-01

    A 40-year-old woman with antiphospholipid syndrome presented with a 5-day history of right upper quadrant (RUQ) pain, radiating posteriorly, associated with fever and vomiting. She was admitted 1-week prior with an upper respiratory infection and erythema multiforme. Clinical assessment revealed sepsis with RUQ tenderness and positive Murphy's sign. Laboratory results showed raised inflammatory markers, along with renal and liver impairment. CT showed bilateral adrenal infarction and inferior vena cava thrombus. The patient was managed for sepsis and started on heparin. Further immunological investigations revealed a diagnosis of systemic lupus erythematous, an exacerbation of which culminated in lupus myocarditis. This case illustrates the importance of promptly recognising adrenal insufficiency in patients with antiphospholipid syndrome and the possible causative agents, which require careful consideration and exclusion to prevent further thrombotic events. It also highlights the importance of undertaking imaging, namely CT, in patients with antiphospholipid syndrome presenting with abdominal pain as well as considering concomitant autoimmune conditions. PMID:27440855

  17. Bilateral Sensorineural Hearing Loss and Polyneuropathy in a Patient with Sweet's Syndrome

    PubMed Central

    Cala, Cather M.; Kole, Lauren; Sami, Naveed

    2015-01-01

    Sweet's syndrome is an inflammatory systemic disease which has been associated with various underlying causes. The disease can involve multiple areas of the body including the skin and neurological system. There have been only two cases which have described otological involvement. This report presents a patient who developed loss of hearing secondary to Sweet's syndrome after developing cutaneous involvement along with peripheral neuropathy. Despite the patient's skin and neuropathy noticing improvement with intravenous immunoglobulin and azathioprine, he required bilateral cochlear implants for partial recovery of his hearing loss. This case highlights the need to recognize Sweet's syndrome as a complicated disease process where the role of otolaryngologists is important in the multidisciplinary coordination of care in both diagnosis and treatment. PMID:26770858

  18. Microform holoprosencephaly with bilateral congenital elbow dislocation; increasing the phenotypic spectrum of Steinfeld syndrome.

    PubMed

    Jones, Gabriela E; Robertson, Lisa; Maniyar, Amit; Shammas, Christos; Phelan, Marie M; Vasudevan, Pradeep C; Tanteles, George A

    2016-03-01

    Steinfeld syndrome (MIM #184705) was first reported in 1982. It is characterised by holoprosencephaly and limb defects, however other anomalies may also be present. Following the initial description, three further cases have been reported in the literature. We report on a 23-year-old girl, with features of microform holoprosencephaly and bilateral congenital elbow dislocation in association with hypoplastic radial heads. She was identified to have a variant in the CDON gene inherited from her father who had ocular hypotelorism, but no other clinical features. We discuss the clinical features of Steinfeld syndrome, and broaden the phenotypic spectrum of this condition. Structural analysis suggests that this variant could lead to destabilisation of binding of CDON with hedgehog proteins. Further work needs to be done to confirm whether mutations in the CDON gene are the cause of Steinfeld syndrome. © 2016 Wiley Periodicals, Inc. PMID:26728615

  19. A delayed presentation of bilateral leg compartment syndrome following non-stop dancing.

    PubMed

    Jefferies, James Gordon; Carter, Tom; White, Tim Oliver

    2015-01-01

    We present the case of a young man with a 48 h delayed presentation of bilateral lower limb acute compartment syndrome (ACS) affecting the anterior compartments following an extended period of dancing at a music festival. On making the diagnosis of ACS, the patient was immediately taken to theatre for fasciotomies and compartmental decompression. Repeat look fasciotomies revealed further necrosis to the muscles of the anterior compartments bilaterally and, effectively, all the muscle bellies within the anterior compartments were excised. The patient has been left with a significant functional deficit and disability. This case highlights the importance of timely diagnosis of ACS as delay in presentation can impact significantly on subsequent functional outcome and quality of life. PMID:25786817

  20. Development of bilateral tension pneumothorax under anesthesia in a Boerhaave's syndrome patient: a case report

    PubMed Central

    Oh, Mi Kyung; Jeon, Woo Jae; Kwon, Yong Deok; Kim, Kyoung Hun

    2016-01-01

    A 33-year-old male visited the emergency room with abdominal pain which developed after a vomiting episode. Based on the pneumomediastinum findings from a chest radiograph and a contrast-enhanced chest and abdominal computed tomography scan, the patient was diagnosed with Boerhaave's syndrome. Preoperative radiologic findings showed no pneumothorax or pleural effusion. Once anesthesia was administered, the patient developed near complete cardiopulmonary collapse due to a bilateral tension pneumothorax, which was treated by bilateral thoracentesis, followed by chest tube insertion. Despite a left side rupture, the damaged right lung was unable to overcome single right ventilation, so the surgery was completed via right thoracotomy. The ruptured site was treated, and the patient was transferred to the intensive care unit. We discuss the anesthetic implications of this disease and how to prevent fatal complications. PMID:27066209

  1. Development of bilateral tension pneumothorax under anesthesia in a Boerhaave's syndrome patient: a case report.

    PubMed

    Oh, Mi Kyung; Jeon, Woo Jae; Cho, Sang Yun; Kwon, Yong Deok; Kim, Kyoung Hun

    2016-04-01

    A 33-year-old male visited the emergency room with abdominal pain which developed after a vomiting episode. Based on the pneumomediastinum findings from a chest radiograph and a contrast-enhanced chest and abdominal computed tomography scan, the patient was diagnosed with Boerhaave's syndrome. Preoperative radiologic findings showed no pneumothorax or pleural effusion. Once anesthesia was administered, the patient developed near complete cardiopulmonary collapse due to a bilateral tension pneumothorax, which was treated by bilateral thoracentesis, followed by chest tube insertion. Despite a left side rupture, the damaged right lung was unable to overcome single right ventilation, so the surgery was completed via right thoracotomy. The ruptured site was treated, and the patient was transferred to the intensive care unit. We discuss the anesthetic implications of this disease and how to prevent fatal complications. PMID:27066209

  2. Bilateral piriformis syndrome in two elite soccer players: Report of two cases.

    PubMed

    Zeren, B; Canbek, U; Oztekin, H H; İmerci, A; Akgün, U

    2015-12-01

    Piriformis syndrome, a relatively rare condition, is described as entrapment of a sciatic nerve at the level of the piriformis muscle. There have been a few reports of bilateral piriformis syndrome in literature. In this study, we present bilateral piriformis syndrome in two professional soccer players from different teams who are symptom free at last follow-up after surgery. In both patients, resting EMG records were read normal, however EMG recording during the activity revealed prolonged H-reflexes. Both patients had no relief from conservative treatment and rehabilitation, therefore surgical treatment was performed. Preoperative mean visual analogue scale (VAS) value was 7, and decreased to 3 at the sixth month follow-up visit and at the longer term follow-up, mean 85months (74-96) it was valued at 1. Both soccer players returned to their active sports lives in the sixth postoperative month. According to Benson's functional evaluation scale, in long-term follow-up, there have been excellent results and both patients resumed their professional carrier for many years (mean 7 years). PMID:26522381

  3. Bilateral Ptosis as the First Presentation of Guillain-Barre Syndrome

    PubMed Central

    TALEBIAN, Ahmad; SOLTANI, Babak; TALEBIAN, Motahhareh

    2016-01-01

    Objective Guillain-Barre syndrome (GBS) is the most common cause of acute weakness in children. It has multiple variant forms with different presentations. A rare initial sign is ptosis. In this study, we present a 10-year-old girl with bilateral ptosis without opthalmoplegia followed by a weakness in extremities with a favourable response to intravenous immunoglobulin. Due to the patient’s initial eyelid levators, myasthenia gravis was ruled out by a Tensilon test and electrophysiological studies. Our report highlights the possibility of GBS as a cause of isolated ptosis, especially in cases without ophthalmoplegia. PMID:27057192

  4. Parvovirus B19-induced acute bilateral carpal tunnel syndrome in twin girls.

    PubMed

    Sakallı, Hale; Baskın, Esra; Dener, Şefik

    2015-01-01

    We describe 2 cases of 6-year-old twin girls presenting with acute carpal tunnel syndrome (CTS) associated with human parvovirus B19 (HPV-B19) infection, as evidenced by serological data and detection of HPV-B19 DNA in blood with use of polymerase chain reaction (PCR). To our knowledge, this is the first time that HPV-B19 infection has been suggested as the causal agent of simultaneous acute bilateral CTS in twins, thus presenting the possibility that similar immunologic responses can be observed in twins during viral infections. PMID:26422355

  5. Concurrence of Stevens-Johnson Syndrome and Bilateral Parotitis after Minocycline Therapy

    PubMed Central

    Yoon, Jimi; Lee, Seung Hun; Kim, Tae-Heung; Choi, Deok-Jin; Kim, Jin-Pyeong; Yoon, Tae-Jin

    2010-01-01

    Minocycline is an antibiotic of tetracycline derivatives that is commonly used in the treatment of moderate to severe acne vulgaris. It has been reported to cause rare adverse events from mild cutaneous eruption to severe forms including drug-induced lupus, serum sickness-like reaction, and hypersensitivity reactions, etc. The risks of adverse events attributed to minocycline have not been ascertained reliably and there are concerns about the safety of minocycline which could possibly result in life-threatening events such as the Stevens-Johnson syndrome. Here we demonstrate an unusual case of Stevens-Johnson syndrome in conjunction with bilateral parotitis after the intake of minocycline in a Korean boy suggesting discreet use of the drug. PMID:21103193

  6. Genetics of primary bilateral macronodular adrenal hyperplasia: a model for early diagnosis of Cushing's syndrome?

    PubMed

    Drougat, Ludivine; Espiard, Stéphanie; Bertherat, Jerôme

    2015-10-01

    Long-term consequences of cortisol excess are frequent despite appropriate treatment after cure of Cushing's syndrome. This might be due to diagnostic delay, often difficult to reduce in rare diseases. The identification of a genetic predisposing factor might help to improve early diagnosis by familial screening. Primary bilateral macronodular adrenal hyperplasia (PBMAH) is a rare cause of Cushing's syndrome. Hypercortisolism in PBMAH is most often diagnosed between the fifth and sixth decades of life. The bilateral nature of the adrenocortical tumors and the occurrence of rare clear familial forms suggest a genetic origin. Indeed, a limited subset of PBMAH can be observed as part of multiple tumors syndromes due to alterations of the APC, Menin or Fumarate Hydratase genes. Rare variants of the phosphodiesterases PDE11A have been associated with PBMAH. The recent identification of ARMC5 germline alterations in 25-50% of PBMAH patients without obvious familial history or associated tumors opens new perspectives. ARMC5 alterations follow the model of a tumor suppressor gene: a first germline inactivating mutation of this 16p located gene is followed by a somatic secondary hit on the other allele (inactivating mutation or allelic loss). Functional studies demonstrate that ARMC5 controls apoptosis and steroid synthesis. The phenotype of index cases patients with the mutation seems more severe than the one of WT index cases. However, phenotype variability within a family is often observed. This review summarizes the genetics of PBMAH, focusing on ARMC5, which offer new perspectives for early diagnosis of Cushing's syndrome. PMID:26264719

  7. Bilateral Painful Ophthalmoplegia: A Case of Assumed Tolosa-Hunt Syndrome.

    PubMed

    Kastirr, Ilko; Kamusella, Peter; Andresen, Reimer

    2016-03-01

    We present the case of a man of 47 years with vertical and horizontal paresis of view combined with periorbital pain that developed initially on the right side but extended after 3-4 days to the left. Gadolinum uptaking tissue in the cavernous sinus was shown by MRI of the orbital region in the T1 spin echo sequence with fat saturation (SEfs) with a slice thickness of 2 mm. As no other abnormalities were found and the pain resolved within 72 hours of treatment with cortison a bilateral Tolosa-Hunt Syndrome (THS) was assumed. THS is an uncommon cause for Painful Ophthalmoglegia (PO) and only few cases of bilateral appearance have been reported. Even though the diagnostic criteria for THS oblige unilateral symptoms we suggest that in patients with bilateral PO THS should not be excluded as a differential diagnosis. Further more when using MRI to detect granulomatous tissue in the orbital region the chosen sequence should be T1 SEfs and slice thickness should possibly be as low as 2 mm, as granulomas are often no larger than 1-2 mm. PMID:27134970

  8. Desmoid tumors of the bilateral breasts in a patient without Gardner syndrome: a case report and review of literature.

    PubMed

    Brown, Charles S; Jeffrey, Brenda; Korentager, Richard; Hughes, Kenneth

    2012-08-01

    Desmoid tumors constitute 0.02% to 0.03% of all tumors. Consequently, few case reports exist for breast desmoids, even fewer identifying bilateral disease. We present a case report of a patient with bilateral breast desmoids and shoulder desmoid without evidence of FAP or Gardner syndrome. This case report explores the clinical, radiographic, pathologic, and treatment elements for desmoid tumors as well as a review of the literature. PMID:21629058

  9. Bilateral lambdoid and sagittal synostosis (BLSS): a unique craniosynostosis syndrome or predictable craniofacial phenotype?

    PubMed

    Hing, Anne V; Click, Eleanor S; Holder, Ursula; Seto, Marianne L; Vessey, Kyle; Gruss, Joseph; Hopper, Richard; Cunningham, Michael L

    2009-05-01

    Multisutural craniosynostosis that includes bilateral lambdoid and sagittal synostosis (BLSS) results in a very characteristic head shape with frontal bossing, turribrachycephaly, biparietal narrowing, occipital concavity, and inferior displacement of the ears. This entity has been reported both in the genetics literature as craniofacial dyssynostosis and in the surgical literature as "Mercedes Benz" syndrome. Craniofacial dyssynostosis was first described in 1976 by Dr. Neuhauser when he presented a series of seven patients with synostosis of the sagittal and lambdoid sutures, short stature, and developmental delay. Over the past 30 years nine additional patients with craniofacial dyssynostosis have been reported in the literature adding to the growing evidence for a distinct craniosynostosis syndrome. The term "Mercedes Benz" syndrome was coined by Moore et al. in 1998 due to the characteristic appearance of the fused sutures on three-dimensional CT imaging. In contrast to the aforementioned reported cases of craniofacial dyssynostosis, all three patients had normal development. Recently, there have been several case reports of patients with BLSS and distinct chromosomal anomalies. These findings suggest that BLSS is a heterogeneous disorder perhaps with syndromic, chromosomal, and isolated forms. In this manuscript we will present the largest series of patients with BLSS and review clinical, CT, and molecular findings. PMID:19396832

  10. Capgras Syndrome in a Patient with Parkinson's Disease after Bilateral Subthalamic Nucleus Deep Brain Stimulation: A Case Report

    PubMed Central

    Kyrtsos, Christina Rose; Stahl, Mark C.; Eslinger, Paul; Subramanian, Thyagarajan; Lucassen, Elisabeth B.

    2015-01-01

    Capgras syndrome is a delusional misidentification syndrome (DMS) which can be seen in neurodegenerative diseases such as Lewy body dementia and, to a lesser extent, in Parkinson's disease (PD). Here, we report the case of a 78-year-old man with a history of idiopathic PD who developed Capgras syndrome following bilateral subthalamic nucleus deep brain stimulation (DBS) implantation. As the risk of DMS has been related to deficits in executive, memory, and visuospatial function preoperatively, this case highlights the importance of continuing to improve patient selection for DBS surgery. Capgras syndrome is a rare potential complication of DBS surgery in PD patients with preexisting cognitive decline. PMID:26078747

  11. [Posterior leukoencephalopathy syndrome presenting with bilateral ataxie optique: a case report].

    PubMed

    Yamashiro, Kazuo; Kunoki, Makiko; Miura, Yoshiharu; Tomiyama, Junji; Mochizuki, Hideki; Mizuno, Yoshikuni

    2005-01-01

    We report a 45 years old right-handed woman who developed acute respiratory distress syndrome (ARDS) after the operation for rupture of an ovarian cyst. One week after the onset of ARDS, she presented visual disturbance and Gerstmann syndrome. MRI T2-weighted images demonstrated abnormal high intensity lesions involving the gray and white matter of the occipital lobes bilaterally extending to the parietal lobes. Based on these findings, she was diagnosed as having posterior leukoencephalopathy syndrome (PLES). Her neurologic symptoms gradually improved, however, she started to complain of difficulty in grasping a cup placed on her peripheral visual fields. Neurological examination revealed no visual disturbance, weakness or cerebellar ataxia. She could easily reach objects presented in the central visual field, however, she could not grasp objects presented peripheral visual fields, while she was looking straight ahead. This disorder was observed both in the right and left visual field, whether she used the right hand or the left hand. We thought she had ataxie optique of Garcin. She was noted to have bilateral both direct and crossed ataxie optique. Ataxie optique is characterized by disturbance in reaching objects presented in the peripheral visual field. The underlying pathophysiologic mechanism is believed to be disconnection of the fibers between the primary visual area and the angular gyrus at the parietooccipital junction. Crossed ataxie optique consisting of difficulty in reaching objects presented in the contralateral visual field believed to be caused by disconnection of crossed pathways of the corpus callosum. Ataxie optique in our patient can be explained by disconnection of both direct and crossed fibers. Ataxie optique is sometimes unrecognized by the patient. Our patient suggests that ataxie optique may well be a symptom of PLES. PMID:15714994

  12. [Bilateral phrenic nerve paralysis, dysautonomia and restrictive cardiomyopathy in a case of POEMS syndrome].

    PubMed

    Delalande, S; Stojkovic, T; Rose, C; Millaire, A; Hurtevent, J F; Vermersch, P

    2002-07-01

    We report a case of POEMS syndrome (Polyneuropathy, Organomegaly, Endocrinopathy, M protein and Skin changes) with unusual clinical features. A 62-year-old woman presented a severe polyneuropathy with dysphonia and vegetative symptoms, including bradycardia and sphincterial disorders. The clinical examination showed facial hyperpigmentation, cachexia, anasarca and splenomegaly. She also presented restrictive cardiomyopathy and endocrine disturbances. Nerve conduction studies revealed a severe demyelinating sensorimotor neuropathy. Cerebrospinal fluid analysis showed an elevated protein level. We detected a biclonal gammapathy (Ig G and Ig A with lambda light chain) and lytic pelvic bone lesions. Later, she developed a severe ventilatory failure due to a bilateral phrenic nerve paralysis leading to a mechanical ventilation. Steroids followed by localized radiotherapy partially improved the respiratory status and stabilized the neuropathy. Phrenic nerve paralysis, restrictive cardiomyopathy, vegetative symptoms and cranial nerve palsy are exceptional in POEMS syndrome. Moreover, this case emphasizes the importance of radiological investigations since the discover of plasmocytoma may improve the prognosis of POEMS syndrome. PMID:12486906

  13. Bilateral Burkitt Lymphoma of the Ovaries: A Report of a Case in a Child with Williams Syndrome

    PubMed Central

    Onimoe, Grace Ifeyinwa; Kahwash, Samir; Termuhlen, Amanda; Gross, Thomas G.; Varga, Elizabeth; Rose, Melissa J.

    2011-01-01

    A 10-year-old female with Williams Syndrome (WS) presented with a two-month history of fatigue, weight loss, and bilateral ovarian masses. Histologic, immunophenotypic, and cytogenetic studies confirmed the diagnosis of Burkitt lymphoma (BL). While there is no established association between the two disorders, this is the third case in the literature of Burkitt lymphoma in a patient with Williams Syndrome. PMID:21687537

  14. Unilateral or bilateral vagotomy induces ovulation in both ovaries of rats with polycystic ovarian syndrome

    PubMed Central

    2013-01-01

    Background Injecting estradiol valerate (EV) to pre-pubertal or adult female rat results in effects similar to those observed in women with polycystic ovarian syndrome (PCOS). One of the mechanisms involved in PCOS development is the hyperactivity of the sympathetic nervous system. In EV-induced PCOS rats, the unilateral sectioning of the superior ovarian nerve (SON) restores ovulation of the innervated ovary. This suggests that, in addition to the sympathetic innervation, other neural mechanisms are involved in the development/maintenance of PCOS. The aims of present study were analyze if the vagus nerve is one of the neural pathways participating in PCOS development. Methods Ten-day old rats were injected with EV dissolved in corn oil. At 24-days of age sham-surgery, unilateral, or bilateral sectioning of the vagus nerve (vagotomy) was performed on these rats. The animals were sacrificed at 90–92 days of age, when they presented vaginal estrous preceded by a pro-estrus smear. Results In EV-induced PCOS rats, unilateral or bilateral vagotomy restored ovulation in both ovaries. Follicle-stimulating hormone (FSH) levels in PCOS rats with unilateral or bilateral vagotomy were lower than in control rats. Conclusions This result suggests that in EV-induced PCOS rats the vagus nerve is a neural pathway participating in maintaining PCOS. The vagus nerve innervates the ovaries directly and indirectly through its synapsis in the celiac-superior-mesenteric ganglion, where the somas of neurons originating in the SON are located. Then, it is possible that vagotomy effects in EV-induced PCOS rats may be explained as a lack of communication between the central nervous system and the ovaries. PMID:23866168

  15. A unilateral purple urine bag syndrome in a patient with bilateral nephrostomy tubes.

    PubMed

    Karim, Adil; Abed, Firas; Bachuwa, Ghassan

    2015-01-01

    A change in the colour of urine is always of clinical significance, and a source of concern for the patient and his physician. Among the different urine colours observed, purple is the least common. Although purple discolouration of a catheter and a urine bag is an uncommon finding, it was reported in the literature as early as 1978, by Barlow and Dickson. We present a unique case of purple urine bag syndrome in a patient with bilateral nephrostomy tubes (NT) and associated urine bags (UB) with only the left nephrostomy tube and urine bag exhibiting the purple colour, which resolved with a course of appropriate antibiotics eradicating the causative bacterial pathogen, and change of NT and UB. PMID:26701992

  16. Bilateral occipital calcification, epilepsy and coeliac disease: clinical and neuroimaging features of a new syndrome.

    PubMed Central

    Magaudda, A; Dalla Bernardina, B; De Marco, P; Sfaello, Z; Longo, M; Colamaria, V; Daniele, O; Tortorella, G; Tata, M A; Di Perri, R

    1993-01-01

    Twenty patients affected by bilateral occipital cortical-subcortical calcification (BOC) are described, 19 (95%) had epilepsy. In 8 of 16 cases studied, intestinal biopsy revealed coeliac disease. Fourteen patients had occipital partial epilepsy with a relatively benign outcome, while 4 patients were affected by a severe form of epilepsy, with very frequent, drug-resistant, generalised and partial seizures with mental deterioration. One patient had a single episode of convulsive status epilepticus at four months of age. The neurological examination was normal in all patients. CT showed flocculo-nodular, cortico-subcortical BOC, without enhancement and without lobar or hemispheric atrophy. MRI was normal. The clinical and neuroimaging features of these patients are different therefore from those with the Sturge-Weber Syndrome. The study confirms a high prevalence of coliac disease in patients with BOC, but the relationship between these two pathologies still needs to be clarified. Images PMID:8350105

  17. Brooke-Spiegler Syndrome with Multiple Scalp Cylindromas and Bilateral Parotid Gland Adenomas

    PubMed Central

    Kalina, Peter; el-Azhary, Rokea

    2012-01-01

    A 62-year-old female presented with numerous soft tissue lesions of her scalp and bilateral preauricular region. Several of these have been biopsied or removed with a diagnosis of cylindromas. Cylindromas are benign tumors with a differentiation towards apocrine sweat glands that increase in number and size throughout life. Multiple scalp cylindromas may coalesce and cover the entire scalp, resulting in the “turban tumor.” These are often associated with the autosomal dominant Brooke-Spiegler syndrome with coexistent facial trichoepitheliomas and spiradenomas. There is a very rare association between cylindromas and basal cell adenoma and adenocarcinoma of the parotid gland, with only 17 reported cases. Ours is the first CT demonstration of both the scalp and parotid gland findings in this uncommon situation. PMID:22606564

  18. 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. PMID:10713649

  19. 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

  20. Single-Session CT-Guided Percutaneous Microwave Ablation of Bilateral Adrenal Gland Hyperplasia Due to Ectopic ACTH Syndrome

    SciTech Connect

    Sarma, Asha Shyn, Paul B.; Vivian, Mark A.; Ng, Ju-Mei; Tuncali, Kemal; Lorch, Jorchen H.; Zaheer, Sarah N.; Gordon, Michael S.; Silverman, Stuart G.

    2015-10-15

    Bilateral adrenalectomy is currently the only available treatment for adrenocorticotropic hormone (ACTH)-dependent Cushing’s syndrome (ectopic ACTH syndrome) that is refractory to pharmacologic therapy. We describe two patients with refractory ectopic ACTH syndrome who were treated with CT-guided percutaneous microwave ablation of both hyperplastic adrenal glands in a single session: One was not a surgical candidate, and the other had undergone unsuccessful surgery. Following the procedure, both patients achieved substantial decreases in serum cortisol, symptomatic improvement, and decreased anti-hypertensive medication requirements.

  1. MRI Findings of Syndrome of Acute Bilateral Symmetrical Basal Ganglia Lesions in Diabetic Uremia: A Case Report and Literature Review

    PubMed Central

    Cao, Xin; Fang, Qiang

    2016-01-01

    The syndrome of acute bilateral basal ganglia lesions is an uncommon clinical occurrence exhibiting acute onset of movement abnormalities, which can be seen almost exclusively among patients with chronic renal failure, especially in the setting of concurrent diabetes mellitus. Symmetrical lesions located in basal ganglia demonstrated in MRI are typical manifestation of this syndrome. Our study includes routine MRI examination, MRS, 3D-ASL, and SWI findings, which have been rarely reported and will contribute to diagnosing more cases about this syndrome. PMID:27493824

  2. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly.

    PubMed

    Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

    2015-10-01

    Ectrodactyly-ectodermal dysplasia and clefting syndrome or "Lobster claw" deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case. PMID:26655010

  3. Bilateral congenital lacrimal fistulas in an adult as part of ectrodactyly-ectodermal dysplasia-clefting syndrome: A rare anomaly

    PubMed Central

    Ghosh, Debangshu; Saha, Somnath; Basu, Sumit Kumar

    2015-01-01

    Ectrodactyly-ectodermal dysplasia and clefting syndrome or “Lobster claw” deformity is a rare congenital anomaly that affects tissues of ectodermal and mesodermal origin. Nasolacrimal duct (NLD) obstruction with or without atresia of lacrimal passage is a common finding of such a syndrome. The authors report here even a rarer presentation of the syndrome which manifested as bilateral NLD obstruction and lacrimal fistula along with cleft lip and palate, syndactyly affecting all four limbs, mild mental retardation, otitis media, and sinusitis. Lacrimal duct obstruction and fistula were managed successfully with endoscopic dacryocystorhinostomy (DCR) which is a good alternative to lacrimal probing or open DCR in such a case. PMID:26655010

  4. Written production in a case of locked-in syndrome with bilateral corticopontic degeneration.

    PubMed

    Gayraud, F; Martinie, B; Bentot, E; Lepilliez, A; Tell, L; Cotton, F; Rode, G

    2015-01-01

    Patients in locked-in syndrome show normal or near normal mental abilities that contrast with the limited motor capacity that hinders voluntary communication. However, eye movements and blinking are usually preserved and can be used to establish a communication system. We report an exceptional case of locked-in syndrome. The aetiology was basilar thrombosis consecutive to a cervical manipulation. In addition, brain MRI performed 23 years later showed a ventral pontine stroke with bilateral corticopontic degeneration. In this study the patient was able to produce written output using a chin-controlled Morse system decoded by a computer. A detailed linguistic analysis of text written over 20 years by the patient was carried out. The data demonstrate that improvements in language performance can be observed even in patients with brain lesions in areas associated with high-level cognitive processes. The data show a decrease of typing, grammatical and lexical errors over time, use of less frequent words, and an increase of more complex linguistic structures. This paper adds to previous findings confirming the value of daily practice and rehabilitation to enhance quality of life in this group of patients. PMID:25347464

  5. Bilateral adrenal cysts and ectopic pancreatic tissue in Beckwith-Wiedemann syndrome: is a conservative approach acceptable?

    PubMed

    Rahmah, R; Yong, J F; Sharifa, N A; Kuhnle, U

    2004-06-01

    Beckwith-Wiedemann syndrome is a common overgrowth syndrome associated with an increased risk of neoplasias which might be explained by the nature and localization of the genetic defect. While malignant tumors are often associated with hemihypertrophy, benign tumors are also found. We report a patient with the typical features of Beckwith-Wiedemann syndrome with two histologically different abdominal tumors, bilateral cystic adrenals and ectopic pancreatic tissue present at birth. In both tumors no malignancy could be detected. Ectopic pancreatic tissue is rarely seen and has been described in Beckwith-Wiedemann syndrome only once. After extirpation of the ectopic pancreatic tissue the cystic adrenals were left in situ since macroscopically no normal adrenal tissue could be identified and separated. Regular ultrasound examinations revealed complete resolution of the cystic adrenals within 24 months. Thus it seems that a conservative approach in selected tumors associated with the Beckwith-Wiedemann syndrome might be acceptable. PMID:15270410

  6. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus.

    PubMed

    Saroj, Gyanendra; Gangwar, Anshul; Dhillon, Jatinder Kaur

    2016-01-01

    Sturge-Weber syndrome (SWS) is a rare, nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. It is believed to be caused by persistence of a vascular plexus around the cephalic portion of the neural tube and is present at birth in about 1 in every 50,000 babies. It is one of the phakomatoses which is often associated with port-wine stains (PWSs) of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. Many people with SWS probably never know they have it. Hypothyroidism is a condition that arises from inadequate release of thyroid-stimulating hormone to stimulate an otherwise normal thyroid gland. This condition is often associated with a deficient secretion of other pituitary hormone, and growth hormone deficiency occurs with an increased prevalence in SWS, presumably secondary to involvement of the hypothalamic-pituitary axis. Diagnosis is made by the presence of a facial PWS and evidence of leptomeningeal angioma either by skull X-ray or computed tomography scan that shows intracranial calcifications. Presently, there is no specific treatment for SWS, and the management of the clinical manifestations and complications is still far from adequate. Here, we report the case of hypothyroidism associated with SWS with oral and facial manifestations in an 11-year-old boy. How to cite this article: Saroj G, Gangwar A, Dhillon JK. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus. Int J Clin Pediatr Dent 2016;9(1): 82-85. PMID:27274162

  7. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus

    PubMed Central

    Saroj, Gyanendra; Gangwar, Anshul

    2016-01-01

    ABSTRACT Sturge-Weber syndrome (SWS) is a rare, nonhereditary developmental condition that is characterized by a hamartomatous vascular proliferation of the brain, resulting in multiple angiomas that occur on the same side due to arteriovenous malformations. It is believed to be caused by persistence of a vascular plexus around the cephalic portion of the neural tube and is present at birth in about 1 in every 50,000 babies. It is one of the phakomatoses which is often associated with port-wine stains (PWSs) of the face, glaucoma, seizures, mental retardation and ipsilateral leptomeningeal angioma. Many people with SWS probably never know they have it. Hypothyroidism is a condition that arises from inadequate release of thyroid-stimulating hormone to stimulate an otherwise normal thyroid gland. This condition is often associated with a deficient secretion of other pituitary hormone, and growth hormone deficiency occurs with an increased prevalence in SWS, presumably secondary to involvement of the hypothalamic-pituitary axis. Diagnosis is made by the presence of a facial PWS and evidence of leptomeningeal angioma either by skull X-ray or computed tomography scan that shows intracranial calcifications. Presently, there is no specific treatment for SWS, and the management of the clinical manifestations and complications is still far from adequate. Here, we report the case of hypothyroidism associated with SWS with oral and facial manifestations in an 11-year-old boy. How to cite this article: Saroj G, Gangwar A, Dhillon JK. Hypothyroidism and Sturge-Weber Syndrome associated with Bilateral Port-wine Nevus. Int J Clin Pediatr Dent 2016;9(1): 82-85. PMID:27274162

  8. Bilateral stenosis of carotid siphon in Hutchinson-Gilford progeria syndrome.

    PubMed

    Narazaki, Ryo; Makimura, Mika; Sanefuji, Masafumi; Fukamachi, Shigeru; Akiyoshi, Hidetaka; So, Hidenori; Yamamura, Kenichiro; Doisaki, Sayoko; Kojima, Seiji; Ihara, Kenji; Hara, Toshiro; Ohga, Shouichi

    2013-08-01

    Hutchinson-Gilford progeria syndrome (HGPS) is a rare premature aging disease, caused by a de novo mutation of lamin-A gene, LMNA G608G. Accumulation of abnormal lamin-A (progerin) compromises nuclear membrane integrity and results in the accelerated senescence. Affected patients show a typical feature of birdlike face, alopecia, sclerotic skin, loss of subcutaneous fat, and short stature with advancing years. Neonatal scleroderma is the first presentation, although early diagnosis is challenging. The leading cause of death is cardio-/cerebro-vascular accidents associated with atherosclerosis. However, not all findings may recapitulate the aging process. We herein report a 9-year-old Japanese male with HGPS who developed cerebral infarction. The genetic study of peripheral blood-derived DNA determined a heterozygous c.1824C>T mutation, p.G608G. Telomere length of lymphocytes was normal. Bilateral stenosis of carotid siphons was prominent, while systemic arteriosclerosis was unremarkable assessed by the ankle-brachial index, carotid ultrasound imaging and funduscopic study. HGPS patients have marked loss and functional defects in vascular smooth muscle cells, leading to the vulnerability to circulatory stress. Symmetrical stenosis of siphons might occur as a distinctive cerebral vasculopathy of HGPS, rather than simple vascular senescence. Peripheral blood study on LMNA G608G and telomere length could screen progerias in infancy for early therapeutic intervention. PMID:23141186

  9. Bilateral alien hand syndrome in cerebrovascular disease: CT, MR, CT angiography, and 99mTc-HMPAO-SPECT findings.

    PubMed

    Serrano-Vicente, Justo; Duran-Barquero, Carmen; Garcia-Bernardo, Lucia; Dominguez-Grande, Maria Luz; Infante-Torre, Jose Rafael; Rayo-Madrid, Juan Ignacio

    2015-03-01

    We report a 65-year-old man with a right cerebral infarction that occurred 15 years ago and a residual left hemiparesis that began with progressive contralateral hemiparesis. During the hospitalization, the patient developed a bilateral alien hand syndrome. Urgent CT, MR, CT angiography, and brain perfusion SPECT were performed that revealed an old right cerebral infarction and a new ischemic lesion in left parietal lobe and adjacent brain territories. PMID:25546190

  10. Anaesthesia management of a case of Jervell and Lange-Nielsen syndrome for minimally invasive bilateral thoracoscopic cervicothoracic sympathectomy.

    PubMed

    Roy, Preety Mittal; Khanna, Sangeeta; Mehta, Yatin; Khan, Ali Z

    2016-06-01

    Long QT syndrome (LQTS) is an arrhythmogenic cardiac disorder resulting from the malfunction of cardiac ion channels. Patient with LQTS may present with syncope, seizures or sudden cardiac death secondary to polymorphic ventricular tachycardia (VT) or torsades de pointes. Patient may be asymptomatic in the pre-operative period but may develop VT for the first time in operation theatre. We are reporting anaesthetic management of a child with LQTS planned for bilateral thoracoscopic cervicothoracic sympathectomy. PMID:27330206

  11. Anaesthesia management of a case of Jervell and Lange-Nielsen syndrome for minimally invasive bilateral thoracoscopic cervicothoracic sympathectomy

    PubMed Central

    Roy, Preety Mittal; Khanna, Sangeeta; Mehta, Yatin; Khan, Ali Z

    2016-01-01

    Long QT syndrome (LQTS) is an arrhythmogenic cardiac disorder resulting from the malfunction of cardiac ion channels. Patient with LQTS may present with syncope, seizures or sudden cardiac death secondary to polymorphic ventricular tachycardia (VT) or torsades de pointes. Patient may be asymptomatic in the pre-operative period but may develop VT for the first time in operation theatre. We are reporting anaesthetic management of a child with LQTS planned for bilateral thoracoscopic cervicothoracic sympathectomy. PMID:27330206

  12. Atypical Wernicke's syndrome sans encephalopathy with acute bilateral vision loss due to post-chiasmatic optic tract edema

    PubMed Central

    Desai, Soaham Dilip; Shah, Diva Sidharth

    2014-01-01

    A middle aged male presented with acute bilateral vision loss, 4 weeks after undergoing gastric bypass surgery for gastric carcinoma. He had normal sensorium, fundoscopy, normal pupillary reaction to light, but had mild opthalmoparesis and nystagmus with ataxia. Magnetic resonance imaging of the brain revealed post-chiasmatic optic tract edema along with other classical features of Wernicke's syndrome. Thiamine supplementation leads to complete resolution of clinical as well as imaging findings. In appropriate clinical settings, a high index of suspicion and early treatment are essential for managing Wernicke's syndrome even in patients with atypical clinical and imaging presentation. PMID:24753673

  13. Bilateral vocal cord paralysis and hypothyroidism as presenting symptoms of Williams-Beuren syndrome: a case report.

    PubMed

    Koren, Ilana; Kessel, Ira; Rotschild, Avi; Cohen-Kerem, Raanan

    2015-09-01

    Williams-Beuren syndrome is a rare neurodevelopmental disorder caused by deletion of 1.5-1.8Mb genes on chromosome 7q11.23. The syndrome was first described as a triad of supra-valvular aortic stenosis, mental retardation, and distinctive facial features. Our patient was referred due to audible inspiratory stridor when he was seven days old. Following endoscopy he was diagnosed with bilateral vocal cord paralysis and was eventually intubated due to respiratory de-compensation followed by tracheotomy. On further workup he was diagnosed with hypothyroidism. Genetic workup supported the diagnosis of Williams-Beuren syndrome. We report here a case with an unusual clinical presentation. PMID:26143126

  14. Colonic adenocarcinoma and bilateral malignant ovarian sex cord tumor with annular tubules in Peutz-Jeghers syndrome.

    PubMed

    Ayadi-Kaddour, A; Bouraoui, S; Bellil, K; Bellil, S; Kchir, N; Zitouna, M M; Haouet, S

    2004-06-01

    Peutz-Jeghers syndrome is characterized by multiple polyps throughout the gastrointestinal tract in association with mucocutaneous pigmentation. Although Peutz-Jeghers syndrome polyps are hamartomas, frequent association of this syndrome with both gastrointestinal and non-gastrointestinal tumours had led to reassessment of the cancer risk in this hereditary disorder. The most common gynaecological tumors in this syndrome are adenoma malignum of the uterine cervix and ovarian sex cord tumor, particularly sex cord tumor with annular tubules. The question of malignant change in a polyp or of the association of gastro intestinal carcinomas still discuss. The authors report a case of Peutz-Jeghers syndrome in a young patient who developed a colonic adenocarcinoma in a hamartomatous polyp together with an incidentally discovered bilateral malignant sex cord tumours. We discuss its association with certain benign and malignant tumors and the risk of rare complications of these hamartomatous polyps. Although malignant tumors are increasingly reported in association with the Peutz-Jeghers syndrome, to our knowledge, there have been no previous reports of such an association in the literature. PMID:15524052

  15. Disseminated cervical adenoma malignum and bilateral ovarian sex cord tumors with annular tubules associated with Peutz-Jeghers syndrome.

    PubMed

    Srivatsa, P J; Keeney, G L; Podratz, K C

    1994-05-01

    Adenoma malignum is a highly differentiated mucinous adenocarcinoma of the cervix with a deceptively innocent histologic appearance but a highly aggressive behavior. We describe a patient who had adenoma malignum associated with Peutz-Jeghers syndrome (PJS) and bilateral ovarian sex cord tumor with annular tubules (SCTAT). The relatively frequent occurrence of cervical adenoma malignum in women with PJS warrants close surveillance by gynecologists for early detection and treatment of this cancer. We report the 16th known case of PJS with adenoma malignum and only the 8th known case of PJS with adenoma malignum and bilateral SCTAT. The highly aggressive nature of cervical adenoma malignum is exemplified, and the difficulties associated with early diagnosis are discussed. The clinical significance of cervical and ovarian tumors associated with PJS is reviewed. PMID:8188091

  16. Antiphospholipid Antibody Syndrome Associated with Graves' Disease Presenting As Inferior Vena Cava Thrombosis with Bilateral Lower Limb DVT.

    PubMed

    Jain, Ankur

    2014-01-01

    We report a case of a 60-year-old lady who presented with bilateral lower limb swelling and a thyroid swelling with clinical features consistent with thyrotoxicosis. Investigations revealed the presence of a thrombus in bilateral external, internal iliac veins, and inferior vena cava extending up to its infrahepatic part. Hormone profile and radioiodine uptake scan confirmed the diagnosis of Graves' disease. Further workup revealed the presence of antiphospholipid antibodies (confirmed after a repeat test at 12 weeks). The patient was treated with antithyroid drugs and anticoagulants. The patient improved with normalization of thyroid function and partial recanalization of the infrahepatic part of inferior vena cava. Hyperthyroidism has been implicated as a potential hypercoagulable state; however, the association of Graves' disease with antiphospholipid antibody syndrome is limited to isolated case reports. This case highlights a new mechanism underlying hypercoagulability associated with Graves' disease. PMID:24812529

  17. Extensive Bilateral Lemierre Syndrome due to Methicillin-Resistant Staphylococcus epidermidis in a Patient with Lung Adenocarcinoma

    PubMed Central

    Choi, Bo Mi; Son, Seong Wan; Park, Chan Kwon; Lee, Sang-Hoon

    2015-01-01

    Lemierre syndrome (LS) is a septic thrombophlebitis of the internal jugular vein (IJV) following an oropharyngeal infection. LS is commonly caused by normal anaerobic flora and treated with appropriate antibiotics and anticoagulation therapy. Although the incidence of disease is very rare, 15% cases of LS are fatal even in the antibiotic era because of disseminated septic thromboemboli. We reported a case of extensive bilateral LS due to methicillin-resistant Staphylococcus epidermidis in a 63-year-old female with lung adenocarcinoma. Initial examination revealed a retropharyngeal abscess; hence, intravenous ceftriaxone and steroid were initiated empirically. However, pulmonary thromboembolism developed and methicillin-resistant S. epidermidis was identified in the bacterial culture. Despite intensive antibiotic and anticoagulation therapies, extensive septic thrombophlebitis involving the bilateral IJV and superior vena cava developed. Adjunctive catheter-directed thrombolysis and superior vena cava stenting were performed and the patient received antibiotic therapy for an additional 4 weeks, resulting in complete recovery. PMID:26175788

  18. Bilateral lid/brow elevation procedure for severe ptosis in Kearns-Sayre syndrome, a mitochondrial cytopathy

    PubMed Central

    Sebastiá, Roberto; Fallico, Ester; Fallico, Matteo; Fortuna, Eduardo; Lessa, Sergio; Neto, Guilherme Herzog

    2015-01-01

    Background The purpose of this work was to determine the effectiveness and possible complications encountered with bilateral fascia lata lid suspension used to correct blepharoptosis in patients with Kearns-Sayre syndrome. Methods This was a retrospective study of seven patients with Kearns-Sayre syndrome who had a minimum of 1 year of follow-up. A bilateral fascia lata sling was used to correct the ptosis. Preoperative and postoperative measurements of the vertical lid fissure width (VFW) and marginal reflex distance (MRD) were performed. The Student’s t-test was used to analyze the results. Results The mean preoperative VFW and MRD measurements were 4±2.45 mm and 0.14±0.92 mm, respectively. The mean postoperative VFW and MRD measurements were 7.71±1.85 mm, and 2.86±1.69 mm, respectively. All preoperative and postoperative values were considered to be statistically significant (P<0.01). Adequate elevation of the lids was obtained in all patients, both functionally and aesthetically. All of the patients showed a mild symmetric postoperative inferior version lagophthalmos, and one patient developed corneal ulceration and scarring due to corneal exposure and a weak Bell’s phenomenon. Conclusion The surgical technique described to correct the blepharoptosis found in patients with Kearns-Sayre syndrome was found to be efficient and relatively safe. The correction should be conservative to decrease the risk of postoperative corneal damage that occurred in one patient. PMID:25565765

  19. Bilateral Common Carotid Artery Occlusion in Spontaneously Hypertensive Rats: A Feasible Animal Model for Ocular Ischemic Syndrome.

    PubMed

    Wang, Yacong; Fan, Yuhua; Zhang, Lihong; Wang, Yi-Xiang J; Qi, Wei; Liang, Willmann; Wang, Chunmei; T W Yew, David; Ye, Cunxi; Sha, Ou

    2016-06-01

    The purpose of this study was to investigate the feasibility of inducing ocular ischemic syndrome in spontaneously hypertensive rats. Hypertensive and normotensive Wistar-Kyoto rats had bilateral occlusion or sham surgery. They were divided into 4 groups: (1) hypertensive-ischemia, (2) hypertensive-sham, (3) normotensive-ischemia, and (4) normotensive-sham. Four months after the operation, the global changes of the eye and pupillary light reflex were assessed. Then each rat was perfused, and randomly one of the bulbuses oculi was prepared as retinal flat mounts for investigation of vascular changes. The opposite eyeball was prepared as a paraffin section for observation of the linear density of retinal ganglion cells and for thickness measurement. One hypertensive-ischemia rat had a cataract in one eye and another rat in the same group had bulbus oculi collapse in one eye. The light reflex disappeared in 13.33% of hypertensive-ischemia rats, and the rest of the hypertensive-ischemia rats and normotensive-ischemia rats had slow reflex. Compared with the respective controls, the peripheral retinal vascular network in hypertensive-ischemia and normotensive-ischemia rats was sparse; linear density of the retinal ganglion cells was significantly reduced; and the retinal thickness was reduced. Compared with normotensive-ischemia rats, the hypertensive-ischemia rats demonstrated more severe changes. After bilateral common carotic artery occlusion, the eyes of hypertensive rats developed various pathological changes similar to those of ocular ischemic syndrome. In conclusion, an animal model for ocular ischemic syndrome can be created by bilateral common carotid artery occlusion in spontaneously hypertensive rats. Anat Rec, 299:806-814, 2016. © 2016 Wiley Periodicals, Inc. PMID:26917224

  20. Spontaneous bilateral corneal perforation in stevens- johnsons syndrome-a challenge in management.

    PubMed

    Md Noh, Umi Kalthum; Then, Kong Yong

    2013-01-01

    A 42-year-old man from Ghana presented with bilateral painful corneal perforations following ingestion of a sulphur-based antibiotic. Emergency bilateral penetrating keratoplasty was performed, with restoration of globe integrity. However, surgical complications arose such as non-healing epithelial defect, secondary infection, graft dehiscence, and mounting intraocular pressure. This case illustrates the challenges faced in managing corneal grafts in patients with already compromised ocular surfaces. PMID:23785259

  1. Bilateral Proliferative Retinopathy Associated With Hoyeraal-Hreidarsson Syndrome, a Severe Form of Dyskeratosis Congenita.

    PubMed

    Allingham, Michael J

    2016-04-01

    Dyskeratosis congenita (DC) is the prototypical member of a family of diseases caused by defective telomere maintenance. These "telomeropathies" also include Hoyeraal-Hreidarsson syndrome (HH) and Revesz syndrome, which are severe forms of dyskeratosis congenita, as well as a subset of idiopathic pulmonary fibrosis, aplastic anemia, and Coats' plus syndrome. Retinopathy has only rarely been reported in DC and HH, but is universally present in Coats' plus and Revesz syndromes. The care of these patients is typically a multidisciplinary effort, and this should include monitoring by an ophthalmologist. PMID:27065378

  2. [A case of Peutz-Jeghers syndrome combined with bilateral breast cancer, an adenocarcinoma of the cervix and ovarian genital cord neoplasms with annular tubules].

    PubMed

    Gloor, E

    1978-05-13

    The clinico-pathological findings are presented in a case of Peutz-Jeghers syndrome associated with a bilateral mammary invasive ductal carcinoma, a well-differentiated mucinous adenocarcinoma of the cervix and microscopic, bilateral ovarian sex cord tumors with annular tubules. The sex cord tumor with annular tubules was described in 1970 by SCULLY, who recognized its striking association with the Peutz-Jeghers syndrome. Two cases of adenocarcinoma of the cervix and another case of uterine adenocarcinoma of unspecified localization associated with Peutz-Jeghers syndrome were found in the literature. It is possible that women with Peutz-Jeghers syndrome run an increased risk of developing adenocarcinoma of the uterine cervix. PMID:644282

  3. A Case of Acute Bilateral Irvine-Gass Syndrome following Uncomplicated Phacoemulsification, Demonstrated with Optical Coherence Tomography

    PubMed Central

    Shields, Melissa K.; Adler, Paul A.; Fuzzard, Dujon R.W.; Chalasani, Rajeeve; Teong, Joanne M.Y.

    2015-01-01

    Purpose To report a case of acute bilateral Irvine-Gass syndrome. Methods This is an observational case report. Results An 82-year-old man with no significant ocular history developed postsurgical pseudophakic cystoid macular edema (CME; Irvine-Gass syndrome) on consecutive phacoemulsification cataract surgeries. His initial first-eye (left) CME developed 25 days after surgery and was managed with topical preparations of dexamethasone 0.1% and ketorolac 0.4%, in addition to a routine post-cataract surgery drop regime. His left CME resolved completely on optical coherence tomography (OCT) by day 100, and he subsequently (after extensive discussion of CME risks) underwent cataract surgery on his right eye. He was commenced prophylactically on dexamethasone, ketorolac and oral indomethacin 25 mg t.d.s. immediately after surgery; however, he later developed CME (OD) on day 32 postoperatively. Within 6 months, he achieved complete resolution of his CME in both eyes. His clinical course was documented with serial OCT studies. Conclusion Irvine-Gass syndrome remains an important differential diagnosis in the evaluation of blurred vision after cataract surgery, despite decreasing incidence. Those who experience CME following their first cataract operation should be counseled about the risks of developing the condition in the contralateral eye, despite prophylactic measures. PMID:26034486

  4. [Successful Treatment of Repeated Bilateral Middle Cerebral Artery Occlusion by Performing Mechanical Thrombectomy in a Patient with Trousseau Syndrome].

    PubMed

    Inoue, Satoshi; Fujita, Atsushi; Mizowaki, Takashi; Uchihashi, Yoshito; Kuroda, Ryuichi; Urui, Seishirou; Kurihara, Eiji; Kohmura, Eiji

    2016-06-01

    We report a patient with Trousseau syndrome who presented with repeated acute middle cerebral artery (MCA) occlusion, which was successfully treated by performing mechanical thrombectomy. A 66-year-old man with a lung lesion and abdominal lymph node swelling experienced a sudden onset of left hemiparesis. Magnetic resonance angiography (MRA) revealed a right MCA occlusion. Perfusion-weighted imaging revealed reduced cerebral blood flow in the right MCA territory. Complete recanalization of the occluded vessel was achieved by performing mechanical thrombectomy, and his symptoms remarkably disappeared. Twenty days after the procedure, he experienced right hemiparesis and total aphasia. MRA revealed a left MCA occlusion, which was located on the contralateral side of the first occlusion. The second mechanical thrombectomy was successfully performed, and complete recanalization was successfully achieved. Right hemiparesis improved after the procedure. Histological examination of the retrieved clots revealed coagulated fibrin and platelets. Cytodiagnosis of pleural effusion revealed adenocarcinoma, and he was diagnosed with lung adenocarcinoma and cancerous pleurisy. Trousseau syndrome was the presumed primary cause of the patient's tendency for thrombogenesis. To the best of our knowledge, there were no reports regarding the repeated use of mechanical thrombectomy for patients with bilateral MCA occlusion caused by Trousseau syndrome. Neuroendovascular therapy can offer good neurological outcomes even in patients with malignant lesions. PMID:27270149

  5. Multiple strokes and bilateral carotid dissections: a fulminant case of newly diagnosed Ehlers-Danlos syndrome type IV.

    PubMed

    Dohle, C; Baehring, J M

    2012-07-15

    Ehlers-Danlos Syndrome is a rare group of inheritable disorders resulting in abnormal collagen production, leading to skin fragility, joint hypermobility and easy bruising. Six major subtypes have been identified, of which Type IV most often leads to neurovascular complications, may lead to inner organ rupture and overall has the worst prognosis. Early recognition followed by genetic testing is key, since this diagnosis will guide decision making in the management of complications, influence the choice of antiplatelet medications versus anticoagulants and allow for potentially affected family members to be identified, undergo genetic testing and reproductive counseling. We here report the case of a 50 year old woman with a fulminant presentation of Ehlers Danlos Syndrome Type IV, including bilateral carotid and vertebral artery dissection, multiple strokes and liver rupture. Of note, this patient did not have a known history or obvious clinical features of connective tissue disease. Genetic testing confirmed the diagnosis. Review of her family history revealed multiple family members with a history of aortic dissection or aneurysm rupture. This case illustrates that Ehlers Danlos Syndrome Type IV is an important differential diagnosis even in adult patients without a known history of connective tissue disease and no prior complications. PMID:22559935

  6. Good visual outcome in an immunocompromised patient with bilateral acute retinal necrosis syndrome: A case report

    PubMed Central

    Marrocos de Aragão, Ricardo E.; Barreira, Ieda M.A.; Arrais, Barbara L.A.; Pereira, Leidiane A.; Ramos, Carine S.

    2013-01-01

    Acute retinal necrosis (ARN) is an uncommon necrotizing, fulminant retinopathy caused by the herpes simplex virus types 1 or 2 or by the varicella zoster vírus with visually devastating consequences. Generally it occurs in patients who are systemically healthy, but occasionally occurs in immunocompromised host. We report a case of bilateral ARN in a patient with AIDS with a good final visual outcome. PMID:25278806

  7. Spinal cord infarction as a rare complication of fat embolism syndrome following bilateral intramedullary nailing of femur fractures.

    PubMed

    Kearsley, RoseMarie; Galbraith, John; Dalton, David; Motherway, Catherine

    2016-01-01

    Fat embolism syndrome (FES) is a rare and potentially fatal complication occurring most often after long bone or pelvic fractures and orthopaedic procedures. It can consist of pulmonary, central nervous system and cutaneous manifestations. The exact pathophysiology of emboli reaching the arterial circulation is poorly understood.1 It is suggested that this may occur by either 'paradoxical' embolism or microembolism.2 3 Its true incidence is unknown but increases in the presence of multiple closed fractures. It can be a diagnostic dilemma for clinicians and if suspected diffusion-weighted MRI is the modality of choice for the investigation of the central nervous system.4 We present the case of a 22-year-old man who developed multifocal cerebral infarcts, a right-sided cerebellar infarct and an infarct in the anterior cord bilaterally at the level of C5-C6 as a result of FES. PMID:27624445

  8. [Charles Bonnet syndrome in an elderly patient with bilateral vision loss, hyperthyroidism and relative digitalis overdose].

    PubMed

    Gödecke-Koch, T; Schlimme, J; Rada, D; Emrich, H M

    2002-05-01

    Charles Bonnet syndrome (CBS) is characterized by the presence of visual hallucinations in elderly, mentally healthy people. We report a visually impaired 90-year-old woman suddenly complaining of visual hallucinations, suffering from hyperthyroidism and a relative digitalis overdose. The diagnosis of CBS could be made after the exclusion of an intoxication and other neurological and psychiatric syndromes. In this case, visual hallucinations ceased without specific psychopharmacological therapy. A brief review of this organic hallucinosis, differential diagnosis, especially hyperthyroidism-induced psychosis, and digitoxin-induced psychosis is given and current therapeutic strategies are suggested. PMID:12078028

  9. [Spontaneous bilateral lens resorption in a case of Hallermann-Streiff syndrome].

    PubMed

    Soriano, J M; Funk, J

    1991-09-01

    The Hallermann-Streiff-syndrome was first described in 1948 by Hallermann and in 1950 by Streiff. The most common features are dyscephalia, cataract, microphthalmia, dental anomalies, hypotrichosis, cutaneous atrophy, and nanism. Anomalies of the eye include cataract, microphthalmia, nystagm, strabism, blue sclera, fundus anomalies and combined anomalies of all segments of the eye. The frequency of cataract is about 90%. Spontaneous resorption of the lens is described in about 8%. We present a five year old girl showing the typical dyscephalia of the Hallermann-Streiff-syndrome (bird face). Her lenses were replaced by opaque membranes. These membranes presumably were remnants of the posterior lens capsule. PMID:1753673

  10. EFFECT OF BILATERAL OOPHORECTOMY ON ADRENOCORTICAL FUNCTION IN WOMEN WITH POLYCYSTIC OVARY SYNDROME (PCOS)

    PubMed Central

    Azziz, Ricardo; Chang, Wendy Y.; Stanczyk, Frank Z.; Woods, Keslie

    2012-01-01

    Objective To determine the impact of ovary-secreted products on adrenocortical function in women with PCOS by studying the adrenocortical response to acute adrenocorticotropic-stimulating hormone (ACTH) stimulation before and after bilateral oophorectomy. Design Prospective study. Setting Tertiary care medical center Participants Fourteen women with PCOS scheduled for bilateral oophorectomy for benign indications, on transdermal estradiol (E2) postoperatively. Interventions Physical exam, blood sampling before and after oophorectomy, measurement of hormone levels. Basal (Steroid0), maximum stimulated (Steroid60), and net increment (ΔSteroid) levels of androstenedione (A4), dehydroepiandrosterone (DHEA), and cortisol (F) before and after ACTH-1–24 stimulation were assessed. Main Outcome Measures Pre- and post-operative basal and ACTH(1–24)-stimulated hormonal levels. Results Total testosterone, free testosterone, and estrone levels decreased, and FSH levels increased significantly following oophorectomy. No significant differences in E2, DHEA sulfate (DHEAS) or sex hormone binding globulin levels were detected. Basal and ACTH-stimulated A4 levels decreased significantly following oophorectomy, and ΔA4 was significantly increased. No significant differences in DHEA0, DHEA60, or F0 levels were detected; F60 and ΔF levels tended to increase following oophorectomy, but the differencesdid not reach significance. Conclusions Ovarian factors do not appear to contribute significantly to the adrenocortical dysfunction of PCOS. PMID:23122827

  11. [Safe and Effective Analgesia with Bilateral Continuous TAP Block for a Patient with Marfan Syndrome after Open Abdominal Aortic Aneurysm Repair].

    PubMed

    Katakura, Yumi; Sakurai, Asako; Endo, Masaru; Hamada, Takako; Nomoto, Mayuko; Yamada, Hiroshi; Takeda, Koji

    2016-06-01

    A patient with Marfan syndrome underwent emergency open abdominal aortic aneurysm repair. She was referred to our department for postoperative analgesia. Taking the risk of possible dural ectasia into consideration, we avoided epidural block. Alternatively, we performed bilateral continuous transversus abdominis plane (TAP) block with sufficient analgesia. Lumbosacral dural ectasia is frequently observed in patients with Marfan syndrome. A few reports described that their fragile dura may contribute to an increased risk of dural puncture and postdural puncture headache (PDPH). Thus, in planning neuraxial block for a patient with Marfan syndrome, the possible consequences of lumbosacral dural ectasia should be considered. A case we herein present shows bilateral continuous TAP block could be a safe and effective alternative to epidural block. PMID:27483660

  12. Successful treatment of Raynaud’s syndrome in a lupus patient with continuous bilateral popliteal sciatic nerve blocks: a case report

    PubMed Central

    Dao, Thuan; Amaro-Driedger, David; Mehta, Jaideep

    2016-01-01

    Raynaud’s syndrome has been treated medically and invasively, sometimes with regional anesthesia leading up to sympathectomy. We demonstrate that regional anesthesia was in this case a useful technique that can allow some patients to find temporary but significant relief from symptoms of Raynaud’s syndrome exacerbation. We present a 43-year-old woman with Raynaud’s syndrome secondary to lupus who was treated with bilateral popliteal nerve block catheters for ischemic pain and necrosis of her feet; this led to almost immediate resolution of her pain and return of color and function of her feet. While medical management should continue to be a front-line treatment for Raynaud’s syndrome, regional anesthesia can be useful in providing rapid dissipation of symptoms and may thus serve as a viable option for short-term management of this syndrome. PMID:27366104

  13. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias.

    PubMed

    Yu, Hyeoh Won; Cho, Won Im; Chung, Hye Rim; Choi, Keun Hee; Yun, Sumi; Cho, Hwan Seong; Shin, Choong Ho; Yang, Sei Won

    2016-03-01

    Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. PMID:27104180

  14. Multiple osteoblastomas in a child with Cushing syndrome due to bilateral adrenal micronodular hyperplasias

    PubMed Central

    Yu, Hyeoh Won; Cho, Won Im; Choi, Keun Hee; Yun, Sumi; Cho, Hwan Seong; Shin, Choong Ho; Yang, Sei Won

    2016-01-01

    Adrenocorticotropin-independent adrenal hyperplasias are rare diseases, which are classified into macronodular (>1 cm) and micronodular (≤1 cm) hyperplasia. Micronodular adrenal hyperplasia is subdivided into primary pigmented adrenocortical disease and a limited or nonpigmented form 'micronodular adrenocortical disease (MAD)', although considerable morphological and genetic overlap is observed between the 2 groups. We present an unusual case of a 44-month-old girl who was diagnosed with Cushing syndrome due to MAD. She had presented with spotty pigmentation on her oral mucosa, lips and conjunctivae and was diagnosed with multiple bone tumors in her femur, pelvis and skull base at the age of 8 years. Her bone tumor biopsies were compatible with osteoblastoma. This case highlights the importance of verifying the clinicopathologic correlation in Cushing syndrome and careful follow-up and screening for associated diseases. PMID:27104180

  15. Remission of severe restless legs syndrome and periodic limb movements in sleep after bilateral excision of multiple foot neuromas: a case report

    PubMed Central

    2010-01-01

    Introduction Restless legs syndrome is a sensorimotor neurological disorder characterized by an urge to move the legs in response to uncomfortable leg sensations. While asleep, 70 to 90 percent of patients with restless legs syndrome have periodic limb movements in sleep. Frequent periodic limb movements in sleep and related brain arousals as documented by polysomnography are associated with poorer quality of sleep and daytime fatigue. Restless legs syndrome in middle age is sometimes associated with neuropathic foot dysesthesias. The causes of restless legs syndrome and periodic limb movements in sleep are unknown, but the sensorimotor symptoms are hypothesized to originate in the central nervous system. We have previously determined that bilateral forefoot digital nerve impingement masses (neuromas) may be a cause of both neuropathic foot dysesthesias and the leg restlessness of restless legs syndrome. To the best of our knowledge, this case is the first report of bilateral foot neuromas as a cause of periodic limb movements in sleep. Case presentation A 42-year-old Caucasian woman with severe restless legs syndrome and periodic limb movements in sleep and bilateral neuropathic foot dysesthesias was diagnosed as having neuromas in the second, third, and fourth metatarsal head interspaces of both feet. The third interspace neuromas represented regrowth (or 'stump') neuromas that had developed since bilateral third interspace neuroma excision five years earlier. Because intensive conservative treatments including repeated neuroma injections and various restless legs syndrome medications had failed, radical surgery was recommended. All six neuromas were excised. Leg restlessness, foot dysesthesias and subjective sleep quality improved immediately. Assessment after 18 days showed an 84 to 100 percent reduction of visual analog scale scores for specific dysesthesias and marked reductions of pre-operative scores of the Pittsburgh sleep quality index, fatigue severity

  16. Bilateral Sturge-Weber and Phakomatosis Pigmentovascularis with Glaucoma, an Overlap Syndrome

    PubMed Central

    Patil, Bharat; Nayak, Bhagabat; Sharma, Reetika; Kumari, Sadhana; Dada, Tanuj

    2015-01-01

    Aim. To report a case of bilateral Sturge-Weber and Phakomatosis pigmentovascularis with secondary glaucoma in a child. Method. Case report. Results. A 4-year-old male child was referred to us for control of intraocular pressure (IOP). Sleeping IOP was 36 mm Hg in right eye and 28 mm Hg in the left eye. The sclera of both the eyes showed bluish black pigmentation—melanosis bulbi. Fundus examination of both eyes showed diffuse choroidal hemangiomas with glaucomatous cupping. Nevus flammeus was present on both sides of face along all the 3 divisions of trigeminal nerve with overlying hypertrophy of skin and on left forearm. Nevus fuscocaeruleus was present on upper trunk. All skin lesions were present since birth and were stationary in nature. CT scan of head revealed left-sided cerebral atrophy. Intraocular pressure was controlled after treatment with topical antiglaucoma medications. Pulsed Dye Laser has been advised by dermatologist for skin lesions. Patient has been advised for regular follow-up. Conclusion. The two overlapping dermatological disorders and their association with glaucoma are a rare entity. Management should be targeted both for dermatological and eye conditions. PMID:26064732

  17. Bilateral Symmetrical Idiopathic Necrotizing Encephalopathy: A New Syndrome in Sprague-Dawley Rats.

    PubMed

    De Jonghe, Sandra; Abbott, David; Vinken, Petra; Moesen, Esther; Feyen, Bianca; Lammens, Lieve; Vynckier, An; De Lahunta, Alexander

    2015-12-01

    This article describes the occurrence of a bilaterally symmetrical encephalopathy in Sprague-Dawley rats, which occurred over the period 2005 to 2012 in our laboratory in both untreated control rats and rats treated with different pharmacologically active compounds. The acute brain lesions consisted of degeneration/necrosis in the ventral areas of the brain mostly with little inflammatory response; in the more rare chronic cases there were numerous lipid-laden macrophages. The areas most consistently affected were the crus cerebri, the ventral midbrain, the pyramids, and the internal capsule. Other areas less frequently affected were the mammillary bodies, the fimbria, the olfactory tubercles, the optic tracts, and the ventral hippocampus. All available data, including clinical signs, gross pathology, clinical pathology, diet, breeding, and housing were collected and are presented. Our investigations did not elucidate the pathogenesis of the lesions, although the infarction-type changes are suggestive of a vascular etiology. To our knowledge, this particular lesion with its consistent distribution pattern has not been reported in the rat literature and its publication is therefore important to the toxicological pathology community, because an unbalanced group distribution in a toxicology study could potentially confound the safety assessment of a compound. PMID:26511844

  18. Fibrolipomatous hamartoma of the median nerve: A cause of acute bilateral carpal tunnel syndrome in a three-year-old child: A case report and comprehensive literature review

    PubMed Central

    Senger, Jenna-Lynn; Classen, Dale; Bruce, Garth; Kanthan, Rani

    2014-01-01

    A three-year-old boy was investigated for inexplicable incessant crying. On examination, his left wrist was mildly swollen (three to four months) and sensitive. Exploration and carpal tunnel decompression of the left wrist with incisional biopsy was performed for the presence of a fusiform swelling intimately associated with the median nerve. Histopathology revealed the presence of enlarged nerve bundles admixed with mature fat cells and diffuse fibroblastic proliferation. Three months later, he underwent urgent contralateral carpal tunnel decompression for a similar presentation. The final diagnosis was bilateral fibrolipomatous hamartoma (FLH) of the median nerves causing acute bilateral compression neuropathy. FLH of the median nerve is an extremely unusual cause of acute bilateral carpal tunnel syndrome in a young child presenting with ‘incessant crying’. A comprehensive review of FLH including epidemiology, etiology, clinical presentation, differential diagnosis, imaging, pathology, treatment and prognosis is discussed. PMID:25332651

  19. Bilateral Pneumothoraces Following BiV ICD Placement: A Case of Buffalo Chest Syndrome

    PubMed Central

    Rali, Aniket S.; Manyam, Harish

    2015-01-01

    Patient: Female, 73 Final Diagnosis: Buffalo chest syndrome Symptoms: — Medication: — Clinical Procedure: Bi-ventricular ICD Specialty: Cardiology Objective: Rare disease Background: Contralateral pneumothorax following device implantation on the left side has been reported in a few cases. The majority of contralateral pneumothoraces showed evidence of atrial perforation on computed tomography (CT), echocardiography, or chest x-rays and required lead revision. To the best of our knowledge there is only 1 other reported case of contralateral pneumothorax without evidence of macro-displacement of the atrial lead. In that case the patient experienced a right-sided pneumothorax on day 1 after undergoing repositioning of the atrial lead. Case Report: The current case is unique on several accounts, including timing of the contralateral pneumothorax and no evidence of associated atrial lead perforation on device interrogation or CT imaging. Furthermore, the appearance of contralateral pneumothorax within 8 hours of clamping of the ipsilateral chest tube argues in favor of a pleuro-pleural fistula. Conclusions: The term ‘buffalo chest’ refers to a single pleural space, with no anatomical separation of the 2 hemithoraces, as seen in an American buffalo or bison. We believe this to be a case of buffalo chest syndrome. PMID:26431396

  20. Bilateral Bone Marrow Edema Syndrome of the Femoral Head with a Unique Onset: A Case Report

    PubMed Central

    Yi, Seung Rim; Kim, Hae Min

    2015-01-01

    Bone marrow edema syndrome (BMES) is a rare condition which mainly affects the hip area. The etiology and pathogenesis of BMES is still unclear. Pain near the affected area, regional osteoporosis, bone marrow edema (identified using magnetic resonance imaging) and spontaneous regression within 6-12 months are the main characteristics of BMES. In this case, a 52-year-old male was diagnosed with BMES of the right hip followed by spontaneous subsiding of symptoms. After 3 years, and under nearly the same social and physical conditions, he was admitted again with newly developed left hip pain and again diagnosed with BMES. We report this rare case since a similar one has not been previously reported in the domestic literature and may be considered valuable for basic research relating to the pathogenesis of BMES.

  1. Bilateral Bone Marrow Edema Syndrome of the Femoral Head with a Unique Onset: A Case Report.

    PubMed

    Yi, Seung Rim; Lee, Ye Hyun; Kim, Hae Min

    2015-12-01

    Bone marrow edema syndrome (BMES) is a rare condition which mainly affects the hip area. The etiology and pathogenesis of BMES is still unclear. Pain near the affected area, regional osteoporosis, bone marrow edema (identified using magnetic resonance imaging) and spontaneous regression within 6-12 months are the main characteristics of BMES. In this case, a 52-year-old male was diagnosed with BMES of the right hip followed by spontaneous subsiding of symptoms. After 3 years, and under nearly the same social and physical conditions, he was admitted again with newly developed left hip pain and again diagnosed with BMES. We report this rare case since a similar one has not been previously reported in the domestic literature and may be considered valuable for basic research relating to the pathogenesis of BMES. PMID:27536636

  2. [Neuromyelitis optica with syndrome of inappropriate secretion of antidiuretic hormone and hypersomnia associated with bilateral hypothalamic lesions: a case report].

    PubMed

    Nakano, Takeshi; Fujimoto, Takeshi; Fukuda, Yasuo; Takahashi, Toshiyuki; Kanbayashi, Takashi

    2011-08-01

    A 31-year-old woman with a 5-year history of recurrent optic neuritis and encephalomyelitis underwent repeated steroid therapy. She developed general malaise and fever in October 2009. Laboratory tests revealed marked reduction in serum Na (106 mEq/L). Because the plasma osmotic pressure was lower than the urinary osmotic pressure and the serum antidiuretic hormone (ADH) level was elevated, she was diagnosed with the syndrome of inappropriate secretion of antidiuretic hormone (SIADH). Magnetic resonance imaging (MRI) revealed high signal intensities on symmetrical T2 weighted and fluid attenuated inversion recovery (FLAIR) images of both sides of the hypothalamus. The serum samples tested positive for the antibody to aquaporin-4 (AQP4). Previously conducted cervical MRI had revealed a longitudinally extending lesion in the cervical cord, and brain MRI had revealed brainstem lesions. We diagnosed the patient with neuromyelitis optica (NMO) according to the revised diagnostic criteria for NMO proposed by Wingerchuk in 2006. Furthermore, she complained of excessive daytime sleepiness. The concentration of orexin in the cerebrospinal fluid was mildly reduced and the orexin levels returned to normal when her sleepiness decreased. ADH and orexin neurons localized in the hypothalamus; hence, we considered the above-mentioned symptoms to be caused by bilateral hypothalamic lesions. PMID:21878727

  3. POST‐SURGICAL REHABILITATION FOLLOWING FASCIOTOMIES FOR BILATERAL CHRONIC EXERTIONAL COMPARTMENT SYNDROME IN A SPECIAL FORCES SOLDIER: A CASE REPORT

    PubMed Central

    Miller, Joseph

    2013-01-01

    Background and Purpose: The etiology of Chronic Exertional Compartment Syndrome (CECS) is still unclear. The most commonly accepted theory suggests that it is a transient but debilitating process where there is an abnormally increased intracompartmental pressure during exercise/exertion due to non‐compliant expansion of the osteofascial tissues. This most commonly occurs in the lower leg. Surgical intervention is often performed for symptom relief. However, there has been limited scientifically‐based publication on post‐surgical rehabilitation, especially with regard to return to function in the military population. The purpose of this case report is to demonstrate the utilization of a recommended post‐operative protocol in a Special Forces Soldier. Case Description: The subject presented as a 25‐year‐old US Army Special Forces Soldier, who failed 8 weeks of conservative management for the diagnosis of CECS and subsequently underwent bilateral lower leg fasciotomies of the anterior and lateral compartments. Outcomes: Following recommended protocol guidelines he was progressed rapidly and within three months deployed without restriction or complications in a demanding combat zone. Discussion: This case report illustrates that following clearly defined, scientifically‐based rehabilitation guidelines helped in addressing all of the involved structures and musculoskeletal dysfunctions that presented following the surgical intervention for CECS in a unique subject. Level of Evidence: 5 PMID:24175149

  4. Endoscopic treatment of bilateral pheochromocytoma in MEN 2A syndrome: case report and review of the literature.

    PubMed

    Iusco, D; Sarli, L; Di Mauro, D; Violi, V; Roncoroni, L

    2007-10-01

    The benefits of laparoscopic adrenalectomy for single adrenal lesion have been well documented in literature; less experience though has been reported with simultaneous bilateral laparoscopic adrenalectomy. This operation is indicated in case of primary hypercortisolism caused by bilateral adrenocortical hyperfunction, Cushing's disease after failure of pituitary surgery, ectopic adrenocorticotropic hormone (ACTH) production by a tumour inaccessible for surgical intervention, and pheochromocytoma when it occurs bilaterally in case of multiple endocrine neoplasia type 2A and 2B. Different laparoscopic approaches have been described to perform this operation, such as the "anterior" approach (transperitoneal), the "lateral" approach (transperitoneal and retroperitoneal), and the "posterior" approach (retroperitoneal). We report a case of bilateral laparoscopic adrenalectomy in a 33 years old female affected with bilateral pheochromocytoma due to multiple endocrine neoplasia type 2A treated with a bilateral transperitoneal laparoscopic adrenalectomy and disease free after 18 months follow-up. PMID:17915049

  5. Multiple bilateral supernumerary mandibular premolars in a non-syndromic patient with associated orthokeratised odontogenic cyst- A case report and review of literature

    PubMed Central

    Kasat, Vikrant O.; Saluja, Harish; Kalburge, Jitendra V.; Kini, Yogesh; Nikam, Atul; Laddha, Ruchi

    2012-01-01

    Multiple supernumerary teeth are very rare, accounting for less than 1% of cases. They are commonly associated with syndromes like Gardner's syndrome and cleidocranial dysostosis and cleft lip and palate. Non-syndromic multiple supernumerary teeth have a predilection to occur in the mandibular premolar region. Orthokeratinized odontogenic cyst (OOC) is a relatively uncommon developmental cyst comprising about 10% of the cases that had been previously implied as odontogenic keratocysts. More than half of the cases of OOC are associated with impacted tooth; but not a single case of OOC associated with supernumerary teeth is reported. Hence, the purpose of this article is to report the first case of multiple supernumerary mandibular premolars associated with OOC in a 35-year-old male and to review the literature associated with multiple bilateral supernumerary mandibular premolars. PMID:23230374

  6. Obstructive sleep apnea syndrome with bilateral papilledema and vision loss in a 3-year-old child.

    PubMed

    Quinn, Anthony G; Gouws, Pieter; Headland, Sophie; Oades, Patrick; Pople, Ian; Taylor, David; Benton, J Sarah; Buncic, J Raymond; Henderson, John; Fleming, Peter

    2008-04-01

    We describe bilateral papilledema and vision loss in a 3-year-old child with obstructive sleep apnea. Although lumbar puncture initially disclosed a normal opening pressure, cerebrospinal fluid (CSF) pressure monitoring during sleep confirmed intermittent episodes of elevated intracranial pressure corresponding to increased airway resistance. The association of obstructive sleep apnea and raised intracranial pressure is recognized in children with craniosynostosis but has not been reported in its absence. PMID:18289895

  7. Metachronous Bilateral Testicular Leydig-Like Tumors Leading to the Diagnosis of Congenital Adrenal Hyperplasia (Adrenogenital Syndrome)

    PubMed Central

    Vukina, Josip; Chism, David D.; Sharpless, Julie L.; Raynor, Mathew C.; Milowsky, Matthew I.; Funkhouser, William K.

    2015-01-01

    A 33-year-old male with a history of left testis Leydig cell tumor (LCT), 3-month status after left radical orchiectomy, presented with a rapidly enlarging (0.6 cm to 3.7 cm) right testicular mass. He underwent a right radical orchiectomy, sections interpreted as showing a similar Leydig cell-like oncocytic proliferation, with a differential diagnosis including metachronous bilateral LCT and metachronous bilateral testicular tumors associated with congenital adrenal hyperplasia (a.k.a. “testicular adrenal rest tumors” (TARTs) and “testicular tumors of the adrenogenital syndrome” (TTAGS)). Additional workup demonstrated a markedly elevated serum adrenocorticotropic hormone (ACTH) and elevated adrenal precursor steroid levels. He was diagnosed with congenital adrenal hyperplasia, 3β-hydroxysteroid dehydrogenase deficiency (3BHSD) type, and started on treatment. Metachronous bilateral testicular masses in adults should prompt consideration of adult presentation of CAH. Since all untreated CAH patients are expected to have elevated serum ACTH, formal exclusion of CAH prior to surgical resection of a testicular Leydig-like proliferation could be accomplished by screening for elevated serum ACTH. PMID:26351608

  8. A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia.

    PubMed

    Blackmore, Kate; Wynne, David M

    2010-08-01

    Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed. PMID:20627328

  9. A case of solitary median maxillary central incisor (SMMCI) syndrome with bilateral pyriform aperture stenosis and choanal atresia.

    PubMed

    Blackmore, Kate; Wynne, David M

    2010-08-01

    Solitary median maxillary central incisor syndrome is a rare disorder involving midline abnormalities. It may present with life threatening respiratory distress in the neonate secondary to nasal malformations. These include pyriform aperture stenosis and choanal atresia. We present the first reported case of simultaneous choanal atresia and pyriform aperture stenosis in a neonate with solitary median maxillary central incisor syndrome. The clinical presentation and the management of congenital pyriform aperture stenosis are discussed. PMID:20626079

  10. Bilateral cellulitis.

    PubMed

    Batra, Vivek; Baras, Alexander

    2015-01-01

    We present a case of bilateral lesions in a 50-year-old man, which were on first impression mistaken for and initially treated as bilateral cellulitis. We propose that bilateral cellulitis, as opposed to unilateral, is rare and that other aetiologies should be considered in evaluating a patient with bilateral lesions. The differential diagnosis includes stasis-dermatitis, lipodermatosclerosis, lymphoedema and vascular lesions such as Kaposi sarcoma, as was identified in this case. Early consultation with dermatology and biopsy in unclear cases mitigates the unnecessary use of prolonged antibiotics, antibiotic resistance and Clostridium difficile infections. HIV testing is an essential screening test in all adults who present with non-specific viral symptoms and rash. PMID:26392449

  11. Bilateral symmetrical adrenal hypermetabolism on FDG PET/CT due to Cushing syndrome in well differentiated neuroendocrine carcinoma.

    PubMed

    Aktas, G E; Soyluoglu Demir, S; Sarikaya, A

    2016-01-01

    The (18)F-FDG PET/CT scan has been suggested for whole-body imaging to identify ectopic adrenocorticotrophic hormone secreting tumours, but there are some challenges involved. The case of a patient is presented, who was admitted with the pre-diagnosis of ectopic ACTH syndrome. On the CT, a nodular lesion was detected in the medial segment of the right lung. The FDG uptake of the lesion seemed to be increased visually, but was not pathological quantitatively (SUVmax: 1.8) on the PET/CT. There was also diffuse increased uptake (SUVmax: 14.2) in the enlarged adrenal glands. The lesion was reported as a possible malignant lesion with low FDG affinity, such as a low grade neuroendocrine tumour, while the diffuse enlarged adrenal glands with high uptake were interpreted as diffusely hyperplasic, due to Cushing's syndrome. The patient was treated with a surgical wedge resection. The histopathological diagnosis confirmed that the tumour was a grade 1 well-differentiated neuroendocrine carcinoma. PMID:26522002

  12. Bilateral anterior lenticonus in a case of Alport syndrome: a clinical and histopathological correlation after successful clear lens extraction

    PubMed Central

    Sonarkhan, Shilpa; Ramappa, Muralidhar; Chaurasia, Sunita; Mulay, Kaustubh

    2014-01-01

    A 29-year-old woman presented with symptoms of gradual, progressive, painless diminution of vision in both eyes for past 8–10 years. On examination, uncorrected visual acuity was 20/60 in the right eye and 20/126 in the left eye. Anterior segment examination was essentially normal except for a conical protrusion in the pupillary axis of the anterior lens capsule with a clear underlying lens. During surgery, central protruded fragile lens capsule was meticulously handled to create well-centered continuous curvilinear capsulorhexis by using paediatric rhexis forceps. Histopathology of excised capsule showed markedly thinned lens capsule with normal epithelium. Electron microscopy of the anterior lens capsule showed multiple linear and irregular zones of dehiscence. Few of these had fibrillar, irregular electron-dense material and vacuoles; and adjacent cells were irregular suggestive of Alport syndrome. Postoperative course was uneventful and visual acuity improved to 20/20 unaided. Clear lens extraction is an effective means of rehabilitating visually symptomatic anterior lenticonus of Alport syndrome. PMID:24969069

  13. Oculocerebral hypopigmentation syndrome (Cross syndrome).

    PubMed

    Ozkan, H; Unsal, E; Köse, G

    1991-01-01

    A typical case of Cross syndrome with hypopigmentation, mental and psychomotor retardation, spasticity, bilateral optic atrophy and dental defects in a three-year-old boy is presented. The clinical features of this rare syndrome are discussed. PMID:1814043

  14. A Case Report of Bilateral Mirror Clubfeet and Bilateral Hand Polydactyly

    PubMed Central

    Nguyen, Mai P; Lawler, Ericka A; Morcuende, Jose A

    2014-01-01

    We report a rare case of a patient with bilateral mirror clubfeet and bilateral hand polydactyly. The patient presented to our orthopaedic clinic with bilateral mirror clubfeet, each with eight toes, and bilateral hands with six fingers and a hypoplastic thumb. The pattern does not fit any described syndrome such as Martin or Laurin-Sandrow syndrome. Treatments by an orthopaedic pediatric surgeon and an orthopaedic pediatric hand surgeon are described. The patient achieved excellent functional and cosmetic outcomes at four year follow-up. PMID:25328478

  15. 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

  16. 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. PMID:26844510

  17. Comparison between Unilateral and Bilateral Ovarian Drilling in Clomiphene Citrate Resistance Polycystic Ovary Syndrome Patients: A Randomized Clinical Trial of Efficacy

    PubMed Central

    Zahiri Sorouri, Ziba; Sharami, Seyede Hajar; Tahersima, Zinab; Salamat, Fatemeh

    2015-01-01

    Background Laparoscopic ovarian drilling (LOD) is an alternative method to induce ovulation in polycystic ovary syndrome (PCOS) patients with clomiphene citrate (CC) resistant instead of gonadotropins. This study aimed to compare the efficacy of unilateral LOD (ULOD) versus bilateral LOD (BLOD) in CC resistance PCOS patients in terms of ovulation and pregnancy rates. Materials and Methods In a prospective randomized clinical trial study, we included 100 PCOS patients with CC resistance attending to Al-Zahra Hospital in Rasht, Guilan Province, Iran, from June 2011 to July 2012. Patients were randomly divided into two ULOD and BLOD groups with equal numbers. The clinical and biochemical responses on ovulation and pregnancy rates were assessed over a 6-month follow-up period. Results Differences in baseline characteristics of patients between two groups prior to laparoscopy were not significant (p>0.05). There were no significant differences between the two groups in terms of clinical and biochemical responses, spontaneous menstruation (66.1 vs. 71.1%), spontaneous ovulation rate (60 vs. 64.4%), and pregnancy rate (33.1 vs. 40%) (p>0.05). Following drilling, there was a significant decrease in mean serum concentrations of luteinizing hormone (LH) (p=0.001) and testosterone (p=0.001) in both the groups. Mean decrease in serum LH (p=0.322) and testosterone concentrations (p=0.079) were not statistically significant between two groups. Mean serum level of follicle stimulating hormone (FSH) did not change significantly in two groups after LOD (p>0.05). Conclusion Based on results of this study, ULOD seems to be equally efficacious as BLOD in terms of ovulation and pregnancy rates (Registration Number: IRCT138903291306N2). PMID:25918587

  18. Genetic mapping to 10q23.3-q24.2, in a large Italian pedigree, of a new syndrome showing bilateral cataracts, gastroesophageal reflux, and spastic paraparesis with amyotrophy.

    PubMed Central

    Seri, M; Cusano, R; Forabosco, P; Cinti, R; Caroli, F; Picco, P; Bini, R; Morra, V B; De Michele, G; Lerone, M; Silengo, M; Pela, I; Borrone, C; Romeo, G; Devoto, M

    1999-01-01

    We have recently observed a large pedigree with a new rare autosomal dominant spastic paraparesis. In three subsequent generations, 13 affected individuals presented with bilateral cataracts, gastroesophageal reflux with persistent vomiting, and spastic paraparesis with amyotrophy. Bilateral cataracts occurred in all affected individuals, with the exception of one patient who presented with a chorioretinal dystrophy, whereas clinical signs of spastic paraparesis showed a variable expressivity. Using a genomewide mapping approach, we mapped the disorder to the long arm of chromosome 10 on band q23.3-q24.2, in a 12-cM chromosomal region where additional neurologic disorders have been localized. The spectrum of phenotypic manifestations in this family is reminiscent of a smaller pedigree, reported recently, confirming the possibility of a new syndrome. Finally, the anticipation of symptoms suggests that an unstable trinucleotide repeat may be responsible for the condition. PMID:9973297

  19. Bilateral patellar tendon-bearing Symes-type prostheses in a severe case of Freeman-Sheldon syndrome in a 21-year-old woman presenting with uncorrectable equinovarus.

    PubMed

    McCormick, Rodger J; Poling, Mikaela I; Chamberlain, Robert L

    2015-01-01

    Described is the use of bilateral patellar tendon-bearing Symes-type prostheses in a severe case of Freeman-Sheldon syndrome (FSS) in a 21-year-old woman presenting with uncorrectable equinovarus and multiple surgical sequela of the lower extremities. FSS is considered a type of myopathic distal arthrogryposis; diagnosis requires the following: microstomia, whistling-face appearance, H-shaped chin dimpling, nasolabial folds, and multiple contractures of the hands and feet. Spinal deformities, metabolic and gastroenterological problems, other dysmorphic craniofacial characteristics, and visual and auditory impairments, are frequent findings. We highlight what we believe to be a unique approach to enhancing the patient's ability to ambulate and engage in physical activity, particularly critical determinates of wellness and long-term functional outcome in FSS, when the feet are non-correctable but not amputated. Important implications exist for physiatrists, other rehabilitation providers and surgeons regarding this likely under-diagnosed syndrome. PMID:26178231

  20. Bilateral Mandibular Supernumerary Canines: A Case Report

    PubMed Central

    Abouei Mehrizi, Ehsan; Semyari, Hassan; Eslami Amirabadi, Gholamreza

    2010-01-01

    Supernumerary teeth are defined as the teeth developed in excess of the number found in a normal dentition. Supernumerary canine is an extremely rare finding particularly in the mandible. This case report presents a 25-year-old female patient with the unique feature of bilateral mandibular supplemental supernumerary canines. The patient was non-syndromic without any other supernumerary teeth. PMID:23346342

  1. A case of bilateral temporal lobe agenesis

    PubMed Central

    Lang, C; Lehrl, S; Huk, W

    1981-01-01

    A 76-year-old man with bilateral temporal lobe agenesis producing clinical features resembling the Robinson syndrome is described. The malformation was discovered during a routine CT examination after the appearance of a homonymous visual field defect. The patient was examined by (neuro) psychological testing. The findings are compared with other reported cases and discussed with regard to cerebral localisation. Images PMID:7288451

  2. Bilateral molariform mandibular second premolars.

    PubMed

    Acharya, Sonu; Kumar Mandal, Pradip; Ghosh, Chiranjit

    2015-01-01

    Macrodontia is a rare dental anomaly that refers to teeth that appear larger than normal. Generalised macrodontia can be associated with certain medical conditions and syndromes. This case report presents clinical and radiographic findings of isolated bilateral macrodontia in a 14-year-old child. The patient was referred to the clinic with local crowding of maxillary and mandibular teeth. Radiographic findings revealed the presence of impacted macrodont mandibular second premolar on one side and erupted macrodontic premolar on the other side and their distinct morphological appearance, characterized by large, multitubercular, and molariform crowns and tapering, single roots. PMID:25685564

  3. Forme fruste of Proteus syndrome: A limited form with bilateral plantar cerebriform collagenomas and varicose veins secondary to a mosaic AKT1 mutation

    PubMed Central

    Wee, Jamie S.; Mortimer, Peter S.; Lindhurst, Marjorie J.; Chong, Heung; Biesecker, Leslie G.; Holden, Colin A.

    2014-01-01

    Importance Proteus syndrome is an extremely rare disorder of mosaic postnatal overgrowth affecting multiple tissues including bone, soft tissue and skin. It typically presents in early childhood with asymmetric and progressive skeletal overgrowth that leads to severe distortion of the skeleton and disability. The genetic basis has recently been identified as a somatic activating mutation in the AKT1 gene, which encodes an enzyme mediating cell proliferation and apoptosis. Observations We present a 33-year-old man that developed plantar cerebriform collagenomas on the soles of both feet and varicose veins in early childhood, in the absence of any skeletal or other connective tissue abnormality. Although the patient did not meet the diagnostic criteria for Proteus syndrome, he was found to have the c.49G>A, p.Glu17Lys AKT1 mutation in lesional skin but not in his blood. Conclusions and Relevance To our knowledge, this is the mildest molecularly confirmed patient with Proteus syndrome, occurring in the absence of the characteristic skeletal overgrowth. These findings extend the spectrum of Proteus syndrome pathology and suggest that somatic mutations late in development and restricted in distribution cause subtle clinical presentations that do not meet the published clinical criteria. PMID:24850616

  4. A rare case of Kearns–Sayre syndrome in a 17-year-old Venezuelan male with bilateral ptosis as the initial presentation

    PubMed Central

    Leal, Mayela; Dhoble, Chetan; Lee, Julie; Lopez, Deinymar; Menéndez, Laura Simonne

    2016-01-01

    Kearns–Sayre syndrome (KSS) was first described in 1958 as ‘a rare neuromuscular disorder defined by a characteristic triad of progressive external ophthalmoplegia, pigmentary retinopathy, atrioventricular block and cerebellar ataxia’. The prevalence rate of KSS is ∼1–3 per 100 000 individuals. Here, we report a rare case of a 17-year-old Venezuelan male with KSS. PMID:26949540

  5. Bilateral taurodontism in primary dentition with hypodontia.

    PubMed

    Surendar, Marappan Natarajan; Pandey, Ramesh Kumar; Khanna, Richa

    2013-01-01

    Taurodontism is a rare dental anomaly in which there is an enlarged pulp chamber at the expense of roots with apical displacement of the pulpal floor, giving it a rectangular shape. It is caused by the failure of Hertwig's epithelial root sheath to invaginate at the proper horizontal level. Taurodontism has been reported as an intraoral feature of several syndromes like Down syndrome, Klinefelter syndrome, Smith-Magenis syndrome, Hurler syndrome, etc. Association of taurodontism with hypodontia in permanent dentition has also been reported. Taurodontism in primary dentition and its association with hypodontia is very rarely reported in the literature. The present case illustrates bilateral taurodontism of primary mandibular molars with hypodontia in maxilla. PMID:23345504

  6. Bilateral diffuse uveal melanocytic proliferation: a management dilemma

    PubMed Central

    Alrashidi, Salah; Aziz, Ayman A; Krema, Hatem

    2014-01-01

    A female patient suffered from gradual decline of vision for few months. She presented with bilateral multiple pigmented choroidal tumours, associated with overlying retinal changes. The clinical presentation suggested bilateral diffuse uveal melanocytic proliferation (BDUMP) syndrome, which is a paraneoplastic disease, although there was no evidence of any concurrent malignancy. The periodic systemic surveillance that was undertaken for the following 4 years failed to reveal any occult cancer. Nevertheless, there has been relentless progressive deterioration in vision as a consequence of BDUMP syndrome. The management of the declining vision in BDUMP syndrome is challenging and controversial. PMID:24855079

  7. [Bilateral segmental neurofibromatosis].

    PubMed

    Rose, I; Vakilzadeh, F

    1991-12-01

    Segmental neurofibromatosis is a rare type of neurofibromatosis. We report a case of bilateral manifestation, review the literature on this extremely uncommon variant, and discuss the possible causative mechanisms and the genetic risk of segmental neurofibromatosis. PMID:1765491

  8. Echinoderms Have Bilateral Tendencies

    PubMed Central

    Zhao, Wenchan; Wang, Sishuo; Lv, Jianhao

    2012-01-01

    Echinoderms take many forms of symmetry. Pentameral symmetry is the major form and the other forms are derived from it. However, the ancestors of echinoderms, which originated from Cambrian period, were believed to be bilaterians. Echinoderm larvae are bilateral during their early development. During embryonic development of starfish and sea urchins, the position and the developmental sequence of each arm are fixed, implying an auxological anterior/posterior axis. Starfish also possess the Hox gene cluster, which controls symmetrical development. Overall, echinoderms are thought to have a bilateral developmental mechanism and process. In this article, we focused on adult starfish behaviors to corroborate its bilateral tendency. We weighed their central disk and each arm to measure the position of the center of gravity. We then studied their turning-over behavior, crawling behavior and fleeing behavior statistically to obtain the center of frequency of each behavior. By joining the center of gravity and each center of frequency, we obtained three behavioral symmetric planes. These behavioral bilateral tendencies might be related to the A/P axis during the embryonic development of the starfish. It is very likely that the adult starfish is, to some extent, bilaterian because it displays some bilateral propensity and has a definite behavioral symmetric plane. The remainder of bilateral symmetry may have benefited echinoderms during their evolution from the Cambrian period to the present. PMID:22247765

  9. Bilateral popliteal arterial dissection.

    PubMed

    Chen, Po-Liang; Ko, Shih-Yu; Tan, Ken-Hing

    2012-01-01

    A clinical feature of bilateral popliteal arterial dissection without involving the descending aorta, bilateral iliac, as well as femoral arteries has never been reported in the past literature. We report a 56-year-old man with hypertension and coronary artery disease who presented to our emergency department with complaints of bilateral knee pain after long-distance walking. Physical examination was notable for elevated blood pressure, but there was no palpable pulsation over dorsalis pedis arteries on his feet. Laboratory evaluation revealed a d-dimer level of 35.2 mg/L (FEU) on the day of the test and 1.2 mg/L one and a half months ago (normal level, <0.55). These findings were suggestive of a recent-onset peripheral arterial occlusive disorder. Computed tomography of the aorta showed bilateral popliteal arterial dissection with arterial intimal flap. Abdominal aorta, bilateral iliac, and femoral arteries remained intact with only arteriosclerotic change. Minimally invasive endovascular stent grafting was then performed. The patient had an uneventful recovery. PMID:21106320

  10. [Bilateral caudate head infarcts].

    PubMed

    Kuriyama, N; Yamamoto, Y; Akiguchi, I; Oiwa, K; Nakajima, K

    1997-11-01

    We reported a 67-year-old woman with bilateral caudate head infarcts. She developed sudden mutism followed by abulia. She was admitted to our hospital 2 months after ictus for further examination. She showed prominent abulia and was inactive, slow and apathetic. Spontaneous activity and speech, immediate response to queries, spontaneous word recall and attention and persistence to complex programs were disturbed. Apparent motor disturbance, gait disturbance, motor aphasia, apraxia and remote memory disturbance were not identified. She seemed to be depressed but not sad. Brain CT and MRI revealed bilateral caudate head hemorrhagic infarcts including bilateral anterior internal capsules, in which the left lesion was more extensive than right one and involved the part of the left putamen. These infarct locations were thought to be supplied by the area around the medial striate artery including Heubner's arteries and the A1 perforator. Digital subtraction angiography showed asymptomatic right internal carotid artery occlusion. She bad had hypertension, diabetes mellitus and atrial fibrillation and also had a left atrium with a large diameter. The infarcts were thought to be caused by cardioembolic occlusion to the distal portion of the left internal carotid artery. Although some variations of vasculature at the anterior communicating artery might contribute to bilateral medial striate artery infarcts, we could not demonstrate such abnormalities by angiography. Bilateral caudate head infarcts involving the anterior internal capsule may cause prominent abulia. The patient did not improve by drug and rehabilitation therapy and died suddenly a year after discharge. PMID:9503974

  11. Bilateral inferior turbinate osteoma.

    PubMed

    Sahemey, R; Warfield, A T; Ahmed, S

    2016-01-01

    Osteomas are the most common benign osteoclastic tumours of the paranasal sinuses. However, nasal cavity and turbinate osteomas are extremely rare. Only nine middle turbinate, three inferior turbinate and one inferior turbinate osteoma cases have been reported to date. The present case report describes the management and follow-up of symptomatic bilateral inferior turbinate osteoma.A 60-year-old female presented with symptoms of bilateral nasal obstruction and right-sided epiphora. Radiological investigation found hypertrophic bony changes involving both inferior turbinates. The patient was managed successfully by endoscopic inferior turbinectomies in order to achieve a patent airway, with no further recurrence of tumour after 3 months postoperatively.To the best of our knowledge, this is the first reported case of bilateral inferior turbinate osteoma. We describe a safe and minimally invasive method of tumour resection, which has a better cosmetic outcome compared with other approaches. PMID:27534890

  12. Efficacy of bilateral pallidotomy.

    PubMed

    Kim, R; Alterman, R; Kelly, P J; Fazzini, E; Eidelberg, D; Beric, A; Sterio, D

    1997-03-15

    Unilateral pallidotomy is a safe and effective treatment for medically refractory bradykinetic Parkinson's disease, especially in those patients with levodopa-induced dyskinesia and severe on-off fluctuations. The efficacy of bilateral pallidotomy is less certain. The authors completed 11 of 12 attempted bilateral pallidotomies among 150 patients undergoing pallidotomy at New York University. In all but one patient, the pallidotomies were separated by at least 9 months. Patients were selected for bilateral pallidotomy if they exhibited bilateral rigidity, bradykinesia, or levodopa-induced dyskinesia prior to treatment or if they exhibited disease progression contralateral to their previously treated side. The Unified Parkinson's Disease Rating Scale (UPDRS) and timed upper-extremity tasks of the Core Assessment Protocol for Intracerebral Transplantation (CAPIT) were administered to all 12 patients in the "off" state (12 hours without receiving medications) preoperatively and again at 6 and 12 months after each procedure. The median UPDRS and contralateral CAPIT scores improved 60% following the initial procedure (p = 0.008, Wilcoxon rank sums test). The second pallidotomy generated only an additional 10% improvement in the UPDRS and CAPIT scores ipsilateral to the original procedure (p = 0.05). Worsened speech was observed in two cases. In the 12th case, total speech arrest was noted during test stimulation. Speech returned within minutes after stimulation was halted. Lesioning was not performed. These results indicate that bilateral pallidotomy has a narrow therapeutic window. Motor improvement ipsilateral to the first lesion leaves little room for further improvement from the second lesion and the risk of speech deficit is greatly enhanced. Chronic pallidal stimulation contralateral to a previously successful pallidotomy may prove to be a safer alternative for the subset of patients who require bilateral procedures. PMID:15096015

  13. Bilateral ovarian Burkitt's lymphoma.

    PubMed

    Gutiérrez-García, L; Medina Ramos, N; García Rodríguez, R; Barber, M A; Arias, M D; García, J A

    2009-01-01

    Primary ovarian lymphoma is a rare entity. We submit a case of a 34-year-old black patient presenting with a bilateral adnexal tumor. She underwent hysterectomy with double salpingo-oophorectomy followed by polychemotherapy treatment. Histology confirmed Epstein-Barr virus-positive bilateral Burkitt's lymphoma. The patient died from septic shock after a month of treatment. Endemic Burkitt's lymphoma has a predilection for the female genital tract, manifesting itself clinically as a pelvic mass and less frequently as a menstrual disorder. It is a rare entity in our environment but should be kept in mind when treating patients of African origin. PMID:19480266

  14. Reversible Acute Parkinsonism and Bilateral Basal Ganglia Lesions in a Diabetic Uremic Patient

    PubMed Central

    Nzwalo, Hipólito; Sá, Francisca; Capela, Carlos; Ferreira, Fátima; Basílio, Carlos

    2012-01-01

    The syndrome of bilateral basal ganglia lesions in diabetic uremic patients is a rare disorder mostly reported in Asians. There are few reports of the syndrome in Caucasians. It manifests as an acute hyperkinetic or hypokinetic extrapyramidal disorder in association with uniform neuroimaging findings of bilateral symmetrical basal ganglia changes in diabetics undergoing hemodialysis. Its pathophysiology remains largely unknown. Thus, we report a typical case of the syndrome in a Caucasian patient who developed an acute and reversible akinetic rigid parkinsonism secondary to bilateral basal ganglia lesions. PMID:23185167

  15. Bilateral dentigerous cysts that involve all four dental quadrants: a case report and literature review

    PubMed Central

    2016-01-01

    Dentigerous cysts are common odontogenic cysts that are associated with the crown of the tooth and typically develop from single lesions. Bilateral and multiple dentigerous cysts are very rare and occur in patients with syndromic conditions. This paper presents a case report of a 15-year-old male patient that experienced non-syndromic bilateral dentigerous cysts that simultaneously occurred in all four dental quadrants around the unerupted third molars. Clinicians should confirm the extent of cystic lesions using a panoramic view and computed tomography, and should keep the possibility of bilateral dentigerous cysts in mind as a potential diagnosis, even in a non-syndromic patient. PMID:27162755

  16. Bilateral inferior petrosal sinus sampling using vasopressin

    PubMed Central

    Kotwal, Narendra; Kumar, Yogesh; Upreti, Vimal; Singh, Amandeep; Garg, M. K.

    2016-01-01

    Context: Anatomical localization of pituitary adenoma can be challenging in adrenocorticotropic hormone (ACTH)-dependent Cushing's syndrome, and bilateral inferior petrosal sinus sampling (BIPSS) is considered gold standard in this regard. Stimulation using corticotrophin-releasing hormone (CRH) improves the sensitivity of BIPSS, however, same is not easily available in India. Therefore, we undertook this study of BIPPS using vasopressin as agent for stimulation owing to its ability to stimulate V3 receptors present on corticotrophs. Aims: To study the tumor localization and lateralization in difficult to localize cases of ACTH-dependent Cushing's syndrome by bilateral inferior petrosal sinus sampling using vasopressin for corticotroph stimulation. Settings and Design: Prospective observational study. Subjects and Methods: Six patients (5 females) meeting inclusion criteria underwent BIPSS using vasopressin for stimulation. Results: All six patients had nonsuppressible overnight and low dose dexamethasone suppression test with elevated plasma ACTH levels suggestive of ACTH-dependent Cushing's syndrome. High dose dexamethasone suppression test showed suppressible cortisol in two cases, and microadenoma was seen in two patients on magnetic resonance imaging pituitary. Contrast enhanced computed tomography of the abdomen showed left adrenal hyperplasia in one case and anterior mediastinal mass with bilateral adrenal hyperplasia another. Using BIPSS four patients were classified as having Cushing's disease that was confirmed histopathologically following surgery. Of the remaining two, one had primary pigmented nodular adrenocortical disease, and another had thymic carcinoid with ectopic ACTH production as the cause of Cushing's syndrome. No serious adverse events were noted. Conclusions: Vasopressin may be used instead of CRH and desmopressin for stimulation in BIPSS. PMID:27186561

  17. Bilateral pisotriquetral loose bodies

    PubMed Central

    Williams, GR; Holland, P; Beazley, J; Hyder, N

    2012-01-01

    Case reports detailing diagnosis and effective treatment of pisotriquetral loose bodies are scarce. This article describes an even rarer case of bilateral pisotriquetral joint loose bodies, explores the relative diagnostic roles of magnetic resonance imaging versus computed tomography, and outlines effective strategies used for the management of this condition drawn from the literature and our own experience. PMID:22507708

  18. Bilateral periorbital ecthyma gangrenosum.

    PubMed

    Ghosheh, Faris R; Kathuria, Sajeev S

    2006-01-01

    We describe a case of bilateral periorbital ecthyma gangrenosum in a diabetic patient with renal failure. Ecthyma gangrenosum is a cutaneous manifestation of Pseudomonas sepsis. We briefly review the pathogenesis of ecthyma gangrenosum and discuss previous reports of periocular involvement. In our patient, conservative measures and supportive care of the periorbital tissue resulted in a good outcome. PMID:17117116

  19. Bilateral Sagittal Split Osteotomy

    PubMed Central

    Monson, Laura A.

    2013-01-01

    The bilateral sagittal split osteotomy is an indispensable tool in the correction of dentofacial abnormalities. The technique has been in practice since the late 1800s, but did not reach widespread acceptance and use until several modifications were described in the 1960s and 1970s. Those modifications came from a desire to make the procedure safer, more reliable, and more predictable with less relapse. Those goals continue to stimulate innovation in the field today and have helped the procedure evolve to be a very dependable, consistent method of correction of many types of malocclusion. The operative surgeon should be well versed in the history, anatomy, technical aspects, and complications of the bilateral sagittal split osteotomy to fully understand the procedure and to counsel the patient. PMID:24872760

  20. Bilateral Presumed Tuberculous Choroiditis

    PubMed Central

    Mohammadi, Naseh; Ghassemi, Fariba; Shojaei, Esfandiar; Moradnejad, Pardis

    2016-01-01

    Purpose: To describe a case of bilateral presumed tuberculous choroiditis which resolved promptly with a short course of antituberculous therapy. Case Report: A 44-year-old lady presented with bilateral visual loss of 8 months’duration. Considering the presence of pan-uveitis, severe macular edema, multifocal leakage on fluorescein angiography, positive tuberculin skin test, and after excluding other diseases, she underwent anti-tuberculous therapy (ATT) for disseminated choroiditis due to presumed ocular tuberculosis. She improved promptly and completely. There were no signs of relapse 18 months after completion of the treatment course. In a young patient with disseminated choroiditis and relatively short duration of symptoms, a course of ATT for 6 months may effectively treat the disease preventing relapse for a considerable period of time. Conclusion: In Presumed Tuberculous chroiditis early diagnosis is crucial for prompt and complete improvement. PMID:27413507

  1. Bilateral renal calculi

    PubMed Central

    Sreenevasan, G

    1974-01-01

    Bilateral renal calculi were present in 114 (10.7%) of 1,070 cases of proved urinary calculus admitted to the Urological Department of the General Hospital, Kuala Lumpur, during the period November 1968—May 1973. The management of bilateral renal calculi is discussed with reference to the first 100 cases in this series. The introduction of renography has greatly facilitated the decision as to which kidney should be operated on first. The management of patients with and without uraemia is discussed and the use of the modified V and V—Y incisions for the removal of staghorn calculi is described. Complications and results are briefly reviewed. ImagesFig. 1Fig. 4Fig. 6Fig. 7 PMID:4845653

  2. Bilateral Mandibular Paramolars

    PubMed Central

    Dhull, Rachita Singh; Panda, Swagatika; Acharya, Sonu; Yadav, Shweta; Mohanty, Gatha

    2014-01-01

    ABSTRACT Supernumerary tooth is a developmental anomaly and has been argued to arise from multiple etiologies. These teeth may remain embedded in the alveolar bone or can erupt into the oral cavity. They can cause a variety of complications in the develo­ping dentition. Supernumerary teeth can present in various forms and in any region of the mandible or maxilla, but have a predisposition for the anterior maxilla. Here is the presentation of a case of unusual location of supernumerary teeth located in between mandibular first and second molar region bilaterally. How to cite this article: Dhull KS, Dhull RS, Panda S, Acharya S, Yadav S, Mohanty G. Bilateral Mandibular Paramolars. Int J Clin Pediatr Dent 2014;7(1):40-42. PMID:25206236

  3. Confabulations after bilateral consecutive strokes of the lenticulostriate arteries.

    PubMed

    Carota, Antonio; Calabrese, Pasquale

    2012-01-01

    We describe the case of a 75-year-old woman who manifested persistent confabulations after two consecutive strokes encompassing the area of the lenticulostriate arteries territory on both hemispheres. Findings reported on this rare clinical syndrome suggest that fantastic confabulations and delusional thoughts may arise after bilateral damage of subcortical nonthalamic structures. PMID:22649343

  4. Confabulations after Bilateral Consecutive Strokes of the Lenticulostriate Arteries

    PubMed Central

    Carota, Antonio; Calabrese, Pasquale

    2012-01-01

    We describe the case of a 75-year-old woman who manifested persistent confabulations after two consecutive strokes encompassing the area of the lenticulostriate arteries territory on both hemispheres. Findings reported on this rare clinical syndrome suggest that fantastic confabulations and delusional thoughts may arise after bilateral damage of subcortical nonthalamic structures. PMID:22649343

  5. Bilateral Nipple Leiomyoma

    PubMed Central

    Kapakli, Mahmut Sertan; Altintoprak, Fatih; Cayırcı, Mine; Manukyan, Manuk Norayk; Kebudi, Abut

    2013-01-01

    Cutaneous leiomyomas are benign smooth muscle neoplasms of the skin. They arise from vascular, arrector pili, genital, and areolar smooth muscles. The most common localizations of cutaneous leiomyomas are the extensor surfaces of the extremities and the trunk. To our knowledge, only few cases of one-sided nipple leiomyomas have been reported, but two-sided nipple leiomyomas have not been presented. For the first time, here, we report a bilateral nipple leiomyoma. PMID:23762734

  6. Compressive Bilateral Filtering.

    PubMed

    Sugimoto, Kenjiro; Kamata, Sei-Ichiro

    2015-11-01

    This paper presents an efficient constant-time bilateral filter that produces a near-optimal performance tradeoff between approximate accuracy and computational complexity without any complicated parameter adjustment, called a compressive bilateral filter (CBLF). The constant-time means that the computational complexity is independent of its filter window size. Although many existing constant-time bilateral filters have been proposed step-by-step to pursue a more efficient performance tradeoff, they have less focused on the optimal tradeoff for their own frameworks. It is important to discuss this question, because it can reveal whether or not a constant-time algorithm still has plenty room for improvements of performance tradeoff. This paper tackles the question from a viewpoint of compressibility and highlights the fact that state-of-the-art algorithms have not yet touched the optimal tradeoff. The CBLF achieves a near-optimal performance tradeoff by two key ideas: 1) an approximate Gaussian range kernel through Fourier analysis and 2) a period length optimization. Experiments demonstrate that the CBLF significantly outperforms state-of-the-art algorithms in terms of approximate accuracy, computational complexity, and usability. PMID:26068315

  7. Bilateral inferior petrosal sinus sampling.

    PubMed

    Zampetti, Benedetta; Grossrubatscher, Erika; Dalino Ciaramella, Paolo; Boccardi, Edoardo; Loli, Paola

    2016-07-01

    Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing's syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88-100% and 67-100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50-70%) makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres. PMID:27352844

  8. Bilateral inferior petrosal sinus sampling

    PubMed Central

    Grossrubatscher, Erika; Dalino Ciaramella, Paolo; Boccardi, Edoardo

    2016-01-01

    Simultaneous bilateral inferior petrosal sinus sampling (BIPSS) plays a crucial role in the diagnostic work-up of Cushing’s syndrome. It is the most accurate procedure in the differential diagnosis of hypercortisolism of pituitary or ectopic origin, as compared with clinical, biochemical and imaging analyses, with a sensitivity and specificity of 88–100% and 67–100%, respectively. In the setting of hypercortisolemia, ACTH levels obtained from venous drainage of the pituitary are expected to be higher than the levels of peripheral blood, thus suggesting pituitary ACTH excess as the cause of hypercortisolism. Direct stimulation of the pituitary corticotroph with corticotrophin-releasing hormone enhances the sensitivity of the procedure. The procedure must be undertaken in the presence of hypercortisolemia, which suppresses both the basal and stimulated secretory activity of normal corticotrophic cells: ACTH measured in the sinus is, therefore, the result of the secretory activity of the tumor tissue. The poor accuracy in lateralization of BIPSS (positive predictive value of 50–70%) makes interpetrosal ACTH gradient alone not sufficient for the localization of the tumor. An accurate exploration of the gland is recommended if a tumor is not found in the predicted area. Despite the fact that BIPSS is an invasive procedure, the occurrence of adverse events is extremely rare, particularly if it is performed by experienced operators in referral centres. PMID:27352844

  9. Synchronous Bilateral Breast Cancers

    PubMed Central

    Subramanyan, Annapurneswari; Radhakrishna, Selvi

    2015-01-01

    Background Bilateral breast cancer (BBC) is not an uncommon entity in contemporary breast clinics. Improved life expectancy after breast cancer treatment and routine use of contra-lateral breast mammography has led to increased incidence of BBC. Our study objective was to define the epidemiological and tumour characteristics of BBC in India. Materials and Methods A total of 1251 breast cancer patients were treated during the period January 2007 to March 2015 and 30 patients were found to have BBC who constituted the study population (60 tumour samples). Synchronous bilateral breast cancers (SBC) was defined as two tumours diagnosed within an interval of 6 months and a second cancer diagnosed after 6 months was labelled as metachronous breast cancer (MBC). Analyses of patient and tumour characteristics were done in this prospective data base of BBC patients. Results Median patient age was 66 years (range 39-85). Majority of the patients had SBC (n=28) and in 12 patients the second tumour was clinically occult and detected only by mammography of the contra-lateral breast. The second tumour was found at lower tumour size compared to the first in 73% of cases and was negative for axillary metastasis in 80% of cases (24/30). Infiltrating ductal carcinoma was the commonest histological type (n=51) and majority of the tumours were ER/PR positive (50/60). Her2 was overexpressed in 13 tumours (21%). Over 70% (22/30) of patients had similar histology in both breasts and amongst them grade concordance was present in about 69% (15/22) of patients. Concordance rates of ER, PR and Her2 statuses were 83%, 80% and 90% respectively. Bilateral mastectomy was the commonest surgery performed in 80% of the patients followed by bilateral breast conservation in 13%. At the end of study period, 26 patients were alive and disease free. Median survival was 29 months (range 3-86 months). Conclusion In most patients with BBC, the second tumour is identified at an early stage than index

  10. Bilateral posterior sternoclavicular dislocation.

    PubMed

    Baumann, Matthias; Vogel, Tobias; Weise, Kuno; Muratore, Tim; Trobisch, Per

    2010-07-01

    Posterior sternoclavicular dislocations are a rare injury, representing <5% of all sternoclavicular dislocations and 1 in 1600 shoulder girdle injuries. Proper imaging with computed tomography and prompt diagnosis are essential steps in preventing potentially lethal complications observed in approximately 3% of all posterior sternoclavicular dislocations. Surgical treatment is necessary if closed reduction fails. With the medial clavicular epiphysis being the last to close (between ages 22 and 25), children and adolescents typically present with epiphyseal fractures rather than joint dislocations. If closed reduction fails, open reduction and internal fixation (ORIF) should be considered in fractures, whereas complex reconstructions with tendon graft procedures have been recommended for joint dislocations. This article presents a case of a traumatic bilateral posterior sternoclavicular dislocation due to an epiphyseal fracture in a 15-year-old boy. To our knowledge, this is the first reported case of a bilateral posterior sternoclavicular dislocation. Attempted closed reduction failed with redislocation after 2 days. The patient subsequently required ORIF. This article describes our technique with anterior retraction of the medial clavicle, closure of the posterior periosteum, and ORIF using nonabsorbable sutures. Postoperative shoulder mobilization was started on day 1. At final follow-up, the patient was completely asymptomatic. PMID:20608625

  11. A 52-Year-Old Male with Bilaterally Duplicated Collecting Systems with Obstructing Ureteral Stones: A Case Report

    PubMed Central

    Scantling, Dane; Ross, Curtis; Altman, Howard

    2013-01-01

    Collecting system duplication is marked by a variety of clinical syndromes. Bilateral and obstructed duplicated systems, particularly with asymmetric levels of duplication, are rare and typically due to ureteric bud development anomalies. The infrequency with which this condition exists makes it a formidable challenge for physicians and patients. To our knowledge, we present the first case report of bilateral obstruction of bilaterally duplicated collecting systems. In our case, a 52-year-old male complaining of low back pain, constipation, urinary urgency and hematuria was found to have bilateral obstructing stones as well as asymmetrical bilateral collecting system duplication. We discuss the natural history of this condition, its consequences and identification. PMID:24917767

  12. Nevus Comedonicus Syndrome

    PubMed Central

    Yadav, Pravesh; Mendiratta, Vibhu; Rana, Shiwangi; Chander, Ram

    2015-01-01

    A case of nevus comedonicus syndrome with atypical cutaneous presentation (widespread involvement without any particular pattern, midline lesions involving lower abdomen and involvement of bilateral pinna), and some unusual skeletal (adduction deformity involving bilateral metatarsal along with medial deviation at the level of tarsometatarsal joint), central nervous system (agenesis of corpus callosum with a interhemispheric cyst), visceral (pancreatic cyst) and neurological manifestations have been illustrated. PMID:26288437

  13. A 12-year-old African American girl with subacute bilateral ophthalmoplegia.

    PubMed

    Bar, Amir; Urbine, Jacqueline; Bahora, Yasmine; Berkenstock, Meghan; Vodzak, Jennifer; Guruprasad, Hamalatha; Sinha, Manisha; Abed, Thair; Legido, Agustín

    2014-06-01

    A twelve-year-old African-American female presented with two week history of progressively worsening headache and fatigue, and vision difficulties for the past week. The physical examination was normal. The neurological evaluation was normal, except for cranial nerves (CN) testing, which showed bilateral restriction of adduction (CN III) and up gaze (CN IV) motions, vertical nystagmus, and left side facial paresis of central origin (CN VII). The bilateral exotropia and ophthalmoplegia are characteristics of WEBINO (Wall-Eyed Bilateral Intranuclear Ophthalmoplegia) syndrome, associated to a brain stem structural lesion. The following causes were evaluated and ruled out: tumor, infection, ischemic stroke, non-infectious inflammation. Pediatric Acquired Demyelinating Syndromes were then considered. Neuromyelitis Optica was ruled out in the absence of neuritis and normal spinal cord MRI. The differential diagnosis between Clinically Isolated Syndrome and Acute Demyelinating Encephalomyelitis, causing an isolated brain stem syndrome, is discussed. PMID:25149958

  14. Simultaneous bilateral spontaneous pneumothorax.

    PubMed

    Graf-Deuel, E; Knoblauch, A

    1994-04-01

    We describe 12 patients with simultaneous bilateral spontaneous pneumothorax (SBSP). They represent 4 percent of patients with spontaneous pneumothorax seen at our hospital from 1971 to 1990. Five of the 12 had no underlying lung disease. In the seven remaining patients, SBSP was secondary to histiocytosis X, lymphangioleiomyomatosis, osteogenic sarcoma with pleural and pulmonary metastases, Hodgkin's disease, mesothelioma, cystic fibrosis, or miliary tuberculosis. Nineteen of the 56 patients with SBSP (34 percent) described in the literature (this series included) had pulmonary disease related to disorders of cells of mesenchymal origin. Emphysema and bullous lung disease were not associated with SBSP. Long-term prognosis was a function of pulmonary status. Four of the patients described herein died during the period reviewed. All suffered from severe underlying disease. In no case was SBSP the main cause of death. With timely treatment, the short-term prognosis is benign even for patients with underlying lung disease. Surgical pleurectomy should be attempted early, especially in SBSP secondary to underlying lung disease. PMID:8162740

  15. Cerebral fat embolism after bilateral total knee replacement arthroplasty -A case report-

    PubMed Central

    Chang, Ri-Na; Lee, Heeseung; Baik, Hee-Jung; Chung, Rack Kyung; Kim, Chi Hyo; Hwang, Tae-Hu

    2010-01-01

    Fat embolism syndrome is a rare and potentially lethal complication most commonly seen in long bone fractures and intramedullary manipulation. The clinical triad of fat embolism syndrome consists of mental confusion, respiratory distress, and petechiae. This study reports a case of cerebral fat embolism syndrome following elective bilateral total knee replacement. After an uneventful anesthesia and initial recovery, the patient developed neurologic symptoms nine hours postoperatively. PMID:21286442

  16. Mosaic Neurocutaneous Disorders and Their Causes.

    PubMed

    Ruggieri, Martino; Praticò, Andrea D

    2015-12-01

    Neurocutaneous disorders are a heterogeneous group of conditions (mainly) affecting the skin [with pigmentary/vascular abnormalities and/or cutaneous tumours] and the central and peripheral nervous system [with congenital abnormalities and/or tumours]. In a number of such disorders, the skin abnormalities can assume a mosaic patterning (usually arranged in archetypical patterns). Alternating segments of affected and unaffected skin or segmentally arranged patterns of abnormal skin often mirror similar phenomena occurring in extra-cutaneous organs/tissues [eg, eye, bone, heart/vessels, lung, kidney and gut]. In some neurocutaneous syndromes the abnormal mosaic patterning involve mainly the skin and the nervous system configuring a (true) mosaic neurocutaneous disorder; or an ordinary trait of a neurocutaneous disorder is sometimes superimposed by a pronounced linear or otherwise segmental involvement; or, lastly, a neurocutaneous disorder can occur solely in a mosaic pattern. Recently, the molecular genetic and cellular bases of an increasing number of neurocutaneous disorders have been unravelled, shedding light on the interplays between common intra- and extra-neuronal signalling pathways encompassing receptor-protein and protein-to-protein cascades (eg, RAS, MAPK, mTOR, PI3K/AKT and GNAQ pathways), which are often responsible of the mosaic distribution of cutaneous and extra-cutaneous features. In this article we will focus on the well known, and less defined mosaic neurocutaneous phenotypes and their related molecular/genetic bases, including the mosaic neurofibromatoses and their related forms (ie, spinal neurofibromatosis and schwannomatosis); Legius syndrome; segmental arrangements in tuberous sclerosis; Sturge-Weber and Klippel-Trenaunay syndromes; microcephaly/megalencephaly-capillary malformation; blue rubber bleb nevus syndrome; Wyburn-Mason syndrome; mixed vascular nevus syndrome; PHACE syndrome; Incontinentia pigmenti; pigmentary mosaicism of the Ito

  17. Acute silicosis with bilateral pneumothorax.

    PubMed

    Srivastava, G N; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa

    2014-01-01

    We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1 year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of acute silicosis as bilateral pneumothorax. PMID:24862410

  18. Acute silicosis with bilateral pneumothorax

    PubMed Central

    Srivastava, G N; Prasad, Rajniti; Meena, Manoj; Hussain, Moosa

    2014-01-01

    We present a case of acute silicosis with bilateral pneumothorax of a 28-year-old man working at a stone crusher factory for 1 year. He presented to the emergency department with cough, respiratory distress and diffuse chest pain. The patient was managed with bilateral intercostal tube drainage under water seal, oxygen inhalation and conservative therapy. On follow-up he showed improvement of resting dyspnoea and was doing well. This case is being reported because of the rare complications of acute silicosis as bilateral pneumothorax. PMID:24862410

  19. Bilateral cerebellar dysfunctions in a unilateral meso-diencephalic lesion.

    PubMed Central

    von Cramon, D

    1981-01-01

    The clinical syndrome of a 65-year-old patient with a slit-shaped right-sided meso-diencephalic lesion was analysed. A cerebellar syndrome with limb-kinetic ataxia, intention tremor and hypotonicity in all extremities as well as ataxic dysarthria was found. The disruption of the two cerebello-(rubro)-thalamic pathways probably explained the signs of bilateral cerebellar dysfunction. The uncrossed ascending limb of the right, and the crossed one of the left brachium conjunctivum may have been damaged by the unilateral lesion extending between caudal midbrain and dorsal thalamus. Images PMID:7241166

  20. Bilateral adrenal gland haemorrhage: an unusual cause

    PubMed Central

    Shenoy, Vasant; Malabu, Usman; Cameron, Donald; Sangla, Kunwarjit

    2014-01-01

    Summary Our patient had drainage of a large amoebic liver abscess. This got complicated by a severe degree of hypotension, which required aggressive fluid resuscitation and hydrocortisone support. Computerised tomography (CT) of the abdomen revealed bilateral adrenal gland haemorrhage (BAH) resulting in primary adrenal gland failure, which was the cause for hypotension. Patient was on long-term warfarin for provoked deep vein thrombosis of lower limb, which was discontinued before the procedure. Thrombophilia profile indicated the presence of lupus anticoagulant factor with prolonged activated partial thromboplastin time (aPTT). Patient was discharged on lifelong warfarin. This case emphasises the need for strong clinical suspicion for diagnosing BAH, rare but life-threatening condition, and its association with amoebic liver abscess and anti-phospholipid antibody syndrome (APLS). Learning points Recognition of BAH as a rare complication of sepsis.APLS can rarely cause BAH. PMID:25276353

  1. Isolated tuberculous lymphadenitis presenting as bilateral buboes

    PubMed Central

    Palanisamy, Arun Prasath; Samuel, Soumya; Vadivel, Sivasubramanian; Kothandapany, Srivenkateswaran

    2015-01-01

    Inguinal and femoral buboes are defined as localized enlargement of lymph nodes in the groin that are painful, and may or may not be fluctuant. We report a case of 42-year-old female who presented with bilateral inguinal swelling of 6 months duration. After a complete evaluation, she was found to be a case of isolated inguinal tuberculous lymphadenitis. There was complete resolution with standard antituberculous therapy. Isolated inguinal tuberculous lymphadenitis though a rare entity in developed countries is not uncommon in developing nations. In this era of syndromic management of sexually transmitted diseases, which carries its own pros and cons, this case report emphasizes the need to look beyond the venereal causes and calls for thorough evaluation and management. PMID:26392662

  2. Metachronous Bilateral Extremity Soft Tissue Sarcomas

    PubMed Central

    Nowrasteh, Ghodratollah; Aziz, Tanim; Assas, Mohammed Al; Nuaimi, Lateefa Al; Marzouqi, Saeeda; Quadri, Asif A.M.; Alrawi, Sadir

    2016-01-01

    Case series Patient: Male, 44 • Male, 58 Final Diagnosis: Soft tissue sarcomas Symptoms: Discomfort • swelling Medication: — Clinical Procedure: Image guided biopsy • metastatic work up • neoadjuvant radiotherapy • radical resection Specialty: Surgery Objective: Rare disease Background: Soft tissue sarcomas (STS) account for approximately 1% of adult malignancies, with 50 to 60% occurring in the extremities. Liposarcoma is the most common type of STS and represent about 20% of total adult sarcomas. There are rare syndromes associated with increased risk of developing STS. Further, chemical compounds such as chlorinated phenols and a few chemotherapeutic drugs have been linked to STS, along with ionizing radiation. Nevertheless, the etiology is uncertain for most of these lesions. Case Report: This report details 2 cases of metachronous bilateral STS of the lower extremities. The first of these presented as a local recurrence of a previously resected right thigh liposarcoma and a new liposarcoma in the left thigh. As mentioned above, among the different subtypes of STS, liposarcoma has the highest tendency for multifocality. The second patient had multifocal metachronous leiomyosarcoma with lung metastases occurring simultaneously with the second presentation. Leiomyosarcoma is another subtype reported to present with multi-focal disease. Conclusions: Despite the rarity of bilateral lesions, their occurrence should not be overlooked in the initial diagnosis and follow-up of the initially detected tumor. Early detection can affect patient survival because their presence predicts unfavorable outcomes. PMID:26744032

  3. Bilateral adrenal masses: a single-centre experience

    PubMed Central

    Bandgar, Tushar; Khare, Shruti; Jadhav, Swati; Lila, Anurag; Goroshi, Manjunath; Kasaliwal, Rajeev; Khadilkar, Kranti; Shah, Nalini S

    2016-01-01

    Background Bilateral adrenal masses may have aetiologies like hyperplasia and infiltrative lesions, besides tumours. Hyperplastic and infiltrative lesions may have coexisting hypocortisolism. Bilateral tumours are likely to have hereditary/syndromic associations. The data on clinical profile of bilateral adrenal masses are limited. Aims To analyse clinical, biochemical and radiological features, and management outcomes in patients with bilateral adrenal masses. Methods Retrospective analysis of 70 patients with bilateral adrenal masses presenting to a single tertiary care endocrine centre from western India (2002–2015). Results The most common aetiology was pheochromocytoma (40%), followed by tuberculosis (27.1%), primary adrenal lymphoma (PAL) (10%), metastases (5.7%), non-functioning adenomas (4.3%), primary bilateral macronodular adrenal hyperplasia (4.3%), and others (8.6%). Age at presentation was less in patients with pheochromocytoma (33 years) and tuberculosis (41 years) compared with PAL (48 years) and metastases (61 years) (P<0.001). The presenting symptoms for pheochromocytoma were hyperadrenergic spells (54%) and abdominal pain (29%), whereas tuberculosis presented with adrenal insufficiency (AI) (95%). The presenting symptoms for PAL were AI (57%) and abdominal pain (43%), whereas all cases of metastasis had abdominal pain. Mean size of adrenal masses was the largest in lymphoma (5.5cm) followed by pheochromocytoma (4.8cm), metastasis (4cm) and tuberculosis (2.1cm) (P<0.001). Biochemically, most patients with pheochromocytoma (92.8%) had catecholamine excess. Hypocortisolism was common in tuberculosis (100%) and PAL (71.4%) and absent with metastases (P<0.001). Conclusion In evaluation of bilateral adrenal masses, age at presentation, presenting symptoms, lesion size, and biochemical features are helpful in delineating varied underlying aetiologies. PMID:27037294

  4. Bilateral internal laryngoceles mimicking asthma.

    PubMed

    Aksoy, Elif A; Elsürer, Cağdaş; Serin, Gediz M; Unal, O Faruk

    2013-05-01

    Laryngocele is an air-filled, abnormal dilation of the laryngeal saccule that extends upward within the false vocal fold, in communication with the laryngeal lumen. A case of 43-year-old male with bilateral internal laryngoceles, who has been treated as asthma for 4 years, is presented. The patient had dyspnea, cough, and excessive phlegm for a month and a late onset stridor. Flexible nasopharyngolaryngoscopy showed bilateral cystic enlargements of the false vocal folds and true vocal folds could not be visualized. Laryngeal CT without contrast enhancement showed bilateral internal laryngoceles. Submucosal total excision of bilateral cystic masses including parts of false vocal folds was performed. The symptoms resolved immediately after surgery. Although the incidence of internal laryngocele is rare, it should be remembered in the differential diagnosis of upper airway problems and diagnostic flexible nasopharnygolaryngoscopy is routinely indicated for airway evaluation in at-risk patients. PMID:24174956

  5. Isolated Bilateral Lacrimal Gland Agenesis.

    PubMed

    Al-Ryalat, Nosaiba T; Ezzat, Jumana W; Ababneh, Osama H; AlRyalat, Saif Aldeen S; Al-Hadidy, Azmy M

    2016-01-01

    A 5-year-old boy presented to the ophthalmology department complaining of absent tearing while crying. Slit-lamp examination showed decreased tear margin film with normal punctae. Orbit magnetic resonance imaging was done and showed bilateral absent lacrimal glands. This is the third case of isolated bilateral lacrimal gland agenesis in the literature. [J Pediatr Ophthalmol Strabismus. 2016;53:e35-e38.]. PMID:27486892

  6. Thoracic Outlet Syndrome Following Breast Implant Rupture

    PubMed Central

    Caplash, Yugesh; Giri, Pratyush; Kearney, Daniel; Wagstaff, Marcus

    2015-01-01

    Summary: We present a patient with bilateral breast implant rupture who developed severe locoregional silicone granulomatous lymphadenopathy. Poly Implant Prothese silicone implants had been used for bilateral breast augmentation 5 years prior. Extracapsular implant rupture and bilateral axillary lymphadenopathy indicated explantation, capsulectomy, and selective lymph node excision. Histology demonstrated silicone lymphadenopathy with no evidence of malignancy. Over the subsequent 12 months, she developed progressive locoregional lymphadenopathy involving bilateral cervical, axillary, and internal mammary groups, resulting in bilateral thoracic outlet syndrome. We report the unusual presentation, progression, and the ultimate surgical management of this patient. PMID:25878942

  7. Simultaneous Bilateral Ophthalmic Artery Chemosurgery for Bilateral Retinoblastoma (Tandem Therapy)

    PubMed Central

    Abramson, David H.; Marr, Brian P.; Francis, Jasmine H.; Dunkel, Ira J.; Fabius, Armida W. M.; Brodie, Scott E.; Mondesire-Crump, Ijah; Gobin, Y. Pierre

    2016-01-01

    Objective Report on the 7-year experience with bilateral ophthalmic artery chemosurgery (OAC-Tandem therapy) for bilateral retinoblastoma. Design Retrospective, single institution study. Subjects 120 eyes of 60 children with bilateral retinoblastoma treated since March 2008. Methods Retrospective review of all children treated at Memorial Sloan Kettering with bilateral ophthalmic artery chemosurgery (Melphalan, Carboplatin, Topotecan, Methotrexate) delivered in the same initial session to both naïve and previously treated eyes. Main Outcome Measures Ocular survival, metastatic disease, patient survival from metastases, second cancers, systemic adverse effects, need for transfusion of blood products, electroretinogram before and after treatment. Results 116 eyes were salvaged (4 eyes were enucleated: 3 because of progressive disease, 1 family choice). Kaplan Meier ocular survival was 99.2% at one year, 96.9% at 2 and 3 years and 94.9% for years 4 through 7. There were no cases of metastatic disease or metastatic deaths with a mean follow-up of 3.01 years. Two children developed second cancers (both pineoblastoma) and one of them died. Transfusion of blood products was required in 3 cases (4 transfusions), 1.9%. Two children developed fever/neutropenia requiring hospitalization (0.95%). ERGs were improved in 21.6% and unchanged after treatment in 52.5% of cases (increase or decrease of less than 25μV). Conclusions Bilateral ophthalmic artery chemosurgery is a safe and effective technique for managing bilateral retinoblastoma-even when eyes are advanced bilaterally, and if both eyes have progressed after systemic chemotherapy. Ocular survival was excellent (94.9% at 8 years), there were no cases of of metastatic disease and no deaths from metastatic disease, but children remain at risk for second cancers. In 21.6% of cases ERG function improved. Despite using chemotherapy in both eyes in the same session, systemic toxicity was low. PMID:27258771

  8. Syndrome in question.

    PubMed

    Dalapicola, Monique Coelho; Veasey, John Verrinder; Lellis, Rute Facchini

    2016-02-01

    Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years. PMID:26982793

  9. Syndrome in question*

    PubMed Central

    Dalapicola, Monique Coelho; Veasey, John Verrinder; Lellis, Rute Facchini

    2016-01-01

    Ross syndrome is a rare disease characterized by peripheral nervous system dysautonomia with selective degeneration of cholinergic fibers. It is composed by the triad of unilateral or bilateral segmental anhidrosis, deep hyporeflexia and Holmes-Adie's tonic pupil. The presence of compensatory sweating is frequent, usually the symptom that most afflicts patients. The aspects of the syndrome are put to discussion due to the case of a male patient, caucasian, 47 years old, with clinical onset of 25 years. PMID:26982793

  10. Bilateral superior rectus transposition for congenital exotropia associated with anomalous medial rectus muscles.

    PubMed

    Kodsi, Sylvia R

    2015-10-01

    Superior rectus transposition to the lateral rectus insertion without inferior rectus transposition has been used to correct esotropic deviations secondary to Duane syndrome and abducens nerve palsy. This is usually combined with an augmented posterior fixation suture of the superior rectus muscle to the lateral rectus muscle and ipsilateral medial rectus recession. We report a child with a large-angle congenital exotropia who was found to have anomalous medial rectus muscles bilaterally. Bilateral superior rectus transposition to the medial rectus insertion with bilateral lateral rectus recessions achieved good ocular alignment in primary position. PMID:26486030

  11. Bilateral carotid and bilateral vertebral artery dissection following facial massage.

    PubMed

    Chakrapani, Andrea L; Zink, Walter; Zimmerman, Robert; Riina, Howard; Benitez, Ronald

    A 50-year-old woman underwent facial massage. After 13 days, she experienced left retro-orbital pain, ptosis, and miosis. Magnetic resonance imaging (MRI) showed stenotic dissection of bilateral cervical internal carotid and vertebral arteries. The intracranial vasculature was intact. She was treated conservatively with long-term oral anticoagulation and remains asymptomatic 18 months later. PMID:18388028

  12. Chronic exertional compartment syndrome of the superficial posterior compartment: Soleus syndrome

    PubMed Central

    Gross, Christopher E; Parekh, Bela J; Adams, Samuel B; Parekh, Selene G

    2015-01-01

    Chronic exertional compartment syndrome (CECS) represents the second most-common cause of exertional leg pain with incidence of 27-33%. CECS of the superficial posterior compartment, or soleus syndrome, is rare and has only been discussed briefly in the literature. We discuss the management of two patients with bilateral soleus syndrome or CECS of the superficial posterior compartment. PMID:26538766

  13. Chronic exertional compartment syndrome of the superficial posterior compartment: Soleus syndrome.

    PubMed

    Gross, Christopher E; Parekh, Bela J; Adams, Samuel B; Parekh, Selene G

    2015-01-01

    Chronic exertional compartment syndrome (CECS) represents the second most-common cause of exertional leg pain with incidence of 27-33%. CECS of the superficial posterior compartment, or soleus syndrome, is rare and has only been discussed briefly in the literature. We discuss the management of two patients with bilateral soleus syndrome or CECS of the superficial posterior compartment. PMID:26538766

  14. Management and outcomes in massive bilateral Wilms’ tumors

    PubMed Central

    Agarwala, Sandeep; Mittal, Deepak; Bhatnagar, Veereshwar; Srinivas, M; Bakhshi, Sameer; Bajpai, Minu; Gupta, Devendra Kumar; Iyer, V. K.; Mohanti, Bidyut K; Thulkar, Sanjay

    2014-01-01

    Purpose: To evaluate the outcome of children with bilateral Wilms’ tumor (BWT) treated on All India Institute of Medical Sciences-Wilms Tumor-99 (AIIMS-WT-99) protocol. Materials and Methods: All children with BWT, registered in our solid tumor clinic from August 1999 through December 2010 were included. Results: Of the 178 fresh cases of Wilms Tumor (WT) treated during this period, 11 (6.2%) had bilateral involvement. All patients except one (12 and 3 cm), had massive bilateral tumors of more than 10 cm on each side. There were eight boys and three girls in the age range 6–30 months. One patient had Denys-Drash syndrome. Twenty renal units were operated upon (12 tumorectomy, five partial nephrectomy, and three nephrectomies), while one patient with inferior vena cava (IVC) thrombus died of renal failure. Tumor spill occurred in three units, lymphnode was positive in two patients. Local recurrence occurred in four patients (six of 18 renal units (33%)—two bilateral and two unilateral). There was one recurrence in the liver that was treated with radio-frequency ablation. The 5-year overall survival (OS) was 90% (95% confidence interval (CI) = 50.8–98.6) and the relapse free survival (RFS) was 38% (95% CI = 6.1–71.6). Conclusion: Massive BWT respond poorly to preoperative chemotherapy, are often not amenable to partial nephrectomy/tumorectomy and have a higher local recurrence rate, giving a poor RFS. PMID:25336802

  15. Bilateral orbital bone infarction in sickle-cell disease.

    PubMed

    Ghafouri, Roya H; Lee, Irene; Freitag, Suzanne K; Pira, Tony N

    2011-01-01

    This is a case of a 2-year-old boy with sickle cell disease who presented with bilateral eyelid swelling, limited extraocular motility, and lateral subperiosteal fluid collection associated with bilateral lateral orbital wall infarctions on MRI. The patient was managed medically with intravenous fluids, analgesics, broad-spectrum antibiotics, systemic steroids, and clinically improved. Patients with sickle cell disease are susceptible to infarction of the orbital bones during vaso-occlusive crises. Orbital wall infarction can lead to acute proptosis and restricted extraocular motility. Orbital wall infarction should be considered in sickle cell patients with orbital diseases so that appropriate treatment can be instituted promptly to prevent the serious sequelae of orbital compression syndrome. PMID:20577135

  16. Female genital tract tumors in Peutz-Jeghers syndrome.

    PubMed

    Chen, K T

    1986-08-01

    Multiple genital tract tumors, including bilateral ovarian sex cord tumors with annular tubules, adenoma malignum of the cervix, and bilateral ovarian mucinous tumors, developed in a female patient with Peutz-Jeghers syndrome. Although each of these tumor types has been reported to occur in this syndrome, the present case appears to be the first in which the syndrome was complicated by tumors of all three types. PMID:3733073

  17. Note: Unshielded bilateral magnetoencephalography system using two-dimensional gradiometers

    NASA Astrophysics Data System (ADS)

    Seki, Yusuke; Kandori, Akihiko; Ogata, Kuniomi; Miyashita, Tsuyoshi; Kumagai, Yukio; Ohnuma, Mitsuru; Konaka, Kuni; Naritomi, Hiroaki

    2010-09-01

    Magnetoencephalography (MEG) noninvasively measures neuronal activity with high temporal resolution. The aim of this study was to develop a new type of MEG system that can measure bilateral MEG waveforms without a magnetically shielded room, which is an obstacle to reducing both the cost and size of an MEG system. An unshielded bilateral MEG system was developed using four two-dimensional (2D) gradiometers and two symmetric cryostats. The 2D gradiometer, which is based on a low-Tc superconducting quantum interference device and wire-wound pickup coil detects a magnetic-field gradient in two orthogonal directions, or ∂/∂x(∂2Bz/∂z2), and reduces environmental magnetic-field noise by more than 50 dB. The cryostats can be symmetrically positioned in three directions: vertical, horizontal, and rotational. This makes it possible to detect bilateral neuronal activity in the cerebral cortex simultaneously. Bilateral auditory-evoked fields (AEF) of 18 elderly subjects were measured in an unshielded hospital environment using the MEG system. As a result, both the ipsilateral and the contralateral AEF component N100m, which is the magnetic counterpart of electric N100 in electroencephalography and appears about 100 ms after the onset of an auditory stimulus, were successfully detected for all the subjects. Moreover, the ipsilateral P50m and the contralateral P50m were also detected for 12 (67%) and 16 (89%) subjects, respectively. Experimental results demonstrate that the unshielded bilateral MEG system can detect MEG waveforms, which are associated with brain dysfunction such as epilepsy, Alzheimer's disease, and Down syndrome.

  18. Bilateral progressive hearing loss and vestibular dysfunction with inner ear antibodies.

    PubMed

    Yukawa, Kumiko; Hagiwara, Akira; Ogawa, Yasuo; Nishiyama, Nobuhiro; Shimizu, Shigetaka; Kawaguchi, Sachie; Nakamura, Mari; Ito, Hiroyuki; Tomiyama, Shunichi; Suzuki, Mamoru

    2010-04-01

    Autoimmune inner ear disease (AIED) is a clinical syndrome of uncertain etiology. We present the neuro-otological findings of 2 cases of bilateral hearing loss, dizziness and the antibody profiles of the inner ears. Case 1 had bilateral progressive hearing loss, vestibular dysfunction and abnormal eye movement as the disease progressed. She had inner ear antibodies against 42 and 58kDa protein antigency on Western blot immune assay, and responded to glycocorticosteroid but not to immunosuppressant treatment. Intratympanic steroid injection temporally eliminated her symptoms. However, she developed idiopathic Cushing's syndrome and underwent labyrinthectomy. Case 2 became deaf as a teenager and experienced dizziness 10 years after becoming deaf. He reacted strongly to 68kDa protein and was a good responder to immunosuppressant with steroid. As we still lack a definitive diagnostic test for AIED, careful observation of the clinical course is critical for differential diagnosis regarding the bilateral progressive hearing loss. PMID:19709829

  19. Bilateral diabetic thigh muscle infarction.

    PubMed

    Barohn, R J; Bazan, C; Timmons, J H; Tegeler, C

    1994-01-01

    A 19-year-old woman with insulin-dependent diabetes mellitus developed pain and tenderness in the medial aspect of the left thigh and calf, followed 1 week later by similar symptoms in the right leg. Technetium 99m pyrophosphate (PYP) radionuclide scans showed increased flow and uptake in the medial thigh muscles. Magnetic resonance imaging (MRI) of the thigh showed increased signal on proton density and T2-weighted images in the medial and lateral thigh compartments. High-resolution B-mode ultrasound showed hyperechoic changes in the anteromedial thigh muscles, with loss of normal myofascial interfaces, and a mixed appearance, bilaterally. Two months later, after the symptoms had begun to resolve, the images had improved. This case documents bilateral diabetic thigh infarction identified by abnormal technetium 99m PYP flow studies, MRI signal, and B-mode ultrasound imaging. PMID:8136579

  20. Bilateral suborbital rash: a dermatologic manifestation of neuropsychiatric disease in a pediatric patient.

    PubMed

    Tong, Lana X; Wang, Yen Tun; Beynet, David

    2014-05-01

    A 10-year-old girl presented with a new onset bilateral suborbital rash. Dermatologic examination revealed violaceous, non-tender, well-demarcated patches with an atypical distribution and pigmentation. After further investigation, a diagnosis of Munchausen syndrome was made and the patient was referred to her primary care provider for further management. PMID:24852783

  1. Bilateral segmental leiomyomas: a case report and review of the literature.

    PubMed

    Suwattee, Pitiporn; Dakin, Cari

    2008-07-01

    Cutaneous leiomyomas are benign tumors of smooth muscles. We report a rare case of bilateral segmental leiomyomas in an 81-year-old man. We also provide a concise review of the literature on leiomyomas, their associations, and genetic defects of multiple cutaneous and uterine leiomyomatosis (MCUL) and hereditary leiomyomatosis and renal cell cancer (HLRCC) syndromes. PMID:18712022

  2. Bilateral posterior cerebral artery infarction.

    PubMed

    Ryan, Davinia; Murphy, Sinead M; Hennessey, Michael J

    2010-01-01

    We report the case of a 70-year-old man who presented with short-term memory impairment and a homonymous left inferior quadrantanopia secondary to simultaneous bilateral posterior cerebral artery (PCA) territory infarction. As in more than a quarter of cases of PCA infarction, no aetiological cause was identified. Unlike the transient nature of symptoms in some cases following unilateral infarction, his deficits persisted on 2-month follow-up. PMID:22798298

  3. Bilateral adrenalectomy in the 21st century: when to use it for hypercortisolism?

    PubMed

    Guerin, Carole; Taieb, David; Treglia, Giorgio; Brue, Thierry; Lacroix, André; Sebag, Frederic; Castinetti, Frederic

    2016-02-01

    Therapeutic options available for the treatment of Cushing's syndrome (CS) have expanded over the last 5 years. For instance, the efficient management of severe hypercortisolism using a combination of fast-acting steroidogenesis inhibitors has been reported. Recent publications on the long-term efficacy of drugs or radiation techniques have also demonstrated low toxicity. These data should encourage endocrinologists to reconsider the place of bilateral adrenalectomy in patients with ACTH-dependent aetiologies of CS; similarly, the indication of bilateral adrenalectomy is reassessed in primary bilateral macronodular adrenal hyperplasia. The objective of this review is to compare the efficacy and side effects of the various therapeutic options of hypercortisolism with those of bilateral adrenalectomy, in order to better define its indications in the 21st century. PMID:26739832

  4. A 19-year-old man with relapsing bilateral pneumothorax, hemoptysis, and intrapulmonary cavitary lesions diagnosed with vascular Ehlers-Danlos syndrome and a novel missense mutation in COL3A1.

    PubMed

    Abrahamsen, Bjørg J; Kulseth, Mari Ann; Paus, Benedicte

    2015-05-01

    A 19-year-old sportsman experienced a right-sided pneumothorax and hemoptysis after having had an intermittent cough and blood-tinged sputum for 2 months. A chest CT scan revealed small cavitary lesions in both lungs. The relapsing pneumothorax was treated with a chest tube twice, as well as surgically after the second relapse. Two months after surgery, the patient developed a cough, fever, and high C-reactive protein levels. At that time, large consolidations had developed in the right lung, while the left lung subsequently collapsed due to pneumothorax. The patient's physical appearance and anamnestic information led us to suspect a genetic connective tissue disease. A sequencing analysis of the COL3A1 gene identified a novel, de novo missense mutation that confirmed the diagnosis of vascular Ehlers-Danlos syndrome (EDS). This atypical presentation of vascular EDS with intrathoracic complications shows that enhanced awareness is required and demonstrates the usefulness of the genetic analyses that are clinically available for several hereditary connective tissue disorders. PMID:25940258

  5. Compensation Following Bilateral Vestibular Damage

    PubMed Central

    McCall, Andrew A.; Yates, Bill J.

    2011-01-01

    Bilateral loss of vestibular inputs affects far fewer patients than unilateral inner ear damage, and thus has been understudied. In both animal subjects and human patients, bilateral vestibular hypofunction (BVH) produces a variety of clinical problems, including impaired balance control, inability to maintain stable blood pressure during postural changes, difficulty in visual targeting of images, and disturbances in spatial memory and navigational performance. Experiments in animals have shown that non-labyrinthine inputs to the vestibular nuclei are rapidly amplified following the onset of BVH, which may explain the recovery of postural stability and orthostatic tolerance that occurs within 10 days. However, the loss of the vestibulo-ocular reflex and degraded spatial cognition appear to be permanent in animals with BVH. Current concepts of the compensatory mechanisms in humans with BVH are largely inferential, as there is a lack of data from patients early in the disease process. Translation of animal studies of compensation for BVH into therapeutic strategies and subsequent application in the clinic is the most likely route to improve treatment. In addition to physical therapy, two types of prosthetic devices have been proposed to treat individuals with bilateral loss of vestibular inputs: those that provide tactile stimulation to indicate body position in space, and those that deliver electrical stimuli to branches of the vestibular nerve in accordance with head movements. The relative efficacy of these two treatment paradigms, and whether they can be combined to facilitate recovery, is yet to be ascertained. PMID:22207864

  6. Macular edema-like change and pseudopapilledema in a case of Scheie syndrome.

    PubMed

    Usui, T; Shirakashi, M; Takagi, M; Abe, H; Iwata, K

    1991-09-01

    We reported a case of Scheie syndrome in which diffuse fine corneal deposits, pigmentary retinal degeneration, pseudopapilledema, and macular edema-like change were observed bilaterally. This is the first report describing macular change in Scheie syndrome. PMID:1836802

  7. Pneumomediastinum and bilateral pneumothorax following near drowning in shallow water

    PubMed Central

    Govindaraj, Santhiya; Viswanathan, Stalin

    2011-01-01

    We report pneumomediastinum, bilateral pneumothorax and acute respiratory distress syndrome in a victim of near drowning who was intoxicated and did not have thoracic or neck trauma. Chest radiograph revealed the above findings, later confirmed by computed tomography. He was in shock and also had gastrointestinal (GI) bleeding and renal dysfunction. With adequate resuscitative measures including fluids, blood transfusions, intercostal tube drainage and mechanical ventilation he made a complete recovery. Good prognostic indicators in near drowning patients include higher Glasgow Coma Scale, short submersion time and quick resuscitative measures even in the presence of serious cardiorespiratory or hemodynamic compromise. PMID:24765331

  8. Pseudohypoaldosteronism presenting with thrombocytosis and bilateral pneumothoraces in an infant.

    PubMed

    Javed, Asma; Leonard, Jennifer M; Cramer, Carl; Kumar, Seema; Kirmani, Salman; Brands, Chad K

    2013-01-01

    Pseudohypoaldosteronism type 1 (PHA-1) is a rare salt-wasting syndrome caused by a peripheral resistance to aldosterone. Here, we describe an unusual presentation of the autosomal dominant PHA-1 featuring bilateral pneumothoraces at birth, thrombocytosis in infancy, and hypercalcemia in addition to the well-described findings of hyponatremia, hyperkalemia, and failure to thrive. These findings contribute to the limited case descriptions of PHA-1 and may suggest additional diagnostic considerations in a neonate presenting with hyperkalemia, hyponatremia, and failure to thrive. PMID:23327809

  9. Bilateral cervical ribs in a Dobermann Pinscher.

    PubMed

    Ricciardi, M; De Simone, A; Gernone, F; Giannuzzi, P

    2015-01-01

    An 11-year-old intact female Doberman Pinscher was presented with the complaint of non-ambulatory tetraparesis. Clinical and neurological examination revealed a caudal cervical spinal cord disfunction (C6-T2 spinal cord segments). Magnetic resonance imaging and computed tomographic (CT) findings of the cervical spine were consistent with caudal cervical spondylomyelopathy (CSM). During the diagnostic work-up for the cervical spine, bilateral bone anomalies involving the seventh cervical vertebra and the first ribs were found on radiographs and CT examination. The rib anomalies found in this dog appear similar to cervical ribs widely described in human medicine. In people, cervical ribs are associated with a high rate of stillbirth, early childhood cancer, and can cause the thoracic outlet syndrome, characterized by neurovascular compression at level of superior aperture of the chest. In dogs, only some sporadic anatomopathological descriptions of cervical ribs exist. In this report the radiographic and CT findings of these particular vertebral and rib anomalies along with their relationships with adjacent vasculature and musculature are shown intravitam in a dog. Specific radiographic and CT findings described in this report may help in reaching a presumptive diagnosis of this anomaly. Finally, their clinical and evolutionary significance are discussed. PMID:25650786

  10. Bilateral lower extremity swelling: black pearl.

    PubMed

    Smithson, Clinton C; Ham, Jared C; Juergens, Andrew L

    2015-12-01

    Iatrogenic pneumothorax secondary to thoracentesis is relatively uncommon but does present to the emergency department (ED). Iatrogenic pneumothoraces developing tension physiology are rare. We report a case of an elderly female patient presenting to the ED with an isolated chief complaint of bilateral leg swelling, beginning the day after a thoracentesis, which was performed 3 days prior for pleural effusions secondary to lung cancer. Given that the patient was hemodynamically stable, not hypoxic, and had a history of chronic obstructive pulmonary disease and recent history of pleural effusions with diminished lung sounds throughout, this was a radiologic diagnosis. Immediately upon diagnosis, a 10F intrapleural catheter was inserted at the second intercostal space in the midclavicular line with successful resolution of the tension phenomenon. The patient tolerated the procedure well, and the catheter was removed on hospital day 2 without recurrence of the pneumothorax. She experienced resolution of her lower extremity swelling and was discharged from the hospital 2 days later. Isolated inferior vena cava syndrome secondary to a subacute tension pneumothorax was likely the cause of the patient's symptoms. This presentation is very rare and is undocumented in the literature. A high degree of suspicion for acute chest pathology should exist in every patient presenting to the ED with history of recent pleural violation. PMID:26003746

  11. [Recovery of Cushing syndrome revealing McCune-Albright syndrome].

    PubMed

    Halioui-Louhaichi, S; Dridi, Y; Azzabi, O; Selmi, I; Fetni, I; Siala, N; Maherzi, A

    2016-01-01

    Cushing syndrome (CS) is a rare feature of McCune-Albright syndrome. Treatments consist of bilateral adrenalectomy followed by lifelong glucocorticoid and mineralocorticoid treatment. However, cases of spontaneous remission of CS have been reported in the literature. We report a case of McCune-Albright syndrome with CS treated with metyrapone for 30 months with prolonged remission after a 12-year follow-up. Adrenalectomy may be avoided in some cases of CS caused by McCune-Albright syndrome. Metyrapone could be a good alternative to surgical treatment. PMID:26552628

  12. Multiresolution Bilateral Filtering for Image Denoising

    PubMed Central

    Zhang, Ming; Gunturk, Bahadir K.

    2008-01-01

    The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique. An important issue with the application of the bilateral filter is the selection of the filter parameters, which affect the results significantly. There are two main contributions of this paper. The first contribution is an empirical study of the optimal bilateral filter parameter selection in image denoising applications. The second contribution is an extension of the bilateral filter: multiresolution bilateral filter, where bilateral filtering is applied to the approximation (low-frequency) subbands of a signal decomposed using a wavelet filter bank. The multiresolution bilateral filter is combined with wavelet thresholding to form a new image denoising framework, which turns out to be very effective in eliminating noise in real noisy images. Experimental results with both simulated and real data are provided. PMID:19004705

  13. Bilateral Adrenal Adenoma Presented As Multiple Metatarsal And Phalangeal Fractures

    PubMed Central

    LiYeung, L L; Lui, T H

    2015-01-01

    Introduction: Symptomatic adrenal adenoma usually presents with systemic symptoms. Depending on the function of the adenoma, the patient can present with pheochromocytoma-like symptoms; primary hyperaldosteronism and Cushing syndrome (weight gain, weakness, depression, and bruising). Case report: A 41 year-old lady presented with multiple metatarsal and phalangeal fractures of the both feet without significant injury. DEXA scan showed evidence of osteoporosis. Investigations showed that the picture was compatible with adrenal Cushing syndrome. Computed tomogram showed bilateral adrenal adenoma. Adrenal cortex scintigraphy with NP-59 scan showed hyperfunctioning right adrenal adenoma. Laproscopic R adrenalectomy was performed and histological study confirmed adrenal cortical adenoma with adjacent cortical atrophy suggestive of a functioning adenoma. Post-operatively, she was put on hydrocortisone replacement and recovered well. Conclusion: Adrenal adenoma can present with insufficiency fractures of the feet. PMID:27299107

  14. Bilateral ECT induces bilateral increases in regional cortical thickness.

    PubMed

    van Eijndhoven, P; Mulders, P; Kwekkeboom, L; van Oostrom, I; van Beek, M; Janzing, J; Schene, A; Tendolkar, I

    2016-01-01

    Electroconvulsive therapy (ECT) is the most effective treatment for patients suffering from severe or treatment-resistant major depressive disorder (MDD). Unfortunately its underlying neurobiological mechanisms are still unclear. One line of evidence indicates that the seizures produced by ECT induce or stimulate neuroplasticity effects. Although these seizures also affect the cortex, the effect of ECT on cortical thickness is not investigated until now. We acquired structural magnetic resonance imaging data in 19 treatment-resistant MDD patients before and after a bilateral ECT course, and 16 healthy controls at 2 time points, and compared changes in cortical thickness between the groups. Our results reveal that ECT induces significant, bilateral increases in cortical thickness, including the temporal pole, inferior and middle temporal cortex and the insula. The pattern of increased cortical thickness was predominant in regions that are associated with seizure onset in ECT. Post hoc analyses showed that the increase in thickness of the insular cortex was larger in responders than in non-responders, which may point to a specific relationship of this region with treatment effects of ECT. PMID:27552587

  15. Multistep joint bilateral depth upsampling

    NASA Astrophysics Data System (ADS)

    Riemens, A. K.; Gangwal, O. P.; Barenbrug, B.; Berretty, R.-P. M.

    2009-01-01

    Depth maps are used in many applications, e.g. 3D television, stereo matching, segmentation, etc. Often, depth maps are available at a lower resolution compared to the corresponding image data. For these applications, depth maps must be upsampled to the image resolution. Recently, joint bilateral filters are proposed to upsample depth maps in a single step. In this solution, a high-resolution output depth is computed as a weighted average of surrounding low-resolution depth values, where the weight calculation depends on spatial distance function and intensity range function on the related image data. Compared to that, we present two novel ideas. Firstly, we apply anti-alias prefiltering on the high-resolution image to derive an image at the same low resolution as the input depth map. The upsample filter uses samples from both the high-resolution and the low-resolution images in the range term of the bilateral filter. Secondly, we propose to perform the upsampling in multiple stages, refining the resolution by a factor of 2×2 at each stage. We show experimental results on the consequences of the aliasing issue, and we apply our method to two use cases: a high quality ground-truth depth map and a real-time generated depth map of lower quality. For the first use case a relatively small filter footprint is applied; the second use case benefits from a substantially larger footprint. These experiments show that the dual image resolution range function alleviates the aliasing artifacts and therefore improves the temporal stability of the output depth map. On both use cases, we achieved comparable or better image quality with respect to upsampling with the joint bilateral filter in a single step. On the former use case, we feature a reduction of a factor of 5 in computational cost, whereas on the latter use case, the cost saving is a factor of 50.

  16. Bilateral Impedance Control For Telemanipulators

    NASA Technical Reports Server (NTRS)

    Moore, Christopher L.

    1993-01-01

    Telemanipulator system includes master robot manipulated by human operator, and slave robot performing tasks at remote location. Two robots electronically coupled so slave robot moves in response to commands from master robot. Teleoperation greatly enhanced if forces acting on slave robot fed back to operator, giving operator feeling he or she manipulates remote environment directly. Main advantage of bilateral impedance control: enables arbitrary specification of desired performance characteristics for telemanipulator system. Relationship between force and position modulated at both ends of system to suit requirements of task.

  17. Symmetric bilateral caudate, hippocampal, cerebellar, and subcortical white matter MRI abnormalities in an adult patient with heat stroke

    PubMed Central

    Schucany, William G.

    2008-01-01

    Heat stroke is the end result of excess heat stress and results in multiorgan dysfunction with a propensity for central nervous system (CNS) injury. Damage to the CNS appears to be the result of multiple mechanisms, including direct heat damage and the initiation of a sepsis-type syndrome. Only a few scattered case reports exist in the literature that document CNS damage via imaging. We present a case with symmetric bilateral magnetic resonance findings in the caudate nuclei, subcortical white matter, hippocampi, and cerebellum. To our knowledge, this is the first case to report symmetric bilateral caudate abnormality and bilateral hippocampal enhancement. PMID:18982090

  18. Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently

    PubMed Central

    Lee, Seung-Eun; Lee, You-Bin; Seok, Hyeri; Shin, In Seub; Eun, Yeong Hee; Kim, Jung-Han; Oh, Young Lyun

    2015-01-01

    A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses. PMID:26248855

  19. Bilateral Adrenocortical Masses Producing Aldosterone and Cortisol Independently.

    PubMed

    Lee, Seung Eun; Kim, Jae Hyeon; Lee, You Bin; Seok, Hyeri; Shin, In Seub; Eun, Yeong Hee; Kim, Jung Han; Oh, Young Lyun

    2015-12-01

    A 31-year-old woman was referred to our hospital with symptoms of hypertension and bilateral adrenocortical masses with no feature of Cushing syndrome. The serum aldosterone/renin ratio was elevated and the saline loading test showed no suppression of the plasma aldosterone level, consistent with a diagnosis of primary hyperaldosteronism. Overnight and low-dose dexamethasone suppression tests showed no suppression of serum cortisol, indicating a secondary diagnosis of subclinical Cushing syndrome. Adrenal vein sampling during the low-dose dexamethasone suppression test demonstrated excess secretion of cortisol from the left adrenal mass. A partial right adrenalectomy was performed, resulting in normalization of blood pressure, hypokalemia, and high aldosterone level, implying that the right adrenal mass was the main cause of the hyperaldosteronism. A total adrenalectomy for the left adrenal mass was later performed, resulting in a normalization of cortisol level. The final diagnosis was bilateral adrenocortical adenomas, which were secreting aldosterone and cortisol independently. This case is the first report of a concurrent cortisol-producing left adrenal adenoma and an aldosterone-producing right adrenal adenoma in Korea, as demonstrated by adrenal vein sampling and sequential removal of adrenal masses. PMID:26248855

  20. Sequentially evolved bilateral epidural haematomas.

    PubMed

    Rochat, P; Johannesen, H H; Poulsgård, L; Bøgeskov, L

    2002-12-01

    Sequentially evolved bilateral epidural haematomas, where the second haematoma evolves after surgical removal of the first haematoma, are rarely reported. We report two cases of this entity. One patient was involved in a road traffic accident and the other was suffering from a head injury after an assault. CT scans showed that both patients had an unilateral epidural haematoma with a thin presumably epidural haemorrhage on the opposite side. Both patients were operated for their epidural haematomas, but did not improve after surgical treatment, and postoperative CT scans revealed evolving of an epidural haematoma on the opposite side. After evacuation of the second epidural haematoma both patients recovered quickly. Sequentially evolved bilateral epidural haematomas are rare, but must be considered in the postoperative intensive care treatment in patients with epidural haematomas. Both cases emphasize the need for intensive care monitoring after an operation for an epidural haematoma and the need for CT scans if the patient does not improve quickly after removal of the haematoma. This is especially important if a small contralateral haematoma is seen on the initial CT scan. PMID:12445923

  1. [Simultaneous bilateral pneumothorax. Case report].

    PubMed

    Paolini, A; Caminiti, F; Tosato, F; Ruggieri, M; Paolini, G; Carnevale, L; Corsini, F; Marano, S; Monsellato, I

    2001-04-01

    A case report of a 44 year-old white man admitted to the surgical unit for a bilateral simultaneous pneumothorax is presented. The pneumothorax occurred on day one after a surgical operation for discal hernia; in the past the patient already presented a right spontaneous pneumothorax at 32 years of age and a left pneumothorax at 37 years of age, both treated with a pleural drainage. A thoracic drain was bilaterally positioned with a good result only in the right side. The persistence of the left pneumothorax induced the authors to perform a postero-lateral thoracotomy bullae excision and pleurectomy with a good postoperative course. After a few months a new right pneumothorax occurred and the patient was treated with a right postero-lateral thoracotomy, bullae resection and pleurectomy. On the basis of the case reported, the authors consider the different opportunities in the treatment of spontaneous pneumothorax in relation to the present knowledges and technologies. Surgical procedure is to be preferred in case of persistence of pneumothorax despite a pleural drain and in case of pneumothorax in high risk subjects. Even if thoracoscopy seems to give better results regarding postoperative pain, it is not always possible with such a method to perform a careful pleurectomy neither to obtain it in all cases (above all in secondary pneumothorax). Every case must then be carefully studied to choose the best treatment at present available. PMID:11353349

  2. Bilateral internal thoracic artery grafting

    PubMed Central

    2013-01-01

    The effectiveness of the left internal mammary artery graft to the anterior descending coronary artery as a surgical strategy has been shown to improve the survival rate and decrease the risk of adverse cardiac events in patients undergoing coronary bypass surgery. These clinical benefits appear to be related to the superior short and long-term patency rates of the internal thoracic artery graft. Although the advantages of using of both internal thoracic arteries (ITA) for bypass grafting have taken longer to prove, recent results from multiple data sets now support these findings. The major advantage of bilateral ITA grafting appears to be improved survival rate, while the disadvantages of complex ITA grafting include the increased complexity of operation, and an increased risk of wound complications. While these short-term disadvantages have been mitigated in contemporary surgical practice, they have not eliminated. Bilateral ITA grafting should be considered the procedure of choice for patients undergoing coronary bypass surgery that have a predicted survival rate of longer than ten years. PMID:23977627

  3. Unusual Bilateral Paramolars Associated with Clinical Complications

    PubMed Central

    Sulabha, A. N.; Sameer, C.

    2015-01-01

    Paramolars are rare supernumerary structures of maxillofacial complex that occur buccally or lingually near the molar row. Predominantly these occur singly; bilateral presentation is very rare. This paper reports two unusual bilateral presentations of paramolars with clinical complication and its management. One of the cases in the present paper also documents the cooccurrence of bilateral paramolars and microdontia of single tooth and one of its paramolars presented with multilobed crown with an anomalous buccal tubercle. PMID:26078890

  4. A clinical analysis of bilateral orbital fracture.

    PubMed

    Roh, Joon Ho; Jung, Jee Woong; Chi, Mijung

    2014-03-01

    Although bilateral orbital fracture can cause serious eyeball and facial skeletal problems, few reports have been issued on the topic. We analyzed the clinical features of bilateral orbital fracture by reviewing the medical records of 147 patients and compared bilateral and unilateral fractures by reviewing the literature.Bilateral orbital fracture was most common in men aged between 50 and 59 years. A traffic accident was the leading cause of trauma, and average time between trauma and surgery was 12.2 days. Bilateral medial fracture accompanied by nasal fracture accounted for the overwhelming majority, and impure blowout fracture in at least 1 eye occurred in 69.4% of the 147 patients. Associated ocular injuries seemed to be similar for bilateral and unilateral fracture. Thirty-five patients (23.8%) had other multiple traumas affecting other than the eyes, and this significantly increased the need for surgery (P < 0.05). Of the 48 patients who underwent surgery, including 4 cases of bilateral surgery, 21 patients who had ocular motility restriction with central diplopia within 30 degrees almost completely recovered. No significant relation between the timing of surgery and improvement was found. Although unilateral surgery was performed in most cases, facial asymmetry related to enophthalmos was unclear at 6 months postoperatively.In summary, bilateral orbital fracture was found to be clinically distinguishable from unilateral fracture in several aspects. We hope these findings provide a reference guide to the approach and management of bilateral orbital fracture. PMID:24514894

  5. Fast bilateral filtering using recursive moving sum

    NASA Astrophysics Data System (ADS)

    Igarashi, Masaki; Ikebe, Masayuki; Shimoyama, Sohsuke; Motohisa, Junichi

    We propose a constant-time algorithm for a bilateral filter. Bilateral filter can be converted into the operation of three-dimensional (3D) convolution. By using recursive moving sum, we can reduce the number of calculations needed to construct a pseudo-Gaussian filter. Applying one-dimensional Gaussian filter to the 3D convolution, we achieved a constant-time bilateral filter. We used a 3-GHz CPU without SIMD instructions, or multi-thread operations. We confirmed our proposed bilateral filter to be processed in constant time. In practical conditions, high PSNR values over 40 dB are obtained.

  6. Bilateral Neck of Femur Fractures in a Bilateral Below-Knee Amputee: A Unique Case.

    PubMed

    Lancer, Hannah R; Smitham, Peter; Ray, Pinak

    2016-01-01

    According to the National Hip Fracture Database, over 64,000 patients were admitted with a hip fracture across England, Wales, and Northern Ireland in 2013, but very few are bilateral, and there are no current cases in the literature of bilateral neck of femur fractures in a patient with bilateral below-knee amputations. We present a case of a 69-year-old bilateral below-knee amputee male admitted to the emergency department with bilateral hip pain and radiological evidence of bilateral displaced neck of femur fractures. The patient subsequently underwent synchronous bilateral total hip replacements under general anaesthetic and an epidural and then went on to make a full recovery. He was discharged 27 days after arrival in hospital. Outpatient follow-up at 3 months has shown that the patient has returned to a similar level of preinjury function and is still able to carry out his daily activities with walking aids and bilateral leg prostheses. PMID:26881162

  7. Bilateral Neck of Femur Fractures in a Bilateral Below-Knee Amputee: A Unique Case

    PubMed Central

    Lancer, Hannah R.

    2016-01-01

    According to the National Hip Fracture Database, over 64,000 patients were admitted with a hip fracture across England, Wales, and Northern Ireland in 2013, but very few are bilateral, and there are no current cases in the literature of bilateral neck of femur fractures in a patient with bilateral below-knee amputations. We present a case of a 69-year-old bilateral below-knee amputee male admitted to the emergency department with bilateral hip pain and radiological evidence of bilateral displaced neck of femur fractures. The patient subsequently underwent synchronous bilateral total hip replacements under general anaesthetic and an epidural and then went on to make a full recovery. He was discharged 27 days after arrival in hospital. Outpatient follow-up at 3 months has shown that the patient has returned to a similar level of preinjury function and is still able to carry out his daily activities with walking aids and bilateral leg prostheses. PMID:26881162

  8. Independent Tumor Origin in Two Cases of Synchronous Bilateral Clear Cell Renal Cell Carcinoma.

    PubMed

    Ji, Zhengguo; Zhao, Jialu; Zhao, Tian; Han, Yuying; Zhang, Yujun; Ye, Haihong

    2016-01-01

    Bilateral renal cell carcinomas (RCCs) pose a challenge for clinical treatment and management. Most bilateral RCCs are sporadic, and do not show a hereditary pattern indicative of VHL syndrome or other inherited cancers. The origin and evolution of these sporadic bilateral RCCs remains elusive. We obtained normal and tumor samples from two male patients suffering from early stage synchronous bilateral clear cell RCC (ccRCC), and analyzed genomic DNA using whole exome sequencing and bisulfite pyrosequencing. We detected distinct 3p loss of heterozygosity (LOH) in both tumors in each patient. Two tumors within the same patient harbored distinct driver mutations and different CpG hypermethylation sites in the VHL promoter. Moreover, tumors exhibit independent evolutionary trajectories. Therefore, distinct 3p LOH, combined with contingent driver gene mutations and independent VHL hypermethylation, led to independent tumor origin and parallel evolution of bilateral ccRCC in these two patients. Our results indicate that tumors in these two cases were not due to common germline oncogenic mutations. They were results of multiple de novo mutations in each kidney, rather than primary ccRCC with contralateral renal metastasis. Therefore, histopathologic and genetic profiling from single tumor specimen may underestimate the mutational burden and somatic heterogeneity of bilateral ccRCCs. PMID:27383411

  9. Independent Tumor Origin in Two Cases of Synchronous Bilateral Clear Cell Renal Cell Carcinoma

    PubMed Central

    Ji, Zhengguo; Zhao, Jialu; Zhao, Tian; Han, Yuying; Zhang, Yujun; Ye, Haihong

    2016-01-01

    Bilateral renal cell carcinomas (RCCs) pose a challenge for clinical treatment and management. Most bilateral RCCs are sporadic, and do not show a hereditary pattern indicative of VHL syndrome or other inherited cancers. The origin and evolution of these sporadic bilateral RCCs remains elusive. We obtained normal and tumor samples from two male patients suffering from early stage synchronous bilateral clear cell RCC (ccRCC), and analyzed genomic DNA using whole exome sequencing and bisulfite pyrosequencing. We detected distinct 3p loss of heterozygosity (LOH) in both tumors in each patient. Two tumors within the same patient harbored distinct driver mutations and different CpG hypermethylation sites in the VHL promoter. Moreover, tumors exhibit independent evolutionary trajectories. Therefore, distinct 3p LOH, combined with contingent driver gene mutations and independent VHL hypermethylation, led to independent tumor origin and parallel evolution of bilateral ccRCC in these two patients. Our results indicate that tumors in these two cases were not due to common germline oncogenic mutations. They were results of multiple de novo mutations in each kidney, rather than primary ccRCC with contralateral renal metastasis. Therefore, histopathologic and genetic profiling from single tumor specimen may underestimate the mutational burden and somatic heterogeneity of bilateral ccRCCs. PMID:27383411

  10. Bilateral treatment for alopecia areata.

    PubMed

    Torchia, Daniele; Schachner, Lawrence A

    2010-01-01

    A 4-year-old, otherwise healthy white girl was referred for a 15-month history of alopecia areata. Anthralin 0.1% cream was prescribed for the left side of the scalp, while corticosteroids for the right side. After 4 months, only the right side of the scalp showed hair regrowth. Half-side strategy, that is, treating one side and managing the other--divided by the mid sagittal suture--as an internal control for no treatment, placebo or other treatment, has been commonly used in clinical studies for decades. In everyday practice, bilateral treatment is useful to evaluate the responsiveness to two topically delivered interventions and diminishes the time necessary to identify an effective one. PMID:20653874

  11. Genetic Syndromes Associated with Craniosynostosis.

    PubMed

    Ko, Jung Min

    2016-05-01

    Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures. It leads not only to secondary distortion of skull shape but to various complications including neurologic, ophthalmic and respiratory dysfunction. Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. Both environmental factors and genetic factors are associated with development of craniosynostosis. Nonsyndromic craniosynostosis accounts for more than 70% of all cases. Syndromic craniosynostosis with a certain genetic cause is more likely to involve multiple sutures or bilateral coronal sutures. FGFR2, FGFR3, FGFR1, TWIST1 and EFNB1 genes are major causative genes of genetic syndromes associated with craniosynostosis. Although most of syndromic craniosynostosis show autosomal dominant inheritance, approximately half of patients are de novo cases. Apert syndrome, Pfeiffer syndrome, Crouzon syndrome, and Antley-Bixler syndrome are related to mutations in FGFR family (especially in FGFR2), and mutations in FGFRs can be overlapped between different syndromes. Saethre-Chotzen syndrome, Muenke syndrome, and craniofrontonasal syndrome are representative disorders showing isolated coronal suture involvement. Compared to the other types of craniosynostosis, single gene mutations can be more frequently detected, in one-third of coronal synostosis patients. Molecular diagnosis can be helpful to provide adequate genetic counseling and guidance for patients with syndromic craniosynostosis. PMID:27226847

  12. Genetic Syndromes Associated with Craniosynostosis

    PubMed Central

    2016-01-01

    Craniosynostosis is defined as the premature fusion of one or more of the cranial sutures. It leads not only to secondary distortion of skull shape but to various complications including neurologic, ophthalmic and respiratory dysfunction. Craniosynostosis is very heterogeneous in terms of its causes, presentation, and management. Both environmental factors and genetic factors are associated with development of craniosynostosis. Nonsyndromic craniosynostosis accounts for more than 70% of all cases. Syndromic craniosynostosis with a certain genetic cause is more likely to involve multiple sutures or bilateral coronal sutures. FGFR2, FGFR3, FGFR1, TWIST1 and EFNB1 genes are major causative genes of genetic syndromes associated with craniosynostosis. Although most of syndromic craniosynostosis show autosomal dominant inheritance, approximately half of patients are de novo cases. Apert syndrome, Pfeiffer syndrome, Crouzon syndrome, and Antley-Bixler syndrome are related to mutations in FGFR family (especially in FGFR2), and mutations in FGFRs can be overlapped between different syndromes. Saethre-Chotzen syndrome, Muenke syndrome, and craniofrontonasal syndrome are representative disorders showing isolated coronal suture involvement. Compared to the other types of craniosynostosis, single gene mutations can be more frequently detected, in one-third of coronal synostosis patients. Molecular diagnosis can be helpful to provide adequate genetic counseling and guidance for patients with syndromic craniosynostosis. PMID:27226847

  13. Submaximal Expression of the Bilateral Deficit

    ERIC Educational Resources Information Center

    McLean, Scott P.; Vint, Peter F.; Stember, Amanda J.

    2006-01-01

    Thirty-six participants performed bilateral and unilateral isometric elbow flexion trials at what they perceived to be 100, 75, 50, and 25% of maximal effort. Absolute bilateral deficits ranged from -16% at 25% effort to -10% at 100% effort. The deficit included a component independent of consciousness and a component inversely related to…

  14. Bilateral microperc in a severe kyphoscoliosis

    PubMed Central

    Dağgülli, Mansur; Penbegül, Necmettin; Dede, Onur; Utanğaç, Mehmet Mazhar

    2016-01-01

    Percutaneous nephrolithotomy is the standard modality for large renal calculi in normal and abnormal renal anatomic situations. This case report describes a 57-year-old male patient who presented with bilateral kidney stones and severe kyphoscoliosis. He had successfully been treated with a bilateral microperc technique. PMID:27011881

  15. CT demonstration of bilateral adrenal hemorrhage

    SciTech Connect

    Ling, D.; Korobkin, M.; Silverman, P.M.; Dunnick, N.R.

    1983-08-01

    Bilateral adrenal hemorrhage with subsequent adrenal insufficiency is a recognized complication of anticoagulant therapy. Because the clinical manifestations are often nonspecific, the antemortem diagnosis of adrenal hemorrhage has been a difficult clinical problem. Computed tomography (CT) provides detailed images of the adrenal glands that are not possible with conventional imaging methods. The CT findings of bilateral adrenal hemorrhage in an anticoagulated patient are reported.

  16. Simultaneous and spontaneous bilateral quadriceps tendons rupture.

    PubMed

    Celik, Evrim Coşkun; Ozbaydar, Mehmet; Ofluoglu, Demet; Demircay, Emre

    2012-07-01

    Simultaneous and spontaneous bilateral quadriceps tendon rupture is an uncommon injury that is usually seen in association with multiple medical conditions and some medications. We report a case of simultaneous and spontaneous bilateral quadriceps tendon rupture that may be related to the long-term use of a statin. PMID:22561379

  17. Spontaneous bilateral adrenal hemorrhage following cholecystectomy

    PubMed Central

    Dahan, Meryl; Lim, Chetana; Salloum, Chady

    2016-01-01

    Postoperative bilateral adrenal hemorrhage is a rare but potentially life-threatening complication. This diagnosis is often missed because the symptoms and laboratory results are usually nonspecific. We report a case of bilateral adrenal hemorrhage associated with acute primary adrenal insufficiency following laparoscopic cholecystectomy. The knowledge of this uncommon complication following any abdominal surgery allows timey diagnosis and rapid treatment. PMID:27275469

  18. Bilateral microperc in a severe kyphoscoliosis.

    PubMed

    Dağgülli, Mansur; Penbegül, Necmettin; Dede, Onur; Utanğaç, Mehmet Mazhar

    2016-03-01

    Percutaneous nephrolithotomy is the standard modality for large renal calculi in normal and abnormal renal anatomic situations. This case report describes a 57-year-old male patient who presented with bilateral kidney stones and severe kyphoscoliosis. He had successfully been treated with a bilateral microperc technique. PMID:27011881

  19. Bilateral pulmonary sequestration: computed tomographic appearance

    SciTech Connect

    Wimbish, K.J.; Agha, F.P.; Brady, T.M.

    1983-04-01

    Intralobar pulmonary sequestration is one manifestation of the wide spectrum of congenital bronchopulmonary foregut malformations. Bilateral intralobar pulmonary sequestration is an exceedingly rare anomaly. Only two pathologically proven cases and one possible case have been reported. We report a case presenting as bilateral paraspinal masses, studied by computed tomograpy (CT) and angiography.

  20. Bilateral parotid swelling: a radiological review

    PubMed Central

    Gadodia, A; Bhalla, A S; Sharma, R; Thakar, A; Parshad, R

    2011-01-01

    Bilateral parotid swelling is not an uncommon occurrence and may pose a challenge for clinicians and radiologists. Numerous causes of bilateral parotid swellings have been identified. The purpose of this pictorial review is to display this wide array with a focus on multimodality approach. PMID:21960397

  1. Sarcoidosis Presenting as Bilateral Vocal Cord Paralysis due to Bilateral Vagal Nerve Involvement.

    PubMed

    Yamasue, Mari; Nureki, Shin-Ichi; Ushijima, Ryoichi; Mukai, Yutaka; Goto, Akihiko; Kadota, Jun-Ichi

    2016-01-01

    We herein report a rare case of sarcoidosis presenting as bilateral vocal cord paralysis due to bilateral vagal nerve involvement. A 72-year-old woman with uveitis of the left eye complained of hoarseness and aspiration due to bilateral vocal cord paralysis. An endobronchial needle aspiration biopsy specimen of the mediastinal lymph nodes showed non-caseating epithelioid cell granuloma. Total protein and cell concentrations in the cerebrospinal fluid were increased. We diagnosed her to have sarcoidosis with bilateral vagal nerve involvement. Corticosteroid therapy improved her symptoms of hoarseness and aspiration. Sarcoidosis should therefore be taken into consideration as a potential cause of bilateral vocal cord paralysis. PMID:27150886

  2. Bilateral Central Retinal Vein Occlusion as Presenting Feature of Chronic Myeloid Leukemia.

    PubMed

    Narang, Subina; Gupta, Panchmi; Sharma, Anuj; Sood, Sunandan; Palta, Anshu; Goyal, Shilpa

    2016-01-01

    Central retinal vein occlusion (CRVO) is a common pathology of the retinal vasculature. Patients with CRVO usually present with a drop in visual acuity. The condition bears no specific therapy; treatment is aimed at the management of potentially blinding complications, of which there are many. With majority of cases being unilateral, bilateral CRVO is usually associated with an underlying systemic illness such as a hyperviscosity syndrome. Here, we present a case of a patient, who presented with a bilateral drop in vision diagnosed as bilateral CRVO on ophthalmic evaluation. Systemic workup revealed the presence of an underlying undiagnosed chronic myeloid leukemia. An initial presentation to the ophthalmologist is a rare occurrence in leukemic patients. This case report highlights the role of the ophthalmologist in diagnosing a potentially life-threatening hematological illness. PMID:27555710

  3. Bilateral Central Retinal Vein Occlusion as Presenting Feature of Chronic Myeloid Leukemia

    PubMed Central

    Narang, Subina; Gupta, Panchmi; Sharma, Anuj; Sood, Sunandan; Palta, Anshu; Goyal, Shilpa

    2016-01-01

    Central retinal vein occlusion (CRVO) is a common pathology of the retinal vasculature. Patients with CRVO usually present with a drop in visual acuity. The condition bears no specific therapy; treatment is aimed at the management of potentially blinding complications, of which there are many. With majority of cases being unilateral, bilateral CRVO is usually associated with an underlying systemic illness such as a hyperviscosity syndrome. Here, we present a case of a patient, who presented with a bilateral drop in vision diagnosed as bilateral CRVO on ophthalmic evaluation. Systemic workup revealed the presence of an underlying undiagnosed chronic myeloid leukemia. An initial presentation to the ophthalmologist is a rare occurrence in leukemic patients. This case report highlights the role of the ophthalmologist in diagnosing a potentially life-threatening hematological illness.

  4. Megacystis, microcolon, intestinal hypoperistalsis syndrome and bilateral streak gonads

    PubMed Central

    Mantan, M.; Singhal, K. K.; Sethi, G. R.; Aggarwal, S. K.

    2011-01-01

    Megacystis, microcolon, intestinal hypoperistalsis is an uncommon condition presenting in neonatal age with features of intestinal obstruction and bladder evacuation abnormalities. We present here an infant girl with the diagnosis consistent with this entity. PMID:21886986

  5. Asplenia Syndrome in a Neonate: A Case Report

    PubMed Central

    Bhalla, Kapil; Yadav, Jaivinder; Mehra, Suchi

    2016-01-01

    Asplenia syndrome is a syndrome of right isomerism or attempted bilateral right sidedness. It includes congenital asplenia in association with complex congenital cyanotic heart disease and situs anomalies of the other thoracoabdominal organs. Herein we report a case of asplenia syndrome so as to highlight and refocus attention on the possibility of this rare syndrome that must be considered in a neonate presenting with congenital cyanotic heart disease.

  6. Asplenia Syndrome in a Neonate: A Case Report.

    PubMed

    Bhalla, Kapil; Singh, Jasbir; Yadav, Jaivinder; Mehra, Suchi

    2016-06-01

    Asplenia syndrome is a syndrome of right isomerism or attempted bilateral right sidedness. It includes congenital asplenia in association with complex congenital cyanotic heart disease and situs anomalies of the other thoracoabdominal organs. Herein we report a case of asplenia syndrome so as to highlight and refocus attention on the possibility of this rare syndrome that must be considered in a neonate presenting with congenital cyanotic heart disease. PMID:27504371

  7. Management of moyamoya syndrome in patients with Noonan syndrome.

    PubMed

    Gupta, Mihir; Choudhri, Omar A; Feroze, Abdullah H; Do, Huy M; Grant, Gerald A; Steinberg, Gary K

    2016-06-01

    A few isolated reports have described an association between Noonan syndrome and cerebrovascular abnormalities, including moyamoya syndrome. These reports have been limited to pediatric patients presenting with recurrent transient ischemic attacks (TIA) or headaches. Management has primarily been pharmacologic, with only one prior report of surgical revascularization to our knowledge. We report four cases of Noonan syndrome patients presenting with headaches and/or sensorimotor strokes in childhood that caused unilateral sensorimotor impairment. Cerebral angiography and MRI revealed bilateral moyamoya syndrome. All patients underwent successful bilateral extracranial-to-intracranial revascularization. The first patient was a 10-year-old girl who presented following a hemorrhagic stroke and recovered well after indirect bypass. The second patient was an adult with a history of childhood stroke whose symptoms progressed in adulthood. She underwent a direct bypass and improved, but continued to experience TIA at her 4 year follow-up. The third patient was a 7-year-old girl with headaches and a new onset TIA who failed pharmacological therapy and subsequently underwent bilateral indirect bypass. The fourth patient was a 24-year-old woman with worsening headaches and an occluded left middle cerebral artery from unilateral moyamoya syndrome. A left sided direct bypass was completed given delayed MRI perfusion with poor augmentation. To our knowledge these are the first reported surgical cases of combined Noonan and moyamoya syndrome. These cases highlight the need to recognize moyamoya syndrome in patients with Noonan syndrome. Early surgical revascularization should be pursued in order to prevent symptom progression. PMID:26778511

  8. Heterochronic bilateral ectopic pregnancy after ovulation induction*

    PubMed Central

    Zhu, Bo; Xu, Gu-feng; Liu, Yi-feng; Qu, Fan; Yao, Wei-miao; Zhu, Yi-min; Gao, Hui-juan; Zhang, Dan

    2014-01-01

    Ectopic pregnancy is identified with the widely-applied assisted reproductive technology (ART). Bilateral ectopic pregnancy is a rare form of ectopic pregnancy which is difficult to be diagnosed at the pre-operation stage. In this paper, we presented an unusual case of heterochronic bilateral ectopic pregnancy after stimulated intrauterine insemination (IUI), where there has been a delay of 22 d between the diagnoses of the two ectopic pregnancies. Literature was reviewed on the occurrence of bilateral ectopic pregnancy during the past four years in the MEDLINE database. We found 16 cases of bilateral ectopic pregnancy reported since 2008, and analyzed the characteristics of those cases of bilateral ectopic pregnancy. We emphasize that ovulation induction and other ARTs may increase the risk of bilateral ectopic pregnancy. Because of the difficulty in identification of bilateral ectopic pregnancy by ultrasonography, the clinician should be aware that the treatment of one ectopic pregnancy does not preclude the occurrence of a second ectopic pregnancy in the same patient and should pay attention to the intra-operation inspection of both side fallopian tubes in any ectopic pregnancy case. PMID:25091994

  9. Bilateral pneumothorax after orthognatic surgery

    PubMed Central

    Bertossi, Dario; Malchiodi, Luciano; Turra, Matteo; Bondi, Vincenzo; Albanese, Massimo; Lucchese, Alessandra; Carinci, Francesco; Nocini, Pierfrancesco

    2012-01-01

    Among complications in orthognathic surgery, the insurgence of pneumothorax is very rare. Pneumothorax is the presence of air or gas in the pleural cavity and it is rare complications in the postoperative oral and maxillofacial surgery patient. The clinical results are dependent on the degree of collapse of the lung on the affected side. Pneumothorax can impair oxygenation and/or ventilation. If the pneumothorax is significant, it can cause a shift of the mediastinum and compromise haemodynamic stability. While 10% of pneumothoraces are asymptomatic, patients often complain of acute chest pain and difficulty breathing. There is a reduction in vital capacity, tachycardia, tachypnoea and a decrease in partial pressure of oxygen with an inability to maintain oxygen saturations. We observed this unusual surgical consequence in a 28-year-old female with negative clinical history and instrumental evaluation after Le Fort I osteotomy and bilateral sagittal split osteotomy (BSSO). No further consequences, no neurological sequelae, no infections and no other osteotomies sequelae were seen. Sudden post-surgical dispnea associated to sub-cutaneous emphysema of the neck and of the thorax must be adequately observed with the aim of monitoring further severe sequelae. The anaesthetic management of the emergency difficult airway in any post-surgical orthognatic treatment can be extremely difficult requiring a multi-disciplinary approach. PMID:23814593

  10. Retroperitoneal laparoscopic bilateral lumbar sympathectomy.

    PubMed

    Segers, B; Himpens, J; Barroy, J P

    2007-06-01

    The first retroperitoneal lumbar sympathectomy was performed in 1924 by Julio Diez. The classic procedure for sympathectomy is open surgery. We report a unilateral laparoscopic retroperitoneal approach to perform bilateral lumbar sympathectomy. This approach was performed for a 43-year-old man with distal arterial occlusive disease and no indication for direct revascularization. His predominant symptoms were intermittent claudication at 100 metres and cold legs. The patient was placed in a left lateral decubitus position. The optical system was placed first in an intra-abdominal position to check that the trocars were well positioned in the retroperitoneal space. The dissection of retroperitoneum was performed by CO2 insufflation. The inferior vena cava was reclined and the right sympathetic chain was individualized. Two ganglia (L3-L4) were removed by bipolar electro-coagulation. The aorta was isolated on a vessel loop and careful anterior traction allowed a retro-aortic pre-vertebral approach between the lumbar vessels. The left sympathetic chain was dissected. Two ganglia (L3-L4) were removed by bipolar electro-coagulation. PMID:17685269

  11. Bilateral symmetry across Aphrodite Terra

    NASA Technical Reports Server (NTRS)

    Crumpler, L. S.; Head, J. W.; Campbell, D. B.

    1987-01-01

    There are three main highland areas on Venus: Beta Regio, Ishtar Terra and Aphrodite Terra. The latter is least known and the least mapped, yet existing analyses of Aphrodite Terra based on available Pioneer-Venus orbiter data suggest that it may be the site of extensive rifting. Some of the highest resolution (30 km) PV data (SAR) included most of the western half of Aphrodite Terra. Recent analysis of the SAR data together with Arecibo range-doppler topographic profiling (10 X 100 km horizontal and 10 m vertical resolution) across parts of Aphrodite, further characterized the nature of possible tectonic processes in the equatorial highlands. The existence of distinct topographic and radar morphologic linear discontinuities across the nearly east-west strike of Aphrodite Terra is indicated. Another prominent set of linear features is distinctly parallel to and orthogonal to the ground tracks of the PV spacecraft and are not included because of the possibility that they are artifacts. Study of the northwest trending cross-strike discontinuities (CSD's) and the nature of topographic and morphologic features along their strike suggest the presence of bilateral topographic and morphologic symmetry about the long axis of Aphrodite Terra.

  12. Bilateral extensor digitorum brevis manus.

    PubMed

    Froelich, John M; Bidgoli-Moghaddam, Mahsa; Moran, Steven L

    2012-09-01

    Dorsal wrist pain and swelling is commonly attributed to a dorsal wrist ganglion. However, based on the authors' experience, a cautious surgeon should keep the uncommonly symptomatic diagnosis of an extensor digitorum brevis manus in their differential despite classic ganglion presentation and suggestive advanced imaging. This article describes a case of a young patient who presented with bilateral symptomatic extensor digitorum brevis manus anomalies that required surgical intervention. An extensor digitorum brevis manus is present in 3% of the population in a classic anatomy study from Japan and is most commonly symptomatic with heavy activity and extremes of wrist extension. Anatomically, the extensor digitorum brevis manus is located in the fourth wrist compartment and most commonly inserts on the index finger extensor mechanism. Examination often reveals a spindle-shaped mass that is palpable distal to the extensor mechanism and moves with extensor tendon motion. Magnetic resonance imaging shows a typical dorsal mass distal to the common extensors with a similar signal as muscle with all image sequencing. Treatment includes activity alterations to relieve symptoms or surgical excision of the muscle belly for refractory cases with care taken to preserve the index extensor mechanism. PMID:22955414

  13. Bilateral sudden hearing loss following habitual abortion: a case report and review of literature.

    PubMed

    Yin, Tuanfang; Huang, Fengying; Ren, Jihao; Liu, Wei; Chen, Xing; Li, Lihua; Xie, Dinghua; Lu, Yongde

    2013-01-01

    Sudden sensorineural hearing loss (SSNHL) is usually unilateral and can be associated with tinnitus and vertigo. The most common causes of this disease are known to be the vascular and viral agents, but immune disorders are involved in the development of sudden deafness. The antiphospholipid syndrome (APS) is an acquired autoimmune system disorder, which is defined as the presence of antiphospholipid antibodies (APA) in the patient's blood, then cause venous and/or arterial thrombosis in various organs of the body, for example, thrombosis can occur in the placenta and/or the inner ear. As a result, it can cause abortion and/or sudden deafness. Bilateral SSNHL following habitual abortion is a rare clinical event. Here, we report a case of 32-year-old woman who presented with bilateral sudden hearing loss following recurrent pregnancy loss (RPL) as the first manifestation of primary antiphospholipid syndrome. Combine the literature, the diagnosis, clinical implication and treatment are discussed. PMID:24040484

  14. [Bilateral segmental neurofibromatosis simulating epidermal nevus].

    PubMed

    Gambichler, T; Küster, W; Wolter, M; Rapp, S; Altmeyer, P; Hoffmann, K

    2000-11-01

    Neurofibromatosis is a neuroectodermal systemic disease. A rare variant of this condition is bilateral segmental neurofibromatosis. A 29-year-old man presented with bilateral papillomatous plaques in the lumbar dermatomes. Clinically, the lesions were very similar to an epidermal nevus but histologic examination revealed superficial neurofibromas. Family history, ophthalmologic and neurologic investigations were unremarkable. The unusual morphologic presentation of bilateral segmental neurofibromas in this case points to the wide clinical spectrum of the disease and the significance of histologic examination in systematic nevoid lesions. PMID:11116852

  15. Bilateral adrenal haemorrhage leading to adrenal crisis

    PubMed Central

    McGowan-Smyth, Sam

    2014-01-01

    A 77-year-old man presented with an acute worsening of chronic back pain. CT showed dense bilateral adrenal glands suggestive of adrenal haemorrhage which was confirmed by MRI. Despite appropriate glucocorticoid replacement for adrenal insufficiency, 7 days after admission this patient suffered an adrenal crisis. Owing to the timely diagnosis, appropriate treatment was given and the patient survived. Large bilateral adrenal haemorrhage however, can lead to cardiovascular collapse and death if not appropriately diagnosed and managed promptly. Despite its rarity, bilateral adrenal haemorrhage should always be considered as a differential for back pain in the setting of an acute illness due to its potentially fatal consequences. PMID:24969071

  16. Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction

    SciTech Connect

    Doody, O.; Adam, W. R.; Foley, P. T.; Lyon, S. M.

    2009-03-15

    Fibromuscular dysplasia (FMD) describes a group of conditions which cause nonatheromatous arterial stenoses, most commonly of the renal and carotid arteries, typically in young women. We report a rare case of bilateral segmental renal infarction secondary to FMD in a young male patient. His initial presentation with loin pain and pyrexia resulted in a delay in the definitive diagnosis of FMD. He was successfully treated with bilateral balloon angioplasty. The delayed diagnosis in this patient until the condition had progressed to bilateral renal infarcts highlights the need for prompt investigation and diagnosis of suspected cases of FMD.

  17. Bilateral perinephric fluid accumulation: an unusual manifestation of pulmonary hypertension--a case report.

    PubMed

    Pentimone, F; Meola, M; Del Corso, L

    1993-06-01

    This is the case report of a forty-seven-year-old man with an ostium secundum atrial septal defect and a very high grade of pulmonary hypertension, associated with a large bilateral perinephric fluid accumulation. The fluid accumulation was remarkably reduced after eleven phlebotomies over a twelve-month period. A pathogenetic relation with the Eisenmenger's syndrome is discussed. No previous report of this association has been found in a survey of the literature. PMID:8503517

  18. Bilateral Second Carpal Row Duplication Associated with Multiple Epiphyseal Dysplasia.

    PubMed

    Cladiere-Nassif, Victoire; Delaroche, Caroline; Pottier, Edwige; Feron, Jean-Marc

    2015-11-01

    We report a case of a 75-year-old woman presenting a hitherto undescribed condition of bilateral second carpal row duplication. She was diagnosed in childhood with both Marfan and Ehlers-Danlos syndromes, with no clear evidence and no further medical follow-up. She presented throughout her life with various articular symptoms, which appeared to be compatible with a diagnosis of multiple epiphyseal dysplasia, and underwent several surgical procedures on her knees and hips. Most recently, she was reporting pain at the base of the fifth metacarpal bone of the left hand. X-ray images and computed tomography (CT) were obtained for exploration and showed a total second row duplication in both carpi, with a total number of 18 carpal bones in each wrist. PMID:26649258

  19. Impaired threat prioritisation after selective bilateral amygdala lesions

    PubMed Central

    Bach, Dominik R.; Hurlemann, Rene; Dolan, Raymond J.

    2015-01-01

    The amygdala is proposed to process threat-related information in non-human animals. In humans, empirical evidence from lesion studies has provided the strongest evidence for a role in emotional face recognition and social judgement. Here we use a face-in-the-crowd (FITC) task which in healthy control individuals reveals prioritised threat processing, evident in faster serial search for angry compared to happy target faces. We investigate AM and BG, two individuals with bilateral amygdala lesions due to Urbach–Wiethe syndrome, and 16 control individuals. In lesion patients we show a reversal of a threat detection advantage indicating a profound impairment in prioritising threat information. This is the first direct demonstration that human amygdala lesions impair prioritisation of threatening faces, providing evidence that this structure has a causal role in responding to imminent danger. PMID:25282058

  20. Bilateral nephrocalcinosis and amelogenesis imperfecta: A case report

    PubMed Central

    Patel, Alok; Jagtap, Chetana; Bhat, Chetan; Shah, Rohan

    2015-01-01

    Amelogenesis imperfecta (AI) is a group of hereditary disorders that affect the quality and/or quantity of dental enamel. This paper describes the clinicopathological features of a patient who was born of nonconsanguineous parents and who presented with oral alterations, including yellow and misshapen teeth, intrapulpal calcifications, delayed tooth eruption, and gum enlargement. Scanning electron microscopy of the teeth revealed hypoplastic enamel, and a renal ultrasound detected bilateral nephrocalcinosis, leading to a diagnosis of AI and nephrocalcinosis syndrome. Since nephrocalcinosis is often asymptomatic and can be associated with impaired renal function, dentists who see children with a generalized and thin hypoplastic AI should consider a renal ultrasound scan and referral to a Nephrologist. Children with nephrocalcinosis should also be considered for a dental check. PMID:26097369

  1. Involuntary masturbation and hemiballismus after bilateral anterior cerebral artery infarction.

    PubMed

    Bejot, Yannick; Caillier, Marie; Osseby, Guy-Victor; Didi, Roy; Ben Salem, Douraied; Moreau, Thibault; Giroud, Maurice

    2008-02-01

    Ischemia of the areas supplied by the anterior cerebral artery is relatively uncommon. In addition, combined hemiballismus and masturbation have rarely been reported in patients with cerebrovascular disease. We describe herein a 62-year-old right-handed man simultaneously exhibiting right side hemiballismus and involuntary masturbation with the left hand after bilateral infarction of the anterior cerebral artery territory. Right side hemiballismus was related to the disruption of afferent fibers from the left frontal lobe to the left subthalamic nucleus. Involuntary masturbation using the left hand was exclusively linked to a callosal type of alien hand syndrome secondary to infarction of the right side of the anterior corpus callosum. After 2 weeks, these abnormal behaviours were completely extinguished. This report stresses the wide diversity of clinical manifestations observed after infarction of the anterior cerebral artery territory. PMID:17961914

  2. [Mandibular advancement: bilateral sagittal split versus -distraction osteogenesis].

    PubMed

    Akkerman, V; Ho, J P; Baas, E M; de Lange, J de

    2015-11-01

    In the 1990s intra-oral distraction osteogenesis (DO) became available as an alternative for bilateral sagittal splitosteotomy (BSSO) for advancement of the mandible. It was thought that DO would lead to more stability in the results and fewer neurosensory disturbances of the inferior alveolar nerve. However, there was no scientific evidence for this assumption. This article describes a number of recently published, prospective studies that demonstrate that BSSO is not inferior to DO with respect to stability and neurosensory disturbances of the inferior alveolar nerve. They also demonstrate that BSSO leads to less pain in patients and to lower total costs. It can be concluded that BSSO should be considered the standard therapy for mandibular advancement up to 10 mm in non-syndromal patients. PMID:26569001

  3. Bilateral endogenous endophthalmitis in disseminated NTM infection: a case report.

    PubMed

    Sinawat, Supat; Yospaiboon, Yosanan; Sinawat, Suthasinee

    2011-05-01

    A 47-year-old Thai female with underlying Sweet's syndrome and disseminated non-tuberculous mycobacterial infection presented with a history of blurred vision on both eyes after being lost to follow-up for eight months. The visual acuity was hand motion on the right eye and counting finger on the left eye. There was moderate inflammation in the anterior chamber and vitreous cavity. Multiple foci of round chorioretinitis were found throughout the fundus on both eyes. The patient denied intravitreous tapping and antibiotic injection. Nevertheless, specimens from several sites were collected for culture and sensitivity test. The result of the culture and sensitivity test revealed rapidly growing mycobacteria on specimens taken from the right inguinal lymph node. The presented case may demonstrate the rare event of bilateral endogenous endophthalmitis with chorioretinitis arising from non-tuberculous mycobacterial infection. The pattern of chorioretinitis demonstrated by the presented report may provide useful clinical information for this rare condition. PMID:21675456

  4. Bilateral ovarian hemangiomas associated with diffuse hemangioendotheliomatosis: a case report.

    PubMed

    Miyauchi, J; Mukai, M; Yamazaki, K; Kiso, I; Higashi, S; Hori, S

    1987-08-01

    A patient who had disseminated vascular tumors involving the bilateral ovaries, bilateral lungs and pleura, pericardium, and mediastinum is reported. The tumors were histologically of the capillary, and partly the cavernous, type of hemangioma. However, endothelial cell growth was prominent in some areas, especially in the lung, and the histology of the lung tumor resembled epithelioid hemangioendothelioma or intravascular bronchiolo-alveolar tumor (IVBAT). In the endocardium of the right atrium, an endothelial tumorous projection was observed, and there were tiny foci of tumor cell nests in the abdominal venous wall. Small lymphangiomas were also found in the subcapsular region of the spleen. These findings suggest that there had been an abnormal proliferation of systemic endothelial cells and that tumors of endothelial cell origin with diverse histological patterns developed with this condition as a background. The autopsy finding of fibrin thrombi in multiple organs as well as laboratory data including thrombocytopenia suggest that this case belongs to the "Kasabach-Merritt syndrome." PMID:3673576

  5. Loin pain hematuria syndrome

    PubMed Central

    Zubair, Adeel S.; Salameh, Hassan; Erickson, Stephen B.; Prieto, Mikel

    2016-01-01

    Loin pain hematuria syndrome (LPHS), first described in 1967, is a rare pain syndrome, which is not well understood. The syndrome is characterized by severe intermittent or persistent flank pain, either unilateral or bilateral, associated with gross or microscopic hematuria. LPHS is a diagnosis of exclusion as there still is not a consensus of validated diagnostic criteria, though several criteria have been proposed. The wide differential diagnosis would suggest a meticulous yet specific diagnostic work-up depending on the individual clinical features and natural history. Several mechanisms regarding the pathophysiology of LPHS have been proposed but without pinpointing the actual causative etiology, the treatment remains symptomatic. Treatment modalities for LPHS are diverse including simple analgesia, opioid analgesic and kidney autotransplantation. This review article summarizes the current understanding regarding the pathophysiology of LPHS along with the steps required for proper diagnosis and a discussion of the different therapeutic approaches for LPHS. PMID:26798473

  6. Loin pain hematuria syndrome.

    PubMed

    Zubair, Adeel S; Salameh, Hassan; Erickson, Stephen B; Prieto, Mikel

    2016-02-01

    Loin pain hematuria syndrome (LPHS), first described in 1967, is a rare pain syndrome, which is not well understood. The syndrome is characterized by severe intermittent or persistent flank pain, either unilateral or bilateral, associated with gross or microscopic hematuria. LPHS is a diagnosis of exclusion as there still is not a consensus of validated diagnostic criteria, though several criteria have been proposed. The wide differential diagnosis would suggest a meticulous yet specific diagnostic work-up depending on the individual clinical features and natural history. Several mechanisms regarding the pathophysiology of LPHS have been proposed but without pinpointing the actual causative etiology, the treatment remains symptomatic. Treatment modalities for LPHS are diverse including simple analgesia, opioid analgesic and kidney autotransplantation. This review article summarizes the current understanding regarding the pathophysiology of LPHS along with the steps required for proper diagnosis and a discussion of the different therapeutic approaches for LPHS. PMID:26798473

  7. Localized neurofibromas in the bilateral orbits.

    PubMed

    Takeuchi, Satoru; Wada, Kojiro; Nagatani, Kimihiro; Nawashiro, Hiroshi

    2013-10-01

    Localized neurofibromas are rare in the orbit and, unlike the more common plexiform neurofibromas, are not typically associated with von Recklinghausen neurofibromatosis. We present a rare case of localized neurofibromas in the bilateral orbits. PMID:24426488

  8. Bilateral persistent pupillary membranes associated with cataract

    PubMed Central

    Ahmad, Syed Shoeb; Binson, Caroline; Lung, Chong Ka; Ghani, Shuaibah Abdul

    2011-01-01

    Summary Exuberant persistent pupillary membranes (PPM) are rare in adult eyes. We report the case of a 53-year-old man diagnosed with bilateral, profuse, persistent pupillary membranes and unilateral cataract. PMID:23362401

  9. Bilateral Coordination of Children who are Blind.

    PubMed

    Rutkowska, Izabela; Lieberman, Lauren J; Bednarczuk, Grzegorz; Molik, Bartosz; Kazimierska-Kowalewska, Kalina; Marszałek, Jolanta; Gómez-Ruano, Miguel-Ángel

    2016-04-01

    The purpose of this study was to evaluate the bilateral coordination in children and adolescents with visual impairments aged 7 to 18 years in comparison to their sighted peers. An additional objective was to identify the influence of sex and age on bilateral coordination. Seventy-five individuals with congenital severe visual impairment (40 girls and 35 boys) comprised the visually impaired group. The Sighted group comprised 139 youth without visual impairment. Subtest 4 of the Bruininks-Oseretsky Test of Motor Proficiency was administered to test bilateral coordination. To analyze the effect of the independent variables in the results obtained in the Subtest 4, four linear regression models were applied according to group and sex. The results indicated that severe visual impairment and lack of visual sensation had a negative effect on the development of participants' bilateral coordination, which however did not depend on sex or age. PMID:27166337

  10. BILATERAL ANTERIOR GLENOHUMERAL DISLOCATION: CLINICAL CASE

    PubMed Central

    Silva, Luís Pires; Sousa, Cristina Varino; Rodrigues, Elisa; Alpoim, Bruno; Leal, Miguel

    2015-01-01

    Bilateral anterior glenohumeral dislocation is a rare occurrence. We present a case of bilateral anterior glenohumeral dislocation caused by a fall. The interest in publishing this case is that this is a clinical rarity with few cases reported in the literature. An 89-year-old female patient was brought to the emergency department after a fall, complaining of intense pain in both shoulders and inability to move them. Objective examination showed clinical signs giving the suspicion of bilateral anterior glenohumeral dislocation, which was confirmed by x-ray imaging. Both dislocations were successfully reduced in the emergency department using the modified Milch technique. When a synchronous and symmetrical force has acted on both shoulders and these are painful with significant functional limitation, the suspicion of bilateral glenohumeral dislocation is a differential diagnosis to be considered, even though it is rare. PMID:27047826

  11. Bilateral total deafness due to pontine haematoma.

    PubMed Central

    Egan, C A; Davies, L; Halmagyi, G M

    1996-01-01

    A 64 year old woman with a predominantly midline pontine tegmental haemorrhage presented with bilateral total deafness. One week later reasonable pure-tone thresholds appeared but she still had total bilateral loss of speech discrimination. At that time contralateral acoustic reflexes were bilaterally absent, whereas ipsilateral acoustic reflexes and waves IV and V of the brainstem auditory evoked potential were bilaterally preserved. It is proposed that this patient's hearing deficit was due to inactivation of the ventral acoustic striae decussating in the trapezoid body. This case supports the contention that in humans the ventral pontine acoustic decussation carries most of the neural signals required for hearing and perhaps all the neural signals required for speech perception. Images PMID:8971114

  12. Bilateral obstruction of bilaterally duplicated collecting systems requiring upper and lower moiety drainage.

    PubMed

    Lynch, J O; Cox, A; Rawal, B; Shelmerdine, S; Vasdev, N; Patel, A

    2016-04-01

    A 60-year-old woman with a history of breast cancer presented with bilateral obstruction of bilaterally duplicated renal collecting systems secondary to extrinsic compression from metastatic pelvic lymphadenopathy. Bilateral JJ ureteric stents were inserted, resulting in some improvement of renal function but a failure to normalise completely. Repeat computed tomography demonstrated bilateral duplex collecting systems with persisting obstruction of the undrained moieties. Selective puncture was performed to decompress the obstructed renal moieties for bilateral nephrostomy catheter insertion. This allowed renal function to improve sufficiently for the patient to be discharged and commence chemotherapy. This is the first reported case of bilaterally obstructed partially duplicated collecting systems and it illustrates the importance of recognising anatomical variants to tailor treatment appropriately. It also highlights the important relationship between urology and interventional radiology in the management of such complex patients. PMID:26985816

  13. Bilateral synchronous plasmacytoma of the testis.

    PubMed

    Narayanan, Geetha; Joseph, Rona; Soman, Lali V

    2016-04-01

    Extramedullary plasmacytoma (EMP) is usually seen in the head and neck regions and in the upper respiratory, gastrointestinal, and central nervous systems. Testis is a rare site for EMP, and bilateral synchronous testicular plasmacytoma occurring as an isolated event at initial presentation has been reported only once previously. We present herein the second such report in a 70-year-old man who underwent bilateral orchidectomy. PMID:27034568

  14. Bilateral synchronous plasmacytoma of the testis

    PubMed Central

    Joseph, Rona; Soman, Lali V.

    2016-01-01

    Extramedullary plasmacytoma (EMP) is usually seen in the head and neck regions and in the upper respiratory, gastrointestinal, and central nervous systems. Testis is a rare site for EMP, and bilateral synchronous testicular plasmacytoma occurring as an isolated event at initial presentation has been reported only once previously. We present herein the second such report in a 70-year-old man who underwent bilateral orchidectomy. PMID:27034568

  15. Bilateral multicystic kidneys--an unusual case.

    PubMed

    Agrawal, Lila; Millard, Marie-Louise; Fairhurst, Joanna; Gilbert, Rodney D

    2002-11-01

    Multicystic dysplasia of the kidneys is a condition whose prognosis is good as it usually presents unilaterally. Bilateral cases are usually fatal in utero. We report a case of bilateral multicystic dysplasia of the kidneys where the lower moiety of the right kidney was spared cystic change. The patient had normal renal function and, following conservative management, remains alive and well 6 months later. PMID:12432443

  16. Bilateral giant juvenile fibroadenomas of the breasts-a rare indication for bilateral skin reducing mastectomy.

    PubMed

    Laitano, Francisco Felipe; Neto, Francisco Laitano; Zerwes, Felipe Pereira

    2016-08-01

    Fibroadenoma is the most common benign tumor of the breast but giant juvenile fibroadenoma represent only 0.5% of all fibroadenomas and when bilateral are much more rare. We describe the case of a 25 years old girl that presented with bilateral giant juvenile fibroadenomas and was treated by bilateral skin reducing mastectomy using the inferior dermal flap, implant, and free nipple graft. PMID:27563567

  17. Bilateral giant juvenile fibroadenomas of the breasts—a rare indication for bilateral skin reducing mastectomy

    PubMed Central

    Neto, Francisco Laitano; Zerwes, Felipe Pereira

    2016-01-01

    Fibroadenoma is the most common benign tumor of the breast but giant juvenile fibroadenoma represent only 0.5% of all fibroadenomas and when bilateral are much more rare. We describe the case of a 25 years old girl that presented with bilateral giant juvenile fibroadenomas and was treated by bilateral skin reducing mastectomy using the inferior dermal flap, implant, and free nipple graft. PMID:27563567

  18. Refractory leg ulcers associated with Klinefelter syndrome.

    PubMed

    Yabuno, Yuto; Tosa, Mamiko; Iwakiri, Itaru; Nomoto, Shunichi; Kaneko, Mayuko; Kuwahara, Kousuke; Hyakusoku, Hiko; Murakami, Masahiro

    2015-01-01

    We present a man with refractory leg ulcers, bilateral varicosis of the lower extremities, and Buerger disease. Autoimmune work-up was negative. However, chromosome analysis showed Klinefelter syndrome (48 XXY). Ulcerative lesions of the lower extremities are a complication of Klinefelter syndrome. To date, the pathogenesis of ulcers in Klinefelter syndrome has not been clarified, but several factors, such as abnormalities of fibrinolysis and prothrombotic states, might be involved. Our present case emphasizes the importance of considering Klinefelter syndrome in the differential diagnosis of a male patient with nonhealing ulcers of the lower extremities. PMID:25797879

  19. Malabsorption Syndromes

    MedlinePlus

    ... syndrome, your small intestine cannot absorb nutrients from foods. Causes of malabsorption syndromes include Celiac disease Lactose intolerance Short bowel syndrome. This happens after surgery to ...

  20. [Simultaneous bilateral spontaneous pneumothorax; report of a case].

    PubMed

    Okubo, Tetsuyuki; Kawada, Masaya; Suzuki, Yoshinori; Kawarada, Yo; Kitashiro, Shuji; Okushiba, Shun-ichi

    2014-04-01

    Simultaneous bilateral spontaneous pneumothorax (SBSP) occurred in a 16-year-old male. He presented with severe dyspnea. Chest X-ray showed bilateral pneumothorax. Computed tomography revealed bilateral apical bullas. Chest tubes were promptly inserted bilaterally. The patient underwent single-stage bilateral video-assisted thoracoscopic surgery( VATS). Bilateral bullectomy was performed sequentially. Saline poured in to test for air leakage did not escape to the opposite pleural cavity, suggesting no pleuro-pleural communication. Post-operative course was uneventful. A bilateral single-stage VATS by a conventional lateral approach with intra-operative patient repositioning is a safe, feasible and familiar technique for treatment of SBSP. PMID:24917168

  1. Bilateral ACL Reconstructions with Hamstring Autografts.

    PubMed

    Panigrahi, Ranajit; Mahapatra, Amita Kumari; Priyadarshi, Ashok; Palo, Nishit; Biswal, Manas R

    2016-07-01

    Bilateral anterior cruciate ligament (ACL) injuries are rare with incidence between 2 and 4%, and presently no definitive guidelines for proper management exist. Ideal treatment protocol remains controversial between a single-stage and two-stage bilateral ACL reconstruction. The purpose of this study is to evaluate the outcome of single-stage bilateral ACL reconstruction with hamstring tendon autografts in bilateral ACL injuries. A prospective study was undertaken including a total of 14 consecutive patients with bilateral ACL deficient knee who underwent single-stage bilateral ACL reconstruction with hamstring tendon autograft with a mean follow-up duration of 28 months (24-38 months). Functional outcomes were evaluated by range of movements, International Knee Documentation Committee (IKDC), Lysholm and Tegner activity score, and stability tests. The mean age was 30 years (range 18-42 years). Average duration of rehabilitation was 8 weeks. Time to return to full-time work and full sports was 5.6 weeks and 6.2 months, respectively. Clinical examination demonstrated full range of motion; a total of 12 patients (86%) had a negative Lachman test and 13 patients (93%) had a negative pivot shift at the final follow-ups. The mean IKDC evaluation score was 89 points, the mean Tegner activity score was 7 points, and the mean Lysholm knee score was 91 points. A total of 12 patients (86%) returned to their preinjury level of activity and an overall greater than 90% satisfaction rate was achieved. Single-stage bilateral ACL reconstruction using hamstring autografts is clinically safe, effective, and cost-effective with better patient compliance and with comparable functional outcome as opposed to two-stage ACL reconstructions. PMID:26408992

  2. Renal dysfunction due to hydronephrosis by SAPHO syndrome: a case report

    PubMed Central

    Kakoki, Katsura; Miyata, Yasuyoshi; Enokizono, Mikako; Uetani, Masataka; Sakai, Hideki

    2015-01-01

    Key Clinical Message Synovitis, Acne, Pustulosis, Hyperostosis, Osteitis (SAPHO) syndrome shows varied pathological symptoms. This is the first report of hydronephrosis due to the mechanical compression of bilateral ureters as a result of SAPHO syndrome. From our experience, MRI is the most useful imaging examination to check the upper urinary tract in SAPHO syndrome. PMID:26331013

  3. Nelson syndrome: definition and management.

    PubMed

    Barber, T M; Adams, E; Wass, J A H

    2014-01-01

    Nelson syndrome is an important complication of treatment with total bilateral adrenalectomy (TBA) for patients with refractory Cushing's disease. Although early cases of Nelson syndrome often presented with the clinical features of large sellar masses, the modern face of Nelson syndrome has changed primarily due to earlier detection (with highly resolved magnetic resonance imaging (MRI) and sensitive ACTH assays) and greater awareness of the condition, resulting in reduced morbidity and mortality. Although lack of administration of neoadjuvant pituitary radiotherapy post-TBA surgery may predict future development of Nelson syndrome, other predictive factors remain controversial. Therefore, Nelson syndrome should be screened for closely and long-term in all patients with a history of Cushing's disease and TBA. The diagnosis of Nelson syndrome remains controversial, and the pathogenesis of this condition is incompletely understood. Current hypotheses include the "released negative feedback" mechansism (residual pituitary corticotropinoma cells are "released" from the negative feedback effects of cortisol following TBA), and the "aggressive corticotropinoma" mechanism (Nelson syndrome is most likely to develop in those patients with refractory treatments - including TBA - for an underlying aggressive corticotropinoma). Effective management of Nelson syndrome with pituitary surgery and radiotherapy is often a challenge. Other therapies (such as Gamma Knife surgery and temozolomide) play an important role and merit further research into their efficacy and placement in the management pathway of Nelson syndrome. PMID:25248597

  4. Bilateral ovarian cystic teratomata mimicking bilateral pure ovarian hemangiomata: case report.

    PubMed

    Feuerstein, I M; Aronson, B L; McCarthy, E F

    1984-01-01

    This report describes a case of bilateral, benign cystic ovarian teratomata which were composed predominantly of cavernous hemangiomatous elements. The right-sided lesion, in particular, mimicked a pure ovarian hemangioma. The clinical presentation, bilaterality of the lesions, the sizes of the hemangiomata, and the subsequent hemangioma of the leg are all of interest in this very rare lesion. PMID:6511164

  5. Bilateral Laparoscopic Partial Nephrectomies: A Case Report

    PubMed Central

    Panuganti, Sravan; Kavoussi, Louis Raphael

    2015-01-01

    Abstract Although laparoscopy is a recognized operative approach to the management of renal masses, there is currently no standardized approach to manage bilateral synchronous renal masses. We present a case of synchronous bilateral renal masses, identified during work-up for flank pain, and managed simultaneously with laparoscopic partial nephrectomies. The patient is a 42-year-old Caucasian male found to have bilateral renal masses during evaluation for left flank pain. Cross-sectional imaging studies showed a 7.0 × 7.3 × 5.2 cm anterior, mid-to-lower pole mass on the left kidney and a 1.5 × 1.9 × 1.6 cm medial lower pole mass on the right kidney. He underwent bilateral laparoscopic partial nephrectomy at the same setting, with an uncomplicated postoperative course. Pathology report revealed clear cell renal-cell carcinoma (ccRCC) on both sides. He had normal renal function and no evidence of recurrence in the first 6 months of follow-up. This case demonstrates the possibility and safety of performing bilateral laparoscopic partial nephrectomies in one operative session. Our review of the literature supports the role of genetic counseling and the need for long-term surveillance in young patients having RCC.

  6. Fahr's Syndrome Associated with Multiple Intracranial Aneurysms: A Case Report.

    PubMed

    Eroglu, Umit; Kahilogullari, Gökmen; Demirel, Altan; Arat, Anıl; Unlu, Agahan

    2016-01-01

    Fahr's Syndrome is characterized by the presence of intracerebral, bilateral and symmetrical calcifications located in bilateral basal ganglia, thalamus, and cerebellum. The etiology is not exactly known. The authors reported a very rare case who had Fahr's Syndrome and intracerebral aneurysms simultaneously. The patient was female and presented with headache. Her examinations revealed aneurysms on the middle cerebral artery, internal carotid artery and ophthalmic artery. That is the first case reported in the literature having multiple intracranial aneurysms and Fahr's Syndrome together. PMID:27400114

  7. Isaac's Syndrome

    MedlinePlus

    ... syndrome (also known as neuromyotonia, Isaacs-Mertens syndrome, continuous muscle fiber activity syndrome, and quantal squander syndrome) is a rare neuromuscular disorder caused by hyperexcitability and continuous firing of ... which include progressive muscle stiffness, continuously contracting ...

  8. Sturge-Weber syndrome - A case report.

    PubMed

    Shaikh, Shahid M; Goswami, Mousumi; Singh, Sanjay; Singh, Darrel

    2015-01-01

    Sturge-Weber syndrome (SWS), also called as encephalotrigeminal angiomatosis is an uncommon congenital neurological disorder & frequent among the neurocutaneous syndromes specifically with vascular predominance. This disorder is characterized by facial capillary malformation & other neurological condition. The oral manifestations are gingival hemangiomatosis restricting to either side in upper and lower jaw, sometimes bilateral. We report a case of SWS with oral, ocular and neurological features. PMID:25853049

  9. Filippi Syndrome: Report of a Rare Case

    PubMed Central

    Goyal, Lata; Bhakhri, Bhanu Kiran; Chug, Ashi

    2015-01-01

    Filippi syndrome is an autosomal recessive condition characterized by syndactyly of fingers and toes, microcephaly, growth retardation and abnormal facies. We are describing a boy who presented with syndactyly, mental retardation, microcephaly, depressed nasal bridge and growth retardation. In addition he had some dental abnormalities like missing bilateral lateral incisors and delayed eruption of teeth. We concluded it to be Filippi syndrome by studying pathognomic clinical features and reviewed the literature. This is the second case report from India. PMID:26813917

  10. Bilateral self-inflicted infectious dacryoadenitis.

    PubMed

    Latasiewicz, Marta; Chang-Sotomayor, Meilin; Alonso-Caldarelli, Claudia; Farias-Plazas, Fabian; Leszczynska, Anna; Gonzalez-Candial, Miguel

    2014-12-01

    The aim of this report is to present a case of a patient with bilateral lacrimal gland abscesses in the course of dacryoadenitis. A 45-year-old female patient with a long history of cocaine abuse presented with bilateral bacterial dacryoadenitis and upper lid inflammation with purulent discharge from a palpebral wound of the right upper lid. The diagnosis was confirmed with microbiology culture and an orbital CT scan, which revealed lacrimal gland abscesses. The patient admitted to vigorous eye scratching, which we believe was the mechanism responsible for the process. The infection resolved on targeted antibiotic therapy. This is the first reported case of bilateral infectious dacryoadenitis produced in a self-inflicted mechanism in a cocaine addict. PMID:25208047

  11. Postpartum Bilateral Sacroiliitis caused by Brucella Infection

    PubMed Central

    Yavuz, Ferdi; Altun, Demet; Ulubay, Mustafa; Firatligil, Fahri Burçin

    2015-01-01

    Early diagnosis of this septic sacroiliitis is difficult because symptoms are nonspecific during the postpartum period. In this case we dicscuss about a patient with bilateral buttock pain unresolved with painkillers and rest, after an induction delivery. A 31-year-old woman was presented to our clinic on the second week of postpartum period with bilateral buttock pain. She was subfebrile and had no apparent abnormality on her pelvic X-ray. The pain was so severe that she was unable to walk properly. Sacroiliac MRI during the acute episode of pain showed bone marrow oedema and fluid within the bilateral sacroiliac joint. She was found seropositive for brucellosis and the patient completely recovered with antibiotherapy treatment. We stopped our patient from breastfeeding when the Rose Bengal test turned out positive. Brucella sacroiliitis should be considered in puerperium period women when buttock pain and difficulty in walking are present and pain is unresponsive to analgesics. PMID:26675497

  12. Bilateral Meckel's cave amyloidoma: a case report.

    PubMed

    Gültaşli, N; van den Hauwe, L; Bruneau, M; D'Haene, N; Delpierre, I; Balériaux, D

    2012-05-01

    Primary solitary amyloidoma of Meckel's cave is rare, and a bilateral location is even more rare. To the best of our knowledge, only 12 cases in the literature have described such a primary lesion, including one case of bilateral involvement of Meckel's cave. We report here on the case of a 57-year-old woman presenting with pseudotumor masses involving both Meckel's caves and responsible for trigeminal neuropathy. The final diagnosis of amyloidoma was made on the basis of histological examination of surgical biopsy specimens. PMID:21641646

  13. Bilateral Atrial Myxoma: A Case Report.

    PubMed

    Susupaus, Attapoom; Foofuengmonkolkit, Kumpoo

    2016-02-01

    Among the rare cardiac tumors, myxoma, which is mostly located in the left atrium, is the most common type. Bilateral atrial myxoma is extremely rare, and requires urgent surgery. The authors report the case of a 34-year-old male, who presented with one month of right hemiparesis and aphasia and subsequently diagnosed with bilateral atrial myxoma based on transthoracic echocardiography. An urgent operation for intra-cardiac tumor removal was performed with the biatrial approach. Once a diagnosis of myxoma has been made, an urgent operation for tumor removal is necessary due to the risk of serious complications, including sudden death from normal blood flow obstruction. PMID:27266240

  14. A case of bilateral ophthalmoplegia while diving.

    PubMed

    Lee, Blair C; Young, Colin R

    2015-01-01

    This case report presents a military diver who became dysphoric and lost consciousness during a routine surface-supplied dive. The patient regained consciousness spontaneously, but the physical exam was notable for bilateral ophthalmoplegia. Full eye movement was regained during hyperbaric oxygen (HBO2) therapy, and the patient subsequently made a full recovery. Equipment and dive profile analysis led to the conclusion of hypercapnia and arterial gas embolism as the probable causes of the diver's symptoms. This is a unique case of isolated bilateral ophthalmoplegia presenting in a diving injury. PMID:26403021

  15. Acute infantile bilateral striatal necrosis: single-photon emission computed tomography (SPECT) imaging and review.

    PubMed

    Zevit, Noam; Steinmetz, Adam; Kornreich, Liora; Straussberg, Rachel

    2007-10-01

    Acute infantile bilateral striatal necrosis is a rarely described acute neurological syndrome associated with radiological findings. Its etiology and pathogenic mechanisms are unknown. Clinically, the syndrome usually follows respiratory illnesses and presents with an array of neurological findings, including axial ataxia, grimacing, mutism, head nodding, and high-pitched cry. This study follows a child with acute infantile bilateral striatal necrosis both clinically and radiologically. In addition, for the first time, the authors describe the serial findings of single-photon emission computed tomography (SPECT) from onset of illness through 20 months. Their findings indicate an initial insult apparent on both magnetic resonance imaging and SPECT localized to the basal ganglia, which, although improved over time, does not fully regress. The residual lesion on SPECT was clinically associated with only mild attention deficit disorder and no motor pathology. The authors review the published literature concerning acute infantile bilateral striatal necrosis and suggest possible mechanisms of this poorly understood and probably underreported condition. PMID:17940250

  16. Unusual Presentation of Recurrent Pyogenic Bilateral Psoas Abscess Causing Bilateral Pulmonary Embolism by Iliac Vein Compression

    PubMed Central

    Ijaz, Mohsin; Sakam, Sailaja; Ashraf, Umair; Marquez, Jose Gomez

    2015-01-01

    Patient: Male, 47 Final Diagnosis: Bilateral psoas abscess • acute lower extremity deep vein thrombosis • bilateral pulmonary embolism Symptoms: Progressive left leg swelling • productive cough with whitish sputum • right flank pain Medication: Antibiotics and anticoagulation Clinical Procedure: CT-guided percutaneous drain placement Specialty: Internal Medicine/Critical Care Objective: Unusual presentation Background: Psoas abscesses are a known cause of back pain, but they have not been reported as a cause of acute lower extremity thromboses and bilateral pulmonary emboli. We report a patient with bilateral psoas abscesses causing extensive pulmonary emboli through compression of the iliac vein. Case Report: A 47-year-old man presented with bilateral leg swelling over 4 weeks. Physical examination revealed a thin male with bilateral leg swelling, extending to the thigh on his left side. He had hemoglobin of 10.5 g/dl, leukocytosis of 16 000/ml, and an elevated D-dimer. A computed tomography (CT) angiogram of his chest showed extensive bilateral pulmonary emboli and infarcts. He remained febrile with vague flank pain, prompting a CT of his abdomen and pelvis that showed large, multiloculated, septated, bilateral psoas abscesses with compression of the left femoral vein by the left psoas abscess and a thrombus distal to the occlusion. Two liters of pus was drained from the left psoas abscess by CT-guidance, and although the Gram staining showed Gram-positive cocci in clusters, cultures from the abscess and blood were negative. A repeat CT showed resolution of the abscesses, and the drain was removed. He was discharged to a nursing home to complete a course of intravenous antibiotics and anticoagulation. Conclusions: Although the infectious complications of psoas abscesses have been described in the literature, the mechanical complications of bilateral psoas abscesses are lacking. It is important to assess for complete resolution of psoas abscesses through

  17. Kasabach – Merritt syndrome: A case report

    PubMed Central

    Osman, Nader M

    2013-01-01

    Kasabach-Merritt syndrome is characterised by the combination of rapidly growing vascular tumour, thrombocytopenia, microangiopathic haemolytic anaemia and consumptive coagulopathy. The blood clotting disorder results from platelets and other clotting factors of the blood being used up within the tumor. We report a two- and- half month old Saudi female infant who presented with epistaxis, conjunctival haemorrage and bilateral periorbital ecchymosis.

  18. Neglected surgically intervened bilateral congenital dislocation of knee in an adolescent

    PubMed Central

    Kumar, Jaswant; Dhammi, Ish Kumar; Jain, Anil K

    2014-01-01

    Neglected bilateral congenital dislocation of knee is unusual. A 12 year old boy presented with inability to walk due to buckling of the knee. The symptoms were present since the child learnt walking. He preferred not to walk. Bilateral supracondylar femoral osteotomy was done at the age of 6 years. Patient had a fixed flexion deformity of both knees, 30° in the right (range of flexion from 30° to 45°) and 45° fixed flexion deformity in left knee respectively (range of flexion from 45° to 65°) when presented to us. The radiological examination revealed bilateral congenital dislocation of knee (CDK). No syndromic association was observed. He was planned for staged treatment. In stage I, the knee joints were distracted by Ilizarov ring fixators and this was followed by open reduction of both the knee joints in stage II. A bilateral supracondylar extension osteotomy was done 18 months after the previous surgery (stage III). The final followup visit at 4 years the patient presented with range of motion 5-100° and 5-80° on the right and left knee respectively with good functional outcome. The case is reported in view of lack of treatment guidelines for long standing neglected CDK in an adolescent child. PMID:24600070

  19. Bilateral Retrobulbar Optic Neuritis Caused by Varicella Zoster Virus in a Patient with AIDS

    PubMed Central

    Duda, Jose F.; Castro, Jose G.

    2015-01-01

    Aims To report on a case of bilateral retrobulbar optic neuritis in a patient with acquired immune deficiency syndrome (AIDS) caused by varicella-zoster virus (VZV); and to review the literature focusing on: cases reported, epidemiology, pathophysiology, diagnosis and treatment. Presentation of Case A 38-year-old woman with AIDS presented with a 10-day history of progressive bilateral visual loss and ocular pain. She had bilateral dilated pupils with no light perception; the fundoscopic examination was normal. Facial herpes zoster lesions appeared on the second day of hospitalization Magnetic resonance imaging (MRI) findings were compatible with a bilateral optic neuritis; the cerebrospinal fluid (CSF) showed pleocytosis, increased proteins and a positive VZV-DNA PCR. She was treated with intravenous acyclovir and corticosteroids and was able, when discharged 2 weeks after admission, to carry out activities of daily living. Discussion VZV retrobulbar optic neuritis has previously been reported in 12 patients with AIDS, more than half of the cases had concomitant herpes zoster and an associated retinopathy. A positive VZV-DNA in the CSF is indicative of VZV infection, initial use of intravenous acyclovir is recommended, and the concomitant use of corticosteroids would be a prudent choice; the duration of antiviral therapy remains undefined. Conclusion VZV retrobulbar optic neuritis in AIDS patients can occur with or without herpes zoster. It is a sight-threatening infectious and inflammatory process requiring the advice of specialists in infectious diseases, ophthalmology, neurology and viral microbiology. PMID:26740936

  20. MANAGEMENT OF BILATERAL FEMORAL NECK FRACTURE IN A NONAGENARIAN PATIENT--CASE REPORT.

    PubMed

    Popescu, D; Trandabaţ, C; Puha, B; Veliceasa, B; Alexa, O

    2016-01-01

    Simultaneous bilateral femoral neck fracture is rare injury. Cases with this type of fracture have been reported in the literature since the 1950s, following the introduction of electroconvulsive therapy which generates violent hip muscle contractions. In young patients' simultaneous bilateral femoral neck fracture results from high energy trauma (car accident or fall from height) in a normal bone. Pathological changes in bone structure occurring in chronic kidney disease, vitamin D deficiency, osteomalacia, osteoporosis, metabolic imbalances and administration of corticosteroids explain the occurrence of this particular type of fracture following low-energy trauma. We present the case of a 90-year-old female patient who suffered a simple fall from her own height resulting in a Garden IV bilateral femoral neck fracture. Our therapeutic option in this patient was bilateral uncemented bipolar hemiarthroplasty in a single session using a single tray of sterile surgical instruments and two sterile drapes. Postoperative outcome was very good, allowing the initiation of functional recovery on the first postoperative day. Uncemented hemiarthroplasty proved to be a good choice in such a patient in the associated diseases may trigger the risk of cardiovascular disturbances specific to bone cement implantation syndrome. PMID:27483720

  1. Opercular syndrome: A case report and review

    PubMed Central

    Desai, Soaham Dilip; Patel, Dipen; Bharani, Sheela; Kharod, Nikhil

    2013-01-01

    We present a 9-year-old boy with history of perinatal asphyxia and neonatal seizures; who presented with delayed development of speech, with predominant dysarthria, dysphagia, and drooling of saliva and unable to protrude tongue along with delayed motor and mental milestones. He had complex partial seizures since last 3 years requiring multiple anti-epileptic drugs. He had dysarthria, nasal twang, and drooling of saliva with difficulty in chewing and swallowing. Hearing and understanding were normal. Bilateral trigemino-facio-linguo-pharyngeal palsy was noticed on voluntary movements with normal jaw jerk with preserved automatic and emotional motor movements. Electroencephalography revealed focal left fronto-temporal epileptiform discharges and brain imaging was suggestive of bilateral cortical and subcortical region encephalomalacia, predominantly involving bilateral opercular region. The clinical and neuroimaging features correspond to bilateral opercular syndrome which could have resulted from the perinatal insult in this case. PMID:24082930

  2. Two Cases of Heerfordt's Syndrome: A Rare Manifestation of Sarcoidosis

    PubMed Central

    Furuta, Yasushi; Fukuda, Satoshi

    2016-01-01

    Heerfordt's syndrome is a rare manifestation of sarcoidosis characterized by the presence of facial nerve palsy, parotid gland enlargement, anterior uveitis, and low grade fever. Two cases of Heerfordt's syndrome and a literature review are presented. Case  1. A 53-year-old man presented with swelling of his right eyelid, right facial nerve palsy, and swelling of his right parotid gland. A biopsy specimen from the swollen eyelid indicated sarcoidosis and he was diagnosed with incomplete Heerfordt's syndrome based on the absence of uveitis. His symptoms were improved by corticosteroid therapy. Case  2. A 55-year-old woman presented with left facial nerve palsy, bilateral hearing loss, and swelling of her bilateral parotid glands. She had been previously diagnosed with uveitis and bilateral hilar lymphadenopathy. Although no histological confirmation was performed, she was diagnosed with complete Heerfordt's syndrome on the basis of her clinical symptoms. Swelling of the bilateral parotid glands and left facial nerve palsy were improved immediately by corticosteroid therapy. Sarcoidosis is a relatively uncommon disease for the otolaryngologist. However, the otolaryngologist may encounter Heerfordt's syndrome as this syndrome presents with facial nerve palsy and swelling of the parotid gland. Therefore, we otolaryngologists should diagnose and treat Heerfordt's syndrome appropriately in cooperation with pneumologists and ophthalmologists. PMID:26885424

  3. [Congenital lumbar hernia and bilateral renal agenesis].

    PubMed

    Barrero Candau, R; Garrido Morales, M

    2007-04-01

    We report a new case of congenital lumbar hernia. This is first case reported of congenital lumbar hernia and bilateral renal agenesis. We review literature and describe associated malformations reported that would be role out in every case of congenital lumbar hernia. PMID:17650728

  4. Fast and Provably Accurate Bilateral Filtering

    NASA Astrophysics Data System (ADS)

    Chaudhury, Kunal N.; Dabhade, Swapnil D.

    2016-06-01

    The bilateral filter is a non-linear filter that uses a range filter along with a spatial filter to perform edge-preserving smoothing of images. A direct computation of the bilateral filter requires $O(S)$ operations per pixel, where $S$ is the size of the support of the spatial filter. In this paper, we present a fast and provably accurate algorithm for approximating the bilateral filter when the range kernel is Gaussian. In particular, for box and Gaussian spatial filters, the proposed algorithm can cut down the complexity to $O(1)$ per pixel for any arbitrary $S$. The algorithm has a simple implementation involving $N+1$ spatial filterings, where $N$ is the approximation order. We give a detailed analysis of the filtering accuracy that can be achieved by the proposed approximation in relation to the target bilateral filter. This allows us to to estimate the order $N$ required to obtain a given accuracy. We also present comprehensive numerical results to demonstrate that the proposed algorithm is competitive with state-of-the-art methods in terms of speed and accuracy.

  5. Computed tomographic findings in bilateral adrenal tuberculosis

    SciTech Connect

    Wilms, G.E.; Baert, A.L.; Kint, E.J.; Pringot, J.H.; Goddeeris, P.G.

    1983-03-01

    The computed tomographic (CT) features of bilateral adrenal tuberculosis are reported in two cases that demonstrate two typical different clinical and morphological manifestations of the disease. The incidence and CT appearance of adrenal tuberculosis are discussed, with emphasis on differential diagnosis.

  6. [Bilateral bronchial rupture: problems of respiratory management].

    PubMed

    Sztark, F; Thicoïpé, M; Favarel-Garrigues, J F; Velly, J F; Lassié, P

    1995-01-01

    The authors report the case of bilateral bronchial rupture in a 39-year-old multiple trauma patient. During the thoracotomy for right main bronchus repair, a partial left bronchial rupture was recognized because of severe hypoxaemia after left selective intubation. PMID:7486281

  7. A Bilateral Traumatic Hip Obturator Dislocation

    PubMed Central

    Karaarslan, Ahmet Adnan; Acar, Nihat; Karci, Tolga; Sesli, Erhan

    2016-01-01

    A case of a bilateral simultaneous traumatic obturator dislocation of both hip joints in an 18-year-old young man following a traffic accident is presented. We reduced the dislocated femoral heads immediately under general anesthesia followed by passive and active exercises and early full-weight bearing mobilization. After 5 years, the result was excellent. PMID:26977327

  8. Bilateral spontaneous hyphema in juvenile xanthogranuloma.

    PubMed

    Vijayalakshmi, P; Shetty, Shashikant; Jethani, Jitendra; Devi, T B Uma

    2006-03-01

    This report describes a rare occurrence of bilateral, spontaneous, nontraumatic hyphema in a 6 weeks old infant, associated with a small, multiple skin lesions. The diagnosis of juvenile xanthogranuloma was confirmed by histopathological examination of the cutaneous lesions. The hyphaema cleared gradually in 2 weeks time with conservative management. PMID:16531672

  9. Bilateral segmental neurofibromatosis diagnosed during pregnancy.

    PubMed

    Maldonado Cid, P; Sendagorta Cudós, E; Noguera Morel, L; Beato Merino, M J

    2011-01-01

    Bilateral segmental neurofibromatosis is a rare subtype of neurofibromatosis type 1 defined by lesions affecting a single segment of the body and crossing the midline, with no systemic involvement. We present a case diagnosed during pregnancy because of the characteristic increase in size of the lesions during this period. PMID:21635828

  10. Cystic fibrosis presenting with bilateral facial palsy.

    PubMed

    Basu, Anna P; Kumar, Prashant; Devlin, Anita M; O'Brien, Christopher J

    2007-07-01

    A 15-week old male infant presented with bilateral lower motor neuron facial palsy of unknown cause. Subsequently his growth deteriorated and he developed progressively worsening cough and wheeze. A diagnosis of cystic fibrosis was confirmed and hypovitaminosis A detected. Improvement of the facial palsy was noted following standard management of cystic fibrosis including vitamin A supplementation. PMID:17287135

  11. Fast and Provably Accurate Bilateral Filtering.

    PubMed

    Chaudhury, Kunal N; Dabhade, Swapnil D

    2016-06-01

    The bilateral filter is a non-linear filter that uses a range filter along with a spatial filter to perform edge-preserving smoothing of images. A direct computation of the bilateral filter requires O(S) operations per pixel, where S is the size of the support of the spatial filter. In this paper, we present a fast and provably accurate algorithm for approximating the bilateral filter when the range kernel is Gaussian. In particular, for box and Gaussian spatial filters, the proposed algorithm can cut down the complexity to O(1) per pixel for any arbitrary S . The algorithm has a simple implementation involving N+1 spatial filterings, where N is the approximation order. We give a detailed analysis of the filtering accuracy that can be achieved by the proposed approximation in relation to the target bilateral filter. This allows us to estimate the order N required to obtain a given accuracy. We also present comprehensive numerical results to demonstrate that the proposed algorithm is competitive with the state-of-the-art methods in terms of speed and accuracy. PMID:27093722

  12. Bilateral giant cyst of the shoulder.

    PubMed

    Agarwal, A; Ferrante, J; Schmidt, R; Eisenbeis, C H

    1987-01-01

    The case of a 61 year old white female with a rapidly progressive rheumatoid arthritis who developed bilateral giant cyst of the shoulder is described here. Arthrographic investigation indicated that these giant cysts were true synovial cysts rather than "pseudocysts". PMID:3427842

  13. Syphilitic aortitis causing bilateral coronary ostial stenosis.

    PubMed

    Hosoba, Soh; Suzuki, Tomoaki; Koizumi, Yusuke; Asai, Tohru

    2011-02-01

    Coronary ostial stenosis in otherwise normal coronary vessels is a rare complication of syphilitic aortitis. A 47-year-old man with no coronary risk factors developed severe isolated ostial stenosis in the left main coronary artery and right coronary artery. He underwent coronary artery bypass grafting using the bilateral internal thoracic arteries and gastroepiploic artery and recovered uneventfully. PMID:21345777

  14. Leriche Syndrome Presenting as Depression with Erectile Dysfunction

    PubMed Central

    Gautam, Priyanka; Saha, Rashmita

    2016-01-01

    Leriche syndrome results from thrombotic occlusion of the abdominal aorta immediately above the site of its bifurcation. Impotence in leriche syndrome is caused due to proximal obstruction, commonly involving isolated common iliac, internal iliac, internal pudendal or dorsalis penis artery. The symptoms of Leriche syndrome include intermittent and bilateral claudication, pallor, coldness and fatigue in lower extremities. Data regarding psychiatric morbidity in Leriche syndrome is unavailable. We hereby report the case of Leriche syndrome, presenting to psychiatry outpatient department with depressive disorder and erectile dysfunction (ED) with focus on dilemmas faced in the diagnosis and management in psychiatry. PMID:27134979

  15. Leriche Syndrome Presenting as Depression with Erectile Dysfunction.

    PubMed

    Bhatia, M S; Gautam, Priyanka; Saha, Rashmita

    2016-03-01

    Leriche syndrome results from thrombotic occlusion of the abdominal aorta immediately above the site of its bifurcation. Impotence in leriche syndrome is caused due to proximal obstruction, commonly involving isolated common iliac, internal iliac, internal pudendal or dorsalis penis artery. The symptoms of Leriche syndrome include intermittent and bilateral claudication, pallor, coldness and fatigue in lower extremities. Data regarding psychiatric morbidity in Leriche syndrome is unavailable. We hereby report the case of Leriche syndrome, presenting to psychiatry outpatient department with depressive disorder and erectile dysfunction (ED) with focus on dilemmas faced in the diagnosis and management in psychiatry. PMID:27134979

  16. [Sturge-Weber syndrome].

    PubMed

    Reith, W; Yilmaz, U; Zimmer, A

    2013-12-01

    Sturge-Weber syndrome (SWS) is a rare congenital disease which affects the brain, the skin and the eyes. It is a sporadically occurring neurocutaneous syndrome affecting the intracerebral veins (venous angiomatosis). The frequency is estimated to be 1 in 50,000 births [1]. The main symptom is intracranial leptomeningeal angiomatosis which mostly affects the occipital and posterior parietal lobes and can occur unilaterally and also bilaterally. Facial cutaneous vascular alterations occur ipsilaterally in the form of port wine stains (nevus flammeus) which are normally found in the catchment area of the trigeminal branch VI. Other clinical symptoms associated with SWS are seizures, glaucoma, headaches, transient neurological stroke-like episodes and cognitive impairment. Neurological cortical symptoms often include hemiparesis, hemiatrophy and hemianopsia. PMID:24292369

  17. Siblings with a syndrome of hydrocephalus with patent aqueduct, growth retardation and associated anomalies.

    PubMed

    Slee, J; Knowles, S; Goldblatt, J

    1999-01-01

    We report on male siblings with hydrocephalus with associated abnormalities including growth retardation, midline cleft palate and bilateral 'fisting' of the hands, providing evidence for a familial syndrome of hydrocephalus and associated anomalies. PMID:10327245

  18. Laparoscopic total extraperitoneal repair of preoperatively diagnosed bilateral obturator and incidental bilateral femoral herniae.

    PubMed

    Malik, Muhammad Usman; Connelly, Tara M; Hamid, Mustafa; Pretorius, Frederik

    2016-01-01

    Obturator hernia (OH), a rare type of hernia, is associated with high morbidity and mortality. Diagnosis is often delayed as clinical symptoms are typically non-specific. OH is frequently associated with other occult inguinopelvic herniae. Early diagnosis is vital to decrease morbidity and mortality. We report the case of a 75-year-old woman who presented to the surgical outpatients' department with non-specific bilateral groin pain radiating to the thighs. CT of the pelvis demonstrated bilateral OH with no radiological evidence of bowel obstruction. Semiurgent elective laparoscopic total extraperitoneal mesh repair was performed. Intraoperative findings confirmed bilateral obturator herniae as well as incidental bilateral femoral herniae. This case highlights the need for a high index of suspicion for such concomitant hernias that, in the presence of OH, may only be identified intraoperatively. PMID:27113790

  19. Bilateral multicystic renal dysplasia with potter sequence. A case with penile agenesis.

    PubMed

    Dursun, Ahmet; Ermis, Bahri; Numanoglu, Varim; Bahadir, Burak; Seckiner, Ilker

    2006-11-01

    Hereditary renal adysplasia (HRA) is a rare autosomal dominant condition. Patients have several other anomalies including Potter facies, thoracic, cardiac, and extremity deformities. The case present dysmorphic facial features such as hypertelorism, prominent epicanthic folds, a flat and broad nose, choanal stenosis, low-set ears, and a receding chin. He had femoral bowing, hypoplastic right tibia and agenesis of the right foot. He had rich and thick skin. He had also a dysplastic empty scrotum, penile agenesis, and anal atresia. The autopsy revealed pulmonary hypoplasia, ventricular septal defect, bilateral multicystic renal dysplasia, agenesis of both ureter and bladder, intraabdominal testicles, and a single umbilical artery. The penile agenesis was first reported, and including the consanguinity in the parents might further delineate the bilateral multicystic HRA. Vater/caudal regression anomalies, Mullerian duct/aplasia, unilateral renal agenesis, and cervicothoracic somite anomalies association, and Coloboma, heart anomaly, choanal atresia, retardation, genital and ear anomalies syndrome has been considered in differential diagnosis. PMID:17106555

  20. A case of midbrain infarction with acute bilateral cerebellar ataxia visualized by diffusion tensor imaging.

    PubMed

    Maya, Yuka; Kawabori, Masahito; Oura, Daisuke; Niiya, Yoshimasa; Iwasaki, Motoyuki; Mabuchi, Shoji

    2016-08-31

    An 85-year-old woman with hypertension was admitted with a sudden onset of gait disturbance and dysarthria. On admission, the patient showed severe bilateral cerebellar ataxia with moderate right medial longitudinal fasciculus (MLF) syndrome. Magnetic resonance (MR) imaging showed an acute infarction in the lower and medial part of midbrain. Diffusion tensor imaging (DTI) started from both cerebellar peduncles revealed that the lesion of the acute infarction matched the decussation of superior cerebellar peduncle where crossing of tract was seen and a part of its tract was interrupted at the site. Interruption of the cerebellum red nuclear path at the medial part of midbrain was considered to be the reason for bilateral cerebellar ataxia and visualization of cerebellum red nuclear path by DTI can give better understanding of the neurological symptom. PMID:27477572

  1. Chronic Bilateral Tibial Stress Fractures with Valgus Treated with Bilateral Intramedullary Nailing: A Case Report

    PubMed Central

    Dailey, Steven K; Archdeacon, Michael T.

    2014-01-01

    Introduction: Stress fractures are overuse injuries most commonly seen in athletes, military recruits, and individuals with endocrine abnormalities. It has been demonstrated that chronic cases of anterior tibial stress fractures refractory to conservative management respond well to intramedullary nailing. To our knowledge, only one report has been published concerning patients with bilateral tibial stress fractures treated with bilateral intramedullary nailing. All patients in the series were high-level athletes. We present the case of a non-athletic patient with chronic bilateral tibial stress fractures and associated deformity successfully treated with bilateral intramedullary nails. Case Report: A 23-year-old Caucasian female full-time student presented with chronic bilateral shin pain for approximately five years. She had failed an extensive regimen of conservative management. She was diagnosed with chronic bilateral tibial stress fractures based on history, physical examination, and radiologic findings. She subsequently underwent sequential intramedullary nailing of her tibiae. Both tibiae were in valgus alignment; however, this did not preclude nail placement. The nails deformed upon insertion into the sclerotic canals to conform to the deformation. Post operatively the tibiae united and patient was relieved of her symptoms. Conclusion: Bilateral intramedullary nailing of chronic bilateral tibial stress fractures should be considered as a treatment option for all patients, not just high-level athletes, who fail a trial of conservative management. Additionally, mild to moderate tibial malalignment does not necessarily preclude tibial nailing as the smaller nails placed in sclerotic canals will likely deform on insertion and conform to the canal. PMID:27298944

  2. Bilateral Chest X-Ray Shadowing and Bilateral leg lesions - A case of Pulmonary Kaposi Sarcoma

    PubMed Central

    Khattak, M

    2016-01-01

    We report a case of a 30 year old gentleman seen on the respiratory ward with no significant past medical history presenting with a three week history of worsening dyspnoea, productive cough, fever, bilateral leg swelling and bilateral leg swelling. Initial differential diagnosis included community-acquired pneumonia, cellulitis and deep vein thrombosis. After much investigation a diagnosis of AIDS-related kaposi's sarcoma with visceral manifestations was made. PMID:27239297

  3. A review of bilateral training for upper extremity hemiparesis.

    PubMed

    Stoykov, Mary Ellen; Corcos, Daniel M

    2009-01-01

    Upper extremity hemiparesis is the most common post-stroke disability. Longitudinal studies have indicated that 30-66% of stroke survivors do not have full arm function 6 months post-stroke. The current gold standard for treatment of mild post-stroke upper limb impairment is constraint-induced therapy but, because of the inclusion criteria, alternative treatments are needed which target more impaired subjects. Bilateral arm training has been investigated as a potential rehabilitation intervention. Bilateral arm training encompasses a number of methods including: (1) bilateral isokinematic training; (2) mirror therapy using bilateral training; (3) device-driven bilateral training; and (4) bilateral motor priming. Neural mechanisms mediating bilateral training are first reviewed. The key bilateral training studies that have demonstrated evidence of efficacy will then be discussed. Finally, conclusions are drawn concerning clinical implications based on the reviewed literature. PMID:19517519

  4. Hardware Implementation of a Bilateral Subtraction Filter

    NASA Technical Reports Server (NTRS)

    Huertas, Andres; Watson, Robert; Villalpando, Carlos; Goldberg, Steven

    2009-01-01

    A bilateral subtraction filter has been implemented as a hardware module in the form of a field-programmable gate array (FPGA). In general, a bilateral subtraction filter is a key subsystem of a high-quality stereoscopic machine vision system that utilizes images that are large and/or dense. Bilateral subtraction filters have been implemented in software on general-purpose computers, but the processing speeds attainable in this way even on computers containing the fastest processors are insufficient for real-time applications. The present FPGA bilateral subtraction filter is intended to accelerate processing to real-time speed and to be a prototype of a link in a stereoscopic-machine- vision processing chain, now under development, that would process large and/or dense images in real time and would be implemented in an FPGA. In terms that are necessarily oversimplified for the sake of brevity, a bilateral subtraction filter is a smoothing, edge-preserving filter for suppressing low-frequency noise. The filter operation amounts to replacing the value for each pixel with a weighted average of the values of that pixel and the neighboring pixels in a predefined neighborhood or window (e.g., a 9 9 window). The filter weights depend partly on pixel values and partly on the window size. The present FPGA implementation of a bilateral subtraction filter utilizes a 9 9 window. This implementation was designed to take advantage of the ability to do many of the component computations in parallel pipelines to enable processing of image data at the rate at which they are generated. The filter can be considered to be divided into the following parts (see figure): a) An image pixel pipeline with a 9 9- pixel window generator, b) An array of processing elements; c) An adder tree; d) A smoothing-and-delaying unit; and e) A subtraction unit. After each 9 9 window is created, the affected pixel data are fed to the processing elements. Each processing element is fed the pixel value for

  5. Nongranulomatous anterior uveitis in a patient with Usher syndrome

    PubMed Central

    Alzuhairy, Sultan Abdulaziz S.; Alfawaz, Abdullah

    2013-01-01

    A 34-year-old female with Usher syndrome, but no family history of similar illness, presented with complaints of vision reduction, redness, and photophobia. Biomicroscopic examination showed mildly injected conjunctivae bilateral, small, round keratic precipitates; bilateral +2 cells with no flare reaction in the anterior chamber; and bilateral posterior subcapsular cataracts. No associated posterior synechiae, angle neovascularization, or iris changes were detected; normal intraocular pressures were obtained. Fundus examination demonstrated waxy pallor of both optic nerves, marked vasoconstriction in retinal vessels, and retinal bone spicule pigment formation, with a normal macula. Electroretinography confirmed the diagnosis of retinitis pigmentosa, optical coherent tomography was normal and otolaryngology consultation was conducted. To our knowledge, an association between Usher syndrome and bilateral nongranulomatous anterior uveitis has not been previously reported, and our purpose is to report this association. PMID:24371428

  6. An Approach to Bilateral Control System for Improvement of Operationality

    NASA Astrophysics Data System (ADS)

    Tanaka, Hiroyuki; Ohnishi, Kouhei

    Many types of bilateral control architectures to transmit force sensation have been proposed. Some control methods obtain good performance. Operationality is not satisfactory, however, when these methods are implemented in bilateral systems with large inertia and friction force. In this paper, a novel bilateral control method is proposed to improve operationality of bilateral systems with large inertia and friction force. The proposed method is compared with a conventional method, and the effectiveness of the proposed method is demonstrated by coordinate transformation and experiments.

  7. [Bilateral testicular metastasis of cancer of the prostate].

    PubMed

    el Moussaoui, A; Sarf, I; Dakir, M; Zamiati, S; Benjelloun, S

    1997-01-01

    Testicular metastasis of prostate cancer rarely occurs. Bilateral localization is exceptional. We report a new case of prostate adenocarcinoma with bilateral testicular metastasis. The diagnosis was made on clinical and ultrasonic arguments, and confirmed on the pathological specimen. Treatment consisted in a bilateral orchidectomy, associated with nonsteroid androgens. PMID:9765784

  8. Nocturnal seizure and simultaneous bilateral shoulder fracture-dislocation.

    PubMed

    Sahbudin, Ilfita; Filer, Andrew

    2016-01-01

    An otherwise fit and well 27-year-old man presented with acute onset unexplained bilateral shoulder pain, and was found to have bilateral shoulder fractures and dislocations on imaging. Although features were atypical, a nocturnal seizure causing the bilateral shoulder fractures was suspected and EEG showed features compatible with epilepsy. PMID:26838296

  9. Bilateral Orbital Abscesses After Strabismus Surgery.

    PubMed

    Dhrami-Gavazi, Elona; Lee, Winston; Garg, Aakriti; Garibaldi, Daniel C; Leibert, Michelle; Kazim, Michael

    2015-01-01

    Infectious orbital complications after strabismus surgery are rare. Their incidence is estimated to be 1 case per 1,100 surgeries and include preseptal cellulitis, orbital cellulitis, subconjunctival and sub-Tenon's abscesses, myositis, and endophthalmitis. This report describes the case of an otherwise healthy 3-year-old boy who underwent bilateral medial rectus recession and disinsertion of the inferior obliques. A few days after surgery, the patient presented with bilateral periorbital edema and inferotemporal chemosis. A series of CT scans with contrast revealed inferotemporal orbital collections OU. The patient immediately underwent transconjunctival drainage of fibrinous and seropurulent collections in the sub-Tenon's space and experienced rapid improvement a few days later. The patient is reported to be in stable condition in a follow-up examination performed more than a year after the reported events. PMID:24896771

  10. Simultaneous, Isolated Traumatic Bilateral Patella Fractures

    PubMed Central

    Madi, Sandesh; Naik, Monappa; Rao, Sharath; Vijayan, Sandeep

    2016-01-01

    Introduction Isolated traumatic fractures of both Patellae, occurring at the same time, in an otherwise healthy person, are very rare. The Patella, as a subcutaneous and a cancellous bone, is vulnerable to injury. However, simultaneous injury to both Patellae without the involvement of any other bony injuries occurs infrequently, and only a few cases have been reported in the literature. Case Presentation We report a rare case of isolated, traumatic bilateral Patella fracture with unusual fracture patterns and briefly review the literature. Conclusions In simultaneous bilateral Patella fractures, good functional outcome can be expected following a stable surgical fixation and a structured rehabilitation regime. However, personality of the fracture determines salvage versus sacrifice of the Patella. PMID:27218047

  11. Bilateral traumatic facial paralysis. Case report.

    PubMed

    Undabeitia, Jose; Liu, Brian; Pendleton, Courtney; Nogues, Pere; Noboa, Roberto; Undabeitia, Jose Ignacio

    2013-01-01

    Although traumatic injury of the facial nerve is a relatively common condition in neurosurgical practice, bilateral lesions related to fracture of temporal bones are seldom seen. We report the case of a 38-year-old patient admitted to Intensive Care Unit after severe head trauma requiring ventilatory support (Glasgow Coma Scale of 7 on admission). A computed tomography (CT) scan confirmed a longitudinal fracture of the right temporal bone and a transversal fracture of the left. After successful weaning from respirator, bilateral facial paralysis was observed. The possible aetiologies for facial diplegia differ from those of unilateral injury. Due to the lack of facial asymmetry, it can be easily missed in critically ill patients, and both the high resolution CT scan and electromyographic studies can be helpful for correct diagnosis. PMID:23541180

  12. Bilateral idiopathic optic neuritis in pregnancy.

    PubMed

    Liu, Tiffany S; Crow, Robert W

    2013-01-01

    A 33-year-old primiparous woman at 31 weeks gestation with no prior history of ocular disease, autoimmune disease, or recent viral infection presented with rapidly decreasing visual acuity to 20/400 OD and counting fingers OS over 11 days. MRI demonstrated mild bilateral optic nerve enhancement. Humphrey 30-2 visual field testing showed bilateral seco-central scotomas. MR venogram, visual evoked potentials, preeclampsia workup, and serum blood tests for folate, B6, B12, Toxoplasmosis, Bartonella, Lyme disease, ACE levels, RPR, and LFTs were all within normal limits. After one-time treatment of IV 125 mg methylprednisolone and spinal tap, visual acuity improved to 20/20 OD and 20/20 OS. PMID:23305442

  13. Peribulbar anesthesia causing bilateral orbital hemorrhage

    PubMed Central

    Garft, Kyla; Burt, Peter; Burt, Benjamin

    2016-01-01

    We report a case of bilateral orbital hemorrhage as a complication of peribulbar anesthesia in a 78 year old man. Initially, unilateral orbital hemorrhage occurred but quickly spread to the contralateral side. Neuroophthalmological assessment revealed a proptosed tense globe with normal retinovascular findings. Visual acuity was adversely affected and this was conservatively managed with no lasting ophthalmic sequela. This patient’s case was reported as it illustrates an unusual complication of bilateral spread of orbital hemorrhage secondary to peribulbar anesthesia. It highlights how early ophthalmic assessment can ensure a good visual outcome in the setting of appropriate ophthalmic monitoring. The mechanisms of orbital hemorrhage spread and appropriate management options are discussed. PMID:27013899

  14. Bilateral calcaneal stress fractures in two cats.

    PubMed

    Cantatore, M; Clements, D N

    2015-06-01

    Two cats that developed bilateral calcaneal stress fractures are reported. One cat developed lameness associated with incomplete fractures at the base of both calcanei, both of which progressed to acute, complete fractures 2 months later. The second cat presented with acute complete calcaneal fracture, with evidence of remodelling of the contralateral calcaneus, which subsequently fractured two years later. The calcaneal fractures were successfully stabilised with lateral bone plates in each case. Stress fractures were suspected because of the bilateral nature, the simple and similar configuration, the consistent location of the fractures, the absence of other signs of trauma in both cases and the suspected insidious onset of the lameness. The feline calcaneus is susceptible to stress fracture, and cats presenting with calcaneal fractures without evidence of trauma should be evaluated for concurrent skeletal pathology. PMID:25929309

  15. Bilateral Nocardia Keratitis after Photorefractive Keratectomy

    PubMed Central

    Faramarzi, Amir; Feizi, Sepehr; Javadi, Mohammad-Ali; Rezaei Kanavi, Mozhgan; Yazdizadeh, Forouzan; Moein, Hamid-Reza

    2012-01-01

    Purpose To report the clinical, confocal scan, and histopathologic features of nocardia keratitis in a patient who developed bilateral infection following photorefractive keratectomy (PRK). Case Report A 23-year-old woman underwent bilateral PRK for low myopia. On postoperative day 3, dense central stromal infiltrates were noticed in both eyes. Empirical antibiotic therapy was initiated which was converted into specific therapy after a definite diagnosis was made based on clinical features and confirmed by confocal scan and histopathologic findings. Clinical and confocal scan features were consistent with the diagnosis of Nocardia keratitis, and topical 2% amikacin eye drops were started. Because of poor response to medical therapy, lamellar keratectomy was performed in both eyes which shortened the treatment course. Histopathologic examination reconfirmed the initial diagnosis. Conclusion Familiarity with clinical and confocal scan features facilitates early diagnosis of Nocardia keratitis leading to proper management and hence a rapid therapeutic response. PMID:23275825

  16. Dravet Syndrome

    MedlinePlus

    ... NINDS Dravet Syndrome Information Page Synonym(s): Severe Myoclonic Epilepsy of Infancy (SMEI) Table of Contents (click to ... Dravet Syndrome? Dravet syndrome, also called severe myoclonic epilepsy of infancy (SMEI), is a severe form of ...

  17. Williams syndrome

    MedlinePlus

    Williams-Beuren syndrome ... Williams syndrome is a rare condition caused by missing a copy of several genes. Parents may not have ... history of the condition. However, a person with Williams syndrome has a 50% chance of passing the disorder ...

  18. Brown Syndrome

    MedlinePlus

    ... Does Brown syndrome cause eye problems besides abnormal eye movements? Some children with Brown syndrome have poor binocular ... In the congenital form of Brown syndrome, the eye movement problem is usually constant and unlikely to resolve ...

  19. Fahr's Syndrome

    MedlinePlus

    ... Awards Enhancing Diversity Find People About NINDS NINDS Fahr's Syndrome Information Page Synonym(s): Familial Idiopathic Basal Ganglia ... is being done? Clinical Trials Organizations What is Fahr's Syndrome? Fahr's Syndrome is a rare, genetically dominant, ...

  20. Cushing syndrome

    MedlinePlus

    ... Cushing disease Cushing syndrome due to adrenal tumor Diabetes Ectopic Cushing syndrome Exogenous Cushing syndrome Kidney stones Pituitary tumor Rheumatoid arthritis Tumor Update Date 10/28/2015 Updated by: ...

  1. A rare case of bilateral aspergillus endophthalmitis.

    PubMed

    Gupta, Saurabh; Loudill, Cameron; Tammara, Anita; Chow, Robert T

    2015-01-01

    Aspergillus endophthalmitis is a devastating inflammatory condition of the intraocular cavities that may result in irreparable loss of vision and rapid destruction of the eye. Almost all cases in the literature have shown an identified source causing aspergillus endophthalmitis as a result of direct extension of disease. We present a rare case of bilateral aspergillus endophthalmitis. A 72-year-old woman with a history of diabetes mellitus, congenital Hirschsprung disease, and recent culture-positive candida pyelonephritis with hydronephrosis status post-surgical stent placement presented with difficulty opening her eyes. She complained of decreased vision (20/200) with pain and redness in both eyes - right worse then left. Examination demonstrated multiple white fungal balls in both retinas consistent with bilateral fungal endophthalmitis. Bilateral vitreous taps for cultures and staining were performed. Patient was given intravitreal injections of amphotericin B, vancomycin, ceftazidime, and started on oral fluconazole. Patient was scheduled for vitrectomy to decrease organism burden and to remove loculated areas of infection that would not respond to systemic antifungal agents. Four weeks after initial presentation, the fungal cultures revealed mold growth consistent with aspergillus. Patient was subsequently started on voriconazole and fluconazole was discontinued due to poor efficacy against aspergillus. Further workup was conducted to evaluate for the source of infection and seeding. Transthoracic cardiogram was unremarkable for any vegetation or valvular abnormalities. MRI of the orbits and sinuses did not reveal any mass lesions or bony destruction. CT of the chest was unremarkable for infection. Aspergillus endophthalmitis may occur because of one of these several mechanisms: hematogenous dissemination, direct inoculation by trauma, and contamination during surgery. Our patient's cause of bilateral endophthalmitis was through an unknown iatrogenic seed

  2. A rare case of bilateral aspergillus endophthalmitis

    PubMed Central

    Gupta, Saurabh; Loudill, Cameron; Tammara, Anita; Chow, Robert T.

    2015-01-01

    Aspergillus endophthalmitis is a devastating inflammatory condition of the intraocular cavities that may result in irreparable loss of vision and rapid destruction of the eye. Almost all cases in the literature have shown an identified source causing aspergillus endophthalmitis as a result of direct extension of disease. We present a rare case of bilateral aspergillus endophthalmitis. A 72-year-old woman with a history of diabetes mellitus, congenital Hirschsprung disease, and recent culture-positive candida pyelonephritis with hydronephrosis status post-surgical stent placement presented with difficulty opening her eyes. She complained of decreased vision (20/200) with pain and redness in both eyes – right worse then left. Examination demonstrated multiple white fungal balls in both retinas consistent with bilateral fungal endophthalmitis. Bilateral vitreous taps for cultures and staining were performed. Patient was given intravitreal injections of amphotericin B, vancomycin, ceftazidime, and started on oral fluconazole. Patient was scheduled for vitrectomy to decrease organism burden and to remove loculated areas of infection that would not respond to systemic antifungal agents. Four weeks after initial presentation, the fungal cultures revealed mold growth consistent with aspergillus. Patient was subsequently started on voriconazole and fluconazole was discontinued due to poor efficacy against aspergillus. Further workup was conducted to evaluate for the source of infection and seeding. Transthoracic cardiogram was unremarkable for any vegetation or valvular abnormalities. MRI of the orbits and sinuses did not reveal any mass lesions or bony destruction. CT of the chest was unremarkable for infection. Aspergillus endophthalmitis may occur because of one of these several mechanisms: hematogenous dissemination, direct inoculation by trauma, and contamination during surgery. Our patient's cause of bilateral endophthalmitis was through an unknown iatrogenic seed

  3. Idiopathic Bilateral External Jugular Vein Thrombosis

    PubMed Central

    Hindi, Zakaria; Fadel, Ehab

    2015-01-01

    Patient: Male, 21 Final Diagnosis: Idiopathic bilateral external jugular vein thrombosis Symptoms: Face engorgement • neck swelling Medication: — Clinical Procedure: None Specialty: Hematology Objective: Unknown ethiology Background: Vein thrombosis is mainly determined by 3 factors, which constitute a triad called Virchow’s triad: hypercoagulability, stasis, and endothelial injury. Venous thrombosis commonly occurs in the lower extremities since most of the blood resides there and flows against gravity. The veins of the lower extremities are dependent on intact valves and fully functional leg muscles. However, in case of valvular incompetency or muscular weakness, thrombosis and blood stasis will occur as a result. In contrast, the veins of the neck, specially the jugulars, have distensible walls which allow flexibility during respiration. In addition, the blood directly flows downward towards the heart. Nevertheless, many case reports mentioned the thrombosis of internal jugular veins and external jugular veins with identified risk factors. Jugular vein thrombosis has previously been associated in the literature with a variety of medical conditions, including malignancy. Case Report: This report is of a case of idiopathic bilateral external jugular vein thrombosis in a 21 year-old male construction worker of Southeast Asian origin with no previous medical history who presented with bilateral facial puffiness of gradual onset over 1 month. Doppler ultrasound and computed tomography were used in the diagnosis. Further work-up showed no evidence of infection or neoplasia. The patient was eventually discharged on warfarin. The patient was assessed after 6 months and his symptoms had resolved completely. Conclusions: Bilateral idiopathic external jugular veins thrombosis is extremely rare and can be an indicator of early malignancy or hidden infection. While previous reports in the literature have associated jugular vein thrombosis with malignancy, the present

  4. Erlotinib-related bilateral anterior uveitis

    PubMed Central

    Ali, Kashif; Kumar, Indu; Usman-Saeed, Muniba; Usman Saeed, Muhammad

    2011-01-01

    The authors report the case of a 68-year-old woman with secondary adenocarcinoma of the lungs from an unknown primary. Erlotinib was started which produced symptoms suggestive of uveitis. Erlotinib was stopped and restarted a month later at a lower dose, which resulted in severe bilateral anterior uveitis. The uveitis settled after stopping erlotinib and treatment with topical steroids and cycloplegics. To the best of the authors’ knowledge, this is the first case of erlotinib-related anterior uveitis. PMID:22694887

  5. Bilateral pedicled gracilis flap for scrotal reconstruction.

    PubMed

    Daigeler, Adrien; Behr, Björn; Mikhail, Bassem Daniel; Lehnhardt, Marcus; Wallner, Christoph

    2016-09-01

    Trauma, infection and cancer can cause severe scrotal defects. The demand to a definitive reconstruction in terms of aesthetics and function is high. Primary closure, skin grafts and local fasciocutaneous flaps do not meet these high demands in most cases. The authors treated a series of patients requiring scrotal reconstruction with bilateral pedicled gracilis muscle flaps and split thickness skin grafts, resulting in satisfying aesthetic and functional outcomes. PMID:27318782

  6. Effective analgesia after bilateral tubal ligation.

    PubMed

    Wittels, B; Faure, E A; Chavez, R; Moawad, A; Ismail, M; Hibbard, J; Principe, D; Karl, L; Toledano, A Y

    1998-09-01

    To evaluate the analgesic efficacy of local anesthetic infiltration, 20 parturients scheduled for elective minilaparotomy and bilateral tubal ligation with either spinal or epidural anesthesia participated in this prospective, randomized, controlled, double-blind trial. All patients received intravenous (iv) metoclopramide 10 mg and ketorolac 60 mg intraoperatively, as well as preincisional infiltration of the infraumbilical skin incision with 0.5% bupivacaine. Infiltration of bilateral uterine tubes and mesosalpinx was performed either with 0.5% bupivacaine (n = 10) or isotonic sodium chloride solution (n = 10). Intravenous meperidine (25 mg every 3 minutes as needed) was given to treat pain in the postanesthesia care unit (PACU). The total amount of meperidine administered in the PACU was significantly larger in the saline group than in the bupivacaine group. Pain scores at 30, 45, 60, 75, and 90 minutes postoperatively and on the 7th postoperative day were significantly lower in the bupivacaine group than in the saline group. During tubal ligation, infiltration of uterine tubes and mesosalpinx with 0.5% bupivacaine significantly enhanced analgesia both immediately postoperatively and on the 7th postoperative day compared with infiltration with sodium chloride. In conclusion, this study proved that during bilateral tubal ligation with either spinal or epidural anesthesia, preemptive analgesia using iv ketorolac, iv metoclopramide, and infiltration of the incised skin and uterine tubes with 0.5% bupivacaine can eliminate pain, nausea, vomiting, or cramping and maintain good analgesia for 7 days postoperatively. PMID:9728841

  7. High Performance, Three-Dimensional Bilateral Filtering

    SciTech Connect

    Bethel, E. Wes

    2008-06-05

    Image smoothing is a fundamental operation in computer vision and image processing. This work has two main thrusts: (1) implementation of a bilateral filter suitable for use in smoothing, or denoising, 3D volumetric data; (2) implementation of the 3D bilateral filter in three different parallelization models, along with parallel performance studies on two modern HPC architectures. Our bilateral filter formulation is based upon the work of Tomasi [11], but extended to 3D for use on volumetric data. Our three parallel implementations use POSIX threads, the Message Passing Interface (MPI), and Unified Parallel C (UPC), a Partitioned Global Address Space (PGAS) language. Our parallel performance studies, which were conducted on a Cray XT4 supercomputer and aquad-socket, quad-core Opteron workstation, show our algorithm to have near-perfect scalability up to 120 processors. Parallel algorithms, such as the one we present here, will have an increasingly important role for use in production visual analysis systems as the underlying computational platforms transition from single- to multi-core architectures in the future.

  8. Bilateral limbic system destruction in man

    PubMed Central

    Feinstein, Justin S.; Rudrauf, David; Khalsa, Sahib S.; Cassell, Martin D.; Bruss, Joel; Grabowski, Thomas J.; Tranel, Daniel

    2010-01-01

    We report here a case study of a rare neurological patient with bilateral brain damage encompassing a substantial portion of the so-called “limbic system.” The patient, Roger, has been studied in our laboratory for over 14 years and the current article presents his complete neuroanatomical and neuropsychological profiles. The brain damage occurred in 1980 following an episode of herpes simplex encephalitis. The amount of destroyed neural tissue is extensive and includes bilateral damage to core limbic and paralimbic regions, including the hippocampus, amygdala, parahippocampal gyrus, temporal poles, orbitofrontal cortex, basal forebrain, anterior cingulate cortex, and insular cortex. The right hemisphere is more extensively affected than the left, although the lesions are largely bilateral. Despite the magnitude of his brain damage, Roger has a normal IQ, average to above average attention, working memory, and executive functioning skills, and very good speech and language abilities. In fact, his only obvious presenting deficits are a dense global amnesia and a severe anosmia and ageusia. Roger's case presents a rare opportunity to advance our understanding of the critical functions underlying the human limbic system, and the neuropsychological and neuroanatomical data presented here provide a critical foundation for such investigations. PMID:19763994

  9. Multilateral, regional and bilateral energy trade governance

    SciTech Connect

    Leal-Arcas, Rafael; Grasso, Costantino; Rios, Juan Alemany )

    2014-12-01

    The current international energy trade governance system is fragmented and multi-layered. Streamlining it for greater legal cohesiveness and international political and economic cooperation would promote global energy security. The current article explores three levels of energy trade governance: multilateral, regional and bilateral. Most energy-rich countries are part of the multilateral trading system, which is institutionalized by the World Trade Organization (WTO). The article analyzes the multilateral energy trade governance system by focusing on the WTO and energy transportation issues. Regionally, the article focuses on five major regional agreements and their energy-related aspects and examines the various causes that explain the proliferation of regional trade agreements, their compatibility with WTO law, and then provides several examples of regional energy trade governance throughout the world. When it comes to bilateral energy trade governance, this article only addresses the European Union’s (EU) bilateral energy trade relations. The article explores ways in which gaps could be filled and overlaps eliminated whilst remaining true to the high-level normative framework, concentrating on those measures that would enhance EU energy security.

  10. [Clinical features of bilateral trigeminal neuralgia].

    PubMed

    Yokosako, Suguru; Takahashi, Yuichi; Kikuchi, Asami; Yoshimura, Chika; Arai, Naoyuki; Ohbuchi, Hidenori; Hirota, Kengo; Hagiwara, Shinji; Tani, Shigeru; Sasahara, Atsushi; Kasuya, Hidetoshi

    2015-02-01

    Among 238 patients with bilateral trigeminal neuralgia(TN)who visited our hospital between April 2007 and June 2014, 5(2%)were surgically treated by microvascular decompression(MVD). The initial symptom was on the right side in four and on both sides in one patient. Intervals between the initial and second onset on the other side(left)were two months, and four, six, and eight years. None of the patients showed involvement of the first branch of the trigeminal nerve. The patients with bilateral TN were younger than the 154 patients with unilateral TN who were treated surgically by MVD in this period(45 vs. 65 years), and the bilateral TN patients predominantly were women(4/5 vs. 99/154). In the surgical field, the trigeminal nerve and root entry zone were compressed more by veins in the bi lateral TN patients than in the unilateral TN(4/5 vs. 60/154, respectively)patients. We could not identify any differences in MRI CISS before versus after the onset of left trigeminal neuralgia, suggesting that compression is not the sole cause of the symptom. PMID:25672553

  11. Bilateral caudate infarct--a case report.

    PubMed

    Lim, J K; Yap, K B

    1999-07-01

    Caudate strokes comprise only a small proportion of all subtypes of strokes. Bilateral caudate infarcts are even rarer and only a few cases have been reported in the literature. We report an 86-year-old woman with bilateral caudate infarcts. She had no past medical history of note. She presented with headache for several days and drowsiness on day of admission. Clinical examination revealed abulia, inability to comprehend or verbalize (acute mutism), right-sided neglect and right-sided hemiparesis. Computed tomographic (CT) scan brain revealed decreased attenuation in both heads of both caudate nuclei with extension across the anterior limb of both internal capsules to involve the lentiform nuclei. Echocardiography showed aortic valve sclerosis, mild mitral and aortic regurgitation and normal left ventricular function. Carotid ultrasound revealed mild stenosis of proximal right internal carotid and left distal common carotid and adjacent proximal internal carotid arteries. She showed initial improvement in the first week, but subsequently had a progressive downward course despite rehabilitation and died 44 days after her stroke. A patient with bilateral caudate infarcts is likely to have poor prognosis for rehabilitation and survival. PMID:10561773

  12. Management of Severe Bilateral Ureteropelvic Junction Obstruction in Neonates with Prenatally Diagnosed Bilateral Hydronephrosis

    PubMed Central

    Kim, Jongwon; Hong, Sungwoo; Park, Chang Hoo; Park, Hongzoo

    2010-01-01

    Purpose The management of prenatally detected bilateral ureteropelvic junction obstruction (UPJO) remains controversial. We attempted to develop a treatment plan for patients with severe bilateral UPJO. Materials and Methods We evaluated the records of 13 patients with prenatally diagnosed grade 3 or more bilateral hydronephrosis that led to the postnatal diagnosis of severe bilateral UPJO. Ultrasonography and 99mtechnetium mercaptoacetyltriglycine (99mTc-MAG3) renal scans were performed within 1 month. Four renal units had grade 3 and 22 had grade 4 hydronephrosis. All 13 patients were managed by unilateral pyeloplasty, and the patients' mean age was 3 months. At 1 month postoperatively, we decided whether delayed surgery in the opposite renal unit was necessary according to the findings of ultrasonography and 99mTc-MAG3 scans. Results Of 13 patients, 11 underwent initial pyeloplasty on renal units with more severe hydronephrosis or lower relative renal function (RRF) on 99mTc-MAG3 scans. The remaining 2 patients simultaneously underwent percutaneous nephrostomy on renal units with a lower RRF and initial pyeloplasty on renal units with a higher RRF. In 5 patients, contralateral hydronephrosis had spontaneously improved at 1 month postoperatively, and 8 patients underwent delayed contralateral pyeloplasty at 2 months postoperatively. Conclusions In children with severe bilateral UPJO, the non-operated renal units with grade 3 and some with grade 4 hydronephrosis improved spontaneously after unilateral pyeloplasty. Therefore, delayed pyeloplasty of the opposite side should be considered at 1 month following initial pyeloplasty. PMID:20856652

  13. Iatrogenic “buffalo chest” bilateral pneumothoraces following unilateral transbronchial lung biopsies in a bilateral lung transplant recipient

    PubMed Central

    Sawalha, Leith; Gibbons, William J.

    2015-01-01

    We present a 54 year old male patient who had a bilateral lung transplant sixteen years ago for Alpha-1 Antitrypsin Deficiency-related emphysema. He was referred for flexible bronchoscopy with transbronchial biopsies to evaluate new mild exertional dyspnea and worsening of his FEV1. Eight transbronchial biopsies were done from the right middle lobe and the right lower lobe. Post procedure he developed bilateral pneumothoces that required emergent bilateral pleural ‘pigtail’ catheters. To our knowledge, this is the first reported case of bilateral pneumothoraces that developed after a unilateral procedure in a bilateral lung transplant recipient relatively late after the transplant. PMID:26236604

  14. Neu-Laxova syndrome: a case report.

    PubMed

    Roy, S; Begum, J; Sharifunnaher, B; Saha, A K; Afroza, S; Islam, M Z

    2014-01-01

    Neu-Laxova syndrome (NLS) is a rare lethal syndrome found in both consanguinous and non-consanguinous couple. This is characterized by terrible face with unusual craniofacial appearance with exophthalmos, spectrum of central nervous system malformation, like microcaphaly, hypoplastic cerebellum, cleft lip/palate, ichthyosis and oedema. The diagnosis is made on the basis of clinical parameter. We report a 4 hour old male term newborn with IUGR of Neu-Laxova syndrome presented with anencephaly, rudimentary cerebellum, exophthalmos of right eye, bilateral cleft lip and palate and cryptorchidism. Anencephaly and cryptorchidism are two recently reported findings of NLS. We are presenting this case in addition from Bangladesh to lend further support to those two new findings as component of Neu-Laxova syndrome. Outcome of this syndrome is not good. Most of the patients are died of infection within hours to days. PMID:24584393

  15. Single stage minimally invasive bilateral video assisted thoracoscopic surgery for simultaneous bilateral primary spontaneous pneumothorax.

    PubMed

    Sachithanandan, A; Nur Ezrin, I; Badmanaban, B

    2012-04-01

    Simultaneous bilateral spontaneous pneumothorax (SBSP) is a very rare life-threatening condition that requires rapid diagnosis and treatment. Most cases are secondary to various underlying lung pathology but a primary SBSP may occur due to rupture of subpleural blebs or bullae. Surgery via an open or minimally invasive approach provides definitive treatment and can be undertaken as a staged or simultaneous procedure. We report our experience with two such rare cases utilizing a single stage minimally invasive bilateral video assisted thoracoscopic (VATS) approach. The pathogenesis of this rare condition and intra-operative technical considerations for a successful outcome are discussed. PMID:22822653

  16. An Unusual Case of Neuralgic Amyotrophy Presenting with Bilateral Phrenic Nerve and Vocal Cord Paresis

    PubMed Central

    Holtbernd, F.; Zehnhoff-Dinnesen, A. am; Duning, T.; Kemmling, A.; Ringelstein, E.B.

    2011-01-01

    Background Neuralgic amyotrophy (brachial plexus neuropathy, brachial plexus neuritis, or Parsonage-Turner syndrome) is an uncommon inflammatory condition typically characterized by acute and severe shoulder pain followed by paresis with muscle weakness and atrophy of the upper limb or shoulder girdle. We report an unusual clinical manifestation of neuralgic amyotrophy, namely bilateral phrenic nerve palsy with concomitant laryngeal paresis. Case Report A 55-year-old male presented with orthopnea and aphonia after an episode of bilateral shoulder pain preceded by an upper respiratory tract infection. Spirometry, chest X-ray and videolaryngoscopy revealed bilateral and simultaneous paresis of the diaphragm and the vocal cords. Clinical examination at admission and at the 2-month follow-up did not show upper limb weakness or atrophy, except for a mild atrophy of the right supraspinatus muscle. An electromyography of the upper limb muscles and nerve conduction studies did not reveal signs of denervation. Analysis of the cerebrospinal fluid and an MRI of the neuraxis were unremarkable. After treatment with prednisolone, vocal cord function markedly improved within 8 weeks, whereas paresis of the diaphragm persisted. Conclusion Shoulder pain followed by diaphragmatic paralysis with dyspnea and hoarseness may be a manifestation of neuralgic amyotrophy even if upper limb or shoulder girdle palsies are absent. PMID:21490716

  17. Bilateral Wilms' tumors: changing concepts in management

    SciTech Connect

    Laberge, J.M.; Nguyen, L.T.; Homsy, Y.L.; Doody, D.P.

    1987-08-01

    Bilaterality is uncommon in Wilms' tumor, being present in 4% to 8% of the cases. We report the combined experience of two children's hospitals in one city over a 20-year period. We encountered nine cases of synchronous bilateral nephroblastoma (National Wilms' Tumor Study 3, stage V). Age at diagnosis ranged from 9 to 41 months (mean 23 months). There were five girls and four boys. Associated findings include nephroblastomatosis in three cases (33%), one of which also had a familial history; undescended testis in two cases; and minor anomalies in two other cases. Surgical treatment consisted of unilateral nephrectomy with contralateral partial nephrectomy or tumorectomy in six cases, nephrectomy with contralateral biopsy only in two cases, and the other patient had bilateral biopsies initially, followed at a later date by partial nephrectomy on one side. All patients received chemotherapy; actinomycin D (AMD) only was used in the oldest case, vincristine and AMD in five cases, to which was added cyclophosphamide in one case and adriamycin in two. Seven patients received radiation therapy. Seven out of the nine patients survived more than 2 years (77%); five are well, off chemotherapy, with no evidence of disease from 4 to 11 years after diagnosis. Two patients suffered from chronic renal failure and one died from complications after renal transplantation more than 19 years after diagnosis. The two patients who died from their disease presented with more advanced tumor. Therefore, the agressiveness of multimodal therapy can be tailored according to stage and histology, and effective chemotherapy allows maximal preservation of renal parenchyma in patients with stage I and II tumors.

  18. Brown-Vialetto-Van Laere syndrome: a rare syndrome in otology.

    PubMed

    Prabhu, H V; Brown, M J K

    2005-06-01

    Brown-Vialetto-Van Laere syndrome, or pontobulbar palsy with deafness, is a rare disorder characterized by bilateral sensorineural deafness and a variety of cranial nerve disorders usually involving the motor components of the lower cranial nerves. Less commonly, spinal motor nerves and upper motor neurons are involved. Familial and sporadic cases have been reported. Based on evidences, this syndrome has been related to autosomal recessive, autosomal dominant and X-linked inheritance. Autoimmune origin has been considered as well. In this paper, we report the case of a 38-year-old female patient who primarily presented with bilateral sensorineural hearing loss and then progressively developed Xth and VIIth cranial nerve paralysis. Brown-Vialetto-Van Laere syndrome was diagnosed with this symptom complex of sensorineural hearing loss and pontobulbar palsy. PMID:15992475

  19. Bilateral discoid medial menisci: a rare phenomenon

    PubMed Central

    Samal, Puspak; Bhagwat, Kishan; Panigrahi, Tapas; Gopinathan, Nirmalraj

    2014-01-01

    Discoid medial meniscus is a relatively rare pathology of the knee joint, with bilateral cases even rarer. Herein, we report the case of a 25-year-old man diagnosed with discoid medial meniscus in the right knee with a horizontal tear. Increased cupping of the medial condyle of the tibia, widening of the medial joint space and the presence of discoid meniscus in the right knee prompted investigation of the asymptomatic left knee with magnetic resonance imaging. The contralateral asymptomatic knee also showed evidence of discoid medial meniscus. The symptomatic knee was successfully treated by arthroscopic partial meniscectomy, with excellent functional outcome. PMID:25273941

  20. Bilateral Ossiculoplasty in 1 Case of Achondroplasia

    PubMed Central

    Jung, Jongyoon; Yang, Chulwon; Lee, Sunkyu

    2013-01-01

    Achondroplasia is the most common skeletal dysplasia and it combines various complications with normal longevity. Hearing disturbance due to otitis media or an ossicular anomaly is one of the most common complications. Conductive hearing loss is suggested as the most common form of hearing loss. Temporal bone and middle ear structures are distorted in achondroplasia because of rotational change of the skull base. Authors experienced a case of an achondroplastic patient with bilateral hearing disturbance. We faced making a potential mistake during the previous operation but a favorable postoperative result occurred. Our experience could be helpful to other clinicians who face achondroplastic patients. PMID:24653923

  1. Bilateral Orbital Metastasis of Prostatic Adenocarcinoma.

    PubMed

    Saadi, Ahmed; Kerkeni, Walid; Bouzouita, Abderrazak; Ayed, Haroun; Gaja, Ali; Cherif, Mohamed; Ben Slama, Riadh; Mnif, Najla; Derouiche, Amine; Chebil, Mohamed

    2016-08-01

    Despite the high incidence of prostate carcinoma, metastases of the uvea are very rare and bilateral localization is even more. We report here the case of a 77-year-old man diagnosed with a metastatic prostate carcinoma. Two months later, he presented a decreased vision in his right eye and blurred vision in the left eye relevant to metastatic lesion on his right iris and left choroidal metastasis. The urologist should evoke possibility of ocular metastasis in patients with prostate cancer presenting visual disorders. PMID:27181244

  2. Bilateral recurrent self-induced keratoconus.

    PubMed

    Koenig, Steven B

    2008-11-01

    The observational case describes bilateral recurrent keratoconus in corneal transplants performed in a patient with self-induced keratoconus secondary to compulsive eye rubbing. Slitlamp findings demonstrated corneal stromal thinning and scarring in the patient's right eye and temporal corneal hydrops in his left eye. Videokeratography of the right eye confirmed the presence of corneal steepening and irregular astigmatism, consistent with the diagnosis of keratoconus involving each transplant. Together with the history of ongoing compulsive eye rubbing, these findings support the concept that chronic mechanical trauma to the cornea may contribute to the development of keratoconus. PMID:18997547

  3. Congenital bilateral eversion of the eyelids.

    PubMed

    Isawumi, Michaeline A; Adeoti, Caroline O; Umar, Ismaila O; Oluwatimilehin, Ilesanmi O; Raji, Ralph A

    2008-01-01

    The authors report a case of congenital bilateral eversion of the eyelids with secondary conjunctival prolapse in a 4-hour-old neonate. Conservative treatment with hypertonic normal saline, lubricants, antibiotics, and padding was instituted. No surgery was performed. Complete eye opening was achieved by the 10th day after birth. Medical management is advantageous in that it is inexpensive, noninvasive, and can be performed even in a center with poor surgical facilities, as is commonly encountered in developing countries. The sociopsychological aspect of the disease, possible pathophysiology, and treatment are discussed. PMID:19043951

  4. Progressive bilateral thinning of the parietal bones

    SciTech Connect

    Cederlund, C.G.; Andren, L.; Olivecrona, H.

    1982-03-01

    Observation of a case of progressive bilateral parietal thinning within a period of 14 years induced us to study skull films of 3 636 consecutive patients. Parietal thinning was found in 86 patients (2.37%). It was more common in women, with a sex ratio of 1:1.9. The mean age of the females was 72 years, and that of the males 63 years. Previous skull films of 25 of these patients were available and showed progression in 10. It is concluded that parietal thinning is a slowly progressive disease of middle-aged and old patients and is not an anatomical variant or congenital dysplasia of the dipole.

  5. Bilateral internuclear ophthalmoplegia following mild head injury.

    PubMed

    Muthukumar, N; Veerarajkumar, N; Madeswaran, K

    2001-05-01

    A 7-year-old child presented with bilateral internuclear ophthalmoplegia (INO) following a trivial head injury. CT was normal. MRI revealed a pontine lesion. Two months after the injury the patient was neurologically normal. INO following head injury is rare. Rarer still is INO following mild head injury. To date, only four cases of INO had been reported following mild head injury; the present case is the fifth and the first in which the lesion was documented using MRI. The relevant literature is reviewed. PMID:11417420

  6. Bilateral sequential Propionibacterium acnes exogenous endophthalmitis.

    PubMed

    Saffra, Norman; Moriarty, Emily; Milman, Tatyana

    2016-12-01

    A 68-year-old man underwent uncomplicated sequential cataract extractions performed more than a year apart. He presented 6 months after the second surgery with persistent intraocular inflammation in both eyes. Cultures from both eyes grew Propionibacterium acnes and he responded well to treatment. Suspicion for delayed-onset post-operative endophthalmitis must remain high in uveitis cases that fail to resolve with anti-inflammatory treatments. The authors believe this is the first reported case of bilateral sequential P. acnes exogenous endophthalmitis. PMID:27220771

  7. Bilateral carotid aneurysms unmasked by severe hypopituitarism.

    PubMed Central

    Michils, A.; Balériaux, D.; Mockel, J.

    1991-01-01

    We describe a patient who initially presented with severe hyponatraemia and grand mal seizures, without any focal neurological symptoms. The final diagnosis was that of giant bilateral carotid aneurysms extending into the sella turcica with anterior hypopituitarism. To the best of our knowledge, this is the first case report of symmetrical carotid aneurysms manifested exclusively by an acute endocrine emergency with none of the concomitant usual focal signs such as headache, failing vision, oculomotor palsy or subarachnoid hemorrhage. Images p286-a Figure 1 PMID:2062778

  8. Prenatal diagnosis of bilateral ectrodactyly and radial agenesis associated with trisomy 10 mosaicism.

    PubMed

    Lévy, Jonathan; Jouannic, Jean-Marie; Saada, Julien; Dhombres, Ferdinand; Siffroi, Jean-Pierre; Portnoï, Marie-France

    2013-01-01

    Ectrodactyly or split hand and foot malformations (SHFMs) are rare malformations of the limbs, characterized by median clefts of the hands and feet, syndactyly, and aplasia and/or hypoplasia of the phalanges. They represent a clinically and genetically heterogeneous disorder, with both sporadic and familial cases. Most of the genomic rearrangements identified to date in some forms of SHFM are autosomal dominant traits, involving various chromosome regions. Bilateral radial ray defects comprise also a large heterogenous group of disorders, including trisomy 18, Fanconi anemia, and thrombocytopenia-absent-radius syndrome, not commonly associated with ectrodactyly. The present paper describes a case of ectrodactyly associated with bilateral radial ray defects, diagnosed in the first trimester of pregnancy, in a fetus affected by trisomy 10. Only four cases of sporadic and isolated ectrodactyly, diagnosed by ultrasonography between 14 and 22 weeks' gestation, have been reported. To our knowledge, the present case is the first report of mosaic trisomy 10 associated with SHFM and radial aplasia. Trisomy 10 is a rare lethal chromosomal abnormality, most frequently found in abortion products. Only six liveborn mosaic trisomy 10 infants, with severe malformations, dead in early infancy, have been reported. A severe clinical syndrome can be defined, comprising ear abnormalities, cleft lip/palate, malformations of eyes, heart, and kidneys, and deformity of hands and feet and most often associated with death neonatally or in early infancy. PMID:23401811

  9. A case of bilateral aldosterone-producing adenomas differentiated by segmental adrenal venous sampling for bilateral adrenal sparing surgery

    PubMed Central

    Morimoto, R; Satani, N; Iwakura, Y; Ono, Y; Kudo, M; Nezu, M; Omata, K; Tezuka, Y; Seiji, K; Ota, H; Kawasaki, Y; Ishidoya, S; Nakamura, Y; Arai, Y; Takase, K; Sasano, H; Ito, S; Satoh, F

    2016-01-01

    Primary aldosteronism due to unilateral aldosterone-producing adenoma (APA) is a surgically curable form of hypertension. Bilateral APA can also be surgically curable in theory but few successful cases can be found in the literature. It has been reported that even using successful adrenal venous sampling (AVS) via bilateral adrenal central veins, it is extremely difficult to differentiate bilateral APA from bilateral idiopathic hyperaldosteronism (IHA) harbouring computed tomography (CT)-detectable bilateral adrenocortical nodules. We report a case of bilateral APA diagnosed by segmental AVS (S-AVS) and blood sampling via intra-adrenal first-degree tributary veins to localize the sites of intra-adrenal hormone production. A 36-year-old man with marked long-standing hypertension was referred to us with a clinical diagnosis of bilateral APA. He had typical clinical and laboratory profiles of marked hypertension, hypokalaemia, elevated plasma aldosterone concentration (PAC) of 45.1 ng dl−1 and aldosterone renin activity ratio of 90.2 (ng dl−1 per ng ml−1 h−1), which was still high after 50 mg-captopril loading. CT revealed bilateral adrenocortical tumours of 10 and 12 mm in diameter on the right and left sides, respectively. S-AVS confirmed excess aldosterone secretion from a tumour segment vein and suppressed secretion from a non-tumour segment vein bilaterally, leading to the diagnosis of bilateral APA. The patient underwent simultaneous bilateral sparing adrenalectomy. Histopathological analysis of the resected adrenals together with decreased blood pressure and PAC of 5.2 ng dl−1 confirmed the removal of bilateral APA. S-AVS was reliable to differentiate bilateral APA from IHA by direct evaluation of intra-adrenal hormone production. PMID:26538381

  10. A case of bilateral aldosterone-producing adenomas differentiated by segmental adrenal venous sampling for bilateral adrenal sparing surgery.

    PubMed

    Morimoto, R; Satani, N; Iwakura, Y; Ono, Y; Kudo, M; Nezu, M; Omata, K; Tezuka, Y; Seiji, K; Ota, H; Kawasaki, Y; Ishidoya, S; Nakamura, Y; Arai, Y; Takase, K; Sasano, H; Ito, S; Satoh, F

    2016-06-01

    Primary aldosteronism due to unilateral aldosterone-producing adenoma (APA) is a surgically curable form of hypertension. Bilateral APA can also be surgically curable in theory but few successful cases can be found in the literature. It has been reported that even using successful adrenal venous sampling (AVS) via bilateral adrenal central veins, it is extremely difficult to differentiate bilateral APA from bilateral idiopathic hyperaldosteronism (IHA) harbouring computed tomography (CT)-detectable bilateral adrenocortical nodules. We report a case of bilateral APA diagnosed by segmental AVS (S-AVS) and blood sampling via intra-adrenal first-degree tributary veins to localize the sites of intra-adrenal hormone production. A 36-year-old man with marked long-standing hypertension was referred to us with a clinical diagnosis of bilateral APA. He had typical clinical and laboratory profiles of marked hypertension, hypokalaemia, elevated plasma aldosterone concentration (PAC) of 45.1 ng dl(-1) and aldosterone renin activity ratio of 90.2 (ng dl(-1) per ng ml(-1 )h(-1)), which was still high after 50 mg-captopril loading. CT revealed bilateral adrenocortical tumours of 10 and 12 mm in diameter on the right and left sides, respectively. S-AVS confirmed excess aldosterone secretion from a tumour segment vein and suppressed secretion from a non-tumour segment vein bilaterally, leading to the diagnosis of bilateral APA. The patient underwent simultaneous bilateral sparing adrenalectomy. Histopathological analysis of the resected adrenals together with decreased blood pressure and PAC of 5.2 ng dl(-1) confirmed the removal of bilateral APA. S-AVS was reliable to differentiate bilateral APA from IHA by direct evaluation of intra-adrenal hormone production. PMID:26538381

  11. Bilateral talus fracture dislocation: is avascular necrosis inevitable?

    PubMed Central

    Balaji G, Gopisankar; Arockiaraj, Justin

    2014-01-01

    Bilateral simultaneous fracture dislocation of the talus is a very rare injury. It occurs usually following high-velocity injuries. We report a 45-year-old man who presented with bilateral talar fracture dislocation following a heavy stone falling on him. On his right side, he had open talus dislocation with lateral process fracture and on his left side he had closed talar neck fracture dislocation. He underwent immediate debridement and external fixator application for his right side and open reduction and internal fixation for his left talus. At the end of 34 months’ follow-up, he was able to walk up to 2 km without pain. His ankle range of movements were restricted bilaterally. Radiographs revealed bilateral avascular necrosis with right side showing complete collapse. We present this case for its rare presentation of bilateral talar fracture dislocation with bilateral avascular necrosis with sequential radiographs. PMID:25155493

  12. Noninvasive testing of asymptomatic bilateral hilar adenopathy

    SciTech Connect

    Carr, P.L.; Singer, D.E.; Goldenheim, P.; Bernardo, J.; Mulley, A.G. )

    1990-03-01

    The diagnostic strategy for asymptomatic patients with persistent bilateral bilar adenopathy often involves invasive procedures. The authors used Bayesian analysis to: (1) estimate the relative prevalences of diseases causing bilateral bilar adenopathy; (2) assess changes in the prevalence of disease by race, the presence of other clinical symptoms, and geography; and (3) determine the value of relevant noninvasive tests, including the angiotensin-converting enzyme (ACE) assay, gallium scan, and purified protein derivative (PPD), in order to assess when a strategy of watchful waiting is appropriate. The analysis indicated that the ACE assay, particularly when paired with the PPD, can identify many patients who might safely be managed without immediate invasive biopsy. Patients who are ACE+ and PPD- have an estimated probability of sarcoidosis of 0.95 or greater; patients who are ACE- and PPD+ have a probability of tuberculosis of 0.86 if black, 0.79 if white. In contrast, gallium scanning has no diagnostic role in this clinical situation. Bronchoscopic or mediastinoscopic biopsy has a limited role for patients who are ACE+ PPD- or ACE- PPD+ because of limited sensitivity. Patients who are both ACE- and PPD-, particularly if white, may have a high enough risk of lymphoma to consider invasive biopsy.

  13. Influence of unilateral weight on bilateral cyclograms

    NASA Astrophysics Data System (ADS)

    Pellicer Costa, Juan José; Dusza, Jacek J.

    2014-11-01

    The paper presents the results of gait parameters as a function of unilateral weight. The object of the research was a woman walking on a stationary surface and carrying in his hand weights from 0 to 15 kg. Her movement was recorded by 6 cameras recording the location of 34 markers placed at appropriate points in the body. 3D reconstruction was performed for each of the reflecting markers. Tested signals were changes in the value the joint angles of ankle, knee and hip. On the basis of about 6 cycles of movement of each load, a model for the average gait cycle was developed. The result of the experiments are graphs of changes the joint angles as a function of time, bilateral cyclograms, synchronized bilateral cyclograms and regression lines. The conclusion of the study is to determine how one-sided load affects gait asymmetry. Simple and easy to interpret method of presentation of results were also shown. Studies were conducted using VICON system.

  14. Bilateral prophylactic mastectomy: issues and concerns.

    PubMed

    Stefanek, M E

    1995-01-01

    At present, the care of women at increased risk of developing breast cancer poses a clinical dilemma and remains an area of controversy. A number of investigators have addressed the pros and cons of prophylactic mastectomy versus close follow-up, utilizing annual mammography, semiannual or even more frequent physical examinations of the breast, and proficient monthly breast self-examinations. Recent efforts to isolate a gene (BRCA1) on chromosome 17q12-21 raise additional concerns about the management of women testing positive for BRCA1 mutations. These women are estimated to have an 85% lifetime risk of developing breast cancer. Testing for BRCA1 mutation carriers may soon be available for population screening. This article describes preliminary studies investigating health care provider and patient perceptions of bilateral prophylactic mastectomy. In addition, a number of research questions remain regarding the efficacy and utilization of bilateral prophylactic mastectomy as a treatment option for women at increased risk of developing breast cancer. These women include those testing positive for BRCA1 mutations. In addition, women with a strong family history opting against testing for BRCA1 mutations may express interest in surgery. PMID:8573451

  15. Atypical Bilateral Fuchs Uveitis: Diagnostic Challenges

    PubMed Central

    Couto, Cristóbal; Hurtado, Erika; Faingold, Dana; Demetrio, Carmen; Schlaen, Ariel; Zas, Marcelo; Zarate, Jorge; Rosetti, Silvia; de Lima, Andrea Paes; Croxatto, Juan Oscar; Chiaradía, Pablo; Burnier, Miguel N.

    2015-01-01

    Bilateral Fuchs uveitis associated with vitreous infiltration and posterior segment involvement requires a thorough diagnostic evaluation. The lack of well-defined diagnostic criteria makes identification of this entity difficult. The aim of this case report was to present the characteristics of a patient with atypical Fuchs uveitis and the procedures needed to rule out the differential diagnosis with specific attention to the utility of in vivo confocal microscopy (IVCM). Case Report One case of chronic bilateral uveitis with severe vitreous opacities is presented. After extensive systemic workup, including vitrectomy, the case had no identifiable systemic etiology. IVCM of the cornea revealed the presence of dendritiform keratic precipitates. Conclusion The diagnosis of Fuchs uveitis is based on clinical findings as no confirmatory laboratory tests are available. A high index of suspicion is key to an early diagnosis, especially in the cases with vitreous opacities and posterior segment manifestations. Auxiliary tests such as IVCM may aid the clinician in the diagnosis of Fuchs uveitis. PMID:26483668

  16. Bilateral Comparison in Chemosensory-Mediated Foraging

    NASA Astrophysics Data System (ADS)

    Webster, D. R.; Rahman, S.; Dasi, L. P.

    2000-11-01

    Motivation is drawn from the need to determine the sensory cues that animals such as blue crabs and lobsters use to track chemical odor plumes to locate food or mates. Major steps forward with this difficult problem can only be achieved through an appreciation of the spatial and temporal variation of concentration fields and the information content available to a forager in the plume. Here we discuss the usefulness of bilateral comparison to an animal tracking a turbulent plume. Instantaneous concentration fields of a chemical plume diffusing in a fully-developed turbulent open channel flow are measured using planar laser-induced fluorescence (PLIF). The plume is released iso-kinetically 25 mm above the smooth bed (z+ = 90), thus transport is mainly due to advection and ambient turbulence. A spatial correlation function in the spanwise direction is a dramatic indicator of the relative position of the centerline and distance from the source. The relative direction of the plume centerline can be estimated from an instantaneous bilateral comparison provided the sensors are separated by a distance that is relatively large compared to the spanwise integral length scale based on the spatial correlation function.

  17. Hiatus Hernia Repair with Bilateral Oesophageal Fixation

    PubMed Central

    Martin, David

    2015-01-01

    Background. Despite advances in surgical repair of hiatus hernias, there remains a high radiological recurrence rate. We performed a novel technique incorporating bilateral oesophageal fixation and evaluated outcomes, principally symptom improvement and hernia recurrence. Methods. A retrospective study was performed on a prospective database of patients undergoing hiatus hernia repair with bilateral oesophageal fixation. Retrospective and prospective quality of life (QOL), PPI usage, and patient satisfaction data were obtained. Hernia recurrence was assessed by either barium swallow or gastroscopy. Results. 87 patients were identified in the database with a minimum of 3 months followup. There were significant improvements in QOL scores including GERD HRQL (29.13 to 4.38, P < 0.01), Visick (3 to 1), and RSI (17.45 to 5, P < 0.01). PPI usage decreased from a median of daily to none, and there was high patient satisfaction (94%). 57 patients were assessed for recurrence with either gastroscopy or barium swallow, and one patient had evidence of recurrence on barium swallow at 45 months postoperatively. There was an 8% complication rate and no mortality or oesophageal perforation. Conclusions. This study demonstrates that our technique is both safe and effective in symptom control, and our recurrence investigations demonstrate at least short term durability. PMID:26065030

  18. Bilateral tension pneumothorax following equipment improvisation.

    PubMed

    Zambricki, Christine; Schmidt, Carol; Vos, Karen

    2014-02-01

    This case report describes an unexpected event that took place as a result of using improvised equipment. The patient, a 16-year-old female undergoing complex oral surgery, suffered bilateral pneumothorax following the improper use of an airway support device. During the immediate postoperative period with the patient still intubated, oxygen tubing was attached to a right angle elbow connector with the port closed and 10 L/minute oxygen flow was administered to the patient in a manner that did not allow the patient to exhale. Within seconds, pneumothorax was apparent as the patient's vital signs deteriorated, visible swelling was noted in the shoulders and neck, and there was an absence of breath sounds on auscultation. This case study has application beyond the immediate discussion of bilateral pneumothorax, serving as a caution about the unintended consequences of equipment improvisation. In addition to highlighting the hazards of providing patient care with a non-standard device, this study also provides a powerful example of the human factors that can contribute to medical errors in the healthcare setting. PMID:24654348

  19. Unusual Bilateral Rim Fracture in Femoroacetabular Impingement

    PubMed Central

    Rafols, Claudio; Monckeberg, Juan Edo; Numair, Jorge

    2015-01-01

    This is a report of one case of bilateral acetabular rim fracture in association with femoroacetabular impingement (FAI), which was treated with a hip arthroscopic procedure, performing a partial resection, a labral reinsertion, and a subsequential internal fixation with cannulated screws. Up to date, there are in the literature only two reports of rim fracture and “os acetabuli” in association with FAI. In the case we present, the pincer and cam resection were performed without complications; the technique used was published previously. With this technique the head of the screw lays hidden by the reattached labrum. We removed partially the fractured rim fragment and the internal fixation of the remaining portion was achieved with a screw. In the event of a complete resection of the fragment, it would have ended with a LCE angle of 18° and a high probability of hip instability. We believe that this bilateral case helps establish the efficacy and reproducibility of the technique described by Larson. PMID:25722907

  20. Bilateral optic nerve head drusen with chorioretinal coloboma in the right eye.

    PubMed

    Dehghani, Ali Reza; Rezaei, Leila; Ghanbari, Heshmatollah

    2016-01-01

    Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic nerve head drusen (ONHD) are white calcareous deposits that are generally asymptomatic. We report a very rare association of both in a healthy patient with no any systemic syndrome. A 16-year-old man was referred to our clinic from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. External ocular and slit lamp examination were normal. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly. Ultrasonography showed an echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and moderate superior field depression in the right eye corresponding to inferior coloboma in funduscopy. Results of general medical and neurologic, cardiologic, and other examinations were normal. To the best our knowledge combination of bilateral ONHD and unilateral chorioretinal coloboma in a healthy patient with no any systemic syndrome has not been published in the literature. We reported this very rare association and recommended examine eyes and other body organs. In such cases that coloboma is associated with ONHD, we should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by the consultant pediatricians and clinical geneticists. PMID:27099847

  1. Bilateral optic nerve head drusen with chorioretinal coloboma in the right eye

    PubMed Central

    Dehghani, Ali Reza; Rezaei, Leila; Ghanbari, Heshmatollah

    2016-01-01

    Chorioretinal coloboma is a congenital defect of the eye caused by improper closure of the embryonic fissure. Optic nerve head drusen (ONHD) are white calcareous deposits that are generally asymptomatic. We report a very rare association of both in a healthy patient with no any systemic syndrome. A 16-year-old man was referred to our clinic from suffering blurred vision. Best corrected visual acuity of the right eye was 6/10 and 10/10 in the left one. External ocular and slit lamp examination were normal. Dilated ophthalmoscopy showed marked swelling in both optic nerves and chorioretinal coloboma in the right eye inferiorly. Ultrasonography showed an echodense structure with acoustic shadowing in both eyes consistent with buried ONHD. Visual field testing showed normal field in the left eye and moderate superior field depression in the right eye corresponding to inferior coloboma in funduscopy. Results of general medical and neurologic, cardiologic, and other examinations were normal. To the best our knowledge combination of bilateral ONHD and unilateral chorioretinal coloboma in a healthy patient with no any systemic syndrome has not been published in the literature. We reported this very rare association and recommended examine eyes and other body organs. In such cases that coloboma is associated with ONHD, we should keep in mind Noonan syndrome. The diagnosis of Noonan syndrome is clinical and confirm by the consultant pediatricians and clinical geneticists. PMID:27099847

  2. Bilateral luxatio erecta with greater tuberosity fracture: a case report

    PubMed Central

    Saxena, Vikas; Pradhan, Pavan

    2013-01-01

    Bilateral shoulder dislocation with greater tuberosity fracture and luxatio erecta, both are rare by themselves, with only few reports of each. We report an unusual case of posttraumatic bilateral symmetrical shoulder dislocation involving luxatio erecta with greater tuberosity fracture in a young male. To our knowledge, this is the first case of symmetrical bilateral shoulder dislocation with greater tuberosity fracture involving luxatio erecta dislocation from Indian subcontinent. PMID:26403880

  3. Simultaneous bilateral spontaneous pneumothoraces: a case of occupational asthma

    PubMed Central

    Khan, Waseem Asrar; Curl-Roper, Thomas; Helm, Jenifer; Houghton, Catherine

    2013-01-01

    Simultaneous bilateral spontaneous pneumothoraces (SBSP) are uncommon. This report presents the case of a previously well 19-year-old man with a diagnosis of SBSP and symptoms suggestive of occupational asthma. Despite bilateral bullectomy and pleurodesis using a video-assisted thoracoscopic surgical technique, the pneumothorax reoccurred unilaterally and open surgery was performed. This case illustrates a rare condition of bilateral pneumothoraces presenting as a first presentation of occupational asthma and the issues surrounding its management. PMID:24027249

  4. Simultaneous bilateral spontaneous pneumothoraces: a case of occupational asthma.

    PubMed

    Khan, Waseem Asrar; Curl-Roper, Thomas; Helm, Jenifer; Houghton, Catherine

    2013-01-01

    Simultaneous bilateral spontaneous pneumothoraces (SBSP) are uncommon. This report presents the case of a previously well 19-year-old man with a diagnosis of SBSP and symptoms suggestive of occupational asthma. Despite bilateral bullectomy and pleurodesis using a video-assisted thoracoscopic surgical technique, the pneumothorax reoccurred unilaterally and open surgery was performed. This case illustrates a rare condition of bilateral pneumothoraces presenting as a first presentation of occupational asthma and the issues surrounding its management. PMID:24027249

  5. Successful Reconstruction of Asymptomatic Bilateral External Carotid Artery Aneurysms.

    PubMed

    Loja, Melissa N; Pevec, William C

    2016-04-01

    True aneurysms of the external carotid artery (ECA) are extremely rare with an unknown incidence and natural history. We present the successful operative management of an asymptomatic 65-year-old man found to have bilateral internal carotid artery stenosis and bilateral ECA aneurysms. His bilateral carotid arteries were reconstructed with bifurcated interposition grafts in a staged fashion. The patient recovered without sequelae and continues to be asymptomatic 1 year after reconstruction. We present the operative management of this rare case. PMID:26802292

  6. Spermatozoa protein alterations in infertile men with bilateral varicocele

    PubMed Central

    Agarwal, Ashok; Sharma, Rakesh; Durairajanayagam, Damayanthi; Cui, Zhihong; Ayaz, Ahmet; Gupta, Sajal; Willard, Belinda; Gopalan, Banu; Sabanegh, Edmund

    2016-01-01

    Among infertile men, a diagnosis of unilateral varicocele is made in 90% of varicocele cases and bilateral in the remaining varicocele cases. However, there are reports of under-diagnosis of bilateral varicocele among infertile men and that its prevalence is greater than 10%. In this prospective study, we aimed to examine the differentially expressed proteins (DEP) extracted from spermatozoa cells of patients with bilateral varicocele and fertile donors. Subjects consisted of 17 men diagnosed with bilateral varicocele and 10 proven fertile men as healthy controls. Using the LTQ-orbitrap elite hybrid mass spectrometry system, proteomic analysis was done on pooled samples from 3 patients with bilateral varicocele and 5 fertile men. From these samples, 73 DEP were identified of which 58 proteins were differentially expressed, with 7 proteins unique to the bilateral varicocele group and 8 proteins to the fertile control group. Majority of the DEPs were observed to be associated with metabolic processes, stress responses, oxidoreductase activity, enzyme regulation, and immune system processes. Seven DEP were involved in sperm function such as capacitation, motility, and sperm-zona binding. Proteins TEKT3 and TCP11 were validated by Western blot analysis and may serve as potential biomarkers for bilateral varicocele. In this study, we have demonstrated for the first time the presence of DEP and identified proteins with distinct reproductive functions which are altered in infertile men with bilateral varicocele. Functional proteomic profiling provides insight into the mechanistic implications of bilateral varicocele-associated male infertility. PMID:25999357

  7. Proximal femoral focal deficiency as a manifestation of Antley-Bixler syndrome: a case report.

    PubMed

    Sulaiman, A R; Nawaz, H; Munajat, I; Sallehudin, A Y

    2007-04-01

    We report a case of the Antley-Bixler syndrome in an 11-year-old girl. She presented with bilateral proximal femoral focal deficiency, right clubfoot, left radiohumeral synostosis, bilateral ear hypoplasia, cleft palate, tongue tie, missing teeth, congenital heart disease, a pelvic kidney with hydronephrosis, and mental retardation. Proximal femoral focal deficiency has never been reported before as a manifestation of Antley-Bixler syndrome. Her mother was exposed to radiation during an intravenous urogram done in the first trimester of pregnancy. Exposure to radiation has not been implicated as a cause of Antley-Bixler syndrome. PMID:17429125

  8. Meckel-Gruber syndrome: A rare and lethal anomaly

    PubMed Central

    Kheir, Abdelmoneim E. M.; Imam, Abdelmutalab; Omer, Ilham M.; Hassan, Ibtsama M.A.; Elamin, Sara A.; Awadalla, Esra A.; Gadalla, Mohammed H.; Hamdoon, Tagwa A.

    2012-01-01

    Meckel-Gruber syndrome is a rare and lethal autosomal recessive disorder characterized by occipital encephalocele, postaxial polydactyly and bilateral dysplastic cystic kidneys. It can be associated with many other conditions. Antenatal ultrasound examination establishes the diagnosis by identifying at least two of the major features described. We describe a female baby who had the typical triad of Meckel-Gruber syndrome and died shortly after birth.

  9. Cushing's syndrome associated with recurrent endometrioid adenocarcinoma of the ovary.

    PubMed Central

    Crawford, S M; Pyrah, R D; Ismail, S M

    1994-01-01

    Ectopic production of adrenocorticotrophic hormone (ACTH) by malignant neoplasms is a well recognised cause of Cushing's syndrome but is extremely rare in ovarian carcinoma. A patient who underwent surgery for ovarian carcinoma followed by a course of chemotherapy is reported. The tumour was a bilateral moderately differentiated endometrioid adenocarcinoma and contained numerous chromogranin immunoreactive endocrine cells as well as small foci of ACTH immunoreactivity. She subsequently presented with Cushing's syndrome in association with extensive pelvic recurrence of the tumour. Images PMID:7962637

  10. Retinal detachment in the Morning Glory syndrome. Pathogenesis and management.

    PubMed

    von Fricken, M A; Dhungel, R

    1984-01-01

    A case of bilateral Morning Glory syndrome is presented, unusual because of a rhegmatogenous posterior pole retinal detachment in the left eye. Posterior pole retinal detachments have been described in association with this syndrome. This case report is the first presentation of a retinal tear located within the excavation surrounding the optic nerve. A surgical management approach is presented wherein vitrectomy and internal gas tamponade are utilized to reattach the retina. PMID:6463401

  11. Carisoprodol withdrawal syndrome resembling neuroleptic malignant syndrome: Diagnostic dilemma

    PubMed Central

    Paul, Gunchan; Parshotam, Gautam L; Garg, Rajneesh

    2016-01-01

    Soma (Carisoprodol) is N-isopropyl-2 methyl-2-propyl-1,3-propanediol dicarbamate; a commonly prescribed, centrally acting skeletal muscle relaxant. Neuroleptic malignant syndrome (NMS) is a potentially life-threatening adverse effect of antipsychotic agents. Although diagnostic criteria for NMS have been established, it should be recognized that atypical presentations occur and more flexible diagnostic criteria than currently mandated, may be warranted. We wish to report a postoperative case of bilateral knee replacement who presented with carisoprodol (Soma) withdrawal resembling NMS that was a diagnostic dilemma. Subsequently, it was successfully treated with oral baclofen in absence of sodium dantrolene.

  12. Becker's Nevus Syndrome

    PubMed Central

    Dasegowda, Sathyanarayana B; Basavaraj, GB; Nischal, KC; Swaroop, MR; Umashankar, NP; Swamy, Suchetha S

    2014-01-01

    Becker's nevus is a cutaneous hamartoma characterized by circumscribed hyperpigmentation with hypertrichosis. Becker's nevus syndrome is an association of Becker's nevus with unilateral breast hypoplasia and muscle, skin, and/or skeletal abnormalities. We here report a case of a 15 year-old female who presented with bilateral Becker's nevus over her groins, thighs, vulva, and in front of the neck from the age of 5 years. She had associated mental retardation, delayed development of mile stones, delayed puberty, dwarfism, depressed nasal bridge, long slender digits, crowding of lateral toes, valgus deformity of first metatarsophalangeal joint, mitral valve prolapse, muddy conjunctiva with hypertrophic and hyperpigmented caruncle of both eyes, ichthyosis, brownish hair, and absence of axillary and pubic hair. On histopathological examination collagen hamartoma underneath the Becker's nevus was found. PMID:25071279

  13. Intracranial Volume in 15 Children with Bilateral Coronal Craniosynostosis

    PubMed Central

    Maltese, Giovanni; Wikberg, Emma; Bernhardt, Peter; Kölby, Lars; Tarnow, Peter E. W.

    2014-01-01

    Background: Intracranial volume (ICV) growth in patients with bilateral coronal craniosynostosis (BCS) is not well described. It is therefore important to evaluate the consequences of cranial surgery in children with this condition. The aim of the present study was to evaluate ICVs in patients operated on for BCS. Methods: A consecutive series of patients with BCS were operated on using spring-assisted cranioplasty, with computed tomography scans in 0.6-mm slices, were included. A MATLAB-based computer program capable of measuring ICV was used. Patients were compared with an age- and gender-matched control group of healthy children. Student’s t test was used for statistical analysis. Results: Fifteen patients (7 girls and 8 boys) with 43 computed tomography scans were identified. The diagnoses were 13 syndromic BCS (3 Apert, 1 Crouzon, 6 Muenke, and 3 Saethre-Chotzen) and 2 nonsyndromic BCS. The mean preoperative volume at the age of 5 months (n = 15) was 887 mL (range, 687–1082). Mean volume at follow-up at the age of 3 years (n = 13) was 1369 mL (range, 1196–1616). In comparison, the mean ICVs for controls at the ages of 5 months (n = 30) and 3 years (n = 26) were 854 mL and 1358 mL, respectively. The differences were not statistically significant (P > 0.05). Conclusions: Patients with BCS were operated on with spring-assisted cranioplasty seem to maintain their age-related ICV at 3 years of age when compared to normal children. PMID:25506526

  14. Hypothenar hammer syndrome followed by systemic sclerosis.

    PubMed

    Lambert, M; Hatron, P Y; Hachulla, E; Devulder, B

    2000-10-01

    We describe the first case of bilateral hypothenar hammer syndrome (HHS) followed by systemic sclerosis (SSc) that was associated with silica exposure (Erasmus syndrome). The patient was a woman smoothing tiles in an earthenware factory who presented with bilateral digital ischemia associated with Raynaud's phenomenon. HHS was diagnosed, based on an angiographic study showing aneurysm of the ulnar arteries and occlusions of multiple digital arteries. Pulmonary silicosis was also diagnosed on pulmonary tomodensitometry. Two years later digital swelling with acroosteolysis developed. The FANA test was positive (titer 1:640) and anticentromere antibody tests were also positive. Esophageal manometry showed dysmotility of the lower esophagus. These findings were consistent with a diagnosis of SSc. PMID:11036855

  15. Bilateral Trade Flows and Income Distribution Similarity

    PubMed Central

    2016-01-01

    Current models of bilateral trade neglect the effects of income distribution. This paper addresses the issue by accounting for non-homothetic consumer preferences and hence investigating the role of income distribution in the context of the gravity model of trade. A theoretically justified gravity model is estimated for disaggregated trade data (Dollar volume is used as dependent variable) using a sample of 104 exporters and 108 importers for 1980–2003 to achieve two main goals. We define and calculate new measures of income distribution similarity and empirically confirm that greater similarity of income distribution between countries implies more trade. Using distribution-based measures as a proxy for demand similarities in gravity models, we find consistent and robust support for the hypothesis that countries with more similar income-distributions trade more with each other. The hypothesis is also confirmed at disaggregated level for differentiated product categories. PMID:27137462

  16. Bilateral Trade Flows and Income Distribution Similarity.

    PubMed

    Martínez-Zarzoso, Inmaculada; Vollmer, Sebastian

    2016-01-01

    Current models of bilateral trade neglect the effects of income distribution. This paper addresses the issue by accounting for non-homothetic consumer preferences and hence investigating the role of income distribution in the context of the gravity model of trade. A theoretically justified gravity model is estimated for disaggregated trade data (Dollar volume is used as dependent variable) using a sample of 104 exporters and 108 importers for 1980-2003 to achieve two main goals. We define and calculate new measures of income distribution similarity and empirically confirm that greater similarity of income distribution between countries implies more trade. Using distribution-based measures as a proxy for demand similarities in gravity models, we find consistent and robust support for the hypothesis that countries with more similar income-distributions trade more with each other. The hypothesis is also confirmed at disaggregated level for differentiated product categories. PMID:27137462

  17. Shared control in bilateral telerobotic systems

    NASA Astrophysics Data System (ADS)

    Bicker, Robert; Ow, Sing M.

    1995-12-01

    The paper describes the implementation of shared control in a force reflecting telerobotic system which has been carried out during the EC TELEMAN project INGRID. The experimental facility which has been developed at Newcastle comprises a Puma 762 robot which can be manually teleoperated by a Puma 260 robot, functioning as a generalized bilateral (or force reflecting) controller. The slave robot has been configured to support several autonomous force controlled tasks, which have been developed specifically for repair and maintenance operations in nuclear and/or other hazardous environments. The control architecture is based on a network of parallel processors, and the man-machine interface provides `soft-switching' of any axis to facilitate mixed mode and shared control which can be configured in both teleoperator and task based modes. A graphic display is also included to provide a visual indication of the magnitude of forces/torques exerted at the tool/environment interface.

  18. Bilateral stony lung: pulmonary alveolar microlithiasis.

    PubMed

    Chandra, Subhash; Mohan, Anant; Guleria, Randeep; Das, Prasenjit; Sarkar, Chitra

    2009-01-01

    A 40-year-old male paddy field worker was referred for exertional shortness of breath and non-productive cough for 4 years. He had been treated for pulmonary tuberculosis twice. Chest radiograph showed extensive bilateral nodular opacities ("sandstorm-like") in the middle and lower lobe. Pulmonary function tests revealed a restrictive ventilatory defect. High resolution CT showed widespread nodular infiltration with "crazy paving" appearance and interrupted black pleura sign. This was confirmed as pulmonary alveolar microlithiasis (PAM) by trans-bronchial lung biopsy, which showed normal respiratory lining epithelium with dilated alveolar spaces containing many calcific bodies, some of which showed concentric calcification. The possibilities of silicosis (due to exposure to husk) and tuberculosis, both of which can mimic PAM clinically and radiologically, made this case a diagnostic challenge. PMID:21686505

  19. Bilateral stony lung: pulmonary alveolar microlithiasis

    PubMed Central

    Chandra, Subhash; Mohan, Anant; Guleria, Randeep; Das, Prasenjit; Sarkar, Chitra

    2009-01-01

    A 40-year-old male paddy field worker was referred for exertional shortness of breath and non-productive cough for 4 years. He had been treated for pulmonary tuberculosis twice. Chest radiograph showed extensive bilateral nodular opacities (“sandstorm-like”) in the middle and lower lobe. Pulmonary function tests revealed a restrictive ventilatory defect. High resolution CT showed widespread nodular infiltration with “crazy paving” appearance and interrupted black pleura sign. This was confirmed as pulmonary alveolar microlithiasis (PAM) by trans-bronchial lung biopsy, which showed normal respiratory lining epithelium with dilated alveolar spaces containing many calcific bodies, some of which showed concentric calcification. The possibilities of silicosis (due to exposure to husk) and tuberculosis, both of which can mimic PAM clinically and radiologically, made this case a diagnostic challenge. PMID:21686505

  20. Bilateral vision loss associated with radiofrequency exposure

    PubMed Central

    Liu, Dianna; Cruz, Franz Marie; Subramanian, Prem S

    2012-01-01

    A 57-year-old otherwise healthy woman presented with painless binocular vision loss 1 week after direct application of radiofrequency energy to her orbits. She had no light perception bilaterally. Pupils were dilated and not reactive to light. Fundoscopic exam initially showed optic disc swelling in the right eye and a normal-appearing disc in the left eye. Magnetic resonance imaging of the brain and orbits showed gadolinium enhancement of both intraorbital optic nerves. She underwent a course of high-dose steroid treatment without recovery of vision. Optic discs were pale 11 weeks after injury. With exclusion of other possible causes, this represents a unique case of irreversible binocular optic nerve damage and blindness secondary to radiofrequency exposure. PMID:23271888

  1. Bilateral mandibular fracture related to osteoradionecrosis

    PubMed Central

    Goyal, Shikha; Mohanti, Bidhu Kalyan

    2015-01-01

    Mandible is the most frequently affected bone during head and neck irradiation. Late changes in the mandible may manifest in the form of reduced bone density, dental caries, loss of spongiosa trabeculations, delayed healing following dental extraction, pathologic fractures, osteoradionecrosis, trismus, growth defects in children or second malignancies. Pathologic fractures of mandibular bone are rare and may be spontaneous or traumatic (following dental extraction). We report the case of a 55-year lady, who had undergone surgery and adjuvant radiotherapy for carcinoma oral tongue T2N0M0 on a cobalt-60 unit and was disease-free. After a follow-up of 8 years post-irradiation, she presented with sudden onset oral pain and inability to open mouth. Pantomogram showed fracture at the junction of body and ramus of the mandible bilaterally. PMID:26097342

  2. Computed tomography findings in bilateral perinephric lymphangiomatosis.

    PubMed

    Hakeem, Aijaz; Gojwari, Tariq A; Reyaz, Sheikh; Rasool, Shubana; Shafi, Hakim; Mufti, Shahida

    2010-01-01

    Perinephric lymphangioma is rare disorder that may be confused with various forms of renal cystic diseases and urinomas. In this disorder a developmental malformation results in failure of developing lymphatic tissue to establish normal communication with the rest of lymphatic system. Once there is restricted drainage of lymphatic fluid the lymphatic channels dilate to form cystic masses that may be unilocular or multilocular and may be seen unilaterally or bilaterally .This condition presents with various signs and symptoms or can be just an incidental finding which in presence of misleading clinical history may be confused with other diseases. CT scan with delayed cuts and USG guided aspiration with biochemical analysis of fluid will help us in arriving to final diagnosis. PMID:20842254

  3. Subacute bilateral visual loss in methylmalonic acidemia.

    PubMed

    Traber, Ghislaine; Baumgartner, Matthias R; Schwarz, Urs; Pangalu, Athina; Donath, Marc Y; Landau, Klara

    2011-12-01

    A 23-year-old woman known to have methylmalonic acidemia (MMA) since birth suffered bilateral visual loss within 5 days. Multiple sclerosis, Leber hereditary optic neuropathy, vasculitis, infections (in particular treponema), and vitamin deficiency were ruled out. The optic nerve head changed from normal in appearance to atrophic. Treatment attempts with high-dose intravenous steroids and coenzyme Q10 combined with vitamin E were ineffective. The patient's underlying disease was metabolically well controlled by strict diet and carnitine supplementation. Toxic damage of both optic nerves due to MMA is the most likely mechanism. MRI showed moderate enhancement of both optic nerves. To our knowledge, this is the first report of a morphological correlate on MRI. PMID:21873889

  4. [Unilateral to bilateral pleurisy: Pleural tuberculosis?].

    PubMed

    Ben Ameur, S; Smaoui, S; Kamoun, F; Chabchoub, I; Kamoun, T; Messaadi, F; Aloulou, H; Hachicha, M

    2016-04-01

    Pleural tuberculosis is the first or second most common form of extrapulmonary tuberculosis as well as the main cause of pleural effusion in many countries. It is rare in young infants and is more common in children over 10 years of age. We report the case of a 19-month-old girl admitted for prolonged fever with unilateral pleural effusion. The mother reported a history of lymph node tuberculosis 6 years previously. Intravenous antibiotics with cefotaxime and vancomycin were started. Thoracocentesis yielded a serosanguinous exudate fluid with a lymphocyte predominance. The tuberculin skin test and PCR GeneXpert(©) on pleural fluid were negative. The initial outcome was favorable, but the chest X-rays 10 days after discharge showed bilateral pleural effusion. Pleural biopsy was proposed but the culture of pleural fluid was positive for Mycobacterium tuberculosis. The child was put under standard treatment for tuberculosis. The outcome was favorable. PMID:26922570

  5. Bilateral Scapulohumeral Ankylosis after Prolonged Mechanical Ventilation.

    PubMed

    van Lotten, Manon L; Schreinemakers, J Rieneke; van Noort, Arthur; Rademakers, Maarten V

    2016-09-01

    This case demonstrates a rarely reported bilateral scapulohumeral bony ankylosis. A young woman developed extensive heterotopic ossifications (HOs) in both shoulder joints after being mechanically ventilated for several months at the intensive care unit in a comatose status. She presented with a severe movement restriction of both shoulder joints. Surgical resection of the bony bridges was performed in 2 separate sessions with a significant improvement of shoulder function afterwards. No postoperative complications, pain, or recurrence of HOs were noted at 1-year follow-up. Mechanical ventilation, immobilization, neuromuscular blockage, and prolonged sedation are known risk factors for the development of HOs in the shoulder joints. Relatively early surgical resection of the HOs can be performed safely in contrary to earlier belief. Afterwards, nonsteroidal anti-inflammatory drugs and/or radiation therapy can be possible treatment modalities to prevent recurrence of HOs. PMID:27583120

  6. Bilateral Scapulohumeral Ankylosis after Prolonged Mechanical Ventilation

    PubMed Central

    Schreinemakers, J. Rieneke; van Noort, Arthur; Rademakers, Maarten V.

    2016-01-01

    This case demonstrates a rarely reported bilateral scapulohumeral bony ankylosis. A young woman developed extensive heterotopic ossifications (HOs) in both shoulder joints after being mechanically ventilated for several months at the intensive care unit in a comatose status. She presented with a severe movement restriction of both shoulder joints. Surgical resection of the bony bridges was performed in 2 separate sessions with a significant improvement of shoulder function afterwards. No postoperative complications, pain, or recurrence of HOs were noted at 1-year follow-up. Mechanical ventilation, immobilization, neuromuscular blockage, and prolonged sedation are known risk factors for the development of HOs in the shoulder joints. Relatively early surgical resection of the HOs can be performed safely in contrary to earlier belief. Afterwards, nonsteroidal anti-inflammatory drugs and/or radiation therapy can be possible treatment modalities to prevent recurrence of HOs. PMID:27583120

  7. Bilateral balanced articulation: science or dogma?

    PubMed

    Farias-Neto, Arcelino; Carreiro, Adriana da Fonte Porto

    2014-06-01

    For more than a hundred years, it has been supposed that canine guidance should be avoided in conventional complete dentures, since it would result in denture instability. Thus, bilateral balanced articulation has been held by many authors as fundamental for treatment success. However, randomized clinical trials have shown that balanced articulation is not as important as previously thought. The issue about which occlusal concept is the most appropriate for individual needs is clinically and economically relevant for both the dentist and dental technician. Therefore, the purpose of this study is to provide an evidence-based update on complete denture occlusion. Clinical Relevance: The issue about which occlusal concept is the most appropriate for individual needs is clinically and economically relevant. PMID:25073224

  8. Synchronous Bilateral Testicular Tumors with Different Histopathology

    PubMed Central

    Anastasiou, Ioannis; Deligiannis, Dimitrios; Skarmoutsos, Ioannis; Karaolanis, Georgios; Palla, Viktoria-Varvara; Nonni, Afrodite; Mitropoulos, Dionysios; Constantinides, Constantinos A.

    2015-01-01

    A 40-year-old male presented to our outpatient department with the chief complaint of a painless mass on his right testis with gradual size increase over the past two months. Physical examination and ultrasound revealed a firm and nontender mass both on the right and on the left testis. The only elevated biomarker was b-hcG (24,7 mIU/mL) and computer tomography (CT) did not reveal any pathology. Bilateral high orchiectomies were performed, without previous frozen storage of the sperm. Histology proved typical seminoma of the left testis and embryonal carcinoma of the right testis. He received two cycles of adjuvant combination chemotherapy with bleomycin, etoposide, and cisplatin. Six months after the operation no residual tumor or recurrence was observed. PMID:26060594

  9. Bilateral adrenal hemorrhage in polycythemia vera.

    PubMed

    Bhandari, Shruti; Agito, Katrina; Krug, Esther I

    2016-01-01

    Bilateral adrenal hemorrhage (BAH) is a rare complication typically seen in critically ill patients, which can lead to acute adrenal insufficiency and death unless it is recognized promptly and treated appropriately. We describe the case of a 64-year-old man with polycythemia vera found to be unresponsive with fever, hypotension, tachycardia, and hypoglycemia. Electrocardiogram showed ST-elevation with elevated troponin, hemoglobin, prothrombin time, and partial thromboplastin time. He required aggressive ventilator and vasopressor support. Despite primary coronary intervention, he remained hypotensive. Random cortisol level was low. He received stress dose hydrocortisone with immediate hemodynamic stability. BAH was highly suspected and was confirmed by non-contrast abdominal computed tomography. Prompt recognition and timely initiated treatment remain crucial to impact the mortality associated with acute adrenal insufficiency. PMID:27609733

  10. Bilateral pigmented villonodular synovitis of the knee

    PubMed Central

    Shah, Samir H.; Porrino, Jack A.; Green, John R.; Chew, Felix S.

    2015-01-01

    Pigmented villonodular synovitis is a disorder resulting in a villous, nodular, or villonodular proliferation of the synovium, with pigmentation related to the presence of hemosiderin. These lesions are almost exclusively benign with rare reports of malignancy. Pigmented villonodular synovitis can occur in a variety of joints and at any age but most often occurs within the knee in the young adult. Pigmented villonodular synovitis is a rare disease entity, and bilateral synchronous or metachronous involvement of a joint is even more uncommon, with few reports previously described in the literature. We present a case of pigmented villonodular synovitis involving both the right and left knee in the same patient, with radiographic imaging, magnetic resonance imaging, photograph and video intraoperative imaging, and pathologic correlation. PMID:26649121

  11. Bilateral mechanical rotational vertebral artery occlusion.

    PubMed

    Dargon, Phong T; Liang, Conrad W; Kohal, Anmol; Dogan, Aclan; Barnwell, Stanley L; Landry, Gregory J

    2013-10-01

    Rotational vertebral artery occlusion, or bow hunter's stroke, is reversible, positional symptomatic vertebrobasilar ischemia. The typical mechanism of action is obstruction of a dominant vertebral artery with contralateral head rotation in the setting of baseline ipsilateral vertebral artery stenosis or occlusion. Here we present a rare case of mechanical occlusion of bilateral patent vertebral arteries manifesting as near syncope with rightward head rotation. Diagnostic cerebral angiography showed dynamic right C5 vertebral occlusion and left C2 vertebral occlusion. The patient underwent right C4/5 transverse process decompression. Postoperative angiogram showed patent flow through the right vertebral artery in neutral position and with head turn with resultant resolution of symptoms. PMID:23465174

  12. Testicular lipomatosis in Cowden's syndrome.

    PubMed

    Woodhouse, Joe B; Delahunt, Brett; English, Sharon F; Fraser, Hamish H; Ferguson, Martin M

    2005-09-01

    Cowden's syndrome is either familial or sporadic and is associated with the predominantly postpubertal development of a variety of cutaneous, stromal and visceral neoplasms. The syndrome is associated with mutations of the PTEN gene and is closely related to Bannayan's syndrome in which macrocephaly and benign tumors, especially lipomas and hemangiomas are pathognomic. In PTEN knockout mice testicular tumors have been reported and for this reason we felt it prudent to examine the testes of our patients with genetically proven Cowden's syndrome. Seven of eight patients who underwent testicular ultrasound were found to have diffuse bilateral hyperechoic lesions. Four patients consented to testicular biopsy and on histological examination multiple foci of adipocytes were found within the testicular interstitium, with no evidence of dysplasia or preclinical malignancy. Immunohistochemical assessment of adipocytes suggested a stromal derivation without evidence of metaplasia from Leydig cells. In one case there was focal atrophy of seminiferous tubules, while in two others there was nodular periorchitis of the tunica albuginea. Biochemical evaluation of testicular function (luteinizing hormone, follicle-stimulating hormone, testosterone, sex hormone binding globulin and free androgen index), prostate-specific antigen and testicular tumor markers were normal, while seminal fluid analysis showed only minor abnormalities. The high incidence of testicular lipomatosis in our adult subjects suggests this to be an important diagnostic criterion for Cowden's syndrome. PMID:15920539

  13. Idiopathic Bilateral Adrenal Hemorrhage in a 63-Year-Old Male: A Case Report and Review of the Literature

    PubMed Central

    Bodukam, Vijay Kumar; Thakur, Kshitij; Singh, Amandeep; Jenkins, Donald; Bahl, Jaya

    2015-01-01

    Adrenal hemorrhage is a largely uncommon condition typically caused by a number of factors including infection, MI, CHF, anticoagulants, trauma, surgery, and antiphospholipid syndrome. Yet, idiopathic bilateral hemorrhage is rare. The authors present a case of a 63-year-old male who presented with abdominal pain that was eventually diagnosed as bilateral adrenal hemorrhages due to an unknown origin. Abdominal CT revealed normal adrenal glands without enlargement, but an MRI displayed enlargement due to hemorrhage in both adrenals. There was no known cause; the patient had not suffered from an acute infection and was not on anticoagulants, and the patient's history did not reveal any of the other known causative factors. The case underscores the importance of keeping bilateral adrenal hemorrhages on the list of differentials even when a cause is not immediately clear. It also raises the question of whether CT is the most sensitive test in the diagnosis of adrenal hemorrhage and whether the diagnostic approach should place greater weight on MRI. The case highlights the need for prompt therapy with steroids once bilateral hemorrhage is suspected to avert the development or progression of adrenal insufficiency. PMID:25973281

  14. Pseudoaminopterin syndrome.

    PubMed

    Kraoua, Lilia; Capri, Yline; Perrin, Laurence; Benmansour, Abdelmajjid; Verloes, Alain

    2012-09-01

    Pseudoaminopterin syndrome or aminopterin syndrome-like sine aminopterin (ASSA syndrome--OMIM 600325] is a rare autosomal recessive syndrome defined by characteristic dysmorphic features, skeletal defects, limb anomalies, cryptorchidism, and growth retardation. The syndrome owes its name to the fact that patients resemble the children exposed to aminopterin or to methotrexate, two dihydrofolate reductase inhibitors used for chemotherapy, or as an abortificient in early pregnancy. Ten patients have been described with pseudoaminopterin syndrome. Their phenotype is variable, and differs from the phenotype resulting from folic acid deprivation, leading to the notion that the pathogenesis may be more complex than simple vitamin deficiency. We report on an Algerian patient with pseudoaminopterin syndrome, review the previously reported cases and confirm that pseudoaminopterin syndrome does not result from a detectable contiguous gene imbalance as high resolution CGH array was normal in this child. PMID:22811276

  15. Usher Syndrome

    MedlinePlus

    Usher syndrome is an inherited disease that causes serious hearing loss and retinitis pigmentosa, an eye disorder ... hearing and vision. There are three types of Usher syndrome: People with type I are deaf from ...

  16. Morquio syndrome

    MedlinePlus

    ... to have children and who have a family history of Morquio syndrome. Counseling is also recommended for families who have a child with Morquio syndrome to help them understand the condition and possible treatments. Prenatal testing is available.

  17. Asperger syndrome

    MedlinePlus

    ... this page: //medlineplus.gov/ency/article/001549.htm Asperger syndrome To use the sharing features on this page, please enable JavaScript. Asperger syndrome is often considered a high functioning form ...

  18. Piriformis syndrome

    MedlinePlus

    ... sciatica; Hip socket neuropathy; Pelvic outlet syndrome; Low back pain - piriformis References Joseph RL, Alleva JT, Hudgins TH. Piriformis syndrome. In: Frontera: Essentials of Physical Medicine and Rehabilitation . 3rd ed. Philadelphia, PA: Elsevier Saunders; 2014:chap ...

  19. Pendred Syndrome

    MedlinePlus

    ... thyroid gland. Pendred syndrome also can affect the vestibular system, which controls balance. Some people with Pendred syndrome will show vestibular weakness when their balance is tested. However, the ...

  20. Turner Syndrome

    MedlinePlus

    Turner syndrome is a genetic disorder that affects a girl's development. The cause is a missing or ... t work properly. Other physical features typical of Turner syndrome are Short, "webbed" neck with folds of ...

  1. Premenstrual syndrome

    MedlinePlus

    ... syndrome. Cochrane Database Syst Rev. 2009;2:CD001396. Lentz GM. Primary and secondary dysmenorrhea, premenstrual syndrome, and premenstrual dysphoric disorder: etiology, diagnosis, management. In: Lentz GM, Lobo RA, Gershenson DM, Katz VL, eds. ...

  2. Cushing syndrome

    MedlinePlus

    ... Cushing syndrome is called exogenous Cushing syndrome . Prednisone, dexamethasone, and prednisolone are examples of this type of ... Blood cortisol levels Blood sugar Saliva cortisol levels Dexamethasone suppression test 24-hour urine for cortisol and ...

  3. Cushing's Syndrome

    MedlinePlus

    ... Cushing's syndrome, also called hypercortisolism , is a rare endocrine disorder caused by chronic exposure of the body's tissues ... removing the tumor while minimizing the chance of endocrine deficiency or long-term ... for Cushing's Syndrome Clinical Trials ...

  4. Hurler syndrome

    MedlinePlus

    Hurler syndrome is a rare disease of metabolism in which a person cannot break down long chains of sugar molecules called glycosaminoglycans (formerly called mucopolysaccharides). Hurler syndrome belongs to a group of diseases called mucopolysaccharidosis, ...

  5. Bloom's Syndrome

    MedlinePlus

    ... Glycogen Storage Disease, Type 1A Joubert Syndrome Maple Syrup Urine Disease and DLD Mucolipidosis IV (MLIV) Nemaline ... Glycogen Storage Disease, Type 1A Joubert Syndrome Maple Syrup Urine Disease and DLD Mucolipidosis IV (MLIV) Nemaline ...

  6. Angelman Syndrome

    MedlinePlus

    ... causes developmental delay and neurological problems. The physician Harry Angelman first delineated the syndrome in 1965, when ... 202-534-3731 Prader-Willi Syndrome Association 8588 Potter Park Drive Suite 500 Sarasota, FL 34238 national@ ...

  7. Asperger syndrome

    MedlinePlus

    Asperger syndrome is often considered a high functioning form of autism. It can lead to difficulty interacting socially, repeat behaviors, and clumsiness. Asperger syndrome is a part of the larger developmental disorder ...

  8. Rett Syndrome

    MedlinePlus

    Rett syndrome is a rare genetic disease that causes developmental and nervous system problems, mostly in girls. It's related to autism spectrum disorder. Babies with Rett syndrome seem to grow and develop normally at first. ...

  9. Warburg micro syndrome in siblings from India

    PubMed Central

    Sekhon, Prabhjot Kaur; Premalatha, R.; Sabapathy, Sarala

    2016-01-01

    Warburg syndrome is a rare disorder characterized by microcephaly, microcornea, congenital cataract, developmental delay, and hypogonadism. Here, we report two siblings from India who presented with developmental delay, microcornea, microphthalmia, and bilateral congenital cataracts, born to the third-degree consanguineously married couple. Both children had hypoplasia of corpus callosum. In this report, we aim to highlight and compare clinical features of these two cases with previously reported cases. PMID:27195044

  10. Imaging findings of Gorlin-Goltz syndrome

    PubMed Central

    Hajalioghli, Parisa; Ghadirpour, Ali; Ataie-Oskuie, Reza; Kontzialis, Marinos

    2015-01-01

    A 15-year-old girl was referred to a dentist complaining of parageusia, bad taste in the mouth, which started 9 months ago. Panoramic X-ray and non-enhanced computed tomography scan revealed multiple bilateral unilocular cysts in the mandible and maxilla, along with calcification of anterior part of the falx cerebri. She was eventually diagnosed with Gorlin-Goltz syndrome based on imaging and histopathologic finding of keratocystic odontogenic tumor. PMID:25610614

  11. Fat embolism syndrome after combined aesthetic surgery.

    PubMed

    Erba, Paolo; Farhadi, Jian; Schaefer, Dirk Johannes; Pierer, Gerhard

    2011-02-01

    Fat embolism syndrome is a rare complication that develops after extended soft tissue disruption by liposuction, in particular if combined with time consuming, multiple procedures. Early signs are non-specific and often not considered, so that diagnosis and correct management may be delayed. We report a case in which liposuction combined with other aesthetic surgical procedures caused a fat embolism syndrome in a 46-year-old woman, which was followed by multiple organ failure and the development of sepsis with perimammary abscesses. Extended liposuction of the abdomen and thighs, bilateral augmentation mammaplasty, and stripping of both greater saphenous veins were combined. PMID:20158423

  12. Sotos syndrome: An interesting disorder with gigantism

    PubMed Central

    Nalini, A.; Biswas, Arundhati

    2008-01-01

    We report the case of a 16-year-old boy diagnosed to have Sotos syndrome, with rare association of bilateral primary optic atrophy and epilepsy. He presented with accelerated linear growth, facial gestalt, distinctive facial features, seizures and progressive diminution of vision in both eyes. He had features of gigantism from early childhood. An MRI showed that brain and endocrine functions were normal. This case is of interest, as we have to be aware of this not so rare disorder. In addition to the classic features, there were two unusual associations with Sotos syndrome in the patient. PMID:19893668

  13. Unilateral Ectrodactyly in a Newborn with Trisomy 18 Syndrome: An Unusual Association.

    PubMed

    Kislal, Fatih Mehmet; Altuntas, Nilgun; Ozdemir, Osman; Ceylaner, Serdar; Kislal, Mustafa Hayri; Andiran, Nesibe

    2015-08-01

    The case of a newborn male with trisomy 18 syndrome, having bilateral syndactyly, aplasia and hypoplasia of the foot digits, unilateral ectrodactyly of the left foot and a prominently dorsiflexed hallux, clenched hand with overlapping fingers and general hypertonia, is presented. There are only 5 cases of trisomy 18 syndrome associated with ectrodactyly in the literature. We present a case of trisomy 18 syndrome with unilateral ectrodactyly of the left foot, which is an infrequent association. PMID:26305313

  14. Globus Pallidus Interna Deep Brain Stimulation in a Patient with Medically Intractable Meige Syndrome

    PubMed Central

    Bae, Dae-Woong; Son, Byung-chul; Kim, Joong-Seok

    2014-01-01

    Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects. PMID:25360233

  15. Hierarchical bilateral filtering based disparity estimation for view synthesis

    NASA Astrophysics Data System (ADS)

    Shin, Hong-Chang; Lee, Gwangsoon; Cheong, Won-Sik; Hur, Namho

    2016-06-01

    In this paper, we introduce a high efficient and practical disparity estimation using hierarchical bilateral filtering for real-time view synthesis. The proposed method is based on hierarchical stereo matching with hardware-efficient bilateral filtering. Hardware-efficient bilateral filtering is different from the exact bilateral filter. The purpose of the method is to design an edge-preserving filter that can be efficiently parallelized on hardware. The proposed hierarchical bilateral filtering based disparity estimation is essentially a coarse-to-fine use of stereo matching with bilateral filtering. It works as follows: firstly, the hierarchical image pyramid are constructed; the multi-scale algorithm then starts by applying a local stereo matching to the downsampled images at the coarsest level of the hierarchy. After the local stereo matching, the estimated disparity map is refined with the bilateral filtering. And then the refined disparity map will be adaptively upsampled to the next finer level. The upsampled disparity map used as a prior of the corresponding local stereo matching at the next level, and filtered and so on. The method we propose is essentially a combination of hierarchical stereo matching and hardware-efficient bilateral filtering. As a result, visual comparison using real-world stereoscopic video clips shows that the method gives better results than one of state-of-art methods in terms of robustness and computation time.

  16. A prospective study of bilateral inguinal hernia repair.

    PubMed Central

    Serpell, J. W.; Johnson, C. D.; Jarrett, P. E.

    1990-01-01

    A prospective study of outcome after inguinal hernia repair in patients undergoing simultaneous repair of bilateral hernias (n = 31), sequential repair of bilateral hernias (n = 5), and unilateral hernia repair (n = 75) is reported. There were no differences in wound complications, post-operative respiratory complications, or other adverse effects in the three groups. Operating time was similar in the unilateral and bilateral simultaneous repairs (median 55 min), but was longer (100 min) for the combination of two sequential repairs. Hospital stay was shortest for patients undergoing unilateral repair (2 days) but was less with bilateral simultaneous repair (4 days) than after two sequential repairs (total of 6 days). There were 12 (11%) wound complications of which five (5%) were infections. There was no difference in complication rate between unilateral and bilateral hernia repair. Postoperative recovery was assessed prospectively and was recorded at 1 month. There was no difference between unilateral and bilateral simultaneous repairs in the number of days before the patient was able to climb stairs easily, drive a car or return to work. The duration of the requirement for analgesia was similar in each group. We conclude that bilateral simultaneous hernia repair can be carried out with no greater morbidity than a unilateral repair, and the return to normal activity is as rapid. Bilateral hernias should be repaired simultaneously rather than sequentially. PMID:2221764

  17. Bilateral congenital ureteral strictures in a young cat

    PubMed Central

    Lee, Namsoon; Choi, Mihyun; Keh, Seoyeon; Oh, Yein; Seo, Jimin; Choi, Heeyeon; Kim, Hyunwook; Yoon, Junghee

    2014-01-01

    An 8-month-old cat was presented with bilateral hydronephrosis. Bilateral ureteral obstructions were identified by diagnostic imaging and confirmed by necropsy. Histopathologic findings revealed polypoid transitional epithelial hyperplasia with chronic lymphoplasmacytic inflammation. This report documents congenital ureteral strictures as a cause of ureteral obstruction in a young cat. PMID:25183890

  18. UNITED STATES/GERMAN TECHNICAL BILATERAL AGREEMENT: PAST, PRESENT & FUTURE

    EPA Science Inventory

    The U.S. Environmental Protection Agency (EPA) and the German Federal Ministry of Education and Research (BMBF) entered into a Bilateral Agreement in 1990 to study each country's efforts in developing and demonstrating remedial technologies. The bilateral agreement is being impl...

  19. Bilateral Cochlear Implantation in Children: Experiences and Considerations

    ERIC Educational Resources Information Center

    Bohnert, Andrea; Spitzlei, Vera; Lippert, Karl L.; Keilmann, Annerose

    2006-01-01

    Between 2000 and 2006, the University Clinic for Ear Nose and Throat and Communication Disorders in Mainz, Germany, performed 41 bilateral cochlear implantations in children. This article addresses some of the factors to be considered in a decision to bilaterally implant a child, including the age of the child at the first implant, the length of…

  20. Ultrasound Diagnosis of Bilateral Quadriceps Tendon Rupture After Statin Use

    PubMed Central

    Nesselroade, Ryan D.; Nickels, Leslie Connor

    2010-01-01

    Simultaneous bilateral quadriceps tendon rupture is a rare injury. We report the case of bilateral quadriceps tendon rupture sustained with minimal force while refereeing a football game. The injury was suspected to be associated with statin use as the patient had no other identifiable risk factors. The diagnosis was confirmed using bedside ultrasound. PMID:21079697

  1. Bilateral nasolabial cysts - case report and review of literature

    PubMed Central

    Patil, Aruna R; Singh, Abhinav Pratap; Nandikoor, Shrivalli; Meganathan, Prabhu

    2016-01-01

    Nasolabial cyst is a non-odontogenic, extraosseous, soft tissue cyst, commonly unilateral, located in the nasolabial fold. Bilateral nasolabial cysts are of rare occurrence. This case report describes the multimodality imaging appearance of bilateral nasolabial cysts with a review of literature.

  2. Simultaneous bilateral quadriceps tendon rupture while playing basketball.

    PubMed

    Shah, M; Jooma, N

    2002-04-01

    Simultaneous bilateral quadriceps tendon rupture is an uncommon injury in healthy people and only a few cases have been reported in athletes. This is the first report of a patient with simultaneous bilateral quadriceps tendon rupture incurred while playing basketball. The injury was surgically repaired and the patient had a good functional outcome. PMID:11916903

  3. Bilateral familial Hirayama disease in a father and daughter

    PubMed Central

    Pandey, Sanjay; Jain, Shruti

    2016-01-01

    We are reporting a case of bilateral familial Hirayama disease where a father and daughter are the affected members of the family with the similar distribution of their weakness and wasting. To the best of our knowledge, bilateral familial Hirayama disease has not been described in father and daughter. PMID:27293344

  4. Chronic Cough and Bilateral Pneumothoraces in a Nonsmoker.

    PubMed

    O'Beirne, Sarah L; Escalon, Joanna G; Arkin, Jordan E; Stiles, Brendon M; Kaner, Robert J; Legasto, Alan C; Narula, Navneet; King, Thomas C

    2016-02-01

    An 82-year-old Japanese nonsmoking man presented with persistent dry cough and small left apical pneumothorax. High resolution CT scan of the chest demonstrated bilateral upper lobe pleuroparenchymal thickening and architectural distortion. Serial imaging revealed mild progression and development of small bilateral pneumothoraces, and pneumomediastinum. A surgical lung biopsy was required to confirm the diagnosis. PMID:26867855

  5. Dumping Syndrome

    MedlinePlus

    ... Disease Organizations​​ (PDF, 341 KB)​​​​​ Alternate Language URL Dumping Syndrome Page Content On this page: What is ... Nutrition Points to Remember Clinical Trials What is dumping syndrome? Dumping syndrome occurs when food, especially sugar, ...

  6. Velocardiofacial Syndrome

    ERIC Educational Resources Information Center

    Gothelf, Doron; Frisch, Amos; Michaelovsky, Elena; Weizman, Abraham; Shprintzen, Robert J.

    2009-01-01

    Velocardiofacial syndrome (VCFS), also known as DiGeorge, conotruncal anomaly face, and Cayler syndromes, is caused by a microdeletion in the long arm of Chromosome 22. We review the history of the syndrome from the first clinical reports almost half a century ago to the current intriguing molecular findings associating genes from the…

  7. Down syndrome

    MedlinePlus

    Down syndrome is a genetic condition in which a person has 47 chromosomes instead of the usual 46. ... In most cases, Down syndrome occurs when there is an extra copy of chromosome 21. This form of Down syndrome is called trisomy 21. ...

  8. Dance Therapy with Physical Therapy for Children with Down Syndrome.

    ERIC Educational Resources Information Center

    Dupont, Blanche Burt; Schulmann, Diana

    This study sought to investigate effects of a dance program on bilateral toe-standing balance and single-point static balance skills of a group of children with Down Syndrome. Thirteen experimental and 10 control group students between the ages of 3 and 13 years were assessed on toe-standing balance and single-point standing balance on the right…

  9. Bilateral Thoracic Ganglion Cyst : A Rare Case Report

    PubMed Central

    Kazanci, Burak; Tehli, Ozkan; Guclu, Bulent

    2013-01-01

    Ganglion cysts usually arise from the tissues around the facet joints. It is usually associated with degenerative cahanges in facet joints. Bilateral thoracic ganglion cysts are very rare and there is no previous case that located in bilateral intervertebral foramen compressing the L1 nerve root associated with severe radiculopathy. We report a 53 years old woman who presented with bilateral groin pain and severe numbness. Magnetic resonance imaging revealed bilateral cystic mass in the intervertebral foramen between 12th thoracal and 1st lumbar vertebrae. The cystic lesions were removed after bilateral exposure of Th12-L1 foramens. The result of hystopathology confirmed the diagnosis as ganglion cyst. The ganglion cyst may compromise lumbar dorsal ganglion when it located in the intervertebral foramen. The surgeon should keep this rare entity in their mind for differential diagnosis. PMID:23908708

  10. Bilateral choroidal melanoma--case analysis and literature review.

    PubMed

    Kowal, Joanna; Strzałka, Anna; Markiewicz, Anna; Romanowska-Dixon, Bożena; Bogdali, Anna

    2015-01-01

    Uveal melanoma is the most common primary intraocular neoplasm in adults. Its bilateral localization is extremely rare. The aim of the paper is analysis of the cases of bilateral uveal melanoma. Five bilateral uveal melanoma patients were diagnosed in the Department of Ophtalmology and Ocular Oncology beetwen 1980 and 2014. Both eyes of four patients were threated with brachytherapy. Final enulcleation of the one eye was performed in three patients. It was the primary treatment in one patient. The presence of uveal melanoma was confirmed by pathological examination in all cases after surgical removal of eyeball and in one after local resection of iris tumor. Metastatic lesions were diagnosed in lungs and liver in two patients. Three patients are still followed-up at our institution. The possibility of bilateral uveal melanoma should considered although it is extremely rare. bilateral uveal melanoma, brachytherapy, enucleation. PMID:26638545

  11. Bilateral Clavicle Fractures: A Report of Three Cases

    PubMed Central

    Khatri, Kavin; Sharma, Vijay; Farooque, Kamran; Sharma, Swati

    2016-01-01

    Bilateral clavicle fractures are uncommonly reported in the literature with the incidence being less than 0.5% of all the clavicle fractures. Bilateral clavicle fractures are caused either by high-energy transfer of compression forces across both shoulder girdles or by a direct trauma to one clavicle followed by that to the other clavicle. These fractures could be missed due to their association with more severe chest injuries or a more symptomatically displaced fracture on one side or due to inadequate chest radiographs. We report three cases of traumatic bilateral clavicle fractures with three modes of injuries in different age groups. All the fractures were treated conservatively with good functional outcomes without any sequelae. Bilateral clavicle fractures should be actively sought by every trauma team with proper clinical examination and chest radiographs including both shoulder joints in high-energy trauma cases or with bilateral shoulder compression injuries. PMID:27504365

  12. Osteomyelitis of Bilateral Femoral Heads After Childbirth: A Case Report

    PubMed Central

    Lee, Kyung Soo; Kong, Sangwon; Kim, Junho; Kim, Taikon; Choi, Chan Beom; Kim, Yee-Suk

    2015-01-01

    Hip and pelvic pain during pregnancy or after delivery is a common problem in young females, and in most cases this problem has a self-limiting course. The patient described in this case suffered from severe hip pain after childbirth. MR imaging study was performed and it showed arthritis of bilateral hip joints and osteomyelitis of femoral heads with an abscess in the surrounding muscle. Infection, such as septic arthritis or osteomyelitis, is an extremely rare cause of peripartum joint pain. The patient's clinical symptoms and laboratory findings improved with antibiotic therapy. However, limitation of motion of the bilateral hip joints persisted although the patient continued rehabilitative therapy for 15 months, and the patient had to undergo bilateral total hip replacement. Hereby, we present a case of severe osteomyelitis and pyogenic arthritis of bilateral femoral heads and hip joints after delivery, which eventually required bilateral total hip replacement. PMID:26161359

  13. Bilateral ethmoidal dural arteriovenous fistula: unexpected surgical diagnosis.

    PubMed

    Ros de San Pedro, Javier; Pérez, Claudio J Piqueras; Parra, Joaquín Zamarro; López-Guerrero, Antonio López; Sánchez, Juan F Martínez-Lage

    2010-12-01

    Dural arteriovenous fistulae (DAVFs) are infrequent lesions, the most common locations of which are the cavernous, sigmoid and transverse sinuses. The cribiform plate is one of the less frequent sites for DAVFs, where they entail a high hemorrhage risk. Feeding arteries for ethmoidal DAVFs can be uni- or bilateral. However, the draining fistulous system has classically been described as unilateral. The authors report the second case in literature of bilateral ethmoidal DAVF, which is defined as that with bilateral draining veins. The present case was diagnosed only after surgical exploration of both cribiform plates. No preoperative radiological test could detect the presence of a bilateral venous draining system from the ethmoidal DAVF. Possible reasons for that lack of presurgical diagnosis are discussed. Bilateral surgical exploration of the anterior cranial fossa is recommended when dealing with ethmoidal DAVFs, even when they seem to be unilateral on preoperative studies. PMID:20727670

  14. A Case of Wolfram Syndrome

    PubMed Central

    Naderian, Gholamali; Ashtari, Fereshteh; Nouri-Mahdavi, Kia; Sajjadi, Valleh

    2010-01-01

    Purpose To report a case of Wolfram syndrome characterized by early onset diabetes mellitus and progressive optic atrophy. Case Report A 20-year-old male patient with diabetes mellitus type I presented with best corrected visual acuity of 1/10 in both eyes with correction of −0.25+1.50@55 and −0.25+1.50@131 in his right and left eyes, respectively. Bilateral optic atrophy was evident on fundus examination. The patient also had diabetes insipidus, neurosensory deafness, neurogenic bladder, polyuria and extra-residual voiding indicating atony of the urinary tract, combined with delayed sexual maturity. Conclusion One should consider Wolfram syndrome in patients with juvenile onset diabetes mellitus and hearing loss. Ophthalmological examination may disclose optic atrophy; urologic examinations are vital in such patients. PMID:22737327

  15. Wolfram syndrome maps to distal human chromosome 4p

    SciTech Connect

    Polymeropoulos, M.H.; Swift, R.; Swift, M.

    1994-09-01

    Wolfram syndrome (MIM 222300) is an autosomal recessive disorder defined by the occurrence of diabetes mellitus and progressive bilateral optic atrophy. Wolfram syndrome homozygotes develop widespread nervous system abnormalities; in particular, they exhibit severe behavioral difficulties that often lead to suicide attempts or psychiatric hospitalizations. The Wolfram syndrome gene also predisposes heterozygous carriers to psychiatric disorders. Since these heterozygotes are common in the general population, the Wolfram syndrome gene may contribute significantly to the overall burden of psychiatric illness. Based on a linkage analysis of 11 families segregating for this syndrome, using microsatellite repeat polymorphisms throughout the human genome, we found the Wolfram syndrome gene to be linked to markers on the short arm of human chromosome 4, with Zmax=6.46 at {theta}=0.02 for marker D4S431.

  16. Bilateral renal vein thrombosis and pulmonary embolism secondary to membranous glomerulonephritis treated with percutaneous catheter thrombectomy and localized thrombolytic therapy

    PubMed Central

    Janda, S. P.

    2010-01-01

    Renal vein thrombosis (RVT) is a rare event but is prevalent in patients with nephrotic syndrome. Bilateral RVT is even rarer. The literature is relatively sparse in terms of the management of RVT because of its rarity and consists of a few case reports and case series. We present a case with bilateral RVT complicated by a pulmonary embolism in a patient with membranous glomerulonephritis (MGN). A 19-year-old female presented with acute flank pain and worsening renal function after a couple of weeks in hospital while being treated with diuretics for anasarca secondary to MGN. Venography was used for diagnosis. The patient underwent percutaneous catheter thrombectomy and localized thrombolysis achieving resolution of pain and improvement of renal function. The patient was then anticoagulated for life with warfarin. PMID:21072156

  17. [A case of bilateral paramedian thalamic infarction in childhood with the sensory disturbance and the sensory loss of taste].

    PubMed

    Tohyama, Jun; Kanazawa, Osamu; Akasaka, Noriyuki; Kamimura, Takanori

    2004-01-01

    Bilateral paramedian thalamic infarcts are characterized by disturbance of consciousness, followed by persisting dementia, decreased spontaneity, apathy, amnesia and paralysis of eye movement. We report a 15-year-old boy with this syndrome, who exhibited transient coma at the onset. In addition to the typical symptoms, he complained of sensory disturbance in the lower extremities and face and the loss of taste sense. MRI showed symmetric paramedian thalamic infarction. There was no lesion in the midbrain. The etiology of infarct in this boy remained unknown despite extensive laboratory and neuroradiological examination. His sensory disturbance in the extremities and face may be due to extensive involvement of the inferolateral area of the thalamus by infarction of the paramedian thalamic artery. This patient illustrates that bilateral paramedian thalamic infarction can occur in a previously healthy child. PMID:14737867

  18. Comparative gait initiation kinematics between simulated unilateral and bilateral ankle hypomobility: Does bilateral constraint improve speed performance?

    PubMed

    Delafontaine, A; Honeine, J-L; Do, M-C; Gagey, O; Chong, R K

    2015-08-31

    Improvement of motor performance in unilateral upper limb motor disability has been shown when utilizing inter-limb coupling strategies during physical rehabilitation. This suggests that 'default' bilateral central motor commands are facilitated. Here, we tested whether this bilateral motor control principle may be generalized to the lower limbs during gait initiation, which involves alternate bilateral actions. Disability was simulated by strapping to produce ankle hypomobility. Healthy adult subjects initiated gait at a self-paced speed with no ankle constraint (control), or with the stance, swing or bilateral ankles strapped. The duration of the anticipatory postural adjustments lengthened and the center of mass instantaneous progression velocity at foot-off decreased when the ankle was strapped. During the step execution phase, progression velocity at foot-contact was higher when both ankles were strapped compared to unilateral strapping of the stance ankle. These findings suggest that bilateral central motor commands are favored during walking tasks. Indeed, unilateral constraint of the stance ankle should compel the central nervous system to adapt specific commands to the constraint and normal sides whereas the 'default' bilateral motor commands would be utilized when both ankles are strapped leading to better kinematics performance. Bilateral in-phase upper limb coordination and bilateral alternating lower limb locomotor movements may share similar control mechanisms. PMID:26197055

  19. Chronic exertional compartment syndrome with medial tibial stress syndrome in twins.

    PubMed

    Banerjee, Purnajyoti; McLean, Christopher

    2011-06-01

    Chronic exertional compartment syndrome and medial tibial stress syndrome are uncommon conditions that affect long-distance runners or players involved in team sports that require extensive running. We report 2 cases of bilateral chronic exertional compartment syndrome, with medial tibial stress syndrome in identical twins diagnosed with the use of a Kodiag monitor (B. Braun Medical, Sheffield, United Kingdom) fulfilling the modified diagnostic criteria for chronic exertional compartment syndrome as described by Pedowitz et al, which includes: (1) pre-exercise compartment pressure level >15 mm Hg; (2) 1 minute post-exercise pressure >30 mm Hg; and (3) 5 minutes post-exercise pressure >20 mm Hg in the presence of clinical features. Both patients were treated with bilateral anterior fasciotomies through minimal incision and deep posterior fasciotomies with tibial periosteal stripping performed through longer anteromedial incisions under direct vision followed by intensive physiotherapy resulting in complete symptomatic recovery. The etiology of chronic exertional compartment syndrome is not fully understood, but it is postulated abnormal increases in intramuscular pressure during exercise impair local perfusion, causing ischemic muscle pain. No familial predisposition has been reported to date. However, some authors have found that no significant difference exists in the relative perfusion, in patients, diagnosed with chronic exertional compartment syndrome. Magnetic resonance images of affected compartments have indicated that the pain is not due to ischemia, but rather from a disproportionate oxygen supply versus demand. We believe this is the first report of chronic exertional compartment syndrome with medial tibial stress syndrome in twins, raising the question of whether there is a genetic predisposition to the causation of these conditions. PMID:21667913

  20. [Worster-Drought syndrome--a specific cerebral palsy syndrome--why is the diagnosis frequently overlooked?].

    PubMed

    Shmueli, Dorit; Gross-Tsur, Varda

    2007-10-01

    Worster-Drought syndrome (WDS) is a developmental disorder presenting as mild tetraplegic cerebral palsy (CP) with severe pseudobulbar palsy, manifested as articulation problems or anarthria, chewing, and swallowing difficulties and severe drooling. Epilepsy, learning disabilities, behavioral disturbances, and other congenital anomalies are common. WDS accounts for about 1% of CP cases. On brain imaging, migrational abnormalities are frequently found, mainly in the opercular area, however normal brain imaging does not exclude the diagnosis. Although children with WDS have serious, early-onset developmental disabilities, the specific diagnosis is frequently delayed or overlooked. There is a lack of awareness of the syndrome and an overlap with similar congenital syndromes, such as the Opercular Syndrome and Congenital Bilateral Perisylvian Syndrome. Two children with WDS are presented, emphasizing the clinical symptoms, natural history of the disorder, etiology and imaging. The similarity between WDS and other specific congenital syndromes will be discussed with the suggestion to unite them all under the name WDS. PMID:17990388

  1. Bilateral cochlear implantation: current concepts, indications, and results.

    PubMed

    Basura, Gregory J; Eapen, Rose; Buchman, Craig A

    2009-12-01

    The optimal treatment for bilateral hearing loss continues to evolve as cochlear implant (CI) and hearing aid technologies advance, as does our understanding of the central auditory system. Ongoing discussions continue on the validity and feasibility of bilateral CI in terms of performance, justification of need, medical/surgical safety concerns, and economics. The purpose of this review article is to provide an update on the advantages and disadvantages of bilateral CI and to provide a discussion on timing (simultaneous vs. sequential), technology (bimodal vs. binaural) and feasibility. Binaural advantages are found in both adult and pediatric bilateral CI recipients, the greatest being the head shadow effect and improvements in localization and loudness summation. This theoretically offers an advantage over their unilateral implanted counterparts in terms of improved sound localization and enhanced speech perception under noisy conditions. Most investigators agree that bilateral stimulation during critical periods of development is paramount for optimizing auditory functioning in children. Currently, bilateral CI is widely accepted as a safe and effective means of bilateral auditory stimulation. PMID:19894280

  2. Speckle reducing bilateral filter for cattle follicle segmentation

    PubMed Central

    2010-01-01

    Background Ultrasound imaging technology has wide applications in cattle reproduction and has been used to monitor individual follicles and determine the patterns of follicular development. However, the speckles in ultrasound images affect the post-processing, such as follicle segmentation and finally affect the measurement of the follicles. In order to reduce the effect of speckles, a bilateral filter is developed in this paper. Results We develop a new bilateral filter for speckle reduction in ultrasound images for follicle segmentation and measurement. Different from the previous bilateral filters, the proposed bilateral filter uses normalized difference in the computation of the Gaussian intensity difference. We also present the results of follicle segmentation after speckle reduction. Experimental results on both synthetic images and real ultrasound images demonstrate the effectiveness of the proposed filter. Conclusions Compared with the previous bilateral filters, the proposed bilateral filter can reduce speckles in both high-intensity regions and low intensity regions in ultrasound images. The segmentation of the follicles in the speckle reduced images by the proposed method has higher performance than the segmentation in the original ultrasound image, and the images filtered by Gaussian filter, the conventional bilateral filter respectively. PMID:21047390

  3. Bilateral follicular cysts in a water buffalo.

    PubMed

    Khan, F A; Nabi, S U; Pande, Megha; Das, G K; Sarkar, M

    2011-03-01

    The present short communication puts on record a case of bilateral, multiple follicular cysts in a water buffalo along with a detailed description of its ovarian biometry and follicular fluid composition. The ovarian weight and biometrical parameters were much higher than in normal cycling buffaloes. A total of three follicular cysts were observed, two on the right ovary and one on the left ovary, measuring 4.9, 3.0 and 2.6 cm yielding 21, 9 and 5 ml of follicular fluid, respectively. The cystic fluid was deep yellow in colour with a viscous consistency. The follicular fluid concentrations of glucose, total protein, cholesterol, acid phosphatase, calcium, phosphorus and progesterone in all the cysts were within the range reported previously in normal buffalo follicular fluid; however, the alkaline phosphatase concentration in cyst 1 and total bilirubin concentration in cysts 1 and 2 were higher than the values in normal follicular fluid. In contrast, the levels of urea nitrogen in cysts 1 and 3, and oestradiol in cyst 3 were lower than the normal values. All the three follicles had an oestradiol to progesterone ratio less than 1. The results of our study suggest that follicular cysts in buffalo are oestrogenically inactive and have an altered concentration of certain biochemical and hormonal constituents. PMID:21086160

  4. Psychosocial Outcomes after Bilateral Hand Transplantation

    PubMed Central

    Singh, Mansher; Oser, Megan; Zinser, Jennifer; Sisk, Geoffroy; Carty, Matthew J; Sampson, Christian; Pribaz, Julian J; Pomahac, Bohdan

    2015-01-01

    Background: Since the first successful hand transplantation in 1998, there have been multiple reports about surgical technique, transplant survival, and immunosuppression. However, very limited published data exist on psychosocial outcomes following hand transplantation. Methods: We report psychosocial outcomes in a patient with bilateral hand transplants at the midforearm level with serial follow-ups over 3.5 years. Different metrics used to study psychosocial outcomes included the following: SF-12, CES-D, Dyadic Adjustment Scale, Rosenberg SE, and EQ-5D. Result: Preoperatively, our patient did not have any evidence of depression (CES-D = 3), had a nonstressful relationship with his spouse (Dyadic Adjustment Scale = 100), and self-esteem was in the normal range (Rosenberg SE = 21). These metrics and his additional scales (SF-12 MCS, EQ-5D, and EQ-VAS) did not change appreciably and were within the normal range for the entire duration of 3.5-year follow-up at all different time points. Conclusion: With the increasing popularity of hand transplantation and the increasing awareness of the importance of psychosocial parameters in overall success, appropriate, comprehensive, and standardized measurements are important. These should be an integral part of patients’ screening and follow-up. PMID:26579339

  5. Rehabilitation for bilateral amputation of fingers

    USGS Publications Warehouse

    Stapanian, Martin A.; Stapanian, Adrienne M.P.; Staley, Keith E.

    2010-01-01

    We describe reconstructive surgeries, therapy, prostheses, and adaptations for a patient who experienced bilateral amputation of all five fingers of both hands through the proximal phalanges in January 1992. The patient made considerable progress in the use of his hands in the 10 mo after amputation, including nearly a 120% increase in the active range of flexion of metacarpophalangeal joints. In late 1992 and early 1993, the patient had "on-top plasty" surgeries, in which the index finger remnants were transferred onto the thumb stumps, performed on both hands. The increased web space and functional pinch resulting from these procedures made many tasks much easier. The patient and occupational therapists set challenging goals at all times. Moreover, the patient was actively involved in the design and fabrication of all prostheses and adaptations or he developed them himself. Although he was discharged from occupational therapy in 1997, the patient continues to actively find new solutions for prehension and grip strength 18 yr after amputation.

  6. Bilateral putaminal necrosis and bronopol toxicity.

    PubMed

    Trivisano, Marina; Carapelle, Elena; Martino, Tommaso; Specchio, Luigi Maria

    2015-01-01

    Among alcohols, methanol intoxication is the most frequently associated with cerebral toxicity, causing retinal damage and putaminal necrosis. This consequence is believed to be due to the transformation of methanol into formic acid. We describe the case of a patient who presented with acute impairment of consciousness and tetraparesis after she had been drinking several bottles of a topical antiseptic solution (Lysoform Medical) containing 2-bromo-2-nitro-1,3-propandiol (bronopol) among excipients, in order to lose weight during previous months. Moreover, she had been on a strict slimming diet. Soon after admission, a severe respiratory and metabolic impairment became rapidly evident, requiring an intensive care unit admission. Cerebral MRI showed the presence of bilateral putaminal necrosis. She recovered in 10 days, surprisingly, without any evident clinical neurological signs. Methanol, also bronopol, when diluted in aqueous solution, at warm temperature and/or higher pH, may release formaldehyde, which is converted into formic acid, a basal ganglia toxic compound. PMID:25697297

  7. Red ear syndrome: literature review and a pediatric case report.

    PubMed

    Moitri, Misha O; Banglawala, Sarfaraz M; Archibald, Jason

    2015-03-01

    Red ear syndrome (RES) is characterized by recurrent unilateral or bilateral painful attacks of the external ear, accompanied by ear redness, burning, or warmth. Proposed etiologies of this rare condition include dysregulation of sympathetic outflow, upper cervical pathology, glossopharyngeal and trigeminal neuralgia, TMJ dysfunction, thalamic syndrome, and primary headache syndromes. Idiopathic cases also exist in the literature. Pediatric cases are particularly rare and more commonly associated with migraine. Given the various potential etiologies, no single treatment is effective in all cases. This paper summarizes the current understanding and management of RES, and describes a case of idiopathic pediatric RES. PMID:25583087

  8. Connectivity-Based Parcellation of the Thalamus Explains Specific Cognitive and Behavioural Symptoms in Patients with Bilateral Thalamic Infarct

    PubMed Central

    Serra, Laura; Cercignani, Mara; Carlesimo, Giovanni A.; Fadda, Lucia; Tini, Nadia; Giulietti, Giovanni; Caltagirone, Carlo; Bozzali, Marco

    2013-01-01

    A novel approach based on diffusion tractography was used here to characterise the cortico-thalamic connectivity in two patients, both presenting with an isolated bilateral infarct in the thalamus, but exhibiting partially different cognitive and behavioural profiles. Both patients (G.P. and R.F.) had a pervasive deficit in episodic memory, but only one of them (R.F.) suffered also from a dysexecutive syndrome. Both patients had an MRI scan at 3T, including a T1-weighted volume. Their lesions were manually segmented. T1-volumes were normalised to standard space, and the same transformations were applied to the lesion masks. Nineteen healthy controls underwent a diffusion-tensor imaging (DTI) scan. Their DTI data were normalised to standard space and averaged. An atlas of Brodmann areas was used to parcellate the prefrontal cortex. Probabilistic tractography was used to assess the probability of connection between each voxel of the thalamus and a set of prefrontal areas. The resulting map of corticothalamic connections was superimposed onto the patients’ lesion masks, to assess whether the location of the thalamic lesions in R.F. (but not in G. P.) implied connections with prefrontal areas involved in dysexecutive syndromes. In G.P., the lesion fell within areas of the thalamus poorly connected with prefrontal areas, showing only a modest probability of connection with the anterior cingulate cortex (ACC). Conversely, R.F.’s lesion fell within thalamic areas extensively connected with the ACC bilaterally, with the right dorsolateral prefrontal cortex, and with the left supplementary motor area. Despite a similar, bilateral involvement of the thalamus, the use of connectivity-based segmentation clarified that R.F.’s lesions only were located within nuclei highly connected with the prefrontal cortical areas, thus explaining the patient’s frontal syndrome. This study confirms that DTI tractography is a useful tool to examine in vivo the effect of focal lesions

  9. Bilateral ureteric stones: an unusual cause of acute kidney injury.

    PubMed

    Sumner, Daniel; Rehnberg, Lucas; Kler, Aaron

    2016-01-01

    A 49-year-old man presented to the accident and emergency department, with a short history of vague abdominal pain, abdominal distension and two episodes of frank haematuria. A plain chest film showed dilated loops of large bowel and blood results on admission showed an acute kidney injury (stage 3). A diagnosis of bowel obstruction was made initially but a CT scan of the abdomen showed bilateral obstructing calculi. After initial resuscitation, the patient had bilateral ultrasound-guided nephrostomies and haemofiltration. He later underwent bilateral antegrade ureteric stenting. A decision will later be made on whether or not he is fit enough to undergo ureteroscopy and laser stone fragmentation. PMID:27030462

  10. Unilateral Direct Carotid Cavernous Fistula Causing Bilateral Ocular Manifestation

    PubMed Central

    Demartini Jr., Zeferino; Liebert, Fernando; Gatto, Luana Antunes Maranha; Jung, Thiago Simiano; Rocha Jr., Carlos; Santos, Alex Marques Borges; Koppe, Gelson Luis

    2015-01-01

    Unilateral carotid cavernous fistula presents with ipsilateral ocular findings. Bilateral presentation is only seen in bilateral fistulas, usually associated with indirect (dural) carotid cavernous fistulas. Direct carotid cavernous fistulas are an abnormal communication between the internal carotid artery and the cavernous sinus. They typically begin with a traumatic disruption in the artery wall into the cavernous sinus, presenting with a classic triad of unilateral pulsatile exophthalmos, cranial bruit and episcleral venous engorgement. We report the case of a 38-year-old male with traumatic right carotid cavernous sinus fistula and bilateral ocular presentation successfully treated by interventional neuroradiology. PMID:26955353

  11. Reduced 123I Ioflupane Binding in Bilateral Diabetic Chorea

    PubMed Central

    Sato, Kenichiro; Hida, Ayumi; Kameyama, Masashi; Morooka, Miyako; Takeuchi, Sousuke

    2016-01-01

    Abstract We report a 64-year-old man with diabetic chorea whom we investigated with dopamine transporter SPECT, 18F FDG PET, 99mTc ethylcysteinate dimer (ECD) SPECT, and 123I metaiodobenzylguanidine (MIBG) scintigraphy. Dopamine transporter SPECT revealed reduced 123I ioflupane binding in the bilateral striatum. 18F FDG PET showed metabolic dysfunction in the bilateral striatum, as shown in earlier studies. 99mTc ECD SPECT revealed reduced brain perfusion in the bilateral caudate nucleus and putamen. 123I MIBG scintigraphy revealed no cardiac sympathetic nerve dysfunction. Our case suggests a possible nigrostriatal presynaptic dopaminergic involvement in diabetic chorea. PMID:26975011

  12. Orthodontic treatment of bilateral geminated maxillary permanent incisors.

    PubMed

    Le Gall, Michel; Philip, Camille; Aboudharam, Gérard

    2011-05-01

    Geminated teeth occur more frequently in the deciduous dentition than in the permanent dentition, with prevalence figures of 0.6% and 0.1%, respectively. Bilateral presentation is rare. A survey of the literature showed that the prevalence estimates for bilateral double teeth range from 0.01% to 0.04% in the deciduous dentition, and 0.05% in the permanent dentition. In this article, we report a rare case of bilateral fusion of the maxillary permanent incisors, discuss possible histogenetic mechanisms related to this anomaly, and explain the treatment management. PMID:21536214

  13. Spheniodal mucocele causing bilateral optic neuropathy and ophthalmoplegia

    PubMed Central

    Selvakumar, Ambika; Mahalaxmi, Balasubramanyam; Ananth, V; Gautam, Cugati

    2014-01-01

    Sphenoid sinus mucocele comprises only 2% of all paranasal sinus mucoceles. In literature, there is a case report on sphenoidal mucocele causing bilateral optic neuropathy, with unilateral partial recovery and cranial nerve palsy, but we did not come across any literature with bilateral optic neuropathy and ophthalmoplegia together caused by spheno-ethmoidal mucocele. We present such a rare case of spheno-ethmoidal mucocele causing bilateral optic neuropathy and unilateral sixth nerve palsy who had postsurgery, unilateral good vision recovery, and complete resolution of sixth nerve palsy. PMID:23571243

  14. A rare case of bilateral bronchial foreign body

    PubMed Central

    Zhao, ZhiGang; Gao, Qian; Song, PengLong

    2015-01-01

    We present the case of a 7-year-old male patient with bilateral bronchial cocklebur fruit aspiration, which he sustained while playing. The patient presented with a triad of cough, wheezing, and decreased breath sounds (decreased in the right lung and absent in the left). These symptoms led to a diagnosis of bilateral bronchial foreign body, which was confirmed by computed tomography three-dimensional reconstruction of the bronchial tree. The patient was on the verge of death during operation but was ultimately rescued. Our therapeutic experience in treating this case of bilateral bronchial cocklebur fruit aspiration may provide a good reference for others. PMID:26101515

  15. Bilateral topographic symmetry patterns across Aphrodite Terra, Venus

    NASA Technical Reports Server (NTRS)

    Crumpler, L. S.; Head, James W.

    1988-01-01

    Topographic profiles have been obtained across Aphrodite Terra to test for bilateral symmetry of the type associated with thermal boundary layer topography at divergent plate boundaries on earth. In addition to a broad bilateral symmetry at a range of angles across Aphrodite Terra, detailed bilateral symmetry is noted within domains between linear discontinuities in directions parallel to the strike of the discontinuities. The results suggest that western Aphrodite Terra is similar to terrestrial oceanic divergent plate boundary environments, and that the cross-strike discontinuities are analogous to oceanic fracture zones rather than strike-slip faults.

  16. Parkinsonism and transient bilateral ptosis in systemic lupus erythematosus

    PubMed Central

    Teoh, P. C.; Richard, A. T. Ng; Wong, P. K.

    1974-01-01

    Many neurological abnormalities have been described in systemic lupus erythematosus (SLE), but transient bilateral ptosis and parkinsonism are rarely encountered. This paper describes a young Malay girl with SLE who develops psychosis, bilateral ptosis and parkinsonism during an exacerbation of her illness. These neurological features disappeared after adequate treatment with cyclophosphamide. Though the pathogenesis of these neurological abnormalities is not clearly known, it is likely that transient bilateral ptosis is due to myoneural dysfunction not unlike that of myasthenia gravis. As for parkinsonism, it can probably be explained on the basis of ‘vasculitis’ of the basal ganglia leading to microinfarcts and encephalomalacia. ImagesFig. 1Fig. 2

  17. Epilepsy in Muenke Syndrome: FGFR3-Related Craniosynostosis

    PubMed Central

    Agochukwu, Nneamaka B.; Solomon, Benjamin D.; Gropman, Andrea L.; Muenke, Maximilian

    2014-01-01

    Epilepsy, a neurologic disorder characterized by the predisposition to recurrent unprovoked seizures, is reported in more than 300 genetic syndromes. Muenke syndrome is an autosomal-dominant craniosynostosis syndrome characterized by unilateral or bilateral coronal craniosynostosis, hearing loss, intellectual disability, and relatively subtle limb findings such as carpal bone fusion and tarsal bone fusion. Muenke syndrome is caused by a single defining point mutation in the fibroblast growth factor receptor 3 (FGFR3) gene. Epilepsy rarely occurs in individuals with Muenke syndrome, and little detail is reported on types of epilepsy, patient characteristics, and long-term outcomes. We present seven patients with Muenke syndrome and seizures. A review of 789 published cases of Muenke syndrome, with a focus on epilepsy and intracranial anomalies in Muenke syndrome, revealed epilepsy in six patients, with intracranial anomalies in five. The occurrence of epilepsy in Muenke syndrome within our cohort of 58 patients, of whom seven manifested epilepsy, and the intracranial anomalies and epilepsy reported in the literature, suggest that patients with Muenke syndrome may be at risk for epilepsy and intracranial anomalies. Furthermore, the impact of Muenke syndrome on the central nervous system may be greater than previously thought. PMID:23044018

  18. Zellweger syndrome with severe malnutrition, immunocompromised state and opportunistic infections.

    PubMed

    Cardoso, Patrícia; Amaral, Maria Emanuel; Lemos, Sónia; Garcia, Paula

    2016-01-01

    Peroxisome biogenesis disorders are related to a spectrum of genetic diseases that range from severe Zellweger syndrome to milder infantile Refsum disease. Zellweger syndrome is characterised by dysmorphic features, severe hypotonia, seizures, failure to thrive, liver dysfunction and skeletal defects. Increased levels of very long chain fatty acids are the biochemical hallmark and the most common mutations found in the PEX1 gene. We report an unusual presentation of Zellweger syndrome in a 2-month-old female infant with severe malnutrition, opportunistic infections, lymphopaenia and a small thymic shadow on chest radiography. With this clinical picture, an initial hypothesis of primary immunodeficiency was considered. It was later confirmed to not be the case. On follow-up, global developmental delay, bilateral optic nerve atrophy and moderate bilateral sensorineural deafness grade II were documented. There were no further infectious complications and we concluded malnutrition was the cause of the infant's immunocompromised state. PMID:27090541

  19. Unexplained neuropsychiatric symptoms in intensive care: A Fahr Syndrome case.

    PubMed

    Calili, Duygu Kayar; Mutlu, Nevzat Mehmet; Mutlu Titiz, Ayse Pinar; Akcaboy, Zeynep Nur; Aydin, Eda Macit; Turan, Isil Ozkocak

    2016-08-01

    Fahr Syndrome is a rare disease where calcium and other minerals are stored bilaterally and symmetrically in the basal ganglia, cerebellar dentate nucleus and white matter. Fahr Syndrome is associated with various metabolic disorders, mainly parathyroid disorders. The presented case discusses a 64-year old male patient admitted to the intensive care unit of our hospital diagnosed with aspiration pneumonia and urosepsis. The cranial tomography examination to explain his nonspecific neurological symptoms showed bilateral calcifications in the temporal, parietal, frontal, occipital lobes, basal ganglia, cerebellar hemisphere and medulla oblongata posteriorly. His biochemical test results also indicated parathormone-calcium metabolic abnormalities. Fahr Syndrome must be considered for a definitive diagnosis in patients with nonspecific neuropsychiatric symptoms and accompanying calcium metabolism disorders in order to control serious morbidity and complications because of neurological damage. PMID:27524543

  20. Slowly progressive anarthria with late anterior opercular syndrome: a variant form of frontal cortical atrophy syndromes.

    PubMed

    Broussolle, E; Bakchine, S; Tommasi, M; Laurent, B; Bazin, B; Cinotti, L; Cohen, L; Chazot, G

    1996-12-01

    We describe eight patients with slowly progressive speech production deficit combining speech apraxia, dysarthria, dysprosody and orofacial apraxia, and initially no other deficit in other language and non-language neuropsychological domains. Long-term follow-up (6-10 years) in 4 cases showed an evolution to muteness, bilateral suprabulbar paresis with automatic-voluntary dissociation and frontal lobe cognitive slowing without generalised intellectual deterioration. Most disabled patients presented with an anterior opercular syndrome (Foix-Chavany-Marie syndrome), and pyramidal or extrapyramidal signs. CT and MRI findings disclosed asymmetric (left > right) progressive cortical atrophy of the frontal lobes predominating in the posterior inferior frontal region, notably the operculum. SPECT and PET revealed a decreased cerebral blood flow and metabolism, prominent in the left posterior-inferior frontal gyrus and premotor cortex, extending bilaterally in the most advanced cases. Pathological study of two cases showed non-specific neuronal loss, gliosis, and spongiosis of superficial cortical layers, mainly confined to the frontal lobes, with no significant abnormalities in the basal ganglia, thalamus, cerebellum, brain stem (except severe neuronal loss in the substantia nigra in one case), and spinal cord. We propose to call this peculiar syndrome Slowly Progressive Anarthria (SPA), based on its specific clinical presentation, and its metabolic and pathological correlates. SPA represents another clinical expression of focal cortical degeneration syndromes, that may overlap with other similar syndromes, specially primary progressive aphasia and the various frontal lobe dementias. PMID:8994103

  1. Overgrowth Syndromes.

    PubMed

    Edmondson, Andrew C; Kalish, Jennifer M

    2015-09-01

    Numerous multiple malformation syndromes associated with pathologic overgrowth have been described and, for many, their molecular bases elucidated. This review describes the characteristic features of these overgrowth syndromes, as well as the current understanding of their molecular bases, intellectual outcomes, and cancer predispositions. We review syndromes such as Sotos, Malan, Marshall-Smith, Weaver, Simpson-Golabi-Behmel, Perlman, Bannayan-Riley-Ruvalcaba, PI3K-related, Proteus, Beckwith-Wiedemann, fibrous dysplasia, Klippel-Trenaunay-Weber, and Maffucci. PMID:27617124

  2. Lutembacher's syndrome

    PubMed Central

    Kulkarni, Sandhya S.; Sakaria, Amit K.; Mahajan, Sanket K.; Shah, Kuldeep B.

    2012-01-01

    The definition of Lutembacher's syndrome has undergone many changes. It refers to combination of congenital Atrial Septal Defect with acquired mitral stenosis. Lutembacher's syndrome is a very rare disease and in the past, it has been either overdiagnosed or misdiagnosed. Here, we will discuss a case of a pregnant lady who developed breathlessness during her third trimester of pregnancy and on detailed examination and investigation, she was found to be having Lutembacher's syndrome. PMID:22629045

  3. Bilateral congenital lumbar hernias in a patient with central core disease--A case report.

    PubMed

    Lazier, Joanna; Mah, Jean K; Nikolic, Ana; Wei, Xing-Chang; Samedi, Veronica; Fajardo, Carlos; Brindle, Mary; Perrier, Renee; Thomas, Mary Ann

    2016-01-01

    Congenital lumbar hernias are rare malformations caused by defects in the development of the posterior abdominal wall. A known association exists with lumbocostovertebral syndrome; however other associated anomalies, including one case with arthrogryposis, have been previously reported. We present an infant girl with bilateral congenital lumbar hernias, multiple joint contractures, decreased muscle bulk and symptoms of malignant hyperthermia. Molecular testing revealed an R4861C mutation in the ryanodine receptor 1 (RYR1) gene, known to be associated with central core disease. This is the first reported case of the co-occurrence of congenital lumbar hernias and central core disease. We hypothesize that ryanodine receptor 1 mutations may interrupt muscle differentiation and development. Further, this case suggests an expansion of the ryanodine receptor 1-related myopathy phenotype to include congenital lumbar hernias. PMID:26684984

  4. Bilateral Ankle and Subtalar Joint Fusion Secondary to Guillain Barré-Induced Foot Drop.

    PubMed

    Seidel, Jay; Mathew, Bindu; Marks, Jeffrey

    2016-01-01

    Guillain-Barré syndrome is a serious disorder that occurs when the body's immune system mistakenly attacks the peripheral nervous system. This leads to nerve inflammation and damage that can cause muscle weakness and/or paralysis, including foot drop. Therapy ranges from supportive measures, such as physical therapy, to surgical intervention. It appears from the published data that these patients, generally, have been left to their own devices, because no known cure is available, necessitating reliance on others for their daily activities and ambulation. We present a unique surgical option, with a follow-up period of 24 months, in which bilateral subtalar and ankle fusion allowed unassisted ambulation to a patient who might otherwise have never walked again. PMID:25128306

  5. Behavioural Variant Frontotemporal Dementia with Bilateral Insular Hypometabolism: A Case Report

    PubMed Central

    Sood, Mamta; Bhad, Roshan; Tripathi, Manjari

    2016-01-01

    Fronto-Temporal Dementia (FTD) is a cluster of syndromes, characterized by progressive deterioration of cognition, language and/or behavioural changes associated with degeneration of the frontal and temporal lobes. A 53-year-old man was admitted with a history of gradually progressive behavioural disturbances, disinhibition, unprovoked anger outbursts, apathy, disorganised behaviour and impaired self-care. A clinical diagnosis of Fronto temporal Dementia (behavioural variant) was made. Extensive investigations found no abnormality except in FDG-PET scan of the brain which revealed hypo metabolism in bilateral anterior insular region. Insula is an important brain area implicated in emotional awareness and behaviour control. Hypo metabolism in insular region in the absence of any structural neuroimaging findings, in a case of behavioural variant of Fronto-temporal dementia suggest that, it might be one of the earliest neurobiological changes occurring in this disorder. PMID:27190928

  6. Diagnosis of osteopetrosis in bilateral congenital aural atresia: Turning point in treatment strategy.

    PubMed

    Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh

    2016-05-01

    Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934

  7. Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis

    PubMed Central

    Han, Kyoung Hee; Park, Ji Youn; Min, Seung-Kee; Ha, Il-Soo; Cheong, Hae Il

    2016-01-01

    Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%–5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS. PMID:27279890

  8. Bilateral atraumatic medial meniscal tears in a 17-year-old rower.

    PubMed

    Taylor, Taryn Lise; Frankovich, Renata; Rumball, Jane

    2009-01-01

    Meniscal injury produces disability in a large portion of the population, and sports injuries are a common cause. Atraumatic meniscal tears may occur after repetitive low-energy loading. Rowing is a highly technical sport and very demanding on an athlete's body. There are numerous reports on patellofemoral and iliotibial band friction syndrome in rowers but there is an extremely low incidence of meniscal tears reported in these athletes. This is a unique case report of a young adolescent athlete who suffered bilateral medical meniscal tears during sporting activity. Rowing is a low impact sport making this an unusual occurrence, especially in a young individual. This case report highlights the importance of considering all training activities when trying to isolate the mechanism of injury in an athlete. PMID:21686387

  9. Non-Familial Cherubism with Bilateral Maxilla and Mandible Involvement - Clinicoradiographic Findings.

    PubMed

    Ali, Ibrahim K; Karjodkar, Freny R; Sansare, Kaustubh; Salve, Prashant; Dora, Amaresh C

    2016-01-01

    Cherubism is a self-limiting non-neoplastic autosomal dominant fibro-osseous syndrome of the jaws. It is occasionally manifested before the age of two years. It occurs in children and more often in boys. It is characterized by notable clinical bilateral swelling of the cheeks due to a bony enlargement of the jaws that impart a characteristic 'cherubic' look. Regression occurs in the course of puberty leaving a few facial deformities and malocclusion. Cherubism might occur in solitary cases or in several members of the family, often in many generations. The reported case is an example of solitary sporadic occurrence within a family, which is a rarely documented condition in the literature. PMID:27588230

  10. Diagnosis of osteopetrosis in bilateral congenital aural atresia: Turning point in treatment strategy

    PubMed Central

    Verma, Ritu; Jana, Manisha; Bhalla, Ashu Seith; Kumar, Arvind; Kumar, Rakesh

    2016-01-01

    Aural atresia is a rare congenital malformation of the external and middle ear. There are several syndromic associations of this anomaly with those involving the first and second branchial arches. Occurrence of aural atresia with sclerosing skeletal dysplasia is unknown and has never been reported. The co-existence of a sclerosing dysplasia can make the surgical treatment in aural atresia difficult and risky; and the auditory improvement may not be as expected. Moreover, internal auditory canal narrowing and hence sensorineural hearing loss in sclerosing dysplasia might add to the already existing conductive hearing loss in such patients. In this case report we have described an unknown association of bilateral microtia with sclerosing skeletal dysplasia (autosomal dominant osteopetrosis) and clinical implications of these two conditions occurring together leading to a change in the management plan. PMID:27170934

  11. Bilateral iliac and popliteal arterial thrombosis in a child with focal segmental glomerulosclerosis.

    PubMed

    Han, Kyoung Hee; Park, Ji Youn; Min, Seung-Kee; Ha, Il-Soo; Cheong, Hae Il; Kang, Hee Gyung

    2016-05-01

    Thromboembolic complications (TECs) are clinically important sequelae of nephrotic syndrome (NS). The incidence of TECs in children is approximately 2%-5%. The veins are the most commonly affected sites, particularly the deep veins in the legs, the inferior vena cava, the superior vena cava, and the renal veins. Arterial thrombosis, which is less common, typically occurs in the cerebral, pulmonary, and femoral arteries, and is associated with the use of steroids and diuretics. Popliteal artery thrombosis in children has been described in cases of traumatic dissection, osteochondroma, Mycoplasma pneumoniae infection, and fibromuscular dysplasia. We report of a 33-month-old girl with bilateral iliac and popliteal arterial thrombosis associated with steroid-resistant NS due to focal segmental glomerulosclerosis. Her treatment involved thrombectomy and intravenous heparinization, followed by oral warfarin for 8 months. Herein, we report a rare case of spontaneous iliac and popliteal arterial thrombosis in a young child with NS. PMID:27279890

  12. Changes in bilateral bispectral index VISTA monitoring system during Wada test.

    PubMed

    Pacreu, S; Vila, E; Rodríguez, C; Arroyo, R; Fernández, S; Fernández, J L

    2014-12-01

    The Wada test is a procedure used in the preoperative assessment before epilepsy surgery in order to determine language lateralization, to assess the post-operative risk of an amnesia syndrome, and to evaluate the risk of material-specific memory deficits, in particular verbal memory deficits. This test involves inserting a cannula into the internal carotid artery via the femoral artery, and then to inject amobarbital to shut down brain function, usually in one of the brain hemispheres. The bilateral bispectral index (BIS) VISTA™ monitoring system (BVMS) was used to detect changes in EEG, and in the power spectrum distribution using the density spectral array (DSA) of both hemispheres. We describe a patient with an agenesis of the A1 segment of the right anterior cerebral artery, scheduled for a Wada test, in whom the BVMS demonstrated its potential value. PMID:24657004

  13. Total dream loss: a distinct neuropsychological dysfunction after bilateral PCA stroke.

    PubMed

    Bischof, Matthias; Bassetti, Claudio L

    2004-10-01

    The term Charcot-Wilbrand syndrome (CWS) denotes dream loss following focal brain damage. We report the first case of CWS, in whom neuropsychological functions, extension of the underlying lesion, and sleep architecture changes were assessed. A 73-year-old woman reported a total dream loss after acute, bilateral occipital artery infarction (including the right inferior lingual gyrus), which lasted for over 3 months. In the absence of sleep-wake complaints and (other) neuropsychological deficits, polysomnography demonstrated an essentially normal sleep architecture with preservation of REM sleep. Dreaming was denied also after repeated awakenings from REM sleep. This observation suggests that CWS (1) can represent a distinct and isolated neuropsychological manifestation of deep occipital lobe damage, and (2) may occur in the absence of detectable REM sleep abnormalities. PMID:15389890

  14. Delayed tumor resection in a 5-year-old child with bilateral Wilms tumor.

    PubMed

    Carmichael, Samuel P; Pulliam, Joseph F; D'Orazio, John A

    2013-01-01

    We describe the case of a 5-year-old girl whose abdominal pain and distension were caused by Wilms tumor of the kidney. Because of the bilateral nature of her disease, she was spared biopsy or initial nephrectomy as part of her treatment course. Rather, she was treated presumptively for Wilms tumor based primarily on radiologic findings. Neoadjuvant chemotherapy consisting of vincristine, dactinomycin and doxorubicin was given to facilitate nephron-sparing surgery for tumor resection. Her initial chemotherapeutic course was complicated by tumor lysis syndrome manifested by elevated serum uric acid and was treated effectively with hyperhydration and alkalization of intravenous fluids. The patient's disease responded well to chemotherapy, and she underwent successful tumor excision after 12 weeks of chemotherapy. The resected tumor was identified as anaplastic Wilms tumor, illustrating that pathologic identification of Wilms tumor is possible even after multiple cycles of neoadjuvant chemotherapy and marked tumor shrinkage. PMID:24964423

  15. Strongyloides stercoralis induced bilateral blood stained pleural effusion in patient with recurrent Non-Hodgkin lymphoma.

    PubMed

    Win, T T; Sitiasma, H; Zeehaida, M

    2011-04-01

    Infections and malignancies are common causes of pleural effusion. Among infectious causes, hyperinfection syndrome of Strongyloides stercoralis may occur in immunosuppressive patient. A 62-year-old man, known case of Non-Hodgkin lymphoma (NHL) was presented with recurrent NHL stage IV and had undergone salvage chemotherapy. Patient subsequently developed pneumonia with bilateral pleural effusion and ascites. We reported rhabditiform larvae of S. stercoralis in pleural fluid of both lungs without infiltration by lymphoma cells. Stool for microscopic examination also revealed rhabditiform larvae of S. stercoralis. This patient was a known case of NHL receiving chemotherapy resulting in immunosuppression state. Although S. stercoralis infection is not very common compared to other parasitic infections, it is common in immunosuppressive patients and may present with hyperinfection. Therefore, awareness of this parasite should be kept in mind in immunosuppressive patients. PMID:21602770

  16. Non-Familial Cherubism with Bilateral Maxilla and Mandible Involvement – Clinicoradiographic Findings

    PubMed Central

    Karjodkar, Freny R; Sansare, Kaustubh; Salve, Prashant; Dora, Amaresh C

    2016-01-01

    Cherubism is a self-limiting non-neoplastic autosomal dominant fibro-osseous syndrome of the jaws. It is occasionally manifested before the age of two years. It occurs in children and more often in boys. It is characterized by notable clinical bilateral swelling of the cheeks due to a bony enlargement of the jaws that impart a characteristic ‘cherubic’ look. Regression occurs in the course of puberty leaving a few facial deformities and malocclusion. Cherubism might occur in solitary cases or in several members of the family, often in many generations. The reported case is an example of solitary sporadic occurrence within a family, which is a rarely documented condition in the literature. PMID:27588230

  17. Moebius syndrome and narcolepsy: A case dissertation☆

    PubMed Central

    Sabaneeff, Lídia; Mendes Motta, Henrique; Castro, Juliana; Tufik, Sergio; Santos Coelho, Fernando Morgadinho

    2014-01-01

    Moebius syndrome (MS) is a congenital syndrome characterized by unilateral or bilateral aplasia of the VI and VII cranial nerves, with consequent convergent strabismus and bilateral peripheral facial paralysis. This syndrome might be associated with diurnal excessive sleepiness and muscular hypotony, mimetizing in this manner, narcolepsy. The diagnostic criteria for narcolepsy depend on the presence of REM sleep during the day. As with patients with MS we do not have ocular movements due to the VI nerve paralysis, the absence of horizontal ocular movements might make it difficult to confirm narcolepsy in these patients. The common clinical characteristics of these patients are due to a possible impairment of the same structures that are affected in the central nervous system. However, the mechanism by which it occurs remains to be fully understood. Further electrophysiological researches are necessary to better clarify the association of these two diseases. The objective of this dissertation is to describe and discuss a case of Moebius syndrome with diurnal excessive sleepiness as a differential diagnosis for narcolepsy. PMID:26483899

  18. Moebius syndrome and narcolepsy: A case dissertation.

    PubMed

    Sabaneeff, Lídia; Mendes Motta, Henrique; Castro, Juliana; Tufik, Sergio; Santos Coelho, Fernando Morgadinho

    2014-03-01

    Moebius syndrome (MS) is a congenital syndrome characterized by unilateral or bilateral aplasia of the VI and VII cranial nerves, with consequent convergent strabismus and bilateral peripheral facial paralysis. This syndrome might be associated with diurnal excessive sleepiness and muscular hypotony, mimetizing in this manner, narcolepsy. The diagnostic criteria for narcolepsy depend on the presence of REM sleep during the day. As with patients with MS we do not have ocular movements due to the VI nerve paralysis, the absence of horizontal ocular movements might make it difficult to confirm narcolepsy in these patients. The common clinical characteristics of these patients are due to a possible impairment of the same structures that are affected in the central nervous system. However, the mechanism by which it occurs remains to be fully understood. Further electrophysiological researches are necessary to better clarify the association of these two diseases. The objective of this dissertation is to describe and discuss a case of Moebius syndrome with diurnal excessive sleepiness as a differential diagnosis for narcolepsy. PMID:26483899

  19. Evaluation of findings in Crouzon's syndrome.

    PubMed

    Altintas, Ayse Gül Koçak; Gül Aksoy, Fatma Gül; Altintas, Cumhur S.; Midillioglu, Inci Koçak; Duman, Sunay

    1999-12-01

    PURPOSE. The purpose of this study was to investigate the ocular, facial and radiological signs of Crouzon's syndrome in a group of older patients who had not undergone previous craniofacial surgery. METHODS. Six cases of Crouzon's syndrome, four of whom belonged to a three-generation family, were examined systemically, ophthalmologically and roentgenographically; five of these cases were additionally evaluated with computed tomography and compared with Apert syndrome. RESULTS. On radiologic evaluation, all cases had synostosis of all cranial sutures and fontanelles and brachycephalic skulls, crowding of the upper teeth due to maxillary hypoplasia, and serious nasal septum deviation. Bilateral ethmoidal, maxillary and sphenoidal chronic sinusitis was found in 50% of cases. Three patients had hypertelorism and two others had a tendency toward hypertelorism. The typical facial appearance with shallow orbits, globe protrusion and exorbitism was present in all cases. Two of them had V-pattern exotropia while the other four were orthophoric. The single case with mental retardation had bilateral cataract. CONCLUSION. Crouzon's syndrome can present with different findings and must be evaluated multidisciplinarly. PMID:12045969

  20. [Bilateral carotid dissection. A not to underestimate cause of neurological loss after road accident].

    PubMed

    Stock, K; Peters, S; Behrmann, C; Spielmann, R P

    2002-10-01

    The dissection of the internal carotid artery is a rare complication of acceleration traumas of the upper spine. 30% of these dissections are caused by road accidents and again less than 30% of these occur bilateral as shown here. The symptoms are fronto-temporal and periorbital starting headaches spreading out to the occiput and Horner's syndrome. Complete hemiplegia as in our case is an impressive exception but the doctor in attendance should think of the carotid dissection. The exclusion of this complication is obligatory because treatment and outcome depend on it.The dynamic effects of bilateral carotid dissections may, as shown here, lead to relapsing cerebral infarctions with persisting neurologic deficits up to manifest hemiparesis. But restitution can be accomplished if early diagnosed by DSA and/or MRI. Therapy of choice is early prevention of persisting neurologic deficits using effective dosed heparin and depending on the residual lumen of the vessel oral anticoagulants or platelet antagonists for one year. PMID:12376897