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Sample records for bilateral perisylvian syndrome

  1. 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

  2. Connectivity between Perisylvian and Bilateral Basal Temporal Cortices

    PubMed Central

    Lesser, Ronald P.; Sinai, Alon; Gaillard, William D.; Franaszczuk, Piotr J.; Crone, Nathan E.

    2012-01-01

    Language processing requires the orchestrated action of different neuronal populations, and some studies suggest that the role of the basal temporal (BT) cortex in language processing is bilaterally distributed. Our aim was to demonstrate connectivity between perisylvian cortex and both BT areas. We recorded corticocortical evoked potentials (CCEPs) in 8 patients with subdural electrodes implanted for surgical evaluation of intractable epilepsy. Four patients had subdural grids over dominant perisylvian and BT areas, and 4 had electrode strips over both BT areas and left posterior superior temporal gyrus (LPSTG). After electrocortical mapping, patients with grids had 1-Hz stimulation of language areas. Patients with strips did not undergo mapping but had 1-Hz stimulation of the LPSTG. Posterior language area stimulation elicited CCEPs in ipsilateral BT cortex in 3/4 patients with left hemispheric grids. CCEPs were recorded in bilateral BT cortices in 3/4 patients with strips upon stimulation of the LPSTG, and in the LPSTG in the fourth patient upon stimulation of either BT area. This is the first in vivo demonstration of connectivity between LPSTG and both BT cortices. The role of BT cortex in language processing may be bilaterally distributed and related to linking visual information with phonological representations stored in the LPSTG. PMID:21715651

  3. Cochlear implantation in a patient with Perisylvian syndrome.

    PubMed

    Smith, Wendy; Axon, Patrick

    2007-06-01

    Perisylvian syndrome is a rare neurological disorder characterised by the partial paralysis of muscles, epilepsy and mild to severe mental retardation. It is associated with hearing loss and delay in language and speech development. This presents additional challenges in the assessment of whether a child is suitable for cochlea implantation. The method to determine whether the hearing loss is of cochlear or central origin and the progress of a child with Perisylvian syndrome who received a cochlear implant is discussed. PMID:17549806

  4. [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

  5. Perisylvian polymicrogyria, infantile spasms and arthrogryposis: the severe end of the spectrum of congenital bilateral perisylvian polymicrogyria.

    PubMed

    De Coene, Anja; Van Coster, Rudy; Verhelst, Helene

    2010-05-01

    Congenital bilateral perisylvian polymicrogyria (CBPP) is the most frequent type of polymicrogyria in children. A 3-month-old male patient is described here with the combination of CBPP, infantile spasms and arthrogryposis. Only four patients have been reported earlier in the literature with this combination. Three of them had epilepsy. These patients represent the more severe phenotype of CBPP, characterized by early onset of symptoms, epilepsy, mental retardation, pseudobulbar palsy and arthrogryposis. PMID:19559633

  6. Anatomical predictors of aphasia recovery: a tractography study of bilateral perisylvian language networks.

    PubMed

    Forkel, Stephanie J; Thiebaut de Schotten, Michel; Dell'Acqua, Flavio; Kalra, Lalit; Murphy, Declan G M; Williams, Steven C R; Catani, Marco

    2014-07-01

    Stroke-induced aphasia is associated with adverse effects on quality of life and the ability to return to work. For patients and clinicians the possibility of relying on valid predictors of recovery is an important asset in the clinical management of stroke-related impairment. Age, level of education, type and severity of initial symptoms are established predictors of recovery. However, anatomical predictors are still poorly understood. In this prospective longitudinal study, we intended to assess anatomical predictors of recovery derived from diffusion tractography of the perisylvian language networks. Our study focused on the arcuate fasciculus, a language pathway composed of three segments connecting Wernicke's to Broca's region (i.e. long segment), Wernicke's to Geschwind's region (i.e. posterior segment) and Broca's to Geschwind's region (i.e. anterior segment). In our study we were particularly interested in understanding how lateralization of the arcuate fasciculus impacts on severity of symptoms and their recovery. Sixteen patients (10 males; mean age 60 ± 17 years, range 28-87 years) underwent post stroke language assessment with the Revised Western Aphasia Battery and neuroimaging scanning within a fortnight from symptoms onset. Language assessment was repeated at 6 months. Backward elimination analysis identified a subset of predictor variables (age, sex, lesion size) to be introduced to further regression analyses. A hierarchical regression was conducted with the longitudinal aphasia severity as the dependent variable. The first model included the subset of variables as previously defined. The second model additionally introduced the left and right arcuate fasciculus (separate analysis for each segment). Lesion size was identified as the only independent predictor of longitudinal aphasia severity in the left hemisphere [beta = -0.630, t(-3.129), P = 0.011]. For the right hemisphere, age [beta = -0.678, t(-3.087), P = 0.010] and volume of the long

  7. Anatomical predictors of aphasia recovery: a tractography study of bilateral perisylvian language networks.

    PubMed

    Forkel, Stephanie J; Thiebaut de Schotten, Michel; Dell'Acqua, Flavio; Kalra, Lalit; Murphy, Declan G M; Williams, Steven C R; Catani, Marco

    2014-07-01

    Stroke-induced aphasia is associated with adverse effects on quality of life and the ability to return to work. For patients and clinicians the possibility of relying on valid predictors of recovery is an important asset in the clinical management of stroke-related impairment. Age, level of education, type and severity of initial symptoms are established predictors of recovery. However, anatomical predictors are still poorly understood. In this prospective longitudinal study, we intended to assess anatomical predictors of recovery derived from diffusion tractography of the perisylvian language networks. Our study focused on the arcuate fasciculus, a language pathway composed of three segments connecting Wernicke's to Broca's region (i.e. long segment), Wernicke's to Geschwind's region (i.e. posterior segment) and Broca's to Geschwind's region (i.e. anterior segment). In our study we were particularly interested in understanding how lateralization of the arcuate fasciculus impacts on severity of symptoms and their recovery. Sixteen patients (10 males; mean age 60 ± 17 years, range 28-87 years) underwent post stroke language assessment with the Revised Western Aphasia Battery and neuroimaging scanning within a fortnight from symptoms onset. Language assessment was repeated at 6 months. Backward elimination analysis identified a subset of predictor variables (age, sex, lesion size) to be introduced to further regression analyses. A hierarchical regression was conducted with the longitudinal aphasia severity as the dependent variable. The first model included the subset of variables as previously defined. The second model additionally introduced the left and right arcuate fasciculus (separate analysis for each segment). Lesion size was identified as the only independent predictor of longitudinal aphasia severity in the left hemisphere [beta = -0.630, t(-3.129), P = 0.011]. For the right hemisphere, age [beta = -0.678, t(-3.087), P = 0.010] and volume of the long

  8. [Case report: bilateral Cushing's syndrome].

    PubMed

    Cheikhrouhou, Héla; Khiari, Karima; Chérif, Lotfi; Ben Abdallah, Néjib; Ben Maïz, Hédi

    2003-04-01

    The authors report a case of a 49-year-old woman presenting a Cushing's syndrome (January 1997). The Magnetic Resonance Image of the pituitary gland revealed a microadenoma without extension in the cavernous sinus and a partial empty sella. The computed tomography scan showed a discreet bilateral adrenal hyperplasia with a left nodule (23 mm in diameter) a second nodule was noted. These data suggested the eventuality of maconodular adrenocortical hyperplasia in long-standing Cushing's disease. We discuss the implications of this finding for diagnosis, treatment and follow-up.

  9. Sequential presentation of bilateral Brown syndrome.

    PubMed

    Sekeroğlu, Hande Taylan; Türkçüoğlu, Peykan; Sanaç, Ali Şefik; Sener, Emin Cumhur

    2012-04-01

    Brown syndrome, characterized by a limitation of elevation in adduction and positive forced duction testing, is usually unilateral but occurs bilaterally in 10% of all cases. It may present as a congenital condition in one eye and develop in the other eye with no apparent cause. We present a case of bilateral Brown syndrome in which the right eye became involved within 1 year of surgery on the left eye for congenital Brown syndrome.

  10. Bilateral carpal tunnel syndrome in Graves' disease.

    PubMed

    Manganelli, P; Pavesi, G; Salaffi, F

    1987-01-01

    Carpal tunnel syndrome (CTS) may be associated with endocrinopathies, such as hypothyroidism and acromegaly. A direct relationship between CTS and hyperthyroidism has recently been suggested. We now report a case in which bilateral CTS developed after treatment of Graves' disease, thus, questioning the possibility of a relationship between these two disease processes.

  11. 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

  12. 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.

  13. Bilateral subclavian steal syndrome with vertigo.

    PubMed

    Yamanaka, Toshiaki; Sawai, Yachiyo; Hosoi, Hiroshi

    2014-06-01

    Subclavian steal syndrome (SSS) is usually caused by unilateral subclavian artery (SA) occlusion, and bilateral SSS is very rare. Takayasu's arteritis (TA) is a chronic granulomatous form of vasculitis that affects the SA, most commonly in women aged 15-40 years. We report a rare case of bilateral SSS due to TA in a 52-year-old woman, who exhibited severe vertigo. Although her blood pressure was within the normal range and did not differ between her arms, Doppler ultrasonography revealed low antegrade blood flow in the right SA and retrograde flow in the left SA. Computed tomography angiography demonstrated complete obstruction of the bilateral SA proximal to the vertebral artery origin. The more marked decrease in the blood flow of the vertebrobasilar artery experienced in bilateral SSS compared with unilateral SSS is considered to have caused the severe rotatory vertigo in the present patient. Since the vascular conditions of TA progressively deteriorate, delayed diagnosis and treatment could result in poor outcomes and unfavorable prognosis. We suggest that SSS with TA might require an early detection and treatment as well as careful follow-up for preventing vertigo and other neurological deficits in the vertebrobasilar arterial region.

  14. [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

  15. 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.

  16. 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.

  17. Bilateral adrenal haemorrhagic infarction in a patient with antiphospholipid syndrome.

    PubMed

    Godfrey, Rebecca Louise; Clark, James; Field, Benjamin

    2014-11-19

    A 68-year-old woman with antiphospholipid syndrome presented with a 3-day history of bilateral loin pain, vomiting, fever and confusion. On examination she was febrile, hypotensive and tachycardic. Investigations revealed raised inflammatory markers, renal impairment and hyponatraemia. Abdominal ultrasound revealed two well-defined heterogeneous areas bilaterally in the region of the adrenal glands. This prompted serum cortisol measurement and a CT of the abdomen. Cortisol was low in the context of sepsis at 48 nmol/L, and CT confirmed bilateral heterogeneous adrenal pathology. The patient was managed for septic shock and adrenal insufficiency. She was recognised to have several risk factors for haemorrhagic infarction of the adrenals: antiphospholipid syndrome, sepsis, postoperative state and anticoagulant therapy. She was discharged well on glucocorticoid and mineralocorticoid therapy and a repeat CT at 4 weeks confirmed the diagnosis of bilateral adrenal infarct and haemorrhage.

  18. 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

  19. Catastrophic antiphospholipid syndrome presenting as bilateral central retinal artery occlusions.

    PubMed

    Saraf, Steven S; Patel, Yogin P; Desai, Ankit; Desai, Uday R

    2015-01-01

    A previously healthy 22-year-old African American woman presented with bilateral vision loss associated with headache. Her ocular examination was significant for bilateral retinal arterial "boxcarring," retinal whitening, retinal hemorrhages, and cherry red spots. She was diagnosed with bilateral central retinal artery occlusions and was hospitalized due to concomitant diagnosis of stroke and hypercoagulable state. She was also found to be in heart failure and kidney failure. Rheumatology was consulted and she was diagnosed with catastrophic antiphospholipid syndrome in association with systemic lupus erythematosus. Approximately 7 months after presentation, the patient's vision improved and remained stable at 20/200 and 20/80. PMID:25722904

  20. Recurrent bilateral occipital infarct with cortical blindness and anton syndrome.

    PubMed

    Kwong Yew, Kiu; Abdul Halim, Sanihah; Liza-Sharmini, Ahmad Tajudin; Tharakan, John

    2014-01-01

    Bilateral cortical blindness and Anton syndrome, are most commonly caused by ischaemic stroke. In this condition, patients have loss of vision but deny their blindness despite objective evidence of visual loss. We report a case of a patient with multiple cardiovascular risk factors who developed recurrent bilateral occipital lobe infarct with Anton syndrome. A suspicion of this condition should be raised when the patient has denial of blindness in the presence of clinical and radiological evidence of occipital lobe injury. Management of this condition should focus on the underlying cause, in which our patient requires secondary stroke prevention and rehabilitation. PMID:24744933

  1. Acute exercise-induced bilateral thigh compartment syndrome.

    PubMed

    Boland, Michael R; Heck, Chris

    2009-03-01

    Acute compartment syndrome of the thigh is rare due to the space's ability to accommodate large volumes of fluid and, with the exception of the lateral septum, its thin compliant linings. This article describes a case of bilateral exercise-induced severe compartment syndrome treated with anterior and posterior fasciotomies. A 29-year-old man was admitted to intensive care with myoglobinuria. His left thigh was evaluated 18 hours later for compartment syndrome. The patient reported that 14 hours prior to initial presentation, he had participated in a 1-hour session of vigorous basketball. He gradually developed bilateral moderately severe thigh pain and tea-colored urine. Physical examination revealed pain secondary to passive stretch of both knees at 20 degrees flexion, plus firm anterior and posterior compartments to palpation. A handheld pressure monitor revealed the following compartment pressures: left anterior 80 mm Hg; left posterior 75 mm Hg; right anterior 45 mm Hg; and right posterior 50 mm Hg. Bilateral emergent anterior and posterior compartment fasciotomies were performed. The patient developed a significant severe distal motor and sensory neurological deficit on the left side, which recovered to 3/5 motor strength and protective sensation. At 6-month follow-up, he ambulated with the assistance of a left ankle foot orthosis. Acute severe compartment syndrome can occur following vigorous exercise. We recommend fasciotomies after exercise-induced acute compartment syndrome rather than initial observation because of the severity of morbidity associated with undertreated compartment syndrome.

  2. 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

  3. 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

  4. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report

    PubMed Central

    Acharya, Sonu; Panda, Swagatika; Sahoo, Sujit Ranjan; Ray, Prayas

    2013-01-01

    ABSTRACT Gorlin's syndrome is a rare disorder transmitted as an autosomal dominant trait. It is characterized by multiple disorders involving multiple systems. We present a case of 11-year-old male child presenting with multiple odontogenic keratocyst to the dental clinic. Retrograde diagnosis of Gorlin-Goltz syndrome was made after clinical and radiological investigation. How to cite this article: Acharya S, Panda S, Dhull KS, Sahoo SR, Ray P. Gorlin Syndrome with Bilateral Polydactyly: A Rare Case Report. Int J Clin Pediatr Dent 2013;6(3):208-212. PMID:25206225

  5. 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

  6. Bilaterally Symmetrical Lower Extremity Compartment Syndrome following Massive Transfusion.

    PubMed

    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.

  7. 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

  8. 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

  9. 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.

  10. 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

  11. 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

  12. 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

  13. 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

  14. Craniofacial Microsomia: Goldenhar Syndrome in Association with Bilateral Congenital Cataract

    PubMed Central

    Shrestha, U. D.; Adhikari, S.

    2015-01-01

    Craniofacial microsomia (CFM) includes a spectrum of malformations primarily involving structures derived from the first and second branchial arches. Patients with hemifacial microsomia and epibulbar dermoids are said to have Goldenhar syndrome (GHS). Four-month-old boy with whitish pupillary reflex presented with the features of GHS in pediatric ophthalmology clinic. The child had ocular and auricular manifestations. There were no vertebral anomalies, but he had bilateral congenital cataract. The peculiarity of this case is the presence of the bilateral total congenital cataract, in association with CFM. There is absence of epibulbar dermoid or lipodermoid in the eyes, although the child had features of GHS. In addition to it, anesthetic intubation was smooth in this case. Any case diagnosed with CFM and/or GHS needs treatment through multidisciplinary approach, consultation in ophthalmology department is one of them. PMID:26635984

  15. Bilateral Lacrimal Gland Lymphoma in Sjögren Syndrome

    PubMed Central

    Ozsan, Nazan; Sahin, Fahri

    2016-01-01

    A 31-year-old female with Primary Sjögren Syndrome (pSS) presented with bilateral puffiness around the eye for 3 years. The lacrimal glands were hypertrophic and edematous bilaterally. Schirmer 1 score was 2 and 1 mm and tear-film break-up time was 3 and 4 seconds, in the right and the left eyes, respectively. An incisional biopsy from the left lacrimal gland revealed diffuse and intense CD20, CD5, and bcl-2 positivity with negative cyclin D1 and CD23 which supported lymphoma. Upon haematology consultation extranodal marginal zone lymphoma diagnosis was made. CHOP (cyclophosphamide, doxorubicin, vincristine, and methyl prednisolone) treatment was initiated. In conclusion, pSS is a well known autoimmune disease in which increased rate of lymphoma is present. Early detection with histopathologic confirmation and multidisciplinary approach with ophthalmology, rheumatology, and haematology are mandatory in these patients. PMID:27738539

  16. 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

  17. [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

  18. 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

  19. Spontaneous bilateral compartment syndrome in a HIV-positive patient.

    PubMed

    Davidson, Donald James; Shaukat, Yasir Mehmood; Jenabzadeh, Reza; Gupte, Chinmay M

    2013-01-01

    Spontaneous bilateral compartment syndrome is a very rare condition but one which requires swift diagnosis and urgent surgical decompression by fasciotomies in order to achieve the best outcome. We present the case of a 31-year-old HIV-positive man. The case highlights the perils of being sidetracked by an atypical clinical history instead of acting on the classical clinical examination findings. We will discuss the presentation and management of this patient, review the literature and highlight the key learning points. The most important learning point being that no matter how atypical the history, if a patient presents with limb pain out of proportion to the injury (with or without pain on passive stretch), sensory changes and a loss of motor power, then a diagnosis of acute compartment syndrome must be considered.

  20. 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

  1. Magnetoencephalography in Landau-Kleffner syndrome.

    PubMed

    Paetau, Ritva

    2009-08-01

    Magnetoencephalography (MEG) detects weak magnetic fields outside the head. Spikes generated on the perisylvian convexity are detected only by electroencephalography (EEG), whereas spikes with intrasylvian generators are selectively seen by MEG. Generators of MEG and EEG spikes are determined using equivalent current dipoles (ECDs) that represent local cortical activity in parallel pyramidal neurons. MEG localizes cortical spike generators with a 1-2-cm spatial accuracy, and with a millisecond time resolution. This allows tracking of neural activity over successive synaptic connections in the cortical network. Both EEG and MEG are necessary for comprehensive spatial and temporal description of perisylvian epileptic networks in the Landau-Kleffner syndrome (LKS). MEG studies suggest that in more than 80% of LKS patients, the bilateral epileptic discharges are generated in the auditory- and language-related perisylvian cortex. Approximately 20% of children with LKS children have a unilateral perisylvian pacemaker that triggers secondary bilateral synchrony of spikes. This 20% may regain considerable language skills after multiple subpial transections (MSTs) of the pacemaker area. Outcome data of a few surgery patients suggest an indispensable role of MEG when planning the most efficient therapy for patients with LKS.

  2. Unusual presentation of Sturge-Weber syndrome: Progressive megalencephaly with bilateral cutaneous and cortical involvement

    PubMed Central

    Mittal, Kundan; Kaushik, Jaya Shankar; Kaur, Gurpreet; Aamir, Mohd; Sharma, Suvasini

    2014-01-01

    The Sturge Weber syndrome is characterized by developmental delay, seizures in infancy, unilateral cutaneous lesions with ipsilateral leptomeningeal enhancement. We report an unusual presentation of Sturge Weber syndrome with bilateral port wine nevus on the trunk and face along with bilateral cortical involvement in a developmentally normal child with progressive megalencephaly. PMID:25024575

  3. Bilateral phlegmasia dolens associated with Trousseau's syndrome: a case report.

    PubMed

    Hasegawa, Satoshi; Aoyama, Tomoki; Kakinoki, Ryosuke; Toguchida, Junya; Nakamura, Takashi

    2008-06-01

    Phlegmasia dolens is a rare disorder caused by massive venous thrombosis. The clinical condition is subclassified as phlegmasia cerulea dolens (PCD) and phlegmasia alba dolens. The 2 forms differ in the venous area affected and remaining blood flow, causing arterial involvement and resulting in a difference in therapeutic strategy and prognosis. In PCD, the mortality rate is high, and amputation for venous gangrene is needed. Phlegmasia alba dolens is a mild form of PCD; the collateral venous system is intact, and the prognosis is better than for PCD. We experienced a rare case of bilateral phlegmasia dolens associated with Trousseau's syndrome. The patient was diagnosed with phlegmasia alba dolens because the artery was not occluded. She was treated with anticoagulant therapy, the placement of a vena cava filter, and a rehabilitation program. The bilateral soleus muscles underwent necrosis, and the occurrence of equinus foot disturbed her walking, but careful rehabilitation with stretching, a range of passive motion exercises by a physical therapist, a tilting table, and ergometer training strengthened the muscles and allowed the patient to walk unaided 8 months after onset. Through this case, we hope to learn more about the pathogenesis of phlegmasia dolens and clinical treatment measures.

  4. Bilaterally cleft lip, limb defects, and haematological manifestations: Roberts syndrome versus TAR syndrome.

    PubMed

    Urban, M; Opitz, C; Bommer, C; Enders, H; Tinschert, S; Witkowski, R

    1998-09-23

    We report on a 13-year-old patient followed since birth. He is the only offspring of young, non-consanguineous German parents. His mother has an isolated left cleft of lip and a cleft palate. At birth, our patient presented with bilaterally cleft lip/cleft palate, phocomelia of upper limbs with normal hands, and mild symmetrical deficiencies of the long bones of the lower limbs. Haematological evaluation demonstrated a leukaemoid reaction during a urinary tract infection as well as intermittent thrombocytopenia and episodes of marked eosinophilia during the first two years of life. Intellectual development has been normal. Comparison with two similar cases from the literature suggests a non-random phenotypic overlap of Roberts syndrome (MIM 268300) and TAR syndrome (MIM 274000). Such clinical constellations may be key observations to understand the genetic relationship of Roberts syndrome and TAR syndrome in future phenotype-genotype correlations. PMID:9788553

  5. McCune-Albright syndrome presenting with unilateral macroorchidism and bilateral testicular masses.

    PubMed

    Khanna, Geetika; Kantawala, Kartikeya; Shinawi, Marwan; Sarwate, Sandhya; Dehner, Louis P

    2010-12-01

    Bilateral synchronous intratesticular masses are rare but can be caused by metastatic disease to the testicle, primary testicular masses or benign etiologies such as congenital adrenal hyperplasia and granulomatous orchitis. We present an unusual case of McCune-Albright syndrome presenting with unilateral testicular enlargement and bilateral testicular masses secondary to Sertoli cell hyperplasia. To our knowledge, this is a unique case of testicular masses secondary to McCune-Albright syndrome. PMID:20607225

  6. Bilateral endoscopic adrenalectomy for Cushing's syndrome in a patient with polycystic liver and kidney disease.

    PubMed

    Niemann, Ulf; Behrend, Matthias

    2004-02-01

    Microadenomectomy via a transsphenoidal approach is today's treatment of choice to achieve normal cortisol blood levels in patients with ACTH-secreting pituitary tumors. Should neurosurgery fail, bilateral adrenalectomy is recommended. Today the endoscopic, transabdominal or retroperitoneal adrenalectomy is regarded as the gold standard for the operation of endocrine-active adrenal tumors. Therefore, in principle, the question of the indication for the endoscopic operation no longer arises but only the question of the technical feasibility in individual cases. We report on a patient with a pituitary-dependent Cushing's syndrome after a twice-repeated unsuccessful transsphenoidal adenomectomy and with additional polycystic kidney and liver disease, who underwent bilateral retroperitoneoscopic adrenalectomy. Despite the massive enlargement of both kidneys, it was possible to safely perform a bilateral retroperitoneoscopic adrenalectomy. In the case of marked bilateral cystic kidneys, a bilateral retroperitoneoscopic adrenalectomy in Cushing's syndrome is technical possible and a safe and effective method of treatment.

  7. Megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome: a case report.

    PubMed

    Kariminejad, Ariana; Radmanesh, Farid; Rezayi, Ali-Reza; Tonekaboni, Seyed-Hasan; Gleeson, Joseph G

    2013-05-01

    This report describes a case of megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome in a 1-year-old boy, born to healthy nonconsanguineous parents. Megalencephaly and bilateral postaxial polydactyly of upper and lower limbs were noted at birth. He had profound developmental delay and moderate hypotonia. Magnetic resonance imaging (MRI) of the brain revealed hydrocephalus, polymicrogyria in both frontal lobes and perisylvian regions, and thin corpus callosum. Array-comparative genomic hybridization was normal. The patient's clinical and radiologic findings fit the classic description of megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome. The possible overlap between megalencephaly-polymicrogyria-polydactyly-hydrocephalus syndrome and other similar conditions is discussed.

  8. Occurrence of bilaterally independent epileptic spasms after a corpus callosotomy in West syndrome.

    PubMed

    Kobayashi, Katsuhiro; Endoh, Fumika; Toda, Yoshihiro; Oka, Makio; Baba, Hiroshi; Ohtsuka, Yoko; Yoshinaga, Harumi

    2016-01-01

    We report a patient with intractable West syndrome whose epileptic spasms (ESs) were initially bilaterally synchronous, as is typical; after a complete corpus callosotomy, however, bilaterally independent ESs originated in either hemisphere. Activity of probable cortical origin associated with ESs was detected by observing ictal gamma oscillations. Brain MRI revealed no structural abnormality before surgery. This case suggests that ESs with a hemispheric origin may appear generalized because of synchronizing effects in the corpus callosum in some patients.

  9. Bilateral compartment syndrome of the anterior thigh following functional fitness exercises: a case report.

    PubMed

    McDonald, Lucas S; Mitchell, Ronald J; Deaton, Travis G

    2012-08-01

    We present a case of delayed, acute bilateral exertional compartment syndrome of the anterior thigh induced by callisthenic exercise. Symptoms consisted of pain out of proportion to examination findings, inability to ambulate, and severe pain with knee flexion. Treatment consisted of bilateral thigh fasciotomies and supportive therapy for concomitant rhabdomyolysis. Full strength, range of motion, and return to all military duties were achieved by 4 months postinjury.

  10. Bilateral sudden sensorineural hearing loss as an initial presentation of myelodysplastic syndrome.

    PubMed

    Lee, Eun Jung; Yoon, Yong Joo

    2012-01-01

    This study reports an unusual case in which myelodysplastic syndrome presented bilateral sudden sensorineural hearing loss as the first symptom of the disease. The aural symptoms and signs such as tinnitus, dizziness, and hearing impairment of a hematologic disease are common. However, sudden hearing loss as the first manifestation of a hematologic disease is extremely rare. A 76-year-old woman presented with bilateral sudden hearing loss. The patient was found to have myelodysplastic syndrome during a workup for her hearing loss. Unfortunately, the patient's hearing loss did not improve after the medical treatment.

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

    PubMed

    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

  12. 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

  13. Bilateral anterior tarsal tunnel syndrome variant secondary to extensor hallucis brevis muscle hypertrophy in a ballet dancer: a case report.

    PubMed

    Tennant, Joshua N; Rungprai, Chamnanni; Phisitkul, Phinit

    2014-12-01

    We present a case of bilateral anterior tarsal tunnel syndrome secondary EHB hypertrophy in a dancer, with successful treatment with bilateral EHB muscle excisions for decompression. The bilateral presentation of this case with the treatment of EHB muscle excision is the first of its type reported in the literature. PMID:25457672

  14. Gómez-López-Hernández Syndrome: A Rare Cause of Bilateral Nonscarring Alopecia.

    PubMed

    Saricam, Merve Hatun; Tekin, Burak; Unver, Olcay; Ekinci, Gazanfer; Ergun, Tulin

    2015-01-01

    Gómez-López-Hernández syndrome is a rare neurocutaneous disorder characterized by the triad of rhombencephalosynapsis, parietal alopecia, and trigeminal anesthesia. We report a 16-year-old girl with bilateral parietotemporal alopecia in whom cranial magnetic resonance imaging revealed rhombencephalosynapsis, suggesting a diagnosis of Gómez-López-Hernández syndrome. Neurologic examination and neuroimaging may be warranted in select patients with parietal alopecia to exclude this uncommon entity. PMID:26391554

  15. 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

  16. 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.

  17. 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…

  18. Bilateral squamosal suture synostosis: A rare form of isolated craniosynostosis in Crouzon syndrome.

    PubMed

    Tandon, Yasmeen K; Rubin, Michael; Kahlifa, Mohamed; Doumit, Gaby; Naffaa, Lena

    2014-07-28

    Craniosynostosis is a pathologic condition which is characterized by the premature fusion of cranial sutures. It may occur alone or in association with other anomalies making up various syndromes. Crouzon syndrome is the most common craniosynostosis syndrome. Bicoronal sutures fusion is most commonly involved in Crouzon syndrome. There have only been a handful of cases of squamosal suture synostosis described in the surgery literature with the few ones described in Crouzon syndrome associated with other types of craniosynostosis. To the best of our knowledge, we are presenting the first case of isolated bilateral squamosal suture synostosis in a patient with Crouzon syndrome in a radiology journal with emphasis on its radiological appearance. PMID:25071892

  19. Bilateral Vision Loss after Delivery in Two Cases: Severe Preeclampsia and HELLP Syndrome

    PubMed Central

    Çelik, Gökhan; Eser, Ahmet; Günay, Murat; Yenerel, Nursal Melda

    2015-01-01

    Two patients with no symptoms of hypertension in their medical history before pregnancy were referred to the obstetrics emergency clinic with hypertension and visual complaints. After physical examination and laboratory tests, one of the patients was diagnosed with severe preeclampsia while the other was diagnosed with HELLP syndrome (Hemolysis-Elevated Liver enzymes-Low Platelets). Ocular examinations were performed after delivery due to the patients’ worsening visual complaints. The severe preeclamptic patient showed bilateral serous retinal detachment (SRD) while the patient with HELLP syndrome showed bilateral macular hemorrhage. Systemic blood pressure control was advised. The patients’ ocular findings and visual acuities improved in the follow-up periods. SRD and macular hemorrhage can be observed in patients with preeclampsia and HELLP syndrome as a result of the disruption of retinal and choroidal vasculature. PMID:27800247

  20. [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.

  1. 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

  2. Bilateral carpal tunnel syndrome and ulnar neuropathy at the elbow in a pizza chef.

    PubMed

    Vimercati, Luigi; Lorusso, Antonio; L'abbate, Nicola; Assennato, Giorgio

    2009-01-01

    A case of bilateral carpal tunnel syndrome and ulnar neuropathy at the elbow in a 22-year-old pizza chef is described. An on-site analysis revealed that job tasks performed by the worker exposed him to a combination of biomechanical risk factors. Patient history and workplace observations suggest that occupational physical exposure may have caused the bilateral entrapment neuropathies. The present report underlines the advisability of a detailed occupational history in the case of entrapment neuropathies of the upper limbs commonly regarded as being related to biomechanical occupational exposure.

  3. Bilateral coxitis in scleroderma-polymyositis overlap syndrome

    PubMed Central

    Berrada, Khadija; Abourazzak, Fatima Ezzahra; Houssaini, Ghita Sqalli; Kadi, Nadira; Tahiri, Latifa; Amrani, Kawthar; Khammar, Zineb; Lahlou, Meriam; Berrady, Rhizlane; Rabhi, Samira; Tizniti, Siham; Bono, Wafaa; Harzy, Taoufik

    2014-01-01

    Joint manifestations in scleroderma (Scl) and polymyositis (PM) are dominated by inflammatory arthralgia. Arthritis is less common and preferentially affects the hands, wrists, knees, and ankles. Involvement of the hip has been rarely reported in the literature. We report a case of coxitis diagnosed in a patient suffering from scleroderma-polymyositis overlap syndrome successfully treated by ultrasound-guided infiltration of triamcinolone hexacetonide PMID:27708891

  4. 46,XY female sex reversal syndrome with bilateral gonadoblastoma and dysgerminoma

    PubMed Central

    DU, XUE; ZHANG, XUHONG; LI, YONGMEI; HAN, YUKUN

    2014-01-01

    Sex reversal syndrome is a rare congenital condition of complete or disordered gonadal development leading to discordance between the genetic, gonadal and phenotypic sexes, including 46,XX and 46,XY. The gonadoblastoma on the Y-chromosome (GBY) region is associated with an increased risk of developing type II germ cell tumors/cancer. The present study reports a unique case of a phenotypically normal female (age 17 years), presenting with primary amenorrhea and later diagnosed with 46,XY female sex reversal syndrome. Following bilateral gonadectomy, bilateral gonadoblastoma and dysgerminoma were diagnosed. Thus, estrogen replacement therapy was administered periodically to promote the development of secondary sexual characteristics and menstruation, and to prevent osteoporosis. A four year follow-up showed no tumor recurrence and a regular menstrual cycle in this patient. PMID:25187804

  5. Acute bilateral useless hand syndrome: a rare presenting manifestation of vitamin B12 deficiency.

    PubMed

    Biyani, Sumant; Jha, Sneh Kumar; Pandey, Suchit; Shukla, Rakesh

    2015-10-16

    We report a case of bilateral useless hand syndrome, a rare presenting manifestation of vitamin B12 deficiency. A 38-year-old man, a strict vegetarian and a teacher by occupation, presented with acute onset clumsiness of both hands while performing fine movements. Detailed history-taking, examination of the patient and relevant investigations (complete blood count, serum vitamin B12 and MRI of the cervical spinal cord) were carried out. Laboratory analysis was suggestive of vitamin B12 deficiency and MRI demonstrated a lesion involving the posterior columns of the cervical cord. The patient was diagnosed as a case of non-compressive cervical myelopathy predominantly involving the posterior column due to vitamin B12 deficiency. Acute bilateral useless hand syndrome can be a rare presenting feature of vitamin B12 deficiency.

  6. Bilateral supplemental permanent maxillary lateral incisors in a non-syndromic patient

    PubMed Central

    Bhullar, Mandeep Kaur; Uppal, Amandeep Singh; Kochhar, Gulsheen Kaur; Singh, Ranjit

    2014-01-01

    A supernumerary tooth is an additional entity to the normal series and can be seen in all quadrants of the jaw. Occurrence may be single or multiple, unilateral or bilateral, erupted or impacted in one or in both jaws. Multiple supernumeraries are rare in individuals with no other associated disease or syndromes. The conditions commonly associated with an increased prevalence of supernumerary teeth include cleft lip and palate, cleidocranial dysplasia and Gardner syndrome. The supplemental supernumerary refers to a duplication of teeth in normal series and is found at the end of a tooth series. A supplemental tooth may closely resemble the teeth of the group to which it belongs or it may bear little resemblance in size or shape to the teeth with which it is associated. This report presents a case with bilateral supplemental upper permanent lateral incisors without any symptoms of associated disorders. PMID:25168674

  7. Bilateral Multifocal Renal Angiomyolipoma Associated with Wunderlich’s Syndrome in A Tuberous Sclerosis Patient

    PubMed Central

    Hulikanthimatt, Kiran Shankar; Awatti, Shreeharsha Mallappa; Narayanrao, Suresh Turuvekere

    2015-01-01

    Renal Angiomyolipoma (renal AML) is a benign clonal neoplasm with a incidence of 0.3-3%, occurring as isolated sporadic entity or in association with Tuberous sclerosis (TS) in 80% cases. Multiple, bilateral renal AML are considered diagnostic of Tuberous sclerosis. Wunderlich’s syndrome, a urological emergency is a spontaneous nontraumatic bleeding into subcapsular and or peri-renal space and is a life threatening complication of renal AML occurring in 10% cases which has to be timely diagnosed and treated. Here, we present an unusual case of TS with bilateral, multifocal renal AML in a 25-year-old female who presented with Wunderlich’s syndrome, which is a rare but life threatening complication that has to be suspected, timely diagnosed and treated. We have discussed the importance of early diagnosis, timely treatment, follow up and education of patient and relatives of the possible complications associated. PMID:26435953

  8. [Atypical symptoms of Fabry's disease: sudden bilateral deafness, lymphoedema and Lown-Ganong-Levine syndrome].

    PubMed

    Undas, Anetta; Ryś, Donata; Wegrzyn, Wojciech; Musiał, Jacek

    2002-11-01

    A 40-year-old man with Fabry disease, confirmed by decreased leukocyte alpha-galactosidase A activity in 2001, complained of sudden bilateral deafness, as evidenced by clinical history and audiometry. Magnetic resonance of the brain revealed features typical of Fabry disease. Other clinical manifestations of the disease included: angiokeratoma, mild proteinuria with normal renal function, lymphoedema of the lower limbs, pre-excitation syndrome, myocardial hypertrophy.

  9. Bilateral multicystic parotid masses in primary Sjögren syndrome.

    PubMed

    Toh, Alex S; Broomfield, Stephen J; Teh, Lee-Suan; Aslam, Muhammad B; Duncan, Gavin; Morar, Pradeep

    2011-10-01

    We report the case of a 48-year-old woman who presented with bilateral parotid gland enlargement as the initial manifestation of primary Sjögren syndrome. Magnetic resonance imaging demonstrated multiple areas of low-intensity signal mixed with foci of high-intensity signal, a finding suggestive of Sjögren syndrome. Autoimmune blood tests for Sjögren syndrome were positive, and a sublabial biopsy confirmed the diagnosis. Investigations for other conditions, including human immunodeficiency virus infection, were negative. After 5 years of follow-up, the parotid swellings remained essentially unchanged. We discuss the etiology of multicystic parotid gland pathology, its involvement in Sjögren syndrome, and the risk of lymphoma.

  10. Loeys-Dietz syndrome with bilateral radial head dislocations: a case report.

    PubMed

    Rustagi, Tarush; Agashe, Mandar V; Dhamele, Jaideep; Aroojis, Alaric J; Mehta, Rujuta

    2013-08-01

    Loeys-Dietz syndrome is characterised by vascular aneurysms, hypertelorism, and a bifid uvula. We report on an 11-year-old boy with Loeys-Dietz syndrome who presented with bilateral radial head dislocations and severe osteopaenia with changes of avascular necrosis in both hips causing an out-toeing, wide gait. Considering the poor prognosis for elbow movement and possible radial head dysplasia, surgical reduction of the radial heads was deferred. A subtrochanteric de-rotation osteotomy of the left hip was performed to improve the gait.

  11. 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.

  12. [Clinical presentations of the secondary bilateral synchronization syndrome in adults with epilepsy].

    PubMed

    Fedin, A I; Generalov, V O; Amcheslavskaia, E V; Mishniakova, L P; Sadykov, T R

    2008-01-01

    One thousand eight hundreds and eighty patients with symptomatic and cryptogenic forms of focal epilepsy have been studied. Ninety patients (4.7%) had the secondary bilateral synchronization (SBS) syndrome in the EEG. Criteria of the syndrome are the presence of focal epileptiform activity in the EEG and SBS detected during the long-term video-EEG monitoring. In this group of patients, secondary generalized tonic-clonic, myoclonic and complex partial seizures were found. The clinical presentations of focal epilepsy were similar to the syndromes of idiopathic epilepsy in most cases. Long-term video-EEG monitoring is a method of choice in the instrumental diagnostics of these forms of epilepsy which are different in genesis and prognosis but similar in the kinematic characteristics.

  13. Hearing rehabilitation with single-stage bilateral vibroplasty in a child with Franceschetti syndrome.

    PubMed

    Sargsyan, Sona; Rahne, Torsten; Kösling, Sabrina; Eichler, Gerburg; Plontke, Stefan K

    2014-05-01

    Hearing is of utmost importance for normal speech and social development. Even children who have mild or unilateral permanent hearing loss may experience difficulties with understanding speech, as well as problems with educational and psycho-social development. The increasing advantages of middle-ear implant technologies are opening new perspectives for restoring hearing. Active middle-ear implants can be used in children and adolescents with hearing loss. In addition to the well-documented results for improving speech intelligibility and quality of hearing in sensorineural hearing loss active middle-ear implants are now successfully used in patients with conductive and mixed hearing loss. In this article we present a case of successful, single-stage vibroplasty, on the right side with the fixation of the FMT on the stapes and PORP CLiP vibroplasty on the left side in a 6-year-old girl with bilateral mixed hearing loss and multiple dyslalia associated with Franceschetti syndrome (mandibulofacial dysostosis). CT revealed bilateral middle-ear malformations as well as an atretic right and stenotic left external auditory canal. Due to craniofacial dysmorphia airway and (post)operative, management is significantly more difficult in patients with a Franceschetti syndrome which in this case favoured a single-stage bilateral procedure. No intra- or postoperative surgical complications were reported. The middle-ear implants were activated 4 weeks after surgery. In the audiological examination 6 months after surgery, the child showed 100% speech intelligibility with activated implants on each side.

  14. [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

  15. 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

  16. Bilateral microphthalmos with unilateral superior cyst in a child with autism and CHARGE syndrome.

    PubMed

    Pushker, Neelam; Tinwala, Sana; Khurana, Saurbhi; Sen, S

    2013-04-01

    A case of autism with CHARGE syndrome with microphthalmos and a superior colobomatous cyst arising from the optic disc is reported. A 7-year-old boy presented with a gradually increasing mass, involving the superior orbit and upper eyelid of right eye of 3 years' duration. Clinical examination revealed bilateral microphthalmos with typical iris coloboma, posterior synechiae, and cataractous lens. Imaging revealed bilateral optic disc colobomas with a superior cyst in the right orbit. Intraoperatively, a single cystic lesion was seen in the superior orbit arising from the optic disc region. Histopathology was suggestive of a colobomatous cyst, positive for glial tissue [glial fibrillary acidic protein (GFAP) positive] and neuroretinal elements (synaptophysin positive) on immunohistochemistry. Genetic analysis revealed a normal karyotype (46, XY).

  17. 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.

  18. 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).

  19. 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

  20. [The trapped popliteal artery syndrome. About one bilateral case (author's transl)].

    PubMed

    Simon, J J; Soppelsa, A

    1981-03-01

    The authors report on a new case of trapped popliteal artery syndrome. It was bilateral and revealed on one side by acute thrombotic ischemia, leading on this occasion to discovery of the opposite trap. They insist on the extremely easy diagnosis based on a dynamic examination of the limb. The distal pulses must be felt in intermediary position, then after tensing of the gastrocnemia by dorsiflexion of the foot and hyper extension of the knee. Blood velocity measurement by Doppler probes and bifemoralangiography, both dynamic, must precede imperative surgery. PMID:7217240

  1. KCOT Occurring in Bilateral Maxillary Sinus in Non-Syndromic Patient

    PubMed Central

    Newaskar, Vilas; Rajmohan, Sushmita; Dashore, Dolly

    2016-01-01

    Odontogenic Keratocyst (OKC) also termed as Keratocystic Odontogenic Tumour (KCOT) (WHO 2005) is a pathology with unique behavior because of which it is under much scrutiny and continued study. The pathology usually presents itself commonly in mandible and less commonly in maxilla. The occurrence of KCOT in maxillary sinus is reported as rare and multiple occurrences are mostly associated along with the presence of Nevoid Basal Cell Carcinoma (NBCC) syndrome. Here, we present a rare case of bilateral Maxillary OKC involving maxillary sinuses, without the presence of NBCC syndrome. An interesting feature of this case is the presence of left upper third molar in ectopic position in maxillary sinus and a vertically impacted right third molar suggesting an origin from the dental lamina. PMID:27656578

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

    PubMed

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

    2010-06-01

    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.

  3. 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

  4. KCOT Occurring in Bilateral Maxillary Sinus in Non-Syndromic Patient.

    PubMed

    Newaskar, Vilas; Verma, Manish; Rajmohan, Sushmita; Dashore, Dolly

    2016-08-01

    Odontogenic Keratocyst (OKC) also termed as Keratocystic Odontogenic Tumour (KCOT) (WHO 2005) is a pathology with unique behavior because of which it is under much scrutiny and continued study. The pathology usually presents itself commonly in mandible and less commonly in maxilla. The occurrence of KCOT in maxillary sinus is reported as rare and multiple occurrences are mostly associated along with the presence of Nevoid Basal Cell Carcinoma (NBCC) syndrome. Here, we present a rare case of bilateral Maxillary OKC involving maxillary sinuses, without the presence of NBCC syndrome. An interesting feature of this case is the presence of left upper third molar in ectopic position in maxillary sinus and a vertically impacted right third molar suggesting an origin from the dental lamina. PMID:27656578

  5. Can response time be trained with bilateral limb training in children with Down syndrome?

    PubMed Central

    Rao, Pratiksha Tilak; Solomon, John M.

    2015-01-01

    Aims: Response time (RT), that is, the time taken to respond is known to be delayed in children with Down syndrome (DS). We performed a pilot study to evaluate whether bilateral limb training can be used to train RT, in children with DS. Settings and Design: 10 children with DS (5 males) were recruited from a special school in a suburban region using convenience sampling. Subjects and Methods: Response time was measured using an indigenously developed RT Analyzer, before and after intervention, from right and left hand. Structured bilateral limb training was given for a period of 4 weeks, using low-cost, locally available materials, in community settings. Statistical Analysis Used: The Wilcoxon signed ranks test was used for statistical analysis. Results: Significant improvements in RT following 4 weeks of intervention were seen in the left hand (P = 0.006) but not in the right hand (P = 0.104). Conclusions: Response time can be trained in children with DS using 4 weeks of bilateral limb training activities using low-cost, locally available materials. PMID:26167016

  6. 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.

  7. 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

  8. 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

  9. 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

  10. 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

  11. A rare differential diagnosis to occupational neck pain: bilateral stylohyoid syndrome.

    PubMed

    Kirchhoff, Gertrud; Kirchhoff, Chlodwig; Buhmann, Sonja; Kanz, Karl-Georg; Lenz, Miriam; Vogel, Tobias; Kichhoff, Rainer Maria

    2006-06-26

    Chronic neck pain is widely prevalent and a common source of disability in the working-age population. Etiology of chronic neck pain includes neck sprain, mechanical or muscular neck pain, myofascial pain syndrome, postural neck pain as well as pain due to degenerative changes. We report the case of a 42 year old secretary, complaining about a longer history of neck pain and limited movement of the cervical spine. Surprisingly, the adequate radiologic examination revealed a bilateral ossification of the stylohyoid ligament complex. Her symptoms remained intractable from conservative treatment consisting of anti-inflammatory medication as well as physical therapy. Hence the patient was admitted to surgical resection of the ossified stylohyoid ligament complex. Afterwards she was free of any complaints and went back to work. Therefore, ossification of the stylohyoid ligament complex causing severe neck pain and movement disorder should be regarded as a rare differential diagnosis of occupational related neck pain.

  12. 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

  13. Congenital Bilateral Retinal Detachment in Two Siblings with Osteoporosis-Pseudoglioma Syndrome.

    PubMed

    Welinder, Lotte G; Robitaille, Johane M; Rupps, Rosemarie; Boerkoel, Cornelius F; Lyons, Christopher J

    2015-01-01

    The birth of a bilaterally blind child is catastrophic for families and a challenging diagnostic and management problem for ophthalmologists. Early identification of the underlying cause and its genetic basis helps initiate possible treatment, delineate prognosis, and identify risks for future pregnancies. In some cases, an early diagnosis can also influence the treatment of other family members. We report two sisters with bilateral retinal detachment and retro-lental masses from birth with no detectable NDP or FZD4 mutations. They were born to parents without detectable retinal anomalies. At 1 year of age, the elder sister had low impact bone fractures, and further evaluation identified severe osteopenia and multiple spinal compression fractures. Molecular testing identified biallelic lipoprotein receptor-related protein 5 (LRP5) mutations (NM_002335.3:c. [889dupA]; [2827 + 1G > A]) confirming a diagnosis of osteoporosis-pseudoglioma (OPPG) syndrome. After this diagnosis, the father and mother were found to have low bone mass and the father started on therapy. We conclude that early detection of LRP5 mutations is important for initiation of treatment of reduced bone density in the patients and their carrier relatives. PMID:25945592

  14. Reporting of allocation method and statistical analyses that deal with bilaterally affected wrists in clinical trials for carpal tunnel syndrome.

    PubMed

    Page, Matthew J; O'Connor, Denise A; Pitt, Veronica; Massy-Westropp, Nicola

    2013-11-01

    The authors aimed to describe how often the allocation method and the statistical analyses that deal with bilateral involvement are reported in clinical trials for carpal tunnel syndrome and to determine whether reporting has improved over time. Forty-two trials identified from recently published systematic reviews were assessed. Information about allocation method and statistical analyses was obtained from published reports and trialists. Only 15 trialists (36%) reported the method of random sequence generation used, and 6 trialists (14%) reported the method of allocation concealment used. Of 25 trials including participants with bilateral carpal tunnel syndrome, 17 (68%) reported the method used to allocate the wrists, whereas only 1 (4%) reported using a statistical analysis that appropriately dealt with bilateral involvement. There was no clear trend of improved reporting over time. Interventions are needed to improve reporting quality and statistical analyses of these trials so that these can provide more reliable evidence to inform clinical practice.

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

    PubMed

    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

    2015-10-01

    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: O ne 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.

  16. 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.

  17. 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

  18. 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

  19. 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.

  20. 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

  1. 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

  2. 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.

  3. 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

  4. [Bilateral reversed palmaris longus muscle--a rare cause of peripheral median nerve compression syndrome. Case report].

    PubMed

    Giunta, R; Brunner, U; Wilhelm, K

    1993-10-01

    A rare case of median nerve compression syndrome outside the carpal tunnel in the distal forearm is reported. A 21-year-old man suffered while working from symptoms of temporary median nerve compression in both forearms; this was caused by hypertrophy of reversed palmaris longus muscles. Resection of the abnormal muscle bellies relieved the symptoms immediately. Only ten similar cases have been reported in the literature, and this is the first case with bilateral symptoms.

  5. 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

  6. Bilateral renal tumors in an adult man with Smith-Magenis syndrome: The role of the FLCN gene.

    PubMed

    Dardour, Leila; Verleyen, Pieter; Lesage, Karl; Holvoet, Maureen; Devriendt, Koen

    2016-10-01

    Smith-Magenis syndrome (SMS) is a contiguous-gene disorder most commonly caused by a deletion of chromosome 17p11.2. We report a 57 year-old man with SMS who presents bilateral renal tumors. This is most likely related to haploinsufficiency of FLCN gene, located in the deleted region, and a known tumor suppressor gene. Haploinsufficiency of FLCN causes Birt-Hogg-Dubé syndrome (BHDS), characterized by pulmonary cysts, renal and skin tumors. The present observation suggests that the follow-up of patients with SMS should also focus on possible manifestations of BHDS.

  7. Lower extremity anterior compartment syndrome complicating bilateral mastectomy and immediate breast reconstruction: A case report and literature review

    PubMed Central

    Tashakkor, A Yashar; Macadam, Sheina A

    2012-01-01

    ‘Well leg compartment syndrome’ refers to compartment syndrome occurring in a nontraumatic setting. This occurs most commonly in the lower limb during surgery performed with the patient in an anatomically vulnerable position. While this complication is well documented in the setting of orthopedic, urological and gynecological surgeries, it is an exceptionally rare complication in plastic surgery; only seven cases have been published on compartment syndrome complicating an operation performed on a supine patient. A case involving a 56-year-old woman who developed an anterior compartment syndrome of her right lower leg following a bilateral mastectomy with immediate breast reconstruction is presented. A detailed literature review is also included. PMID:23730157

  8. 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.

  9. [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

  10. 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

  11. 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

  12. A case of WAGR syndrome in association with developmental glaucoma requiring bilateral Baerveldt glaucoma implants and subsequent tube repositioning

    PubMed Central

    Akagi, Tadamichi; Yoshikawa, Munemitsu; Nakanishi, Hideo; Yoshimura, Nagahisa

    2015-01-01

    Glaucoma drainage device implantation is efficacious for the treatment of pediatric glaucoma patients when multiple angle surgeries fail. However, tube touching of the corneal endothelium is one of the major postoperative complications to deal with. A 15-month-old male patient with Wilms’ tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome was diagnosed with bilateral developmental glaucoma. He underwent Baerveldt glaucoma implant (BGI) surgeries in both eyes after multiple failed trabeculotomies. The tube in his right eye was touching the cornea 15 months after BGI surgery. To avoid corneal endothelium damage, BGI tube repositioning with scleral fixation was performed without serious complications. The bilateral BGI surgeries achieved successful intraocular pressure reduction for over 2 years and tube repositioning with scleral fixation of BGI tube was successful for BGI tube malposition. Although careful attention to intraocular pressure and tube malposition is essential after glaucoma drainage device implantation, especially in pediatric cases, BGI surgery is effective in the management of developmental glaucoma following unsuccessful angle surgeries. PMID:26109842

  13. A case of WAGR syndrome in association with developmental glaucoma requiring bilateral Baerveldt glaucoma implants and subsequent tube repositioning.

    PubMed

    Akagi, Tadamichi; Yoshikawa, Munemitsu; Nakanishi, Hideo; Yoshimura, Nagahisa

    2015-01-01

    Glaucoma drainage device implantation is efficacious for the treatment of pediatric glaucoma patients when multiple angle surgeries fail. However, tube touching of the corneal endothelium is one of the major postoperative complications to deal with. A 15-month-old male patient with Wilms' tumor, aniridia, genitourinary anomalies, and mental retardation (WAGR) syndrome was diagnosed with bilateral developmental glaucoma. He underwent Baerveldt glaucoma implant (BGI) surgeries in both eyes after multiple failed trabeculotomies. The tube in his right eye was touching the cornea 15 months after BGI surgery. To avoid corneal endothelium damage, BGI tube repositioning with scleral fixation was performed without serious complications. The bilateral BGI surgeries achieved successful intraocular pressure reduction for over 2 years and tube repositioning with scleral fixation of BGI tube was successful for BGI tube malposition. Although careful attention to intraocular pressure and tube malposition is essential after glaucoma drainage device implantation, especially in pediatric cases, BGI surgery is effective in the management of developmental glaucoma following unsuccessful angle surgeries.

  14. Fulminant bilateral acute retinal necrosis syndrome associated with viral encephalitis: A case report

    PubMed Central

    Zhou, Chunkui; Zhu, Lijun; Fang, Shaokuan

    2016-01-01

    Herpes simplex virus (HSV) is the most common cause of acute viral encephalitis. Acute retinal necrosis (ARN) is a rapidly progressing and potentially blinding eye disease that may be induced by HSV. The present case study reports the very rare case of a patient with herpes simplex encephalitis (HSE) combined with acute retinal necrosis (ARN). A 47-year-old woman was admitted to hospital with persistent high fever and somnolence for 5 days. Magnetic resonance imaging showed abnormal signals in the right medial temporal lobes, and HSV-1 was identified in the serum and cerebrospinal fluid. Five days later, despite treatment with intravenous acyclovir and partial improvement in consciousness, the patient suddenly developed blurred vision and bilateral visual pain. Fundus fluorescence angiography revealed bilateral vessel obstruction and flaky reduced fluorescence. ARN was diagnosed clinically. Dexamethasone was administered as an anti-inflammatory adjunct to intravenous acyclovir therapy. The visual acuity of the patient was reduced to mere light perception a further 4 days later. The present case indicates that HSE may be complicated with ARN, causing a reduction in visual acuity to mere light perception within a very short time.

  15. Fulminant bilateral acute retinal necrosis syndrome associated with viral encephalitis: A case report

    PubMed Central

    Zhou, Chunkui; Zhu, Lijun; Fang, Shaokuan

    2016-01-01

    Herpes simplex virus (HSV) is the most common cause of acute viral encephalitis. Acute retinal necrosis (ARN) is a rapidly progressing and potentially blinding eye disease that may be induced by HSV. The present case study reports the very rare case of a patient with herpes simplex encephalitis (HSE) combined with acute retinal necrosis (ARN). A 47-year-old woman was admitted to hospital with persistent high fever and somnolence for 5 days. Magnetic resonance imaging showed abnormal signals in the right medial temporal lobes, and HSV-1 was identified in the serum and cerebrospinal fluid. Five days later, despite treatment with intravenous acyclovir and partial improvement in consciousness, the patient suddenly developed blurred vision and bilateral visual pain. Fundus fluorescence angiography revealed bilateral vessel obstruction and flaky reduced fluorescence. ARN was diagnosed clinically. Dexamethasone was administered as an anti-inflammatory adjunct to intravenous acyclovir therapy. The visual acuity of the patient was reduced to mere light perception a further 4 days later. The present case indicates that HSE may be complicated with ARN, causing a reduction in visual acuity to mere light perception within a very short time. PMID:27698716

  16. Bilateral vestibulopathy.

    PubMed

    Strupp, M; Feil, K; Dieterich, M; Brandt, T

    2016-01-01

    The leading symptoms of bilateral vestibulopathy (BVP) are postural imbalance and unsteadiness of gait that worsens in darkness and on uneven ground. There are typically no symptoms while sitting or lying under static conditions. A minority of patients also have movement-induced oscillopsia, in particular while walking. The diagnosis of BVP is based on a bilaterally reduced or absent function of the vestibulo-ocular reflex (VOR). This deficit is diagnosed for the high-frequency range of the angular VOR by a bilaterally pathologic bedside head impulse test (HIT) and for the low-frequency range by a bilaterally reduced or absent caloric response. If the results of the bedside HIT are unclear, angular VOR function should be quantified by a video-oculography system (vHIT). An additional test supporting the diagnosis is dynamic visual acuity. Cervical and ocular vestibular-evoked myogenic potentials (c/oVEMP) may also be reduced or absent, indicating impaired otolith function. There are different subtypes of BVP depending on the affected anatomic structure and frequency range of the VOR deficit: impaired canal function in the low- and/or high-frequency VOR range only and/or otolith function only; the latter is very rare. The etiology of BVP remains unclear in more than 50% of patients: in these cases neurodegeneration is assumed. Frequent known causes are ototoxicity mainly due to gentamicin, bilateral Menière's disease, autoimmune diseases, meningitis and bilateral vestibular schwannoma, as well as an association with cerebellar degeneration (cerebellar ataxia, neuropathy, vestibular areflexia syndrome=CANVAS). In general, in the long term there is no improvement of vestibular function. There are four treatment options: first, detailed patient counseling to explain the cause, etiology, and consequences, as well as the course of the disease; second, daily vestibular exercises and balance training; third, if possible, treatment of the underlying cause, as in bilateral

  17. Peripheral osteoma bilateral of the mandible without association with Gardner syndrome.

    PubMed

    Guimarães, Karis Barbosa; Cavalcante, Josuel Raimundo; Ferraro-Bezerra, Marcelo; Silva, Daniela Nascimento; de Holanda Vasconcellos, Ricardo José; do Egito Vasconcelos, Belmiro Cavalcanti

    2012-03-01

    Periosteal osteoma is a rare benign pathologic lesion in the jaws and the mandible is the anatomic region most frequently involved. Elective treatment consists of surgical excision. The aim of this report was to report a rare case of bilateral periosteal osteoma involving the mandible--a painless, firm, and well-circumscribed lesion, with more bony prominence on the left than on the right side. The patient had no history of facial trauma or systemic changes that could intervene with the progression of the lesion. It was diagnosed as a periosteal osteoma, and surgical excision was performed. No recurrence after 4 years of follow-up was demonstrated. Surgical excision of the periosteal osteoma was demonstrated to be a useful technical strategy that simplifies and accelerates the surgical procedures and probably contributes to establish harmony of the jaws. Periodical clinical and radiographic follow-ups after surgery are advised.

  18. Arthroscopic treatment of bilateral snapping scapula syndrome: a case report and review of the literature.

    PubMed

    Freche, Sven; Juch, Franziska; Nusselt, Thomas; Delank, Karl Stefan; Hagel, Alexander

    2015-01-01

    Snapping scapula syndrome represents a rare entity in shoulder surgery. Clinically, it presents as shoulder pain and loud crepitus during shoulder movement. Moreover, glenohumeral bursitis can cause additional pain. Snapping scapula syndrome is caused by an increased angulation of the superomedial part of the scapula combined with bursitis. Other common causes such as subscapular osteochondroma or rib fracture non-unions were excluded. We report a 24-year-old female patient with persisting shoulder pain and disturbing crepitus during movement of the left shoulder. Radiographic examination revealed increased angulation of the superomedial scapula on both sides and MRI, bursitis of the left shoulder. The patient was successfully treated with minimally invasive arthroscopic trimming of the prominent osseous formation at the left scapula combined with bursectomy. Additionally, specific pain-adapted physiotherapeutic exercises of both shoulders were performed. The patient had a pain-free shoulder movement without crepitus on both sides at the 6th month follow-up. PMID:25803260

  19. 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

  20. 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

  1. Homozygous FOXE3 mutations cause non-syndromic, bilateral, total sclerocornea, aphakia, microphthalmia and optic disc coloboma

    PubMed Central

    Buentello-Volante, Beatriz; McKibbin, Martin; Rocha-Medina, J. Alberto; Fernandez-Fuentes, Narcis; Koga-Nakamura, Wilson; Ashiq, Aruna; Khan, Kamron; Booth, Adam P.; Williams, Grange; Raashid, Yasmin; Jafri, Hussain; Rice, Aine; Inglehearn, Chris F.; Zenteno, Juan Carlos

    2010-01-01

    Purpose To investigate the genetic basis of recessively-inherited congenital, non syndromic, bilateral, total sclerocornea in two consanguineous pedigrees, one from the Punjab province of Pakistan and the other from the Tlaxcala province of Mexico. Methods Ophthalmic examinations were conducted on each family member to confirm their diagnosis and magnetic resonance imaging (MRI) or ultrasonography of the eyes was performed on some family members. Genomic DNA was analyzed by homozygosity mapping using the Affymetrix 6.0 SNP array and linkage was confirmed with polymorphic microsatellite markers. Candidate genes were sequenced. Results A diagnosis of autosomal recessive sclerocornea was established for 7 members of the Pakistani and 8 members of the Mexican pedigrees. In the Pakistani family we established linkage to a region on chromosome 1p that contained Forkhead Box E3 (FOXE3), a strong candidate gene since FOXE3 mutations had previously been associated with various anterior segment abnormalities. Sequencing FOXE3 identified the previously reported nonsense mutation, c.720C>A, p.C240X, in the Pakistani pedigree and a novel missense mutation which disrupts an evolutionarily conserved residue in the forkhead domain, c.292T>C, p.Y98H, in the Mexican pedigree. Individuals with heterozygous mutations had no ocular abnormalities. MRI or ultrasonography confirmed that the patients with sclerocornea were also aphakic, had microphthalmia and some had optic disc coloboma. Conclusions This is the fourth report detailing homozygous FOXE3 mutations causing anterior segment abnormalities in human patients. Previous papers have emphasized aphakia and microphthalmia as the primary phenotype, but we find that the initial diagnosis – and perhaps the only one possible in a rural setting – is one of non-syndromic, bilateral, total sclerocornea. Dominantly inherited anterior segment defects have also been noted in association with heterozygous FOXE3 mutations. However the absence

  2. [Herpetiform bilateral epithelial corneal dystrophy caused by Tyrosinemia (Richner-Hanhart-Syndrome) (author's transl)].

    PubMed

    Jaeger, W; Gallasch, G; Schnyder, U W; Lutz, P; Schmidt, H

    1978-10-01

    Nutritional Tyrosinemia in animal experiments (Schweizer, Burns a. o.) caused an epithelial corneal dystrophy. The corresponding clinical picture is the Richner-Hanhart-Syndrome with herpetiform epithelial corneal dystrophy, palmo-plantar-keratosis and -- in some case -- a later developing oligophrenia. Goldsmith and coworkers suceeded to find out that all these symptoms are combined with tyrosinemia presumably caused ty one congenital enzyme defect. -- Personal clinical observations of such patients demonstrate that as well the corneal as the dermatological symptoms could be cured by diet, if the diagnosis is made in childhood. Corneal symptoms are still absent with a tyrosinemia as high as 10 mg%. Dietetic formula was found out to maintain this level, which is low enough to avoid the symptoms of Richner-Hanhart-Syndrome and certainly high enough to avoid symptoms of nutritional deficiency. One might expect that oligophrenia will not develop if this diet is used consequently. -- Since the first corneal symptoms develop already during the first years of life the ophthalmologist ought to know this etiology. The levels of tyrosinemia are so exorbitant (30--50 mg%) that the laboratory diagnosis is possible without any difficulty. If the diagnosis is only made in adult patients dietetic therapy is of limited value.

  3. 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

  4. Polymicrogyria and infantile spasms in a patient with 1p36 deletion syndrome.

    PubMed

    Saito, Yoshiaki; Kubota, Masaya; Kurosawa, Kenji; Ichihashi, Izumi; Kaneko, Yuu; Hattori, Ayako; Komaki, Hirofumi; Nakagawa, Eiji; Sugai, Kenji; Sasaki, Masayuki

    2011-05-01

    A 3-months-old boy presented with partial seizures that soon evolved into infantile spasms. Magnetic resonance imaging revealed bilateral perisylvian polymicrogyria with right-sided predominance. ACTH therapy successfully controlled epilepsy and electroencephalograms were normalized. Conventional G-banded chromosomal analysis was performed due to his distinctive features and a derivative chromosome 1 derived from parental balanced translocation with a karyoptype of 46,XY,der(1)t(1;4)(p36.23;q35) was detected. Fluorescent in situ hybridization analysis confirmed the deleted region of 1p36 as large as 8.6Mb. This is the first delineation of concurrent complications of infantile spasms and polymicrogyria in patient with 1p36 deletion. 1p36 deletion syndrome should be broadly recognized as a differential diagnosis of regional polymicrogyria and/or infantile spasms.

  5. Bilateral gustatory rhinorrhea following bilateral parotidectomy: a case report.

    PubMed

    Stevens, H E; Doyle, P J

    1988-06-01

    To the authors' knowledge, no cases of gustatory rhinorrhea following parotidectomy have been reported. A case is presented of a 28-year-old woman who underwent bilateral parotidectomies within a four-year period for congenital sialectasia and secondary infection. Both operations were complicated by Frey's syndrome and she also developed bilateral gustatory rhinorrhea. The successful management of this problem with bilateral vidian neurectomies and the refractory nature of her Frey's syndrome, finally controlled with tympanic neurectomies, are discussed. PMID:3398109

  6. 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

  7. 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. PMID:27536636

  8. Bilateral meningoencephaloceles with cerebrospinal fluid rhinorrhea after facial advancement in the Crouzon syndrome

    PubMed Central

    Panuganti, Bharat A.; Leach, Matthew

    2015-01-01

    Background: Cerebrospinal fluid (CSF) rhinorrhea and encephaloceles are rare complications of craniofacial advancement procedures performed in patients with craniofacial dysostoses (CD) to address the ramifications of their midface hypoplasia including obstructed nasal airway, exorbitism, and impaired mastication. Surgical repair of this CSF rhinorrhea is complicated by occult elevations in intracranial pressure (ICP), potentially necessitating open, transcranial repair. We report the first case in otolaryngology literature of a patient with Crouzon syndrome with late CSF rhinorrhea and encephalocele formation after previous LeFort III facial advancement surgery. Objectives: Describe the case of a patient with Crouzon syndrome who presented with CSF rhinorrhea and encephaloceles as complications of Le Fort III facial advancement surgery. Review the literature pertaining to the incidence and management of post-operative CSF rhinorrhea and encephaloceles. Analyze issues related to repair of these complications, including occult elevations in ICP, the utility of perioperative CSF shunts, and the importance of considering alternative repair schemes to the traditional endonasal, endoscopic approach. Methods: Review of the literature describing CSF rhinorrhea and encephalocele formation following facial advancement in CD, focusing on management strategies. Results: CSF rhinorrhea and encephalocele formation are rare complications of craniofacial advancement procedures. Occult elevations in ICP complicate the prospect of permanent surgical repair, potentially necessitating transcranial repair and the use of CSF shunts. Though no consensus exists regarding the utility of perioperative CSF drains, strong associations exist between elevated ICP and failed surgical repair. Additionally, the anatomic changes in the frontal and ethmoid sinuses after facial advancement present a challenge to endoscopic repair. Conclusion: Otolaryngologists should be aware of the possibility of

  9. An unusual presentation of Guillain-Barré syndrome in a young man with bilateral upper extremity weakness.

    PubMed

    McNicholas, N; O'Dowd, S; McNamara, B; O'Toole, O

    2015-09-08

    A 30-year-old man attended the emergency department with a 4-day history of progressive, bilateral upper limb weakness. He had mild shortness of breath and occasional swallowing difficulties. One month prior to presentation, he had flu-like symptoms and diarrhoea. Examination revealed upper limb hypotonia, symmetrical distal arm weakness and hyporeflexia. Power and reflexes in the lower limbs were normal. Nerve conduction studies and lumbar puncture demonstrated features consistent with Guillain-Barré syndrome (GBS). The patient was treated with a 5-day course of intravenous immunoglobulins. He improved significantly over the next 2 weeks. Breathing and swallow function did not deteriorate and required no further intervention. He had a sustained improvement, and remained at baseline 1 year later. Work-up for underlying structural, infectious, inflammatory and paraneoplastic aetiologies were negative. Serum antiganglioside antibodies were positive for the anti-GT1a IgG isotype supporting the clinical diagnosis of the pharyngeal-cervical-brachial variant of GBS.

  10. 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

  11. Subclinical Cushing's syndrome in patients with bilateral compared to unilateral adrenal incidentalomas: a systematic review and meta-analysis.

    PubMed

    Paschou, Stavroula A; Kandaraki, Eleni; Dimitropoulou, Fotini; Goulis, Dimitrios G; Vryonidou, Andromachi

    2016-02-01

    The purpose of this study was to systematically review the literature for studies that have investigated possible differences in the prevalence of subclinical Cushing's syndrome (SCS) and related clinical implications between patients with unilateral (UAI) and bilateral adrenal incidentalomas (BAI) and to meta-analyze the best evidence available. Electronic databases PubMed and EMBASE were systematically searched. Main study outcome was the prevalence of SCS in patients with UAI and BAI. Secondary outcomes were the prevalence of obesity, diabetes, glucose intolerance, hypertension, dyslipidemia, and osteoporosis in patients with UAI and BAI. Risk differences (RD) or mean differences (MD) and 95 % confidence intervals (CIs) were calculated. Meta-analysis was conducted using Review Manager (RevMan 5.3). Six studies were included in the meta-analysis involving in total 1239 patients, 968 with UAI, and 271 with BAI. Patients with UAI had lower prevalence of SCS compared with those with BAI [RD (95 % CI) -0.13 (-0.22 to -0.05), I (2) = 42 %]. The mass diameter of UAI did not differ from BAI (the size of the largest lesion) [MD (95 % CI) -0.45 (-1.09 to 0.19), I (2) = 91 %]. The prevalence of obesity [MD (95 % CI) 0.42 (-0.53 to 1.37), I (2) = 4 %], diabetes [RD (95 % CI) -0.04 (-0.11 to 0.04), I (2) = 0 %], hypertension [RD (95 % CI) 0.00 (-0.18 to 0.18), I (2) = 75 %], and dyslipidemia [RD (95 % CI) -0.02 (-0.16 to 0.13), I (2) = 50 %] did not differ between UAI and BAI. The present meta-analysis provided evidence that patients with BAI present a higher prevalence of SCS compared to patients with UAI.

  12. Parallel versus Serial Processing Dependencies in the Perisylvian Speech Network: A Granger Analysis of Intracranial EEG Data

    ERIC Educational Resources Information Center

    Gow, David W., Jr.; Keller, Corey J.; Eskandar, Emad; Meng, Nate; Cash, Sydney S.

    2009-01-01

    In this work, we apply Granger causality analysis to high spatiotemporal resolution intracranial EEG (iEEG) data to examine how different components of the left perisylvian language network interact during spoken language perception. The specific focus is on the characterization of serial versus parallel processing dependencies in the dominant…

  13. Beyond the amygdala: Linguistic threat modulates peri-sylvian semantic access cortices.

    PubMed

    Weisholtz, Daniel S; Root, James C; Butler, Tracy; Tüscher, Oliver; Epstein, Jane; Pan, Hong; Protopopescu, Xenia; Goldstein, Martin; Isenberg, Nancy; Brendel, Gary; LeDoux, Joseph; Silbersweig, David A; Stern, Emily

    2015-12-01

    In this study, healthy volunteers were scanned using functional magnetic resonance imaging (fMRI) to investigate the neural systems involved in processing the threatening content conveyed via visually presented "threat words." The neural responses elicited by these words were compared to those elicited by matched neutral control words. The results demonstrate that linguistic threat, when presented in written form, can selectively engage areas of lateral temporal and inferior frontal cortex, distinct from the core language areas implicated in aphasia. Additionally, linguistic threat modulates neural activity in visceral/emotional systems (amygdala, parahippocampal gyrus and periaqueductal gray), and at earlier stages of the visual-linguistic processing stream involved in visual word form representations (ventral occipitotemporal cortex). We propose a model whereby limbic activation modulates activity at multiple nodes along the visual-linguistic-semantic processing stream, including a perisylvian "semantic access network" involved in decoding word meaning, suggesting a dynamic interplay between feedforward and feedback processes. PMID:26575986

  14. Segregation of lexical and sub-lexical reading processes in the left perisylvian cortex.

    PubMed

    Roux, Franck-Emmanuel; Durand, Jean-Baptiste; Jucla, Mélanie; Réhault, Emilie; Reddy, Marion; Démonet, Jean-François

    2012-01-01

    A fundamental issue in cognitive neuroscience is the existence of two major, sub-lexical and lexical, reading processes and their possible segregation in the left posterior perisylvian cortex. Using cortical electrostimulation mapping, we identified the cortical areas involved on reading either orthographically irregular words (lexical, "direct" process) or pronounceable pseudowords (sublexical, "indirect" process) in 14 right-handed neurosurgical patients while video-recording behavioral effects. Intraoperative neuronavigation system and Montreal Neurological Institute (MNI) stereotactic coordinates were used to identify the localization of stimulation sites. Fifty-one reading interference areas were found that affected either words (14 areas), or pseudo-words (11 areas), or both (26 areas). Forty-one (80%) corresponded to the impairment of the phonological level of reading processes. Reading processes involved discrete, highly localized perisylvian cortical areas with individual variability. MNI coordinates throughout the group exhibited a clear segregation according to the tested reading route; specific pseudo-word reading interferences were concentrated in a restricted inferior and anterior subpart of the left supramarginal gyrus (barycentre x = -68.1; y = -25.9; z = 30.2; Brodmann's area 40) while specific word reading areas were located almost exclusively alongside the left superior temporal gyrus. Although half of the reading interferences found were nonspecific, the finding of specific lexical or sublexical interferences is new evidence that lexical and sublexical processes of reading could be partially supported by distinct cortical sub-regions despite their anatomical proximity. These data are in line with many brain activation studies that showed that left superior temporal and inferior parietal regions had a crucial role respectively in word and pseudoword reading and were core regions for dyslexia.

  15. 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

  16. A Rare Interstitial Duplication of 8q22.1–8q24.3 Associated with Syndromic Bilateral Cleft Lip/Palate

    PubMed Central

    Rezek, Regina Ferreira; Rodrigues Abbas, Ana Angélica; Forte Mazzeu, Juliana; Duarte Miranda, Siliana Maria; Velloso-Rodrigues, Cibele

    2014-01-01

    We present a rare case of 8q interstitial duplication derived from maternal balanced translocations in a patient with bilateral cleft lip and palate in syndromic form associated with other congenital malformations. G-banding cytogenetic analysis revealed a chromosomal abnormality in the form of the karyotype 46,XX der(22)t(8;22)(q22.1;p11.1)mat. Chromosome microarray analysis evidenced a 49 Mb duplicated segment of chromosome 8q with no pathogenic imbalances on chromosome 22. Two siblings also carry the balanced translocation. We have compared this case with other “pure” trisomies of 8q patients reported in the literature and with genome wide association studies recently published. This work highlights the involvement of chromosome 8q in orofacial clefts. PMID:25506438

  17. Bilateral Entry and Release of Middle East Respiratory Syndrome Coronavirus Induces Profound Apoptosis of Human Bronchial Epithelial Cells

    PubMed Central

    Tao, Xinrong; Hill, Terence E.; Morimoto, Chikao; Peters, Clarence J.; Ksiazek, Thomas G.

    2013-01-01

    The newly emerged Middle East respiratory syndrome coronavirus (MERS-CoV) infects human bronchial epithelial Calu-3 cells. Unlike severe acute respiratory syndrome (SARS)-CoV, which exclusively infects and releases through the apical route, this virus can do so through either side of polarized Calu-3 cells. Infection results in profound apoptosis within 24 h irrespective of its production of titers that are lower than those of SARS-CoV. Together, our results provide new insights into the dissemination and pathogenesis of MERS-CoV and may indicate that the virus differs markedly from SARS-CoV. PMID:23824802

  18. Heterogeneity of bilateral renal agenesis.

    PubMed Central

    Fitch, N.

    1977-01-01

    Bilateral and unilateral renal agenesis may be expressions of single dominant gene. Chromosome abnormalities may be present and the renal agenesis may be part of a syndrome of multiple abnormalities. Apparently normal relatives of affected individuals should be screened by intravenous pyelography before genetic counselling given. PMID:844022

  19. 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

  20. Bilateral pallidotomy for treatment of Parkinson's disease induced corticobulbar syndrome and psychic akinesia avoidable by globus pallidus lesion combined with contralateral stimulation

    PubMed Central

    Merello, M; Starkstein, S; Nouzeilles, M; Kuzis, G; Leiguarda, R

    2001-01-01

    OBJECTIVE—Posteroventral pallidotomy (PVP) has proved to be an effective method for the treatment of Parkinson's disease. However, data on bilateral procedures are still limited. To assess the effects of bilateral globus pallidus (GPi) lesion and to compare it with a combination of unilateral GPi lesion plus contralateral GPi stimulation (PVP+PVS), an open blind randomised trial was designed.
METHODS—A prospective series of patients with severe Parkinson's disease refractory to medical treatment, and severe drug induced dyskinesias, were randomised either to simultaneous bilateral PVP or simultaneous PVP+PVS. All patients were assessed with the core assessment programme for intracerebral transplantation (CAPIT), and a comprehensive neuropsychological and neuropsychiatric battery both before surgery and 3 months later.
RESULTS—The severe adverse effects found in the first three patients subjected to bilateral PVP led to discontinuation of the protocol. All three patients developed depression and apathy. Speech, salivation, and swallowing, as well as freezing, walking, and falling, dramatically worsened. By contrast, all three patients undergoing PVP+PVS had a significant motor improvement.
CONCLUSION—Bilateral simultaneous lesions within the GPi may produce severe motor and psychiatric complications. On the other hand, a combination of PVP+ PVS significantly improves parkinsonian symptoms not associated with the side effects elicited by bilateral lesions.

 PMID:11606671

  1. 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

    2015-07-15

    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.

  2. Effects of passive correction of scapular position on pain, proprioception, and range of motion in neck-pain patients with bilateral scapular downward-rotation syndrome.

    PubMed

    Ha, Sung-min; Kwon, Oh-yun; Yi, Chung-hwi; Jeon, Hye-seon; Lee, Won-hwee

    2011-12-01

    The effects of passive correction of scapular position (PCSPT) on pain, proprioception, and range of motion (ROM) were investigated in neck-pain patients with bilateral scapular downward-rotation (SDR). Fifteen neck-pain patients with bilateral SDR were recruited from a workplace based work-conditioning center. The intensity of pain felt was quantified using a visual analogue scale. Kinematic data for ROM and joint-position error (JPE) were analyzed using a 3-dimensional motion-analysis system. Differences in pain, JPE, and ROM with and without PCSPT were assessed using a paired t-test. PCSPT significantly decreased JPE and neck pain during active neck rotation and significantly increased neck-rotation ROM (p < 0.05). These findings suggest that PCSPT results in decreased neck pain and improved neck-rotation ROM and proprioception during active neck rotation in neck-pain patients with bilateral SDR. PMID:21705260

  3. A syndrome of hypohidrotic ectodermal dysplasia with normal teeth, peculiar facies, pigmentary disturbances, psychomotor and growth retardation, bilateral nuclear cataract, and other signs.

    PubMed Central

    Freire-Maia, N; Fortes, V A; Pereira, L C; Opitz, J M; Marcalle, F A; Cavalli, I J

    1975-01-01

    This paper describes a 7-year-old girl with trichodysplasia, normal teeth, onychogryposis, hypohidrosis, psychomotor and growth retardation, dry and warm skin with follicular hyperkeratosis, pigmentary disturbances (hyper- and hypochromic spots), bilateral nuclear cataract, dermatoglyphic anomalies, and other signs. This condition is considered a new form of ectodermal dysplasia. Images PMID:1177286

  4. Somato-motor haptic processing in posterior inner perisylvian region (SII/pIC) of the macaque monkey.

    PubMed

    Ishida, Hiroaki; Fornia, Luca; Grandi, Laura Clara; Umiltà, Maria Alessandra; Gallese, Vittorio

    2013-01-01

    The posterior inner perisylvian region including the secondary somatosensory cortex (area SII) and the adjacent region of posterior insular cortex (pIC) has been implicated in haptic processing by integrating somato-motor information during hand-manipulation, both in humans and in non-human primates. However, motor-related properties during hand-manipulation are still largely unknown. To investigate a motor-related activity in the hand region of SII/pIC, two macaque monkeys were trained to perform a hand-manipulation task, requiring 3 different grip types (precision grip, finger exploration, side grip) both in light and in dark conditions. Our results showed that 70% (n = 33/48) of task related neurons within SII/pIC were only activated during monkeys' active hand-manipulation. Of those 33 neurons, 15 (45%) began to discharge before hand-target contact, while the remaining neurons were tonically active after contact. Thirty-percent (n = 15/48) of studied neurons responded to both passive somatosensory stimulation and to the motor task. A consistent percentage of task-related neurons in SII/pIC was selectively activated during finger exploration (FE) and precision grasping (PG) execution, suggesting they play a pivotal role in control skilled finger movements. Furthermore, hand-manipulation-related neurons also responded when visual feedback was absent in the dark. Altogether, our results suggest that somato-motor neurons in SII/pIC likely contribute to haptic processing from the initial to the final phase of grasping and object manipulation. Such motor-related activity could also provide the somato-motor binding principle enabling the translation of diachronic somatosensory inputs into a coherent image of the explored object.

  5. 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

  6. Bilateral dacryoceles associated with bilateral alacrimia with punctal and canalicular agenesis

    PubMed Central

    Gupta, Himika; Kane, Shubhada; Balasubramaniam, Vidyashankar

    2013-01-01

    To report a rare case of a 19 year old female presenting with bilateral dacryocele and punctal and canalicular agenesis without epiphora. After clinical and radiological examination, the patient was found to have bilateral lacrimal gland agenesis as well. No other dental, otological or systemic abnormality suggestive of lacrimo-auricular-dento-digital syndrome was detected. She was managed with bilateral dacryocystectomy. Algorithm for managing such nonconventional lacrimal outflow dysgenesis based on presenting symptoms and co existing epiphora is proposed. Rationale of preferring lacrimal sac excision instead of dacryocystorhinostomy in this case is also discussed. PMID:24526864

  7. Bilateral lower limb polio, chronic obstructive pulmonary disease, and recurrent acute coronary syndrome in a poly tobacco user: a preventable triple tragedy.

    PubMed

    Aggarwal, Ramesh; Dwivedi, Shridhar

    2012-01-01

    Non communicable diseases in most of the developing countries have surpassed the morbidity and mortality arising from communicable diseases. However there are people who continue to suffer from the residual disabilities of some communicable disease acquired at younger age like polio and develop non communicable diseases like COPD and coronary syndrome at older age primarily because of their tobacco habits. Both of these combination of communicable and non communicable diseases are preventable if timely preventive measures and healthy life style is adopted. This case highlights one such case where patient despite suffering from polio and restrictive lung disease started using tobacco and suffered from obstructive lung disease and coronary syndrome.

  8. Bilateral lesions of CA1 and CA2 fields of the hippocampus are sufficient to cause a severe amnesic syndrome in humans.

    PubMed Central

    Kartsounis, L D; Rudge, P; Stevens, J M

    1995-01-01

    A patient is reported in whom a classic amnesic syndrome developed as a result of repeated episodes of cerebral ischaemia, accompanied by seizures. The amnesia was very severe for both old and newly acquired memories and the critical lesions defined by MRI were circumscribed areas confined to CA1 and CA2 fields of both hippocampi. Images PMID:7608720

  9. 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

  10. Acute bilateral isolated foot drop: Report of two cases

    PubMed Central

    Kertmen, H.; Gürer, B.; Yimaz, E. R.; Sekerci, Z.

    2015-01-01

    Foot drop is defined as the weakness of the foot and ankle dorsiflexion. Acute unilateral foot drop is a well-documented entity, whereas bilateral foot drop is rarely documented. Slowly progressing bilateral foot drop may occur with various metabolic causes, parasagittal intracranial pathologies, and cauda equina syndrome. Acute onset of bilateral foot drop due to disc herniation is extremely rare. Here we present two cases of acute bilateral foot drop due to disc herniation. The first patient was a 45-year-old man presented with acute bilateral foot drop, without any sign of the cauda equina syndrome. Lumbar magnetic resonance imaging of the patient revealed L4-5 disc herniation. To our knowledge, this is the first presented case of acute bilateral foot drop without any signs of cauda equina syndrome caused by L4-5 disc herniation. The second patient was a 50-year-old man who was also presented with acute bilateral foot drop, and had T12-L1 disc herniation with intradural extension. Also this is the first presented case of T12-L1 disc herniation with intradural extension causing acute bilateral foot drop. We performed emergent decompressive laminectomy to both of the patients and extrude disc materials were excised. Both of the patients were recovered with favorable outcome. PMID:25972945

  11. Bilateral taurodontism in primary dentition with hypodontia

    PubMed Central

    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

  12. Bilateral Ovarian Fibrothecoma Associated with Ascites, Bilateral Pleural Effusion, and Marked Elevated Serum CA-125

    PubMed Central

    Loué, Védi André Serges; Gbary, Eléonore; Koui, Sylvanus; Akpa, Bédi; Kouassi, Adélaide

    2013-01-01

    Background. The risk of ovarian cancer is increased in the association of ovarian tumor, ascites, and hydrothorax with the significant elevated tumor marker CA-125. However, this association can be observed in a rare clinical and benign pathological entity, that is Demons-Meigs' syndrome. Objective. To describe a rare case of Demons-Meigs' syndrome observed in our department. Methods. A black African woman of 35 years old, seventh gravida and fourth parous, underwent a total abdominal hysterectomy with bilateral salpingoophorectomy for large bilateral ovarian masses associated with significant ascites, bilateral pleural effusion, and particular highly elevated tumor marker CA-125 (1835 UI/mL) in a pronounced general alteration condition. Results. The postoperative course was uneventful characterized by a complete remission of hydrothorax and ascites with normal level of CA-125 three months after tumor excision. Histology of both masses revealed a bilateral ovarian fibrothecoma, a benign tumor of the ovary, thus confirming the diagnosis of Demons-Meigs' syndrome. Conclusion. The Demons-Meigs syndrome, although it strongly mimics the clinical picture of malignant metastatic ovarian cancer, remains a disease with benign prognosis after surgical tumor resection. This is a rare condition that must be known and recognized by practitioners to avoid unnecessary practices. PMID:23431489

  13. Bilateral Anterior Shoulder Dislocation

    PubMed Central

    Siu, Yuk Chuen; Lui, Tun Hing

    2014-01-01

    Introduction: Unilateral anterior shoulder dislocation is one of the most common problems encountered in orthopedic practice. However, simultaneous bilateral anterior dislocation of the shoulders is quite rare. Case Presentation: We report a case of a 75-year-old woman presented with simultaneous bilateral anterior shoulder dislocation following a trauma, complicated with a traction injury to the posterior cord of the brachial plexus. Conclusions: Bilateral anterior shoulder dislocation is very rare. The excessive traction force during closed reduction may lead to nerve palsy. Clear documentation of neurovascular status and adequate imaging before and after a reduction should be performed. PMID:25685749

  14. 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

  15. Bilateral inferior turbinate osteoma

    PubMed Central

    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

  16. 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

  17. Subsequent bilateral thalamic haemorrhage

    PubMed Central

    Perez, Jesus; Scherle, Claudio; Machado, Calixto

    2009-01-01

    Simultaneous or subsequent bilateral thalamic haemorrhage is rare, and most reported cases are from Asian countries. An 80-year-old white Cuban man, with a history of arterial hypertension, suffered sudden onset of right hemiparesis. Computed tomography (CT) scan showed a left posteromedial thalamic haemorrhage. Two days later his condition suddenly deteriorated: blood pressure was 220/105 mm Hg, he was stuporous and tetraplegic, respiration was ataxic, and his gaze was fixed and deviated downward and inward. CT scan showed haemorrhages in both thalami, extending to the ventricles. 32 h later the patient died. There are few previous publications of simultaneous or subsequent bilateral thalamic haemorrhages and this is the first report involving a Hispanic patient. Prognosis in patients with bilateral thalamic haemorrhage is poor, and the mechanism underlying the development of subsequent and symmetrical bleeding is not clear. PMID:21709830

  18. Treatment of bilateral urolithiasis.

    PubMed

    Matsushita, K; Tanikawa, K; Masuda, A; Matsunaga, J; Matsuzaki, S

    1992-12-01

    Patients with bilateral urolithiasis diagnosed at their first visit were followed for at least one year after the start of treatment. In this retrospective study of 123 patients, the basic metabolic workup revealed no specific underlying cause of simultaneously occurring bilateral nephrolithiasis, and none of the currently used therapeutic regimes proved to be efficient in attaining clinically acceptable stone-free rates at the 3, 6 and 12-month follow-ups. Of 38 patients treated with extracorporeal shockwave lithotripsy (SWL), 21 underwent treatment of both kidneys and 10 (48%) were free of stones bilaterally after 12 months. All of the 17 patients treated with unilateral SWL failed to achieve a stone-free state on the contralateral side. In another group, treated with medication alone to minimize risks of stone recurrence, only 3 of 26 (11.5%) patients were stone-free on both sides 12 months after the start of medication. Since we achieved a stone-free state of both kidneys in no more than 12% of the non-medicated cases, it seems warranted to treat bilateral urolithiasis with SWL more frequently, particularly when patients cannot return regularly to the stone clinic for a longterm follow-up.

  19. Bilateral undescended testes.

    PubMed

    Snyder, H M

    1993-01-01

    The management of boys with undescended testes appears to be an increasingly common problem. While Scorer (1964) [11] reported an incidence of 0.8% of undescended testes in boys of one year of age, a recent survey (Radcliffe Hospital, Oxford, 1986) [18] suggests the incidence has risen to as high as 1.6%. While the etiology may be varied, it is quite clear that many cases represent disturbances of the hypothalamic-pituitary-gonadal axis and may represent a forme fruste of hypogonadotropic hypogonadism [3]. Bilateral undescended testes might logically appear to represent the worst end of such an endocrinopathy. Indeed, a review of the experience at The Children's Hospital of Philadelphia [12] of boys with bilateral undescended testes indicated a greater number of CNS abnormalities (P = 0.0000) than in boys with unilateral undescended testes (Table 1). Also consistent with bilateral cryptorchid testes representing the most severe end of the spectrum was the reported findings from that study that bilateral undescended testes also exhibit a significantly greater number of associated G-U anomalies (P = 0.0004).

  20. Bilateral renal lymphoangiomatosis

    PubMed Central

    Raed, Alqahtani; Sultan, Alkhateeb; Bader, Al-Mutairi

    2015-01-01

    Introduction Renal lymphangiomatosis is a rare congenital benign disease of renal lymphatic system, here we are presenting a very rare form of disease which is bilateral form. Presentation of the case A young adult presented to our clinic after being referred from primary care clinic with intermittent bilateral flank pain and no other symptoms after extensive radiological investigations diagnosis has been made and confirmed by radiological finding of disease. Active treatment usually preserved for complex cases and for the complications of the disease but in our patient as needed analgesia worked well in controlling his intermittent pain and his wish not to pursue any intervention. The vague presentation with initial imaging rising suspicion of renal tumor or complex renal cyst might cause psychological street on the patient, which our patient had, but reassurance after extensive radiological work up relive that's stress. Discussion Although it is very rare disease to be bilateral but wide variety of other differential diagnoses make importance of disease recognition and accurate diagnosis is the key. Conclusion Renal lymphangiomatosis is a rare benign disease of renal lymphatic, which usually affect one side, but bilateral form is very rare form, which may raise the suspicions of genetic form of renal malignancy. Accurate diagnosis requires work up to role out malignant and other renal tumor, which require active surgical management. PMID:26719997

  1. 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

  2. Bilateral compressive optic neuropathy secondary to bilateral sphenoethmoidal mucoceles.

    PubMed

    Newton, N; Baratham, G; Sinniah, R; Lim, A

    1989-01-01

    We have presented a rare case of bilateral posterior sphenoethmoidal sinus mucoceles with bilateral compressive optic neuropathy. While the duration of compression was variably present over a 10-month period, there were nevertheless significant improvements in visual acuity of the right eye and visual fields bilaterally following extensive optic nerve decompression.

  3. 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

  4. Bilateral parotid neoplasms.

    PubMed

    Williams, C

    1980-09-01

    We are reporting what we believe to be the second case of bilateral parotid neoplasm occuring synchronously with differing histologies. We believe that this neoplasm may represent a specific propensity of parotid tissue in a given individual to develop neoplasia in a multipotential glandular organ subject to many neoplastic diseases. There also exists the possibility that this condition may represent an immune deficiency specific to the parotid gland. The case reports that have been reviewed demonstrate that a secondary neoplasm may arise from benign mixed tumors. We question whether this is an example of monistic origin of acinic cell tumor from a mixed-cell tumor or, perhaps, dualistic expression for this salivary gland to develop a multiplicity of anaplastic and metaplastic growth variants. In approaching a case with bilateral parotid involvement, the physician must be aware of the rare possibility that benign and malignant tumors may coexist. He must be prepared to proceed with more radical extirpation than simple superficial parotidectomy.

  5. Bilateral combined laryngocele

    PubMed Central

    Suqati, Abrar A.; Alherabi, Ameen Z.; Marglani, Osama A.; Alaidarous, Tariq O.

    2016-01-01

    Laryngocele is an uncommon condition that represents a benign dilatation of the laryngeal saccule with air and/or fluid, arising in the region of the laryngeal ventricle. Laryngoceles, or laryngomucocele can be classified as internal, or combined. The aim of presenting this rare case of a bilateral combined laryngocele, are to emphasize the importance of diagnostic laryngoscopy in upper airway pathologies evaluation, increase awareness in the general otolaryngologist community, and to highlight the external surgical method. PMID:27464869

  6. [Spontaneous bilateral Petit hernia].

    PubMed

    Fontoura, Rodrigo Dias; Araújo, Emerson Silveira de; Oliveira, Gustavo Alves de; Sarmenghi Filho, Deolindo; Kalil, Mitre

    2011-01-01

    Petit's lumbar hernia is an uncommon defect of the posterior abdominal wall that represents less than 1% of all abdominal wall hernias. It is more often unilateral and founded in young females, rarely containing a real herniated sac. There are two different approaches to repair: laparoscopy and open surgery. The goal of this article is to report one case of spontaneous bilateral lumbar Petit's hernia treated with open surgery.

  7. Multispectral bilateral video fusion.

    PubMed

    Bennett, Eric P; Mason, John L; McMillan, Leonard

    2007-05-01

    We present a technique for enhancing underexposed visible-spectrum video by fusing it with simultaneously captured video from sensors in nonvisible spectra, such as Short Wave IR or Near IR. Although IR sensors can accurately capture video in low-light and night-vision applications, they lack the color and relative luminances of visible-spectrum sensors. RGB sensors do capture color and correct relative luminances, but are underexposed, noisy, and lack fine features due to short video exposure times. Our enhanced fusion output is a reconstruction of the RGB input assisted by the IR data, not an incorporation of elements imaged only in IR. With a temporal noise reduction, we first remove shot noise and increase the color accuracy of the RGB footage. The IR video is then normalized to ensure cross-spectral compatibility with the visible-spectrum video using ratio images. To aid fusion, we decompose the video sources with edge-preserving filters. We introduce a multispectral version of the bilateral filter called the "dual bilateral" that robustly decomposes the RGB video. It utilizes the less-noisy IR for edge detection but also preserves strong visible-spectrum edges not in the IR. We fuse the RGB low frequencies, the IR texture details, and the dual bilateral edges into a noise-reduced video with sharp details, correct chrominances, and natural relative luminances. PMID:17491451

  8. 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

  9. 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.

  10. 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

  11. Bilateral maculopathy associated with Pierre Robin sequence.

    PubMed

    Witmer, Matthew T; Vasan, Ryan; Levy, Richard; Davis, Jessica; Chan, R V Paul

    2012-08-01

    Pierre Robin sequence has been associated with a number of ocular complications, including myopia, strabismus, Möbius syndrome, nasolacrimal duct obstruction, glaucoma, cataract, microphthalmos, coloboma of choroid, and retinal detachment. We report a 10-day-old boy who presented with micrognathia, glossoptosis, and cleft palate as well as multiple congenital anomalies. Ophthalmic examination was notable for bilateral maculopathy, with focal areas of retinal and retinal pigment epithelial atrophy. The association of Pierre Robin sequence and maculopathy has been reported only twice previously.

  12. Bilateral Pseudoexfoliation Deposits on Intraocular Lens Implants

    PubMed Central

    Bonafonte Marquez, Elena; Bonafonte Royo, Sergio

    2015-01-01

    We present a rare case of bilateral pseudoexfoliative deposits on both intraocular lens (IOL) implants in an 83-year-old woman with no other associated pathology, 5 years after cataract surgery. Pseudoexfoliation syndrome is the most common cause of secondary open-angle glaucoma worldwide and these deposits are usually found on the natural lens. The fact that pseudoexfoliative deposits have been found on IOL implants implies the need for a thorough examination in pseudophakic patients, for it could be the only sign of secondary glaucoma. PMID:25713742

  13. [Anesthesiologic peculiarities in bilateral pheochromocytoma and suspected multiple endocrine neoplasia].

    PubMed

    Hack, G; Mihanovic, N; Helpap, B

    1985-10-01

    Based on a case report with surgical removal of bilateral giant phaeochromocytomas with heredofamilial affliction and suspicion of multiple endocrine neoplasia type II (MEN-II syndrome) and of malignancy, the anaesthetic implications are discussed in a patient inadvertently not prepared by antiadrenergic treatment. It is pointed out that in MEN II syndrome, a combination of phaeochromocytoma and medullary carcinoma of the thyroid, surgical removal of the phaeochromocytoma merits utmost priority before thyreoidectomy. The most important laboratory parameters in the diagnosis of MEN II syndrome are pre- and postoperative determinations of calcitonin and carcinoembryonal antigen (CEA).

  14. Bilateral accessory thoracodorsal artery.

    PubMed

    Natsis, Konstantinos; Totlis, Trifon; Tsikaras, Prokopios; Skandalakis, Panagiotis

    2006-09-01

    The subscapular artery arises from the third part of the axillary artery and gives off the circumflex scapular and the thoracodorsal arteries. Although anatomical variations of the axillary artery are very common, the existence of a unilateral accessory thoracodorsal artery has been described in the literature only once. There are no reports of bilateral accessory thoracodorsal artery, in the literature. In the present study, a bilateral accessory thoracodorsal artery, originating on either side of the third part of the axillary artery, is described in a 68-year-old female cadaver. All the other branches of the axillary artery had a typical origin, course, distribution and termination. This extremely rare anatomical variation apart from the anatomical importance also has clinical significance for surgeons in this area. Especially, during the dissection or mobilization of the latissimus dorsi that is partly used for coverage problems in many regions of the body and also in dynamic cardiomyoplasty, any iatrogenic injury of this accessory artery may result in ischemia and functional loss of the graft.

  15. [Metachronous bilateral Wilms' tumor].

    PubMed

    Mambié Meléndez, M; Guibelalde Del Castillo, M; Nieto Del Rincón, N; Rodrigo Jiménez, D; Femenia Reus, A; Román Piñana, J M

    2002-03-01

    Wilms' tumor occurs in 5-10 % of all cases of nephroblastoma. The metachronous form represents 2-3 % of cases. Most (96.2 %) metachronous tumors appear within the first 5 years of the primary tumor. Associated malformations are more common in bilateral cases. Metachronous tumors are a therapeutic challenge. We describe the case of an 11-year-old girl with left hemihypertrophy. The diagnosis was metachronous relapse of Wilms' tumor 7 years after the first diagnosis. The patient received five courses of preoperative chemotherapy and tumorectomy was performed. Because of post-surgical complications, nephrectomy was performed on her only kidney. Since she is anephric, the patient is in chronic renal failure and is dependent on dialysis. Treatment with carboplatin and etoposide was continued after surgery and the patient is currently in complete remission. The appearance of a metachronous Wilms' tumor 5 years after that of the primary tumor is rare. When a contralateral tumour develops, chemotherapy must be given until the size of the tumor is reduced in order to preserve renal function and avoid dialysis. In patients with chronic renal failure caused by bilateral nephrectomy, ongoing treatment with dialysis support can be achieved through the choice of effective drugs and knowledge of their pharmacokinetics and pharmacodynamics.

  16. [Trichorhinophalangeal syndrome. Apropos of 4 cases discovered in adults].

    PubMed

    Madoule, P; Le Balc'h, T; Petrel, P; Doyon, D

    1984-02-01

    Of four adult patients with the trichorhinophalangeal syndrome, 3 were from one family, one had associated major femoral trochlear dysplasia and bilateral mandibular condyle dysplasia, and another a bilateral coxa plana. PMID:6716338

  17. Bilateral macrostomia associated with aqueductal stenosis and glial heterotopias.

    PubMed

    Pepe, Ernesto; Petricig, Paola; Peretta, Paola; Cinalli, Giuseppe

    2007-09-01

    We report on an Italian boy, born to normal and nonconsanguineous parents with a prenatal diagnosis of ventriculomegaly and subependymal glial heterotopias. At birth bilateral macrostomia was diagnosed without other evident facial anomalies. Magnetic resonance imaging (MRI) showed triventricular hydrocephalus and aqueductal stenosis and confirmed the nodules of glial heterotopia. The bilateral macrostomia was surgically corrected with the vermilion square flap method and W-plasty technique and follow up MRI at 6 months showed mild increase of ventricular dilatation without signs of active hydrocephalus. The association between macrostomia and hydrocephalus has been reported only in rare cases of complex malformative syndromes but never with isolated macrostomia.

  18. Reversible posterior encephalopathy syndrome associated with micronodular adrenocortical disease and Cushing syndrome.

    PubMed

    Lodish, Maya; Patronas, Nicholas J; Stratakis, Constantine A

    2010-01-01

    We report a 6-year-old girl with ACTH-independent Cushing syndrome secondary to bilateral adrenal hyperplasia; she presented with hypertension and seizures, and magnetic resonance imaging shows changes consistent with posterior reversible encephalopathy syndrome.

  19. Pediatric isolated bilateral iliac aneurysm.

    PubMed

    Chithra, R; Sundar, R Ajai; Velladuraichi, B; Sritharan, N; Amalorpavanathan, J; Vidyasagaran, T

    2013-07-01

    Aneurysms are rare in children. Isolated iliac artery aneurysms are very rare, especially bilateral aneurysms. Pediatric aneurysms are usually secondary to connective tissue disorders, arteritis, or mycotic causes. We present a case of a 3-year-old child with bilateral idiopathic common iliac aneurysms that were successfully repaired with autogenous vein grafts.

  20. Bilateral Adrenal Incidentalomas: A Case Report and Review of Diagnostic Challenges

    PubMed Central

    Carlson, Anders L.; Marney, Annis M.; Anderson, Scott R.; Gilbert, Matthew P.

    2013-01-01

    Incidentally discovered adrenal masses (incidentalomas) are common and present challenges both in diagnosis and management. When incidentally discovered adrenal masses are bilateral, a refined diagnostic approach is warranted since bilateral disease is more likely to be pathologic. We review a case of a 34-year-old man with incidentally discovered bilateral adrenal nodules. A comprehensive diagnostic strategy led to the diagnosis of bilateral pheochromocytoma caused by von Hippel-Lindau syndrome. He was successfully treated with bilateral laparoscopic adrenalectomy and has recovered well. While the initial diagnostic approach is similar to the unilateral incidentaloma, additional testing and/or genetic testing should be considered in the case of the bilateral adrenal mass. PMID:23401807

  1. Bilateral pseudogout 8 years after bilateral total knee arthroplasty.

    PubMed

    Levi, Gabriel S; Sadr, Kamran; Scuderi, Giles R

    2012-11-01

    This article presents the clinical features of crystal arthropathy after knee replacement. The current literature on pseudogout and gout after both total and partial knee replacement is summarized. A case of bilateral pseudogout 8 years after initial total knee arthroplasty (TKA) is used to highlight the clinical characteristics and treatment options for this underrecognized condition. Presentation mimicked a late septic joint arthroplasty with sudden onset of pain and effusion. The patient was treated successfully with an arthrotomy, debridement, synovectomy, polyethylene insert exchange, oral steroids, and nonsteroidal anti-inflammatories. There are no other reported cases of bilateral pseudogout after bilateral TKA.

  2. 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

  3. Do patients with incidentally discovered bilateral adrenal nodules represent an early form of ARMC5-mediated bilateral macronodular hyperplasia?

    PubMed

    Emms, Holly; Tsirou, Ioanna; Cranston, Treena; Tsagarakis, Stylianos; Grossman, Ashley B

    2016-09-01

    Bilateral adrenal macronodular hyperplasia (BMAH) is a rare form of Cushing's syndrome characterised by the presence of bilateral secretory adrenal nodules and hypercortisolism. Familial studies support a genetic basis for BMAH, and the disease has been linked to mutations in ARMC5, a gene shown to have a tumour suppressor-like action in the development of adrenal nodules. This study aimed to investigate whether ARMC5 mutations play a role in the development of incidentally discovered bilateral adrenal nodules. We investigated 39 patients with incidentally discovered bilateral adrenal nodules >0.8 cm in diameter who underwent extensive biochemical testing to look for signs of subclinical hypercortisolism. Genomic DNA was analysed by Sanger sequencing, using primers targeted to ARMC5 transcripts. Of the 39 patients included in our study, three were identified as having variants in ARMC5. Two of these are unlikely to be clinically significant, but there is evidence that the third mutation, Chr16:g.31476122;c.1778G>C (p.Arg593Pro), may be pathogenic. Another variant, affecting the same amino-acid residue c.1777C>T (p.Arg593Trp), has been identified previously in two studies of BMAH patients, where it has been shown to segregate with disease in one BMAH family. This patient had biochemical evidence of hypercortisolism in the absence of overt Cushing's syndrome, and underwent bilateral adrenalectomy separated in time. The presence of a probably clinically significant mutation in ARMC5 in one patient with bilateral adrenal incidentalomas adds to the growing body of evidence in support of ARMC5 as a critical mediator of adrenal nodule development. In addition, the absence of significant ARMC5 mutations in 38 of our patients represents an important negative finding, demonstrating the degree of variability within the pathogenesis of adrenal nodule development. PMID:27306888

  4. Bilateral and symmetrical tinea mammae.

    PubMed

    Yilmaz, Murat; Kavak, Ayse; Yamaner, Nalan Jale

    2013-09-14

    Tinea corporis has rarely been reported in some locations such as on the breast skin as unilaterally. Herein, we present a case of bilateral tinea mammae, which has not been reported before in English language literature to our knowledge.

  5. 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

  6. Bilateral Renal Vein Thrombosis due to Elevated Factor VIII Levels.

    PubMed

    Patole, Shalom; Ramya, I

    2015-08-01

    Recent evidence has shown that high level of factor VIII is associated with increased risk of thromboembolism. High factor VIII levels are associated with a seven-fold increase in the risk of venous thrombosis. Renal vein thrombosis is usually associated with nephrotic syndrome, procoagulant state or oral contraceptive pills. We report a case of a lady who presented with bilateral renal vein thrombosis due to high factor VIII levels and oral contraceptive pills (OCP) use. PMID:27604446

  7. [Bilateral sarcoidosis of parotid glands].

    PubMed

    Hahn, Pernille; Krogdahl, Annelise; Godballe, Christian

    2012-04-23

    We describe an unusual case of sarcoidosis in which the patient presented with a bilateral swelling of the parotid salivary glands and no other manifestation of the disease. Sarcoidosis is a multisystem granulomatous disorder of unknown cause in which there may be multiple exocrine involvement, including the salivary glands. This case emphasises the importance of including sarcoidosis in the differential diagnosis of bilateral parotid swelling. PMID:22533935

  8. Lehman syndrome: a new syndrome for pierre robin sequence.

    PubMed

    Correia-Sá, Inês; Horta, Ricardo; Neto, Tiago; Amarante, José; Marques, Marisa

    2015-05-01

    Lehman syndrome, or lateral meningocele syndrome, is characterized by facial dysmorphism, multiple lateral meningoceles, and skeletal abnormalities. Only nine cases have been described. We present a case of a 2-year-old boy presenting with micrognathia, glossoptosis, and hypertelorism as well as associated severe obstructive sleep apnea. He was submitted to bilateral mandibular distraction with external nonresorbable devices to correct Pierre Robin sequence (PRS). Later, multiple lateral meningoceles were identified, and a diagnosis of Lehman syndrome was made. Lehman syndrome must be considered in syndromic infants with PRS. Distraction osteogenesis is a safe procedure that is effective as a first choice in the treatment of patients with Lehman syndrome presenting with micrognathia.

  9. Thoracic outlet syndrome following breast implant rupture.

    PubMed

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

    2015-03-01

    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

  10. 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

  11. 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

  12. Spontaneous bilateral quadriceps tendon rupture.

    PubMed

    Vigneswaran, N; Lee, K; Yegappan, M

    2007-11-01

    Spontaneous bilateral quadriceps tendon ruptures are uncommon. We present a 30-year-old man with end-stage renal failure, who sustained this injury, and subsequently had surgical repair of both tendons on separate occasions. He has since regained full range of movement of both knees.

  13. 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

  14. 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.

  15. Bilateral Ureteral Obstruction in Children after Appendectomy

    PubMed Central

    Grande, M.; Lisi, G.; Bianchi, D.; Bove, P.; Miano, R.; Esser, A.; De Sanctis, F.; Neri, A.; Grande, S.; Villa, M.

    2015-01-01

    Acute renal failure due to bilateral ureteral obstruction is a rare complication after appendectomy in children. We report a case of bilateral ureteric obstruction in a 14-year-old boy nine days after surgery for an acute appendicitis. After saline-filling of the urinary bladder, transabdominal ultrasound demonstrated bilateral hydronephrosis of moderate degree. No abscess was found with CT but presence of millimetric stones on both distal ureters was shown, with bilateral calyceal dilatation. Cystoscopy revealed inflammatory changes in the bladder base. Following introduction of bilateral ureteric stents, there was rapid normalisation of urinary output and serum creatinine. PMID:26295001

  16. 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.

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

    PubMed

    Govindaraj, Santhiya; Viswanathan, Stalin

    2011-07-01

    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.

  18. Laparoscopic Cortical Sparing Adrenalectomy for Pediatric Bilateral Pheochromocytoma: Anesthetic Management

    PubMed Central

    Rajappa, Geetha Chamanhalli; Anandaswamy, Tejesh Channasandra

    2014-01-01

    Introduction: Pheochromocytoma is a catecholamine-secreting tumor, which is seen rarely in children. These tumors predominantly secrete norepinephrine and epinephrine. They might be familial and associated with hereditary tumors such as Von Hippel-Lindau syndrome and multiple endocrine neoplasia type II. Case Presentation: The child might present with a spectrum of clinical manifestation including hypertension, headache, visual disturbances, and behavioral problems. A meticulous preoperative preparation is essential for a stable intraoperative and postoperative outcome Conclusions: We described successful perioperative management of a child who underwent bilateral laparoscopic cortical sparing adrenalectomy and a repeated surgery for the residual tumor removal. PMID:24790902

  19. 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

  20. Spontaneous bilateral kidney rupture during autologous stem cell transplantation in a patient affected by amyloidosis

    PubMed Central

    Ferrannini, Michele; Vischini, Gisella; De Angelis, Gottardo; Giannakakis, Konstantinos; Arcese, William

    2011-01-01

    Kidney spontaneous rupture is not a recognized complication neither for amyloidosis nor of autologous stem cell transplantation (ASCT). A 46-year-old white woman, affected by nephrotic syndrome, was diagnosed as AL amyloidosis by renal biopsy. We report the singular case of a bilateral spontaneous kidney rupture during ASCT for AL with renal rescue. PMID:25984105

  1. 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.

  2. 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

  3. Bilateral Advantages in Subitizing With Visual Masking.

    PubMed

    Pryor, Campbell G; Howe, Piers D L

    2015-01-01

    Performance on a range of visual-processing tasks has been shown to improve when information is split bilaterally across the left and right visual hemifields rather than being restricted to a single visual hemifield. However, a recent study by Delvenne et al. found no such bilateral advantage for subitizing, which is our ability to rapidly and accurately enumerate small quantities of objects. This finding is particularly surprising, as it contradicts the prediction of FINgers of INSTantiation theory that subitizing should benefit from bilateral presentation. Our study investigated the issue by determining if there are any circumstances where a bilateral advantage for subitization occurs. Contrary to Delvenne et al., we found that subitizing could show bilateral advantages, but only when the display was backward-masked. We discuss these findings in relation to how the rate of encoding and the time available for this encoding may affect bilateral advantages in subitizing. A general model is proposed under which bilateral advantages could be explained.

  4. 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

  5. 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

  6. Bilateral ECT induces bilateral increases in regional cortical thickness

    PubMed Central

    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

  7. 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.

  8. [Congenital bilateral vocal cord paralysis].

    PubMed

    Meyer, Lars Christian; Godballe, Christian

    2009-01-12

    Congenital bilateral vocal cord paralysis (CBVCP) is a rare but potentially life-threatening condition and awareness of the condition is necessary to ensure early diagnosis and treatment. This case describes a 25-month-old boy suffering from CBVCP. The main symptoms at birth were inspiratory stridor combined with a normal voice and feeding problems. The difficulties in achieving the right diagnosis are demonstrated, and the treatment so far, including tracheotomy and a feeding tube, is outlined. The importance of fibre optic laryngoscopy in both diagnosis and control is stressed. PMID:19174021

  9. Independent bilateral primary bronchial carcinomas

    PubMed Central

    Chaudhuri, M. Ray

    1971-01-01

    Independent bilateral primary bronchial carcinomas are not common. Since Beyreuther's description in 1924, 16 well-documented cases of independent primary bronchial carcinomas of different histology have been described. From 1965 to 1970, eight cases were seen at the London Chest Hospital. In order to make the diagnosis of a second primary bronchial carcinoma, each tumour should be malignant and neither should be a metastasis from the other. To meet this last criterion, the histopathological features of the two tumours must be different. Many cases have been described in the literature as double primary bronchial carcinomas where the second primary had the same histological features as the first. Images PMID:4327711

  10. Amelogenesis imperfecta with bilateral nephrocalcinosis

    PubMed Central

    Poornima, P; Katkade, Shashikant; Mohamed, Roshan Noor; Mallikarjuna, Rachappa

    2013-01-01

    A 12-year-old patient presented with a severe delay of eruption in permanent maxillary and mandibular incisors. On examination, there was over-retained primary teeth and delayed eruption of permanent teeth. Retained primary teeth showed light yellow discolouration whereas permanent teeth were distinct yellow with thin or little enamel. Subsequent imaging revealed all the premolars except maxillary left first premolar showed signs of intra-alveolar coronal resorption, nephrocalcinosis with bilateral multiple calculi and small papillary tip calcifications, marked increase in alkaline phosphatase. Subsequent dental treatment for restoring the functional and aesthetic requirement followed by appropriate treatment for renal problem was undertaken. PMID:23709541

  11. Heterogeneity in Waardenburg syndrome.

    PubMed Central

    Hageman, M J; Delleman, J W

    1977-01-01

    Heterogeneity of Waardenburg syndrome is demonstrated in a review of 1,285 patients from the literature and 34 previously unreported patients in five families in the Netherlands. The syndrome seems to consist of two genetically distinct entities that can be differentiated clinically: type I, Waardenburg syndrome with dystopia canthorum; and type II, Waardenburg syndrome without dystopia canthorum. Both types have an autosomal dominant mode of inheritance. The incidence of bilateral deafness in the two types of the syndrome was found in one-fourth with type I and about half of the patients with type II. This difference has important consequences for genetic counseling. Images Fig. 7 Fig. 8 Fig. 9 PMID:331943

  12. 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

  13. [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

  14. [Bilateral occipital infarction with central homonymous hemianopia].

    PubMed

    Ishikawa, H; Tanabe, Y

    1991-09-01

    A 45-year-old man of bilateral occipital infarction with central homonymous hemianopia is reported. He was admitted to our hospital with complaints of visual loss and large central scotoma on both eyes. Pupillary light reaction and ocular fundi were normal. On admission, bilateral retrobulbar optic neuritis was suspected. However, congruous, irregular central scotomas with vertical step were observed in both eyes with Goldmann perimetry. X-ray computed tomography (CT) and magnetic resonance imaging (MRI) revealed the infarction in bilateral occipital tips. Bilateral central homonymous hemianopia is considered to be rare and a case in which occipital lobe lesions were detected with X-ray CT and MRI has not been reported. We stress that bilateral central scotomas with vertical step indicate bilateral occipital lesions.

  15. 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

  16. 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

  17. 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

  18. Successful Bilateral Composite Ear Reattachment

    PubMed Central

    2014-01-01

    Summary: A successful bilateral ear composite graft nonmicrosurgical reattachment is presented. In cases where suitable vessels are unavailable for microsurgical revascularization, the reconstructive challenge can be formidable for salvaging the unique anatomic and aesthetic structure of the ear. The case is presented of an 18-year-old woman who was a victim of an assault wherein both of her ears were intentionally amputated by her attacker. She underwent successful surgical reattachment followed by a postoperative regimen of hyperbaric oxygen, cooling, and meticulous wound care. The patient achieved 100% survival of her left ear graft and 95% survival of her right ear graft. Clinical photographs at 18 months are presented, along with a discussion of the possible implications for other reconstructive applications. PMID:25289367

  19. CUSHING'S SYNDROME

    PubMed Central

    Wilkinson, Allan B.

    1961-01-01

    Sixteen cases of verified Cushing's syndrome, and twelve cases of probable Cushing's syndrome were reviewed and data on them were compared with various reports on Cushing's syndrome in the literature. The diagnosis hinges upon a high index of suspicion, and one or several of the major criteria may be lacking. Ultimate establishment of correct diagnosis should be based largely on the clinical features, although stimulation and suppression tests may help to confirm a clinical diagnosis. In well-established clinical cases, with borderline laboratory confirmation, exploration may be justified, especially if tests fail to identify a specific cause. In cases of adrenal cortical tumor, all pathological tissue should be removed if possible, with great care to support and stimulate the remaining atrophic adrenal gland during and following operation. In cases of bilateral adrenal cortical hyperplasia, the problem is one of how much to remove. At present most investigators advocate radical subtotal resection, leaving less than 10 per cent of one side. ImagesFigure 1.Figure 2.Figure 3.Figure 4.Figure 5.Figure 6. PMID:13785315

  20. Tortuosity of the retinal vessels in Aarskog syndrome (faciogenital dysplasia).

    PubMed

    Pizio, H F; Scott, M H; Richard, J M

    1994-03-01

    Aarskog syndrome (faciogenital dysplasia) is a genetic growth disorder characterized by short stature, cryptorchidism, shawl scrotum, and dysmorphic facial features. Ophthalmic findings include hypertelorism, blepharoptosis, strabismus, and ophthalmoplegia. The authors report a patient with Aarskog syndrome and bilateral retinal vessel tortuosity. This is the first retinal anomaly associated with Aarskog syndrome.

  1. 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

  2. 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

  3. 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.

  4. 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

  5. Postlaminectomy Bilateral Lumbar Intraspinal Synovial Cysts

    PubMed Central

    Cho, Sung Ik; Lee, Jung Hwan

    2016-01-01

    Lumbar intraspinal synovial cysts are included in the difference diagnosis of lumbar radiculopathy. Developing imaging modalities has result in increased reporting about these lesions. However, the case of bilateral new lumbar intraspinal synovial cysts after laminectomy has been rarely reported. We report of a rare case with bilateral lumbar intraspinal synovial cysts after laminectomy, requiring surgical excision. PMID:27799997

  6. 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.

  7. Extrapyramidal syndrome

    PubMed Central

    Panda, Akhila Kumar; Bala, Kiran; Bhirud, Lomesh

    2014-01-01

    Organophosphate (OP) poisoning is a common occurrence in the rural areas of developing countries like India. Acute cholinergic crisis is one of the important causes of mortality related to OP poisoning. Delayed peripheral neuropathy, extrapyramidal syndromes and neuropsychiatric manifestations are the major consequences of secondary neuronal damage. This case illustrates a 14-year-old girl who ingested 50 mL of OP pesticide and developed extrapyramidal symptoms in the form of parkinsonism and hand dystonia in spite of immediate medical attention. MRI of the brain with T2, fluid attenuated inversion recovery and diffusion-weighted sequences revealed bilateral symmetrical basal ganglia hyperintensities. Further follow-up revealed a significant clinical improvement with marked resolutions of the brain lesions. The reversible extrapyramidal symptoms with disappearance of neuroimaging findings without neuropathy or neuropsychiatric manifestations are unusual in OP poisoning. PMID:24398867

  8. Meibomian gland dysfunction in floppy eyelid syndrome.

    PubMed

    Gonnering, R S; Sonneland, P R

    1987-01-01

    A 41-year-old obese man presented with bilateral ocular irritation and clinical findings consistent with floppy eyelid syndrome. Light- and electron-microscopic examination of tarsus removed at surgical correction revealed cystic degeneration and squamous metaplasia of the meibomian glands, along with abnormal keratinization and granuloma formation. These findings, not reported before in this syndrome, suggest that meibomian gland dysfunction with attendant qualitative abnormalities of the tear film, may be partly responsible for the keratoconjunctivitis seen in this syndrome.

  9. 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

  10. 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.

  11. [True hermaphroditism with bilateral ovotestis].

    PubMed

    Güitrón, A; Forsbach, G; González, J M; Garza, J G; Vázquez, J; Espinoza, G

    1998-01-01

    A nineteen years old woman with ambiguous external genitalia was studied. This condition had been previously identified as a newborn, but her parents refused medical attention and it was reared as a girl. At 12-years, she began spontaneous mammary development, appearing pubic and axillary hair, and clitoral enlargement. The menarche occurred at 15-years and it was followed by irregular periods. Physical examination, showed absence of hirsutism and acne, normal mammary development equivalent to grade V of Tanner. The external genitalia showed fused labio-scrotal folds with an small introitus. The urethral meatus was absent and was later located inside the introitus. There was a big phallus similar to an adult penis with a normal glans, flexed by a chordee. Hormonal determinations discarded congenital adrenal hyperplasia. The karyotype was 46,XX and testosterone levels were in adult male range. Pelvic ultrasonography disclosed a normal uterus and both gonads in confirmed by laparoscopy identifying bilateral ovotestis. Testicular tissue was removed and plastic reconstruction of female genitals was done. PMID:9528217

  12. 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.

  13. 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

  14. 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

  15. 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

  16. Two case reports of bilateral adrenal myelolipomas

    PubMed Central

    Yang, Yu; Ye, Lin-Yang; Yu, Bo; Guo, Jia-Xiang; Liu, Qian; Chen, Yun

    2015-01-01

    Primary adrenal myelolipoma is a rare, non-functioning adrenal benign tumor that is composed of mature adipose tissue and a variable amount of haemopoietic elements. Clinically, it is difficult to get diagnosed with adrenal myelolipoma because the patient usually doesn’t have obvious symptoms and signs in early stage. In the present study, two cases of primary bilateral adrenal myelolipomas are reported. Clinical presentation, imaging diagnostic features, histopathological changes and surgical treatments of the two patients are discussed. Preoperative diagnostic imaging examinations (B-mode ultrasonography, computed tomography and magnetic resonance imaging sans) assisted getting a prediction diagnosis of bilateral adrenal myelolipomas. A two-stage surgery was used to successfully excise bilateral adrenal myelolipomas in the two patients. Conventional open adrenalectomy was applied to remove the adrenal myelolipomas greater than 6 cm, and laparoscopic adrenalectomy was performed to excise the adrenal tumors smaller than 6 cm. Bilateral adrenal myelolipomas of the two patients were finally confirmed by postoperative histopathological examinations. Understanding clinical, imaging diagnostic and histopathological features of bilateral adrenal myelolipomas will facilitate timely diagnosis and treatment of this condition. Surgical removal of bilateral adrenal myelolipomas is safe, curative and beneficial. The two-stage surgery appears to be the best treatment option for the patients with bilateral adrenal myelolipomas because it achieves optimal treatment effectiveness with minimized sequelae. PMID:26380835

  17. 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.

  18. 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

  19. 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

  20. Bilateral dacryocystoceles in a pregnant woman.

    PubMed

    Hirabayashi, Kristin E; Yang, Elizabeth; Echegoyen, Julio; Yoon, Steven J; Tao, Jeremiah P

    2014-01-01

    The authors describe, for the first time, bilateral, sequential large dacryocystoceles during pregnancy and review the literature for this presentation. A 26-year-old, 15-week pregnant woman presented with OD epiphora, diplopia, and pain in the setting of an inferomedial orbital mass. Surgical exploration and histopathology were consistent with a dacryocystocele, and a dacryocystorhinostomy was curative. She returned at 34-week gestation, with an identical presentation on the left side. Review of the literature reveals that dacryocystoceles occasionally present in adults; however, bilateral involvement may be unusual. Bilateral dacryocystoceles have not been previously reported in a pregnant woman.

  1. 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.

  2. Simultaneous bilateral decortications via video-assisted thoracic surgery for bilateral empyema

    PubMed Central

    Nose, Naohiro; Anami, Toshiki

    2014-01-01

    Introduction Bilateral empyema is a rare and life-threatening condition that is difficult to treat. We herein report a case of bilateral empyema that was treated with simultaneous bilateral decortications via video-assisted thoracic surgery (VATS). Presentation of case A 38-year-old female complained of chest pain, dyspnea, and high grade fever lasting two weeks. Computed tomography revealed bilateral notching pleural effusion and pneumonia with atelectasis. Bilateral thoracic drainage was performed. From the right chest, white pus was drained, and Streptococcus anginosus was identified. The left drainage fluid was serous, and no bacteria were identified. We diagnosed the patient with right empyema and left para-pneumonic effusion consequent to pneumonia. Because conservative therapies could not resolve the inflammatory findings, simultaneous bilateral VATS decortications were performed. Both thoracic cavities had loculated pleural effusion. In contrast to the preoperative findings, white pus was found in not only the right, but also the left thoracic cavity. She had an uncomplicated postoperative course and recovered. Discussion Bilateral empyema that has developed to the fibrinopleural phase is difficult to treat with drains alone. Bilateral VATS decortications helped to make a definitive diagnosis and treat both sides simultaneously. Conclusion Simultaneous bilateral VATS decortications should be considered as a feasible and effective procedure for bilateral empyema that is refractory to medical treatment. PMID:25528031

  3. Occult secretion of acth and bilateral adrenalectomy: sometimes the messenger has to be shot.

    PubMed

    Chicharro, Sandra; Lecube, Albert; Mesa, Jordi

    2008-12-01

    Cushing's syndrome due to ectopic adrenocorticotrophic hormone (ACTH) secretion is infrequent, the most frequent cause being bronchial carcinoid; nevertheless, in a considerable proportion of cases (up to 16%), the origin of ACTH secretion is not found. Treatment of Cushing's syndrome due to ectopic ACTH secretion is surgical resection of the tumor. However, surgical excision is not always feasible, either because the tumor has not been located, or because it is unresectable; in these cases the question is when to carry out a bilateral adrenalectomy. We present a case of ACTH-dependent hypercortisolism, in which the ectopic origin of ACTH secretion was established from the results of complementary tests (suppression tests, petrosal sinus sampling, imaging); nevertheless, none of the imaging tests was able to identify the secreting tumor. Consequently, after a reasonable interval, a bilateral adrenalectomy was performed, with good clinical results.

  4. 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.

  5. 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.

  6. Bilateral areolar and periareolar pityriasis versicolor.

    PubMed

    Sárdy, Miklós; Korting, Hans Christian; Ruzicka, Thomas; Wolff, Hans

    2010-08-01

    An adolescent boy presented with isolated, symmetrical, bilateral areolar and periareolar pityriasis versicolor. This extremely rare condition should be considered in the differential diagnosis of light brown patches on the areolae.

  7. Bilateral giant abdominoscrotal hydroceles in childhood.

    PubMed

    Serels, S; Kogan, S

    1996-05-01

    There is a paucity of cases in the literature describing the abdominoscrotal hydrocele (ASH). We report the diagnostic and therapeutic aspects of a rapidly expanding giant bilateral ASH in a 4-month-old boy.

  8. Impaired threat prioritisation after selective bilateral amygdala lesions.

    PubMed

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

    2015-02-01

    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.

  9. [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

  10. 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

  11. [Symptomatic cyst of the pancreas and asymptomatic bilateral phaeochromocytoma

    PubMed

    Karvar, S; Breidert, M; Nagel, M; Kirsch, C; Pinkert, J; Ehninger, G

    2001-01-01

    Symptomatic cyst of the pancreas and asymptomatic bilateral phaeochromocytoma. HISTORY AND CLINICAL FINDINGS: A 39-year-old woman was admitted to our department of gastroenterology with recurrent epigastrical pain. Ten years previously the diagnosis of von Hippel-Lindau (VHL)-syndrome has been established. Two years before a germ line mutation in exon 3 of the VHL-tumour suppressor gene has been detected. The patient has a healthy son with a normal VHL-gene and four healthy siblings who had refused a genetic blood test. INVESTIGATIONS: At abdominal ultrasound at the head of the pancreas three 4 2 cm large cysts and in the region of the left adrenal gland a 2,9 2,7 cm large tumor were visible. MRI of the abdomen revealed in addition a 2,2 1,5 cm large tumour of the right adrenal gland. An asymptomatic biadrenal phaeochromocytoma was detected by elevated urine catecholamines and 123I-MIBG-scintigraphy. TREATMENT AND COURSE: The cysts of the pancreas were punctured under endosonographical control and analysis of the cyst fluid was not suspicious of a malignant cystic tumour. The patient had no further abdominal complaints. After oral treatment with the alpha-blocker phentolamine the biadrenal phaeochromocytoma was treated by retroperitoneal laparoscopic surgery in an organ-sparing fashion. Postoperatively ACTH-stimulating test revealed a normal cortisol response. CONCLUSION: Adrenocortical function can be preserved by la-paroscopic adrenal-sparing surgery in bilateral phaeochromocytoma.

  12. 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

  13. Bilateral giant abdominoscrotal hydroceles complicated by appendicitis.

    PubMed

    Yarram, Sai G; Dipietro, Michael A; Graziano, Kathleen; Mychaliska, George B; Strouse, Peter J

    2005-12-01

    Abdominoscrotal hydrocele is a rare entity, with fewer than 100 cases reported in children. Bilateral abdominoscrotal hydroceles are even less common, with 14 cases reported in children. Various complications of abdominoscrotal hydrocele have been reported in the literature. We present a 4-month-old boy with bilateral giant abdominoscrotal hydroceles who developed appendicitis apparently because of obstruction from the right hydrocele. We discuss the various imaging modalities used to establish the diagnosis and plan the operative approach.

  14. [Multiple bilateral renal hydatidosis. A case report].

    PubMed

    Bennani, S; Ait Bolbarod, A; el Mrini, M; Benjelloun, S

    1995-01-01

    The authors report one case of multiple bilateral hydatid cyst of kidney associated to brain and spleen localizations, after intra cardiac rupture. They point out the rarity of the bilateral kidney localization of hydatidosis, explain the dissemination mode, and stress the role of computed tomography for the pre-operative diagnosis. The treatment is above all surgical and adapted to each case. The post-operative results are satisfying. Complementary medical treatment could be useful. PMID:7486851

  15. [Bilateral rupture of the quadriceps tendon].

    PubMed

    Modrego, Francisco J; Molina, Juan

    2004-01-01

    Simultaneous, bilateral, and spontaneous rupture of the quadriceps tendon is a very rare injury. Individuals with this injury are usually predisposed by chronic renal disease with secondary hyperparathyroidism, gout, diabetes, and lupus erythematosus. Often, primary diagnostic confusion can lead to a delay in treatment. Two cases of a bilateral lesion of the quadriceps tendon, that were treated surgically using the technique of Scuderi, followed by an intense rehabilitation programme, are presented. Surgical treatment yields satisfactory results.

  16. Simultaneous bilateral tibial tubercle avulsion fracture.

    PubMed

    Maar, D C; Kernek, C B; Pierce, R O

    1988-11-01

    Bilateral simultaneous tibial tubercle avulsion fractures are extremely rare. The present case was a 16-year-old boy who sustained bilateral simultaneous tibial tubercle avulsion fractures (Watson-Jones Type III) from jumping while playing basketball. Both fractures were treated successfully by open reduction and internal fixation with screws. Three years later, the patient had the screws removed because of knee pain and tenderness over the screws.

  17. 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

  18. 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

  19. Isolated lower lip fistulas in Van der Woude syndrome.

    PubMed

    Etöz, Osman A; Etöz, Abdullah

    2009-09-01

    Van der Woude syndrome (VWS) is a dominantly inherited disease of orofacial region. Characteristic features of this syndrome are bilateral lower lip sinuses along with cleft lip or palate deformity. However, isolated lower lip pits in VWS without any cleft syndrome is uncommon. Lip pits in VWS are usually asymptomatic; however, patients may complain of watery drainage and/or infection. In this report, asymptomatic isolated lower lip sinuses without any cleft syndrome in a patient and his father are presented. PMID:19816310

  20. 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. PMID:27504371

  1. 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

  2. An unusual presentation of Kabuki syndrome: clinical overlap with CHARGE syndrome.

    PubMed

    Verhagen, Judith M A; Oostdijk, Wilma; Terwisscha van Scheltinga, Cecilia E J; Schalij-Delfos, Nicoline E; van Bever, Yolande

    2014-09-01

    Kabuki syndrome is a rare genetic disorder characterized by intellectual disability and multiple congenital anomalies, including short stature, peculiar facial appearance, skeletal anomalies, a variety of visceral malformations and abnormal dermatoglyphic patterns. We describe a case of Kabuki syndrome presenting with atypical features, consisting of bilateral microphthalmia, coloboma, anal atresia and panhypopituitarism, showing considerable phenotypic overlap with CHARGE syndrome. This report demonstrates that clinical follow-up and molecular genetic testing can be useful for establishing the correct diagnosis. PMID:24862881

  3. Apparently new "anophthalmia-plus" syndrome in sibs.

    PubMed

    Fryns, J P; Legius, E; Moerman, P; Vandenberghe, K; Van den Berghe, H

    1995-08-28

    The index patient of this report is a 17-week-gestation female fetus with bilateral anophthalmia, bilateral cleft lip/cleft palate, macrotia with bilateral lateral facial cleft, large open sacral neural tube defect, and uterus unicornis. Parents were normal and nonconsanguineous with an unremarkable family history. Their first child, a 4-year-old boy, is normal. The second child, a 2 1/2-year-old boy, has bilateral anophthalmia and an abnormal left ear with absent lobule as the sole additional anomaly. These 2 sibs seem to be the first examples of a new "anophthalmia-plus" syndrome apparently inherited as autosomal-recessive.

  4. 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

  5. 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

  6. Lenz micropthalmia syndrome with associated orbital cysts.

    PubMed

    Rafailov, Leon; Dattilo, Michael; Shinder, Roman

    2014-01-01

    An 8-year-old boy presented for oculoplastic evaluation of bilateral microphthalmia. He had multiple other congenital anomalies, including microcephaly, wide-spaced teeth, sloping shoulders, protruding ears, syndactyly, a posterior urethral valve, cystic dysplasia of the kidneys, and a bicuspid aortic valve. Taken together, these findings supported the diagnosis of Lenz microphthalmia syndrome. CT of the orbits revealed bilateral microphthalmic globes with associated colobomatous cysts. To the authors' knowledge, this is the first reported case of Lenz microphthalmia syndrome with associated orbital cysts.

  7. 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

  8. Bilateral Sequential Pneumolabyrinth Resulting from Nose Blowing

    PubMed Central

    Lee, Joong Seob; Kwon, Sae Young; Kim, Ji Heui

    2015-01-01

    Pneumolabyrinth describes a condition with entrapped air in the labyrinth and usually occurs in temporal bone fractures that involve the otic capsule. While sporadic cases of bilateral pneumolabyrinth have been reported, cases lacking head trauma are very rare. We report the case of a 43-year-old man who had sudden hearing loss bilaterally after blowing his nose at an interval of 1 year. Although conservative management for the right ear and exploratory tympanotomy with sealing of the possible site of perilymphatic leakage in the left ear were performed, hearing outcome was poor in both ears. To our knowledge, this is the first case of bilateral pneumolabyrinth occurring as a result of nose blowing. PMID:26771019

  9. Bilateral Sequential Pneumolabyrinth Resulting from Nose Blowing.

    PubMed

    Lee, Joong Seob; Kwon, Sae Young; Kim, Ji Heui; Kim, Hyung-Jong

    2015-12-01

    Pneumolabyrinth describes a condition with entrapped air in the labyrinth and usually occurs in temporal bone fractures that involve the otic capsule. While sporadic cases of bilateral pneumolabyrinth have been reported, cases lacking head trauma are very rare. We report the case of a 43-year-old man who had sudden hearing loss bilaterally after blowing his nose at an interval of 1 year. Although conservative management for the right ear and exploratory tympanotomy with sealing of the possible site of perilymphatic leakage in the left ear were performed, hearing outcome was poor in both ears. To our knowledge, this is the first case of bilateral pneumolabyrinth occurring as a result of nose blowing. PMID:26771019

  10. Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome, LKS) and regressive autistic disorders with epileptic EEG abnormalities: the continuing debate.

    PubMed

    Deonna, Thierry; Roulet-Perez, Eliane

    2010-10-01

    Early-onset acquired epileptic aphasia (Landau-Kleffner syndrome) may present as a developmental language disturbance and the affected child may also exhibit autistic features. Landau-Kleffner is now seen as the rare and severe end of a spectrum of cognitive-behavioural symptoms that can be seen in idiopathic (genetic) focal epilepsies of childhood, the benign end being the more frequent typical rolandic epilepsy. Several recent studies show that many children with rolandic epilepsy have minor developmental cognitive and behavioural problems and that some undergo a deterioration (usually temporary) in these domains, the so-called "atypical" forms of the syndrome. The severity and type of deterioration correlate with the site and spread of the epileptic spikes recorded on the electroencephalogram within the perisylvian region, and continuous spike-waves during sleep (CSWS) frequently occur during this period of the epileptic disorder. Some of these children have more severe preexisting communicative and language developmental disorders. If early stagnation or regression occurs in these domains, it presumably reflects epileptic activity in networks outside the perisylvian area, i.e. those involved in social cognition and emotions. Longitudinal studies will be necessary to find out if and how much the bioelectrical abnormalities play a causal role in these subgroup of children with both various degrees of language and autistic regression and features of idiopathic focal epilepsy. One has to remember that it took nearly 40 years to fully acknowledge the epileptic origin of aphasia in Landau-Kleffner syndrome and the milder acquired cognitive problems in rolandic epilepsies.

  11. Baller-Gerold syndrome associated with dextrocardia.

    PubMed

    Ceylan, A; Peker, E; Dogan, M; Tuncer, O; Kirimi, E

    2011-01-01

    Baller-Gerold Syndrome (BGS) is a rare autosomal recessive disorder that is apparent at birth. The disorder is characterized by distinctive malformations of the skull and facial area and bones of the forearms and hands. We report a 4 year old boy in whom the clinical features of craniosynostosis and bilateral absent thumbs and radii led to a diagnosis of Baller-Gerold syndrome. Physical examination revealed that the heart was localized to the right side. Echocardiography confirmed dextrocardia. Dextrocardia has not previously been reported with Baller-Gerold syndrome. To the best of our knowledge, this is the first reported case of Baller-Gerold syndrome associated with dextrocardia.

  12. [Bilateral choroidal osteoma--a case report].

    PubMed

    Jędrychowska-Jamborska, Justyna; Kulig-Stochmal, Agnieszka; Markiewicz, Anna; Jakubowska, Barbara; Romanowska-Dixon, Bożena

    2014-01-01

    Choroidal osteoma is a an extremely rare (especially located bilaterally), benign, intraocular tumor, the type of choristoma. It occurs between 2-3 decades of life, women are particularly vulnerable. The main complication in 1/3 cases is a subretinal neovascularization which may cause bleeding. The gradually progressive decalcification develops within the tumour over time, which causes atrophy of the retinal pigment epithelium and Bruch's membrane deformity. The article presents a case of a 26-year-old woman with bilateral choroidal osteoma complicated by subretinal hemorrhage; the diagnosis was based on clinical examination (biomicroscopy and indirect ophthalmoscopy) as well as specialised tests including: ultrasonography, optical coherence tomography, and fluorescein angiography.

  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. Inducing comprehension in the bilateral poor reader.

    PubMed

    Rattan, G; Dean, R S; Lowrie, R E

    1987-09-01

    The comprehension effects of changes in the spatial configuration of prose were examined with reading disabled children who differed in laterality preference. Specifically, 24 learning disabled boys were presented with prose materials in a standard, phrased, and backward (right to left) fashion. Measures of comprehension showed that text in the phrased and backward conditions differentially facilitated comprehension for the more bilateral subjects but had little effect on more right lateralized subjects. The results were discussed in terms of the instructional implications of alterations in the visual-spatial arrangement of prose materials for bilateral readers.

  15. Idiopathic harlequin syndrome: a pediatric case.

    PubMed

    Jain, Puneet; Arya, Ravindra; Jaryal, Ashok; Gulati, Sheffali

    2013-04-01

    Harlequin syndrome, Harlequin sign, Holmes-Adie syndrome, and Ross syndrome lie on a spectrum of partial dysautonomias affecting facial sudomotor, vasomotor, and pupillary responses. These syndromes have imprecise clinical boundaries and overlap syndromes are known. We report a 9-year-old girl who presented with anhidrosis over the right half of her face and the left side of her body, with compensatory hyperhidrosis on the contralateral side. She was noted to have bilateral tonic pupils and normal muscle stretch reflexes with other features suggestive of autonomic dysfunction. Investigations to rule out secondary causes were noncontributory. Her clinical presentation can be categorized as partial overlap between Harlequin syndrome and Holmes-Adie syndrome. PMID:22638078

  16. Application of electrophysiological methods and magnetic resonance tomographic angiography in the differentiation between hemifacial spasm and Meige syndrome.

    PubMed

    Huang, Chuyi; Miao, Suhua; Chu, Heling; Muheremu, Aikeremujiang; Wu, Jinting; Zhou, Rongsong; Zuo, Huancong; Ma, Yu

    2016-05-01

    Bilateral hemifacial spasm and Meige syndrome can be easily confused due to their similar clinical manifestation. Here, we aimed to investigate the application of electrophysiological methods and magnetic resonance tomographic angiography (MRTA) in the differentiation between hemifacial spasm and Meige syndrome. 10 patients with bilateral hemifacial spasm and 9 patients with Meige syndrome received electrophysiological monitoring of nerves. There were two males and eight females with bilateral hemifacial spasm, aged 16-58 years with a course of 5-54 months. For the patients with Meige syndrome, there were three males and six females, aged 51-68 years with a course of 12-36 months. All patients received conventional MRTA of the brain blood vessels before decompression. We found that all patients with Meige syndrome showed synchronous contraction of bilateral orbicularis oculi muscles and (or) burst discharge from orbicularis oris muscles in surface electromyography (sEMG). However, those with hemifacial spasm presented with bilaterally asynchronous burst discharge. Electromyography for patients with Meige syndrome did not record abnormal muscle response (AMR), but recorded AMR for those with bilateral hemifacial spasm. The offending vessels were compressed in patients with hemifacial spasm in MRTA, while MRTA results were generally negative for those with Meige syndrome. Combining sEMG and AMR detection in EMG and MRTA, bilateral hemifacial spasm can be differentiated from Meige syndrome with a reduction of misdiagnosis rate.

  17. 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

  18. [Giant vertebro-basilar aneurysm. Frontal syndrome].

    PubMed

    Rosa, A; Mizon, J P; Sevestre, H

    1991-01-01

    A 72-year-old man presented with an apparent frontal syndrome. He also had bilateral trigeminal neuralgia, a pyramidal syndrome of all 4 limbs, balance disturbances, a horizontal nystagmus when looking to the left and a right velopalatine paralysis. CT scan with contrast showed a hyperdense rounded lesion in the left cerebello-pontine angle. Cerebral angiography showed this to be a large aneurysm of the end of the vertebral arteries. The patient died suddenly. Autopsy confirmed the site and presence of the aneurysm. Balance disturbances, the pyramidal syndrome and velopalatine paralysis could all be explained by brain stem compression and the bilateral nature of the trigeminal neuralgia by compression of the trigemino-thalamic tract. The apparent frontal syndrome, the authors suggest could have resulted from subacute raised intracranial pressure.

  19. 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

  20. Bilateral renal agenesis in an alpaca cria

    PubMed Central

    Gerard, Mathew P.; Spaulding, Kathy A.; Geissler, Kyleigh A.; Anderson, Kevin L.

    2006-01-01

    Abstract A 3-day-old male alpaca cria was presented for lack of vigor and failure to urinate since birth. Based on the history, laboratory data, ultrasonographs, surgical findings, and postmortem examination, the cria was diagnosed with bilateral renal agenesis and hypoplastic bladder, a congenital condition rarely seen in veterinary medicine. PMID:16579043

  1. Bilateral low condylectomy of the mandible.

    PubMed

    Worthington, P

    1980-03-01

    The operation of low condylectomy of the mandible is seldom performed bilaterally. The indications and consequences are reviewed, and a case is reported in which the adverse sequelae commonly attributed to this procedure were avoided over an 11-year follow-up period. PMID:6928301

  2. [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

  3. Bilateral maxillary paramolars: a case report.

    PubMed

    Dhull, Kanika Singh; Acharya, Sonu; Ray, Prayas; Yadav, Shweta; Prabhakaran, Sheeja Devi

    2012-01-01

    Supernumerary teeth are a common clinical and radiographic finding and may produce occlusal and dental problems. Supernumerary teeth can present in various forms and in any region of the mandible or maxilla, but have a predisposition for the anterior maxilla. The purpose of this paper was to present a case of supernumerary teeth located in the maxillary molar region bilaterally. PMID:22828764

  4. Bilateral chylothorax, chylopericardium and chylous ascitis

    PubMed Central

    Kashyap, Anil; Mahajan, Vineet; Whig, Jagdeep; Gupta, Sushil

    2011-01-01

    Non-Hodgkin’s lymphoma (NHL) can commonly present as chylothorax and rarely as chylopericardium. Here we are presenting a case of a 21-years-old female with bilateral chylothorax, chylopericardium and chylous ascites all together finally diagnosed to have NHL as the etiology. To the best of our knowledge, it has been reported very infrequently. PMID:21712926

  5. "Ostrich sign" indicates bilateral vertebral artery dissection.

    PubMed

    Rose, David Z; Husain, M Rizwan

    2012-11-01

    Vertebral artery dissections (VADs) comprise about 2% of ischemic strokes and can be associated with trauma, chiropractic manipulation, motor vehicle collisions, whiplash, amusement park rides, golfing, and other motion-induced injuries to the neck. We present a case of bilateral extracranial VAD as a complication of conducting an orchestra. To our knowledge, this has not been documented in the literature. Conceivably, vigorous neck twisting in an inexperienced, amateur conductor may place excessive rotational forces upon mobile portions of the verterbral arteries, tear the intima, deposit subintimal blood that extends longitudinally, and cause neck pain and/or posterior fossa ischemic symptoms. Magnetic resonance angiography examinations of axially oriented slices of bilateral VADs resemble the face of an ostrich. This observation is similar to the "puppy sign," in which bilateral internal carotid artery dissections resemble the face of a dog. Craniocervical dissections of either the carotid or vertebral arteries have the potential to form an aneurysm, cause artery-to-artery embolism, or completely occlude the parent artery, resulting in an ischemic stroke. Because bilateral VADs in axial magnetic resonance angiographic sections stand out like the eyes of an ostrich, and because the fast identification of VADs is so critical, we eponymize this image the "ostrich sign."

  6. Management of Bilateral Carotid Occlusive Disease

    PubMed Central

    Jadhav, Ashutosh P.; Ducruet, Andrew F.; Jankowitz, Brian T.; Jovin, Tudor G.

    2016-01-01

    Background Symptomatic bilateral internal carotid occlusive disease is a rare but potentially devastating entity. Medical therapy alone is associated with high rates of mortality and recurrent stroke. The optimal management of this disease remains poorly understood. Methods A retrospective review of a prospectively maintained database was conducted for patients who presented with an acute stroke in the setting of bilateral carotid occlusive disease between May and October 2013. Results We identified 3 patients. The admission National Institutes of Health Stroke Scale score ranged from 4 to 7. All patients had small- to moderate-sized infarcts in the anterior circulation on presentation. Angiography confirmed bilateral internal carotid occlusions with collateral filling via the posterior communicating artery and retrograde filling via external carotid artery supply to the ophthalmic artery. All patients were initially managed with permissive hypertension and anticoagulation followed by carotid angioplasty and stenting. At 1-year follow-up, all patients demonstrated a modified Rankin scale score of 0-1. Conclusions Carotid stenting may be a safe and effective therapy for patients presenting with symptomatic bilateral carotid occlusions. PMID:27051405

  7. 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

  8. [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.

  9. 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.

  10. Synchronous bilateral breast cancer in a male

    PubMed Central

    Rubio Hernández, María Caridad; Díaz Prado, Yenia Ivet; Pérez, Suanly Rodríguez; Díaz, Ronald Rodríguez; Aleaga, Zaili Gutiérrez

    2013-01-01

    Male breast cancer, which represents only 1% of all breast cancers, is occasionally associated with a family history of breast cancer. Sporadic male breast cancers presenting with another primary breast cancer are extremely rare. In this article, we report on a 70-year-old male patient with bilateral multifocal and synchronous breast cancer and without a family history of breast cancer. PMID:24319497

  11. Bilateral canalicular adenoma of the parotid gland.

    PubMed

    Liess, Benjamin D; Lane, Robert V; Frazier, Shellaine; Zitsch, Robert P

    2006-03-01

    Canalicular adenoma is a rare benign salivary gland tumor that occurs almost exclusively in the upper lip. Rarely, this benign tumor may occur multifocally in the oral cavity. We report a case of canalicular adenoma in bilateral parotid glands, discuss histological characteristics, and review this tumor.

  12. Bilateral nasolabial cysts: a case report.

    PubMed

    Lee, Jennifer; Christmas, Peter I

    2009-06-01

    The nasolabial cyst is one of the rarer soft tissue cysts, and presents beneath the alae of the nose. There have been few reports of an association between nasolabial cysts and dacryocystitis (an infection of the nasolacrimal sac). This case report describes a female who presented with bilateral nasolabial cysts and longstanding symptoms of epiphora (watery eye).

  13. Bilateral nonfistulous congenital coronary arterial aneurysms.

    PubMed

    Wilson, C S; Weaver, W F; Zeman, E D; Forker, A D

    1975-02-01

    A 15 year old boy collapsed and died after participating in a basketball game. Autopsy revealed bilateral congenital coronary arterial aneurysms. The diagnosis was made post mortem but, retrospectively, might have been suspected during life, even before angiography. The clues to the correct diagnosis were chest pain, a systolic and diastolic murmur and a mass on the right heart border in the chest roentgenogram.

  14. 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

  15. 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

  16. Auriculotemporal Syndrome (Frey Syndrome).

    PubMed

    Motz, Kevin M; Kim, Young J

    2016-04-01

    Frey syndrome is a common sequela of parotidectomy, and although it is not frequently manifested clinically, it can cause significant morbidity for those affected. Frey syndrome results from synkinetic autonomic reinnervation by transected postganglionic parasympathetic nerve fiber within the parotid gland to the overlying sweat glands of the skin. Many surgical techniques have been proposed to prevent the development of Frey syndrome. For those who develop clinical symptoms of Frey syndrome, objective testing can be performed with a Minor starch-iodine test. Some of the current methods to prevent and treat symptomatic Frey syndrome are reviewed. PMID:26902982

  17. Increased cardiovascular mortality following early bilateral oophorectomy

    PubMed Central

    Rivera, Cathleen M.; Grossardt, Brandon R.; Rhodes, Deborah J.; Brown, Robert D.; Roger, Véronique L.; Melton, L. Joseph; Rocca, Walter A.

    2008-01-01

    Objective To investigate the mortality associated with cardiovascular diseases and the effect of estrogen treatment in women who underwent unilateral or bilateral oophorectomy before menopause. Design We conducted a cohort study with long-term follow-up of women in Olmsted County, MN, who underwent either unilateral or bilateral oophorectomy before the onset of menopause from 1950 through 1987. Each member of the oophorectomy cohort was matched by age to a referent woman from the same population who had not undergone any oophorectomy. We studied the mortality associated with cardiovascular disease in a total of 1,274 women with unilateral oophorectomy, 1,091 women with bilateral oophorectomy, and 2,383 referent women. Results Women who underwent unilateral oophorectomy experienced a reduced mortality associated with cardiovascular disease compared with referent women (hazard ratio [HR], 0.82; 95% confidence interval [CI], 0.67–0.99; P = 0.04). By contrast, women who underwent bilateral oophorectomy before age 45 years experienced an increased mortality associated with cardiovascular disease compared with referent women (HR, 1.44; 95% CI, 1.01–2.05; P = 0.04). Within this age stratum, the HR for mortality was significantly elevated in women who were not treated with estrogen through age 45 years or longer (HR, 1.84; 95% CI, 1.27–2.68; P = 0.001) but not in women treated (HR, 0.65; 95% CI, 0.30–1.41; P = 0.28; test of interaction, P = 0.01). Mortality was further increased after excluding deaths associated with cerebrovascular causes. Conclusions Bilateral oophorectomy performed before age 45 years is associated with increased cardiovascular mortality, especially with cardiac mortality. However, estrogen treatment may reduce this risk. PMID:19034050

  18. Adult-onset bulbar ptosis in Joubert syndrome

    PubMed Central

    Burt, Benjamin; Levine, Johanan; Le, Kim

    2012-01-01

    In this case report, we describe a case of adult-onset bulbar ptosis in a patient with Joubert syndrome. Joubert syndrome is a rare neurodevelopmental disorder with malformations in cerebellum and brainstem. Many ocular abnormalities have been noted in Joubert syndrome, but the association of this syndrome with adult-onset ptosis has not been described to date. This 24-year-old Joubert patient developed a cerebrospinal fluid cyst in her midbrain. She had signs of bilateral third nerve palsy and abducens palsy in the left eye. The bilateral central third nerve palsy causing functional blindness secondary to severe bilateral levator palsy was treated successfully with silicone sling frontalis suspension, as the seventh nerve nucleus was not involved. PMID:22291457

  19. 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

  20. 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

  1. Tourette syndrome

    MedlinePlus

    Gilles de la Tourette syndrome; Tic disorders - Tourette syndrome ... Tourette syndrome is named for Georges Gilles de la Tourette, who first described this disorder in 1885. The disorder is likely passed down through families. The syndrome may be linked to ...

  2. 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

  3. Bilateral congenital pseudoarthrosis of the clavicles in a newborn

    PubMed Central

    Kalagiri, Ram R.; Hemingway, Martha; Beeram, Madhava R.

    2016-01-01

    Bilateral congenital pseudoarthrosis of the clavicles is extremely rare. We report a case of this entity presenting in the neonatal period. We highlight the importance of the differential diagnosis when clavicular fracture shows no evidence of healing or occurs bilaterally.

  4. Incidental finding of bilateral papillary thyroid carcinoma in a patient with primary hyperparathyroidism.

    PubMed

    Alavi, Mehr Sadat; Azarpira, Negar; Mojallal, Mehra

    2010-01-01

    Primary hyperparathyroidism (PHP) affects 0.5%-1% of the adult population and presents with classical signs of renal lithiasis, cholecystolithiasis, gastrointestinal ulcerations, depression, and osteoporosis. Parathyroid adenoma, hyperplasia and rarely carcinoma are the underlying pathology. Synchronous thyroid and parathyroid pathologies are described in multiple endocrine neoplasia. We report a case of a 47 years old woman with non-syndromic concomitant occurrence of bilateral non-medullary thyroid carcinoma diagnosed by histopathology, and with PHP confirmed by (99m)Tc-MIBI scintigraphy, hypercalcemia and elevated serum parathyroid hormone. A head and neck surgeon needs to be aware of the possible coexistence of thyroid and parathyroid lesions. To our knowledge, this is the first report of concomitant PHP and bilateral papillary thyroid cancer in the literature. In conclusion, it is optimal to remove both tumors in one operative procedure. Therefore careful thyroid evaluation should be considered for all patients with PHP.

  5. 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

  6. 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.

  7. First Report of AIDS-Related Burkitt's Lymphoma Presenting as Bilateral Orbital Masses

    PubMed Central

    Alves de Almeida, Monica; Nesi Cardoso Migliano Porto, Juliana; de Brito Lyra, Ana Carolina; Calheiros Leite, Luiz Arthur; Basilio-de-Oliveira, Rodrigo Panno; Neves-Motta, Rogério; Correa, Andréa Ramos; Raphael de Almeida Ferry, Fernando; Grechi Pacheco, Ronaldo

    2016-01-01

    Burkitt's lymphoma (BL) is an aggressive B-cell non-Hodgkin's lymphoma and one of the fastest growing tumors in humans. It is an acquired immunodeficiency syndrome- (AIDS-) defining disease and occurs with relatively preserved CD4 cell counts. It rarely affects the orbital region in the setting of AIDS. We report unusual presentation of a fatal case of AIDS-associated BL in a 42-year-old female patient with severe CD4 cell depletion who presented with dramatic fast growing (within days) bilateral orbital masses leading to striking facial deformities. To the best of our knowledge, this is the first report of bilateral orbital involvement in AIDS-associated BL.

  8. Progressive supranuclear palsy syndrome presenting as progressive nonfluent aphasia: a neuropsychological and neuroimaging analysis.

    PubMed

    Rohrer, Jonathan D; Paviour, Dominic; Bronstein, Adolfo M; O'Sullivan, Sean S; Lees, Andrew; Warren, Jason D

    2010-01-30

    There is currently considerable interest in the clinical spectrum of progressive nonfluent aphasia (PNFA) and progressive supranuclear palsy (PSP) and the intersection of these two entities. Here, we undertook a detailed prospective clinical, neuropsychological, and neuroimaging analysis of 14 consecutive patients presenting with PNFA to identify cases meeting clinical criteria for PSP. These patients had further detailed assessment of extrapyramidal and oculomotor functions. All patients had high-resolution MR brain volumetry and a cortical thickness analysis was undertaken on the brain images. Four patients presenting with PNFA subsequently developed features of a PSP syndrome, including a typical oculomotor palsy. The neuropsychological profile in these cases was similar to other patients with PNFA, however, with more marked reduction in propositional speech, fewer speech errors, less marked impairment of literacy skills but more severe associated deficits of episodic memory and praxis. These PSP-PNFA cases had less prominent midbrain atrophy but more marked prefrontal atrophy than a comparison group of five patients with pathologically confirmed PSP without PNFA and more prominent midbrain atrophy but less marked perisylvian atrophy than other PNFA cases. In summary, although the PSP-PNFA syndrome overlaps with PNFA without PSP, certain neuropsychological and neuroanatomical differences may help predict the development of a PSP syndrome. PMID:20077483

  9. Capgras syndrome: a reduplicative phenomenon.

    PubMed

    Alexander, M P; Stuss, D T; Benson, D F

    1979-03-01

    A patient recovering from a severe head injury developed a prolonged Capgras syndrome in which he believed his wife and five children had been replaced by nearly identical substitutes. Although this phenomenon is considered a functional disorder in the psychiatric literature, recent reports postulate an organic basis. Recent studies of the comparable neurologic disorder, reduplicative paramnesia, have stressed the importance of bilateral frontal and right hemisphere pathology. Neuropsychologic and neuroradiologic data in our patient revealed this combination, suggesting that the Capgras syndrome may be a form of reduplicative paramnesia with the same pathologic substrate.

  10. 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

  11. [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.

  12. Bilateral internal jugular vein ectasia: a report of two cases.

    PubMed

    Gendeh, B S; Dhillon, M K; Hamzah, M

    1994-03-01

    Internal jugular vein ectasia is a venous anomaly commonly presenting as a unilateral neck swelling in children and adults. Literature reports of bilateral presentation are rare. Bilateral Doppler ultrasonography is the diagnostic investigation of choice. The possible pathology, aetiology and management are discussed. Conservative management of bilateral cases is recommended in uncomplicated cases.

  13. 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

  14. The cerebral hemispheres and bilateral neural nets.

    PubMed

    Cook, N D; Beech, A R

    1990-06-01

    A high-level cognitive dichotomy ("language and context") is reviewed in relation to empirical findings concerning the functions of the human cerebral hemispheres. We argue that the right hemisphere's involvement in the generation of connotative and contextual information in parallel with the denotative and literal language functions of the left hemisphere provides an important insight into the organization of viable cognitive systems. The role of the corpus callosum in producing the dichotomy is discussed. Finally, the generation of asymmetrical activity in structurally symmetrical, bilateral neural nets is described. The model demonstrates how complementary memory states can be generated in bilateral nets without assuming different modes of information processing, provided that the nets have inhibitory, homotopic connections. Unlike excitatory connections, inhibitory connections are sufficient to generate asymmetric hemispheric activity without postulating intrinsic differences between the cerebral hemispheres.

  15. 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.

  16. Neuropsychological sequelae of bilateral posteroventral pallidotomy

    PubMed Central

    Turner, K; Reid, W; Homewood, J; Cook, R

    2002-01-01

    Methods: 17 patients with Parkinson's disease were evaluated with a neuropsychological battery before and six months after bilateral pallidotomy. A comparison group (n = 8) was also assessed at six month intervals. Outcome variables were tests of memory, language, visuospatial function, attention, executive skills, and depression. Results: Despite a large number of variables studied, a significant postsurgical change was found only in performance of the tower of London task, a measure of planning abilities. The effect size of this change was larger than that of the comparison group, and a reliable change index score established that 5 of 13 surgical patients had statistically reliable reductions in planning performance. Conclusions: Patients with a young age of onset and long duration of Parkinson's disease who underwent bilateral pallidotomy had a relatively circumscribed reduction in neuropsychological functioning, being limited to motor planning efficiency. These data suggest that the cognitive role of the posteroventral globus pallidus is limited, at least in people with Parkinson's disease. PMID:12235317

  17. [Bilateral acetabulum fracture after suffering sport trauma].

    PubMed

    Trost, P; Kollersbeck, C; Pelitz, M; Walcher, T; Genelin, F

    2013-07-01

    This case study describes a 37-year-old male who suffered a bilateral transverse acetabulum fracture with a fracture of the posterior wall and a double-sided dorsal hip dislocation in combination with a left-sided femoral head fracture (Pipkin IV) while skiing in a "fun park". The accurate diagnosis and presurgical planning was made by means of a computed tomography (CT) scan and a subsequent 3D reconstruction. After a primarily executed shielded repositioning of the bilateral hip dislocationearly secondary and anatomical reconstruction of the double-sided acetabulum fracture was possible using the Kocher-Langenbeck approach. A consistent physiotherapy as well as rehabilitation finally led to a positive clinical result for the patient.

  18. BILATERAL LUXATIO ERECTA, A CASE REPORT

    PubMed Central

    Acosta, César Augusto Xavier; da Silva Resch, Elemar; Rodrigues, Rafael

    2015-01-01

    Inferior shoulder dislocation (luxactio erecta) is a rare lesion affecting approximately 0.5% of dislocations of this joint. The vast majority of these cases occur unilaterally. In September 2004, a 43 year old man was brought to the emergency room of the University Hospital of Santa Maria (HUSM) complaining of bilateral shoulder pain and inability to lower the arms, as a result of a fall with the arms abducted, while carrying out work activities. After physical examination and radiological exams, it was found that the patient had a bilateral inferior shoulder dislocation. He was referred to the surgical ward and after intravenous sedation, both shoulders were reduced by closed reduction using the traction-countertraction maneuver. The patient was discharged the day after the reduction. Both arms were immobilized with a velpeau sling in total adduction and intrarotation, with instructions to maintain immobilization for three weeks. The patient was also advised to receive physiotherapy. PMID:27047840

  19. 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

  20. 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

  1. Bilateral synergies in foot force production tasks.

    PubMed

    Sarabon, Nejc; Markovic, Goran; Mikulic, Pavle; Latash, Mark L

    2013-05-01

    We analysed the effects of task symmetry during bilateral accurate force production tasks performed by the two feet. In particular, we tested a hypothesis that bilateral deficit would lead to higher indices of synergies defined as co-varied adjustments in the two forces across trials that reduced total force variability. The subjects produced steady-state force followed by a quick force pulse into the target. The two feet could be acting both into plantar flexion and into dorsiflexion (symmetrical tasks), or in opposite directions (asymmetrical task). We used the framework of the uncontrolled manifold hypothesis to quantify two variance components, one of which did not change total force (V UCM), while the other did (V ORT). Synergy indices during the asymmetrical task were higher than in either symmetrical task. The difference was due to higher V UCM (compared to the symmetrical plantar flexion task) or lower V ORT (compared to the symmetrical dorsiflexion task). The synergy index showed a drop (anticipatory synergy adjustment, ASA) starting 100-150 ms prior to the force pulse initiation. The ASA tended to be shorter and of a smaller magnitude for the asymmetrical task. This is the first demonstration of bilateral synergies during accurate force production by the legs. We conclude that bilateral deficit has no or weak effects on two-leg synergies. The results fit the earlier introduced scheme with two groups of neural variables defining average performance of a redundant system and patterns of co-variation among its elemental variables, respectively. PMID:23568657

  2. Bilateral optic neuritis due to malaria.

    PubMed

    Chacko, Joseph G; Onteddu, Sanjeeva; Rosenbaum, Eric R

    2013-09-01

    Malaria is a mosquito-borne infectious disease caused by protists of the genus Plasmodium. Malaria is widespread in tropical regions around the equator, including much of sub-Saharan Africa, Asia, and the Americas, and uncommonly seen in the developed world. Although a variety of ocular manifestations have been linked to malaria, optic neuritis is rare. We report a patient who developed bilateral optic neuritis after he was treated successfully for acute falciparum malaria.

  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. Ovarian remnant with bilateral duplicate ureters.

    PubMed

    Lyons, Thomas L; Adolph, Allyson J; Winer, Wendy K

    2003-08-01

    A 27-year-old woman had a history of acute chronic pelvic pain. She had had a previous salpingo-oophorectomy for an endometrioma. A computerized tomographic scan showed a left adnexal mass. She was known to have bilateral duplicate ureters shown on intravenous pyelogram. She underwent laparoscopy and retroperitoneal dissection of endometriosis with excision of the mass from the pelvic sidewall. The final pathology was consistent with a hemorrhagic corpus luteal cyst.

  5. Bilateral Brodie's abscess at the proximal tibia.

    PubMed

    Buldu, Halil; Bilen, Fikri Erkal; Eralp, Levent; Kocaoglu, Mehmet

    2012-08-01

    Brodie's abscess is a form of subacute osteomyelitis, which typically involves the metaphyses of the long tubular bones, particularly in the tibia. The diagnosis is usually made incidentally, as there are no accompanying symptoms or laboratory studies. Bilateral involvement at the proximal tibia is unusual. However, orthopaedic surgeons should be aware of this entity, as it may present without symptoms. Checking the contralateral limb for concomitant Brodie's abscess is recommended.

  6. 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

  7. Fetal alcohol syndrome and the developing socio-emotional brain.

    PubMed

    Niccols, Alison

    2007-10-01

    Fetal alcohol syndrome (FAS) is currently recognized as the most common known cause of mental retardation, affecting from 1 to 7 per 1000 live-born infants. Individuals with FAS suffer from changes in brain structure, cognitive impairments, and behavior problems. Researchers investigating neuropsychological functioning have identified deficits in learning, memory, executive functioning, hyperactivity, impulsivity, and poor communication and social skills in individuals with FAS and fetal alcohol effects (FAE). Investigators using autopsy and brain imaging methods have identified microcephaly and structural abnormalities in various regions of the brain (including the basal ganglia, corpus callosum, cerebellum, and hippocampus) that may account for the neuropsychological deficits. Results of studies using newer brain imaging and analytic techniques have indicated specific alterations (i.e., displacements in the corpus callosum, increased gray matter density in the perisylvian regions, altered gray matter asymmetry, and disproportionate reductions in the frontal lobes) in the brains of individuals prenatally exposed to alcohol, and their relations with brain function. Future research, including using animal models, could help inform our knowledge of brain-behavior relations in the context of prenatal alcohol exposure, and assist with early identification and intervention. PMID:17669569

  8. Sparse MEG source imaging in Landau-Kleffner syndrome.

    PubMed

    Zhu, Min; Zhang, Wenbo; Dickens, Deanna; Ding, Lei

    2011-01-01

    Epilepsy patients with Landau-Kleffner syndrome (LKS) usually have a normal brain structure, which makes it a challenge to identify the epileptogenic zone only based on magnetic resonance imaging (MRI) data. A sparse source imaging technique called variation based sparse cortical current density (VB-SCCD) imaging was adopted here to reconstruct cortical sources of magnetoencephalography (MEG) interictal spikes from an LKS patient. Realistic boundary element (BE) head and cortex models were built by segmenting structural MRI. 148-channel MEG was recorded for 10 minutes between seizures. Total 29 epileptiform spikes were selected for analysis. The primary cortical sources were observed locating at the left intra- and perisylvian cortex. Multiple extrasylvian sources were identified as the secondary sources. The spatio-temporal patterns of cortical sources provide more insights about the neuronal synchrony and propagation of epileptic discharges. Our observations were consistent with presurgical diagnosis for this patient and observation of aphasia in LKS. The present results suggest that the promising of VB-SCCD technique in assisting with presurgical planning and studying the neural network for LKS in determining the lateralization of epileptic origins. It can further be applied to non-invasively localize and/or lateralize eloquent cortex for language for epilepsy patients in general in the future.

  9. Bilateral leg edema in an older woman.

    PubMed

    Thaler, H W; Pienaar, S; Wirnsberger, G; Roller-Wirnsberger, R E

    2015-01-01

    Bilateral leg edema is a frequent symptom in older people and an important concern in geriatric medicine. Further evaluation is frequently not performed and simple therapy with diuretics is prescribed. Particularly in older patients, long-term use of diuretics can lead to severe electrolyte imbalances, volume depletion, and falls. In this case report we want to focus the physicians' attention on the necessity to determine the cause and show a correspondingly effective treatment for bilateral leg edema in older people. A thorough approach is required to recognize diseases and to avoid adverse drug events as geriatric patients often show an atypical presentation or minor symptoms. The cause of swollen legs is often multifactorial; therefore, the patient's individual history and an appropriate physical examination are important. Depending on the clinical symptoms, evaluation including basic laboratory tests, urinalysis, chest radiography, and echocardiogram may be indicated. The most probable cause of bilateral edema in older patients is chronic venous insufficiency. Heart failure is also a common cause. Other systemic causes such as renal disease or liver disease are much rarer. Antihypertensive and anti-inflammatory drugs can frequently cause leg edema, but the incidence of drug-induced leg swelling is unknown. With the help of this special case we tried to develop an approach to the diagnosis of symmetric leg edema in older patients, a problem frequently neglected in geriatric medicine.

  10. 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.

  11. 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

  12. 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.

  13. Adams-Oliver syndrome associated with cutis marmorata telangiectatica congenita and congenital cataract: a case report.

    PubMed

    Fayol, Laurence; Garcia, Patricia; Denis, Danièle; Philip, Nicole; Simeoni, Umberto

    2006-04-01

    A female infant presented with Adams-Oliver syndrome (AOS), intrauterine growth retardation, severe cutis marmorata telangiectatica congenita, bilateral congenital cataract, and periventricular lesions. The here-reported association of bilateral congenital cataract with AOS is original. Adams-Oliver syndrome is a genetic defect that causes a vasculopathy and leads to a variety of phenotypes. This observation further supports the current understanding of the physiopathology of AOS. PMID:16586236

  14. Ultrasound diagnosis of bilateral cataracts in a fetus with possible cerebro-ocular congential muscular dystrophy during the routine second trimester anomaly scan.

    PubMed

    Drought, Alexandra; Wimalasundera, Ruwan; Holder, Susan

    2015-08-01

    The finding of bilateral congenital cataracts in the fetus is rare. We report bilateral congenital cataracts detected during the routine second trimester anomaly scan, which subsequently were found to be associated with other congenital anomalies and the parents opted for a termination of pregnancy. At post-mortem, Muscle-Eye Brain disease or Walker-Warburg Syndrome was considered likely, which are autosomal recessive congenital muscular dystrophy disorders associated with cerebral, cerebellar, muscle and eye anomalies. On ultrasound, bilateral cataracts appear as echogenic, solid areas within the fetal orbits. The examination of the fetal face and orbits plays an important role in confirming fetal well-being antenatally. We propose that it should become a routine part of the structural survey of fetal anatomy during the obstetric anomaly scan. This is especially important in pregnancies previously affected by fetal cataracts or pregnancies at risk of rare genetic syndromes. PMID:27433255

  15. Ultrasound diagnosis of bilateral cataracts in a fetus with possible cerebro-ocular congential muscular dystrophy during the routine second trimester anomaly scan.

    PubMed

    Drought, Alexandra; Wimalasundera, Ruwan; Holder, Susan

    2015-08-01

    The finding of bilateral congenital cataracts in the fetus is rare. We report bilateral congenital cataracts detected during the routine second trimester anomaly scan, which subsequently were found to be associated with other congenital anomalies and the parents opted for a termination of pregnancy. At post-mortem, Muscle-Eye Brain disease or Walker-Warburg Syndrome was considered likely, which are autosomal recessive congenital muscular dystrophy disorders associated with cerebral, cerebellar, muscle and eye anomalies. On ultrasound, bilateral cataracts appear as echogenic, solid areas within the fetal orbits. The examination of the fetal face and orbits plays an important role in confirming fetal well-being antenatally. We propose that it should become a routine part of the structural survey of fetal anatomy during the obstetric anomaly scan. This is especially important in pregnancies previously affected by fetal cataracts or pregnancies at risk of rare genetic syndromes.

  16. Tako-tsubo cardiomyopathy in a patient with bilateral lesions in the dorsal medulla.

    PubMed

    Berganzo, Koldo; Ciordia, Roberto; Gómez-Esteban, Juan C; Tijero, Beatriz; Agundez, Marta; Velasco, Fernando; Valle, Maria A; Zarranz, Juan J

    2011-02-01

    Tako-tsubo-like cardiomyopathy (TTC) is much more common than originally thought. The exact pathophysiology of TTC is unclear. The most accepted theory proposes myocardial stunning of neurogenic origin, supported by the frequent antecedent of emotional or physical stress, suggesting a catecholamine-mediated mechanism. We present a patient with this syndrome and bilateral damage of the dorsal medulla oblongata likely affecting both solitary tract nuclei. Our case points to a link between baroreflex failure and TTC, highlighting the important role of sympathetic discharge in the pathophysiology of TTC. PMID:20963467

  17. Bilateral Granulomatous and Fibrinoheterophilic Otitis Interna due to Pseudomonas aeruginosa in a Captive Little Bustard ( Tetrax tetrax ).

    PubMed

    Scala, Christopher; Langlois, Isabelle; Lemberger, Karin

    2015-06-01

    A captive juvenile little bustard ( Tetrax tetrax ) was presented for acute onset of right head tilt and right circling. The bird failed to respond to supportive care and systemic antibiotic therapy. A bilateral granulomatous and fibrinoheterophilic otitis interna due to Pseudomonas aeruginosa was diagnosed postmortem by histopathologic examination and bacterial culture. In bustards, Pseudomonas species have been documented in the normal bacterial flora of the oropharynx and are frequently reported in upper respiratory tract infections. This is the first report of a peripheral vestibular syndrome due to P aeruginosa otitis interna in a bustard species. Pseudomonas aeruginosa should be included as a possible cause of otitis and peripheral vestibular syndrome in bustards.

  18. Muencke syndrome with cleft lip and palate.

    PubMed

    Anderson, Peter J; Snell, Broughton; Moore, Mark H

    2013-07-01

    Muencke syndrome results from mutations in the FGFR3 gene, and although it is well recognized that the clinical presentation is variable, the important key finding includes coronal synostosis. We present a family where a mother with proven FGFR3 Pro250Arg mutation gave birth to identical twins both of whom had craniosynostosis but had coexisting bilateral cleft lip and palate. We believe that this is the first description of clefting occurring in conjunction with Muencke syndrome and so further extends the range of phenotypic variation that can occur in this syndrome. PMID:23851839

  19. Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome

    PubMed Central

    D'Egidio, Gianni; Wan, Cynthia; Baxter, Alan; Rosenberg, Hans

    2016-01-01

    Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS) with a rare complication of compartment syndrome. Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb) of 220 g/L), hypotension, acute renal failure, and bilateral calf pain. Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion) and respiratory (ventilator) support in the ICU was provided until resolution of intravascular fluid extravasation. Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients.

  20. Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome

    PubMed Central

    D'Egidio, Gianni; Wan, Cynthia; Baxter, Alan; Rosenberg, Hans

    2016-01-01

    Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS) with a rare complication of compartment syndrome. Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb) of 220 g/L), hypotension, acute renal failure, and bilateral calf pain. Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion) and respiratory (ventilator) support in the ICU was provided until resolution of intravascular fluid extravasation. Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients. PMID:27688917

  1. Compartment Syndrome as a Result of Systemic Capillary Leak Syndrome.

    PubMed

    Kyeremanteng, Kwadwo; D'Egidio, Gianni; Wan, Cynthia; Baxter, Alan; Rosenberg, Hans

    2016-01-01

    Objective. To describe a single case of Systemic Capillary Leak Syndrome (SCLS) with a rare complication of compartment syndrome. Patient. Our patient is a 57-year-old male, referred to our hospital due to polycythemia (hemoglobin (Hgb) of 220 g/L), hypotension, acute renal failure, and bilateral calf pain. Measurements and Main Results. The patient required bilateral forearm, thigh, and calf fasciotomies during his ICU stay and continuous renal replacement therapy was instituted following onset of acute renal failure and oliguria. Ongoing hemodynamic (Norepinephrine and Milrinone infusion) and respiratory (ventilator) support in the ICU was provided until resolution of intravascular fluid extravasation. Conclusions. SCLS is an extremely rare disorder characterized by unexplained episodic capillary hyperpermeability, which causes shift of volume and protein from the intravascular space to the interstitial space. Patients present with significant hypotension, hemoconcentration, hypovolemia, and oliguria. Severe edema results from leakage of fluid and proteins into tissue. The most important part of treatment is maintaining stable hemodynamics, ruling out other causes of shock and diligent monitoring for complications. Awareness of the clinical syndrome with the rare complication of compartment syndrome may help guide investigations and diagnoses of these critically ill patients. PMID:27688917

  2. Bilateral Symmetry in Morphogenesis of Embryos

    PubMed Central

    Jehle, Herbert

    1970-01-01

    It is suggested that differentiated embryonic cells have a high specificity of molecular constitution as regards the surface layers surrounding their cellular membranes. Correspondingly, specific interface energies may characterize the early contacts between different cell types. The question is raised whether the morphology of the developing embryo may be understood in terms of cellular arrangements which minimize the total interface energy. Bilateral symmetry prevalent in early embryonic development of higher animals might be understood on the basis of the adoption of such a minimum energy principle if, in addition, one assumes that embryonic development is uniquely determined for a particular species. PMID:5272310

  3. 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.

  4. Acute quadriplegia due to bilateral cerebral metastases.

    PubMed

    Levine, D N; Black, P M; Kleinman, G M; Ojemann, R

    1981-03-01

    A 51-year-old man developed severe quadriparesis without sensory loss after a fall on the occiput. When he was treated with corticosteroids, the weakness worsened slightly for 2 days, but he then progressively improved and ultimately walked unassisted. Two months later, postmortem examination disclosed metastatic tumors in the superior portion of each precentral gyrus. The subjacent white matter was edematous. No spinal cord abnormalities were found. The clinical, radiologic, and neuropathologic findings suggest that the quadriplegia resulted from these bilateral cerebral lesions and not from spinal cord dysfunction. The rapid development of cerebral edema, perhaps precipitated by trauma, seemed to account for the acute onset of symptoms in this case.

  5. 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 ...

  6. 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 ...

  7. 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, ...

  8. Cushing syndrome

    MedlinePlus

    Hypercortisolism; Cortisol excess ... The most common cause of Cushing syndrome is taking too much glucocorticosteroid medicine. This form of Cushing syndrome is called exogenous Cushing syndrome . Prednisone, dexamethasone, and prednisolone are ...

  9. Williams syndrome

    MedlinePlus

    Williams-Beuren syndrome ... Williams syndrome is caused by not having a copy of several genes. Parents may not have any family history of the condition. However, people with Williams syndrome have a 50% chance of passing the ...

  10. Posterior bilateral condylar displacement: its diagnosis and treatment.

    PubMed

    Weinberg, L A

    1976-10-01

    One type of condylar displacement (posterior bilateral) was discussed as an etiologic factor in TMJ dysfunction. Joint noise, tenderness on muscle palpation, and acute TMJ pain are all considered signs of TMJ dysfunction. Any joint noise is considered to be an early dysfunctional symptom because of its higher incidence in association with palpable muscle pain or acute TMJ dysfunction. Sometimes the joint noise will immediately precede acute muscle pain and/or fluctuate with the painful symptoms. The treatment of bilateral posterior condyle displacement has been described. The mandibular anterior teeth were shortened and the maxillary posterior occlusion adjusted so that the mandible could be respositioned in an anterior position without increasing the vertical dimension of occlusion. A silver-plated maxillary cast was obtained and mounted on a semiadjustable articulator (Hanau) with a face-bow. The mandibular cast was mounted in the dysfunctional (retruded) centric relation. The articulator was moved into a protrusive position by the amount of anterior correction that is needed to reposition the condyles into the middle of the fossae symmetrically on both sides. The original TMJ radiographs provide the necessary information for this clinical judgment. Acrylic resin was placed in the space created between the condylar sphere and stop on the articulator. An acrylic resin temporary repositioning prosthesis constructed on the metal cast has two functions. It provides a therapeutic trial for the anterior condylar respositioning, and it holds the mandible in the therapeutic position while TMJ radiographs confirm the corrective position of the condyles in the fossae. After a successful 6 to 8 week trial period with remission of symptoms, a gold prosthesis was constructed on the same cast in the same therapeutic position. It remains to be seen whether, after several years, the condylar suspension system changes from a dysfunctional centric relation to a new functional

  11. Landau-Kleffner syndrome is not an eponymic badge of ignorance.

    PubMed

    Hirsch, Edouard; Valenti, Maria Paola; Rudolf, Gabrielle; Seegmuller, Caroline; de Saint Martin, Anne; Maquet, Pierre; Wioland, Norma; Metz-Lutz, Marie-Noëlle; Marescaux, Christian; Arzimanoglou, Alexis

    2006-08-01

    In a 1992 editorial article, Landau expressed the hope of collective agreement in the medical community about Landau-Kleffner syndrome (LKS) in terms of diagnosis criteria, etiology, pathophysiology and rational therapy. Since then, neurophysiological and neuroimaging studies have led to the view that LKS is an acquired aphasia, secondary to an epileptic disturbance affecting a cortical area involved in verbal processing. This fits with the hypothesis of a "functional ablation" caused by epileptic activity. Under these criteria, epileptic aphasia becomes a subgroup of the continuous spike-waves syndrome in which epileptic discharges originate from the temporal cortex. Genetic predisposition for KLS could be related to hyperexcitability and synchronization of interneurons within the perisylvian cortices, which generate the spike-waves. Activation of these waves during NREM sleep, following thalamo-cortical uncoupling, might then alter the blood brain barrier and provoke an autoimmune reaction. Interneuron hyperactivity might in turn have an antiepileptic protective effect, associated with the inhibition of a specific function, and spike-waves activity over the long term might eventuate in focal atrophy. This morphological defect might explain the poor verbal outcome in some cases of LKS. From this study we recommend a multicenter control study of good design and methodology be carried out to compare the efficacies of early versus delayed (3 months) corticosteroid treatment in patients with typical LKS that is being treated by clobazam (or diazepam) monotherapy.

  12. Bilateral Spontaneous Midsubstance Patellar Tendon Rupture after Bilateral Total Knee Arthroplasty

    PubMed Central

    Rajani, Amyn; Dash, Kumar Kaushik; Mahajan, Neetin P; Kumar, Ritesh

    2016-01-01

    Introduction: Patellar tendon rupture can occur due to multiple causes ranging from inflammatory pathologies to episodes of trauma. Extensor mechanism rupture is a rare complication of total knee arthroplasty (TKA). In most of these cases, the failure occurs as avulsion of patellar tendon from tibial tuberosity. We report a rare case with bilateral mid-substance patellar tendon rupture one month after bilateral total knee arthroplasty. Case Presentation: A 69-year-old male was operated for bilateral grade 4 osteoarthritis. On day 30 post-operative, he sustained bilateral patellar tendon rupture while getting up from toilet. He had a history of multiple steroid injections in the knee, which could have affected the tendon. The other etiologies could be inherent weakness of tendon due to diabetes and old age and micro-trauma/stretch associated with sudden correction of previous deformity by TKA. The management in our case was done by primary repair along with augmentation by autologous semitendinosus graft and suture anchor. Conclusion: The operating surgeon must be aware of the possibility of patellar tendon rupture following total knee arthroplasty. This will help the surgeon in early recognition and preparedness to handle such complications, should they arise. Surgeons may consider advising caution to both patient and rehabilitation team in cases with old age, chronic diabetes mellitus, and with a history of steroid injections. PMID:27703942

  13. 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.

  14. Bilateral song production in domestic canaries.

    PubMed

    Suthers, Roderick A; Vallet, Eric; Tanvez, Aurélie; Kreutzer, Michel

    2004-09-01

    We studied the mechanism of song production in the outbred common or domestic canary (Serinus canaria). The contribution that each side of the syrinx makes to song was investigated by observing the effect of unilaterally occluding the left or right primary bronchus, followed by section of the ipsilateral branch of the tracheosyringeal nerve. In other birds with a bilaterally intact vocal system we monitored airflow through each side of the syrinx, together with subsyringeal pressure, during spontaneous song. Song production by domestic canaries is not strongly lateralized as it is in the conspecific song-bred waterslager strain. Some syllables are produced entirely on the left or right side of the syrinx, whereas others contain sequential contributions from each side. Low fundamental frequencies are produced with the left syrinx and high frequencies by the right syrinx, increasing the frequency range of domestic canary song compared to that of the waterslager strain. Midrange frequencies can be generated by either side. Syllables at repetition rates below about 25 s(-1) were accompanied by minibreaths, which were usually bilateral. Unilateral minibreaths were typically on the left side. At higher syllable repetition rates, minibreaths were replaced by a respiratory pattern of pulsatile expiration. Our data show that strong unilateral dominance in song production, present in the waterslager strain, is not a trait of the species as a whole and that the pattern of song lateralization can be altered by selective breeding for particular song characteristics.

  15. 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.

  16. Hiatus Hernia Repair with Bilateral Oesophageal Fixation.

    PubMed

    Mendis, Rajith; Cheung, Caran; 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

  17. [Synchronous bilateral breast cancer in a male].

    PubMed

    García-Mejido, José Antonio; Delgado-Jiménez, Carmen; Gutiérrez-Palomino, Laura; Sánchez-Sevilla, Miguel; Iglesias-Bravo, Eva; Caballero-Fernández, Virginia

    2013-01-01

    antecedentes: el cáncer de mama en el hombre es una enfermedad con baja incidencia, que se reduce aún más cuando es bilateral sincrónica. Existen pocas publicaciones en los últimos años. Objetivo: establecer pautas para el tratamiento de este cáncer, aunque sea infrecuente. Caso clínico: paciente masculino de 75 años de edad, con tumores en ambas mamas, que se le resecaron completamente con exéresis de ganglios palpables. El estudio histopatológico informó que se trataba de un carcinoma ductal infiltrante no especificado. Se indicó tratamiento adyuvante con tamoxifeno y radioterapia; en la actualidad está libre de enfermedad. Conclusiones: el carcinoma mamario bilateral sincrónico en el varón es una enfermedad poco frecuente. Su tratamiento principal es la cirugía, de ahí la importancia del diagnóstico temprano. En la mayoría de los casos se requiere quimioterapia y radioterapia adyuvante porque suelen diagnosticarse en un estadio avanzado.

  18. 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.

  19. 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

  20. 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

  1. The Kluver-Bucy syndrome.

    PubMed

    Góscínski, I; Kwiatkowski, S; Polak, J; Orlowiejska, M; Partyk, A

    1997-09-01

    Evolution of psychological disorders following head injury including memory disorders and other cognitive ones are common. The best known are psychiatric disturbances of various kind after lesions of frontal lobes. Cognitive, behavioural and emotional disorders are not usually seen in patients with bilateral temporal lesions. In our Department of Neurotraumatology we observed 4 patients with post-traumatic lesions localized bitemporally. They developed Kluver-Bucy syndrome--rarity in human pathology--with combination of three or more the following syndromes: increased oral activity, hypersexuality, hypermetamorphosis, memory disorders, placidity, loss of people recognition, bulimia. Several symptoms responded dramatically to carbamazepine. We conclude that it may be a useful agent in treatment of this unusual syndrome.

  2. The bilateral advantage for famous faces: interhemispheric communication or competition?

    PubMed

    Baird, Lyndsay M; Burton, A Mike

    2008-04-01

    The bilateral advantage for the perception of famous faces was investigated using a redundant target procedure. In experiment 1 we compared simultaneous presentation of stimuli (a) bilaterally and (b) one above the other in the central field. Results showed a redundancy advantage, but only when faces were presented bilaterally. This result lends support to the notion of interhemispheric communication using cross-hemisphere representations. Experiment 2 examined the nature of such communication by comparing bilateral presentation of identical face images, with bilateral presentation of different images of the same person. When asked to make a familiar/unfamiliar face judgement, participants showed evidence for a redundancy advantage under both bilateral conditions. This suggests that the nature of the information shared in interhemispheric communication is abstract, rather than being tied to superficial stimulus properties.

  3. [Moebius syndrome: therapeutic proposals from 2 cases].

    PubMed

    Braye, F; Souchere, B; Franc, C; Freidel, M

    1996-12-01

    Moebius syndrome is a congenital bilateral palsy of the sixth and seventh cranial nerves. It results a total absence of facial expression and a severe strabismus. Social life is greatly disturbed. Other anomalies may be associated, especially other cranial palsies and Poland syndrome. The etiology of this syndrome isn't clearly established. Stem necrosis secondary to a vascular deficiency is often admitted. We report two observations. We emphasize the importance of a complete maxillo-facial treatment including maxillo-mandibular anomaly. Both patient underwent orthognathic surgery. The first one for class II and the second for class III anomaly. One patient underwent a facial reanimation by temporal muscle transfer. Orthognathic surgery must be realized prior to facial reanimation. A correction of the strabismus is possible. Moebius syndrome is a rare (200 observations) but very severe malformation. Maxillofacial surgery is able to improve the morphological and relational aspect of Moebius syndrome.

  4. A young woman with bilateral breast cancer: identifying a genetic cause and implications for management.

    PubMed

    de Bruin, Monique A; Ford, James M; Kurian, Allison W

    2013-05-01

    Breast cancer is a common manifestation of an underlying genetic susceptibility to cancer, and 5% to 10% of all breast cancers are associated with a germline mutation in a known risk allele. Detection of mutations has implications for targeted screening and prevention strategies for probands, and for genetic counseling and testing of their family members. This report presents a case involving a 35-year-old woman with no family history of breast or ovarian cancer who presented with a palpable right breast lump. Imaging revealed multiple bilateral breast masses and right axillary adenopathy, and core needle biopsies showed invasive ductal carcinoma in both the right and left breast. This report discusses the appropriate genetics evaluation for a patient with bilateral breast cancer at a young age, including testing for mutations in BRCA1 and BRCA2, followed, if negative, by consideration of testing for mutations in TP53 (Li-Fraumeni syndrome). Given the specialized counseling and testing needs of patients with Li-Fraumeni syndrome, and the implications for targeted screening strategies if a mutation is found, referral to a cancer genetics expert is strongly recommended.

  5. 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

  6. Weismann-Netter-Stuhl syndrome: a family report.

    PubMed

    Alp, Hayrullah; Atabek, Mehmet Emre; Pirgon, Özgür

    2009-01-01

    Weismann-Netter-Stuhl (WNS) syndrome is a rare skeletal anomaly that affects the diaphyseal part of both the tibiae and fibulae with posterior cortical thickening and anteroposterior bowing. This anomaly is usually bilateral and symmetrical. The patients are generally of short stature. In some cases, a family history suggesting genetic transmission of a mutation with an unknown locus has been reported. In this paper we present an infant with WNS syndrome with bilateral involvement of the femur. Similar clinical findings were defined in three other family members.

  7. 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

  8. 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

  9. 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

  10. Catel-Manzke palatodigital syndrome in a second trimester female foetus with nuchal oedema, costovertebral anomalies and radial ray defect.

    PubMed

    Petit, P; Moerman, P; Legius, E; Fryns, J P

    1994-01-01

    We present a female fetus with combination of Pierre Robin anomaly and nuchal oedema, bilateral radial defects, multiple hand malformations including bilateral hyperphalangy, brachymesophalangy, costovertebral abnormalities, and complex cardiac malformation. The present findings constitute a true MCA syndrome with uncertain pattern of inheritance. PMID:7888142

  11. Interocular symmetry analysis of bilateral eyes.

    PubMed

    Li, Yi; Bao, Fang Jun

    2014-05-01

    The purpose of this study was to evaluate the interocular symmetry of several biometric parameters between both eyes. The symmetry between the right and left eye of 397 subjects in 14 biometric parameters, spherical equivalent of refractive error (SE), Jackson crossed cylinder power of refractive error astigmatism with axes at 90° and 180° (RJ0) and at 45° and 135° (RJ45), best-corrected visual acuity (BCVA), average corneal curvature (CC), Jackson crossed cylinder power of corneal astigmatism (CJ0 and CJ45), corneal asphericity coefficient (Q), intraocular pressure (IOP), central corneal thickness (CCT), axial length (AL), anterior chamber depth (ACD), lens thickness (LT) and vitreous chamber depth (VCD), was assessed by comparative data analysis. Aside from RJ0 (p = 0.00), RJ45 (p = 0.02) and Q (p = 0.00), the overall interocular differences of other biometric parameters between fellow eyes were not significant (p > 0.05). The interocular correlation and Bland-Altman plots showed a good agreement between fellow eyes in 14 biometric parameters. Correlations between interocular differences in SE and that in RJ0 (p = 0.03), CC (p = 0.00), AL (p = 0.00) and VCD (p = 0.00) were statistically significant. There were similar strong linear relationships between refractive error astigmatism vectors and corneal astigmatism vectors in bilateral eyes. There were negative correlations of RJ45 and CJ45 between bilateral eyes. A potentially clinically important interocular symmetry in SE, BCVA, CC, CJ0, CJ45, IOP, CCT, AL, ACD, LT and VCD is found in this research, while the differences of RJ0, RJ45 and Q between left and right eyes seem a bit large. The negative interocular relationships of RJ45 and CJ45 demonstrate moderate mirror symmetry exists among fellow eyes. High interocular symmetry in bilateral eyes may be helpful in intraocular lens power calculation, intraocular pressure evaluation, post-operative visual acuity and refraction

  12. [Charles Bonnet syndrome: a case presentation].

    PubMed

    Cumurcu, Tongabay; Elbozan Cumurcu, Birgül; Cam Celikel, Feryal

    2005-01-01

    Charles Bonnet syndrome comprises the triad of visual hallucinations, visual sensory deprivation, and preserved cognitive status. This paper discusses a case diagnosed as Charles Bonnet syndrome, involving visual hallucinations secondary to bilateral primary optic atrophy. An 80-year-old female with normal cognitive functions in the presence of primary optic atrophy and visual hallucinations was diagnosed with Charles Bonnet syndrome. The patient, having had poor vision since childhood, had lost it totally in the last year. Her vision had not improved following cataract operations in both eyes 6 months previously. Her vision was at the level of hand movements. In biomicroscopic examination, bilateral pseudoaphakia was found. Since fundus examination showed bilateral primary optic atrophy in the presence of visual hallucinations, a psychiatric consultation was requested. In her psychiatric examination, she had had hallucinations for the last two years, first elementary and then complex in character. Her cognitive functions were normal with no pathology in her neurologic examination. Routine investigations and neuroradiologic examinations were normal. She had no past history of any personal or familial psychiatric or systemic physical disorder. She was given olanzapine 5 mg daily and was followed up. This syndrome, defined as visual hallucinations in the presence of preserved cognitive functions and deprived vision, requires further research.

  13. 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

  14. 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

  15. 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

  16. Bilateral adrenal hemorrhage in polycythemia vera

    PubMed Central

    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

  17. A very rare bilateral adrenal tumor.

    PubMed

    Toniato, Antonio; Boschin, Isabella Merante; Pelizzo, Maria Rosa

    2014-04-01

    We report a case of very rare adrenal tumor. A 54-year-old patient was classified as affected by bilateral adrenal incidentaloma that surprisingly, on histology resulted solitary fibrous tumors. Solitary fibrous tumor (SFT) is an uncommon mesenchymal neoplasm. Only five cases of localization of SFT in adrenal gland are reported in the literature, while the frequency of retroperitoneum localization is more frequent, about 30 cases. Immunohistochemically, SFT can be positive for CD34 antigen, vimentin, CD99, and bcl-2 and usually negative for cytokeratins, chromogranin A, NSE, neurofilaments, synoptophysin, and S-100. Surgical excision remains the main treatment in fact the recurrence is locoregional and correlated with positive margins due to incomplete excision, while distant metastases are correlated with atypical or malignant features.

  18. Trismus Due to Bilateral Coronoid Hyperplasia

    PubMed Central

    Choi, Moon Gi; Kim, Dong Hyuck; Ki, Eun Jung; Cheon, Hae Myung

    2014-01-01

    Bilateral coronoid hyperplasia causes painless progressive trismus, resulting from coronoid process impingement on the posterior aspect of the zygomatic bone. The etiology of coronoid hyperplasia is unclear, with various theories proposed. An endocrine stimulus, increased temporalis activity, trauma, genetic inheritance and familial occurrence have all been proposed, but no substantive evidence exists to support any of these hypotheses. Multiplanar reformatting of axial scans and 3-dimensional reconstruction permit precise reproduction of the shape and size of the coronoid and malar structures, and relationships of all structures of the temporal and infratemporal fossae. This case shows remarkably increased mouth opening by coronoidectomy in a patient who complained of trismus due to hyperplasia of coronoid process. PMID:27489829

  19. Cherubism With Bilateral Mandible and Maxilla Involvement

    PubMed Central

    Yu, Zhaoyang; Zhai, Miao; Gan, Wei; Zhang, Hong; Zhou, Yuxia; Wen, Haixia

    2015-01-01

    Abstract Cherubism is a rare, nonneoplastic, self-limiting fibro-osseous that occurs in children. Affected children usually appear normal at birth. Lesions are characterized by the replacement of bone with fibrovascular tissue containing many multinucleated giant cells. Most studies have reported cherubism to be familial and with bilateral involvement of the mandibles. The authors describe a nonfamilial case of cherubism, involving both the mandible and the maxilla, in a 4-year-old female child with slowly enlarging, painless, symmetrical swelling of both cheeks. Cherubism is a rare disease that is usually limited to the mandible, but the maxilla may be involved. Computed tomography scan and biopsy are helpful for early diagnosis. PMID:26656340

  20. 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

  1. 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

  2. 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

  3. Framing effect following bilateral amygdala lesion.

    PubMed

    Talmi, Deborah; Hurlemann, René; Patin, Alexandra; Dolan, Raymond J

    2010-05-01

    A paradigmatic example of an emotional bias in decision making is the framing effect, where the manner in which a choice is posed--as a potential loss or a potential gain--systematically biases an ensuing decision. Two fMRI studies have shown that the activation in the amygdala is modulated by the framing effect. Here, contrary to an expectation based on these studies, we show that two patients with Urbach-Wiethe (UW) disease, a rare condition associated with congenital, complete bilateral amygdala degeneration, exhibit an intact framing effect. However, choice preference in these patients did show a qualitatively distinct pattern compared to controls evident in an increased propensity to gamble, indicating that loss of amygdala function does exert an overall influence on risk-taking. These findings suggest either that amygdala does contribute to decision making but does not play a causal role in framing, or that UW is not a pure lesion model of amygdala function.

  4. 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.

  5. 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

  6. 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

  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. 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

  9. [Bilateral infarction of the caudate nuclei].

    PubMed

    Mrabet, A; Mrad-Ben Hammouda, I; Abroug, Z; Smiri, W; Haddad, A

    1994-01-01

    We report the case of a 57-year-old right-handed woman, with a history of hypertension, who, in February 1990, suddenly developed behavioral and cognitive abnormalities. Prior to the onset of her illness she had been normal. On examination, neuropsychological testing (Wechsler Mental Test, Wechsler Adult Intelligence Scale Revised, Knox Cube Test) elicited attention abnormalities, decreased recent memory, apathy, reduced spontaneity and initiative and left hemiparesia. CT scan showed small low density areas in the head of both caudate nuclei and right internal capsule, indicating infarction. Two years later, the deficit had partially resolved. Apathy persisted; psychometry showed an IQ of 57. Bilateral damage to the head of the caudate nuclei disrupt cortical-subcortical connections. The caudate nucleus is an essential component of basal ganglia-thalamo-cortical circuitry and its contribution to cognitive functions and behavior appears to be important. PMID:7801044

  10. 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

  11. Renal function after bilateral extracorporeal shockwave lithotripsy.

    PubMed

    Cass, A S

    1994-12-01

    We studied renal function an average of 44 months after simultaneous bilateral renal SWL in 56 patients. No cases of clinically apparent acute renal failure occurred in the early postoperative period. The glomerular filtration rate (GFR) was calculated using an empiric formula having a significant correlation with measured creatinine clearance, and a change of 20% or greater was considered a clinically significant deterioration in renal function. Of the seven patients with a preoperative serum creatinine concentration of > 1.5 mg/dL, six had an average increase of 35% in postoperative GFR attributable to relief of obstruction, while one had a 30% reduction in GFR. Among 49 patients with a preoperative serum creatinine concentration of 1.5 mg/dL or less, there was an increase in postoperative GFR in 22 patients (45%), no change in 3 (6%), and a decrease in 24 (49%), who had a higher number of multiple renal stones (p < 0.05) and of repeat SWL (p = 0.08). Nine of them (18%) had a clinically significant decrease in GFR of > 20%. A review of the literature showed a long-term reduction of function in the individual human kidney after SWL in some cases of a solitary kidney and in some cases with an untreated contralateral kidney. Because there is no evidence that an untreated contralateral kidney aids the long-term recovery of the function of a treated kidney in all cases, simultaneous or separate bilateral renal SWL would not influence this long-term reduction in renal function, which was felt to occur with multiple renal stones and repeat SWL.

  12. [Bilateral ovarian Burkitt's lymphoma. A case presentation].

    PubMed

    Briseño-Hernández, Andrés Alejandro; Quezada-López, Deissy Roxana; Castañeda-Chávez, Agar; Dassaejv Macías-Amezcua, Michel; Pintor-Belmontes, Julio Cesar

    2014-01-01

    Antecedentes: el linfoma de Burkitt es una forma agresiva de los linfomas no Hodgkin de células B que ocurre con mayor frecuencia en niños y adultos jóvenes; el linfoma de ovario puede aparecer como lesión primaria o, más comúnmente, como una metástasis. Las lesiones primarias de ovario son manifestaciones raras que corresponden a 0.5% de los linfomas no Hodgkin y 1.5% de los tumores de ovario. Caso clínico: paciente femenina de 31 años de edad, con debilidad generalizada, incapacidad para la marcha, disnea, hiporexia, fiebre, diaforesis, pérdida de 20 kg de peso, abdomen plano, con dolor abdominal; Ca125 610 U/mL. La tomografía computada abdominal mostró un gran tumor de aspecto sólido que afectaba a la cavidad pélvica derecha. Se extirparon los tumores ováricos bilaterales. Desde el punto de vista microscópico ambas lesiones muestran una imagen en "cielo estrellado" compuesta por un patrón de infiltrado monótono de linfocitos mezclada con macrófagos de citoplasma amplio y claro, abundantes mitosis atípicas, zonas de necrosis y hemorragia. La inmunohistoquímica reveló positividad para CDI0 y CD20, negativo con CD3, índice de proliferación Ki67 alto. Se diagnosticó linfoma tipo Burkitt bilateral de ovario. Conclusiones: el linfoma de Burkitt de ovario bilateral es poco frecuente, con variabilidad de presentación; el dolor abdominal y los tumores abdominales son lo más frecuente. El pronóstico a corto plazo de las pacientes es malo, por lo que es necesario conocer esta afección para poder establecer el diagnóstico temprano.

  13. Conscious attention, meditation, and bilateral information transfer.

    PubMed

    Bob, Petr; Zimmerman, Elizabeth M; Hamilton, Elizabeth A; Sheftel, Jenna G; Bajo, Stephanie D; Raboch, Jiri; Golla, Megan; Konopka, Lukasz M

    2013-01-01

    Recent findings indicate that conscious attention is related to large-scale information integration of various brain regions, including both hemispheres, that enables integration of parallel distributed modalities of processed information. There is also evidence that the level of information transference related to integration or splitting among brain regions, and between hemispheres, establishes a certain level of efficiency of the information processing. The level of information transference also may have modulatory influences on attentional capacity that are closely linked to the emotional arousal and autonomic response related to a stimulus. These findings suggest a hypothesis that changes in conscious attention, specifically during meditation could be reflected in the autonomic activity as the left-right information transference calculated from bilateral electrodermal activity (EDA). With the aim to compare conscious attention during meditation with other attentional states (resting state, Stroop task, and memory task), we performed bilateral EDA measurement in 7 healthy persons during resting state, Stroop task, neurofeedback memory test, and meditation. The results indicate that the information transference (ie, transinformation) is able to distinguish those attentional states, and that the highest level of the transinformation has been found during attentional processing related to meditation, indicating higher level of connectivity between left and right sides. Calculations other than pointwise transinformation (PTI) performed on EDA records, such as mean skin conductance level or laterality index, were not able to distinguish attentional states. The results suggest that PTI may present an interesting method useful for the assessment of information flow, related to neural functioning, that in the case of meditation may reflect typical integrative changes in the autonomic nervous system related to brain functions and focused attentional processing.

  14. [Irreversible bilateral amaurosis secondary to the course of ethmofrontal mucocele].

    PubMed

    Vallés, H; Palomar, A; Blanc, J; Sevil, J; Fumanal, L

    1990-01-01

    We present a carrier of a mucocele ethmofrontal patient's case that relapsed after surgical treatments and that, subsequently, it caused an intense bilateral and amaurosis. This was attributed to the bilateral atrophy of the optician nerve secondary to the evolution of the own mucocele. It discussed the frequency of this kind of complications in the bibliographical revision made.

  15. 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. PMID:27413273

  16. 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.

  17. Bilateral osteomas and exostoses of the internal auditory canal.

    PubMed

    Schutt, Christopher A; Guo, Jennifer N; Bagwell, Kenneth A; Bulsara, Ketan R; Malhotra, Ajay; Michaelides, Elias

    2015-01-01

    Osteomas and exostoses are benign tumors of the bone that occur in the head and neck region but are rarely found within the internal auditory canal (IAC). In this report, we review the literature on bony lesions of the IAC and present two cases: one case of bilateral compressive osteomas and one case of bilateral compressive exostoses of the IAC.

  18. Simultaneous Bilateral Transient Osteoporosis of the Hip without Pregnancy.

    PubMed

    Okada, Yasuaki; Tsukada, Sachiyuki; Saito, Masayoshi; Tasaki, Atsushi

    2016-01-01

    Transient osteoporosis of the hip (TOH) is a rare disorder characterized by acute severe coxalgia and temporary osteopenia in the proximal femur. Although most cases were unilateral or staged bilateral TOH, some authors reported that the pregnant patients simultaneously had TOH in their bilateral hips. However, there has been no report of simultaneous bilateral TOH in the patient without pregnancy. A 25-year-old Japanese woman without pregnancy had acute simultaneous bilateral hip pain. Plain X-ray of the bilateral hips did not show a periarticular osteopenia. However, magnetic resonance image obtained one week after the onset demonstrated increased T2-weighted signal intensity and decreased T1-weighted signal intensity in the bilateral femoral heads. She was treated conservatively, and follow-up magnetic resonance image at seven weeks after the onset returned to normal bone marrow signal intensity. Her bilateral coxalgia subsided gradually. At one year after the onset, she had no sign of symptomatic flair. Our experience with this case indicates that recognizing the possibility of simultaneous bilateral TOH is important unless the patient is pregnant, and magnetic resonance image is predictable test to make a diagnosis of TOH, even in the absence of abnormal finding on plain X-ray. PMID:27648329

  19. Bilateral Symmetrical Congenital Giant Becker's Nevus: A Rare Presentation

    PubMed Central

    Rao, Angoori Gnaneshwar

    2015-01-01

    Becker's nevus is a focal epidermal hypermelanotic disorder. It morphologically presents as unilateral, hyperpigmented, hypertrichotic patch on upper trunk, proximal upper extremities and arms. However, Becker's nevus presenting as bilateral, symmetrical patches is rare. Herein, we report a rare case of giant Becker's nevus with bilateral symmetrical presentation in an adult male. PMID:26538733

  20. 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...

  1. Leg stiffness: comparison between unilateral and bilateral hopping tasks.

    PubMed

    Brauner, Torsten; Sterzing, Thorsten; Wulf, Mathias; Horstmann, Thomas

    2014-02-01

    Leg stiffness is a predictor of athletic performance and injury and typically evaluated during bilateral hopping. The contribution of each limb to bilateral leg stiffness, however, is not well understood. This study investigated leg stiffness during unilateral and bilateral hopping to address the following research questions: (1) does the magnitude and variability of leg stiffness differ between dominant and non-dominant legs? (2) Does unilateral leg stiffness differ from bilateral leg stiffness? and (3) Is bilateral leg stiffness determined by unilateral leg stiffness? Thirty-two physically active males performed repeated hopping tests on a force platform for each of the three conditions: bilateral hopping, unilateral hopping on the dominant leg, and unilateral hopping on the non-dominant leg. Leg stiffness was estimated as the ratio of the peak vertical force and the maximum displacement using a simple 1-D mass-spring model. Neither the magnitude nor variability of leg stiffness differed between dominant and non-dominant limbs. Unilateral leg stiffness was 24% lower than bilateral stiffness and showed less variability between consecutive hops and subjects. Unilateral leg stiffness explained 76% of the variance in bilateral leg stiffness. We conclude that leg stiffness estimates during unilateral hopping are preferable for intervention studies because of their low variability. PMID:24290613

  2. Traumatic bilateral basal ganglia hematoma: A report of two cases

    PubMed Central

    Bhargava, Pranshu; Grewal, Sarvpreet Singh; Gupta, Bharat; Jain, Vikas; Sobti, Harman

    2012-01-01

    Traumatic Basal ganglia hemorrhage is relatively uncommon. Bilateral basal ganglia hematoma after trauma is extremely rare and is limited to case reports. We report two cases of traumatic bilateral basal ganglia hemorrhage, and review the literature in brief. Both cases were managed conservatively. PMID:23293672

  3. [Bilateral anterior uveiopapillitis, suspicious of Lyme disease--case report].

    PubMed

    Nicula, Cristina; Nicula, D; Rusu, Ioana; Popescu, Raluca

    2013-01-01

    We present the case of a patient which associated bilateral anterior uveitis manifestations with those of bilateral anterior inflammatory optic neuropathy. We followed the evolution of the case under treatment and we discussed the differential diagnosis and the association of the two ocular pathologies.

  4. Simultaneous Bilateral Transient Osteoporosis of the Hip without Pregnancy

    PubMed Central

    Saito, Masayoshi; Tasaki, Atsushi

    2016-01-01

    Transient osteoporosis of the hip (TOH) is a rare disorder characterized by acute severe coxalgia and temporary osteopenia in the proximal femur. Although most cases were unilateral or staged bilateral TOH, some authors reported that the pregnant patients simultaneously had TOH in their bilateral hips. However, there has been no report of simultaneous bilateral TOH in the patient without pregnancy. A 25-year-old Japanese woman without pregnancy had acute simultaneous bilateral hip pain. Plain X-ray of the bilateral hips did not show a periarticular osteopenia. However, magnetic resonance image obtained one week after the onset demonstrated increased T2-weighted signal intensity and decreased T1-weighted signal intensity in the bilateral femoral heads. She was treated conservatively, and follow-up magnetic resonance image at seven weeks after the onset returned to normal bone marrow signal intensity. Her bilateral coxalgia subsided gradually. At one year after the onset, she had no sign of symptomatic flair. Our experience with this case indicates that recognizing the possibility of simultaneous bilateral TOH is important unless the patient is pregnant, and magnetic resonance image is predictable test to make a diagnosis of TOH, even in the absence of abnormal finding on plain X-ray. PMID:27648329

  5. 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

  6. Bilateral congenital pseudoarthrosis of the clavicles in a newborn

    PubMed Central

    Kalagiri, Ram R.; Hemingway, Martha; Beeram, Madhava R.

    2016-01-01

    Bilateral congenital pseudoarthrosis of the clavicles is extremely rare. We report a case of this entity presenting in the neonatal period. We highlight the importance of the differential diagnosis when clavicular fracture shows no evidence of healing or occurs bilaterally. PMID:27695169

  7. 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…

  8. Simultaneous Bilateral Transient Osteoporosis of the Hip without Pregnancy

    PubMed Central

    Saito, Masayoshi; Tasaki, Atsushi

    2016-01-01

    Transient osteoporosis of the hip (TOH) is a rare disorder characterized by acute severe coxalgia and temporary osteopenia in the proximal femur. Although most cases were unilateral or staged bilateral TOH, some authors reported that the pregnant patients simultaneously had TOH in their bilateral hips. However, there has been no report of simultaneous bilateral TOH in the patient without pregnancy. A 25-year-old Japanese woman without pregnancy had acute simultaneous bilateral hip pain. Plain X-ray of the bilateral hips did not show a periarticular osteopenia. However, magnetic resonance image obtained one week after the onset demonstrated increased T2-weighted signal intensity and decreased T1-weighted signal intensity in the bilateral femoral heads. She was treated conservatively, and follow-up magnetic resonance image at seven weeks after the onset returned to normal bone marrow signal intensity. Her bilateral coxalgia subsided gradually. At one year after the onset, she had no sign of symptomatic flair. Our experience with this case indicates that recognizing the possibility of simultaneous bilateral TOH is important unless the patient is pregnant, and magnetic resonance image is predictable test to make a diagnosis of TOH, even in the absence of abnormal finding on plain X-ray.

  9. 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.

  10. 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

  11. 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

  12. Rare cause of paraparesis: bilateral obturator neuropathy after hysterosalpingectomy.

    PubMed

    López-Blanco, Roberto; Mejía-Jiménez, Inmaculada; de Fuenmayor-Fernández de la Hoz, Carlos Pablo; Ruiz-Morales, Juan

    2015-01-01

    Bilateral obturator nerve injury during pelvic surgery is an infrequent cause of lower limb paraparesis. We report the case of a 45-year-old woman with a large uterine leiomyoma who underwent simple total hysterectomy and bilateral salpingectomy. At 24 h after the surgery, the patient noticed loss of muscle strength when adducting both legs. She had no problem with other movements and no sensory or sphincter abnormalities. Neurological examination confirmed that there was loss of strength only in the adductor muscles, with preserved sensory function and reflexes, suggesting bilateral obturator nerve involvement. Pelvic MRI showed a small postsurgical haematoma in the Douglas recess, but far from the obturator nerves. 2 weeks later, electromyography showed positive sharp waves and low motor unit recruitment in the adductor magnus muscles, confirming acute, bilateral obturator nerve neuropathy. The few cases of bilateral obturator neuropathy that have been reported were mostly related to abdominopelvic interventions. PMID:26689250

  13. Bilateral Clavicle Fractures: A Report of Three Cases.

    PubMed

    Lakhotia, Devendra; Khatri, Kavin; Sharma, Vijay; Farooque, Kamran; Sharma, Swati

    2016-06-01

    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

  14. 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

  15. Misdiagnosis of bilateral tubal pregnancy: a case report

    PubMed Central

    2014-01-01

    Introduction The incidence of bilateral tubal pregnancy is rising due to the increase of pelvic inflammatory disease and assisted reproductive techniques. Because the clinical manifestations of bilateral tubal pregnancy are not specific, we often ignore inspection of the other fallopian tube when focusing on the lesions, which may cause misdiagnosis. Case presentation A 33-year-old Chinese woman presented with vaginal bleeding after menopause and with an abnormality found by transvaginal ultrasound scan for which she underwent laparoscopy and salpingectomy. Unfortunately, she had to undergo a repetitive laparoscopic salpingotomy for the other tubal pregnancy due to misdiagnosis of her bilateral tubal pregnancy. Conclusions The incidence of unusual presentations of ectopic pregnancies has risen. Surgeons should always keep in mind the possibility of bilateral tubal pregnancy. An attentive examination of the pelvis, especially the two fallopian tubes, is necessary to avoid missing bilateral tubal pregnancy. PMID:25312677

  16. Sturge–Weber syndrome – A case report

    PubMed Central

    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

  17. The Syndrome of Familial Hypoparathyroidism, Sensorineural Deafness and Renal Dysplasia.

    PubMed

    Meena, Ratti Lal; Maloo, Sudheer Kumar; Samar, Neera; Ruhela, Asim; Saini, Subhash

    2015-06-01

    The syndrome of familial hypoparathyroidism, sensorineural deafness, and renal dysplasia (HDR syndrome) is inherited as an autosomal dominant trait, caused by haploinsufficiency of the GATA3 gene in chromosome 10p. Although first described years ago, but the disease is considered to be very rare. Patients usually present with hypocalcemia, tetany, or afebrile convulsions at any age. Hearing loss is usually bilateral, range from mild to profound impairment. Renal disease includes dysplasia, hypoplasia or aplasia.

  18. Anti-aquaporin-4 antibody-positive dorsal midbrain syndrome.

    PubMed

    Lee, Juyoun; Jeong, Seong-Hae; Park, Sang Min; Sohn, Eun Hee; Lee, Ae Young; Kim, Jae-Moon; Jo, Hyun-Jin; Lee, Yeon-Hee; Kim, Ji-Soo

    2015-04-01

    Neuromyelitis optica spectrum disorders (NMOSD) can cause various ocular motor disorders in addition to optic neuritis. Ocular motor findings associated with NMOSD include spontaneous vertical and gaze-evoked nystagmus, wall-eyed bilateral internuclear ophthalmoplegia, and trochlear nerve palsy. The association between dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity has not been reported. Here, we report a patient displaying typical dorsal midbrain syndrome and anti-aquaporin-4 antibody seropositivity.

  19. 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. PMID:27625493

  20. Carisoprodol withdrawal syndrome resembling neuroleptic malignant syndrome: Diagnostic dilemma.

    PubMed

    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. PMID:27625493

  1. Exercise-induced compartment syndrome: case report.

    PubMed

    Klodell, C T; Pokorny, R; Carrillo, E H; Heniford, B T

    1996-06-01

    Exercise-induced rhabdomyolysis is a frequent event occurring after severe forms of exercise. This is usually a short-lived, uncomplicated phenomenon that is seldom of any clinical significance. The rare progression of this muscle injury to compartment syndrome is, however, a limb- and life-threatening condition that typically presents in the anterior compartment of the lower leg. A case is reported of a young man who participated in physical activity well beyond his normal level of exertion and subsequently developed bilateral lower extremity compartment syndrome requiring surgical decompression. To our knowledge, this is the only description of this complication occurring in a multicompartment, bilateral distribution. The combination of the rarity and morbidity of this condition, as well as the multitude of very common benign injuries that present in the same manner as the problem discussed, make this insult especially dangerous.

  2. Bilateral, atraumatic, proximal tibiofibular joint instability.

    PubMed

    Morrison, Troy D; Shaer, James A; Little, Jill E

    2011-01-01

    Dislocation of the tibiofibular joint is rare and usually results from a traumatic event. Only 1 case of atraumatic proximal tibiofibular joint instability in a 14-year-old girl has been reported in the literature, however this condition might occur more frequently than once thought. A wide range of treatment options exist for tibiofibular dislocations. Currently, the first choice is a conservative approach, and when this fails, surgical means such as resection of the fibula head, arthrodesis, and reconstruction are considered. However, no consensus exists on the most effective treatment. This article reports a unique case of bilateral, atraumatic, proximal tibia and fibular joint instability involving a 30-year-old man with a 20-year history of pain and laxity in the right knee. The patient had no trauma to his knees; he reported 2 immediate family members with similar complaints, which suggests that this case is likely congenital. After conservative approaches proved to be ineffective, the patient underwent capsular reconstruction using free autologous gracilis tendon. At 6-month postoperative follow-up, the patient was pain free with no locking and instability. He then underwent surgery on the left knee. At 1-year follow-up after the second surgery, the patient had no symptoms or restrictions in mobility. We provide an alternative surgical approach to arthrodesis and resection for the treatment of chronic proximal tibiofibular instability. In the treatment of chronic tibiofibular instability, we believe that reconstruction of the tibiofibular joint is a safe and effective choice.

  3. Unilateral and bilateral nasal resistances: a supplement.

    PubMed

    Naito, K; Cole, P; Humphrey, D

    1990-06-01

    Three hundred and thirty-four measurements of bilateral and unilateral nasal resistance (at delta P 1.0 cm H2O and by time averaging) in 233 adults were carried out by posterior rhinomanometry with a head-out body plethysmograph. Total nasal resistances, calculated by the equation of Ohm's Law for parallel resistors from measured unilateral resistances, were compared with measured total nasal resistances. The time averaged total nasal resistances calculated by use of Ohm's Law for parallel resistors were closer to direct measurements than resistances at delta P 1.0 cm H2O calculated from the same equation. We attempted to fit calculated total nasal resistance with direct measurements by modification of the equation of Ohm's Law for parallel resistors to T = 0.96[R x L/(R + L)]0.92 in the time averaged nasal resistance and T = 1.07[R x L/(R + L)]0.77 in resistance at delta P 1.0 cm H2O (T: total nasal resistance, R: nasal resistance on the right side, L: nasal resistance on the left side). Calculated total nasal resistances from the above equations agreed closely with direct measurements.

  4. 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.

  5. 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.

  6. 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

  7. 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

  8. Unilateral hyperhydrosis in Pourfour du Petit syndrome.

    PubMed

    Kara, Murat; Dikmen, Erkan; Akarsu, Cengiz; Birol, Ahu

    2004-08-01

    Upper limp hyperhydrosis is an idiopathic disease with bilateral involvement. However, Pourfour du Petit syndrome, the opposite of Horner syndrome, may result in unilateral upper limb hyperhydrosis. It occurs following hyperactivity of the sympathetic cervical chain as a consequence of irritation secondary to trauma. We report herein two cases with Pourfour du Petit syndrome showing unilateral upper limb hyperhydrosis. The patients presented with right-sided mydriasis and ipsilateral hemifacial hyperhydrosis. The onset of disease was followed by a trauma in both patients. They underwent upper thoracic sympathectomy with favorable outcome. A history of an antecedent trauma in patients with unilateral upper limb hyperhydrosis and anisocoria may imply a possible diagnosis of Pourfour du Petit syndrome. PMID:15296919

  9. Anophthalmia-Waardenburg syndrome with expanding phenotype: does neural crest play a role?

    PubMed

    Galasso, Cinzia; Bombardieri, Roberta; Cerminara, Caterina; Stranci, Giuseppe; Curatolo, Paolo

    2007-11-01

    We describe a child with bilateral anophthalmia, limb anomalies, skin lesions, cerebral malformations, epilepsy, and mental retardation. This patient, according to eponymous classification, should fit into the Anophthalmia-Waardenburg syndrome, although he also presents cutaneous and cerebral manifestations never reported in this syndrome until now. These clinical findings could be explained by the new classification of brain malformations, which takes into account the role of neural crest in Waardenburg syndrome.

  10. 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 ...

  11. LEOPARD syndrome

    MedlinePlus

    LEOPARD syndrome is a very rare inherited disorder in which there are problems with the skin, face, ... LEOPARD syndrome is inherited as an autosomal dominant trait. This means the person only needs the abnormal ...

  12. 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 ...

  13. 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 ...

  14. Metabolic Syndrome

    MedlinePlus

    Metabolic syndrome is a group of conditions that put you at risk for heart disease and diabetes. These ... doctors agree on the definition or cause of metabolic syndrome. The cause might be insulin resistance. Insulin is ...

  15. 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 ...

  16. 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. ...

  17. Piriformis syndrome

    MedlinePlus

    Pseudosciatica; Wallet sciatica; Hip socket neuropathy; Pelvic outlet syndrome; Low back pain - piriformis ... Sciatica is the main symptom of piriformis syndrome. Other symptoms include: Tenderness or a dull ache in ...

  18. 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@ ...

  19. 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…

  20. Exophthalmos: A Forgotten Clinical Sign of Cushing's Syndrome.

    PubMed

    Giugni, Aldo Schenone; Mani, Shylaja; Kannan, Subramanian; Hatipoglu, Betul

    2013-01-01

    Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia. PMID:23555062

  1. Exophthalmos: A Forgotten Clinical Sign of Cushing's Syndrome.

    PubMed

    Giugni, Aldo Schenone; Mani, Shylaja; Kannan, Subramanian; Hatipoglu, Betul

    2013-01-01

    Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia.

  2. Exophthalmos: A Forgotten Clinical Sign of Cushing's Syndrome

    PubMed Central

    Giugni, Aldo Schenone; Mani, Shylaja; Kannan, Subramanian

    2013-01-01

    Exophthalmos is typically associated with Graves' ophthalmopathy. Although originally described by Harvey Cushing, exophthalmos is an underappreciated sign of Cushing's syndrome. We present a case of a 38-year-old female who presented with severe bilateral proptosis and was subsequently diagnosed with Cushings disease. We discuss the possible mechanisms causing exophthalmos in patients with either endogenous or exogenous hypercortisolemia. PMID:23555062

  3. 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

  4. Spheniodal mucocele causing bilateral optic neuropathy and ophthalmoplegia.

    PubMed

    Selvakumar, Ambika; Mahalaxmi, Balasubramanyam; Ananth, V; Gautam, Cugati

    2014-04-01

    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.

  5. Bilateral impacted femoral neck fracture in a renal disease patient.

    PubMed

    Devkota, Pramod; Ahmad, Shiraz

    2013-09-01

    Spontaneous bilateral femoral neck facture in a renal disease patient is not common. We report a case of 47-year-old female patient with chronic renal failure and on regular hemodialysis for the past 5 years who sustained bilateral impacted femoral neck fracture without history of trauma and injury and refused any surgical intervention. The patient was mobilised on wheel chair one year after the fractures. The cause of the fracture and the literature review of the bilateral femoral neck fracture in renal disease are discussed.

  6. 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.

  7. Bilateral Femoral Neck Stress Fracture in Child: A Case Report

    PubMed Central

    Lee, Gun-Woo; Yoon, Taek-Rim; Eshnazarovich, Eshnazarov Kamolhuja

    2016-01-01

    A femoral neck stress fracture in child is rare, particularly in bilateral case. It is easy to miss initially or may be misdiagnosed. The authors experienced a case of bilateral femoral neck stress fracture in a 10-year-old boy with bilateral hip. The patient was successfully healed by conservative treatment. We report this rare case with a review of the literature. A femoral neck stress fracture should be included in the differential diagnosis in children who present with sustained hip or groin pain. PMID:27777920

  8. Bilateral medial patellofemoral ligament reconstruction in high-level athletes.

    PubMed

    Kuroda, Yuichi; Matsushita, Takehiko; Matsumoto, Tomoyuki; Kawakami, Yohei; Kurosaka, Masahiro; Kuroda, Ryosuke

    2014-10-01

    This report presents two cases of high-level athletes with bilateral patellar dislocations who were able to return to their preinjury level of activity after bilateral medial patellofemoral ligament (MPFL) reconstruction, without any major complications. Patient 1 was a 19-year-old male volleyball player for a top-level college volleyball team, and patient 2 was a 24-year-old woman who was a member of a national-level adult softball team. MPFL reconstruction could be an effective treatment for bilateral patellar dislocation in high-level athletes. Level of evidence V.

  9. 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

  10. Reconstruction of bilateral tendoachilles with fascia lata graft.

    PubMed

    Saxena, Vikas; Pradhan, Pavan; Yadav, Ashok; Nathani, Neeraj

    2013-11-01

    A 19 year old male presented with progressive enlargement of both tendoachilles for 2 years and difficulty in walking for 3 months. The neurological history and examination revealed progressive mental deterioration and ataxia. The blood investigation revealed hypercholesterolemia. We report this rare case of cerebrotendinous xanthomatosis with bilateral tendoachilles enlargement, which was treated by excision of bilateral tendoachilles and reconstruction with fascia lata. The American Orthopedic Foot and Ankle Society hindfoot score was 93/100 bilaterally and the subjective evaluation of the patient showed very good results. PMID:24379473

  11. Bilateral heterotopic ossification after bilateral hip arthroplasty in a geriatric patient.

    PubMed

    Tabert, I; Lekkos, K; Dettmer-Flügge, A; Schmidt, D; Gogol, M

    2011-12-01

    The case of a geriatric patient with total hip arthroplasty for coxarthrosis and an inpatient fall 12 days after the first operation is reported. Six weeks after the first operation, the patient reported new pain in the area of both hips and thighs. X-ray and scintigraphy confirmed the diagnosis of bilateral heterotopic ossification. NSAID therapy was started, and rapid improvement was observed. At discharge, the patient was able to walk with aids in- and outside. At the 12-month follow-up, x-ray control showed Brooker state 3 for the right and 4 for the left hip. Walking ability did not change during follow-up. PMID:22159834

  12. Sotos syndrome.

    PubMed

    Juneja, A; Sultan, A

    2011-12-01

    Sotos syndrome is a well-defined childhood overgrowth syndrome characterized by pre- and postnatal overgrowth, developmental delay, advanced bone age, and a typical facial gestalt including macrodolichocephaly with frontal bossing, frontoparietal sparseness of hair, apparent hypertelorism, downslanting palpebral fissures, and facial flushing. This report presents a case of Sotos syndrome in a 5½-year-old child. PMID:22169837

  13. 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…

  14. 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, ...

  15. 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. ...

  16. Is bilateral congenital anorchia genetically determined?

    PubMed

    Parigi, G B; Bardoni, B; Avoltini, V; Caputo, M A; Bragheri, R

    1999-10-01

    Bilateral congenital anorchia (BCA) can be defined as complete absence of testicular tissue in a patient with male normal phenotype and karyotype. On the basis of familial occurrences of BCA a possible genetic aetiology has been hypothesised, i.e. mutations of the SRY gene which initiates the genetic cascade leading to testis development in mammals. The aim of the study is to assess this hypothesis. Eight boys affected by BCA have been studied; a normal monozygotic twin of one of the patients, a boy and a girl acted as controls. A normal 46, XY karyotype was detected in all patients; 3 had hypoplasia of the scrotum and 2 of the penis. Hormonal data were available for 5 patients: Prader's stimulation test to HCG showed in all lack of testosterone response, and 4 out of 5 had elevated FSH and LH levels. Complete absence of testicular tissue was confirmed in all by surgical exploration. DNA was sampled by Jeanpierre modified extraction method and amplification by polymerase chain reaction. The expected segment of 750 basepairs of the SRY gene, included between the two oligonucleotide primers Xes 10 and Xes 11, was found in all patients. SRY gene is present in our BCA patients as well as in normal boys, and therefore BCA does not seem related to an anomaly of the opening reading frame sequence of the SRY gene. Nevertheless, familial occurrences of BCA continue to suggest a genetic aetiology: further studies must therefore evaluate the possibility of punctiform mutations of the SRY gene, by direct sequentiation, and exclude abnormalities in the critical region DSS/AHC of the X chromosome, recently discovered as one of the loci involved in the differentiation of the male gonad.

  17. The Kluver-Bucy syndrome.

    PubMed

    Gościński, I; Kwiatkowski, S; Polak, J; Orłowiejska, M

    1997-01-01

    Evolution of psychological disorders following head injury including memory and other cognitive disorders are common. The best known are psychiatric disturbances of various kinds after lesions of the frontal lobes. Cognitive, behavioural and emotional disorders are not usually seen in patients with bilateral temporal lesions. In our Department of Neurotraumatology we have observed 4 patients with posttraumatic lesions localized bitemporally. They developed Kluver-Bucy syndrome-rarity in human pathology-combined with three or more of the following symptoms and signs: increased oral activity, hypersexuality, hypermetamorphosis, memory disorders, placidity, loss of people recognition, bulimia. Several symptoms responded dramatically to carbamazepine. We conclude that it may be a useful agent in the treatment of this unusual syndrome.

  18. 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

  19. 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.

  20. Bilateral pulmonary emboli after bilateral mastectomy in a 15-year-old boy with hypogonadism: A case report

    PubMed Central

    Piggott, JR; Yazdani, Arjang

    2010-01-01

    Pulmonary emboli are rare, yet serious, complications of body contouring surgery. When they occur, they more often follow as complications of long, invasive procedures in adults. The present report details a case of bilateral pulmonary emboli in an obese 15-year-old boy with hypogonadism undergoing bilateral mastectomy for gynecomastia. The diagnosis of bilateral pulmonary emboli was made on the basis of clinical presentation and positive ventilation/perfusion scan. The patient responded well to heparin anticoagulation treatment. The relevance of pediatric obesity, pediatric body contouring surgery and the risk of thromboembolic events in pediatric patients are discussed. PMID:22131848

  1. Refeeding syndrome.

    PubMed

    Fernández López, M T; López Otero, M J; Alvarez Vázquez, P; Arias Delgado, J; Varela Correa, J J

    2009-01-01

    Refeeding syndrome is a complex syndrome that occurs as a result of reintroducing nutrition (oral, enteral or parenteral) to patients who are starved or malnourished. Patients can develop fluid-balance abnormalities, electrolyte disorders (hypophosphataemia, hypokalaemia and hypomagnesaemia), abnormal glucose metabolism and certain vitamin deficiencies. Refeeding syndrome encompasses abnormalities affecting multiple organ systems, including neurological, pulmonary, cardiac, neuromuscular and haematological functions. Pathogenic mechanisms involved in the refeeding syndrome and clinical manifestations have been reviewed. We provide suggestions for the prevention and treatment of refeeding syndrome. The most important steps are to identify patients at risk, reintroduce nutrition cautiously and correct electrolyte and vitamin deficiencies properly.

  2. Isolated bilateral fractures of zygomatic arches: report of a case.

    PubMed

    Ho, V

    1994-12-01

    Isolated unilateral fractures of the zygomatic arch are uncommon. Isolated bilateral fractures of the zygomatic arches are even more rare. Indeed a search of the literature failed to find any previous report of this fracture pattern.

  3. Venous outflow of the brain after bilateral complete jugular ligation.

    PubMed

    Ensari, Serdar; Kaptanoğlu, Erkan; Tun, Kağan; Gün, Taylan; Beşkonakli, Etem; Celikkanat, Serdar; Dere, Hüseyin; Cekirge, Saruhan

    2008-01-01

    A case of a patient with bilateral internal, external, posterior external and anterior jugular vein ligations and excisions performed in the neck due to a larynx tumor is presented. Radical neck dissection is a standard otorhinolaryngological procedure in the management of head and neck cancer patients with bilateral lymph node metastasis to the neck. Sacrifice of both internal and external jugular veins bilaterally has been recognized as a dangerous approach leading to intracranial hypertension with subsequent neurological sequela and death. In this report, we aimed to demonstrate how venous outflow from the brain diverts after jugular venous system obliteration. After bilateral jugular vein ligations, digital subtraction angiography (DSA) showed that the venous drainage route of the brain had been diverted from the jugular veins to the vertebral venous plexus. PMID:18382980

  4. Bilateral thalamic infarct as a diagnosed conversion disorder.

    PubMed

    Lok, Ugur; Yalin, Oner; Odes, Ramazan; Bozkurt, Selim; Gulacti, Umut

    2013-05-01

    Bilateral thalamic infarcts are an uncommon type of cerebral infarct. Bilateral paramedian thalamic infarctions may lead to a severe impairment of consciousness. The sudden onset of a lethargy or comatose state, in the absence of motor deficits, easily evokes the idea of a subarachnoid hemorrhage. Other patients present with behavior changes, disorientation in space and time, memory loss, or thought disorders. We believe that bilateral thalamic infarction is often missed in emergency department (ED) in relatively young patients, especially when magnetic resonance imaging is not performed. In these cases, the patient can be discharged with various psychiatric diagnoses. We suggest that bilateral thalamic infarct should be considered in patients in the ED with new diagnoses of conversion disorder.

  5. [A Case of Synchronous Malignant Pheochromocytomas in Bilateral Adrenal Glands].

    PubMed

    Usui, Kimitsugu; Hirasawa, Terukazu; Kobayashi, Masataka; Shioi, Kouichi; Kobayashi, Kazuki; Sakai, Naoki; Noguchi, Sumio; Tsuura, Yukio

    2016-06-01

    We present a case of synchronous malignant pheochromocytoma in bilateral adrenal glands. A 73- year-old man presented to our hospital with bilateral adrenal masses incidentally found during abdominal ultrasonography examination for an unrelated issue. The patient had a 30-year history of hypertension and paroxysmal atrial fibrillation. Computed tomography and magnetic resonance imaging showed heterogeneous tumors in bilateral adrenal glands and an enlarged para-aortic lymph node. Hormonal examinations revealed a high value of urinary catecholamines. Metaiodobenzylguanidine (MIBG) scintigraphy showed increased uptake in bilateral adrenal glands and the lymph node. Both adrenal tumors and the node were surgically removed. Pathological examination revealed histologically distinct tissue between the two adrenal tumors. The patient received five cycles of adjuvant chemotherapy, consisting of cyclophosphamide, vincristine, and dacarbazine. The patient has been in remission for 32 months following surgical treatment. PMID:27452493

  6. Rare bilateral presentation of morning glory disc anomaly.

    PubMed

    Dedhia, Chintan Jethalal; Gogri, Pratik Yeshwant; Rani, Padmaja Kumari

    2016-01-01

    A 4-year-old girl presented with a history of poor vision and oscillation of both eyes since infancy. Ocular examination revealed the best corrected visual acuity of 2/60 in right eye and 3/60 in left eye. Horizontal pendular nystagmus was present in both eyes. Fundus examination revealed morning glory disc anomaly (MGDA) bilaterally. Radiographic imaging of the brain revealed central nervous system anomalies. The guarded visual prognosis was explained and the patient referred for low vision rehabilitation and advised yearly follow-up. MGDA is very rarely bilateral. We aim to highlight the distinguishing features of bilateral MGDA from other excavated optic nerve head anomalies which could also present bilaterally but vary in their associations, management and prognosis. PMID:27571914

  7. Genetics Home Reference: congenital bilateral absence of the vas deferens

    MedlinePlus

    ... mucus clogs the vas deferens as they are forming, causing them to deteriorate before birth. In instances ... BILATERAL APLASIA OF Sources for This Page Cuppens H, Cassiman JJ. CFTR mutations and polymorphisms in male ...

  8. [A Case of Synchronous Malignant Pheochromocytomas in Bilateral Adrenal Glands].

    PubMed

    Usui, Kimitsugu; Hirasawa, Terukazu; Kobayashi, Masataka; Shioi, Kouichi; Kobayashi, Kazuki; Sakai, Naoki; Noguchi, Sumio; Tsuura, Yukio

    2016-06-01

    We present a case of synchronous malignant pheochromocytoma in bilateral adrenal glands. A 73- year-old man presented to our hospital with bilateral adrenal masses incidentally found during abdominal ultrasonography examination for an unrelated issue. The patient had a 30-year history of hypertension and paroxysmal atrial fibrillation. Computed tomography and magnetic resonance imaging showed heterogeneous tumors in bilateral adrenal glands and an enlarged para-aortic lymph node. Hormonal examinations revealed a high value of urinary catecholamines. Metaiodobenzylguanidine (MIBG) scintigraphy showed increased uptake in bilateral adrenal glands and the lymph node. Both adrenal tumors and the node were surgically removed. Pathological examination revealed histologically distinct tissue between the two adrenal tumors. The patient received five cycles of adjuvant chemotherapy, consisting of cyclophosphamide, vincristine, and dacarbazine. The patient has been in remission for 32 months following surgical treatment.

  9. [Bilateral stage nephrectomy as a preparation for kidney transplantation].

    PubMed

    Mayer, P; Bauer, H W; Land, W

    1982-03-01

    The indication for nephrectomy prior to kidney transplantation has changed. Contrary to the formerly in nearly all transplantation centres practiced one-time two-sided nephrectomy, at present the removal of the patient's own kidneys prior to transplantation is done only if strictly indicated. In contrast to other transplantation centres which, if indicated, still today carry out bilateral nephrectomy prior to transplantation is done only if strictly indicated. In contrast to other transplantation centres which, if indicated, still today carry out bilateral nephrectomy prior to transplantation, since 1977 the transplantation centre of Munich prefers the two-time bilateral nephrectomy. Technique, surgical approach, indication and results of our method are presented. In the course of the discussion the disadvantages of the one-time bilateral nephrectomy are opposed to the advantages of the two-time method.

  10. Bilateral infrapatellar tendon rupture in a patient with jumper's knee.

    PubMed

    Rosenberg, J M; Whitaker, J H

    1991-01-01

    This is the fourth case report of bilateral infrapatellar tendon rupture as a result of indirect trauma in a patient without systemic disease. This is the only report we have found of jumper's knee leading to simultaneous infrapatellar tendon ruptures.

  11. Unilateral Arm Urticaria Presenting as a Paraneoplastic Manifestation of Metachronous Bilateral Breast Cancer

    PubMed Central

    Kasi, Pashtoon Murtaza; Hieken, Tina J.; Haddad, Tufia C.

    2016-01-01

    Various paraneoplastic syndromes (PNS) are reported to be associated with breast cancer and can range from mild dermatological symptoms to severe neurological complications. Neurological and dermatological manifestations tend to be the more commonly seen paraneoplastic manifestations, albeit both are relatively rare. Diagnosis of the underlying malignancy is often delayed since the presence and severity of paraneoplastic manifestations are not dependent on the tumor size or stage. Herein, we describe a unique case of unilateral arm urticaria presenting as a paraneoplastic manifestation of metachronous bilateral breast cancer. Similar reports and other PNS associated with breast cancer are described. Recognition of PNS associated with underlying malignancies and age-appropriate screening can facilitate diagnosis of the underlying occult malignancy. Resection of the underlying malignancy can lead to resolution and/or improvement of the PNS for some patients. PMID:26933416

  12. 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

  13. 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

  14. 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

  15. Behavioural Variant Frontotemporal Dementia with Bilateral Insular Hypometabolism: A Case Report.

    PubMed

    Mahapatra, Ananya; Sood, Mamta; Bhad, Roshan; Tripathi, Manjari

    2016-04-01

    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

  16. 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

  17. 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.

  18. 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

  19. 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.

  20. 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

  1. Statin-induced bilateral foot drop in a case of hypothyroidism

    PubMed Central

    Chaudhary, Neera; Duggal, Ashish Kumar; Makhija, Prashant; Puri, Vinod; Khwaja, Geeta Anjum

    2015-01-01

    Muscle involvement is a common manifestation of both clinical and subclinical hypothyroidism, with serum creatine kinase (CK) elevation being probably the most common manifestation, and is seen in up to 90% of patients, but is usually mild (less than 10 times the upper limit of normal). Rhabdomyolysis is a distinctively uncommon presentation of hypothyroidism described usually in the setting of precipitating events such as strenuous exercise, alcohol, or statin use. Rarely rhabdomyolysis and myoedema seen in hypothyroidism can be complicated by the development of anterior compartment syndrome leading to neurovascular compression. We describe a case of a patient with hypothyroidism who developed acute onset bilateral foot drop on initiation of statins. This case highlights the need for cautious use of statins in patients at risk for rhabdomyolysis. PMID:26425013

  2. 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.

  3. 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

  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.

  5. Bilateral sensorineural deafness and hydrocephalus due to foramen of Monro obstruction in sibs: A newly described autosomal recessive disorder

    SciTech Connect

    Chudley, A.E.; McCullough, C.; McCullough, D.W.

    1997-01-31

    We identified a Canadian-Mennonite family in which a brother and sister have hydrocephalus due to obstruction at the foramen of Monro and profound bilateral sensorineural deafness. This appears to be a unique combination of anomalies and, to our knowledge, has not been reported previously. Both parents and a brother are phenotypically normal. The parents are second cousins. Thus, on the basis of consanguinity, affected sibs of both sexes, and in the absence of evidence for intrauterine infections or other adverse perinatal events, this syndrome is likely inherited in an autosomal recessive fashion. 37 refs., 5 figs.

  6. [Bilateral renal vein thrombosis and acute renal failure due to inferior vena cava filter thrombosis. Report of one case].

    PubMed

    Vega, Jorge; Díaz, Rienzi

    2014-11-01

    Bilateral renal vein thrombosis is an unusual etiology of acute renal failure and usually is associated with nephrotic syndrome. We report a 77-year-old man, consulting in the emergency room for anuria that appeared 24 hours after a syncope. The patient was carrier of an inferior vena cava filter prophylactically installed 17 months earlier and was not receiving anticoagulation. Serum creatinine on admission was 5.45 mg/dl and blood urea nitrogen was 54 mg/dl. Computed tomography and Doppler ultrasonography showed an extensive thrombosis of inferior vena cava and both renal veins. Heparin therapy was started with a rapid recovery of renal function and diuresis.

  7. Bilateral basal ganglia lesions in patients with end-stage diabetic nephropathy.

    PubMed

    Li, Jordan Y Z; Yong, Tuck Y; Sebben, Ruben; Khoo, Eewin; Disney, Alex P S

    2008-02-01

    Acute movement disorder associated with reversible bilateral basal ganglia lesions is an increasingly recognized syndrome in patients with end-stage renal disease, especially in the setting of concurrent diabetes mellitus. We report an elderly man with end-stage diabetic nephropathy treated by daily automated peritoneal dialysis who developed subacute symptoms of gait disturbance, dysarthria, dysphagia and lethargy. Computed tomography and magnetic resonance imaging of the head revealed bilateral symmetrical basal ganglia lesions. Repeat imaging 3 weeks later showed that these lesions had regressed spontaneously. However, his neurological symptoms improved slowly. These findings were similar to 23 other cases in the literature. Review of these cases shows that clinical features were predominantly bradykinesia, gait disturbance and concurrent metabolic acidosis (observed in 90% of cases). The pathogenesis of this condition has not been clearly defined, but uraemia may be an aggravating factor in predisposed patients, particularly in the presence of diabetic microvascular disease. There is no specific treatment for this condition; supportive measures are the mainstay of management. In the majority of patients, neurological improvement lags behind regression of basal ganglia lesions seen with neuroimaging, and the long-term outcome is variable.

  8. The role of bilateral adrenalectomy in the treatment of refractory Cushing's disease.

    PubMed

    Wong, Anni; Eloy, Jean Anderson; Liu, James K

    2015-02-01

    Cushing's syndrome (CS) results from sustained exposure to excessive levels of free glucocorticoids. One of the main causes of CS is excessive adrenocorticotropic hormone (ACTH) secretion by tumors in the pituitary gland (Cushing's disease [CD]). Cushing's disease and its associated hypercortisolism have a breadth of debilitating symptoms associated with an increased mortality rate, warranting urgent treatment. Currently, the first line of treatment for CD is transsphenoidal surgery (TSS), with excellent long-term results. Transsphenoidal resections performed by experienced surgeons have shown remission rates ranging from 70% to 90%. However, some patients do not achieve normalization of their hypercortisolemic state after TSS and continue to have persistent or recurrent CD. For these patients, various therapeutic options after failed TSS include repeat TSS, radiotherapy, medical therapy, and bilateral adrenalectomy (BLA). Bilateral adrenalectomy has been shown to be a safe and effective treatment modality for persistent or recurrent CD with an immediate and definitive cure of the hypercortisolemic state. BLA was traditionally performed through an open approach, but since the advent of laparoscopic adrenalectomy, the laparoscopic approach has become the surgical method of choice. Advances in technology, refinement in surgical skills, competency in adrenopathology, and emphasis on multidisciplinary collaborations have greatly reduced morbidity and mortality associated with adrenalectomy surgery in a high-risk patient population. In this article, the authors review the role of BLA in the treatment of refractory CD. The clinical indications, current surgical and endocrinological results reported in the literature, surgical technique (open vs laparoscopic), drawbacks, and complications of BLA are discussed.

  9. Role of bilateral inferior petrosal sinus sampling (BIPSS) in the diagnosis of Cushing's disease in a patient with double superior vena cava.

    PubMed

    Tashi, Sonam; Ng, Keng Sin

    2015-08-01

    Cushing's syndrome is known to have a wide spectrum of clinical presentation with debilitating consequences and morbidity if not diagnosed and treated in time. Sometimes the diagnosis of Cushing's syndrome can be challenging to the endocrinologist, especially when the usual battery of biochemical tests and advanced cross-sectional imaging is negative or inconclusive. We described a case in which the use of bilateral inferior petrosal sinus sampling (BIPSS) was conclusive albeit being technically challenging (due to a rare incidental finding of double superior vena cava) and invasive in nature.

  10. Extrauterine Intrapartum Treatment (EXIT) in bilateral primary fetal hydrothorax.

    PubMed

    Henry, P Y; Aravindan, C S; Sivakumar, K; Krishna, H R

    2009-01-01

    Primary Foetal Hydrothorax (PFHT), is an intrathoracic collection of fluid in the fetus, which may be present on either side or even bilaterally. Advances in foetal diagnostics now allow consideration of the Ex-utero Intrapartum Treatment (EXIT) procedure for PFHT. Ex-utero Intrapartum Treatment (EXIT) allows therapeutic interventions on the neonate while maintaining fetoplacental circulation and thereby maintaining oxygenation. We report two cases of bilateral PFHT managed successfully with EXIT procedure.

  11. Giant Bilateral Juvenile Fibroadenoma of the Breast in Prepubescent Girl.

    PubMed

    Khan, Salma; Khan, Momna; Rafique, Sadia

    2015-10-01

    Juvenile fibroadenoma accounts for 4% of the total fibroadenomas. Giant juvenile fibroadenoma is found in only 0.5% of all fibroadenomas. The authors report a 10-year girl presenting with progressive enlargement of both breasts for one year. Based on clinical findings and Fine Needle Aspiration Cytology (FNAC), a diagnosis of bilateral giant juvenile fibroadenomas of breast was made. She underwent bilateral lumpectomy with breast conservation and made uneventful postoperative recovery. PMID:26522216

  12. Bilateral keratocystic odontogenic tumor: A report of two cases

    PubMed Central

    Srivatsan, K. S.; Kumar, Vikas; Mahendra, Ashish; Singh, Preeti

    2014-01-01

    The designation “keratocyst” was used to describe any jaw cyst in which keratin was formed to a large extent. A rare incidence of bilateral mandibular cysts (odontogenic keratocysts) was related to third molar teeth. Herein, we report two cases of bilateral keratocystic odontogenic tumor in a 22-year-old male and 15-year-old female, which was diagnosed by a series of investigations and treated appropriately. PMID:25298727

  13. Bilateral keratocystic odontogenic tumor: A report of two cases.

    PubMed

    Srivatsan, K S; Kumar, Vikas; Mahendra, Ashish; Singh, Preeti

    2014-01-01

    The designation "keratocyst" was used to describe any jaw cyst in which keratin was formed to a large extent. A rare incidence of bilateral mandibular cysts (odontogenic keratocysts) was related to third molar teeth. Herein, we report two cases of bilateral keratocystic odontogenic tumor in a 22-year-old male and 15-year-old female, which was diagnosed by a series of investigations and treated appropriately.

  14. Syncronized Warthin's tumor in bilateral parotid gland and nasopharynx.

    PubMed

    Güçlü, Oğuz; Muratlı, Aslı; Karatağ, Ozan; Dereköy, Fevzi Sefa; Uludağ, Ahmet

    2012-01-01

    Warthin's tumor is a benign salivary gland tumor of the parotid gland. Although bilateral or multicentric involvement of the parotid gland is common, extraparotid involvement is seen rarely. The nasopharynx is an unusual region for extraparotid involvement. In this article, we present a 52-year-old male case with a synchronized Warthin's tumor in the bilateral parotid gland and nasopharynx, and we discuss the clinical management of the disease.

  15. Acute bilateral vision loss in emergency department: A case report.

    PubMed

    Tanrikulu, Ceren Sen; Hocagil, Hilal; Kaya, Ural; Hocagil, Abdullah Cuneyt

    2016-03-01

    Stroke occurs due to the interruption of blood flow to the brain and it is divided into ischemic and hemorrhagic. In the ischemic strokes, while the most commonly affected vessel is median cerebral artery (MCA), it is particularly affected bilateral posterior cerebral artery (PCA) is very rare condition. In this study, a case of sudden loss of vision and bilateral occipital infarct associated with bilateral vertebral system pathology and methylene tetrahydrofolate reductase (MTHFR) gene mutation were reported. A 62-year-old man was admitted with sudden loss of vision complaint starting 10 h before applying to emergency department. The patient was oriented and cooperative. On neurological examination, there was complete loss of vision in the right eye and only a response to light in the left eye. On the brain computerized tomography (CT), ischemic lesions were observed in the bilateral occipital areas and on magnetic resonance imaging (MRI), there were foci showing diffusion limitation in cortico-subcortical areas of bilateral parieto-occipital region. On the detailed examination at the clinic, MTHFR (a1298c) gene mutation was detected. Bilateral occipital infarction is rare and its diagnosis can be difficult because of its atypical symptoms. Therefore, occipital infarction should be suspected when the only sign is isolated vision loss in patients with risk factor for thromboembolism in their history and detailed visual-neurological examination of these patients should be performed. PMID:27239639

  16. Functional effects of bilateral form deprivation in monkeys.

    PubMed

    Harwerth, R S; Smith, E L; Paul, A D; Crawford, M L; von Noorden, G K

    1991-07-01

    Psychophysical methods were used to study the effects of binocular form deprivation, initiated at 1 month of age, on the visual sensitivities of young monkeys. All the monkeys reared with bilateral form deprivation for 7 weeks or longer had reduced spatial contrast sensitivity for both eyes. Although the contrast sensitivity deficits of the bilaterally form-deprived monkeys generally were larger for one eye than the other, the magnitudes of the deficits were small compared with those produced by similar periods of unilateral form deprivation. For other monocular vision functions investigated, temporal contrast sensitivity and increment-threshold spectral sensitivity, the data for the bilaterally form-deprived animals showed only minor variations from those of the control monkeys. However, none of the bilaterally form-deprived monkeys had binocular vision on either measures of binocular summation or stereodetection, even if the animal had normal monocular vision functions. Therefore, these results show that monocular sensory deficits caused by abnormal early visual experience as a result of bilateral form deprivation are much less severe than those caused by unilateral form deprivation. The differences in the severity of visual deficits may be attributed to the consequences of anomalous binocular competition associated with unilateral form deprivation that was minimized during bilateral form deprivation. Thus, these results illustrate that anomalous binocular competition is more detrimental to the developing visual system of infants than direct deprivation per se. PMID:2071342

  17. Simultaneous vs staged bilateral video-assisted thoracoscopic surgery.

    PubMed

    Yim, A P

    1996-10-01

    It is generally thought that simultaneous bilateral chest surgery carries a high morbidity. We reviewed the results of simultaneous (under one anesthesia) vs staged bilateral video-assisted thoracoscopic surgery (VATS) from a single institution over a 35-month period. From September 1992 to July 1995, we performed simultaneous bilateral VATS on 37 patients (31 males, six females, age ranging from 15 to 55 years) with spontaneous pneumothorax (20) for bleb resections and pleurodesis; thoracodorsal sympathectomy (12) for palmar hyperhidrosis and vasospastic disease; and metastatic sarcomas (five) for wedge lung resections. During the same period, nine patients with metachronous bilateral spontaneous pneumothorax had staged procedures, as did two with digital ischemic ulcers for sympathectomy and three with metastatic pulmonary osteosarcomas for resection. Mean postoperative hospital stays in days for the simultaneous groups were 3.3 for spontaneous pneumothorax, 2.1 for sympathectomy, and 1.5 for wedge resection, compared to 2.9, 2.5, and 2.2 for the staged groups, respectively (p > 0.05 by Mann-Whitney U tests). Likewise, pain assessment by visual analogue scale as well as analgesic requirement showed no significant difference between the simultaneous and the staged groups. We conclude that simultaneous VATS is not associated with increased morbidity or prolonged hospital stay compared to the staged counterparts and provides an attractive alternative to the median sternotomy, bilateral posterolateral thoracotomy, or transternal (clam-shell) thoracotomy for selected cases of simultaneous bilateral lung surgery. PMID:8864102

  18. Laparoscopic Adrenalectomy in a Patient of Von Hippel Lindau Syndrome with Ventriculo-Peritoneal Shunt-Anaesthetic management

    PubMed Central

    Mungasuvalli, Nagaraj Channappa; Chinnappa, Jithendra; Iyer, Sadasivan Shanker; Thammanna, Prathima Padavarahalli

    2014-01-01

    Von Hippel Lindau (VHL) syndrome has a predilection to manifest multiple haemangioblastomas in the retina and central nervous system. We report a rare case of raised intracranial pressure during bilateral laparoscopic adrenalectomy for pheochromocytoma in a patient of VHL syndrome who had a ventriculoperitoneal shunt. PMID:25386452

  19. [The symptomatology of frontal and temporal lobe damages].

    PubMed

    Mizuno, Tomoyuki; Takeda, Katsuhiko

    2009-11-01

    In this paper, we review the symptoms associated with damages to the frontal and/or temporal lobes. Similarities and differences between the symptoms observed after a stroke and in frontotemporal lobar degeneration are also discussed. Frontal lobe damages may lead to various apraxic disorders, including limb-kinetic, ideomotor, gait, buccofacial, and ocular motor apraxia. Language dysfunction can arise from perisylvian lesions as well as from extra-perisylvian regions. Broca's aphasia, Wernicke's aphasia, pure word deafness, and aphemia are typical examples of disorders caused by damages to the perisylvian region. Transcortical motor and anomic aphasias are mostly associated with damages to the extra-perisylvian region. Although it has been reported that executive dysfunction is associated with damages to the frontal lobe, it remains to be determined whether there is a cause-and-effect relationship between the 2. A combination of memory, attention, emotional, and mood disorders may underlie executive dysfunction. Patients with lesions in the inferior temporal lobe often present with various types of agnosia. Visual agnosia is common in semantic dementia, but is infrequent after a stroke in the temporal lobe. Prosopagnosia is a rare consequence of damages to the temporal lobe. Bilateral and right-sided lesions are likely to cause this disorder than left-sided lesions. Although, prosopagnosia is less frequently observed than visual agnosia in semantic dementia, it still is one of the common features of the disease. Bilateral injuries to the mesial temporal lobes have been known to induce a marked amnesic syndrome. It is devastating in that the patient can remember virtually nothing new. However, memories acquired before the injuries are mostly conserved and the patient can still learn motor skills. PMID:19938677

  20. EphA4-Mediated Ipsilateral Corticospinal Tract Misprojections Are Necessary for Bilateral Voluntary Movements But Not Bilateral Stereotypic Locomotion

    PubMed Central

    Serradj, Najet; Paixão, Sónia; Sobocki, Tomasz; Feinberg, Mitchell; Klein, Rüdiger; Kullander, Klas

    2014-01-01

    In this study, we took advantage of the reported role of EphA4 in determining the contralateral spinal projection of the corticospinal tract (CST) to investigate the effects of ipsilateral misprojections on voluntary movements and stereotypic locomotion. Null EphA4 mutations produce robust ipsilateral CST misprojections, resulting in bilateral corticospinal tracts. We hypothesize that a unilateral voluntary limb movement, not a stereotypic locomotor movement, will become a bilateral movement in EphA4 knock-out mice with a bilateral CST. However, in EphA4 full knock-outs, spinal interneurons also develop bilateral misprojections. Aberrant bilateral spinal circuits could thus transform unilateral corticospinal control signals into bilateral movements. We therefore studied mice with conditional forebrain deletion of the EphA4 gene under control by Emx1, a gene expressed in the forebrain that affects the developing CST but spares brainstem motor pathways and spinal motor circuits. We examined two conditional knock-outs targeting forebrain EphA4 during performance of stereotypic locomotion and voluntary movement: adaptive locomotion over obstacles and exploratory reaching. We found that the conditional knock-outs used alternate stepping, not hopping, during overground locomotion, suggesting normal central pattern generator function and supporting our hypothesis of minimal CST involvement in the moment-to-moment control of stereotypic locomotion. In contrast, the conditional knock-outs showed bilateral voluntary movements under conditions when single limb movements are normally produced and, as a basis for this aberrant control, developed a bilateral motor map in motor cortex that is driven by the aberrant ipsilateral CST misprojections. Therefore, a specific change in CST connectivity is associated with and explains a change in voluntary movement. PMID:24719100