Sample records for bilateral perisylvian syndrome

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

    PubMed

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

    2015-03-01

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

  2. Bilateral periventricular nodular heterotopia with amniotic band syndrome.

    PubMed

    Ruggieri, Martino; Spalice, Alberto; Polizzi, Agata; Roggini, Mario; Iannetti, Paola

    2007-06-01

    The amniotic (constriction) band syndrome is characterized by distal ring constrictions, intrauterine amputations, and acrosyndactyly. External constriction by amniotic bands is the generally accepted mechanism: early amniotic rupture leads to formation of mesodermal fibrous strands that constrict, entangle, and amputate distal portions of limbs. Etiology is heterogeneous. Anecdotal cases involving central nervous system abnormalities (e.g., acrania, anencephaly, polymicrogyria, congenital bilateral perisylvian syndrome, neuronal heterotopia, septo-optic dysplasia, and spinal cord tethering) have been reported. We describe a 9-year-old girl with typical features of constriction band syndrome localized to the lower limbs who had also profound mental retardation and drug-resistant epilepsy associated with bilateral periventricular nodular heterotopia (a brain malformation of neuronal migration and proliferation caused by mutations in the X-linked filamin 1 gene [FLN1] on chromosome Xq28). The karyotype was normal, as was mutational screening for FLN1. The occurrence of bilateral periventricular nodular heterotopia in the context of amniotic band syndrome is novel (chance occurrence of both: 0.000004%). PMID:17560504

  3. Sturge-Weber syndrome with bilateral intracranial calcification

    Microsoft Academic Search

    E Boltshauser; J Wilson; R D Hoare

    1976-01-01

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

  4. A new case of megalencephaly and perisylvian polymicrogyria with post-axial polydactyly and hydrocephalus: MPPH syndrome

    Microsoft Academic Search

    Marina Colombani; Mondher Chouchane; Gaelle Pitelet; Laure Morales; Patrick Callier; Jean-Pierre Pinard; Laurence Lion-François; Christel Thauvin-Robinet; Francine Mugneret; Frédéric Huet; Laurent Guibaud; Laurence Faivre

    2006-01-01

    We report a new case of megalencephaly and polymicrogyria with post-axial polydactyly and hydrocephalus (MPPH syndrome) in an 18-month-old girl. She was the first child of healthy non-consanguineous parents and measurements at birth were +3 standard deviations (S.D.) for weight, +2 S.D. for length and +4 S.D. for OFC. Ultrasound scan at 24 weeks of gestation (WG) showed mild ventriculomagaly with

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

    PubMed Central

    Zuki?, Sanela; Sinanovi?, Osman; Hodži?, Renata; Mujagi?, Svjetlana; Smajlovi?, Edina

    2014-01-01

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

  6. Progressive bilateral anterior sacral meningoceles in Marfan syndrome

    Microsoft Academic Search

    R. J. Scheck; T. Schramm; K.-P. Gloning; T. Vogl; E. Ostermayer

    1995-01-01

    Anterior sacral meningoceles (ASM) in Marfan syndrome are rare. They may cause constipation, urinary frequency, dysmenorrhea, and low back pain or numbness. This report describes bilateral ASM at the level of S1, S2 and S3 in a woman with Marfan syndrome who was admitted to the gynaecology department for evaluation of left lower abdominal pain. The magnetic resonance appearance of

  7. Catastrophic Antiphospholipid Syndrome Presenting as Bilateral Central Retinal Artery Occlusions

    PubMed Central

    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

  8. 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

  9. 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

  10. Perisylvian language networks of the human brain

    Microsoft Academic Search

    Marco Catani; Derek K. Jones; Dominic H. ffytche

    2005-01-01

    Early anatomically based models of language consisted of an arcuate tract connecting Broca's speech and Wernicke's comprehension centers; a lesion of the tract resulted in conduction aphasia. However, the heterogeneous clinical pre- sentations of conduction aphasia suggest a greater complexity of perisylvian anatomical connections than allowed for in the classical anatomical model. This article re-explores perisylvian language connectivity using in

  11. Bilateral Wünderlich Syndrome Caused by Spontaneous Rupture of Renal Angiomyolipomas

    PubMed Central

    Sotošek, Stanislav; Marki?, Dean; Španjol, Josip; Krpina, Kristian; Kneževi?, Siniša; Mari?i?, Anton

    2015-01-01

    Wünderlich syndrome (WS) is a urological emergency characterized by retroperitoneal hemorrhage. In most cases, bleeding occurs from a renal angiomyolipoma (AML) and may be the first manifestation of the disease. We report a female patient with bilateral WS due to the metachronous rupture of renal AMLs. Because the patient was stable and the tumor was not malignant, treatment was conservative. Follow-up revealed the full recovery of kidney function and the resolution of the hematoma. PMID:25793141

  12. Bilateral wünderlich syndrome caused by spontaneous rupture of renal angiomyolipomas.

    PubMed

    Sotošek, Stanislav; Marki?, Dean; Španjol, Josip; Krpina, Kristian; Kneževi?, Siniša; Mari?i?, Anton

    2015-01-01

    Wünderlich syndrome (WS) is a urological emergency characterized by retroperitoneal hemorrhage. In most cases, bleeding occurs from a renal angiomyolipoma (AML) and may be the first manifestation of the disease. We report a female patient with bilateral WS due to the metachronous rupture of renal AMLs. Because the patient was stable and the tumor was not malignant, treatment was conservative. Follow-up revealed the full recovery of kidney function and the resolution of the hematoma. PMID:25793141

  13. Epilepsy surgery in bilateral Sturge-Weber syndrome

    Microsoft Academic Search

    Ingrid E. B. Tuxhorn; Heinz W. Pannek

    2002-01-01

    Two infants with severe drug refractory focal epilepsy caused by Sturge-Weber syndrome and extensive cerebral leptomeningeal angiomatosis were referred for preoperative video-electroencephalographic evaluation. Brain imaging with computed tomography and gadolinium-enhanced magnetic resonance imaging demonstrated bilateral disease in both children with a predominance of involvement of one hemisphere. Clinical examination and neurophysiology with ictal video recording demonstrated epileptogenesis from one hemisphere.

  14. 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

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

    PubMed

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

    2014-04-01

    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

  16. Bilateral type-I duane syndrome with multiple anamolies: a case report.

    PubMed

    Varma, Chaitanya; Aroor, Shrikiran; Mundkur, Suneel C; Annamalai, Karthick

    2012-10-01

    The Duane syndrome is a strabismus syndrome which is characterized by congenital non-progressive horizontal ophthalmoplegia which primarily affects the abducens nerve. Approximately 70% of the individuals with the Duane syndrome have an isolated disease. We have described here, a case of bilateral Duane syndrome with associated anamolies. PMID:23205369

  17. Posterior circulation hyperperfusion syndrome after bilateral vertebral artery intracranial stenting.

    PubMed

    Zhang, Renliang; Zhou, Guangyi; Xu, Gelin; Liu, Xinfeng

    2009-01-01

    Hyperperfusion syndrome is a well-documented clinical complication after endarterectomy and carotid stenting. However, little is known about this complication immediately after vertebral artery stenting. A 51-year-old woman who had repeated episodes of dizziness, nausea, and vomiting was diagnosed with vertebrobasilar insufficiency. Diffusion-weighted magnetic resonance imaging showed a lesion in the posterior inferior cerebellar artery territory. The patient underwent bilateral vertebral stenting due to severe stenosis in both intracranial segments of the vertebral arteries. Three hours after the procedure, she had severe headache with vomiting and gradually went into a coma. An urgent brain computed tomographic scan showed hemorrhage in the right cerebellum and subarachnoid region. Also, transcranial Doppler revealed approximate doubling of blood flow velocity in vertebral artery area compared with her baseline value. Cerebral blood flow and blood pressure monitoring, together with intensive antihypertensive therapy, are necessary to reduce the risk of hyperperfusion syndrome after vertebral stenting. Moreover, the safety and efficacy of intracranial vertebral stenting should be further reevaluated by large-scale randomized trials. PMID:18973983

  18. Beckwith-Wiedemann syndrome and bilateral adrenal pheochromocytoma: sonography and MRI findings.

    PubMed

    Baldisserotto, Matteo; Peletti, Adriana Barcellos; Angelo de Araújo, Manoel; Pertence, Ana Paula Cardoso; Dora, Marcelo Dourado; Maciel, Elines Oliva; Gaiger, Ana Maria

    2005-11-01

    Beckwith-Wiedemann syndrome is characterized by a group of clinical abnormalities, the most frequent of which are omphalocele, macroglossia, gigantism, neonatal hypoglycemia and umbilical hernia. The association of this syndrome with malignant tumors is well documented. We report a child with this syndrome associated with bilateral adrenal pheochromocytoma. PMID:15983774

  19. Bilateral diffuse choroidal hemangiomas with unilateral facial nevus flammeus in Sturge–Weber Syndrome

    Microsoft Academic Search

    Arezo Amirikia; Ingrid U Scott; Timothy G Murray

    2000-01-01

    PURPOSE: To report bilateral choroidal hemangiomas associated with unilateral facial nevus flammeus in Sturge–Weber syndrome.METHODS: Case report.RESULTS: A 6-year-old male with a left facial nevus flammeus and a history of a left cerebral angioma had been followed 2 years for increasing esotropia. Examination demonstrated bilateral diffuse choroidal hemangiomas with overlying exudative retinal detachments. After bilateral external beam radiotherapy, the retinal

  20. Bilateral conjunctival retention cysts in the aftermath of Stevens-Johnson syndrome

    PubMed Central

    Rajaraman, Revathi; Raghavan, Anita; Palanisamy, Manikandan

    2008-01-01

    In this case report, we describe the rare occurrence of bilateral conjunctival retention cysts in a child with Stevens-Johnson syndrome. The case was managed conservatively as there were no functional or cosmetic problems. PMID:18158410

  1. Large-sized bilateral axillary artery aneurysms in a patient with marfan syndrome: a case report.

    PubMed

    Jun, Feng; Shun, Zhang Yu; Min, Liu Ya; Xue, Qu Gen; Yan, Tian Hong; Hao, Qin; Li, Xiao Xin

    2010-08-01

    A 46-year-old man presented with large bilateral aneurysm of the axillary arteries combined with Marfan syndrome. Treatment consisted of axillary aneurysm resection and vessel replacement. Postoperative computed tomographic angiography confirmed good flow in the bilateral axillary artery, and the patient recovered without complication. PMID:20719737

  2. Cerebrofacial venous metameric syndrome (CVMS) 3: Sturge-Weber syndrome with bilateral lymphatic\\/venous malformations of the mandible

    Microsoft Academic Search

    N. Ramli; M. Sachet; C. Bao; P. Lasjaunias

    2003-01-01

    We present a case of Sturge-Weber syndrome with a bilateral lymphatic\\/venous malformation of the mandible. Modern biology suggests an explanation for such a case. The classification of cerebrofacial venous metameric syndromes (CVMS) enables us to recognise this lesion as involving the most caudal of the cranial metamere (CVMS 3).

  3. Single Bilateral Dexamethasone Implant in Addition to Panretinal Photocoagulation and Oral Azathioprine Treatment in IRVAN Syndrome

    PubMed Central

    Saatci, Ali Osman; Ayhan, Ziya; Take?, Ömer; Yaman, Aylin; Bajin, F. Meltem Söylev

    2015-01-01

    The idiopathic retinal vasculitis, aneurysms and neuroretinitis (IRVAN) syndrome is a disease characterized by multiple retinal macroaneurysms, neuroretinitis and peripheral capillary nonperfusion. Visual loss may result from either ischemia-related complications or macular involvement. Treatment is not always rewarding. We report a case with stage 2 IRVAN syndrome who was successfully treated with a single bilateral intravitreal dexamethasone implant in addition to panretinal photocoagulation and systemic azathioprine treatment. PMID:25802506

  4. Delayed appearance of bilateral Morgagni herniae in a child with Down's syndrome

    Microsoft Academic Search

    R. Kubiak; C. Platen; E. Schmid; R. Gruber; K.-H. Ludwig; W. Rauh

    1998-01-01

    This case report describes the delayed presentation of bilateral Morgagni herniae in a 4 1\\/2-year-old boy with Down's syndrome.\\u000a The authors emphasize the difficulties in establishing the diagnosis, although ultrasonography appears to be valu?able. A\\u000a review of the literature suggests an association between Morgagni hernias and trisomy 21.

  5. [A bilateral diaphragmatic paralysis due to Parsonage and Turner syndrome--its evolution over eight years].

    PubMed

    Guinard, S; Huchot, E; Couturaud, F; Quiot, J-J; L'hévéder, G; Mialon, P; de Saint Martin, L; Le Gal, G; Leroyer, C

    2008-06-01

    The diaphragmatic paralysis is a rare disease whose causes and evolving forms are numerous. We report the development to eight years of paralysis diaphragmatic bilateral attributed to a Parsonage-Turner syndrome: the lack of recovery is proved by respiratory functional follow-up. The therapeutic possibilities, limited, are discussed. PMID:18656787

  6. Deafness due to bilateral endolymphatic sac tumours in a case of von Hippel-Lindau syndrome

    Microsoft Academic Search

    G Kempermann; H P Neumann; R Scheremet; B Volk; W Mann; J Gilsbach; R Laszig

    1996-01-01

    A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal

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

    PubMed Central

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

    2014-01-01

    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

  8. Bilateral Gluteal Compartment Syndrome Following Right Total Knee Revision: A Case Report

    PubMed Central

    Osteen, Kristie D.; Haque, Shireen H.

    2012-01-01

    Background Gluteal compartment syndrome is a rare occurrence traditionally found in settings of extended immobilization. Thrombolytics and medications with myositis as a potential side effect have also been implicated in a few isolated cases of spontaneous compartment syndrome. Early signs are pain on passive stretching and pain out of proportion to physical examination findings. Failure to recognize and definitively treat compartment syndrome within the first 24 to 36 hours can lead to permanent limb loss and morbidity from a host of systemic complications such as hyperkalemia, renal failure, and sepsis. Case Report We report a case of bilateral gluteal compartment syndrome in a 52-year-old patient following a right total knee revision. On postoperative day 2, physical examination after the patient became agitated and in severe distress from bilateral buttock pain showed that the right and left gluteal regions were tense, hard, and erythematous. Creatinine phosphokinase and liver function tests were significantly elevated. Following emergency fasciotomy, physicians thoroughly reviewed the operative course, medication history, and imaging studies. We withdrew simvastatin, a medication associated with spontaneous compartment syndrome, from our patient's daily medications. By day of discharge, both creatinine phosphokinase and liver function problems were decreasing, and the gluteal pain had significantly resolved. The etiology of bilateral gluteal compartment syndrome in our patient could have been a combination of intraoperative length and positioning with simvastatin-induced myositis. Obesity presented an additional risk factor. Conclusion This case highlights the importance of identifying patients at increased risk of compartment syndrome in the preoperative assessment and following them with more intensive intraoperative and postoperative monitoring. PMID:22778678

  9. Serial neuroimaging in tolosa-hunt syndrome with acute bilateral complete ophthalmoplegia.

    PubMed

    Sugie, Kazuma; Morikawa, Masami; Taoka, Toshiaki; Hirano, Makito; Ueno, Satoshi

    2011-01-01

    Tolosa-Hunt syndrome (THS) is a very rare, relapsing, and remitting painful ophthalmoplegia caused by nonspecific granulomatous inflammation in the cavernous sinus. To our knowledge, bilateral complete, simultaneous palsies of all 3 cranial nerves associated with extraocular movement have not been reported. We describe the first such patient with bilateral THS that responded quickly to corticosteroid therapy. A 54-year-old man presented with a periorbital and frontal headache with acute bilateral severe blepharoptosis and fixed eyes, which dramatically responded to corticosteroid therapy. He had diabetes mellitus type II. Brain MRI showed granulomatous inflammation in both cavernous sinuses and thickening of the surrounding dura mater of the cranial base, suggesting the coexistence of focal hypertrophic cranial pachymeningitis. Our experience indicates that steroid therapy with strict control of blood sugar should be considered in patients with THS complicated by diabetes. MRI is a valuable tool for serially monitoring the response of lesions to treatment in THS. PMID:19453827

  10. Bilateral pleural effusion with APLA positivity in a case of rhupus syndrome

    PubMed Central

    Saha, Kaushik; Saha, Arnab; Mitra, Mrinmoy; Panchadhyayee, Prabodh

    2014-01-01

    Rhupus syndrome is a rare syndrome characterized by overlap of rheumatoid arthritis (RA) and systemic lupus erythematosus (SLE). Our patient was a diagnosed case of RA and developed SLE 2 years after. She was a middle-aged woman, presented with bilateral pleural effusion with exacerbation of skin and joint symptoms of SLE. We diagnosed the case as tubercular pleural effusion by positive Mycobacterium tuberculosis in bactec 460 culture. She had also anti-phospholipid antibody positivity without any symptoms and signs of thrombosis. PMID:25378850

  11. Chronic bilateral common carotid artery occlusion: a model for ocular ischemic syndrome in the rat

    Microsoft Academic Search

    Daniel Lavinsky; Nice Sarmento Arterni; Matilde Achaval; Carlos Alexandre Netto

    2006-01-01

    Background  Ocular ischemic syndrome is a devastating eye disease caused by severe carotid artery stenosis. The reduction of blood flow\\u000a produced by bilateral common carotid artery occlusion (BCCAO) of rats for 7 days induces events related to gliosis with no\\u000a evident histological damage. However, retinal degeneration and cellular death occur after 90 days of BCCAO. Our purpose has\\u000a been to investigate

  12. PHACE syndrome with growth hormone deficiency and absence of bilateral internal carotid arteries: a case report.

    PubMed

    Altin, Hakan; Alp, Hayrullah; Sap, Fatih; Karata?, Zehra; Baysal, Tamer; Karaaslan, Sevim

    2012-01-01

    PHACE syndrome is characterized by posterior fossa malformations (P), large facial hemangiomas (H), arterial anomalies (A), cardiac anomalies or coarctation of aorta (C), and eye anomalies (E) and has striking female predominance. Endocrine abnormalities have recently been described in these patients, involving the thyroid and pituitary glands. We report the case of a 2-year-old girl with the clinical features of PHACE with absence of bilateral internal carotid arteries and isolated growth hormone deficiency. PMID:22010790

  13. Isolated Bilateral Ptosis as an Early Sign of Guillain-Barré Syndrome

    PubMed Central

    Imam, Yahia Z.

    2013-01-01

    Background. Guillain-Barré syndrome (GBS) has many variants with distinct presentations. Ptosis as an initial presentation is rare. Case Report. We describe a young female with bilateral ptosis without ophthalmoplegia as the initial presentation of Guillain-Barré ptosis in an anti-GQ1b IgG antibody negative patient with a favorable outcome to intravenous immunoglobulins. Objectives. Our paper highlights the importance of recognizing GBS as a potential etiology in a patient presenting with isolated ptosis, particularly since the course of GBS can be more dramatic than in the anti-GBQ1b syndromes such as ophthalmoparesis without ataxia and Miller Fisher syndrome or ocular myasthenia. Conclusion. This is the first paper of anti-GBQ1b antibody negative GBS presenting with isolated ptosis without ophthalmoparesis. GBS should be included in the list of differential diagnosis of such presentations. PMID:23585975

  14. Bilateral adrenal cystic lymphangiomas in nevoid basal cell carcinoma (Gorlin-Goltz) syndrome: US, CT, and MR findings.

    PubMed

    Mortelé, K J; Hoier, M R; Mergo, P J; Ros, P R

    1999-01-01

    We present a case of bilateral adrenal cystic lymphangioma in a patient with the Gorlin-Goltz syndrome. This case is unique as it is the first illustrated case (US, CT, and MR findings) of a cystic lymphangioma arising within the adrenal gland. In addition, the coexistence of cystic adrenal lymphangioma with the Gorlin-Goltz syndrome has not been described previously. PMID:10433287

  15. Topographical functional connectivity pattern in the Perisylvian language networks

    Microsoft Academic Search

    H.-D. Xiang; H. M. Fonteijn; D. G. Norris; P. Hagoort

    2010-01-01

    We performed a resting-state functional connectivity study to investigate directly the functional correlations within the perisylvian language networks by seeding from 3 subregions of Broca's complex (pars opercularis, pars triangularis, and pars orbitalis) and their right hemisphere homologues. A clear topographical functional connectivity pattern in the left middle frontal, parietal, and temporal areas was revealed for the 3 left seeds.

  16. Bilateral deficits in fine motor control ability and manual dexterity in women with fibromyalgia syndrome.

    PubMed

    Pérez-de-Heredia-Torres, Marta; Martínez-Piédrola, Rosa M; Cigarán-Méndez, Margarita; Ortega-Santiago, Ricardo; Fernández-de-Las-Peńas, César

    2013-04-01

    The aim of the current study was to investigate fine motor control ability and manual dexterity women with fibromyalgia syndrome (FMS) without symptoms in the upper extremity compared to healthy women. Subtests of the Purdue Pegboard Test (one-hand, bilateral and assembly) and of the Jebsen-Taylor hand-function test (writing, turning cards, picking up small, light and large heavy objects, simulated feeding and stacking checkers) were evaluated bilaterally in 20 women with FMS (aged 35-55 years) without symptoms in the upper limb and 20 age- and hand dominance-matched healthy women. Differences between sides and groups were analysed with several analysis of variance (ANOVA). The ANOVA revealed significant differences between groups (P < 0.001) and sides (P = 0.007) for one-hand pin placement subtest: women with FMS showed bilateral worse scores than controls. Patients also exhibited significantly lower scores in bilateral pin placement and assembly subtests when compared to healthy controls (P < 0.001). The ANOVA also revealed significant differences between groups for writing, turning over cards, picking up small objects, stacking checkers, picking up large light objects and picking up large heavy objects (all, P < 0.001): women with FMS needed more time for these subtests than healthy women with both hands. No difference for simulated feeding was found between groups. Our findings revealed bilateral deficits in fine motor control ability and manual dexterity in patients with FMS without symptoms in the upper extremity. These deficits are not related to the clinical features of the symptoms supporting an underlying central mechanism of altered motor control. PMID:23354668

  17. Bilateral stellate ganglion blockade for recalcitrant oral pain from Burning Mouth Syndrome: a case report.

    PubMed

    Walega, David R; Smith, Clark; Epstein, Joel B

    2014-01-01

    Burning Mouth Syndrome (BMS) is a chronic painful disorder characterized by unremitting bilateral burning oral pain often associated with taste abnormalities and complaints of dry mouth. The diagnosis is made by history and symptom presentation in the absence of an identifiable cause or oral lesion. It is commonly seen in perimenopausal women but is also seen in men, and is considered a small-fiber neuropathy. Management can be challenging and few effective treatments are available. This article presents a case report of stellate ganglion blockade as a treatment for recalcitrant pain from BMS. PMID:24822241

  18. Deafness due to bilateral endolymphatic sac tumours in a case of von Hippel-Lindau syndrome.

    PubMed Central

    Kempermann, G; Neumann, H P; Scheremet, R; Volk, B; Mann, W; Gilsbach, J; Laszig, R

    1996-01-01

    A case of bilateral endolymphatic sac tumours is reported. In a patient with von Hippel-Lindau syndrome, tumour growth in the right cerebellopontine angle caused deafness. The tumour was removed and classified as a metastasis from a thyroid carcinoma. However, on thyroidectomy no primary neoplasm could be found. Eight years later a similar tumour was operated on in the left petrosal bone. Histological appearance, immunocytochemical findings, and the clinical context gave evidence that the tumours had to be reclassified as endolymphatic sac tumours--extremely rare entities. The report supports the hypothesis, suggested by the few earlier case reports, that endolymphatic sac tumours could be one of the inherent tumour manifestations in von Hippel-Lindau syndrome. Images PMID:8795608

  19. Rotational vertebral artery syndrome: 3D kinematics of nystagmus suggest bilateral labyrinthine dysfunction.

    PubMed

    Marti, Sarah; Hegemann, Stefan; von Büdingen, Hans-Christian; Baumgartner, Ralf W; Straumann, Dominik

    2008-05-01

    Whether the rotational vertebral artery syndrome (RVAS), consisting of attacks of vertigo, nystagmus and tinnitus elicited by head-rotation induced compression of the dominant vertebral artery (VA), reflects ischemic dysfunction of uni- or bilateral peripheral or central vestibular structures, is still debated. We report on a patient with bilateral high-grade carotid stenoses, in whom rightward headrotation led to RVAS symptoms including a prominent nystagmus. Three-dimensional kinematic analysis of the nystagmus pattern, recorded with search coils, revealed major downbeat nystagmus with minor horizontal and torsional components. Magnetic resonance angiography demonstrated a hypoplastic right VA terminating in the posterior inferior cerebellar artery, a dominant left VA, and a hypoplastic P1-segment of the left posterior cerebral artery (PCA) that was supplied by the left posterior communicating artery (PCoA). The right PCA and both anterior inferior cerebellar arteries were supplied by the basilar artery. The right PCoA originated from the right internal carotid artery. Color duplex sonography showed severe reduction of diastolic blood flow velocities in the left VA during RVAS attacks. The nystagmus pattern can be best explained by vectorial addition of 3D sensitivity vectors of stimulated right and left anterior and horizontal semicircular canals with slightly stronger stimulation on the left side. We hypothesize that in RVAS, compression of dominant VA leads to acute vertebrobasilar insufficiency with bilateral, but asymmetric ischemia of the superior labyrinth. With regard to RVAS etiology, our case illustrates a type of pure vascular RVAS. Severity of attacks markedly decreased after successful bilateral carotid endarterectomy. PMID:18274804

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

    PubMed

    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

  1. 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

  2. Conn's syndrome and bilateral renal artery stenosis in the presence of multiple renal arteries.

    PubMed

    Glodny, B; Cromme, S; Wörtler, K; Herwig, R; Kisters, K; Winde, G

    2001-04-01

    We report the case of a 42-year-old male who was admitted to our hospital after an acute hypertensive crisis despite four-way anti-hypertensive therapy. The renal scintigraphy, the excretory urogram and the biochemical profile performed two years before were unremarkable, except for slightly elevated serum creatinine and plasma aldosterone, in presence of normal aldosterone/renin ratio. The renal arterial angiography that was performed despite a second unremarkable scintigraphy revealed high-grade bilateral arterial stenosis in the presence of multiple renal arteries. Following dilatation of the left stenosis, the aldosterone/renin ratio was pathologic. Recumbent and orthostatic aldosterone values were 830 pg/ml and 1824 pg/ml, respectively, and recumbent and orthostatic renin values were 0.82 and 1.21 ng angiotensin I/ml/h, respectively. The abdominal computed tomography performed to investigate a possible concomitant Conn's syndrome resulted in the detection of a left adrenal tumor. After resection of the lesion, plasma-aldosterone levels normalized and a pronounced rise in serum potassium levels was observed. Following angioplasty of the right renal artery stenosis, blood pressure could easily be managed with combined beta and calcium channel blocker therapy. Particularly in cases of bilateral (but also in the presence of unilateral) renal artery stenosis in association with Conn's syndrome, all the available screening methods for these disorders can fail. In cases of poor response to combination hypertensive therapies, renal arteriography and a fludrocortisone-suppression test should be performed in order to rule out both renal arterial stenosis and Conn's syndrome, even in the absence of clinical and biochemical findings suspicious for either disorder. PMID:11383914

  3. [Eclampsia and total bilateral amaurosis in a woman subsequently diagnosed with reversible posterior leukoencephalopathy syndrome].

    PubMed

    Andrés, M A Arteche; Ortiz-Gómez, J R; Mansob, A M Martín; Martí, I Plaja; García, S Hernández; Ortigosa, F Sos

    2008-05-01

    Eclampsia is a complication of preeclampsia and is characterized by the appearance of grand mal seizures and/or coma, in the absence of any other neurological abnormalities. Neither focal neurological deficit nor prolonged coma tends to develop following a crisis. Eclampsia should therefore lead us to consider other clinical entities that may require special treatment. We report the case of a pregnant woman who presented total bilateral loss of vision following a grand mal seizure. The patient was subsequently diagnosed with reversible posterior leukoencephalopathy syndrome, which has clinical and radiologic manifestations linked to several causes, such as hypertensive encephalopathy, eclampsia, kidney failure, and immunosuppressant therapy. The syndrome involves headache, altered states of consciousness, changes in vision (including blindness), and seizures; these symptoms generally coincide with a rapid increase in blood pressure. Diagnosis requires neuroimaging, and the typical finding is edema in the posterior zones of the brain hemispheres. The most widely accepted hypothesis concerning the pathophysiologic mechanism underlying this syndrome is failure of cerebral autoregulation with development of vasogenic edema. The prognosis is good and the alterations usually resolve completely with appropriate treatment, which is the same as for the management of eclampsia, with strict monitoring of blood pressure. PMID:18661690

  4. Bilateral hypertrophic olivary nucleus degeneration on magnetic resonance imaging in children with Leigh and Leigh-like syndrome

    PubMed Central

    Taly, A B; Sonam, K; Govindaraju, C; Arvinda, H R; Gayathri, N; Bharath, M M Srinivas; Ranjith, D; Nagappa, M; Sinha, S; Khan, N A; Thangaraj, K

    2014-01-01

    Objective: Bilateral hypertrophic olivary degeneration on brain MRI has been reported in a few metabolic, genetic and neurodegenerative disorders, including mitochondrial disorders. In this report, we sought to analyse whether bilateral symmetrical inferior olivary nucleus hypertrophy is specifically associated with mitochondrial disorders in children. Methods: This retrospective study included 125 children (mean age, 7.6?±?5 years; male:female, 2.6:1) diagnosed with various metabolic and genetic disorders during 2005–2012. The routine MRI sequences (T1 weighted, T2 weighted and fluid-attenuated inversion–recovery sequences) were analysed for the presence of bilateral symmetrical olivary hypertrophy and central tegmental tract or dentate nuclei signal changes. The other imaging findings and the final diagnoses were noted. Results: The cohort included patients with Leigh and Leigh-like syndrome (n?=?25), other mitochondrial diseases (n?=?25), Wilson disease (n?=?40), Type 1 glutaric aciduria (n?=?14), maple syrup urine disease (n?=?13), giant axonal neuropathy (n?=?5) and L-2 hydroxy glutaric aciduria (n?=?3). Bilateral inferior olivary nucleus hypertrophy was noted in 10 patients, all of whom belonged to the Leigh and Leigh-like syndrome group. Conclusion: Bilateral hypertrophic olivary degeneration on MRI is relatively often, but not routinely, seen in children with Leigh and Leigh-like syndrome. Early detection of this finding by radiologists and physicians may facilitate targeted metabolic testing in these children. Advances in knowledge: This article highlights the occurrence of bilateral hypertrophic olivary nucleus degeneration on MRI in children with Leigh and Leigh-like syndrome, compared with other metabolic disorders. PMID:24470583

  5. Brooke-spiegler syndrome with multiple scalp cylindromas and bilateral parotid gland adenomas.

    PubMed

    Kalina, Peter; El-Azhary, Rokea

    2012-01-01

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

  6. 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

  7. Bilateral mini-open decompression in the treatment of carpal tunnel syndrome caused by persistent median artery: case report.

    PubMed

    Acioly, Marcus André; Maior, Paolo Souto; Telles, Carlos; de Aguiar, Guilherme Brasileiro

    2013-12-01

    Carpal tunnel syndrome (CTS) is a common peripheral entrapment neuropathy that is caused by increased pressure within the carpal tunnel resulting in a chronic process of median nerve ischemia and segmental demyelination. We report on a patient with bilateral patent persistent median artery (PMA) affected by typical symptomatic CTS who was treated by bilateral mini-open decompression with complete improvement. For PMA-associated CTS, standard decompression is considered the treatment of choice, whereas resection should be reserved for pathological PMA. Mini-open decompression can be an alternative, as we have verified in this first description. PMID:23504667

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

    PubMed

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

    2015-03-01

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

  9. Unbalanced der(5)t(5;20) translocation associated with megalencephaly, perisylvian polymicrogyria, polydactyly and hydrocephalus.

    PubMed

    Verkerk, Annemieke J M H; Schot, Rachel; van Waterschoot, Laura; Douben, Hannie; Poddighe, Pino J; Lequin, Maarten H; de Vries, Linda S; Terhal, Paulien; Hahnemann, Johanne M D; de Coo, Irenaeus F M; de Wit, Marie-Claire Y; Wafelman, Leontien S; Garavelli, Livia; Dobyns, William B; Van der Spek, Peter J; de Klein, Annelies; Mancini, Grazia M S

    2010-06-01

    The combination of megalencephaly, perisylvian polymicrogyria, polydactyly and hydrocephalus (MPPH) is a rare syndrome of unknown cause. We observed two first cousins affected by an MPPH-like phenotype with a submicroscopic chromosome 5q35 deletion as a result of an unbalanced der(5)t(5;20)(q35.2;q13.3) translocation, including the NSD1 Sotos syndrome locus. We describe the phenotype and the deletion breakpoints of the two MPPH-like patients and compare these with five unrelated MPPH and Sotos patients harboring a 5q35 microdeletion. Mapping of the breakpoints in the two cousins was performed by MLPA, FISH, high density SNP-arrays and Q-PCR for the 5q35 deletion and 20q13 duplication. The 5q35 deletion area of the two cousins almost completely overlaps with earlier described patients with an atypical Sotos microdeletion, except for the DRD1 gene. The five unrelated MPPH patients neither showed submicroscopic chromosomal aberrations nor DRD1 mutations. We reviewed the brain MRI of 10 Sotos patients and did not detect polymicrogyria in any of them. In our two cousins, the MPPH-like phenotype is probably caused by the contribution of genes on both chromosome 5q35 and 20q13. Some patients with MPPH may harbor a submicroscopic chromosomal aberration and therefore high-resolution array analysis should be part of the diagnostic workup. PMID:20503325

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

    PubMed Central

    Jain, Ankur

    2014-01-01

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

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

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

    2015-01-01

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

  13. Early presentation of bilateral gonadoblastomas in a Denys-Drash syndrome patient: a cautionary tale for prophylactic gonadectomy.

    PubMed

    Patel, Payal R; Pappas, John; Arva, Nicoleta C; Franklin, Bonita; Brar, Preneet Cheema

    2013-01-01

    Mutation of the Wilms tumor gene (WT1) is associated with two well-described syndromes called Denys-Drash (DDS) and Frasier (FS). Both are associated with nephropathy and ambiguous genitalia and have overlapping clinical and molecular features. The known risk of Wilms tumor in DDS and gonadoblastoma (GB) in FS patients requires tumor surveillance. The literature reports the occurrence of GB in DDS as lower than FS. This case highlights a very early presentation of bilateral GB in DDS and the consideration of early prophylactic gonadectomy at the time of diagnosis with DDS. PMID:23729537

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

    PubMed Central

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

    2015-01-01

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

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

    PubMed Central

    Marrocos de Aragăo, 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

  16. Anatomical characteristics and surgical treatment of bilateral congenital upper eyelid entropion in an infant with neonatal progeroid syndrome.

    PubMed

    Yaz?c?, Bülent; Toka, Fatih; Çömez, Arzu T

    2014-01-01

    Neonatal progeroid syndrome (NPS) is a rare, autosomal recessive disorder characterized by an aged face, wrinkled skin, decreased subcutaneous fat, and neonatal teeth. The authors describe herein a 2-month-old infant with NPS who underwent eyelid surgery for bilateral upper eyelid entropion and stromal keratitis. The preoperative examination revealed thin, narrow, and soft tarsal plates. The eyelids were large and floppy, and there was no preaponeurotic fat tissue. The entropion was successfully treated by the levator muscle advancing onto the tarsus, anterior lamellar repositioning, horizontal eyelid shortening, and canthal resuspension. Upper eyelid entropion may occur relatively frequently in infants with NPS, and the special anatomical features of the eyelid should be considered while performing entropion surgery. PMID:24807800

  17. 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.

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

    Microsoft Academic Search

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

    2006-01-01

    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

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

    PubMed Central

    2010-01-01

    Introduction Restless legs syndrome is a sensorimotor neurological disorder characterized by an urge to move the legs in response to uncomfortable leg sensations. While asleep, 70 to 90 percent of patients with restless legs syndrome have periodic limb movements in sleep. Frequent periodic limb movements in sleep and related brain arousals as documented by polysomnography are associated with poorer quality of sleep and daytime fatigue. Restless legs syndrome in middle age is sometimes associated with neuropathic foot dysesthesias. The causes of restless legs syndrome and periodic limb movements in sleep are unknown, but the sensorimotor symptoms are hypothesized to originate in the central nervous system. We have previously determined that bilateral forefoot digital nerve impingement masses (neuromas) may be a cause of both neuropathic foot dysesthesias and the leg restlessness of restless legs syndrome. To the best of our knowledge, this case is the first report of bilateral foot neuromas as a cause of periodic limb movements in sleep. Case presentation A 42-year-old Caucasian woman with severe restless legs syndrome and periodic limb movements in sleep and bilateral neuropathic foot dysesthesias was diagnosed as having neuromas in the second, third, and fourth metatarsal head interspaces of both feet. The third interspace neuromas represented regrowth (or 'stump') neuromas that had developed since bilateral third interspace neuroma excision five years earlier. Because intensive conservative treatments including repeated neuroma injections and various restless legs syndrome medications had failed, radical surgery was recommended. All six neuromas were excised. Leg restlessness, foot dysesthesias and subjective sleep quality improved immediately. Assessment after 18 days showed an 84 to 100 percent reduction of visual analog scale scores for specific dysesthesias and marked reductions of pre-operative scores of the Pittsburgh sleep quality index, fatigue severity scale, and the international restless legs syndrome rating scale (36 to 4). Polysomnography six weeks post-operatively showed improved sleep efficiency, a marked increase in rapid eye movement sleep, and marked reductions in hourly rates of both periodic limb movements in sleep with arousal (135.3 to 3.3) and spontaneous arousals (17.3 to 0). Conclusion The immediate and near complete remission of symptoms, the histopathology of the excised tissues, and the marked improvement in polysomnographic parameters documented six weeks after surgery together indicate that this patient's severe restless legs syndrome and periodic limb movements in sleep was of peripheral nerve (foot neuroma) origin. Further study of foot neuromas as a source of periodic limb movements in sleep and as a cause of sleep dysfunction in patients with or without concomitant restless legs syndrome, is warranted. PMID:20849622

  20. Varicocele-Caused Progressive Damage in Bilateral Testis and Sertoli Cell-Only Syndrome in Homolateral Testis in Rats

    PubMed Central

    Liu, Jianjun; Ding, Degang; Liu, Jie

    2014-01-01

    Background We aimed to investigate whether varicocele (VC) in rats can cause Sertoli cell-only syndrome (SCOS). Material/Methods Forty adolescent SD rats were randomly divided into 4 groups: 4-weeks control group, 4-weeks experimental group, 12-weeks control group, and 12-weeks experimental group. Left varicocele models were introduced by partially ligating left kidney veins for the experimental groups, and the sham surgery groups as controls were executed with exactly the same surgery as in the experimental groups except for the ligation. Rats in control and experimental groups for 4 and 12 weeks were killed after laparotomy at 4 and 12 weeks, respectively, the testes were taken out and fixed in fixative containing 4% polyformaldehyde, then were stained by hematoxylin and eosin (HE). The density and viability of sperm were analyzed by computer-aided sperm analysis. Results Compared with rats in 4-weeks and 12-weeks control group, histological structures of bilateral testes in both experimental groups were impaired, most of them showing as focal focuses. The pathological changes of testes in rats of the 12-weeks experimental group were bilateral, and included atrophy of seminiferous tubules, turbulence of spermatogenic cells in seminiferous tubules, defluvium of most spermatogenic cells, abortion of spermatogenesis, and degradation of spermatogenic epithelia. One rat in the 12-weeks experimental group was shown having SCOS, with the spermatogenic cells in seminiferous tubules completely flaked, degraded, or absent, and only Sertoli cells lined the seminiferous tubules. Conclusions Laboratory VC caused progressive impairment of homolateral testes, and SCOS could be induced when the damage was severe. Our results indicate that asthenozoospermia, azoospermia, and SCOS can be prevented by the earlier treatment of VC. PMID:25313556

  1. 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

  2. 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

    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

  3. 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 of any abnormalities in the FOXE3 heterozygotes described suggests that genetic background and environmental factors plays a role in the penetrance of the mutant allele. PMID:20664696

  4. Focal cortical atrophy syndromes.

    PubMed

    Black, S E

    1996-07-01

    The topography of Alzheimer's disease (AD) and its effects on language, perception, and praxis are briefly reviewed as background to the focal cortical atrophy syndromes, including primary progressive aphasia (PPA), posterior cortical atrophy (PCA), and corticobasal degeneration (CBD). Simplistically speaking, there are two main pathological and neuroimaging phenotypes associated with these syndromes. One is frontotemporal degeneration (FTD), a nonspecific spongiform degeneration, with gliosis and neuronal loss, sometimes with Pick cells and bodies, which is usually selective for frontal and anterior temporal cortex. The other is Alzheimer's disease, in which amyloid plaques and neurofibrillary tangles initially develop in the hippocampal region, and spread to the lateral temporal and parietal neocortex and then to the frontal cortex. In the case of PPA, left perisylvian dysfunction is usually evident at presentation clinically and on neuroimaging. Nonfluent progressive aphasia tends to progress anteriorly and is usually associated with FTD. In fluent progressive aphasia, the pathology may progress anteriorly due to FTD or posteriorly, reflecting AD. In PCA, the visual association cortex is targeted bilaterally, often more so on the right, and the pathology is usually indicative of AD. CBD is an asymmetric akinetic-rigid syndrome associated with apraxia, in which swollen achromatic neurons are present in the frontoparietal cortex and substantia nigra on light microscopy, suggesting to some that it may fall within the spectrum of FTD and Pick's disease. The application of new molecular biological techniques, however, suggest that CBD, FTD, and Pick's Disease may be pathologically distinct. The clinico-pathological features of each syndrome are reviewed and compared to those typical for AD, and single photon emission computerized tomography scans representative for each are illustrated. PMID:8811996

  5. Bilateral abducens palsy in a case of cytomegalovirus-associated Guillain–Barré syndrome

    Microsoft Academic Search

    Masahiko Kishi; Ryuji Sakakibara; Emina Ogawa; Fuyuki Tateno; Osamu Takahashi; Michiaki Koga

    :  Ophthalmoparesis in cytomegalovirus (CMV)-associated Guillain–Barré syndrome (GBS) is rare. We treated a 37-year-old woman\\u000a with CMV-GBS who presented with an acute onset of generalized weakness and numbness in the extremities, followed by facial\\u000a diplegia, which led to mechanical ventilation. She had increased IgM and IgG-type antibodies against CMV in the serum and\\u000a increased IgM-type serum anti-GM2 ganglioside antibody was also

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

    PubMed Central

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

    2015-01-01

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

  7. Total Joint Replacement for Severe Bilateral Condylar Resorption-Gorham-Stout Syndrome.

    PubMed

    Kunjur, Jayanth; Hirani, Murtaza; Manisali, Mehmet; Matthews, Nigel Shaun

    2015-06-01

    Gorham-Stout syndrome is an extremely rare bone disorder characterized by osteolytic bony resorption. It is of unknown etiology and thought to result from localized endothelial proliferation of lymphatic vessels that cause the bony resorption. Diagnosis of Gorham-Stout can only be made after excluding other common causes of bony resorption such as those caused by infection, inflammation, malignancy, and endocrine. The clinical symptoms vary from minor pain and swelling to non-healing fractures from minor injuries. Due to the rarity of the disease, the overlap of symptoms with other diseases, and uncertain etiology, very few cases of Gorham-Stout syndrome have been reported. The disease is characterized by gradual resorption of bone particularly the bones of shoulder, skull, thorax, and spine, although long bones may also be affected. We present the case of a 45-year-old male patient who presented to the maxillofacial department with marked facial asymmetry leading to functional and aesthetic problems, showing massive osteolysis of both his mandibular condyles and majority of his ramus. PMID:26080240

  8. Functional organization of perisylvian activation during presentation of sentences in preverbal infants

    Microsoft Academic Search

    Ghislaine Dehaene-Lambertz; Lucie Hertz-Pannier; Jessica Dubois; Sébastien Mériaux; Alexis Roche; Mariano Sigman; Stanislas Dehaene

    2006-01-01

    We examined the functional organization of cerebral activity in 3-month-old infants when they were listening to their mother language. Short sentences were presented in a slow event-related functional MRI paradigm. We then parsed the infant's network of perisylvian responsive regions into functionally distinct regions based on their speed of activation and sensitivity to sentence repetition. An adult-like structure of functional

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

    PubMed Central

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

    2012-01-01

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

  10. 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…

  11. 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

  12. Pierre Robin syndrome associated with bilateral femoral aplasia, shortening of upper extremities, right kidney agenesis and left kidney malformation

    Microsoft Academic Search

    J. Handži?-?uk; M. Bagatin; V. ?uk

    1997-01-01

    A 5-year-old female infant with a extremely rare combination of Pierre Robin syndrome and multiple malformations of the limbs and kidneys is presented. Orthopaedic, hearing, intellectual and speech disabilities and treatment difficulties are discussed.

  13. A novel missense mutation of NSDHL in an unusual case of CHILD syndrome showing bilateral, almost symmetric involvement

    Microsoft Academic Search

    Arne König; Rudolf Happle; Regina Fink-Puches; Hans Peter Soyer; Dorothea Bornholdt; Hartmut Engel; Karl-Heinz Grzeschik

    2002-01-01

    The CHILD syndrome (MIM 308050), an acronym for congenital hemidysplasia with ichthyosiform nevus and limb defects, is an X-linked dominant trait with lethality for male embryos. Recently, we elucidated the underlying gene defect by demonstrating point mutations in NSDHL (NAD[P]H steroid dehydrogenase-like protein) at Xq28 in 6 patients with classic CHILD syndrome. The most striking clinical feature is an inflammatory

  14. Mapping phonemic processing zones along human perisylvian cortex: an electro-corticographic investigation

    PubMed Central

    Molholm, Sophie; Mercier, Manuel R.; Liebenthal, Einat; Schwartz, Theodore H.; Ritter, Walter; Foxe, John J.; De Sanctis, Pierfilippo

    2015-01-01

    The auditory system is organized such that progressively more complex features are represented across successive cortical hierarchical stages. Just when and where the processing of phonemes, fundamental elements of the speech signal, is achieved in this hierarchy remains a matter of vigorous debate. Non-invasive measures of phonemic representation have been somewhat equivocal. While some studies point to a primary role for middle/anterior regions of the superior temporal gyrus (STG), others implicate the posterior STG. Differences in stimulation, task and inter-individual anatomical/functional variability may account for these discrepant findings. Here, we sought to clarify this issue by mapping phonemic representation across left perisylvian cortex, taking advantage of the excellent sampling density afforded by intracranial recordings in humans. We asked whether one or both major divisions of the STG were sensitive to phonemic transitions. The high signal-to-noise characteristics of direct intracranial recordings allowed for analysis at the individual participant level, circumventing issues of inter-individual anatomic and functional variability that may have obscured previous findings at the group level of analysis. The mismatch negativity (MMN), an electro-physiological response elicited by changes in repetitive streams of stimulation, served as our primary dependent measure. Oddball configurations of pairs of phonemes, spectro-temporally matched non-phonemes, and simple tones were presented. The loci of the MMN clearly differed as a function of stimulus type. Phoneme representation was most robust over middle/anterior STG/STS, but was also observed over posterior STG/SMG. These data point to multiple phonemic processing zones along perisylvian cortex, both anterior and posterior to primary auditory cortex. This finding is considered within the context of a dual stream model of auditory processing in which functionally distinct ventral and dorsal auditory processing pathways may be engaged by speech stimuli. PMID:23708059

  15. Sex-Linked Differences in the Anatomy of the Perisylvian Language Cortex: A Volumetric MRI Study of Gray Matter Volumes

    Microsoft Academic Search

    Tracey A. Knaus; Angela M. Bollich; David M. Corey; Lisa C. Lemen; Anne L. Foundas

    2004-01-01

    Perisylvian regions important for auditory processing include Heschl's gyrus (HG), the planum temporale (PT), the posterior superior temporal gyrus (pSTG), and the posterior ascending ramus (PAR). Sex-linked differences in language functions and anatomy have been suggested. To examine sex-linked differences, the authors used MRI to measure HG, PT, pSTG, and PAR volumes. Sex differences were found in right HG and

  16. Complete bilateral ophthalmoplegia with unilateral facial palsy in a child with anti-GQ1b syndrome.

    PubMed

    Kauser, Hina; Jain, Puneet; Sharma, Suvasini; Aneja, Satinder

    2015-02-01

    The classical phenotype of Miller Fisher syndrome is characterized by ophthalmoplegia, ataxia and areflexia. However, less extensive forms have been described. The authors report a 14-y-old boy with positive anti-GQ1b antibodies with unusual clinical findings. He presented with headache, double vision and vomiting for 7 d. Examination revealed complete opththalmoplegia, right lower-motor-neuron facial palsy, no limb weakness or cerebellar signs and normal fundus. CSF examination and MRI brain were normal. Electrophysiological studies showed normal limb nerve conduction studies, low CMAP amplitude of right facial nerve, abnormal blink reflex and negative repetitive-nerve-stimulation test. Anti-GQ1b antibodies were positive. The child was managed conservatively with gradual complete recovery. The patients with positive anti-GQ1b antibodies who do not demonstrate the full complement of the Miller Fisher syndrome triad have been reported previously. However, unilateral facial palsy has not been reported previously. This report further expands the phenotypic spectrum of anti-GQ1b syndrome. PMID:24997143

  17. CAD/CAM bilateral ear prostheses construction for Treacher Collins syndrome patients using laser scanning and rapid prototyping.

    PubMed

    Ciocca, Leonardo; De Crescenzio, Francesca; Fantini, Massimiliano; Scotti, Roberto

    2010-06-01

    Ear defects in patients affected by Treacher Collins syndrome necessitate the replacement of the existing anatomic residuals of the ears with custom-made prostheses. This paper describes a multidisciplinary protocol involving both medicine and computer-aided design/computer-aided manufacturing for manufacturing ear prostheses. Using innovative prototyping technologies together with conventional silicone processing procedures, a step-by-step procedure is presented. The complete workflow includes laser scanning of the defective regions of a patient's face, the use of 3D anatomic models from an ear digital library and rapid prototyping of both substructures for bar anchoring and moulds for silicone processing. PMID:19844817

  18. Functional organization of perisylvian activation during presentation of sentences in preverbal infants

    PubMed Central

    Dehaene-Lambertz, Ghislaine; Hertz-Pannier, Lucie; Dubois, Jessica; Mériaux, Sébastien; Roche, Alexis; Sigman, Mariano; Dehaene, Stanislas

    2006-01-01

    We examined the functional organization of cerebral activity in 3-month-old infants when they were listening to their mother language. Short sentences were presented in a slow event-related functional MRI paradigm. We then parsed the infant's network of perisylvian responsive regions into functionally distinct regions based on their speed of activation and sensitivity to sentence repetition. An adult-like structure of functional MRI response delays was observed along the superior temporal regions, suggesting a hierarchical processing scheme. The fastest responses were recorded in the vicinity of Heschl's gyrus, whereas responses became increasingly slower toward the posterior part of the superior temporal gyrus and toward the temporal poles and inferior frontal regions (Broca's area). Activation in the latter region increased when the sentence was repeated after a 14-s delay, suggesting the early involvement of Broca's area in verbal memory. The fact that Broca's area is active in infants before the babbling stage implies that activity in this region is not the consequence of sophisticated motor learning but, on the contrary, that this region may drive, through interactions with the perceptual system, the learning of the complex motor sequences required for future speech production. Our results point to a complex, hierarchical organization of the human brain in the first months of life, which may play a crucial role in language acquisition in our species. PMID:16968771

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

    PubMed Central

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

    1999-01-01

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

  20. Bilateral paraganglioma.

    PubMed

    Trivedi, Rushikesh Yogesh; Leong, Samuel C

    2012-01-01

    A 41-year-old man presented with bilateral, painless, non-pulsatile left-sided neck swelling. This was noticed incidentally on a routine medical check-up. He had no significant medical history and did not take any regular medications. Urinary catecholamines and routine blood tests were all normal, and when he had been examined he was normotensive. The MRI scan revealed an enhancing lesion on the left side of the neck as seen in figure 1; it measured 4 cm×3 cm and had a 'salt and pepper' appearance causing splaying of the internal and external carotid arteries at the level of the bulb. There was a similar enhancing lesion on the right side, measuring 2.6 cm×2 cm at the level of the carotid bifurcation and 5 cm× 2.6 cm at the jugular bulb, this is seen in figure 1. A diagnosis of bilateral paraganglioma was made. PMID:23144338

  1. Coats' plus: a progressive familial syndrome of bilateral Coats' disease, characteristic cerebral calcification, leukoencephalopathy, slow pre- and post-natal linear growth and defects of bone marrow and integument.

    PubMed

    Crow, Y J; McMenamin, J; Haenggeli, C A; Hadley, D M; Tirupathi, S; Treacy, E P; Zuberi, S M; Browne, B H; Tolmie, J L; Stephenson, J B P

    2004-02-01

    In 1988 we reported two sisters with bilateral Coats' disease, sparse hair, dystrophic nails, and primeval splashes of intracranial calcification. We now provide an update on this family documenting the occurrence of skeletal defects comprising abnormal bone marrow, osteopenia, and sclerosis with a tendency to fractures, a mixed cerebellar and extrapyramidal movement disorder, infrequent epileptic seizures, leukodystrophic changes, and postnatal growth failure. Additionally, we present two previously unreported individuals from Ireland and Switzerland with the identical disorder which we designate Coats' plus. Since our original publication a number of other authors have described, frequently as a "new" syndrome, cases with a variable combination of the same features observed in our patients. We review this literature and suggest that the phenotypic overlap with dyskeratosis congenita may provide a clue to the molecular aetiology of this multisystem disorder. PMID:15002047

  2. Case report: Three-trocar technique for bilateral laparoscopic nephropexy.

    PubMed

    Chekulaev, Dimitri; Dayma, Thierry; Abecassis, Jean-Paul; Peyromaure, Michaël

    2007-01-01

    Nephroptosis is a rare syndrome, which affects the right kidney in the majority of cases. In the current report, a case of bilateral nephroptosis with an unusual presentation is presented. We performed bilateral laparoscopic nephropexy using a three-trocar approach. The intervention was successful, with a short operative time and minimal blood loss. The clinical presentation of our patient and the surgical technique for bilateral laparoscopic nephropexy are described. PMID:17263609

  3. Bilateral Molariform Mandibular Second Premolars

    PubMed Central

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

    2015-01-01

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

  4. Plasma ACTH responses to cortisol infusion are similar in patients with primary hypoadrenalism and patients studied some years after bilateral adrenalectomy for Cushing's syndrome.

    PubMed

    Atkinson, A B; Beacom, R; Kennedy, A L; Hadden, D R; Sheridan, B

    1989-05-01

    It has been suggested that the first-phase feedback of steroid feedback on ACTH secretion is abnormal in Cushing's disease patients studied after adrenalectomies. We have performed hydrocortisone infusions (100 mg over 2 h) in 15 patients who had previously undergone bilateral adrenalectomy for Cushing's disease. None had had any pituitary-directed therapy and none had a pituitary macroadenoma. Their responses were compared with a group of seven hypoadrenal patients. A significant rise in serum cortisol was observed between 5 and 10 min in both groups. The first significant fall in ACTH occurred between 30 and 45 min in both groups. There was no abnormality of first-phase feedback in the Cushing's group and our results do not therefore support previous speculation that such an abnormality provides evidence for a higher central nervous system (CNS) aetiology of Cushing's disease. PMID:2557986

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

    PubMed

    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

  6. Connections of the ventral granular frontal cortex of macaques with perisylvian premotor and somatosensory areas: anatomical evidence for somatic representation in primate frontal association cortex.

    PubMed

    Preuss, T M; Goldman-Rakic, P S

    1989-04-01

    In macaque monkeys with injections of tritiated amino acids or horseradish peroxidase in the ventrolateral granular frontal cortex, we observed extensive anterograde and retrograde labeling of the premotor and somatosensory cortex in and around the lateral sulcus. Comparable labeling was not present with large and small control injections of the dorsal granular cortex. Cytoarchitectonic evaluation of the perisylvian cortex in the three cases examined in detail indicated that labeled areas included the ventral premotor cortex (area 6V); the precentral opercular and orbitofrontal opercular areas (PrCO and OFO); the second somatosensory area (S-II); the opercular cortex immediately anterior to S-II, possibly corresponding to area 2 of the S-I complex; and the central part of the insular cortex, including portions of the granular and dysgranular insular fields (Ig, Idg). Labeling was particularly dense and extensive in areas 6V, S-II, and OFO. Lighter labeling was also present in the rostral inferior parietal lobule (areas 7b and POa). The distribution of label within perisylvian areas was not uniform: certain parts were heavily labeled, while other parts were lightly labeled or unlabeled. Comparison of label distribution with published accounts of the somatotopy of these areas indicates that forelimb and orofacial representations were selectively labeled. Further, our results, taken together with other recent anatomical findings (e.g., Matelli et al.: Journal of Comparative Neurology 251:281-298, 1987; Barbas and Pandya: Journal of Comparative Neurology 256:211-228, 1987) suggest strongly that there is a network of interconnected forelimb and orofacial representations in macaque cortex, involving the ventral granular frontal cortex, area 6V, OFO, opercular area 2, S-II, the central insula, and area 7b. Each injection of frontal cortex which labeled the perisylvian somatic cortex involved the cortex of the ventral rim of the principal sulcus (PSvr). The cortex surrounding the PSvr does not stand out as a distinct area in Nissl-stained material. However, examination of myelin-stained sections prepared from uninjected hemispheres with the Gallyas technique revealed the existence of a distinct zone centered on the PSvr. This myeloarchitectonic area, which we term area 46vr, is more heavily myelinated than the ventral bank and fundus of the principal sulcus (area 46v) but is less heavily myelinated than the ventral (inferior) convexity (area 12). Involvement of area 46vr in our injections was probably responsible for the strong labeling observed in perisylvian somatic areas.(ABSTRACT TRUNCATED AT 400 WORDS) PMID:2708598

  7. Simultaneous Bilateral or Unilateral Carpal Tunnel Release?

    PubMed Central

    Osei, Daniel A.; Calfee, Ryan P.; Stepan, Jeffrey G.; Boyer, Martin I.; Goldfarb, Charles A.; Gelberman, Richard H.

    2014-01-01

    Background: Over 60% of patients with carpal tunnel syndrome present with symptoms and findings of nerve compression in both hands. Our goal was to compare patient-rated difficulties in performing activities of daily living in the early postoperative period between those undergoing bilateral carpal tunnel release and those undergoing unilateral carpal tunnel release. Methods: This prospective cohort study enrolled consecutive patients with bilateral carpal tunnel syndrome undergoing bilateral carpal tunnel release (n = 47) or unilateral carpal tunnel release (n = 41). Patient function and disease severity were measured by an abbreviated form of the Disabilities of the Arm, Shoulder and Hand questionnaire, QuickDASH, and the Boston Carpal Tunnel Questionnaire at baseline, at postoperative visit 1 at a mean time (and standard deviation) of 10 ± 3 days, and at postoperative visit 2 at a mean time (and standard deviation) of 30 ± 6 days. Patients rated their difficulty in completing fifteen activities of daily living each day for the first postoperative week. Patients reported the factors that influenced their choice of surgery. Results: There was no difference in baseline function or disease severity between the two groups with regard to QuickDASH and the Boston Carpal Tunnel Questionnaire. Patients in both groups improved after carpal tunnel release with no difference between groups either at postoperative visit 1 for QuickDASH (p = 0.97) and the Boston Carpal Tunnel Questionnaire (p = 0.86) or at postoperative visit 2 for QuickDASH (p = 0.43) and the Boston Carpal Tunnel Questionnaire (p = 0.34). Patients undergoing bilateral carpal tunnel release had more difficulty only during postoperative days 1 to 2 in opening jars (p = 0.03), cooking (p = 0.008), and doing household chores (p = 0.02). Patients in the two groups did not differ (p > 0.05) in their abilities to perform activities of daily living necessary for personal hygiene or independence on any day during the first seven days following surgery with regard to using the bathroom, bathing, dressing, or eating. Although the most common reason why patients chose bilateral carpal tunnel release was to avoid two surgical procedures (42%), the most common reason why patients chose unilateral carpal tunnel release was concern for self-care (36%). Conclusions: Patients with bilateral carpal tunnel syndrome can anticipate more severe functional impairment during the first few postoperative days with bilateral carpal tunnel release compared with unilateral carpal tunnel release, but limitations beyond postoperative day 2 or 3 are similar for bilateral and unilateral carpal tunnel release. Level of Evidence: Therapeutic Level II. See Instructions for Authors for a complete description of levels of evidence. PMID:24897736

  8. Syndrome in question. MAGIC syndrome.

    PubMed

    Nascimento, Ana Cláudia Mendes do; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Hélio Amante

    2014-01-01

    The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder. PMID:24626673

  9. 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

  10. [Large bilateral lung opacities].

    PubMed

    Mezghani, S; Abdelghani, A; Trabelsi, A; Hayouni, A; Garrouche, A; Benzarti, M; Jerray, M

    2005-12-01

    We report the case of a 42-year-old woman operated for neurofibroma of the scalp on two occasions who consulted for bilateral chest pain which reveated two voluminous bilateral pulmonary masses. Biopsy of one of these masses was in favor of secondary or primary leiomyosarcoma. The association of Von Recklinghausen's disease and soft tissue sarcoma is rare: most often neurofibroma which degenerates is observed. Other types of sarcoma, like leiomyosarcoma, have been described. PMID:16449930

  11. Sirenomelia: the mermaid syndrome

    Microsoft Academic Search

    I. V. Meisheri; V. S. Waigankar; M. P. Patel; A. Naregal; S. Ramesh; P. Muthal

    1996-01-01

    Sirenomelia, or mermaid syndrome, is the extreme form of caudal regression syndrome. We present another case of this fascinating anomaly with fused lower limbs, absent external genitalia, and absent genitourinary system. The patient could not be salvaged because of bilateral renal agenesis. Detailed autopsy findings and a review of the literature are presented.

  12. Mermaid and Potter's Syndrome Occurring Simultaneously

    Microsoft Academic Search

    E. N. Liatsikos; P. Perimenis; K. Dandinis; E. Kaladelfou; G. A. Barbalias

    1999-01-01

    We herein report a case of a female embryo who died in utero and at autopsy she was found to have bilateral renal agenesis with the extrarenal manifestations of Potter's syndrome together with mermaid syndrome which is a rare combination. From all the anomalies of the upper urinary tract bilateral renal agenesis seems to have a cardinal role in the

  13. Bilateral Pseudomonas aeruginosa endophthalmitis following bilateral simultaneous cataract surgery.

    PubMed

    Kashkouli, Mohsen Bahmani; Salimi, Shabnam; Aghaee, Hossein; Naseripour, Masood

    2007-01-01

    A bilateral simultaneous cataract surgery (BSCS) was performed on a 67-year-old man. The surgeon had not changed the surgical settings in between the two procedures for the two eyes. The patient developed fulminant bilateral endophthalmitis a day following the BSCS. Intravitreal culture grew Pseudomonas aeruginosa . The source of infection was not found. Immediate bilateral vitrectomy and intravitreal, subconjunctival, topical and systemic antibiotic did not save the eyes. Patient ended up with bilateral visual loss. PMID:17699948

  14. Bilateral Pseudomonas aeruginosa endophthalmitis following bilateral simultaneous cataract surgery

    PubMed Central

    Salimi, Shabnam; Aghaee, Hossein; Naseripour, Masood

    2007-01-01

    A bilateral simultaneous cataract surgery (BSCS) was performed on a 67-year-old man. The surgeon had not changed the surgical settings in between the two procedures for the two eyes. The patient developed fulminant bilateral endophthalmitis a day following the BSCS. Intravitreal culture grew Pseudomonas aeruginosa . The source of infection was not found. Immediate bilateral vitrectomy and intravitreal, subconjunctival, topical and systemic antibiotic did not save the eyes. Patient ended up with bilateral visual loss. PMID:17699948

  15. Bilateral pisotriquetral loose bodies.

    PubMed

    Williams, G R; Holland, P; Beazley, J; Hyder, N

    2012-04-01

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

  16. Bilateral Rasmussen encephalitis

    Microsoft Academic Search

    Yuguang Guan; Guoming Luan; Jian Zhou; Xingzhou Liu

    2011-01-01

    Rasmussen encephalitis (RE) is a rare, chronic, inflammatory neurological disorder that usually affects one hemisphere of the brain. RE is characterized by frequent and severe seizures, progressive neurological dysfunction, and unilateral brain atrophy. This article documents a case of RE with bilateral involvement occurring at the age of 2. The patient's seizures consisted of focal motor seizures (epilepsia partialis continua

  17. Bilateral traumatic quadriceps tendon rupture

    Microsoft Academic Search

    C. Holm

    1999-01-01

    Summary  Bilateral quadriceps tendon rupture is a rare condition. In most cases the patients with bilateral quadriceps tendon rupture\\u000a have a general degenerative disease. This case story present a middle-aged male, who became a traumatic bilateral quadriceps\\u000a tendon rupture. Though treated acutely and trained intensively he did not achieve full range of motion.

  18. Bilateral pheochromocytoma during the postpartum period

    Microsoft Academic Search

    Lalita Wattanachanya; Udomsak Bunworasate; Wanee Plengpanich; Natnicha Houngngam; Patinut Buranasupkajorn; Sarat Sunthornyothin; Vorasuk Shotelersuk; Thiti Snabboon

    2009-01-01

    Background  Pheochromocytoma manifesting during pregnancy is uncommon but it is responsible for a high maternal and fetal mortality rate,\\u000a especially when unrecognized. Most cases of pheochromocytoma are sporadic but they can be part of hereditary autosomal dominant\\u000a syndromes.\\u000a \\u000a \\u000a \\u000a Case  We describe a case of bilateral pheochromocytoma in a term-pregnant patient with a previous history of medullary thyroid carcinoma\\u000a (MTC). Her genetic study

  19. Bilateral ocular osseous choristomas.

    PubMed

    Moon, Jeung Hee; Yoon, Dae Young; Choi, Chul Soon; Yun, Eun Joo; Yoon, Eun Joo; Park, Sang Joon; Seo, Young Lan; Kim, Byoung Jin

    2005-11-01

    Choristoma is a benign tumour defined as normal tissue in an ectopic location. Osseous choristoma, one subtype of this entity, occurring within the orbit has rarely been reported in the world literature. We report a 6-year-old girl with bilateral ocular osseous choristomas who presented with palpable nodules protruding from both upper lids. The radiological and clinical findings are described and previous reports are reviewed. PMID:16001272

  20. Compressive Bilateral Filtering.

    PubMed

    Sugimoto, Kenjiro; Kamata, Sei-Ichiro

    2015-11-01

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

  1. Ocular Findings of Beals Syndrome

    Microsoft Academic Search

    Sugie Takaesu-Miyagi; Hiroshi Sakai; Tadashi Shiroma; Kazuhisa Hayakawa; Yusei Funakoshi; Shoichi Sawaguchi

    2004-01-01

    Background In this report of two Okinawan patients with Beals syndrome and accompanying ocular complications, the symptoms of Beals syndrome and Marfan syndrome are compared. The etiology of these two syndromes is considered in relation to fibrillin. Cases Case 1 was a 5-year-old boy who showed blue sclera and bilateral enlargement of optic disc cupping. Case 2 was a 24-year-old

  2. Rotational vertebral artery syndrome

    Microsoft Academic Search

    Sarah Marti; Stefan Hegemann; Hans-Christian von Büdingen; Ralf W. Baumgartner; Dominik Straumann

    2008-01-01

    Whether the rotational vertebral artery syndrome (RVAS), consisting of attacks of vertigo, nystagmus and tinnitus elicited\\u000a by head-rotation induced compression of the dominant vertebral artery (VA), reflects ischemic dysfunction of uni- or bilateral\\u000a peripheral or central vestibular structures, is still debated. We report on a patient with bilateral high-grade carotid stenoses,\\u000a in whom rightward headrotation led to RVAS symptoms including

  3. Choroid Plexus Size in Young Children with Sturge-Weber Syndrome

    Microsoft Academic Search

    Paul D. Griffiths; Susan Blaser; Mitra B. Boodram; Derek Armstrong; Derek Harwood-Nash

    1996-01-01

    PURPOSE: To assess the size of the choroid plexus in young children with unilateral and bilateral Sturge-Weber syndrome. METHODS: Subjects included 15 children 4 years old or younger with Sturge-Weber syndrome. Eleven cases were unilateral and four were bilateral. Unilateral or bilateral involvement was determined by the distribution of abnormal leptomeningeal enhancement on MR images. The diameters of the choroid

  4. Severe Acute Orthopnea: Ipilimumab-Induced Bilateral Phrenic Nerve Neuropathy.

    PubMed

    Jinnur, Praveen; Lim, Kaiser G

    2015-08-01

    Ipilimumab is a monoclonal antibody used in the treatment of unresectable or metastatic melanoma. Several immune-related adverse events including potential fatal events have been reported following its use. We report a case of a 66-year-old man who presented with severe acute exertional dyspnea and orthopnea following administration of ipilimumab for metastatic melanoma. Although various peripheral neuropathy syndromes associated with ipilimumab have been reported, bilateral phrenic nerve paralysis has not been previously reported. This case also highlights the clinical features of bilateral phrenic nerve neuropathy. Pulmonologists have to be aware of these unusual immune-related respiratory adverse events in patients being treated with monoclonal antibodies. PMID:25956728

  5. Traumatic bilateral quadriceps tendon rupture

    Microsoft Academic Search

    Lars Hansen; Sřren Larsen; Troels Laulund

    2001-01-01

    Bilateral spontaneous quadriceps tendon rupture is a very rare event, with only an estimated 40 cases reported in the literature.\\u000a We report a case of bilateral spontaneous quadriceps tendon rupture and review the literature. The reviewed literature recommends\\u000a early repair; therefore, early diagnosis is crucial. Reportedly, up to 50% of spontaneous bilateral quadriceps ruptures are\\u000a misdiagnosed at first, resulting in

  6. Bilateral Rasmussen encephalitis.

    PubMed

    Guan, Yuguang; Luan, Guoming; Zhou, Jian; Liu, Xingzhou

    2011-02-01

    Rasmussen encephalitis (RE) is a rare, chronic, inflammatory neurological disorder that usually affects one hemisphere of the brain. RE is characterized by frequent and severe seizures, progressive neurological dysfunction, and unilateral brain atrophy. This article documents a case of RE with bilateral involvement occurring at the age of 2. The patient's seizures consisted of focal motor seizures (epilepsia partialis continua in the fingers on the right hand); tonic convulsions of the right arm and head turning to the right with impairment of consciousness; and secondarily generalized tonic-clonic seizures. Antiepileptic drugs did not effectively control the seizures. The pathology of the left frontoparietal lobe biopsy of the abnormal MRI signal showed typical neuronal loss, abundant lymphocytic infiltration into the perivascular space, and numerous microglial nodules. Bipolar electrocoagulation on functional cortexes, which failed to alter the course of the illness, was followed by high-dose prednisone (about 2 weeks worth), without improvement. Both functional hemispherectomies and anatomical hemispherectomies failed to halt the course of the illness, and contralateral seizures developed and became apparent after the operation, suggesting bilateral RE. PMID:21216675

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

    PubMed

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

    2014-06-01

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

  8. 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

  9. 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

  10. Familial bilateral glomus jugulare tumors

    Microsoft Academic Search

    E. T. Tali; R. N. Sener; E. Ibis; H. Alper; M. Ariyurek

    1991-01-01

    Glomus jugulare tumors may be bilateral or more commonly associated with a glomus tumor in another location. These tumors can also have a familial distribution which appears to be autosomal dominant. In this paper, two brothers are presented with bilateral glomus jugulare tumors. Such occurence appears to be a unique familial manifestation.

  11. Iridoschisis and bilateral lens subluxation associated with periocular eczema.

    PubMed

    Adler, Richard A; Weinberg, Robert S

    2004-01-01

    We present a 53-year-old man with bilateral lens subluxation, unilateral iridoschisis, and a long-standing history of periocular eczema. Although a case of unilateral lens subluxation and ipsilateral iridoschisis has been described recently, the pathogenesis of this simultaneous occurrence remains unclear. Our case raises questions about the relationship among these clinical observations and suggests that these findings may exist as part of an unrecognized oculodermal syndrome. PMID:14967294

  12. 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

  13. Syndrome In Question*

    PubMed Central

    do Nascimento, Ana Cláudia Mendes; Gaspardo, Daniela Barros Cortez; Cortez, Tatiana Mimura; Miot, Hélio Amante

    2014-01-01

    The authors present a male 40-year-old patient with established diagnosis of Behçet's disease which had evolved to recurrent bilateral auricular polychondritis crises. MAGIC syndrome (mouth and genital ulcers with inflamed cartilage) is rare and groups together patients with this clinical picture without necessarily fulfilling the clinical criteria for Behçet's disease or relapsing polychondritis, demonstrating an independent disorder. PMID:24626673

  14. Bilateral Polydactyly in a foal.

    PubMed

    Carstanjen, Bianca; Abitbol, Marie; Desbois, Christophe

    2007-06-01

    The following case report describes the diagnosis and surgery of bilateral polydactyly of unknown origin in a colt. A 7-month-old Berber colt was referred for cosmetic and curative excision of supernumerary digits. Radiographic examination revealed bilateral polydactyly and welldeveloped first carpal bones. Surgery consisted of an osteotomy of both second metacarpal bones combined with an amputation of the supernumerary digits. The follow-up at 18 months after surgery revealed a sound horse with an excellent cosmetic outcome. PMID:17519577

  15. A MITF Mutation Associated with a Dominant White Phenotype and Bilateral Deafness in German Fleckvieh Cattle

    PubMed Central

    Philipp, Ute; Lupp, Bettina; Mömke, Stefanie; Stein, Veronika; Tipold, Andrea; Eule, Johanna Corinna; Rehage, Jürgen; Distl, Ottmar

    2011-01-01

    A dominantly inherited syndrome associated with hypopigmentation, heterochromia irides, colobomatous eyes and bilateral hearing loss has been ascertained in Fleckvieh cattle (German White Fleckvieh syndrome). This syndrome has been mapped to bovine chromosome (BTA) 22 using a genome-wide association study with the bovine high density single nucleotide polymorphism array. An R210I missense mutation has been identified within microphthalmia-associated transcription factor (MITF) as responsible for this syndrome. The mutation is located in the highly conserved basic region of the protein and causes a negative-dominant effect. SOX10 and PAX3 promoter binding site mutations in MITF could be ruled out as causative for the German White Fleckvieh syndrome. Molecular characterization of this newly detected bovine syndrome means a large animal model is now available for the Tietz syndrome in humans. PMID:22174915

  16. Bilateral synchronous male breast cancer

    PubMed Central

    Nwashilli, Nnamdi J.; Ugiagbe, Ezekiel E.

    2015-01-01

    Bilateral synchronous breast cancer is extremely rare. A 75-year-old man presented with a right breast mass, which ulcerated and a lump in the left breast. Right breast examination revealed a breast ulcer 7×10cm with everted edges and complete nipple destruction. The left breast showed a hard lump measuring 4×5cm in the nipple-areolar area, unattached to skin, or underlying structure. There was no palpable axillary lymph node bilaterally. A wedge biopsy of right breast ulcer and excision of the left breast lump confirmed bilateral invasive ductal carcinoma - Grade 2 tumor in both breasts. He had bilateral simple mastectomy and chemotherapy; defaulted for 18 months during treatment, and re-presented with bilateral tumor recurrence. The importance of this case report is to create more awareness that breast cancer can occur in males just as in females, though the incidence is rare in males. Early presentation and compliance with treatment modality provide a better outcome. PMID:25737181

  17. [Norrie-Wardburg syndrome].

    PubMed

    Skevas, A; Kastanioudakis, I; Daniilidis, B; Exarchakos, G

    1992-10-01

    We describe a case of a 25-year old patient with typical Norrie-Warburg Syndrome. From the first year of his life he was found to be blind, with bilateral sensorineural loss of hearing. Audiological examination showed symmetrical moderate bilateral sensorineural hearing loss. His hearing loss was refractory to treatment for the last eight years. Because of timely diagnosis of hearing loss and timely fitting of a hearing aid, the patient could study at school and graduate from university education. Disease carriers who are clinically healthy can be identified only via chromosome analysis. PMID:1418232

  18. Bilateral Multiple Level Lateral Meningocoele

    PubMed Central

    Kumar, B. E. Panil; Hegde, Kishor V.; Kumari, G. Lalitha; Agrawal, Amit

    2013-01-01

    Lateral meningocoele is a very rare disorder characterized by extensions of the dura and arachnoid through an enlarged neural foramen. We report a case of a 23-year-old female with deformity of spine who presented with low back pain and no neurological deficits. A whole spine magnetic resonance imaging showed multiple well-defined cystic masses involving dorsal, lumbar, and sacral spinal levels bilaterally, with dural ectasia and neural foraminal widening suggestive of bilateral multiple level lateral meningocoele. The patient is being managed conservatively and is on regular follow-up. PMID:23607071

  19. Duane retraction syndrome type 1 with usher syndrome type 2: an unreported association.

    PubMed

    Khurana, Bhawna Piplani; Khurana, Aruj Kumar; Grover, Sumit

    2015-01-01

    Duane retraction syndrome is characterized by globe retraction and palpebral fissure narrowing on adduction, with restriction of abduction, adduction, or both. Usher syndrome type 2 consists of congenital bilateral sensorineural hearing loss and retinitis pigmentosa. The authors present a case with a yet unreported association between Duane retraction syndrome type 1 and Usher syndrome type 2. [J Pediatr Ophthalmol Strabismus. 2015;52:e26-e29.]. PMID:25955520

  20. Retinocytoma Associated with Bilateral Retinoblastoma

    Microsoft Academic Search

    Naseripour M; Akbarzadeh S

    Purpose: To report a case of retinocytoma associated with bilateral retinoblastoma. Case Report: A 3-year-old girl presenting with left eye exotropia was referred to our ophthalmic oncology service for a second opinion. Funduscopy under general anesthesia revealed one prominent retinocytoma associated with five discrete retinoblastomas in the left eye and three discrete retino- blastomas in the right eye. Conclusion: Although

  1. Bilateral spontaneous cerebrospinal fluid otorrhea

    Microsoft Academic Search

    Nobumitsu Honda; Yoshihisa Okouchi; Hidemitsu Sato; Tetsuji Sanuki; Naohito Hato; Naoaki Yanagihara; Shingo Murakami; Kiyofumi Gyo

    2004-01-01

    We present a rare case of bilateral cerebrospinal fluid (CSF) otorrhea via multiple bony defects in the left tegmen and a single defect with the herniated brain tissue on the right side. Initially, the patient complained of left hearing loss and fullness and was diagnosed with serous otitis media. After myringotomy, the pulsating watery discharge suggested CSF otorrhea. Five months

  2. Rubinstein-Taybi syndrome: Cranial MR imaging findings

    Microsoft Academic Search

    R. Nuri Sener

    1995-01-01

    Rubinstein-Taybi syndrome is a syndrome of mental retardation associated with broad thumbs and big toes. The patients have a characteristic flat face with a beaked nose. This paper deals with brain changes in a patient with this syndrome, studied by MR imaging, which mainly consist of bilateral rolandic cortical clefts and diminished white matter, which probably account for mental retardation.

  3. Nontraumatic fat embolism syndrome in sickle cell anemia

    Microsoft Academic Search

    Darlene P. Horton; Donna M. Ferriero; William C. Mentzer

    1995-01-01

    A 14-year-old girl with sickle cell disease and nephrotic syndrome developed bone pain, followed by pulmonary edema, seizures, coma, and bilateral flaccid paralysis. Fat embolism syndrome was diagnosed by cranial magnetic resonance imaging and an exchange transfusion was performed. Within 3 months, all symptoms had resolved. It is concluded that fat embolism syndrome must be considered as a possible cause

  4. Bilateral Wada test: Amobarbital or propofol? J. Curot a,c,

    E-print Network

    47 patients who underwent a bilateral intracarotid procedure, performed with amobarbital (18 inhibits the ipsilateral cerebral hemisphere, in order to isolate the contralateral hemisphere and assess to prevent the risk of global amnesic syndrome as in patient HM. Nowadays, the traditional Wada test needs

  5. 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.

  6. 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

  7. 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, Bjřrg 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

  8. Blepharokeratoconjunctivitis in Cockayne syndrome.

    PubMed

    Bhojwani, Rajan; Lloyd, I Chris; Alam, Suemyaa; Ashworth, Jane

    2009-01-01

    Cockayne syndrome is a multisystemic, autosomal recessive disease resulting from abnormalities of DNA repair. Ocular manifestations are common, particularly congenital cataract and retinal dystrophy. This study describes a previously unreported association of blepharokeratoconjunctivitis (BKC) in Cockayne syndrome. The authors conducted a retrospective case review of patients with Cockayne syndrome between 1997 and 2006. The ocular manifestations were documented. All cases were bilaterally aphakic from congenital cataract surgery. Four cases of BKC with resultant corneal changes were identified. Two other cases of BKC without corneal changes were also noted. There were no cases of corneal ulceration or visually significant scarring. These findings are clinically important because many patients with Cockayne syndrome wear contact lenses for the refractive correction of aphakia with a resultant risk of corneal ulceration. PMID:19496505

  9. [Idiopathic bilateral patellar tendon rupture].

    PubMed

    Choufani, C; Barthélemy, R; Danis, J; Demoures, Th; Rigal, S

    2015-04-01

    In the absence of systemic disease, specific treatment or sport tendonitis, simultaneous bilateral patellar tendon rupture is rare. Often missed on the first glance, it represents a diagnostic difficulty that should not be overlooked at the initial medical visit. The loss of active extension of the lower limb and a radiographic patella alta, even in a bilateral context, should raise suspicion of this diagnosis. It is then necessary to search for predisposing causes and to evoke the differential, or frequently associated, diagnoses. The present report illustrates these diagnostic difficulties and summarizes some clinical considerations that might help to avoid neglecting these different elements at the first medical visit (positive diagnosis, associated lesions, favouring factors). PMID:26054172

  10. Bilateral Lhermitte-Duclos disease.

    PubMed

    Bozbuga, Mustafa; Gulec, Ilker; Suslu, Hikmet Turan; Bayindir, Cicek

    2010-01-01

    Lhermitte-Duclos disease (LDD) is a pathologic entity with progrediating, diffuse hypertrophy chiefly of the stratum granulosum of the cerebellum. Typically LDD is a unilateral lesion of the cerebellum or in vermis. Here we report a case of LDD with bilateral lesions of cerebellar hemispheres managed surgically. A 28-year-old woman presented with one-year history of progressive headache, nausea, vomiting, and blurred vision. Neurologic examination revealed a bilateral mild papilledema, mild dysmetria, and dysdiadochokinesia. The cerebellar lesions caused moderate mass effect in posterior fossa with hydrocephalus, and Chiari type I malformation. We performed the suboccipital-retrosigmoid approach, and removed completely the left intracerebellar mass. Symptoms related to elevated intracranial pressure disappeared in a short period postoperatively. PMID:20508358

  11. Multiresolution bilateral filtering for image denoising.

    PubMed

    Zhang, Ming; Gunturk, Bahadir K

    2008-12-01

    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

  12. Olmsted syndrome--a rare syndrome with oral manifestations.

    PubMed

    Batra, Puneet; Shah, Naseem

    2004-05-01

    Olmsted syndrome is a rare, congenital condition characterized by severe palmo-planter keratosis, periorificial keratosis, and hypotrichosis. Though orofacial keratosis is one of the consistent findings of Olmsted syndrome, it has never been reported in the dental literature. We report a case of Olmsted syndrome in an eight-year-old boy who presented with massive and crippling palmoplanter keratosis and bilateral oral lesions in the form of keratotic plaques at the corners of the mouth, as well as on the dorsum of the tongue. PMID:15153872

  13. Post-ictal Klüver-Bucy syndrome after temporal lobectomy.

    PubMed Central

    Anson, J A; Kuhlman, D T

    1993-01-01

    In both animals and humans, Klüver-Bucy syndrome is produced by bilateral temporal lobectomy. It is characterised by hypersexuality, visual agnosia, strong oral tendencies, dietary changes, and hypermetamorphosis. Recurrent, postictal Klüver-Bucy syndrome occurred transiently after seizures in a female who had undergone unilateral temporal lobectomy. The pathophysiological mechanism may have been postictal dysfunction of the remaining temporal lobe, producing a transient functional bilateral temporal lobectomy. Images PMID:8459250

  14. Management of ocular complications of Vogt-Koyanagi-Harada syndrome

    Microsoft Academic Search

    Irfan Perente; Canan Asli Utine; Hanefi Cakir; Vedat Kaya; Ilknur Tugal Tutkun; Omer Faruk Yilmaz

    2009-01-01

    Background Vogt-Koyanagi-Harada syndrome (VKH) is a multisystem disorder, characterized by the T-cell-mediated autoimmune process directed\\u000a against melanocytic antigens in the ocular, nervous, auditory and integumentary systems. The ocular hallmarks of the disease\\u000a involve severe bilateral panuveitis associated with exudative retinal detachment. Case report We report a pediatric case of probable VKH Syndrome with isolated ocular findings, in which bilateral vitritis,

  15. Amelogenesis imperfecta with bilateral nephrocalcinosis.

    PubMed

    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

  16. 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

  17. 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

  18. Bilateral simultaneous ancient schwannomas of the orbit.

    PubMed

    Sales-Sanz, Marco; Sanz-Lopez, Andrea; Romero, Jose Antonio Canto

    2007-01-01

    An unusual case of purely cystic bilateral ancient schwannomas of the orbit is reported. Complete ophthalmologic examination and CT was performed. Diagnosis was established by excisional biopsy of both tumors, including clinicopathologic study. Histologic examination showed a bilateral purely cystic ancient schwannoma. This case underlines the importance of considering neural tumors, including schwannomas, in the differential diagnosis of both cystic and bilateral orbital tumors. As far as we know, no other case has been previously reported. PMID:17237700

  19. Bilateral orbital emphysema from compressed air injury

    Microsoft Academic Search

    Tina Li; Mahmood F Mafee; Deepak P Edward

    1999-01-01

    PURPOSE: To describe a patient who developed bilateral subconjunctival and orbital emphysema after an automobile tire explosion.METHOD: Case report.RESULTS: A 60-year-old man sustained bilateral ocular injury after a tire explosion. Ophthalmic examination disclosed bilateral subconjunctival air, with no visible conjunctival laceration. Computed tomography showed orbital emphysema, with no evidence of orbital fracture. Follow-up examination 2 weeks after the injury disclosed

  20. 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.

  1. Metabolic Syndrome

    MedlinePLUS

    ... applies to a condition known as metabolic syndrome. Metabolic Syndrome Is an Early Warning Sign Metabolic syndrome isn' ... 2 diabetes down the road. What Exactly Is Metabolic Syndrome? Metabolic syndrome is a collection of problems that ...

  2. Beals Syndrome

    MedlinePLUS

    ... Boards & Staff Annual Report & Financials Contact Us Donate Marfan & Related Disorders What is Marfan Syndrome? What are ... the syndrome. How does Beals syndrome compare with Marfan syndrome? People with Beals syndrome have many of ...

  3. Bilateral multifocal renal oncocytoma in pregnancy.

    PubMed

    Wentzel, Schalk W; Vermeulen, Lodewikus P

    2012-10-10

    Renal oncocytomas are the most common benign solid renal tumor, accounting for 3-7% of renal neoplasms. Oncocytomas are multifocal in 2-12% and bilateral in 4-14% of cases. Multifocal bilateral oncocytomas represent only 1.4% of renal cases. We present an extraordinary case of a patient with multifocal bilateral renal oncocytomas during pregnancy. An electronic literature search revealed fewer than 30 reports of on cases of bilateral multifocal renal oncocytomas, none of them occurring in pregnancy. The management of this patient differed from the recommended guidelines for renal masses suspected to be malignant because elective caesarean section and nephrectomy in the second trimester was refused. PMID:23372918

  4. Unilateral vs. Bilateral Damage 1 Running head: UNILATERAL VS. BILATERAL DAMAGE

    E-print Network

    McClelland, James L. "Jay"

    Scoville produced a profound memory loss in patient HM by removing the hippocampus bilaterally. Scoville, 1955; Scoville & Milner, 2000). Why are there such different consequences of unilateral and bilateral

  5. A Rare Entity: Bilateral First Rib Fractures Accompanying Bilateral Scapular Fractures

    PubMed Central

    Gulbahar, Gultekin; Kaplan, Tevfik; Turker, Hasan Bozkurt; Gundogdu, Ahmet Gokhan; Han, Serdar

    2015-01-01

    First rib fractures are scarce due to their well-protected anatomic locations. Bilateral first rib fractures accompanying bilateral scapular fractures are very rare, although they may be together with scapular and clavicular fractures. According to our knowledge, no case of bilateral first rib fractures accompanying bilateral scapular fractures has been reported, so we herein discussed the diagnosis, treatment, and complications of bone fractures due to thoracic trauma in bias of this rare entity. PMID:26175916

  6. SPEECH INTELLIGIBILITY IN NOISE AND CHILDREN WITH BILATERAL CIS: THE EFFECT OF BILATERAL EXPERIENCE

    E-print Network

    Litovsky, Ruth

    ., Lake, J. (In Press, 2005). Bilateral cochlear implants in children: localization acuity measured@waisman.wisc.edu SUMMARY Bilateral cochlear implants (BICIs) are somewhat experimental since the advantages are difficult at determinig whether children, who receive bilateral implants in sequential procedures, exhibit similar effects

  7. Surgical management of Gerhardt syndrome.

    PubMed

    Chiril?, M; Mure?an, R; Cosgarea, M; Tomescu, E

    2010-01-01

    Adduction bilateral vocal fold immobility syndrome may be due by both recurrent laryngeal nerves paralysis--Gerhardt syndrome--and all intrinsic laryngeal muscles paralysis--Riegel syndrome. Etiology of Gerhardt syndrome is thyroid surgery, intubation's maneuver, trauma, neurological disorders, extrala-ryngeal malignancies. The manifestations of Gerhardt syndrome are inspiratory dyspnea and slightly influenced voicing by paramedian vocal folds paralysis with an important narrowing of the airway at the glottic level. The surgical procedures for enlargement of the glottic space can be classified in many ways and their major characteristics are: changes at the glottic level; surgical approach: open neck or endoscopic, with or without opening of the mucosal lining; the need for tracheostomy; the equipment used. The aim of this review is to expound the variety of interventions through the last century marked by the development of the diagnostic methods, the anesthesia and the surgical armament with sophisticated instruments and technologies. PMID:20726297

  8. 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. PMID:24131950

  9. International bilateral water treaty formation

    NASA Astrophysics Data System (ADS)

    Espey, Molly; Towfique, Basman

    2004-05-01

    A logistic model is estimated to determine factors that have influenced the formation of bilateral international water treaties over the last 60 years. Results indicate that the larger a water basin is as a percent of a country's size, the more likely the country is to form a treaty regarding its management. On the other hand, the more control over the water basin any given country has, the less likely it will be involved in a treaty. The results also support aspects of two competing theories about the role of country differences, that in some cases they serve as obstacles to treaty formation yet in other cases they encourage treaty formation to formalize management when informal arrangements are not adequate.

  10. Bilateral shotgun pellet pulmonary emboli

    PubMed Central

    Huebner, Stephen; Ali, Sayed

    2012-01-01

    Intravascular migration of bullets and other foreign bodies is a rare but known complication of penetrating trauma. Missile embolization can represent a diagnostic challenge because it may present in various and unexpected ways. We present the case of a 54-year-old female who sustained shotgun pellet emboli to the pulmonary arteries following a left upper extremity gunshot wound and related vascular surgery. The case illustrates bilateral embolization, and the embolic events occurred following surgery. Embolization should be considered in evaluating patients with gunshot wounds, particularly if there are anomalous symptoms or the projectile is not found in the original, or expected, location. Close attention to the location of the foreign bodies on serial radiographs may reveal the diagnosis of intravascular embolization. PMID:22690290

  11. Multiresolution Bilateral Filtering for Image Denoising

    Microsoft Academic Search

    Ming Zhang; Bahadir K. Gunturk

    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

  12. Bilateral parotid swelling: a radiological review

    PubMed Central

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

    2011-01-01

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

  13. Simultaneous and spontaneous bilateral quadriceps tendons rupture.

    PubMed

    Celik, Evrim Co?kun; Ozbaydar, Mehmet; Ofluoglu, Demet; Demircay, Emre

    2012-07-01

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

  14. Bilateral multicystic kidneys – an unusual case

    Microsoft Academic Search

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

    2002-01-01

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

  15. Non Syndromic Familial Bilateral Decidious Taurodontism – A First Case Report

    PubMed Central

    Panigrahi, Rajat G.; K. T., Srilatha; Bhuyan, Ruchi; Bhuyan, Sanat K.

    2014-01-01

    Taurodontism is anatomical and morphological alteration in the shape of the tooth. In taurodontism the crowns appear larger and the roots shorter compared to the normal anatomy of the respective tooth. The incidence of taurodontism in deciduous is very low and is also not very common in permanent dentition. The endodontic treatment protocol of taurodontic tooth is challenging. Taurodontic tooth requires a very cautious approach in identifying the root canal orientation and presence of any extra or lateral canals. PMID:25654041

  16. Malignant vascular disease of the kidney: Nature of the lesions, mediators of disease progression, and the case for bilateral nephrectomy

    Microsoft Academic Search

    Piero Ruggenenti; Giuseppe Remuzzi

    1996-01-01

    A case of thrombotic microangiopathy presenting as a hemolytic uremic syndrome complicated by untreatable hypertension and ultimately requiring bilateral nephrectomy is discussed. Severe hypertension and renal failure may complicate the course of vascular diseases of the kidney, including thrombotic microangiopathy, chronic hypertension, and scleroderma. Toxins, pressure stress, and immune material may trigger the initial injury to vascular endothelium. The malignant

  17. Bilateral cochlear implants in children R. Litovskya,*, P. Johnstonea

    E-print Network

    Litovsky, Ruth

    Bilateral cochlear implants in children R. Litovskya,*, P. Johnstonea , A. Parkinsonb , R. Petersc of listening with bilateral cochlear implants (CIs) improves the ability of children to hear speech in noise reserved. Keywords: Bilateral; Cochlear; Implantation; Pediatric; Binaural; Experience 1. Introduction

  18. Simultaneous Bilateral Cochlear Implantation in Adults: A Multicenter Clinical Study

    E-print Network

    Litovsky, Ruth

    Simultaneous Bilateral Cochlear Implantation in Adults: A Multicenter Clinical Study Ruth Litovsky- neous" bilateral cochlear implantation (both im- plants placed during a single surgical procedure sensori- neural hearing loss. Performance with the bilateral cochlear implants, using the same speech

  19. Sjögren syndrome

    MedlinePLUS

    Xerostomia-Sjögren syndrome; Keratoconjunctivitis sicca - Sjögren; Sicca syndrome ... in children. Primary Sjögren syndrome is defined as dry eyes and dry mouth without another autoimmune disorder. Secondary ...

  20. Contiguous bilateral head and neck paragangliomas in a carrier of the SDHB germline mutation.

    PubMed

    Collins, Nicole; Dietzek, Alan

    2012-01-01

    Paragangliomas are extremely rare neoplasms with multicentric presentation usually linked to familial tumor syndromes. This patient presented with the uncommon combination of concurrent bilateral carotid body tumors and a unilateral glomus jugulare mass that demonstrated vascular continuity. During treatment, the patient was found to be heterozygous for the SDHB germline mutation, an autosomal dominant genotype of the familial paraganglioma syndromes associated with increased malignancy. The unique profile of the SDHB patient as regards primary evaluation, surgical considerations, and extended surveillance was explored and has led to a proposed treatment algorithm for these patients. PMID:21820839

  1. Post-ictal Klüver-Bucy syndrome after temporal lobectomy

    Microsoft Academic Search

    J A Anson; D T Kuhlman

    1993-01-01

    In both animals and humans, Klüver-Bucy syndrome is produced by bilateral temporal lobectomy. It is characterised by hypersexuality, visual agnosia, strong oral tendencies, dietary changes, and hypermetamorphosis. Recurrent, postictal Klüver-Bucy syndrome occurred transiently after seizures in a female who had undergone unilateral temporal lobectomy. The pathophysiological mechanism may have been postictal dysfunction of the remaining temporal lobe, producing a transient

  2. Phenotypic variation and genetic heterogeneity in Léri-Weill syndrome

    Microsoft Academic Search

    Simone Schiller; Stephanie Spranger; Birgit Schechinger; Maki Fukami; Sabine Merker; Stenvert LS Drop; Jochen Tröger; Hans Knoblauch; Jürgen Kunze; Jörg Seidel; Gudrun A Rappold

    2000-01-01

    Léri-Weill syndrome (LWS) or dyschondrosteosis represents a short stature syndrome characterised by the mesomelic shortening of the forearms and lower legs and by bilateral Madelung deformity of the wrists. Recently, mutations in the pseudoautosomal homeobox gene SHOX have been shown to be causative for this disorder. This gene has previously been described as the short stature gene implicated in Turner

  3. Keratoconus associated with floppy eyelid syndrome.

    PubMed

    Donnenfeld, E D; Perry, H D; Gibralter, R P; Ingraham, H J; Udell, I J

    1991-11-01

    Floppy eyelid syndrome is a recently described entity, which characteristically involves overweight individuals. The characteristic findings are an upper lid that may be readily everted, tarsal laxity, and diffuse papillary conjunctival changes. The cause of floppy eyelid syndrome is believed to be a mechanical disorder due to the eversion of the lids while sleeping. The cause of keratoconus remains uncertain. There are strong proponents to a mechanical etiology for this disease. The authors report five cases of floppy eyelid syndrome with concomitant keratoconus. One patient with bilateral keratoconus had bilateral symmetric floppy eyelid syndrome. The other four patients had asymmetric keratoconus and floppy eyelid syndrome. In all four patients, the keratoconus was significantly worse in the eye with the more severe case of floppy eyelid syndrome. In addition, these four patients all gave a history of sleeping with their head facing predominantly on the side with the floppy eyelid syndrome and keratoconus. Two patients with keratoconus and floppy eyelid syndrome were able to undergo successful contact lens rehabilitation of their keratoconus after treatment of the floppy eyelid syndrome. PMID:1800928

  4. Bilateral nephrocalcinosis and amelogenesis imperfecta: A case report

    PubMed Central

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

    2015-01-01

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

  5. Tourette Syndrome

    MedlinePLUS

    What Is Tourette Syndrome? Tourette syndrome is a condition that affects a person's central nervous system and causes tics. Tics are ... few months or a year. Continue Who Gets Tourette Syndrome? Tourette syndrome can affect people of all ...

  6. Tourette Syndrome

    MedlinePLUS

    NINDS Tourette Syndrome Information Page Condensed from Tourette Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Trials Organizations Additional resources from MedlinePlus What is Tourette Syndrome? Tourette syndrome (TS) is a neurological disorder ...

  7. Fanconi syndrome

    MedlinePLUS

    De Toni-Fanconi syndrome ... Fanconi syndrome can be caused by faulty genes, or it may result later in life due to kidney damage. Sometimes the cause of Fanconi syndrome is unknown. Common causes of Fanconi syndrome ...

  8. Fibromuscular Dysplasia Presenting with Bilateral Renal Infarction

    SciTech Connect

    Doody, O., E-mail: orla_doody@hotmail.co [Alfred Hospital, Department of Radiology (Australia); Adam, W. R. [University of Melbourne and Goulburn Valley Health, School of Rural Health (Australia); Foley, P. T.; Lyon, S. M. [Alfred Hospital, Department of Radiology (Australia)

    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.

  9. 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

  10. 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

  11. 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

  12. 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.

  13. Bilateral ureteral stricture from polyarteritis nodosa.

    PubMed

    Hefty, T R; Bonafede, P; Stenzel, P

    1989-03-01

    A case of bilateral, asynchronous ureteral stricture from polyarteritis nodosa is described. Two cases of unilateral ureteral stricture from polyarteritis nodosa have been reported previously. Ureteral obstruction not associated with retroperitoneal fibrosis is rare with polyarteritis nodosa. PMID:2563780

  14. [Gillespie syndrome: 2 familial cases].

    PubMed

    Boughamoura, L; Yacoub, M; Abroug, M; Chabchoub, I; Bouguezzi, R; Charfeddine, L; Amri, F; Essoussi, A-S

    2006-10-01

    We report 2 familial cases of Gillespie syndrome in an 8-year-old girl and her brother 16 months old. They had both congenital aniridia, cerebellar ataxia and mental retardation. In the girl, pupillar dilation in the 2 eyes and delay in different milestones development were elicited at 2 years. She was summoned at the birth of her brother that had pronounced floppiness and the same ocular abnormalities. Ophtalmological exam confirmed partial and bilateral aniridia in both sibs. Brain MRI showed vermis atrophy in the girl and an hypoplasic inferior vermis in her brother. Apropos of these case reports, we make a brief update about this extremely rare genetic syndrome. PMID:16919425

  15. Wernicke's syndrome after bariatric surgery

    Microsoft Academic Search

    J. SALAS-SALVADÓ; P. GARCÍA-LORDA; G. CUATRECASAS; A. BONADA; X. FORMIGUERA; D. DEL CASTILLO; M. HERNÁNDEZ; J. M. OLIVÉ

    2000-01-01

    Two young females with severe morbid obesity presented with Wernicke's syndrome after Roux-en-Y gastro-jejunum bypass had been performed. The first patient had recurrent vomiting and dyplopia two months post-surgery. Physical examination indicated bilateral ophthalmoparesia with conserved convergence and ataxia. The second patient had frequent vomiting episodes over the previous three months together with lower limb hypotonia, myoclonia and generalised tonicoclonic

  16. A case of Kallmann syndrome

    Microsoft Academic Search

    Maria Luisa Cecilia R Arkoncel; Francis Raymond P Arkoncel; Frances Lina Lantion-Ang

    2011-01-01

    Kallmann syndrome (KS), a rare genetic disorder, refers to the association between hypogonadotropic hypogonadism and anosmia or hyposmia due to abnormal migration of olfactory axons and gonadotropin-releasing hormone producing neurons.The authors report a case of a 26-year-old Filipino male who presented with eunuchoid body proportion, absence of facial and axillary hair and sparse pubic hair, micropenis and bilaterally descended prepubertal

  17. [The Marinescu-Sjögren syndrome].

    PubMed

    P?nescu, C

    1991-01-01

    The patient, 54 years old, has an evolutive bilateral congenital cataract, with lens opacities, initially placed in posterior cortex and which developed to a total cataract. The patient also present oligophrenia, spinocerebral ataxia and trophic disturbances with kyphoscoliosis and extremities deforms. We insist on the research of the trophic and neurological disturbances at the patients with congenital cataract for the elucidation of some etiopathogenic aspects in the degenerative syndrome described by Marinescu. PMID:1845442

  18. Cowden Syndrome Presenting as Breast Cancer: Imaging and Clinical Features

    PubMed Central

    Seo, Mirinae; Ahn, Hye Shin; Moon, Hyeong-Gon

    2014-01-01

    Cowden syndrome is an uncommon, autosomal dominant disease which is characterized by multiple hamartomas of the skin, mucous membrane, brain, breast, thyroid, and gastrointestinal tract. The diagnosis of Cowden syndrome implicates an increased risk of developing breast cancer. We report a case of a 22-year-old woman with Cowden syndrome that presented as breast cancer with concomitant bilateral exuberant benign masses in both breasts. PMID:25246819

  19. Bilateral quadriceps tendon rupture — A case report

    Microsoft Academic Search

    T. K. Kaar; M. O’Brien; P. Murray; G. B. Mullan

    1993-01-01

    Summary  The diagnosis of rupture of the quadriceps tendon is made relatively infrequently and bilateral simultaneous rupture is a\\u000a rarity. We report a case of spontaneous bilateral rupture of the quadriceps tendon in a patient with documented chronic renal\\u000a insufficiency and secondary hyperparathyroidism. A predisposing cause to the condition as obtained in this case, has been\\u000a described in cases previously published

  20. New syndromic entity of situs inversus totalis.

    PubMed

    Mubashir, M A; Sabry, M A; Farah, S; Haseeb, N; Quasrawi, B; al-Busairi, W; al-Dabbous, R; al-Awadi, S A; Farag, T I

    1999-01-01

    A 22-year-old Bedouin female with MCA/MR has been recently ascertained. She showed profound mental retardation, proportionate short stature, facial dysmorphism, spastic quadreparesis, bilateral taliper equinovarus, brachydactyly, situs inversus totalis, and MRI findings of cerebellar/midbrain migration defects. The described phenotype represents a new syndromic situs inversus with a characteristic Facio-Cerebro-Skeleto-Cardiac phenotype. PMID:10327248

  1. Anterior Plagiocephaly in an Atypical Case of Apert Syndrome

    PubMed Central

    Gupta, Madhumita; Pai, Ashwin Alke; Bhattacharya, Abhimanyu; Ramachandra, Ravi; Sawarappa, Raghavendra; Mohapatra, Subhakanta; Kanoi, Aditya

    2013-01-01

    Apert syndrome is a congenital craniosynostosis syndrome comprising of bilateral coronal synostosis , symmetric syndactyly of hands and feet and midface hypoplasia. We present an atypical phenotype of this syndrome with right sided unilateral coronal synostosis. However, type I apert hand and other clinical and radiological features suggestthe diagnosis. Genetic analysis revealed an absence of the specific missense mutations in the FGFR 2 gene that is found in patients with this syndrome. We conclude that this patient represented a rare atypical variant of Apert syndrome. Further analysis is required to map the associated genotype. PMID:25489515

  2. [Sturge-Weber syndrome].

    PubMed

    Reith, W; Yilmaz, U; Zimmer, A

    2013-12-01

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

  3. Cauda equina syndrome.

    PubMed

    Orendácová, J; Cízková, D; Kafka, J; Lukácová, N; Marsala, M; Sulla, I; Marsala, J; Katsube, N

    2001-08-01

    Single or double-level compression of the lumbosacral nerve roots located in the dural sac results in a polyradicular symptomatology clinically diagnosed as cauda equina syndrome. The cauda equina nerve roots provide the sensory and motor innervation of most of the lower extremities, the pelvic floor and the sphincters. Therefore, in a fully developed cauda equina syndrome, multiple signs of sensory disorders may appear. These disorders include low-back pain, saddle anesthesia, bilateral sciatica, then motor weakness of the lower extremities or chronic paraplegia and, bladder dysfunction. Multiple etiologies can cause the cauda equina syndrome. Among them, non-neoplastic compressive etiologies such as herniated lumbosacral discs and spinal stenosis and spinal neoplasms play a significant role in the development of the cauda equina syndrome. Non-compressive etiologies of the cauda equina syndrome include ischemic insults, inflammatory conditions, spinal arachnoiditis and other infectious etiologies. The use of canine, porcine and rat models mimicking the cauda equina syndrome enabled discovery of the effects of the compression on nerve root neural and vascular anatomy, the impairment of impulse propagation and the changes of the neurotransmitters in the spinal cord after compression of cauda equina. The involvement of intrinsic spinal cord neurons in the compression-induced cauda equina syndrome includes anterograde, retrograde and transneuronal degeneration in the lumbosacral segments. Prominent changes of NADPH diaphorase exhibiting, Fos-like immunoreactive and heat shock protein HSP72 were detected in the lumbosacral segments in a short-and long-lasting compression of the cauda equina in the dog. Developments in the diagnosis and treatment of patients with back pain, sciatica and with a herniated lumbar disc are mentioned, including many treatment options available. PMID:11311464

  4. Use of adrenal arterial embolization in severe ACTH-dependent Cushing's syndrome

    Microsoft Academic Search

    S. B. Blunt; M. Pirmohamed; V. K. Chatterjee; J. M. Burrin; D. J. Allison; G. F. Joplin

    1989-01-01

    The management of a patient with severe Cushing's syndrome due to ectopic ACTH produced by a medullary carcinoma of the thyroid is described. Initial treatment with maximal adrenolytic medical therapy and two attempts at bilateral adrenal venous infarction had failed to control the disease, and she was at that time unfit for surgery. Subsequent use of bilateral adrenal arterial embolization

  5. Functioning endometrium and endometrioma in a patient with mayer-rokitanski-kuster-hauser syndrome.

    PubMed

    Kawano, Yasushi; Hirakawa, Tomoko; Nishida, Masakazu; Yuge, Akitoshi; Yano, Mitsutake; Nasu, Kaei; Narahara, Hisashi

    2014-01-01

    Mayer-Rokitanski-Kuster-Hauser (MRKH) syndrome is a rare disease. A 27-year-old woman was admitted for primary amenorrhea and cyclic pelvic pain. Magnetic resonance imaging (MRI) revealed bilateral Müllerian remnants with functioning endometrium and a pelvic mass considered to be an endometriotic cyst. Bilateral Müllerian remnants were removed, and right ovarian cystectomy was performed at laparoscopic surgery. Accurate evaluation before the operation and informed consent are necessary to treat patients with MRKH syndrome. PMID:25336999

  6. [Cushing's syndrome: diagnostic exploration].

    PubMed

    Tabarin, A; Roger, P

    The diagnosis of Cushing's syndrome is one of the most perplexing and controversial problems in endocrinology. However, significant advances in the diagnosis procedures have been made in the past decade. The diagnostic studies involved in the evaluation of patients with suspected Cushing's syndrome fall into two categories: confirming the presence of true hypercortisolism and establishing the precise aetiology. Diagnosis of Cushing's syndrome: ambulatory screening relies on the overnight 1 mg dexamethasone test. Negative tests are confirmed by measuring cortisol in two 24-hour urine samples. If cortisol excretion is slightly above normal, a 48-hour low-dose dexamethasone suppression test or an intravenous infusion dexamethasone suppression test are required. Diagnosis of the aetiology of Cushing's syndrome: the first step is to establish if the hypercortisolism is ACTH-dependent or not. This step is solved by measuring plasma ACTH and cortisol in the late afternoon. Computed tomography scanning of the adrenal glands is required in ACTH-independent Cushing's syndrome. A unilateral tumour will be demonstrated in most of cases. If bilateral lesions are found, dynamic testing using cortisol releasing factor and/or metyrapone must be performed to confirm the ACTH-independency of the syndrome. In ACTH-dependent Cushing's syndrome, the major difficulty is to distinguish between a pituitary source and an ectopic source of ACTH secretion. Magnetic resonance imaging of the pituitary with gadolinium enhancement must be preferred to computed tomography scanning but its sensitivity is not better than 70-80% and false positives can occur. When no macroscopic pituitary lesion can be detected, bilateral inferior petrosal sinus sampling coupled to CRH injection for ACTH measurement will indicate the source of ACTH secretion. If this test indicates the patient has Cushing's disease, pituitary trans-sphenoidal surgery can be performed. If the test indicates the patient has ectopic ACTH-secretion, a cervico-thoraco-abdominal scanning is necessary to identify the tumour. In the case of occult tumour the hypercortisolism must be controlled by pharmacological agents and the imaging investigations must be repeated at appropriate intervals. PMID:8127815

  7. COMPARISON OF BILATERAL VERSUS UNILATERAL VARICOCELECTOMY IN MEN WITH PALPABLE BILATERAL VARICOCELES

    Microsoft Academic Search

    DOUGLAS SCHERR; MARC GOLDSTEIN

    1999-01-01

    PurposeThe left varicocele is usually larger in men with bilateral varicoceles. We hypothesized that most of the benefit of varicocelectomy would derive from repair of the larger varicocele. To test this hypothesis we prospectively compared the effect of unilateral versus bilateral microsurgical varicocelectomy in men with large (grade III) or moderate (II) left varicocele associated with small but palpable (I)

  8. 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. PMID:20637551

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

    PubMed Central

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

    2014-01-01

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

  10. Tourette Syndrome

    MedlinePLUS

    ... first described the condition in 1885. What Is Tourette Syndrome? Tourette syndrome (TS) is a neurological disorder ... is still being studied. Back Continue Dealing With Tourette Syndrome Many people don't understand what TS ...

  11. 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 ...

  12. Down Syndrome

    MedlinePLUS

    ... NICHD Research Information Clinical Trials Resources and Publications Down Syndrome: Condition Information Skip sharing on social media links Share this: Page Content What is Down syndrome? Down syndrome describes a set of cognitive and ...

  13. Usher Syndrome

    MedlinePLUS

    ... for Usher syndrome. The best treatment involves early identification so that educational programs can begin as soon ... syndrome. Other areas of study include the early identification of children with Usher syndrome, treatment strategies such ...

  14. Metabolic Syndrome

    MedlinePLUS

    ... page from the NHLBI on Twitter. What Is Metabolic Syndrome? Metabolic (met-ah-BOL-ik) syndrome is the ... three metabolic risk factors to be diagnosed with metabolic syndrome. A large waistline. This also is called abdominal ...

  15. Metabolic syndrome

    MedlinePLUS

    Metabolic syndrome is a name for a group of risk factors that occur together and increase the chance ... Metabolic syndrome is becoming very common in the United States. Doctors are not sure whether the syndrome is ...

  16. Bilateral Tessier's 7 Cleft with Maxillary Duplication.

    PubMed

    Chauhan, Dinesh Singh; Guruprasad, Yadavalli

    2015-03-01

    Tessier's 7 cleft or lateral facial cleft are unusual lesions that result from failure of the embryonic mandibular and maxillary process of the first branchial arch to fuse properly and form the corners of the mouth. It may be seen alone or in combination with other anomalies, accompanied by varying degrees of severity. We report an extremely rare case of bilateral Tessier's 7 cleft along with maxillary duplication, macrostomia, bilateral posterior maxillary cleft, and mandibular retrusion in an 18-year-old male patient. PMID:25838682

  17. Second-Side Surgery in Superior Canal Dehiscence Syndrome

    PubMed Central

    Agrawal, Yuri; Minor, Lloyd B.; Schubert, Michael C.; Janky, Kristen L.; Davalos-Bichara, Marcela; Carey, John P.

    2014-01-01

    Objective Bilateral superior canal (SC) dehiscence syndrome poses a challenge because bilateral SC dehiscence (SCD) plugging might be expected to result in oscillopsia and disability. Our aims were as follows: 1) to evaluate which symptoms prompted patients with bilateral SCD syndrome (SCDS) to seek second-side surgery, and 2) to determine the prevalence of disabling imbalance and oscillopsia after bilateral SC plugging. Study Design Prospective observational study. Setting Tertiary referral center. Patients Five patients with bilateral SCDS based on history, audiometric and physiologic testing, and computed tomographic findings. This includes all of our patients who have had second-side plugging surgery to date. Intervention(s) Bilateral sequential middle fossa craniotomy and plugging of SCs. Main Outcome Measure(s) Cochleovestibular symptoms, cervical and ocular vestibular-evoked myogenic potential testing, dizziness handicap inventory, short-form 36 Health Survey, dynamic visual acuity testing. Results The most common symptoms prompting second-side surgery were sound- and pressure-induced vertigo and autophony. Three of the 5 patients reported that symptoms shifted to the contralateral ear immediately after plugging the first side, whereas in 2 patients, contralateral symptoms developed several years after the first SC plugging. Two of 4 patients experienced ongoing oscillopsia after bilateral SCDS surgery; however, all patients reported relief from their SCD symptoms and were glad that they had pursued bilateral surgery. Conclusion In patients with bilateral SCDS, sound- and pressure-induced vertigo most commonly prompted second-side surgery. Despite some degree of oscillopsia after bilateral SCDS surgery, patients were very satisfied with second-side surgery, given their relief from other SCDS symptoms. PMID:22158019

  18. Use of adrenal arterial embolization in severe ACTH-dependent Cushing's syndrome.

    PubMed

    Blunt, S B; Pirmohamed, M; Chatterjee, V K; Burrin, J M; Allison, D J; Joplin, G F

    1989-08-01

    The management of a patient with severe Cushing's syndrome due to ectopic ACTH produced by a medullary carcinoma of the thyroid is described. Initial treatment with maximal adrenolytic medical therapy and two attempts at bilateral adrenal venous infarction had failed to control the disease, and she was at that time unfit for surgery. Subsequent use of bilateral adrenal arterial embolization enabled medical therapy to produce sufficient control of the Cushing's syndrome to allow bilateral adrenalectomy to be performed successfully, with resultant cure of the excessive cortisol production. PMID:2602257

  19. Neu-Laxova syndrome: a case report.

    PubMed

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

    2014-01-01

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

  20. 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

  1. Persistent bilateral proatlantal type II artery

    PubMed Central

    Zarghouni, Mehrzad; Marichal, Daniel

    2013-01-01

    Vascular anomalies and variants are common in patients undergoing imaging studies, and patients with these anomalies are generally asymptomatic. Remnants of fetal carotid-basilar circulation are rarely identified. We report a rare case of persistent type 2 bilateral proatlantal arteries, in which the patient presented with dizziness. PMID:23382615

  2. Persistent bilateral proatlantal type II artery.

    PubMed

    Zarghouni, Mehrzad; Marichal, Daniel

    2013-01-01

    Vascular anomalies and variants are common in patients undergoing imaging studies, and patients with these anomalies are generally asymptomatic. Remnants of fetal carotid-basilar circulation are rarely identified. We report a rare case of persistent type 2 bilateral proatlantal arteries, in which the patient presented with dizziness. PMID:23382615

  3. BILATERAL NEONATAL BREAST ENLARGEMENT ASSOCIATED WITH MASTITIS

    Microsoft Academic Search

    R. D. Wammanda

    A 1- month old baby girl presented with bilateral enlargement of the breast noticed at 2 weeks of life. The grandmother, following their tradition, commenced twice daily massaging of both breasts with warm water. This is to expel the mother's breast milk which they believe the baby have sucked into her own breast. The baby's breast continued to increase in

  4. A bilateral foot drop due to neuroschistosomiasis

    Microsoft Academic Search

    Joyce Wilbers; Albert Idema; Anja Gijtenbeek

    2010-01-01

    Schistosomiasis is a parasitic disease, uncommon in Europe and the USA. We present an unusual case of intracerebral schistosomiasis,\\u000a presenting with a bilateral foot drop. We describe unique magnetic resonance spectroscopy characteristics that can contribute\\u000a to diagnosis and follow up of a neuroschistosomiasis infection.

  5. A bilateral foot drop due to neuroschistosomiasis.

    PubMed

    Wilbers, Joyce; Idema, Albert; Gijtenbeek, Anja

    2010-05-01

    Schistosomiasis is a parasitic disease, uncommon in Europe and the USA. We present an unusual case of intracerebral schistosomiasis, presenting with a bilateral foot drop. We describe unique magnetic resonance spectroscopy characteristics that can contribute to diagnosis and follow up of a neuroschistosomiasis infection. PMID:20127348

  6. Hypersexuality following bilateral thalamic infarction: case report

    Microsoft Academic Search

    Eduardo G. Mutarelli; Antonio M. P. Omuro; Tarso Adoni

    2006-01-01

    Hypersexuality is a rare but well recognized condition following brain injury. It has been described secondarily to dysfunction in the hypothalamus, the temporal and frontal lobes. We re p o rt a 63 y e a r-old man that developed neuropsychological disturbances with hypersexuality as a prominent feature , disinhibition and moderate memory loss, hypersomnia and irritability after a bilateral

  7. Bilateral Facial Paralysis Caused by Bilateral Temporal Bone Fracture: A Case Report and a Literature Review

    PubMed Central

    Eliçora, Sultan ?evik; Dinç, Aykut Erdem; Bi?kin, Sultan; Damar, Murat; Bilgin, Ergin

    2015-01-01

    Bilateral facial paralysis caused by bilateral temporal bone fracture is a rare clinical entity, with seven cases reported in the literature to date. In this paper, we describe a 40-year-old male patient with bilateral facial paralysis and hearing loss that developed after an occupational accident. On physical examination, House-Brackmann (HB) facial paralysis of grade 6 was observed on the right side and HB grade 5 paralysis on the left. Upon temporal bone computed tomography (CT) examination, a fracture line exhibiting transverse progression was observed in both petrous temporal bones. Our patient underwent transmastoid facial decompression surgery of the right ear. The patient refused a left-side operation. Such patients require extensive monitoring in intensive care units because the presence of multiple injuries means that facial functions are often very difficult to evaluate. Therefore, delays may ensue in both diagnosis and treatment of bilateral facial paralysis. PMID:26175920

  8. Metastatic leiomyosarcoma presenting as bilateral, multifocal breast masses.

    PubMed

    Vasan, Neil; Saglam, Ozlen; Killelea, Brigid K

    2012-01-01

    Here we describe a case of metastatic leiomyosarcoma presenting as bilateral, multifocal breast masses. This case represents the convergence of three rare entities: leiomyosarcoma of unknown primary origin, metastases to the breast and bilateral, multicentric breast disease. PMID:23220834

  9. Ehlers-Danlos syndrome and congenital heart anomalies.

    PubMed

    Maeda, T; Suzuki, Y; Haeno, S; Asada, M; Hiramatsu, R; Tanaka, F; Okada, M; Suzuki, T

    1996-03-01

    Two sisters with Ehlers-Danlos syndrome, inherited as an autosomal recessive trait, and congenital heart disease are herein reported. One was a 20-year-old woman with Ehlers-Danlos syndrome and multiple aphthous stomatitis, bronchial asthma, an emphysematous lung, a ventricular septal defect and a bilateral inguinal hernia due to hyperextensibility and joint hypermobility. The other was a 17-year-old girl with the same syndrome and an atrial septal defect, a ventricular septal defect and patent ductus arteriosus. The combination of Ehlers-Danlos syndrome and congenital heart anomalies in these siblings suggest a common genetic defect to be the cause of these diseases. PMID:8785453

  10. Metabolic Syndrome

    MedlinePLUS

    ... you know? One in five Americans has the metabolic syndrome. WhO is At risk fOr the metAbOlic syndrOme? The syndrome runs in families and is more ... High blood pressure Most people who have the metabolic syndrome feel healthy and may not have any signs ...

  11. 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

  12. CDK19 is disrupted in a female patient with bilateral congenital retinal folds, microcephaly and mild mental retardation

    Microsoft Academic Search

    Arijit Mukhopadhyay; Jamie M. Kramer; Gerard Merkx; Dorien Lugtenberg; Dominique F. Smeets; Merel A. W. Oortveld; Ellen A. W. Blokland; Jyoti Agrawal; Annette Schenck; Hans van Bokhoven; Erik Huys; Eric F. Schoenmakers; C. Erik van Nouhuys; Frans P. M. Cremers

    2010-01-01

    Microcephaly, mental retardation and congenital retinal folds along with other systemic features have previously been reported\\u000a as a separate clinical entity. The sporadic nature of the syndrome and lack of clear inheritance patterns pointed to a genetic\\u000a heterogeneity. Here, we report a genetic analysis of a female patient with microcephaly, congenital bilateral falciform retinal\\u000a folds, nystagmus, and mental retardation. Karyotyping

  13. One-stage clipping of bilateral middle cerebral artery aneurysms via the bilateral pterional keyhole approach.

    PubMed

    Maruyama, Keisuke; Kurita, Hiroki; Yamaguchi, Ryuichi; Noguchi, Akio; Shiokawa, Yoshiaki

    2013-01-01

    Five patients aged 55 to 73 years (mean 63 years) underwent one-stage clipping for unruptured aneurysms in the bilateral middle cerebral arteries (mean size 4.5 mm, range 2 to 7 mm) via the bilateral pterional keyhole approach in our institute. Important points are as follows: the head is affixed with no rotation; one side manipulation is started 5 minutes after the other side to avoid conflict of surgical instruments; a 5-cm curvilinear skin incision is made inside the hairline and pterional keyhole craniotomy is made bilaterally using 2 burr holes; the whole operating table is rotated 15 degrees to one side to facilitate the microsurgical trans-sylvian approach and aneurysm clipping; the operating table is rotated to the other side for the contralateral procedure; and particular care is taken to avoid bilateral brain injury. This approach provided minimum but sufficient working space required for trans-sylvian dissection. Aneurysm neck clipping was safely performed in a mean operation time of 5 hours 17 minutes. No complications occurred and satisfactory cosmetic results were obtained in all patients. Postoperative neuroimaging studies exhibited bilateral complete clipping with minimal intracranial air content and minimum consequences of brain retraction. One-stage clipping via the pterional keyhole approach is a safe and effective therapeutic option for small bilateral aneurysms. PMID:23524497

  14. Vestibular Evoked Myogenic Potentials in Patients with Idiopathic Bilateral Vestibulopathy

    Microsoft Academic Search

    Masaki Matsuzaki; Toshihisa Murofushi

    2001-01-01

    Idiopathic bilateral vestibulopathy (IBV) is an acquired bilateral peripheral vestibular disorder of unknown cause. Three patients diagnosed as IBV by neuro-otological examination were reported. They underwent vestibular evoked myogenic potential (VEMP) testing which reflects the functionality of the sacculo-collic pathway. As a result, 2 of the 3 patients showed bilateral absence of VEMPs and one showed unilateral absence. The VEMPs

  15. Cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy

    PubMed Central

    Kahraman, Hasan; Tokur, Mahmut; Sayar, Hamide; Inci, Mehmet Fatih

    2013-01-01

    Cryptogenic organising pneumonia is not considered in the differential diagnosis of bilateral hilar and mediastinal lymphadenopathy. We submitted a patient presenting with bilateral hilar and mediastinal lymphadenopathy. We suspected diagnosis of sarcoidosis, but the patient was diagnosed as cryptogenic organising pneumonia with the histological result. This is the second case report of cryptogenic organising pneumonia presenting with bilateral hilar and mediastinal lymphadenopathy. PMID:23761506

  16. Bilateral robot system on the real-time network structure

    Microsoft Academic Search

    Yutaka Uchimura; Takahiro Yakoh

    2004-01-01

    This paper presents a bilateral robot system, which is driven by the static friction-free drive system and implemented on the real-time network structure. The goal is to realize a force reflecting bilateral teleoperation with haptic impression transmission over computer networks. The paper considers two subjects relating to the bilateral robot. The first is static friction, which degrades the performance of

  17. Massive bilateral nephroblastomatosis in a 13-year-old girl

    Microsoft Academic Search

    E. Pichler; O. A. Jfirgenssen; E. Balzar; W. F. Pinggera; A. Wolf; O. Wagner; G. Reinartz; H. Czembirek; G. Syré

    1982-01-01

    A 13-year-old girl on chronic hemodialysis with renal failure thought to be due to polycystic renal disease, underwent bilateral nephrectomy as a pretransplant procedure. Microscopic examination of the grossly enlarged nodular kidneys revealed a bilateral diffuse tumor infiltration which was not Wilms tumor. Eventually the diagnosis of bilateral nephroblastomatosis was established. This is apparently unique at this age without coexistent

  18. 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 its position in the window as well as the pixel value for the central pixel of the window. The absolute difference between these two pixel values is calculated and used as an address in a lookup table. Each processing element has a lookup table, unique for its position in the window, containing the weight coefficients for the Gaussian function for that position. The pixel value is multiplied by the weight, and the outputs of the processing element are the weight and pixel-value weight product. The products and weights are fed to the adder tree. The sum of the products and the sum of the weights are fed to the divider, which computes the sum of products the sum of weights. The output of the divider is denoted the bilateral smoothed image. The smoothing function is a simple weighted average computed over a 3 3 subwindow centered in the 9 9 window. After smoothing, the image is delayed by an additional amount of time needed to match the processing time for computing the bilateral smoothed image. The bilateral smoothed image is then subtracted from the 3 3 smoothed image to produce the final output. The prototype filter as implemented in a commercially available FPGA processes one pixel per clock cycle. Operation at a clock speed of 66 MHz has been demonstrated, and results of a static timing analysis have been interpreted as suggesting that the clock speed could be increased to as much as 100 MHz.

  19. Wall-eyed bilateral internuclear ophthalmoplegia: review of pathogenesis, diagnosis, prognosis and management.

    PubMed

    Wu, Yu-Tai; Cafiero-Chin, Malinda; Marques, Cathy

    2015-01-01

    Wall-eyed bilateral internuclear ophthalmoplegia (WEBINO) is an uncommon disorder of ocular motility that possesses a unique spectrum of clinical findings, consisting of primary gaze exotropia, adduction impairment and nystagmus of the abducting eye. WEBINO is a variant of internuclear ophthalmoplegia (INO) sharing similar pathophysiology and aetiologies. Much of the literature published on internuclear ophthalmoplegia and its variants focuses on aetiology and pathophysiology, whereas there has been less information addressing prognosis and management. This review will provide current perspectives on the pathogenesis, prognosis and management of WEBINO syndrome. PMID:25308307

  20. Systematized inflammatory epidermal nevus with symmetrical involvement: An unusual case of CHILD syndrome?

    Microsoft Academic Search

    Regina Fink-Puches; Hans Peter Soyer; Gerhard Pierer; Helmut Kerl; Rudolf Happle

    1997-01-01

    The CHILD syndrome ( c ongenital h emidysplasia with i chthyosiform nevus and l imb d efects) is usually characterized by lateralization of all associated anomalies. It has been assumed that the event of X-inactivation coincides and interferes with a clone of organizer cells controlling a large developmental field. A 16-year-old girl with bilateral manifestations of CHILD syndrome is described.

  1. Connexin 26 (GJB2) mutations as a cause of the KID syndrome with hearing loss

    Microsoft Academic Search

    Alessandro Terrinoni; Andrea Codispoti; Valeria Serra; Ernesto Bruno; Biagio Didona; Mauro Paradisi; Steven Nisticň; Elena Campione; Bianca Napolitano; Laura Diluvio; Gerry Melino

    2010-01-01

    KID syndrome (MIM 148210) is an ectodermal dysplasia characterized by the occurrence of localized erythematous scaly skin lesions, keratitis and severe bilateral sensorineural deafness. KID syndrome is inherited as an autosomic dominant disease, due to mutations in the gene encoding gap junction protein GJB2 (connexin 26, Cx26). Cx26 is a component of gap junction channels in the epidermis and in

  2. Surgical management of the adrenal glands in the multiple endocrine neoplasia type II syndrome

    Microsoft Academic Search

    Jon A. van Heerden; Glen W. Sizemore; J. Aidan Carney; Clive S. Grant; William H. ReMine; Sheldon G. Sheps

    1984-01-01

    During a 30-year period (1951–1981), seventeen patients underwent bilateral adrenalectomy for established adrenal medullary disease with catecholamine excess. Fourteen patients had the MEN IIa syndrome and 3 had the MEN IIb syndrome. There was no major operative morbidity and no operative mortality.

  3. Efficacy and tolerability outcomes after punctal occlusion with silicone plugs in dry eye syndrome

    Microsoft Academic Search

    Mini Balaram; Debra A Schaumberg; M. Reza Dana

    2001-01-01

    PURPOSE: Silicone punctal plugs are widely used to conserve moisture in tear-deficient eyes. We studied the efficacy and tolerability of punctal plugs in symptom reduction and improvement of ocular surface disease parameters in dry eye syndrome.METHODS: We reviewed the records of 50 consecutive patients with dry eye syndrome who were symptomatic despite maximally tolerable lubricant therapy and had bilateral lower

  4. Bilateral Psoas Abscess in the Emergency Department

    PubMed Central

    Tomich, Eric B.; Della-Giustina, David

    2009-01-01

    We present the case of a 45-year-old female who presented multiple times to the emergency department with acute low back pain and was subsequently diagnosed with bilateral psoas muscle abscess. Psoas abscess is an uncommon cause of acute low back pain that is associated with high morbidity and mortality. The onset of symptoms is frequently insidious and the clinical presentation vague. Proper diagnosis requires vigilance of the physician to recognize signs in the history and physical examination that are suggestive of a potentially serious spinal condition and initiate further workup. While most patients with acute low back pain have a benign etiology, this case report demonstrates the challenge of diagnosing a patient with bilateral psoas abscess who had few known risk factors and symptoms typical of mechanical low back pain. PMID:20046252

  5. Bilateral psoas abscess in the emergency department.

    PubMed

    Tomich, Eric B; Della-Giustina, David

    2009-11-01

    We present the case of a 45-year-old female who presented multiple times to the emergency department with acute low back pain and was subsequently diagnosed with bilateral psoas muscle abscess. Psoas abscess is an uncommon cause of acute low back pain that is associated with high morbidity and mortality. The onset of symptoms is frequently insidious and the clinical presentation vague. Proper diagnosis requires vigilance of the physician to recognize signs in the history and physical examination that are suggestive of a potentially serious spinal condition and initiate further workup. While most patients with acute low back pain have a benign etiology, this case report demonstrates the challenge of diagnosing a patient with bilateral psoas abscess who had few known risk factors and symptoms typical of mechanical low back pain. PMID:20046252

  6. Bilateral calcaneal stress fractures in two cats.

    PubMed

    Cantatore, M; Clements, D N

    2015-06-01

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

  7. Bilateral Nocardia Keratitis after Photorefractive Keratectomy

    PubMed Central

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

    2012-01-01

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

  8. Morphoimmunohistochemical characteristics of synchronous bilateral breast cancer.

    PubMed

    Todorov, S S; Kit, O I; Bosenko, E S; Kas'yanenko, V N; Lushnikova, E L; Nepomnyashchikh, L M

    2014-09-01

    We performed a complex morphoimmunohistochemical study of synchronous bilateral breast cancer (diagnosed in 0.69% cases in women of the postproductive period after the age of 50). The molecular genetic characteristics of this cancer were detected and the algorithm of diagnostic studies was suggested. We have demonstrated that bilateral breast cancer was in 75% cases estrogen- and progesterone-positive, but androgen-negative. The HER-2/neu status of the tumor was negative in 75% cases, but expression of HER-2/neu was detected in the tumor of one mammary gland in 20% cases. Membrane expression of E-cadherin in infiltrative ductal cancer cells significantly decreased up to its complete disappearance with the decrease in tumor cell differentiation degree. The data provide new insight into prognostic value of the studied biomarkers and help to develop treatment protocol with consideration for the molecular biology of this group of tumors. PMID:25257431

  9. Bilateral breast masses with a rare etiology.

    PubMed

    Thieringer, Friederike; Sartorius, Gideon; Kalf, Katrin; Heinzelmann, Viola; Vetter, Marcus

    2013-01-01

    Breast masses have a variety of benign and malignant etiologies. We present the case of a 28-year-old woman with bilateral large painful breast masses that developed rapidly in the three weeks before first presentation. Further investigation revealed bilateral ovarian masses. Biopsies of both ovarian masses were taken, and the pathology reported Burkitt's lymphoma. Additional staging with a PET scan was suggestive of bone marrow involvement, but bone marrow biopsy was negative. Examination of the cerebrospinal fluid did not identify malignant cells. The patient underwent CODOX-M/IVAC chemotherapy, and a complete response was demonstrated after one cycle of treatment. Six months after finishing chemotherapy the patient remained in complete remission. To our knowledge this is the first case reporting simultaneous involvement of breast, ovaries, and bones in Burkitt's lymphoma. Gynecologists and oncologists should be aware of this pattern. Polychemotherapy treatment must be initiated rapidly with curative intent. PMID:24066248

  10. Bilateral brachial plexus compressive neuropathy (crutch palsy).

    PubMed

    Raikin, S; Froimson, M I

    1997-01-01

    Brachial plexus compressive neuropathy following the use of axillary crutches (crutch palsy) is a rare but well-recognized entity. Most reported cases involve the posterior cord of the brachial plexus in children and have resolved spontaneously within 8-12 weeks. We recently treated a 36-year-old man who was using axillary crutches for mobilization after a supracondylar femoral fracture. Bilateral posterior cord (predominantly radial nerve) compressive neuropathy subsequently developed, with lesser involvement of the ulnar and median nerves. The patient had little to no improvement clinically 8 weeks after the estimated onset of the palsy, and an electromyogram at that time confirmed the presence of a severe axonotmesis lesion of the radial, median, and ulnar nerves bilaterally. The patient was treated with static cock-up wrist splinting and discontinuation of the axillary crutches. Return of sensory and motor function was delayed but occurred within 9 months. PMID:9057152

  11. Bilateral Wallerian Degeneration of the Pontocerebellar Tracts

    PubMed Central

    Hekimoglu, Azad; Suer Dogan, Ihsaniye; Turan, Aynur; Oztekin, Mehmet Fevzi; Hekimoglu, Baki

    2015-01-01

    Wallerian degeneration is the process of progressive demyelination and disintegration of the distal axonal segment following the transection of the axon or damage to the neuron. We report a case of a patient with Wallerian degeneration of the pontocerebellar tracts. She had a history of a pontine infarction 3 months ago. Wallerian degeneration of pontocerebellar tracts is seen bilaterally and symmetrically and is more visible in the middle cerebellar peduncles. Along the middle cerebellar peduncles hyperintense signal was detected on T2 weighted images. Wallerian degeneration of pontocerebellar tracts is a rare entity. It can occur bilaterally after a large pontine infarction. Magnetic resonance imaging seems to be the most effective technique for detection of Wallerian degeneration. In this report we want to mention this rare entity and to prevent wrong diagnosis. PMID:26171260

  12. Bilateral adrenal infarction in Crohn's disease

    PubMed Central

    Khandelwal, Ashish; Krishna, J. Sateesh; Khandelwal, Kanika; Virmani, Vivek; Ryan, John

    2013-01-01

    Adrenal infarction is an uncommon cause of adrenal insufficiency. We herein present unique occurrence of bilateral adrenal infarction detected on imaging in a young female with known history of Crohn's disease. The patient responded well to steroids and is on follow up. To our knowledge, this is the first case reported in English literature of adrenal infarct associated with Crohn's disease as extraintestinal manifestation. PMID:24083186

  13. Simultaneous Bilateral Anterior Approach Total Hip Arthroplasty.

    PubMed

    Gondusky, Joseph S; Pinkos, Kevin A; Choi, Leera; Patel, Jay J; Barnett, Steven; Gorab, Robert S

    2015-07-01

    Simultaneous bilateral total hip arthroplasty (THA) has been performed successfully, with good outcomes and low complication rates reported. Most published studies on the topic used anterolateral or posterior surgical approaches. The anterior approach is performed under live fluoroscopy with the patient supine, obviating the need for patient repositioning during bilateral surgery. The authors report their experience with simultaneous bilateral anterior approach total hip arthroplasty. The authors retrospectively reviewed data for 75 patients (150 hips). Mean follow-up was 26 months (range, 5-60 months). Mean patient age was 59 years and the majority were American Society of Anesthesiology class 2 (range, 1-3). Mean total surgical time was 144 minutes (72 minutes per hip). Mean blood loss was 565 mL. Mean hospital length of stay was 2.75 days (range, 1-4 days). Ninety-six percent of patients were able to ambulate on postoperative day 1. Sixty-eight percent of patients were discharged to home. Mean Harris Hip Score improved from 50 to 97. All patients noted a return to preoperative level of activity or higher. Complications included 1 atraumatic minimally displaced trochanteric fracture occurring 2 weeks postoperatively, 1 pulmonary embolism on postoperative day 3 treated without sequelae, 1 episode of postoperative atrial fibrillation, and 4 minor local wound complications. No readmission, infection, nerve palsy, dislocation, reoperation, or death occurred. Anterior approach THA has the advantage of a single supine position for bilateral simultaneous surgery and the current study supports its use in appropriate patients. [Orthopedics. 2015; 38(7):e611-e615.]. PMID:26186324

  14. Bilateral hearing loss following a retrobulbar block

    Microsoft Academic Search

    Ronald B. George; Jason Hackett

    2005-01-01

    Purpose  Regional anesthesia is the most commonly used ophthalmological anesthetic technique in Canada and the United States. Brainstem\\u000a anesthesia is not an uncommon complication of retrobulbar blocks. Anesthesiologists are a prominent element in the ophthalmology\\u000a suite, in part due to the complications possible with regional anesthesia. This is the first reported case of complete bilateral\\u000a hearing loss following a retrobulbar block.

  15. Tuberose sclerosis and bilateral renal carcinoma.

    PubMed

    Honey, R J; Honey, R M

    1977-11-01

    A case report is presented of an 18-year-old girl of normal intellect with tuberose sclerosis and multiple bilateral renal carcinomas. These tumours were successfully treated surgically by right nephrectomy and left partial nephrectomy. The usual renal lesion in these patients is the angiomyolipoma, which is a hamartoma. Renal carcinoma associated with tuberose sclerosis is extremely rare, and the mode of behaviour of these tumours may be unusual. PMID:588942

  16. Bilateral Pregnancy Luteoma: A Case Report

    PubMed Central

    Nanda, Annu; Gokhale, Uday A.; Pillai, G Rajasekharan

    2014-01-01

    Pregnancy luteoma is a non-neoplastic lesion of the ovary occurring during pregnancy and is usually discovered incidentally at the time of a cesarean section or during postpartum tubal ligation. An accurate diagnosis is important for the mother and the fetus as it can be confused with ovarian malignancy leading to unnecessary surgery. We report a case of a pregnant female who was discovered to have bilateral enlarged ovaries at the time of emergency cesarean section. PMID:25337317

  17. Multilateral, regional and bilateral energy trade governance

    SciTech Connect

    Leal-Arcas, Rafael; Grasso, Costantino; Rios, Juan Alemany (Queen Mary Univ. of London (United Kingdom))

    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.

  18. Lumbar discal cyst causing bilateral radiculopathy

    PubMed Central

    Hyung-Jun, Kwak; Dae-Yong, Kim; Tae-Ho, Kim; Ho-Sang, Park; Jae-Sung, Kim; Jae-Won, Jang; Jung-Kil, Lee

    2011-01-01

    Background: Discal cyst is a rare lesion that can result in clinical symptoms typical of disc herniation manifesting as a unilateral single nerve root lesion. To the best of the authors’ knowledge, this is the first reported case of discal cyst resulting in bilateral radiculopathy. Case Description: A 48-year-old female presented with bilateral sciatica and neurogenic claudication for 3 months. Magnetic resonance imaging revealed an extradural cystic lesion compressing the ventral aspect of the thecal sac at the level of the L3-L4 intervertebral disc. The lesion showed low and high signal intensities on T1- and T2-weighted images, respectively. Total excision of the cyst was achieved after a left hemipartial laminectomy of L3, and an obvious communication with the disc space was found. Bilateral sciatica was immediately resolved after surgery, and was sustained at the two-year follow-up. The histological diagnosis was consistent with a discal cyst. Conclusions: Although a discal cyst is extremely rare, the possibility of a discal cyst should be considered in differential diagnosis of patients with radiculopathy, particularly when encountering any extradural mass lesion ventral to the thecal sac. Surgical resection is the most employed therapeutic method for symptomatic lumbar discal cysts. PMID:21427789

  19. 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.

  20. [Clinical features of bilateral trigeminal neuralgia].

    PubMed

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

    2015-02-01

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

  1. 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. PMID:25013154

  2. Cat eye syndrome.

    PubMed

    Sharma, Deepak; Murki, Srinivas; Pratap, Tejo; Vasikarla, Madhavi

    2014-01-01

    A full-term female baby, a product of non-consanguineous marriage, was born at 37?weeks of gestation with a birth weight of 2.08?kg. Antenatal scan at 31?weeks revealed complex congenital heart disease with a hypoplastic right ventricle, pulmonary atresia and an intact septum. Immediately after birth, the infant was shifted to the nursery and was started on intravenous fluids and infusion prostaglandin E1 (Alprostidil). On examination, she had microcephaly, periorbital puffiness, a long philtrum, a broad nasal bridge and retrognathia, up slanting palpebral fissures, widely spaced nipples, a sacral dimple and right upper limb postaxial polydactyly. Postnatal echocardiography confirmed a large ostium secundum atrial septal defect with left to right shunt, right ventricle hypoplasia, pulmonary atresia with an intact septum and a large vertical patent ductus arteriosus. Ophthalmological examination showed a bilateral chorioretinal coloboma sparing disc and fovea. Karyotyping showed an extra small marker chromosome suggestive of the Cat eye syndrome. PMID:24842361

  3. Bilateral symmetrical supernumerary heads of biceps brachii with rare pectoralis major insertion.

    PubMed

    Fraser, Patrick Richard; Howard, Levi William; Rosales, Armando Aviles; Guttmann, Geoffrey David

    2015-04-01

    During a routine dissection of a 51-year-old Caucasian male cadaver, bilateral symmetrical supernumerary heads (SH) of the biceps brachii muscles with insertion into the pectoralis major (PM) muscles were discovered. Multiple case reports have documented supernumerary heads for the biceps brachii; however, none have shown bilateral insertion into the pectoralis major. This study describes a previously undocumented variation of the SH that has potential for clinical impact. Healthcare providers could be confounded by patients presenting with shoulder pain or muscle tears as a result of the anomaly. Furthermore, MRI studies on patients with possible shoulder muscle tears could reveal unexpected results. Such cases would warrant consideration of SH anomaly and treatment should be adjusted accordingly. The significant bulk and angle of the SH insertion on the PM we observed changes force vectors which would have an unknown effect in performance, surgical interventions and pain syndromes. A second biceps brachii anomaly was observed on the left brachium in addition to the bilateral SH. We postulate that these variants provide the potential for clinical complications regarding muscular injury to these aforementioned muscle groups. PMID:24817560

  4. Learning about Down Syndrome

    MedlinePLUS

    ... genetic terms used on this page Learning About Down Syndrome What is Down syndrome? What are the symptoms ... syndrome Additional Resources for Down Syndrome What is Down syndrome? Down syndrome is a chromosomal condition related to ...

  5. Bilateral atticoantral chronic suppurative otitis media presenting as bilateral cutaneous mastoid fistulas.

    PubMed

    Bhat, Kemmannu Vikram; Udayashankar, Shankarappa Gangadharaiah; Venkatesha, Belur Keshavamurthy; Kumar, Praveen

    2009-10-01

    Postauricular cutaneous mastoid fistula secondary to chronic suppurative otitis media (CSOM) is an unusual complication. Bilateral manifestation along with primary acquired cholesteatoma and atelectasis of the pars tensa as an end-stage complication in the natural course of atticoantral CSOM is rare. This kind of complication has a very morbid effect on the ear, and it poses a therapeutic challenge in terms of eradicating disease and restoring function. In this article, we describe the unusual course of an atticoantral CSOM that (1) began as a primary acquired cholesteatoma simultaneously in both ears, (2) proceeded to automastoidectomy and a severe mixed hearing loss bilaterally, and (3) ended with the development of bilateral cutaneous mastoid fistulas that served to arrest the further progression of the disease process on its own. This case serves as a good demonstration of how a ventilating mastoid fistula can change the natural course of atticoantral CSOM and abort the occurrence of deadly complications. PMID:19826984

  6. Premenstrual syndrome

    MedlinePLUS

    Brown I, O'Brien PMS, Marjoribanks I, Wyatt K. Selective serotonin reuptake inhibitors for premenstrual syndrome. Cochrane Database Syst Rev. 2009;2:CD001396. Lentz GM. Primary and secondary dysmenorrhea, premenstrual syndrome, and ...

  7. Tourette Syndrome

    MedlinePLUS

    If you have Tourette syndrome, you make unusual movements or sounds, called tics. You have little or no control over them. Common tics ... words, spin, or, rarely, blurt out swear words. Tourette syndrome is a disorder of the nervous system. ...

  8. Rett Syndrome

    MedlinePLUS

    ... is Rett Syndrome? Rett syndrome is a childhood neurodevelopmental disorder that affects females almost exclusively. The child ... antiepileptic drugs may be used to control seizures. Occupational therapy, physiotherapy, and hydrotherapy may prolong mobility. Some children ...

  9. 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 ...

  10. Klinefelter syndrome

    MedlinePLUS

    47 X-X-Y syndrome ... have two XX chromosomes. Boys normally have an X and a Y chromosome. Klinefelter syndrome is when ... boy is born with at least one extra X chromosome. Usually, this occurs due to one extra ...

  11. Asperger Syndrome

    MedlinePLUS

    ... have certain genetic or chromosomal conditions, such as fragile X syndrome  or tuberous sclerosis . 11-14 When taken ... and autistic behavior in children and adolescents with fragile X syndrome. Am J Ment Retard. 2008; 113(1): 44- ...

  12. Hunter syndrome

    MedlinePLUS

    ... Mild to no mental deficiency Both forms: Carpal tunnel syndrome Coarse features of the face Deafness (gets ... Airway obstruction Carpal tunnel syndrome Hearing loss that gets worse over time Loss of ability to complete daily living activities Joint stiffness that ...

  13. Goodpasture syndrome

    MedlinePLUS

    Goodpasture syndrome is a rare disease that can involve quickly worsening kidney failure and lung disease. Some ... Goodpasture syndrome is an autoimmune disorder . It occurs when the immune system mistakenly attacks and destroys healthy ...

  14. Joubert Syndrome

    MedlinePLUS

    ... sponsored a symposium on Joubert syndrome in 2002. Research priorities for the disorder were outlined at this meeting. NIH Patient Recruitment for Joubert Syndrome Clinical Trials At NIH Clinical Center Throughout the U.S. ...

  15. Ascher's syndrome: A rare case report

    PubMed Central

    Chandravanshi, Shivcharan Lal; Mishra, Vinay

    2015-01-01

    An 18-year-old Indian girl with upper lip deformity presented with on and off painless swelling of her both upper eyelids for 3 years. Clinical evaluation revealed bilateral blepharochalasis, narrowing of horizontal palpebral fissure, decreased outer intercanthal distance, iris coloboma, cleft soft palate, bifid uvula, sensorineural deafness and double upper lip. Clinical examination of the thyroid, thyroid hormone assay and ultrasonography revealed normal thyroid gland structure and function. Ascher's syndrome was diagnosed. To our knowledge, this is the first reported case of Ascher's syndrome associated with iris coloboma, heterochromia iridum, and narrowing of horizontal palpebral fissure and decreased outer intercanthal distance secondary to lengthening of lateral canthal ligament. PMID:25971175

  16. A new case of TEMPI syndrome

    PubMed Central

    Mohammadi, Farnaz; Wolverson, Michael K.; Bastani, Bahar

    2012-01-01

    We present an interesting case of a woman with new onset hypertension and abdominal fullness who was found to have huge bilateral perinephric fluid collections. Extensive workup revealed that she had secondary polycythemia, extensive truncal and proximal extremities telangiectasia and IgA-lambda monoclonal gammopathy of underdetermined significance. We believe that this is one of the rare cases consistent with the recently described TEMPI syndrome.

  17. Ocular findings in arteriohepatic dysplasia (Alagille's syndrome).

    PubMed

    Romanchuk, K G; Judisch, G F; LaBrecque, D R

    1981-04-01

    The ocular findings in a father and two offspring with arteriohepatic dysplasia are reported in this paper. All three people had bilateral posterior embryotoxon, Axenfeld's anomaly and a pigmentary retinopathy. Other ocular findings were exotropia, an ectopic pupil, band keratopathy, choroidal folds, anomalous optic discs and infantile myopia. Persons with arteriohepatic dysplasia typically present with prolonged neonatal jaundice. Cognizance of the associated eye findings helps distinguish this syndrome from other types of familial intrahepatic cholestasis. PMID:6796244

  18. Adrenocortical Overexpression of Gastric Inhibitory Polypeptide Receptor Underlies Food-Dependent Cushing's Syndrome

    Microsoft Academic Search

    JOHANNE TREMBLAY; PAVEL HAMET; WOUTER W. DE HERDER

    2010-01-01

    Abnormal responsiveness of adrenocortical cells to gastric inhib- itory polypeptide (GIP) in food-dependent Cushing's syndrome sug- gested that adrenal expression of ectopic, overexpressed, or mutated GIP receptor (GIPR) underlies this syndrome. The expression of GIPR was studied by RT-PCR in human adrenal tissues from two patients with GIP-dependent Cushing's syndrome (adenoma, bilateral hyper- plasia), five fetal or adult controls, one

  19. Globus Pallidus Interna Deep Brain Stimulation in a Patient with Medically Intractable Meige Syndrome

    PubMed Central

    Bae, Dae-Woong; Son, Byung-chul; Kim, Joong-Seok

    2014-01-01

    Medical therapies in patients with Meige syndrome, including botulinum toxin injection, have been limited because of incomplete response or adverse side effects. We evaluated a patient with Meige syndrome who was successfully treated with deep brain stimulation (DBS) in the globus pallidus interna (GPi). This case report and other previous reports suggest that bilateral GPi DBS may be an effective treatment for medically refractory Meige syndrome, without significant adverse effects. PMID:25360233

  20. Metabolic syndrome.

    PubMed

    Cho, L W

    2011-11-01

    Metabolic syndrome is a clustering of different risk factors that collectively increases the risk of developing cardiovascular disease and type 2 diabetes mellitus. The syndrome itself is associated with various metabolic abnormalities, including insulin resistance, non-alcoholic fatty liver disease, obstructive sleep apnoea, male hypogonadism and polycystic ovary syndrome. This review aims to discuss recent developments related to the syndrome, including the associated metabolic complications and goals for therapeutic strategies. PMID:22173246

  1. Fulminant crural compartment syndrome preceded by psychogenic polydipsia.

    PubMed

    Ulstrup, Anton; Ugleholdt, Randi; Rasmussen, Jeppe Vejlgaard

    2015-01-01

    We report a case of bilateral anterolateral crural compartment syndrome elicited by hyponatraemia and psychogenic polydipsia. The unusual constellation of clinical findings and diminished pain expression made initial diagnostic procedures challenging. The possible pathogenesis and treatment options are discussed. Impairment of lower extremity function at follow-up was serious and permanent. PMID:25976194

  2. 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 5˝-year-old child. PMID:22169837

  3. Aase syndrome

    MedlinePLUS

    Aase-Smith syndrome; Hypoplastic anemia/Triphalangeal thumb syndrome ... Jones KL, ed. Aase syndrome. In: Smith's Recognizable Patterns Of Human Malformation. 6th ed. Saunders. 2005. Clinton C, Gazda HT. Diamond-Blackfan Anemia. 2009 Jun 25 [Updated 2013 Jul ...

  4. 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…

  5. 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. ...

  6. Mechanism of highly synchronized bilateral hippocampal activity.

    PubMed

    Wang, Y; Toprani, S; Tang, Y; Vrabec, T; Durand, D M

    2014-01-01

    In vivo studies of epileptiform discharges in the hippocampi of rodents have shown that bilateral seizure activity can sometimes be synchronized with very small delays (<2 ms). This observed small time delay of epileptiform activity between the left and right CA3 regions is unexpected given the physiological propagation time across the hemispheres (>6 ms). The goal of this study is to determine the mechanisms of this tight synchronization with in-vitro electrophysiology techniques and computer simulations. The hypothesis of a common source was first eliminated by using an in-vitro preparation containing both hippocampi with a functional ventral hippocampal commissure (VHC) and no other tissue. Next, the hypothesis that a noisy baseline could mask the underlying synchronous activity between the two hemispheres was ruled out by low noise in-vivo recordings and computer simulation of the noisy environment. Then we built a novel bilateral CA3 model to test the hypothesis that the phenomenon of very small left-to-right propagation delay of seizure activity is a product of epileptic cell network dynamics. We found that the commissural tract connectivity could decrease the delay between seizure events recorded from two sides while the activity propagated longitudinally along the CA3 layer thereby yielding delays much smaller than the propagation time between the two sides. The modeling results indicate that both recurrent and feedforward inhibition were required for shortening the bilateral propagation delay and depended critically on the length of the commissural fiber tract as well as the number of cells involved in seizure generation. These combined modeling/experimental studies indicate that it is possible to explain near perfect synchronization between the two hemispheres by taking into account the structure of the hippocampal network. PMID:24262205

  7. [Cortical deafness following bilateral hypertensive putaminal hemorrhage].

    PubMed

    Nakayama, T; Nobuoka, H; Wada, S; Matsukado, Y

    1986-06-01

    A case of cortical deafness is reported, which was caused by two episodes of bilateral hypertensive putaminal hemorrhage. Such a case has not appeared in literature up to this time. The patient was a right-handed, 69-year-old man, admitted to us after the stroke with right hemiparesis and disturbance of consciousness. CT scans showed right putaminal hemorrhage. After recovery of consciousness, the patient complained of hearing difficulty of the right ear. The right hemiparesis improved and he was followed ambulatorily after two months hospitalization. He returned to his work and was able to drive a car. His hearing difficulty of the right ear progressed and became stone deaf six months after onset of the initial stroke. Two years after the first stroke, he was attacked by the second stroke with left hemiparesis. CT scans showed left putaminal hemorrhage larger than the previous one in the right. When the patient recovered consciousness, he complained of total deafness. Audiometry test showed that hearing was lost completely to both high and low notes, and there was absence of bone conduction bilaterally. Auditory brain stem response test revealed normal pattern from wave I through wave V bilaterally. Middle latency response showed normal component Po on the right ear and it was absent on the left. The component Pa was not identified on both ears. He showed skill and reliance on lip reading two years after the onset of the complete deafness. Now he can speak fluently and communicate with lip reading and written language, although no improvement of the complete deafness being noticed.(ABSTRACT TRUNCATED AT 250 WORDS) PMID:3730198

  8. Multifocality and Bilaterality of Papillary Thyroid Microcarcinoma

    PubMed Central

    So, Yoon Kyoung; Kim, Myung Woo

    2015-01-01

    Objectives Papillary thyroid carcinomas frequently occur as two or more separate foci within the thyroid gland (18%-87%). However, those multifocal tumors are easy to be undetected by preoperative radiologic evaluations, which lead to remnant disease after initial surgery. We aimed to study the incidence of multifocal papillary thyroid microcarcinomas (PTMCs), diagnostic accuracy of preoperative radiologic evaluation, predictive factors, and the chance of bilateral tumors. Methods Two hundred and seventy-seven patients with PTMC were included in this study. All patients underwent total thyroidectomy as an initial treatment. Medical records, pathologic reports, and radiological reports were reviewed for analysis. Results Multifocal PTMCs were detected in 100 of 277 patients (36.1%). The mean number of tumors in each patient was 1.6±1.1, ranging from 1 to 10. The additional tumor foci were significantly smaller (0.32±0.18 cm) than the primary tumors (0.63±0.22 cm) (P<0.001). There was no significant relationship between primary tumor size and the presence of contralateral tumors. With more tumors detected in one lobe, there was greater chance of contralateral tumors; 18.8% with single tumor focus, 30.2% with 2 tumor foci, and 46.2% with 3 or more tumor foci in one lobe. Sensitivity of preoperative sonography was 42.7% for multifocal tumors and 49.0% for bilateral tumors. With multivariate analysis, nodular hyperplasia was the only significant factor for multifocal tumors. Conclusion In cases of PTMCs, the incidence of multifocal tumors is high. However, additional tumor foci are too small to be diagnosed preoperatively, especially under the recent guidelines on radiologic screening tests for papillary thyroid carcinoma. Multifocal PTMCs have high risk of bilateral tumors, necessitating more extensive surgery or more thorough follow-up.

  9. [Ocular anomalies in Alagille's syndrome].

    PubMed

    Ricci, B; Lepore, D; Iossa, M; Santo, A; Chiaretti, A

    1991-01-01

    The authors examine a family, in which two brothers were affected by a severe expression of arteriohepatic dysplasia (ADH, Alagille syndrome), an autosomal dominant disorder associated with intrahepatic cholestasis, characteristic facial appearance, congenital embryotoxon. One of these two cases presented a keratoconus and both had retinal pigmentary degeneration with pigment clumping. The father showed a benign expression of ADH, including the characteristic facial appearance and posterior embryotoxon. Another brother presented only retinal pigmentary abnormalities and a bilateral arcus senilis-like corneal opacity, without any other clinical sign of ADH. The presence of posterior embryotoxon in all the cases of Alagille syndrome confirms that this sign is a hallmark of ADH, also in its benign expression. PMID:1779119

  10. Bilateral synchronous rupture of the quadriceps tendon

    Microsoft Academic Search

    P. Ellanti; N. Davarinos; S. Morris; J. Rice

    Background  Bilateral simultaneous rupture of the quadriceps tendon is a rare entity. They are often associated with degenerative changes\\u000a of the tendons and predisposing conditions such as diabetes or excessive steroid use. They most commonly tend to occur in\\u000a patients of 40 years of age or older.\\u000a \\u000a \\u000a \\u000a \\u000a Materials and methods  We describe a case of a 67-year-old man with simultaneous rupture of both

  11. Bilateral discoid medial menisci: a rare phenomenon.

    PubMed

    Samal, Puspak; Bhagwat, Kishan; Panigrahi, Tapas; Gopinathan, Nirmalraj

    2014-09-01

    Discoid medial meniscus is a relatively rare pathology of the knee joint, with bilateral cases even rarer. Herein, we report the case of a 25-year-old man diagnosed with discoid medial meniscus in the right knee with a horizontal tear. Increased cupping of the medial condyle of the tibia, widening of the medial joint space and the presence of discoid meniscus in the right knee prompted investigation of the asymptomatic left knee with magnetic resonance imaging. The contralateral asymptomatic knee also showed evidence of discoid medial meniscus. The symptomatic knee was successfully treated by arthroscopic partial meniscectomy, with excellent functional outcome. PMID:25273941

  12. Bilateral temporal bone xanthoma. Case report.

    PubMed

    Muthusamy, Kalai A; Azmi, Khairul; Narayanan, Prepageran; Rajagopalan, Raman; Rahman, Nazarina Abdul; Waran, Vicknes

    2008-02-01

    Xanthoma formation is frequently seen over the subcutaneous tissue of extensor surfaces and tendons that have received minor trauma or friction in patients with hypercholesterolemia. However, temporal bone xanthomas with intracranial extension are uncommon. To the best of the authors' knowledge, this is the second report in the literature in which bilateral extension of a xanthoma is described. Xanthomas of the temporal bone are benign lesions, and complete or even partial removal is effective. The predisposing cause of the lesion should also be treated. PMID:18240935

  13. Urgent Bilateral Endoscopic Marsupialization for Respiratory Distress due to Bilateral Dacryocystitis in a Newborn

    PubMed Central

    Kim, Hochang; Park, Jongyeop; Jang, Jaeho; Chun, Junwoo

    2014-01-01

    Abstract We describe an infant with respiratory distress due to bilateral dacryocystoceles and dacryocystitis who was successfully treated with urgent bilateral endoscopic marsupialization. A male infant was brought to our outpatient department 7 days after birth, with red, acutely inflamed swellings near the medial canthal area of both eyes. From birth, there had been bluish swelling near the medial canthal area, and redness and swellings developed within 3 days. On physical examination, the child was afebrile but showed respiratory distress with coarse breathing sound. That day, the infant was admitted and treated with intravenous cefotaxime 150 mg. After withholding oral intake for appropriate preoperative fasting, urgent bilateral probing with endoscopy was done. On endoscopy, huge bilateral congenital dacryocystoceles were found. Because of its huge size, the inferior surface of the cyst was touching the nasal floor, which made probe unable to perforate the wall of dacryocystocele. Therefore, an endoscopy-assisted marsupialization of dacryocystoceles and bicanalicular silicone intubation were performed. Both swellings and erythema subsided within 48 hours postoperatively, and the patient was discharged after 72 hours from treatment. PMID:24799096

  14. Metachronous bilateral mucoepidermoid carcinoma of the parotid gland

    Microsoft Academic Search

    John C. Muthusami; Pranay Gaikwad; John P. Raj; Deepak T. Abraham; Meera Thomas; Veracious Cornerstone

    2008-01-01

    We report a very rare case of bilateral muco-epidermoid carcinoma of the parotid gland that underwent bilateral parotidectomy\\u000a with neck dissections and radiotherapy. This case has done well for three years and suggests that metachronous bilateral mucoepidermoid\\u000a carcinoma of the parotid gland, if treated as per the merits of each side, has a reasonable survival.

  15. Pediatric bilateral large concurrent thalamic glioblastoma: An unusual case report

    PubMed Central

    Pandey, Nityanand; Singh, Pankaj Kumar; Mahapatra, Ashok K.; Kakkar, Aanchal; Sharma, Bhawani Shankar

    2014-01-01

    Bilateral thalamic tumors are rare. Pediatric bilateral thalamic glioblastomas are even rarer, only five cases reported in the English literature till date. The clinical presentation, natural history, and prognosis of pediatric thalamic tumors are still relatively obscure. In this article, we report an 8-year-old patient with large bilateral thalamic glioblastomas and briefly discuss its clinical presentation, possible modalities of management, and prognosis, in the light of available literature. PMID:24891914

  16. 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.

  17. Bilateral pneumothoraces complicating reduction mammoplasty: a case report

    PubMed Central

    2013-01-01

    Background Bilateral pneumothoraces after cosmetic breast surgery are rare and sporadically reported in the literature. Case presentation A 65-year-old female patient developed bilateral pneumothoraces after bilateral breast reduction surgery. Emergent chest tube thoracostomy was performed on both sides. The chest drains were removed on the fourth day (left side) and sixth day (right side), and the patient was discharged after 7 days of hospitalization without any further complications. Conclusion To our knowledge, the English-language literature contains no other reports of bilateral pneumothoraces after reduction mammoplasty. PMID:23890488

  18. Hiatus Hernia Repair with Bilateral Oesophageal Fixation

    PubMed Central

    Martin, David

    2015-01-01

    Background. Despite advances in surgical repair of hiatus hernias, there remains a high radiological recurrence rate. We performed a novel technique incorporating bilateral oesophageal fixation and evaluated outcomes, principally symptom improvement and hernia recurrence. Methods. A retrospective study was performed on a prospective database of patients undergoing hiatus hernia repair with bilateral oesophageal fixation. Retrospective and prospective quality of life (QOL), PPI usage, and patient satisfaction data were obtained. Hernia recurrence was assessed by either barium swallow or gastroscopy. Results. 87 patients were identified in the database with a minimum of 3 months followup. There were significant improvements in QOL scores including GERD HRQL (29.13 to 4.38, P < 0.01), Visick (3 to 1), and RSI (17.45 to 5, P < 0.01). PPI usage decreased from a median of daily to none, and there was high patient satisfaction (94%). 57 patients were assessed for recurrence with either gastroscopy or barium swallow, and one patient had evidence of recurrence on barium swallow at 45 months postoperatively. There was an 8% complication rate and no mortality or oesophageal perforation. Conclusions. This study demonstrates that our technique is both safe and effective in symptom control, and our recurrence investigations demonstrate at least short term durability. PMID:26065030

  19. Bilateral clavicle osteomyelitis: A case report

    PubMed Central

    Saglam, Fevzi; Saglam, Seymanur; Gulabi, Deniz; Eceviz, Engin; Elmali, Nurzat; Yilmaz, Mustafa

    2014-01-01

    INTRODUCTION Osteomyelitis of the clavicle is rare. Infection occurs from hematogenous spread or trauma. In adults infection is usually secondary due to an exogenous cause such as open fractures, surgery (iatrogenic) or spread from local tissue with infection. PRESENTATION OF CASE The case is presented here of a 50-year old female with bilateral clavicular fractures, who was operated on with open reduction and internal fixation. At the 6-month follow-up, she had complaints of bilateral osteomyelitis which was successfully treated with resection of the infected segment of the bone, and antibiotic impregnated collagen. DISCUSSION Predisposing factors include diabetes, intravenous drug abuse, tuberculosis or immune suppression. Management involves the removal of bone fixation, debridement of the bone and if there is a defect, coverage with a muscle flap is applied. CONCLUSION In cases of clavicular osteomyelitis where infection continues despite debridement and antibiotic therapy, excision of the sequestered clavicular section is a successful treatment approach and has been seen to improve quality of life without any functional loss. PMID:25460439

  20. A family with one child with acrocallosal syndrome, one child with anencephaly-polydactyly, and parental consanguinity

    Microsoft Academic Search

    S. Cataltepe; E. Tuncbilek

    1992-01-01

    A 2-year-old boy with typical features of the acrocallosal syndrome is presented. His parents are first degree cousins and their first pregnancy resulted in spontaneous abortion whereas the offspring of their second pregnancy was an anencephalic baby with bilateral postaxial polydactyly of the hands. The possibility of including anencephaly in the spectrum of the CNS malformations of the acrocallosal syndrome

  1. Long-term low-dose ketoconazole treatment in bilateral macronodular adrenal hyperplasia

    PubMed Central

    Comte-Perret, Sophie; Zanchi, Anne

    2014-01-01

    Summary Medical therapy for Cushing's syndrome due to bilateral macronodular adrenal hyperplasia (BMAH) is generally administered for a limited time before surgery. Aberrant receptors antagonists show inconsistent efficacy in the long run to prevent adrenalectomy. We present a patient with BMAH, treated for 10 years with low doses of ketoconazole to control cortisol secretion. A 48-year-old woman presented with headaches and hypertension. Investigations showed the following: no clinical signs of Cushing's syndrome; enlarged lobulated adrenals; normal creatinine, potassium, and aldosterone; normal urinary aldosterone and metanephrines; elevated urinary free cortisol and steroid metabolites; and suppressed plasma renin activity and ACTH. A screening protocol for aberrant adrenal receptors failed to show any illegitimate hormone dependence. Ketoconazole caused rapid normalisation of cortisol and ACTH that persists over 10 years on treatment, while adrenals show no change in shape or size. Ketoconazole decreases cortisol in patients with Cushing's syndrome, and may prevent adrenal overgrowth. Steroid secretion in BMAH is inefficient as compared with normal adrenals or secreting tumours and can be controlled with low, well-tolerated doses of ketoconazole, as an alternative to surgery. Learning points Enlarged, macronodular adrenals are often incidentally found during the investigation of hypertension in patients harboring BMAH. Although laboratory findings include low ACTH and elevated cortisol, the majority of patients do not display cushingoid features.Bilateral adrenalectomy, followed by life-long steroid replacement, is the usual treatment of this benign condition, and alternative medical therapy is sought. Therapy based on aberrant adrenal receptors gives disappointing results, and inhibitors of steroidogenesis are not always well tolerated.However, ketoconazole at low, well-tolerated doses appeared appropriate to control adrenal steroid secretion indefinitely, while preventing adrenal overgrowth. This treatment probably constitutes the most convenient long-term alternative to surgery. PMID:25535576

  2. Exercise Induced Rhabdomyolysis with Compartment Syndrome and Renal Failure

    PubMed Central

    Bhalla, Mary Colleen; Dick-Perez, Ryan

    2014-01-01

    Exertional rhabdomyolysis is sequela that is occasionally seen after strenuous exercise. The progression to compartment syndrome or renal failure is a rare complication that requires prompt recognition and treatment to prevent morbidity (Giannoglou et al. 2007). We present a case of a 22-year-old college football player who presented to the emergency department (ED) after a typical leg workout as part of his weight conditioning. He was found to have rhabdomyolysis with evidence of renal insufficiency. His condition progressed to bilateral compartment syndrome and renal failure requiring dialysis. After bilateral fasciotomies were performed he had resolution of his compartment syndrome. He continued to be dialysis dependent and had no return of his renal function at discharge 12 days after admission. PMID:25105034

  3. AMSAN variant of Guillain Barre syndrome progressing to chronic inflammatory demyelinating polyneuropathy in a patient with Marfan's syndrome and pulmonary tuberculosis.

    PubMed

    Soehardy, Z; Yuhanisa, A; Thein, S S; Rohana, A G; Fauzi, A R; Norlinah, M I; Hamidon, B B; Rozaidi, S W

    2005-12-01

    We report a 40-year-old man who has Marfan's syndrome and was recently diagnosed to have pulmonary tuberculosis when he presented with chronic cough. He was admitted with bilateral lower limb weakness which was ascending in nature. He eventually required ventilation. It was initially thought to be isoniazid-neuropathy. However, stopping the drug did not improve the condition and the patient developed bilateral lower motor neuron 7th cranial nerve palsy. Nerve conduction, MRI and CSF studies were done to confirm a first case report of AMSAN variant progressing to CIDP in a patient with Marfan's syndrome and pulmonary tuberculosis. PMID:16515122

  4. 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 prediction at the time the fellow eye is undergoing refractive surgery. PMID:24734876

  5. Learning about Velocardiofacial Syndrome

    MedlinePLUS

    ... terms used on this page. Learning About Velocardiofacial Syndrome What is velocardiofacial syndrome (VCFS)? What are the ... Syndrome Additional Resources for VCFS What is velocardiofacial syndrome? Velocardiofacial syndrome (VCFS) is a genetic condition that ...

  6. [Capgras' syndrome].

    PubMed

    Ben-Zion, I Z; Levine, K; Shiber, A

    1997-09-01

    We present 3 cases of Capgras' syndrome-a delusional disorder in which the patient believes that 1 (or more) of his acquaintances has been replaced by an imposter who appears as a double. 2 were schizophrenics and 1 had depression with psychotic features. This syndrome is rare in our practice, but we do not know if this is due to lack of awareness of the condition, or to the possibility that it is a culture-related syndrome. We suggest that although the syndrome has lost some of it's significance, it is still worth making the diagnosis because of the medical and psychological implications this condition carries. PMID:9461686

  7. Interstitial Keratitis, Vertigo, and Vasculitis: Typical Cogan's Syndrome.

    PubMed

    Azami, Ahad; Maleki, Nasrollah; Kalantar Hormozi, Mohammadreza; Tavosi, Zahra

    2014-01-01

    Cogan's syndrome (CS) is a chronic inflammatory disorder of unknown etiology that most commonly affects young adults. Clinical hallmarks are bilateral interstitial keratitis and vestibuloauditory dysfunction. Association between CS and systemic vasculitis as well as aortitis also exists. The diagnosis of CS is based upon presence of characteristic inflammatory eye disease and vestibuloauditory dysfunction. We describe classic Cogan's syndrome in a 47-year-old female from Ardabil. The patient was admitted with headache, vertigo, nausea, vomiting, right leg claudication, musculoskeletal pains, bilateral hearing loss, and blindness for the past two months. Ophthalmologic examination revealed that visual acuity was 0.1 bilaterally. Conjunctival hyperemia, bilateral cataract, and interstitial keratitis were detected with a slit lamp examination. Pure tone audiogram (PTA) and auditory brain stem response (ABR) showed bilateral sensorineural hearing loss. The other differential diagnosis of CS was studied and ruled out. Pulse i.v. methylprednisolone and cyclophosphamide were given and were followed by oral prednisolone and cyclophosphamide. Clinical follow-up showed partial improvement. PMID:24715922

  8. An Unusual Case of Bilateral Granulomatous Mastitis

    PubMed Central

    Pistolese, C. A.; Di Trapano, R.; Girardi, V.; Costanzo, E.; Di Poce, I.; Simonetti, G.

    2013-01-01

    Idiopathic granulomatous mastitis (IGM) is an uncommon benign disorder of the breast. At clinical examination, IGM is characterized by an inflammatory process of the breast, usually unilateral. Possible clinical findings are palpable mass with erythematous skin, pain, sterile abscesses, fistula and nipple retraction. Mammography and ultrasound findings are not specific for IGM. Magnetic resonance imaging (MRI) is a useful tool for the differential diagnosis; it is also necessary to delineate the exact extension of the disease and to plan the correct treatment. Final diagnosis is histological. We described an unusual case of IGM with bilateral involvement in a patient with history of pacemaker implantation and IGM typical clinical symptoms. Mammography, ultrasound, and MRI examinations were performed to identify the inflammatory disorder and to plan the correct therapy. Imaging features were correlated with final histological diagnosis of IGM. PMID:23781373

  9. Bilateral choroidal neovascularization after pegylated interferon therapy.

    PubMed

    Mahar, Pir Salim; Memon, Abdul Sami

    2015-04-01

    A30-year-old male presented with gradual decrease in vision in his both eyes after the use of interferon alpha for his hepatitis-C. Ocular examination of patient included, Best Corrected Visual Acuity (BCVA), fundus photographs, Fundus Fluorescein Angiography (FFA) and Optical Coherence Tomography (OCT). His BCVAwas recorded as 6/18 in the right eye and 6/12 in the left eye. The anterior segments were unremarkable, with normal intraocular Pressure (IOP) in his both eyes. Both fundi revealed the presence of bilateral Choroidal Neovascularization (CNV), confirmed on FFAand OCT. To the best of authors' knowledge, simultaneous presence of CNV in both eyes is not reported in the literature. PMID:25933463

  10. 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.

  11. A new image denoising framework based on bilateral filter

    Microsoft Academic Search

    Ming Zhang; Bahadir K. Gunturk

    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 in addition to some other applications. There are two main contributions of this paper. First, we provide an empirical study of the optimal parameter selection for the bilateral filter in image denoising applications. Second, we present

  12. Adaptive bilateral filter and Bayesian threshold based image denoising

    Microsoft Academic Search

    G. Santhanamari; V. R. Vijaykumar; A. V. V. B. Rao

    2010-01-01

    In this paper a hybrid denoising algorithm which combines adaptive bilateral Filter and Bayesian thresholding for digital images corrupted by Gaussian noise is proposed. The wavelet filter bank is used to decompose the image into approximation sub band and detail sub band. The adaptive bilateral filter is applied to approximation sub band and Bayesian thresholding is applied to detail sub

  13. Ultrasound Diagnosis of Bilateral Quadriceps Tendon Rupture After Statin Use

    PubMed Central

    Nesselroade, Ryan D.; Nickels, Leslie Connor

    2010-01-01

    Simultaneous bilateral quadriceps tendon rupture is a rare injury. We report the case of bilateral quadriceps tendon rupture sustained with minimal force while refereeing a football game. The injury was suspected to be associated with statin use as the patient had no other identifiable risk factors. The diagnosis was confirmed using bedside ultrasound. PMID:21079697

  14. Bilateral synchronous quadriceps tendon rupture: a case report

    Microsoft Academic Search

    Varatharaj Mounasamy; Robert C. Chadderdon; Candice McDaniel; Mark C. Willis

    2008-01-01

    Bilateral spontaneous rupture of quadriceps tendons is rare and is usually associated with predisposing comorbid conditions.\\u000a We report an uncommon case of bilateral synchronous rupture of the quadriceps tendon after a ground level fall in a 51-year-old\\u000a male, 8 years after renal transplant.

  15. Simultaneous bilateral quadriceps tendon rupture while playing basketball

    Microsoft Academic Search

    M Shah; N Jooma

    2002-01-01

    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.

  16. Postural fluctuations during pointing from a unilateral or bilateral stance

    Microsoft Academic Search

    Ing-Shiou Hwang; Chien-Ting Huang; Rong-Ju Cherng; Chien-Chun Huang

    2006-01-01

    An experiment was conducted to compare the effects of bilateral and unilateral stance on postural fluctuations and intralimb coordination during active balance control. Fifteen participants stood bilaterally and unilaterally while conducting a pointing task with an outstretched arm. Excursion of center of foot pressure (CoP) and limb movements were recorded with a force plate and eight dual-axis accelerometers, respectively. Compared

  17. Reconciling Bilateral Trade Data for Use in GTAP

    Microsoft Academic Search

    Mark Gehlhar

    1996-01-01

    Bilateral trade flows are reported by both importers and exporters. Large discrepancies in reported import\\/export trade flows can be found when these two reports are compared. The GTAP Data Base requires consistency between the export flow and its corresponding import flow for all partner pairs. Therefore, bilateral trade data in its reported form cannot be directly used for GTAP. Various

  18. Bilateral robot system on the real time network structure

    Microsoft Academic Search

    Yutaka Uchimura; Kouhei Ohnishi; Takahiro Yakoh

    2002-01-01

    This paper presents a bilateral robot system, which is driven by a static friction free drive system and implemented on the real time network structure. The goal is to realize a force feedback teleoperation with haptic impression transmission over computer networks. The paper considers two subjects relating to the bilateral robot. The first is static friction, which degrades the performance

  19. Closed cervical spine trauma associated with bilateral vertebral artery injuries

    Microsoft Academic Search

    P. Kloen; J. D. Patterson; B. I. Wintman; R. M. Ozuna; G. W. Brick

    1999-01-01

    Bilateral vertebral artery injuries in closed cervical spine injuries are uncommon, but early recognition and treatment are important to prevent neurological deterioration. A case of bilateral vertebral injuries in a 35-year-old motor vehicle accident victim is presented, and the current literature is reviewed.

  20. Should lung volume reduction for emphysema be unilateral or bilateral?

    Microsoft Academic Search

    Robert J. McKenna; Matthew Brenner; Richard J. Fischel; Arthur F. Gelb

    1996-01-01

    Both unilateral and bilateral lung volume reduction procedures are being advocated for treatment of severe, generalized emphysema. We analyzed the results of 166 consecutive patients who underwent unilateral (n = 87) or bilateral (n = 79) thoracoscopic stapled lung volume reductions to help define the role for these procedures. There was no statistically significant difference in the operative mortality (3.5%

  1. 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. PMID:25896777

  2. Recovery from bilateral renal vein thrombosis on supportive management alone

    Microsoft Academic Search

    Ashok Kumar; Deepika Chaudhary; V. Bhargava

    1995-01-01

    Renal vein thrombosis (RVT) may be fatal, particularly when it occurs bilaterally. We report a neonate who recovered from bilateral RVT on supportive management alone. A 2450 g male infant was born at term gestation to a non-diabetic mother. The anmiotic fluid contained particulate mecoilium. The Apgar scores were 1 and 3 at 1 and 5 minutes, respecfivelyl Postnatal course

  3. Neuromuscular fatigue differs following unilateral vs bilateral sustained submaximal

    E-print Network

    Paris-Sud XI, Université de

    Neuromuscular fatigue differs following unilateral vs bilateral sustained submaximal contractions 1 of Medicine & Science in Sports Volume 21, Issue 2, pages 268­276, April 2011 Additional Information(Show All. and Lepers, R. (2011), Neuromuscular fatigue differs following unilateral vs bilateral sustained submaximal

  4. Bilateral temporomandibular joint dislocation with locked mandibular impaction.

    PubMed

    Hynes, Sally L; Jansen, Leigh A; Brown, D Ross; Courtemanche, Douglas J; Boyle, James C

    2012-02-01

    Bilateral anterior temporomandibular joint dislocation is very rare, with only 2 reported cases published. In the present report, we describe a healthy 25-year-old man from Haida Gwaii, in British Columbia, Canada, who was transferred to our tertiary trauma center with life-threatening complications of a bilateral anterior temporomandibular joint dislocation with locked mandibular impaction. PMID:22260912

  5. Down Syndrome What causes Down syndrome?

    E-print Network

    Palmeri, Thomas

    04/13 Down Syndrome What causes Down syndrome? Individuals with Down syndrome usually have an extra chromosome 21. Down syndrome occurs in about 1 in every 700 to 1,000 births. The chance of giving birth to a baby with Down syndrome increases if the mother is over 35 years old. Down syndrome affects both males

  6. A New Image Denoising Framework Based on Bilateral Filter.

    PubMed

    Zhang, Ming; Gunturk, Bahadir K

    2008-01-28

    The bilateral filter is a nonlinear filter that does spatial averaging without smoothing edges; it has shown to be an effective image denoising technique in addition to some other applications. There are two main contributions of this paper. First, we provide an empirical study of the optimal parameter selection for the bilateral filter in image denoising applications. Second, we present an extension of the bilateral filter: multi-resolution bilateral filter, where bilateral filtering is applied to low-frequency subbands of a signal decomposed using an orthogonal wavelet transform. Combined with wavelet thresholding, this new image denoising framework turns out to be very effective in eliminating noise in real noisy images. We provide experimental results with both simulated data and real data. PMID:20445823

  7. A new image denoising framework based on bilateral filter

    NASA Astrophysics Data System (ADS)

    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 in addition to some other applications. There are two main contributions of this paper. First, we provide an empirical study of the optimal parameter selection for the bilateral filter in image denoising applications. Second, we present an extension of the bilateral filter: multi-resolution bilateral filter, where bilateral filtering is applied to low-frequency subbands of a signal decomposed using an orthogonal wavelet transform. Combined with wavelet thresholding, this new image denoising framework turns out to be very effective in eliminating noise in real noisy images. We provide experimental results with both simulated data and real data.

  8. Osteomyelitis of Bilateral Femoral Heads After Childbirth: A Case Report

    PubMed Central

    Lee, Kyung Soo; Kong, Sangwon; Kim, Junho; Kim, Taikon; Choi, Chan Beom; Kim, Yee-Suk

    2015-01-01

    Hip and pelvic pain during pregnancy or after delivery is a common problem in young females, and in most cases this problem has a self-limiting course. The patient described in this case suffered from severe hip pain after childbirth. MR imaging study was performed and it showed arthritis of bilateral hip joints and osteomyelitis of femoral heads with an abscess in the surrounding muscle. Infection, such as septic arthritis or osteomyelitis, is an extremely rare cause of peripartum joint pain. The patient's clinical symptoms and laboratory findings improved with antibiotic therapy. However, limitation of motion of the bilateral hip joints persisted although the patient continued rehabilitative therapy for 15 months, and the patient had to undergo bilateral total hip replacement. Hereby, we present a case of severe osteomyelitis and pyogenic arthritis of bilateral femoral heads and hip joints after delivery, which eventually required bilateral total hip replacement. PMID:26161359

  9. 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

  10. A Case of Neuromyelitis Optica Masquerading as Miller Fisher Syndrome

    PubMed Central

    Furutani, Yuka; Hata, Masayuki; Miyamoto, Kazuaki; Moribata, Yusaku; Yoshimura, Nagahisa

    2014-01-01

    A 22-year-old woman presented with double vision that she had experienced since an infection 2 weeks previously. A neurological examination showed limited bilateral eye abduction, mimicking Miller Fisher syndrome. However, T2-weighted magnetic resonance imaging of her brain revealed hyperintense areas in the tegmentum of the pons, including the abducens nucleus, and her serum anti-aquaporin-4 antibody test was positive. She was finally diagnosed with neuromyelitis optica. Intravenous high-dose steroid therapy immediately improved the patient's abduction palsy, but bilateral optic neuritis manifested during the treatment. Subsequent treatment with plasma exchange improved her optic neuritis symptoms. PMID:25408664

  11. Klinefelter Syndrome

    MedlinePLUS

    ... Is It? Klinefelter syndrome can cause problems with learning and sexual development in guys. It's a genetic condition (meaning a person is born with it). Klinefelter syndrome only affects males. It happens because of a difference deep inside the body's cells, in microscopic centers called ...

  12. HELLP Syndrome

    MedlinePLUS

    ... have your baby. HELLP stands for Hemolysis, Elevated Liver enzyme levels and a Low Platelet count. These are problems that can occur in women who have this syndrome. Women who have HELLP syndrome may have bleeding problems, liver problems and blood pressure problems that can hurt ...

  13. Genetics Home Reference: Noonan syndrome

    MedlinePLUS

    ... use for Noonan syndrome? familial Turner syndrome Female Pseudo-Turner Syndrome Male Turner Syndrome Noonan-Ehmke syndrome pseudo-Ullrich-Turner syndrome Turner-like syndrome Turner's phenotype, ...

  14. Fatal fat embolism syndrome in a case of isolated L1 vertebral fracture-dislocation.

    PubMed

    Yamauchi, Koun; Fushimi, Kazunari; Ikeda, Tsuneko; Fukuta, Masashi

    2013-11-01

    Although fat embolism syndrome is a well-known complication of fractures of the long bones or pelvis, fat embolism syndrome occurring subsequent to fracture of the lumbar spine is rare. We report a fatal case of fat embolism syndrome characterized by fat and bone marrow embolism that occurred 36 h after an isolated fracture-dislocation of the L1 vertebra. A postmortem examination was performed and pathological finding demonstrated fat and bone marrow tissue which were disseminated in the bilateral pulmonary arteries. We need to be aware of the possibility of fat embolism syndrome as a complication of spinal fractures, including isolated vertebral body fractures. PMID:23412313

  15. 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. PMID:25639327

  16. [Characterization of bilateral superior canal dehiscence].

    PubMed

    Boleas Aguirre, María Soledad; Migliaccio, Americo; Carey, John

    2007-11-01

    In the superior canal dehiscence syndrome, patients can have sound- or pressure-induced vertigo and oscillopsia. They may also present conductive hearing loss or higher than normal bone conduction thresholds. Clinical manifestations are due to the effect of a third mobile window in the inner ear created by the dehiscence. Diagnosis is based on clinical manifestations, vertical and rotatory nystagmus induced by sound and pressure reflecting SSC stimulation, reduced threshold and increased amplitude of vestibular evoked myogenic potentials (VEMP) and temporal bone CT scan images showing the SSC dehiscence. Characteristic eye movements can be recorded with the scleral search coil technique. PMID:17999910

  17. Bilateral eyelid erythema associated with false eyelash glue.

    PubMed

    Mimura, Tatsuya

    2013-03-01

    We report an unusual case of bilateral eyelid erythema caused by eyelash glue. A 22-year-old woman presented with a 3-day history of bilateral eyelid dermatitis after attaching false eyelashes by using latex-containing glue. Slit-lamp examination revealed erythema and swelling of the upper lids of both eyes. The skin prick test was positive for eyelash glue and her total tear IgE score was high. To the best of our knowledge, this is the first report of bilateral eyelid dermatitis caused by eyelash glue. PMID:22519514

  18. [Bilateral external auditory canal cholesteatoma - a case report].

    PubMed

    Roszkowska, Anna; Morawska-Kochman, Monika; Kubacka, Marzena; Dorobisz, Karolina; Kr?cicki, Tomasz

    2015-06-22

    The external auditory canal cholesteatoma is a rare and difficult to diagnose disease. Symptoms are similar to the external ear canal inflammation and can be masked by retained cerumen. In the article we described a case of 22 year old, mentally impairment women with bilateral otorrhea, in the past treated for external ear canal inflammation. The otoscopic examination showed bilateral congenital auditory canal stenosis, with masses obstructing ear canals. Imaging and histopathologic studies revealed bilateral external ear canal cholesteatoma. The patient was qualified for surgery, which, together with a guardian, did not consent. Presented case indicates the necessity for widening the diagnostic of recurrent external ear canal inflammation. PMID:26098654

  19. Bilateral cerebellopontine angle lesions not always NF2: diagnostic pitfall.

    PubMed

    Rao, Ahsan; Lawrie, Alistair; Bodkin, Peter; Tighe, Jane; Kamel, Mahmoud

    2012-04-01

    Bilateral internal auditory canal (IAC) tumours are almost exclusively associated with bilateral vestibular schwannomas, and there is very little, if anything, that can mimic this appearance. We present a very rare case of a 75-year-old gentleman who initially presented with bilateral IAC tumours and later diagnosed as an isolated primary CNS myeloma without systemic involvement. This is a very rare presentation and has important diagnostic and therapeutic implications. He was treated with a combination of lenalidomide and dexamethasone. The treatment was well tolerated but with limited response. Although rare, metastasis should be considered as a differential diagnosis of IAC lesions. PMID:21970782

  20. Male Breast Cancer: Presenting as Synchronous, Large, Bilateral Masses

    PubMed Central

    Chougule, P. G.; Khatib, Wasim; Shukla, Dhirajkumar B.; Jagtap, Swati Sunil

    2014-01-01

    Male breast cancer is a very rare neoplasm which accounts for 1% of all breast cancers. A 70-year-old male presented with a rapidly growing, bilateral breast masses with large size, surface ulceration and bloody discharge. Synchronous bilateral breast cancer was diagnosed by using fine needle aspiration cytology, mammography, ultrasonography and incisional biopsy. Histopathological studies revealed invasive ductal carcinoma (not otherwise specified), which was of grade III in left breast and of grade II in right breast. We are presenting this case with its clinico-pathological findings, as synchronous bilateral breast cancer occurs extremely rarely in males. PMID:24959456

  1. A rare case of bilateral bronchial foreign body

    PubMed Central

    Zhao, ZhiGang; Gao, Qian; Song, PengLong

    2015-01-01

    We present the case of a 7-year-old male patient with bilateral bronchial cocklebur fruit aspiration, which he sustained while playing. The patient presented with a triad of cough, wheezing, and decreased breath sounds (decreased in the right lung and absent in the left). These symptoms led to a diagnosis of bilateral bronchial foreign body, which was confirmed by computed tomography three-dimensional reconstruction of the bronchial tree. The patient was on the verge of death during operation but was ultimately rescued. Our therapeutic experience in treating this case of bilateral bronchial cocklebur fruit aspiration may provide a good reference for others.

  2. Bilateral renal artery thrombosis secondary to acute necrotizing pancreatitis.

    PubMed

    Thajudeen, Bijin; Budhiraja, Pooja; Bracamonte, Erika R

    2013-10-01

    Renal artery thrombosis is a rare, but serious and often under-diagnosed condition. We report a case of bilateral renal artery thrombosis secondary to acute necrotizing pancreatitis. A 66-year-old female presented with abdominal pain and acute kidney injury (AKI). A renal biopsy showed organized intraluminal thrombi and a computer tomography scan of the abdomen showed bilateral renal artery thrombosis. Emergent laprotomy showed necrosed pancreas. Doppler studies showed deep vein thrombosis of the lower extremities and internal jugular vein thrombosis. Workup for hypercoagulability was unremarkable. The final diagnosis was AKI secondary to bilateral renal artery thrombosis probably due to hypercoagulability of acute necrotizing pancreatitis. PMID:26064514

  3. Geleophysic dysplasia associated with bilateral angle closure glaucoma

    PubMed Central

    Saricaoglu, Murat Sinan; Güven, Dilek; Karakurt, Ahmet; Hasiripi, Hikmet

    2013-01-01

    In this case report, we present occurrence of bilateral angle closure glaucoma in a 9-year-old girl with geleophysic dysplasia. Bilateral YAG laser iridotomy was applied, but intraocular pressure (IOP) remained at high levels, necessitating bilateral trabeculectomy with mitomycin C. On her follow-up examinations for 3 years, IOP remained in the mid-20s with no need for further intervention or antiglaucoma medication. There are few reports describing the ocular findings of geleophysic dysplasia in literature. To our knowledge, this is the first case report describing an application of glaucoma surgery and its results at geleophysic dysplasia. PMID:23514648

  4. Bilateral activity and callosal connections in the somatosensory cortex.

    PubMed

    Iwamura, Y; Taoka, M; Iriki, A

    2001-10-01

    Earlier studies recording single neuronal activity in the postcentral somatosensory cortex of monkeys converged in suggesting that the bilateral receptive fields were related exclusively to the body midline including the trunk, perioral face, and oral cavity. These neurons were recorded mostly in the rostral part of the gyrus, areas 3b and 1. However, the authors recently found a substantial number of neurons with bilateral receptive fields on extremities, hand/digits, shoulders/arms, or legs/feet in the caudalmost part (areas 2 and 5) of the postcentral gyrus. The authors review these results and discuss functional implications of the bilateral representation in the postcentral somatosensory cortex. PMID:11597101

  5. 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.

  6. Bilateral renal artery thrombosis secondary to acute necrotizing pancreatitis

    PubMed Central

    Thajudeen, Bijin; Budhiraja, Pooja; Bracamonte, Erika R.

    2013-01-01

    Renal artery thrombosis is a rare, but serious and often under-diagnosed condition. We report a case of bilateral renal artery thrombosis secondary to acute necrotizing pancreatitis. A 66-year-old female presented with abdominal pain and acute kidney injury (AKI). A renal biopsy showed organized intraluminal thrombi and a computer tomography scan of the abdomen showed bilateral renal artery thrombosis. Emergent laprotomy showed necrosed pancreas. Doppler studies showed deep vein thrombosis of the lower extremities and internal jugular vein thrombosis. Workup for hypercoagulability was unremarkable. The final diagnosis was AKI secondary to bilateral renal artery thrombosis probably due to hypercoagulability of acute necrotizing pancreatitis.

  7. Kousseff syndrome: A fifth case?

    SciTech Connect

    Laux, R.A. [Foundation for Blood Research, Portland, ME (United States); Hamilton, W.; Pinette, M. [Maine Medical Center, Portland, ME (United States)] [and others

    1994-09-01

    Kousseff originally described three siblings with an open sacral myelomeningocele, conotruncal cardiac malformations, low-set, posteriorly rotated ears, retrognathia, a short neck with a low posterior hairline, and renal agenesis as a new autosomal recessive condition. Open neural tube lesions and complex conotruncal cardiac defects are relatively common malformations, both as isolated defects and individually as components of syndromes, but they have been found together only rarely, as part of chromosomal syndromes or following maternal exposures. Toriello et al. reported a fourth case and suggested the eponym Kousseff syndrome for myelomeningcocele, conotruncal defects and minor facial abnormalies. We report a fifth probable case. This male infant was born by spontaneous vaginal delivery at 38 weeks gestation to a 23-year-old G{sub 2}P{sub 1001} mother. Pregnancy was complicated by an elevated alpha-fetoprotein at 16 weeks gestation, followed by an ultrasound diagnosis of an open disease. After birth, physical examination also revealed dysmorphic facies, with a bulbous nose and low-set, posteriorly rotated ears, bilateral 5th finger clinodactyly and hypotonia. Echocardiogram demonstrated complex conotruncal malformations. The patient underwent closure of the myelomeningocele but died at one month of age. Chromosomal analysis was normal (46,XY). Because conotruncal heart defects have been associated with deletions on chromosome 22, FISH analysis using a probe for the DiGeorge syndrome on the long arm of chromosome 22 was performed. It indicated no detectable deletion within this critical region on 22q11. Nonetheless there remains the possibility of a gene (or genes) located on 22q that could produce findings of this rare multiple congenital anomaly syndrome when disrupted. Therefore, further investigation on this chromosome is warranted.

  8. Bilateral putaminal necrosis and bronopol toxicity.

    PubMed

    Trivisano, Marina; Carapelle, Elena; Martino, Tommaso; Specchio, Luigi Maria

    2015-01-01

    Among alcohols, methanol intoxication is the most frequently associated with cerebral toxicity, causing retinal damage and putaminal necrosis. This consequence is believed to be due to the transformation of methanol into formic acid. We describe the case of a patient who presented with acute impairment of consciousness and tetraparesis after she had been drinking several bottles of a topical antiseptic solution (Lysoform Medical) containing 2-bromo-2-nitro-1,3-propandiol (bronopol) among excipients, in order to lose weight during previous months. Moreover, she had been on a strict slimming diet. Soon after admission, a severe respiratory and metabolic impairment became rapidly evident, requiring an intensive care unit admission. Cerebral MRI showed the presence of bilateral putaminal necrosis. She recovered in 10 days, surprisingly, without any evident clinical neurological signs. Methanol, also bronopol, when diluted in aqueous solution, at warm temperature and/or higher pH, may release formaldehyde, which is converted into formic acid, a basal ganglia toxic compound. PMID:25697297

  9. 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.

  10. A longitudinal study in adults with sequential bilateral cochlear implants: Time course for individual ear and bilateral performance

    PubMed Central

    Reeder, Ruth M; Firszt, Jill B; Holden, Laura K; Strube, Michael J

    2013-01-01

    Purpose Examine rate of progress in the second implanted ear as it relates to the first implanted ear and to bilateral performance in adult sequential cochlear implant recipients. Additionally, identify factors that contribute to patient outcomes. Methods A prospective longitudinal study in 21 adults who received bilateral, sequential cochlear implants. Testing occurred at six intervals: pre-bilateral through 12 months post-bilateral implantation. Measures evaluated speech recognition in quiet and noise, localization and perceived benefit. Results Second ear performance was similar to first ear performance by six months post-bilateral implantation. Bilateral performance was generally superior to either ear alone. However, participants with shorter second ear length of deafness (< 20 years) had more rapid early improvement and better overall outcomes than those with longer second ear length of deafness (> 30 years). All participants reported bilateral benefit. Conclusions Adult cochlear implant recipients demonstrated benefit from second ear implantation for speech recognition, localization and perceived communication function. Since performance outcomes were related to length of deafness, shorter time between surgeries may be warranted to reduce negative length-of-deafness effects. Future study may clarify the impact of other variables such as pre-implant hearing aid use, particularly for individuals with longer periods of deafness. PMID:24686778

  11. Goldenhar syndrome.

    PubMed

    Sharma, Neeraj; Passi, Sidhi

    2013-01-01

    Goldenhar syndrome is a syndrome of complex structures developing from first and second branchial arches during blastogenesis. The etiology of this rare disease is not fully understood, as it has shown itself variable genetically and of unclear causes. The disorder is characterized by a wide spectrum of symptoms and physical features that may vary greatly in range and severity from case to case. Here we present a unique case of Goldenhar syndrome with absence of left condyle, hypoplasia of the zygomatic bone, no pneumatization of the mastoid process, underdeveloped mandible, bifid tongue and the skin tags in the preauricular area. PMID:23852257

  12. Supracardiac total anomalous pulmonary venous connection with bilateral (right and left) vertical veins and bilateral obstruction.

    PubMed

    Gavali, Seema A; Phadke, Milind S; Kerkar, Prafulla G

    2013-10-01

    This report describes a rare form of obstructed supracardiac total anomalous pulmonary venous connection with two vertical veins (right and left) draining the right- and the left-sided pulmonary veins respectively without formation of a common chamber and with bilateral obstruction. Surgery for these patients is technically challenging due to the absence of a common chamber, and the prognosis is worse than for patients with a common chamber. Also, it is important to be aware of this entity so that an accurate noninvasive preoperative diagnosis by echocardiography alone without invasive cardiac catheterization is possible. This facilitates emergency surgical repair without delay, which is crucial to improvement of the outcome. PMID:23354149

  13. A novel mitochondrial DNA 8597T>C mutation of Leigh syndrome: report of one case.

    PubMed

    Tsai, Jeng-Dau; Liu, Chin-San; Tsao, Teng-Fu; Sheu, Ji-Nan

    2012-02-01

    Leigh syndrome is an early-onset progressive neurodegenerative disorder with a characteristic neuropathology consisting of focal, bilateral lesions in one or more areas of the central nervous system. The brain images of Leigh syndrome are characterized by markedly symmetrical involvement, most frequently of the putamen. We report a 2-year-old girl with Leigh syndrome manifested as acute onset of altered level of consciousness. Brain magnetic resonance images showed abnormal signal intensity over the bilateral basal ganglia and cerebellar dentate nuclei. Despite normal biochemical studies, in particular serum lactate levels, magnetic resonance spectroscopy demonstrated a downward doublet lactate peak. The diagnosis of Leigh syndrome was subsequently confirmed by genetic study which showed a novel mutation at 8597T>C of the mitochondrial ATPase6 gene. PMID:22348497

  14. Sound localization and interaural time sensitivity with bilateral cochlear implants

    E-print Network

    Poon, Becky Bikkei

    2006-01-01

    Bilateral cochlear implantation is becoming more common as clinicians attempt to provide better sound-source localization and speech reception in noise for cochlear implant (CI) users. While some improvement over the ...

  15. Bilateral primary adrenal non-Hodgkin's lymphoma without adrenal insufficiency

    PubMed Central

    Simpson, William Greg; Babbar, Paurush; Payne, Lynnetta Faith

    2015-01-01

    We are presenting a rare case of bilateral adrenal non-Hodgkin's lymphoma (NHL) that presented as a primary malignancy. An 83-year-old man presented with newly discovered bilateral adrenal incidentalomas, fatigue, and 30 pound weight loss. Of the 116 cases of primary adrenal NHL reported, over half have presented bilaterally and occur with adrenal insufficiency. Therefore, the finding of bilateral adrenal masses requires an urgent work-up of the functional status of the adrenal gland as well as a thorough analysis of the imaging characteristics seen on noncontrast computed tomography (CT) in order to maximize patient survival. Adrenal function testing was normal. Repeat CT imaging revealed rapidly growing lesions with high attenuations; both masses >10 HU. Histological examination of core biopsies discovered malignant lymphoma with no known past history of lymphoma. Our case coincides with the literature, which states that a mass with attenuation >10 HU in the adrenal glands has a high risk of malignancy. PMID:25837497

  16. Interactions between bilaterally paired components of the cockroach circadian system

    Microsoft Academic Search

    Terry L. Page

    1978-01-01

    The hypothesis that bilaterally redundant, and mutually coupled circadian pacemakers are located near the lobula of the optic lobes of the cockroach,Leucophaea maderae, was investigated in a series of lesion experiments.

  17. Early deprivation impairs the development of balance and bilateral coordination.

    PubMed

    Roeber, Barbara J; Gunnar, Megan R; Pollak, Seth D

    2014-07-01

    This study examined balance and bilateral coordination skills in a sample of internationally adopted, post-institutionalized (PI) children. We compared the performance of these PI children to two age-matched groups. One was a group of children who were internationally adopted from foster care (FC). The second group consisted of non-adopted children being raised in their birth families, who served as controls (Control). Both PI and FC children scored lower than control children on balance, while PI children scored lower than both FC and control children on bilateral coordination. These results suggest that aspects of institutional rearing impact the development of bilateral coordination, while factors common to internationally adopted children other than institutionalization impact the development of balance. Region of birth (Asia, Latin/South America, Russia/Eastern Europe) did not moderate associations between institutional duration and bilateral coordination. PMID:24014461

  18. Bartter syndrome

    MedlinePLUS

    ... to reabsorb sodium. Persons affected by Bartter syndrome lose too much sodium through the urine. This causes a rise in the level of the hormone aldosterone, and makes the kidneys remove too much potassium from the body. This ...

  19. Marfan Syndrome

    MedlinePLUS

    ... Loeys-Dietz is more likely to cause fatal aortic aneurysms, and treatment for the two is different. A ... shown that the blood pressure medication losartan prevents aortic aneurysms in a mouse model of Marfan syndrome. Studies ...

  20. Aicardi syndrome

    MedlinePLUS

    ... and 5 months. The condition causes jerking (infantile spasms), a type of childhood seizure. Aicardi syndrome may ... missing Female sex Seizures (typically beginning as infantile spasms) Sores on the retina (retinal lesions) or optic ...

  1. Sjögren's Syndrome

    MedlinePLUS

    ... effects of saliva, you may develop more dental decay (cavities) and mouth infections. Sjögren’s syndrome can also ... mouth makes you extremely prone to progressive dental decay (cavities). Water. Take sips of water or another ...

  2. Rett Syndrome

    MedlinePLUS

    ... Rett syndrome is a neurodevelopmenal disorder that affects girls almost exclusively. It is characterized by normal early ... occur, although breathing usually improves during sleep. Some girls also display autistic-like symptoms such as loss ...

  3. Behcet's Syndrome

    MedlinePLUS

    Behcet's syndrome is a disease that involves inflammation of the blood vessels. It causes problems in many parts of the body. The most common symptoms ... National Institute of Arthritis and Musculoskeletal and Skin Diseases

  4. Down Syndrome

    MedlinePLUS

    ... or problems with their heart, stomach or eyes. Intelligence ranges from low normal to very retarded (slow ... a baby who has Down syndrome will be. Intelligence ranges from low normal to very retarded (slow ...

  5. Alagille Syndrome

    MedlinePLUS

    ... bile ducts in the liver. Bile that cannot flow through the deformed ducts builds up in the ... syndrome involves five distinct findings, including reduced bile flow, congenital heart disease, bone defects, a thickening of ...

  6. Ohtahara Syndrome

    MedlinePLUS

    ... have primarily tonic seizures, but may also experience partial seizures, and rarely, myoclonic seizures. Ohtahara syndrome is ... a characteristic pattern of high voltage spike wave discharge followed by little activity. This pattern is known ...

  7. Sotos Syndrome

    MedlinePLUS

    ... threatening disorder and patients may have a normal life expectancy. The initial abnormalities of Sotos syndrome usually resolve as the growth rate becomes normal after the first few years of life. Developmental delays may improve in the school-age ...

  8. Marfan Syndrome

    MedlinePLUS

    ... surgery. Some people may also choose to have surgery for cosmetic reasons. Anyone with heart problems associated with Marfan syndrome (especially anyone who's had heart surgery) should always take antibiotics before going to the ...

  9. Menkes syndrome

    MedlinePLUS

    Menkes syndrome is an inborn error of metabolism in which cells in the body can absorb copper, but are unable to release it. ... Houwen RHJ. Disorders in the Transport of Copper, Iron, Magnesium, Manganese, Selenium, and Zinc. In: Saudubray J- ...

  10. Hurler Syndrome

    MedlinePLUS

    ... the body how to work properly. In Hurler syndrome, the body has a defective gene and cannot make an important enzyme. Enzymes are proteins inside cells that break down larger building block chemicals into smaller ones. When ...

  11. Down Syndrome

    MedlinePLUS

    ... Special help, such as physical therapy and speech therapy , can give kids a boost with their walking and talking skills. Continue Do a Lot of People Have Down Syndrome? About 1 out of every 800 babies born ...

  12. Aortoduodenal syndrome.

    PubMed

    Takagi, H; Watanabe, T; Umemoto, T

    2015-10-01

    Duodenal obstruction caused by abdominal aortic aneurysm (AAA), i.e. "aortoduodenal syndrome," first described by Osler in 1905 is a rare clinical entity, with only several dozens of cases reported in the literature. In the present paper, we systematically searched literature and reviewed them. Databases including MEDLINE and EMBASE were searched from January 1981 to April 2014 using Web-based search engines (PubMed and OVID). Eighteen papers reported 21 cases with aortoduodenal syndrome. Mean age of patients was 74.5±8.4 years, 71.4% of patients were men, and mean AAA diameter was 7.2±2.1 cm. We also discussed confusion between aortoduodenal syndrome and "superior mesenteric artery syndrome" associated with AAA. PMID:25216355

  13. Sjogren's Syndrome

    MedlinePLUS

    ... and destroy the glands that produce tears and saliva. Sjögren's syndrome is also associated with rheumatic disorders ... increasing knowledge and understanding of the disorder, improving diagnostic techniques, testing interventions, and finding ways to treat, ...

  14. Hepatorenal syndrome.

    PubMed

    Bataller, R; Ginčs, P; Guevara, M; Arroyo, V

    1997-01-01

    Hepatorenal syndrome is a common complication in patients with advanced cirrhosis and ascites characterized not only by renal failure but also by marked alterations in systemic hemodynamics and vasoactive systems. Renal failure is due to a marked hypoperfusion of the kidney secondary to renal vasoconstriction. The pathogenesis of hepatorenal syndrome is not completely known but it is thought to be the extreme manifestation of the underfilling of the arterial circulation secondary to an arterial vasodilation, located mainly in the splanchnic circulation. Recently, a new definition and diagnostic criteria of hepatorenal syndrome have been proposed, which has stimulated research in this field. Prognosis of patients with hepatorenal syndrome is very poor. Liver transplantation is the only effective treatment but it is not applicable in all patients due to short survival. New therapies developed during the last few years, such as the use of systemic vasoconstrictors or transjugular intrahepatic portosystemic shunts appear promising, but their usefulness should be evaluated in prospective investigations. PMID:9308128

  15. Isaac's Syndrome

    MedlinePLUS

    ... typically caused by antibodies that bind to potassium channels on the motor nerve. Issacs' syndrome is only ... neurological conditions that can be caused by potassium channel antibodies. Is there any treatment? Anticonvulsants, including phenytoin ...

  16. Hyperventilation syndrome

    Microsoft Academic Search

    Richard E. Brashear

    1983-01-01

    The hyperventilation syndrome, primary alveolar hyperventilation and respiratory alkalosis accompanied by various signs and\\u000a symptoms, occurs in about 6–11% of the general patient population. The causes of hyperventilation are: 1) organic and physiologic\\u000a and, 2) psychogenic (emotional\\/habit). Hyperventilation syndrome excludes hyperventilation that is compensatory or caused\\u000a by organic or physiologic factors. Acute or chronic anxiety is usually considered the predominant

  17. Bilateral, simultaneous rupture of the quadriceps tendon: a diagnostic pitfall?

    Microsoft Academic Search

    T. Neubauer; M. Wagner; T. Potschka; M. Riedl

    2007-01-01

    Bilateral, simultaneous quadriceps tendon rupture (QTR) represents a rare entity and delay in establishing the correct diagnosis\\u000a is not uncommon. Another three cases are reported here and in all the correct diagnosis was missed initially. A review of\\u000a the English and German literature retrieved 105 cases of bilateral, simultaneous QTR and in 32 patients (30.5%) the correct\\u000a diagnosis was established

  18. Bilateral thoracoscopic thymectomy using a novel positioning system.

    PubMed

    Caronia, Francesco; Fiorelli, Alfonso; Monte, Attilio Lo

    2014-11-01

    Several techniques of bilateral video-assisted thoracoscopic extended thymectomy have been proposed, and each has its own proponents. We summarize our experience in 20 patients who underwent bilateral video-assisted thoracoscopic extended thymectomy, using a new patient positioning that amplifies the thoracoscopic view of the cardiophrenic regions which are often difficult to visualize with standard techniques. In all cases, en-bloc thymectomy with complete dissection of the mediastinal fatty tissue was achieved without sternal retractors or additional incisions. PMID:24887902

  19. Ultrasound assessment of bilateral symmetry in dorsal Lisfranc ligament.

    PubMed

    Marshall, Joshua J; Graves, Nathan C; Rettedal, David D; Frush, Katherine; Vardaxis, Vassilios

    2013-01-01

    Bilateral symmetry of the ligaments is a common assumption used as an intrasubject control for clinical diagnosis. The present study investigated the bilateral symmetry of the dorsal Lisfranc ligament (dLL) using ultrasound. Data were acquired from 50 asymptomatic subjects in a seated position at a loaded calf raise machine equipped with a force plate. The testing conditions included low, medium, and high stress at 0° and 15° abducted foot positions. Images of the dLL were captured and measured using a 10.0-MHz ultrasound transducer and custom written MATLAB software, respectively. The data were analyzed using paired t tests to compare the bilateral measurements of the dLL length under all test conditions. The bilateral pooled dLL length was 7.01 ± 1.38 mm and showed a moderate correlation with the foot length and width. No bilateral differences were found in the dLL length under any of the stress loads in the abducted position or under the medium and high stress load in the rectus position. However, the low stress load rectus position demonstrated a significant bilateral difference in the dLL length (p = .005). The smallest bilateral difference was observed at the 15° abducted position under medium stress (measurement error mean -0.062 mm). Our data suggest that the contralateral dLL length can be used as an intrasubject control for clinical purposes. However, we recommend that the dLL length measurements should be taken in weightbearing position with the foot in the abducted position under medium stress (bilateral stance), reducing potential strain-induced asymmetry. PMID:23522739

  20. Bilateral adrenal non-Hodgkin's lymphoma with adrenal insufficiency

    PubMed Central

    Ellis, R; Read, D

    2000-01-01

    A 74 year old women presented with lethargy and weight loss and was found to have profound adrenal insufficiency and bilateral adrenal mass lesions. Histological examination revealed non-Hodgkin's lymphoma. There was no evidence of lymphoma outside the adrenal glands. Isolated bilateral adrenal masses may rarely be due to primary adrenal non-Hodgkin's lymphoma, which is often associated with adrenal insufficiency.???Keywords: lymphoma; adrenal insufficiency PMID:10908383

  1. 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

  2. Laser arytenoidectomy in the treatment of bilateral vocal cord paralysis

    Microsoft Academic Search

    Z. Szmeja; J. G. Wójtowicz

    1999-01-01

    The introduction of the CO2 surgical laser into laryngeal microsurgery has made resection of the posterior vocal cord together\\u000a with the arytenoid cartilage possible. Since November 1990, 30 arytenoidectomies, 17 partial cordectomies and 18 bilateral\\u000a cordectomies as described by Kashima were performed by means of a CO2 laser in patients with bilateral paralyses of the vocal cords. In this group

  3. Presumed topiramate-induced bilateral acute angle-closure glaucoma

    Microsoft Academic Search

    James T Banta; Kara Hoffman; Donald L Budenz; Elizenda Ceballos; David S Greenfield

    2001-01-01

    PURPOSE: We describe a case of bilateral angle-closure glaucoma associated with oral topiramate therapy.METHODS: Interventional case report. Case report with echographic illustration.RESULTS: A 51-year-old man developed bilateral acute angle-closure glaucoma 2 weeks after beginning topiramate therapy for bipolar affective disorder. Laser peripheral iridotomy was performed in the right eye without resolution of the acute attack. Echography revealed lens thickening and

  4. Recurrent Bilateral Breast Abscess Due to Nontuberculous Mycobacterial Infection

    PubMed Central

    Yoo, Hyunkyung; Choi, Sang Hyun; Kim, Sei Joong; Cho, Young Up; Choi, Suk Jin

    2014-01-01

    Since recurrent bilateral breast infection due to nontuberculous mycobacterium (NTM) is rare, its diagnosis is easily overlooked; in addition, complete recovery is often difficult to achieve. We report a case of recurrent bilateral infection in a 35-year-old woman who had completed treatment for NTM. Although various infectious diseases show similar clinical conditions and imaging findings, recurrences should raise suspicion of NTM infection, and this possibility should be considered in differential diagnoses. PMID:25320630

  5. Alagille syndrome.

    PubMed Central

    Krantz, I D; Piccoli, D A; Spinner, N B

    1997-01-01

    Alagille syndrome (OMIM 118450) is an autosomal dominant disorder associated with abnormalities of the liver, heart, eye, skeleton, and a characteristic facial appearance. Also referred to as the Alagille-Watson syndrome, syndromic bile duct paucity, and arteriohepatic dysplasia, it is a significant cause of neonatal jaundice and cholestasis in older children. In the fully expressed syndrome, affected subjects have intrahepatic bile duct paucity and cholestasis, in conjunction with cardiac malformations (most frequently peripheral pulmonary stenosis), ophthalmological abnormalities (typically of the anterior chamber with posterior embryotoxon being the most common), skeletal anomalies (most commonly butterfly vertebrae), and characteristic facial appearance. Inheritance is autosomal dominant, but expressivity is highly variable. Sibs and parents of probands are often found to have mild expression of the presumptive disease gene, with abnormalities of only one or two systems. The frequency of new mutations appears relatively high, estimated at between 15 and 50%. The disease gene has been mapped to chromosome 20 band p12 based on multiple patients described with cytogenetic or molecular rearrangements of this region. However, the frequency of detectable deletions of 20p12 is low (less than 7%). Progress has been made in the molecular definition of an Alagille syndrome critical region within the short arm of chromosome 20. We will review the clinical, genetic, cytogenetic, and molecular findings in this syndrome. Images PMID:9039994

  6. Tics and Tourette Syndrome

    MedlinePLUS

    MENU Return to Web version Tics and Tourette Syndrome Overview What is Tourette syndrome? Tourette syndrome is a type of tic disorder. Children who have Tourette syndrome will repeat both movements ...

  7. What Are Myelodysplastic Syndromes?

    MedlinePLUS

    ... How many people get myelodysplastic syndromes? What are myelodysplastic syndromes? Myelodysplastic syndromes (MDS) are conditions that occur when ... of blood vessels caused by cuts or bruises. Myelodysplastic syndromes In MDS, some of the cells in the ...

  8. Myelodysplastic Syndromes (MDS)

    MedlinePLUS

    ... syndromes (MDS) Email this page Print this page Myelodysplastic syndromes (MDS) Myelodysplastic syndromes (MDS) are a group of diseases that affect ... other types are severe and life-threatening. Tweet Myelodysplastic syndromes (MDS) Symptoms of MDS How transplant can treat ...

  9. Tethered Spinal Cord Syndrome

    MedlinePLUS

    NINDS Tethered Spinal Cord Syndrome Information Page Table of Contents (click to jump to sections) What is Tethered Spinal Cord Syndrome? Is ... being done? Clinical Trials Organizations What is Tethered Spinal Cord Syndrome? Tethered spinal cord syndrome is a neurological ...

  10. Down Syndrome (For Parents)

    MedlinePLUS

    About Down Syndrome Down syndrome (DS), also called Trisomy 21, is a condition in which extra genetic material causes delays ... rises to about 1 in 100. Continue How Down Syndrome Affects Kids Kids with Down syndrome tend to ...

  11. Carpal Tunnel Syndrome

    MedlinePLUS

    ... important to know the difference. What is carpal tunnel syndrome? Carpal tunnel syndrome is possibly the most ... permanent nerve and muscle damage. What causes carpal tunnel syndrome? Carpal tunnel syndrome may occur in patients ...

  12. Carpal Tunnel Syndrome

    MedlinePLUS

    NINDS Carpal Tunnel Syndrome Information Page Condensed from Carpal Tunnel Syndrome Fact Sheet Table of Contents (click to jump to sections) ... Organizations Additional resources from MedlinePlus What is Carpal Tunnel Syndrome? Carpal tunnel syndrome is a painful condition ...

  13. Hyperimmunoglobulin E syndrome

    MedlinePLUS

    Job syndrome; Hyper IgE syndrome ... Hyperimmunoglobulin E syndrome is also called Job syndrome, after the biblical character Job whose faithfulness was tested by an affliction with draining skin sores and pustules . People with this ...

  14. Bilateral Traumatic Globe Luxation with Optic Nerve Transection

    PubMed Central

    Tok, Levent; Tok, Ozlem Yalcin; Argun, Tugba Cakmak; Yilmaz, Omer; Gunes, Alime; Unlu, Elif Nisa; Sezer, Sezgin; Ibisoglu, Seda; Argun, Mehmet

    2014-01-01

    Purpose The purpose of this study was to document clinical findings and management of a patient with bilateral globe luxation and optic nerve transection. Materials and Methods A 25-year-old female patient was admitted to the emergency department with bilateral traumatic globe luxation following a motor vehicle accident. Results Visual acuity testing showed no light perception. The right pupil was dilated and bilaterally did not react to light. The globes were bilaterally intact. A computed tomography scan revealed Le Fort type II fractures, bilateral optic nerve transection and disruption of all extraocular muscles. The globes of the patient were bilaterally reduced into the orbit. However, the patient developed phthisis bulbi in the right eye at month 3. Conclusion Globe luxation presents a dramatic clinical picture, and may lead to the development of severe complications due to the concomitance of complete optic nerve dissection and multiple traumas. Even if the luxated globe is repositioned into the orbit, there is still an increased risk of the development of phthisis due to ischemia. PMID:25606034

  15. Bilateral parotid enlargement due to malnutrition under the influence of the media in an adolescent in Lithuania.

    PubMed

    Mieliauskaite, Diana; Venalis, Algirdas; Graziene, Vida; Kirdaite, Gailute

    2007-07-01

    The elimination of censorship for the media in post-communist countries in transition has contributed to increases in the prevalence of several medical problems. Children and adolescents are particularly vulnerable to the messages conveyed through the media, which influence their perceptions and behaviour. We describe a case of bilateral parotid enlargement due to malnutrition under the influence of self-prescribed diet in an adolescent. A 15-year-old girl reported to our institution under suspicion of Sjögren's syndrome for medical advice. Two months ago she developed persistent bilateral parotid enlargement and a dry mouth. Her medical history revealed a weight loss due to "self-prescribed" reduce diet. Social questioning clarified high use of the media and influence on the body concept and self image. On extra oral examination, a diffuse parotid enlargement was seen bilaterally. The examination of the mouth showed a low moisture level of the intraoral mucosa. The unstimulated whole salivary flow rate was 2 ml in 15 min. Laboratory findings evidenced anemia (107 g/l). The serum albumin concentration indicated a reduced level (28 g/l). Search for antinuclear antibodies, anti-SSA antibodies, anti-SSB, -Sm, -RNP and anti-double-stranded DNA antibodies was negative. Evaluation for antibodies against hepatitis C, cytomegalovirus and Epstein-Barr virus infection and HIV rendered negative results. A histopathologic examination of labial salivary gland biopsy revealed a picture of sialoadenosis. From the above investigations, a diagnosis of sialoadenosis due to malnutrition was made. PMID:17198741

  16. Use of the loud sound stimulation test in diagnosis of semicircular canal dehiscence syndrome

    Microsoft Academic Search

    Ya-Feng Yu; Yi-Bo Zhang; Chun-Fu Dai; Fang-Lu Chi

    2011-01-01

    Semicircular canal dehiscence (SCD) syndrome is rare, and its diagnosis is a significant challenge in clinical practice. Our\\u000a aim was to explore application of the loud sound stimulation test for diagnosing SCD syndrome. Eight cases of superior semicircular\\u000a canal dehiscence (SSCD), among them two patients had bilateral dehiscences and one case of lateral semicircular canal dehiscence\\u000a (LSCD). A total of

  17. Evaluation of the loin pain\\/hematuria syndrome treated by renal autotransplantation or radical renal neurectomy

    Microsoft Academic Search

    AG Sheil; AK Chui; DJ Verran; J Boulas; LS Ibels

    1998-01-01

    Some patients with the loin pain\\/hematuria syndrome suffer incapacitating flank pain. No effective therapy has been reported. Uncertainty persists concerning the authenticity of the pain and the role of surgery in treatment. Forty-six patients with loin pain\\/hematuria syndrome and intractable pain were evaluated following treatment either by renal autotransplantation (30 patients, 10 bilaterally) or by renal denervation (20 patients, four

  18. Simultaneous bilateral elbow dislocation with bilateral medial epicondyle fractures in a 13-year-old female gymnast with hyperlaxity.

    PubMed

    Bauer, Stefan; Dunne, Ben; Whitewood, Colin

    2012-01-01

    Bilateral simultaneous elbow dislocations are extremely rare and have only been described in 12 cases. In the paediatric population unilateral elbow dislocations are rare with 3-6% of all elbow injuries and there are only few studies describing this injury exclusively in children. There is only one case report of a paediatric patient who sustained a simultaneous bilateral elbow dislocation with medial epicondyle fractures. We present a second paediatric case of simultaneous bilateral elbow dislocation with associated displaced bilateral medial epicondyle fractures in a gymnast with joint hyperlaxity (3 of 5 Wynne-Davies criteria) treated with closed reduction and short-term immobilisation (3 weeks). The patient returned to full trampoline gymnastics between 4 and 5 months postinjury and made an uneventful recovery. PMID:23234820

  19. The red ear syndrome

    PubMed Central

    2013-01-01

    Red Ear Syndrome (RES) is a very rare disorder, with approximately 100 published cases in the medical literature. Red ear (RE) episodes are characterised by unilateral or bilateral attacks of paroxysmal burning sensations and reddening of the external ear. The duration of these episodes ranges from a few seconds to several hours. The attacks occur with a frequency ranging from several a day to a few per year. Episodes can occur spontaneously or be triggered, most frequently by rubbing or touching the ear, heat or cold, chewing, brushing of the hair, neck movements or exertion. Early-onset idiopathic RES seems to be associated with migraine, whereas late-onset idiopathic forms have been reported in association with trigeminal autonomic cephalalgias (TACs). Secondary forms of RES occur with upper cervical spine disorders or temporo-mandibular joint dysfunction. RES is regarded refractory to medical treatments, although some migraine preventative treatments have shown moderate benefit mainly in patients with migraine-related attacks. The pathophysiology of RES is still unclear but several hypotheses involving peripheral or central nervous system mechanisms have been proposed. PMID:24093332

  20. Novel subtype of idiopathic bilateral vestibulopathy: bilateral absence of vestibular evoked myogenic potentials in the presence of normal caloric responses

    Microsoft Academic Search

    Chisato Fujimoto; Toshihisa Murofushi; Yasuhiro Chihara; Mitsuya Suzuki; Tatsuya Yamasoba; Shinichi Iwasaki

    2009-01-01

    To characterize clinical features of those patients who showed an absence of vestibular evoked myogenic potential (VEMP) responses\\u000a in the presence of normal caloric responses bilaterally, we reviewed clinical records of 1,887 consecutive outpatients who\\u000a complained of balance problems, and identified three patients, who showed absent VEMPs in the presence of normal caloric responses\\u000a bilaterally with unknown causes. All three

  1. Surgical correction of ptosis in ocular fibrosis syndrome.

    PubMed Central

    Liu, C; Ohri, R; Frongia, G; Collin, R

    1994-01-01

    The surgical management of ptosis is reported in seven patients suffering from the ocular fibrosis syndrome. Satisfactory results were obtained with bilateral Crawford type brow suspension with autologous fascia lata in six patients and bilateral Fox type brow suspension with stored fascia lata in a young child. As patients with ocular fibrosis syndrome usually exhibit little or no Bell's phenomenon, corneal exposure can become a problem after brow suspension. It was recommended that the lids are left just closed on the operating table at the end of the operation. None of the patients required a subsequent procedure to lower an overcorrection of the ptosis. The routine prescription of ocular lubricants for 2 months after ptosis correction is advocated. Urgent brow suspension in young children using non-autologous materials should only be considered if there is a risk of amblyopia. Images PMID:8199112

  2. Vascular Parkinsonism: Deconstructing a Syndrome.

    PubMed

    Vizcarra, Joaquin A; Lang, Anthony E; Sethi, Kapil D; Espay, Alberto J

    2015-06-01

    Progressive ambulatory impairment and abnormal white matter (WM) signal on neuroimaging come together under the diagnostic umbrella of vascular parkinsonism (VaP). A critical appraisal of the literature, however, suggests that (1) no abnormal structural imaging pattern is specific to VaP; (2) there is poor correlation between brain MRI hyperintensities and microangiopathic brain disease and parkinsonism from available clinicopathologic data; (3) pure parkinsonism from vascular injury ("definite" vascular parkinsonism) consistently results from ischemic or hemorrhagic strokes involving the SN and/or nigrostriatal pathway, but sparing the striatum itself, the cortex, and the intervening WM; and (4) many cases reported as VaP may represent pseudovascular parkinsonism (e.g., Parkinson's disease or another neurodegenerative parkinsonism, such as PSP with nonspecific neuroimaging signal abnormalities), vascular pseudoparkinsonism (e.g., akinetic mutism resulting from bilateral mesial frontal strokes or apathetic depression from bilateral striatal lacunar strokes), or pseudovascular pseudoparkinsonism (e.g., higher-level gait disorders, including normal-pressure hydrocephalus with transependimal exudate). These syndromic designations are preferable over VaP until pathology or validated biomarkers confirm the underlying nature and relevance of the leukoaraiosis. © 2015 International Parkinson and Movement Disorder Society. PMID:25997420

  3. Neuroimaging and clinical characterization of Sotos syndrome.

    PubMed

    Türkmen, S; ?ahin, S; Koçer, N; Peters, H; Mundlos, S; Tüysüz, B

    2015-01-01

    Sotos syndrome is a well-known overgrowth syndrome characterized by excessive growth during childhood, macrocephaly, distinctive facial appearance and learning disability. This disorder is caused by mutations or deletions in NSD1 gene. The aim of this study is to examine the relationship between the neuroimaging and clinical features of children with Sotos syndrome. Six Turkish children with Sotos syndrome were followed up about 3-7 years. The diagnosis was confirmed with molecular genetic analysis. We identified the pathogenic NSD1 mutation including three novel in all patients. All the patients had a characteristic facial gestalt of Sotos syndrome consisting of triangular face with prominent forehead, frontoparietal sparseness of hair and small nose. However, the degree of psychomotor and intellectual development was variable. Severe learning defect and speech delay were remarkable in two patients. The neuroimaging analysis showed abnormalities in four of six patients including bilateral large ventricles, thinning of the corpus callosum and persistent cavum septum pellucidum et vergae. Typical craniofacial appearance is the primary finding for the diagnosis of the disease even in the infantile period. However, the degree of psychomotor and intellectual development is very variable and does not correlate with the neuroimaging findings. PMID:26043501

  4. A case of prune belly syndrome.

    PubMed

    Xu, Wei; Wu, Hui; Wang, Dong-Xuan; Mu, Zhi-Hong

    2015-06-01

    Prune belly syndrome (PBS) is a rare congenital disorder characterized by deficient abdominal wall muscles, urinary tract malformation, and, in males, cryptorchidism. We present a case of PBS in China. The patient was a newborn baby boy who had wrinkled, "prune-like" abdominal skin, bilateral cryptorchidism, and urinary system malformation, complicated with hypoplasia of the lung and branch of the coronary artery-right ventricular fistula. His kidney function was inadequate. The patient subsequently died at age 28 days due to septicemia from a severe urinary tract infection. PMID:23639747

  5. Van der Woude Syndrome with Short Review of the Literature

    PubMed Central

    Deshmukh, Pallavi K.; Deshmukh, Kiran; Mangalgi, Anand; Patil, Subhash; Hugar, Deepa; Kodangal, Saraswathi Fakirappa

    2014-01-01

    Van der Woude syndrome (VWS) is a rare autosomal dominant condition with high penetrance and variable expression. Clinical manifestation of this autosomal dominant clefting syndrome includes bilateral midline lower lip pits, cleft lip, and cleft palate along with hypodontia. These congenital lip pits appear as a malformation in the vermilion border of the lip, with or without excretion. Discomfort caused by spontaneous or induced drainage of saliva/mucus when pressure is applied or during a meal as well as poor aesthetic match is one of the main complaints of patients with congenital lip fistula. The pits are treated by surgical resection. Dentists should be aware of the congenital lip pits as in Van der Woude syndrome because they have been reported to be associated with a variety of malformations or other congenital disorders. Here, the authors report a rare case of Van der Woude syndrome with short review of the literature. PMID:25050184

  6. Brown-McLean Syndrome in a Pediatric Patient

    PubMed Central

    Tourkmani, Abdo Karim; Martinez, Jaime D.; Berrones, David; Juárez-Domínguez, Brenda Y.; Beltrán, Francisco; Galor, Anat

    2015-01-01

    The purpose of this manuscript is to report the case of a 12-year-old patient who presented for routine ophthalmic examination after congenital cataract surgery performed at 2 months of age. The patient was diagnosed with bilateral Brown-McLean syndrome by slit lamp examination. No treatment was required because the patient was asymptomatic and had a clear central cornea. This is the first described case of Brown-McLean syndrome in a pediatric patient, representing the importance of clinical examination in the pediatric age group after cataract surgery because of the risk for patients of developing peripheral edema. PMID:26034485

  7. Acquired Brown's syndrome in a patient with systemic lupus erythematosus.

    PubMed Central

    Alonso-Valdivielso, J L; Alvarez Lario, B; Alegre López, J; Sedano Tous, M J; Buitrago Gómez, A

    1993-01-01

    A 27 year old woman with systemic lupus erythematosus (SLE) developed vertical diplopia with an apparent bilateral inferior oblique muscle palsy, resulting in a limitation of elevation of the globe in adduction. It resolved with systemic steroid treatment. A transient tenosynovitis affecting the superior oblique tendons was the probable underlying pathological mechanism. This is the first described case of Brown's syndrome associated with SLE. Images PMID:8427518

  8. Gluteal Compartment Syndrome following Vascular and Neurological Injuries

    PubMed Central

    Hafez, Mahmoud A.; Radwan, Moustafa

    2014-01-01

    Gluteal compartment syndrome is a serious but rare condition that has recently been increasingly reported in literature. This report presents two cases that followed neurological and vascular injuries (first case: an injury of the superior gluteal artery; second case: neurological injury bilaterally). A high index of suspicion and attention is needed to early diagnose this condition due to the possibility of developing serious and potentially fatal complications and difficulty in management. PMID:24711822

  9. Hughes-Stovin Syndrome and Massive Hemoptysis: A Management Challenge

    PubMed Central

    Al-Zeedy, Khalfan; Jayakrishnan, B.; Rizavi, Dawar; Alkaabi, Juma

    2015-01-01

    Hughes-Stovin syndrome is a very rare clinical entity characterized by pulmonary artery aneurysms and deep vein thrombosis (DVT). Here we report the case of a 53-year-old man, admitted to Sultan Qaboos University Hospital, Muscat, Oman, with bilateral pulmonary artery aneurysms and lower-limb DVT who developed massive hemoptysis. He was managed successfully with high-dose steroids in combination with cyclophosphamide. PMID:25830003

  10. [Livedoid vasculitis in a patient with antiphospholipid syndrome].

    PubMed

    Serra, Sara; Saavedra, M Joăo; Salvador, M Joăo; Reis, J Pedro; Malcata, A

    2010-01-01

    The authors present a clinical case of a 30 year old male patient admitted to the hospital for recurrent cyanosis and feet pain lasting for one year. In addition he presented bilateral purpuric lesions in the lateral maleolar region, one of which with ulceration. The finding of anticardiolipin antibodies associated with intraluminal thrombosis in the dermal vessels, allowed to conclude for Antiphospholipid syndrome. The cutaneous changes identified are named livedoid vasculitis. PMID:20711098

  11. Peripheral gangrene complicating idiopathic and recessive hemolytic uremic syndromes

    Microsoft Academic Search

    Bernard S. Kaplan; Clotilde D. Garcia; Russell W. Chesney; William E. Segar; Katia Giugno; Roberto Chem

    2000-01-01

    Three patients with hemolytic uremic syndrome (HUS) developed peripheral gangrene. Bilateral carotid artery thromboses occurred\\u000a in one of these patients after recovery from HUS. One patient had a long history of juvenile rheumatoid arthritis. In the\\u000a second patient, a flu-like illness preceded the onset of HUS. The third was one of two sisters, with the HUS appearing more\\u000a than 1

  12. [POEMS syndrome].

    PubMed

    Rose, C; Mahieu, M; Hachulla, E; Facon, T; Hatron, P Y; Bauters, F; Devulder, B

    1997-07-01

    POEMS syndrome is an acronym defined by Bardwick (Polyneuropathy, Organomegaly, Endocrinopathy, Monoclonal component and Skin changes). Other various clinical and biological features are reported: edema, cachexia, microangiopathic glomerulopathy, most rarely pulmonary hypertension, cutaneous necrosis. Thrombocytosis or polycythemia may be a prominent feature. POEMS syndrome is sometimes associated with lymphoproliferative disorder. Castelman-like disease is frequently observed as pathologic findings on lymph nodes. Distinction between POEMS syndrome and osteosclerotic myeloma is delicate. The rate of the monoclonal protein is modest-always less than 30 g/L-and is almost of the lambda light chain class. In contrast to multiple myeloma this syndrome is rarely associated with hypercalcemia, skeletal fracture, renal involvement and increasing of M component during evolution. Bone marrow plasmocytosis is usually less than 15% and the kinetic phenotype and genetic characteristics of the plasma cell remain those found in monoclonal gammopathy of undetermined significance. The pathophysiology of this syndrome remains largely unknown but overproduction of pro-inflammatory cytokines are reported, especially TNF alpha, IL-6 and IL-1 beta. Some clinical manifestations seem to be cytokine related. Polyneuropathy and cachexia are the main cause of death. A part corticosteroid and cure of solitary bone lesion, treatment is disappointing and survival is 60% at five years. PMID:9255373

  13. Sleep Disordered Breathing in Isolated Unilateral and Bilateral Diaphragmatic Dysfunction

    PubMed Central

    Khan, Akram; Morgenthaler, Timothy I.; Ramar, Kannan

    2014-01-01

    Introduction: The effect of isolated unilateral or bilateral diaphragmatic dysfunction (DD), in the absence of a generalized neuromuscular disorder, on sleep disordered breathing (SDB) is not well understood. The type of positive airway pressure (PAP) device needed to treat SDB in patients with isolated DD is also not well established. Methods: We retrospectively analyzed data on patients with isolated unilateral or bilateral DD who were referred for polysomnography (PSG) for clinical symptoms or abnormal oximetry between 1994 and 2006. Results: We found 66 patients who met criteria, of whom 74.2% were males with an average age of 58.8 ± 10.9 years. 56 had isolated unilateral DD, and 10 had isolated bilateral DD. All had significant SDB with an apnea-hypopnea index (AHI) of 26.6 ± 28.4. There were no significant differences in PSG measures, arterial blood gas analysis, pulmonary function tests, or echocardiographic data, except for lower maximal inspiratory pressure in patients with bilateral DD compared to unilateral DD (40.2% ± 17.8% vs. 57.7% ± 20.5%, p = 0.02). Control of SDB with continuous PAP (CPAP) was possible in only 37.9% of patients with the rest requiring bilevel PAP (BPAP). Patients with isolated bilateral DD and SDB were 6.8 times more likely to fail CPAP than those with unilateral DD (p = 0.03). Conclusions: Most patients with isolated DD failed CPAP and required BPAP. Patients with bilateral DD were more likely to require BPAP than those with unilateral DD. Patients with isolated DD should be considered for in-lab titration to determine adequacy of therapy. Citation: Khan A, Morgenthaler TI, Ramar K. Sleep disordered breathing in isolated unilateral and bilateral diaphragmatic dysfunction. J Clin Sleep Med 2014;10(5):509-515. PMID:24910552

  14. Spontaneous simultaneous bilateral malignant glaucoma of a patient with no antecedent history of medical or surgical eye diseases

    PubMed Central

    Jarade, Elias F; Dirani, Ali; Jabbour, Elyse; Antoun, Joelle; Tomey, Karim F

    2014-01-01

    Purpose Malignant glaucoma, or aqueous misdirection syndrome, is a condition characterized by sudden intraocular pressure (IOP) elevation, and it is usually unilateral and induced by ocular surgical intervention or by medical therapy. Here, we report a case of simultaneous bilateral malignant glaucoma in a young patient with no history of any ocular diseases. Case report A case of a 24-year-old female with no apparent previous history of ocular medical or surgical conditions was referred to our hospital because of recent bilateral IOP elevation associated with a severe drop in vision and shallow anterior chamber with no posterior segment anomalies detected by ocular ultrasound in both eyes. Yttrium aluminum garnet (YAG) laser iridotomy dropped the IOP only temporarily and the patient received topical atropine treatment with combined trabeculectomy and anterior vitrectomy. Results In this case, the patient had a typical presentation of bilateral malignant glaucoma and her IOP dropped only temporarily following laser iridotomy to rise again shortly thereafter. Also, deepening of the anterior chamber and IOP decrease after topical atropine was very supportive of the diagnosis of malignant glaucoma. Successful management with trabeculectomy and limited vitrectomy also affirmed the diagnosis of malignant glaucoma. Conclusion This is a very rare case of bilateral malignant glaucoma in a young adult without any prior eye conditions; only one similar case has been reported in the literature. We propose our own theory regarding this simultaneous occurrence of the pathology based on previously published studies about the presence of communication between the two eyes along the cerebrospinal fluid pathways. PMID:24920881

  15. MRI findings in Tolosa–Hunt syndrome before and after systemic corticosteroid therapy

    Microsoft Academic Search

    Sinan Çakirer

    2003-01-01

    Tolosa–Hunt syndrome (THS) is characterized by painful ophthalmoplegia due to a granulomatous inflammation in the cavernous sinus. Corticosteroid therapy dramatically resolves both the clinical and radiological findings of THS. We present MRI findings of six patients with a clinical history of at least one episode of unilateral or bilateral orbital-periorbital pain, clinical findings of associated paresis of one or more

  16. Metrorrhagia and precocious puberty revealing primary hypothyroidism in a child with Down’s syndrome

    Microsoft Academic Search

    W Chemaitilly; C Thalassinos; S Emond; E Thibaud

    2003-01-01

    We report a child with Down’s syndrome in whom metrorrhagia and precocious puberty revealed primary autoimmune hypothyroidism. The patient had a decreased growth velocity, exaggerated weight gain, bone age delay, and bilaterally enlarged multicystic ovaries. Delays in the diagnosis and treatment of hypothyroidism can lead to this peculiar presentation.

  17. Synovial osteochondromatosis in hereditary arthro-ophthalmopathy (Wagner-Stickler syndrome)

    Microsoft Academic Search

    Bernhard Tins; Victor Cassar-Pullicino

    2003-01-01

    A case of bilateral synovial osteochondromatosis in a patient with hereditary arthro-ophthalmopathy is presented. The osteochondral lesions were largely calcified in one joint and largely chondromatous in the other. Typical features of hereditary arthro-ophthalmopathy are reviewed and it is hypothesised that the abnormal collagen in this syndrome is responsible for the development of synovial osteochondromatosis. Synovial manifestations of skeletal dysplasias

  18. Slowly progressive Foix-Chavany-Marie syndrome as a precursor of a primary progressive aphasia

    Microsoft Academic Search

    Ingo Uttner; Johannes Brettschneider; Alexander Unrath; Axel Riecker

    Foix-Chavany-Marie syndrome (FCMS) is a rare neurological condition usually associated with bilateral ischemic lesions of the anterior frontoparietal operculum. Here, we present a patient with slowly progressive FCMS owing to focal brain atrophy who developed a language disorder similar to that seen in a primary progressive aphasia.

  19. Slowly progressive Foix-Chavany-Marie syndrome as a precursor of a primary progressive aphasia.

    PubMed

    Uttner, Ingo; Brettschneider, Johannes; Unrath, Alexander; Riecker, Axel

    2012-05-01

    Foix-Chavany-Marie syndrome (FCMS) is a rare neurological condition usually associated with bilateral ischemic lesions of the anterior frontoparietal operculum. Here, we present a patient with slowly progressive FCMS owing to focal brain atrophy who developed a language disorder similar to that seen in a primary progressive aphasia. PMID:22321363

  20. Mastoid pneumatization and aging in children with Pierre-Robin syndrome and in the cleft palate population out of syndrome

    Microsoft Academic Search

    J. Handži?-?uk; V. ?uk; M. Gluhini?

    1999-01-01

    We examined the characteristics of mastoid pneumatization in the Pierre-Robin syndrome (PRS) and non-PRS cleft palate population\\u000a in relation to age. There were 14 patients with PRS (median age, 5 years), 7 patients with bilateral cleft lip-palates (BCLP:\\u000a median age, 6 years), 29 patients with unilateral cleft lip-palates (UCLP: median age, 6 years) and 15 patients with isolated\\u000a cleft palates

  1. Crush syndrome.

    PubMed

    Gonzalez, Dario

    2005-01-01

    The assessment, management, and treatment of the entrapped victim are critical skills needed to ensure a successful outcome. Individuals have been trapped in the "rubble" for even short periods of time only to succumb to predictable consequences of muscle compression injury. The clinician should be prepared to address issues of crush syndrome (including compartment syndrome) proactively and aggressively. The history of this disease is clear and well documented both in the military literature and in the earthquake rescue reviews. The key to management is managing and predicting clinical conditions before they present themselves. The potential exists in the urban environment (with the potential of building collapses) to have patients with crush syndrome that far exceed local medical capabilities should be part of modern disaster planning. This article reviews the various body systems and presents management and assessment strategies for the clinician. PMID:15640677

  2. [Kallmann syndrome].

    PubMed

    Mokosch, A; Bernecker, C; Willenberg, H S; Neumann, N J

    2011-10-01

    The Kallmann syndrome is a very rare congenital association of gonadotropin-releasing hormone deficiency and hyposmia or anosmia. Clinically it is characterized by low serum concentrations of testosterone and inadequate low levels of luteinizing hormone and follicle-stimulating hormone as well as incomplete sexual maturation, lack of secondary sexual features (facial and body hair growth, deepening of the voice), micropenis and sometimes even cryptorchidism. The reduced or absent sense of smell is typical for the Kallmann syndrome and distinguishes this syndrome from other causes of hypogonadotropic hypogonadism. Additional findings may include synkinesia, hearing loss, unilateral renal aplasia, brachy- or syndactyly, agenesis of corpus callosum, cleft palate and dental agenesis. A 19-year-old man presented to our male infertility clinic with delayed sexual maturation, eunuchoid habitus, micropenis, cryptorchidism, erectile dysfunction and absence of ejaculation, anemia and osteoporosis as well as low serum concentrations of luteinizing hormone, follicle-stimulating hormone and testosterone in combination with hyposmia. PMID:21918848

  3. Compartment syndromes

    NASA Technical Reports Server (NTRS)

    Mubarak, S. J.; Pedowitz, R. A.; Hargens, A. R.

    1989-01-01

    The compartment syndrome is defined as a condition in which high pressure within a closed fascial space (muscle compartment) reduces capillary blood perfusion below the level necessary for tissue viability'. This condition occurs in acute and chronic (exertional) forms, and may be secondary to a variety of causes. The end-result of an extended period of elevated intramuscular pressure may be the development of irreversible tissue injury and Volkmann's contracture. The goal of treatment of the compartment syndrome is the reduction of intracompartmental pressure thus facilitating reperfusion of ischaemic tissue and this goal may be achieved by decompressive fasciotomy. Controversy exists regarding the critical pressure-time thresholds for surgical decompression and the optimal diagnostic methods of measuring intracompartmental pressures. This paper will update and review some current knowledge regarding the pathophysiology, aetiology, diagnosis, and treatment of the acute compartment syndrome.

  4. Bilateral dorsolateral thalamic lesions disrupts conscious recollection.

    PubMed

    Edelstyn, Nicola M J; Hunter, Ben; Ellis, Simon J

    2006-01-01

    In an earlier study we disputed the claim that the mediodorsal thalamic nucleus is critical for familiarity. We reported patient (QX) who showed a severe deficit in conscious recollection, and behavioural problems (disinhibition, emotional lability) with relative sparing of familiarity-aware memory following a left mediodorsal thalamic lesion. More recent MR imaging has revealed that QX's lesions are more extensive than previously reported and involve both dorsolateral thalamic nuclei, and whilst there is evidence of left mediodorsal thalamic damage, it is not the main focus of damage. This paper reports a full analysis of QX's thalamic pathology alongside a more detailed investigation of his recognition memory, using yes/no and forced-choice procedures, and executive function. The results revealed impairments in yes/no recognition and conscious recollection rates of famous, artist and unknown names. In addition to the previously noted behavioural disinhibition and emotional lability, a deficit in spontaneous planning ability was evident on the Zoo Map Test (subtest of the Bahavioural Assessment of the Dysexecutive Syndrome). Forced-choice recognition, familiarity estimates and remote memory showed higher levels of preservation. The findings indicate that the dorsolateral thalamus is part of the extended hippocampal circuit which is causally critical only for recall and conscious recollection of complex associations rather than for the more automatic processes linked with novelty detection. PMID:16253293

  5. Flammer syndrome

    PubMed Central

    2014-01-01

    The new term Flammer syndrome describes a phenotype characterized by the presence of primary vascular dysregulation together with a cluster of symptoms and signs that may occur in healthy people as well as people with disease. Typically, the blood vessels of the subjects with Flammer syndrome react differently to a number of stimuli, such as cold and physical or emotional stress. Nearly all organs, particularly the eye, can be involved. Although the syndrome has some advantages, such as protection against the development of atherosclerosis, Flammer syndrome also contributes to certain diseases, such as normal tension glaucoma. The syndrome occurs more often in women than in men, in slender people than in obese subjects, in people with indoor rather than outdoor jobs, and in academics than in blue collar workers. Affected subjects tend to have cold extremities, low blood pressure, prolonged sleep onset time, shifted circadian rhythm, reduced feeling of thirst, altered drug sensitivity, and increased general sensitivity, including pain sensitivity. The plasma level of endothelin-1 is slightly increased, and the gene expression in lymphocytes is changed. In the eye, the retinal vessels are stiffer and their spatial variability larger; the autoregulation of ocular blood flow is decreased. Glaucoma patients with Flammer syndrome have an increased frequency of the following: optic disc hemorrhages, activated retinal astrocytes, elevated retinal venous pressure, optic nerve compartmentalization, fluctuating diffuse visual field defects, and elevated oxidative stress. Further research should lead to a more concise definition, a precise diagnosis, and tools for recognizing people at risk. This may ultimately lead to more efficient and more personalized treatment. PMID:25075228

  6. Serotonin syndrome.

    PubMed

    Bodner, R A; Lynch, T; Lewis, L; Kahn, D

    1995-02-01

    We describe a patient treated with trazodone, isocarboxazid, and methylphenidate hydrochloride who developed confusion, agitation, poor concentration, rigidity, myoclonus, involuntary movements, orthostatic hypotension, and hyperreflexia. CK was normal, and the syndrome resolved spontaneously over 12 hours. The serotonin syndrome occurs following the use of serotomimetic agents (serotonin reuptake inhibitors, tricyclic and tetracyclic antidepressants, tryptophan, 3,4-methylenedioxy-methamphetamine, dextromethorphan, meperidine, S-adenosylmethionine) alone or in combination with monoamine oxidase inhibitors. It is characterized by various combinations of myoclonus, rigidity, hyperreflexia, shivering, confusion, agitation, restlessness, coma, autonomic instability, low-grade fever, nausea, diarrhea, diaphoresis, flushing, and rarely, rhabdomyolysis and death. PMID:7854515

  7. Unilateral versus Bilateral Instrumentation in Spinal Surgery: A Systematic Review

    PubMed Central

    Molinari, Robert W.; Saleh, Ahmed; Molinari, Robert; Hermsmeyer, Jeff; Dettori, Joseph R.

    2015-01-01

    Study Design?Systematic review. Clinical Questions?(1) What is the comparative efficacy of unilateral instrumentation compared with bilateral instrumentation in spine surgery? (2) What is the safety of unilateral instrumentation compared with bilateral instrumentation in spine surgery? Methods?Electronic databases and reference lists of key articles were searched up to September 30, 2014, to identify studies reporting the comparative efficacy and safety of unilateral versus bilateral instrumentation in spine surgery. Studies including recombinant human bone morphogenetic protein 2 as adjunct therapy and those with follow-up of less than 2 years were excluded. Results?Ten randomized controlled trials met the inclusion criteria: five compared unilateral with bilateral instrumentation using open transforaminal or posterior lumbar interbody fusion (TLIF/PLIF), one used open posterolateral fusion, and four used minimally invasive TLIF/PLIF. There were no significant differences between unilateral and bilateral screw instrumentation with respect to nonunion, low back or leg pain scores, Oswestry Disability Index, reoperation, or complications. Conclusions?The existing literature does not identify significant differences in clinical outcomes, union rates, and complications when unilateral instrumentation is used for degenerative pathologic conditions in the lumbar spine. The majority of published reports involve single-level lumbar unilateral instrumentation.

  8. Cryptococcal meningitis presenting with bilateral complete ophthalmoplegia: a case report

    PubMed Central

    2014-01-01

    Background Cryptococcus neoformans is saprophytic encapsulated yeast. Infection is acquired by inhalation of the organism and could be asymptomatic or limited to the lungs, specially in the immunocompetent host. Cryptococcal meningitis is a serious opportunistic infection among post transplant recipients. Cranial nerve palsies and ophthalmoplegia are well known complications of this disease, but bilateral complete ophthalmoplegia is a very rare presentation. Case Presentation A Sri Lankan young male, who is a post kidney transplant recipient, presented with bilateral complete ophthalmoplegia and subsequently was diagnosed to have cryptococcal meningitis based on Indian ink stain and culture of cerebrospinal fluid (CSF). His magnetic resonance imaging (MRI) showed bilateral multiple nodular lesions in both basal ganglia and thalami. Brainstem imaging was normal. Conclusions Cryptococcal meningitis is a serious fungal infection in post transplant patients. It should be suspected in any immunocompromised patient with fever, headache and focal neurological signs. Bilateral thalamic lesions, inflammation and invasion of the cranial nerves and raised intracranial pressure were thought to be possible mechanisms resulting in bilateral complete ophthalmoplegia in this patient. PMID:24885277

  9. Severe early bilateral macular edema following fingolimod therapy.

    PubMed

    Coppes, Oscar Jim Michael; Gutierrez, Ismael; Reder, Anthony T; Ksiazek, Susan; Bernard, Jacqueline

    2013-07-01

    We report a case of bilateral macular edema (ME) within 10 days of starting fingolimod 0.5mg therapy in a patient with Multiple Sclerosis (MS). The complication resolved without treatment as demonstrated by sequential Optical Coherence Tomography (OCT). Fingolimod is a sphingosine-1-phosphate receptor modulator that reduces lymphocyte presence in the CNS. In pivotal trials, ME, a known complication of fingolimod, typically occurred unilaterally with onset at approximately 3 months. A 60y/o AA female, diagnosed with MS in 1977, started oral fingolimod treatment on 05/31/2011. Baseline screening with OCT and ophthalmology evaluation showed no ME. On 06/10, she developed bilateral blurry vision and discontinued fingolimod. On 06/27, OCT revealed severe bilateral ME. Later OCT exams showed a progressive decrease in Central Foveal Thickness (CFT) and Macular Volume (MV), without specific treatment other than discontinuation of fingolimod. On 7/27, CFT, MV, and Visual Acuity (VA) were similar to baseline. This is the first reported case of bilateral, early onset ME following fingolimod treatment at the current FDA-approved dose of 0.5mg. Diabetes, a known risk factor for ME, may have contributed to her early, bilateral involvement. Our case provides further support for earlier OCT, in conjunction with ophthalmic examinations, for at-risk patients on fingolimod, and suggests that cessation of fingolimod may be associated with resolution of ME. PMID:25877733

  10. Bilateral anterior uveitis in a patient with bacterial meningitis.

    PubMed

    Otomo, Kazuyoshi; Kaburaki, Toshikatsu; Shigeeda, Takashi; Takamoto, Mituko; Kawashima, Hidetoshi; Araie, Makoto

    2012-08-01

    We report a case of bilateral iridocyclitis accompanied by bacterial meningitis in an immunocompetent patient. Case report. A 48 year-old healthy female visited our hospital with strong headache, fever, bilateral hyperemia, and blurred vision in both eyes. A slit-lamp examination revealed moderate cells and flare in the anterior chamber of both eyes, with fine keratoprecipitates. There were no obvious inflammatory changes in the vitreous, retina, and optic disc of both eyes. Elevation of peripheral blood white blood cells, C-reactive protein, and an elevated number of cerebrospinal fluid (CSF) cells suggested bacterial meningitis. The patient was admitted to our hospital and received intravenous antibiotics. Finally, a CSF culture revealed infection with gram-positive rods, suspected Listeria monocytogenes, confirming bacterial meningitis. For iridocyclitis, we prescribed betamethasone eyedrops and 0.5 % tropicamide eyedrops with intravenous adminstration of systemic antibiotics. 3 days later, her headache and bilateral hyperemia disappeared. This case is better described as sterile reactive uveitis rather than endogenous bacterial endophthalmitis, because bilateral anterior uveitis was resolved without chronic uveitis, iris atrophy, and vitreous opacity. When clinicians see patients with meningitis and bilateral anterior uveitis, sterile reactive uveitis should be considered in the differential diagnosis of uveitis. PMID:22638923

  11. Polymicrogyria and deletion 22q11.2 syndrome: window to the etiology of a common cortical malformation.

    PubMed

    Robin, Nathaniel H; Taylor, Clare J; McDonald-McGinn, Donna M; Zackai, Elaine H; Bingham, Peter; Collins, Kevin J; Earl, Dawn; Gill, Deepak; Granata, Tiziana; Guerrini, Renzo; Katz, Naomi; Kimonis, Virginia; Lin, Jean-Pierre; Lynch, David R; Mohammed, Shehla N; Massey, Roger F; McDonald, Marie; Rogers, R Curtis; Splitt, Miranda; Stevens, Cathy A; Tischkowitz, Marc D; Stoodley, Neil; Leventer, Richard J; Pilz, Daniela T; Dobyns, William B

    2006-11-15

    Several brain malformations have been described in rare patients with the deletion 22q11.2 syndrome (DEL22q11) including agenesis of the corpus callosum, pachygyria or polymicrogyria (PMG), cerebellar anomalies and meningomyelocele, with PMG reported most frequently. In view of our interest in the causes of PMG, we reviewed clinical data including brain-imaging studies on 21 patients with PMG associated with deletion 22q11.2 and another 11 from the literature. We found that the cortical malformation consists of perisylvian PMG of variable severity and frequent asymmetry with a striking predisposition for the right hemisphere (P = 0.008). This and other observations suggest that the PMG may be a sequela of abnormal embryonic vascular development rather than a primary brain malformation. We also noted mild cerebellar hypoplasia or mega-cisterna magna in 8 of 24 patients. Although this was not the focus of the present study, mild cerebellar anomalies are probably the most common brain malformation associated with DEL22q11. PMID:17036343

  12. A case of cochlear implantation in a patient with Epstein syndrome.

    PubMed

    Nabekura, Takashi; Nagano, Yuki; Matsuda, Keiji; Tono, Tetsuya

    2015-04-01

    Epstein syndrome is a rare autosomal dominant platelet disorder characterized by thrombocytopenia, giant platelets, and sensorineural hearing loss. It is included among four overlapping syndromes, the others being May-Hegglin anomaly, Fechtner syndrome, and Sebastian syndrome. It is now established that all four disorders are caused by mutations in the MYH9 gene. We report the case of a patient with Epstein syndrome in whom bilateral profound hearing impairment developed and cochlear implantation was carried out. A cochlear implant was successfully used with a speech discrimination score of 100% on a Japanese sentence recognition test. This report offers the second description of the performance of a cochlear implant in a patient with Epstein syndrome. This case study may offer hope for patients and their family members with this kind of mutation. PMID:25293679

  13. Prune Belly syndrome: A rare case report

    PubMed Central

    Samal, Sunil Kumar; Rathod, Setu

    2015-01-01

    Prune Belly syndrome (PBS) is a rare congenital anomaly of uncertain etiology almost exclusive to males. We report a case of term male baby born to a 39-year-old grand multipara with previous four normal vaginal births. There was no history of genetic or congenital anomaly in her family. Examination of the baby revealed hypotonia, deficient abdominal muscle, cryptorchidism, palpable kidney, and bladder. Ultrasound examination of the abdomen revealed bilateral gross hydronephrosis and megaureter. Provisional diagnosis of PBS was made and the baby was admitted in neonatal intensive care units for further management. Routine antenatal care with ultrasonography will help in detecting renal anomalies, which can be followed postnatally. Early diagnosis of this syndrome and determining its optimal treatment are very important in helping to avoid its fatal course. PMID:25810678

  14. Repair of bilateral cleft lip and its variants

    PubMed Central

    Mulliken, John B.

    2009-01-01

    The surgeon who lifts a scalpel to repair a bilateral cleft lip and nasal deformity is accountable for: 1) precise craftsmanship based on three-dimensional features and four-dimensional changes; 2) periodic assessment throughout the child's growth; and 3) technical modifications during primary closure based on knowledge gained from long-term follow-up evaluation. These children should not have to endure the stares prompted by nasolabial stigmata that result from outdated concepts and technical misadventures. The principles for repair of bilateral complete cleft lip have evolved to such a level that the child's appearance should be equivalent to, or surpass, that of a unilateral complete cleft lip. These same principles also apply to the repair of the variants of bilateral cleft lip, although strategies and execution differ slightly. PMID:19884685

  15. Bilateral foot pain in a collegiate distance runner.

    PubMed

    Denegar, C R; Siple, B J

    1996-01-01

    A 19-year-old, white female, college freshman cross-country runner presented to the athletic training center complaining of bilateral plantar foot pain 1 week into the cross-country season. She had been treated for bilateral plantar fasciitis twice in the previous 3 years, and initial evaluation suggested a recurrence. A failure to respond to treatment led us to suspect other causes. Subsequent diagnostic imaging (bone scan, CT scan) revealed bilateral navicular stress fractures. Navicular stress fractures are probably more common than once believed. This case study describes the course of evaluation and treatment in relation to the common presentation, etiology, problems of diagnosis, and treatment of navicular stress fractures. PMID:16558375

  16. Multiple Symmetric Lipomatosis (Madelung's Disease) Presenting as Bilateral Huge Gynecomastia

    PubMed Central

    Jang, Jae Hoon; Lee, Anbok; Han, Sang-Ah; Ryu, Jung-Kyu

    2014-01-01

    Multiple symmetric lipomatosis (MSL), or Madelung's disease, is a rare disease of unknown etiology. It is characterized by the presence of loose adipose tissue deposits localized in the cervical region and upper body. MSL presenting as bilateral huge gynecomastia is an extremely rare phenomenon. The present report describes a case of MSL in a 66-year-old man. The patients presented with bilateral breast bulging. He had a history of cigarette and alcohol use. His condition was treated with a bilateral nipple-sparing mastectomy. MSL can present as a form of gynecomastia, for its accurate diagnosis and proper treatment of MSL, increasing awareness of the clinical characteristics of the disease is required, especially amongst breast surgeons. Herein, we review the literature and discuss the clinical characteristics, pathology, and surgical treatment of MSL. PMID:25548590

  17. Idiopathic bilateral adrenal haemorrhage related to acute adrenal insufficiency

    PubMed Central

    Ogino, Jun; Toda, Joe; Onitsuka, Shiro; Hashimoto, Naotake

    2013-01-01

    A 54-year-old woman presented with sudden epigastralgia and left back pain. She had no significant history. Laboratory data showed mild inflammation and no liver or renal dysfunction. Abdominal CT showed left adrenal enlargement and haemorrhage. Hydrocortisone therapy was started to prevent adrenal insufficiency before laboratory findings for ACTH (adrenocorticotropic hormone) and cortisol levels. On the second hospital day, abdominal CT showed additional right adrenal enlargement and haemorrhage. The serum cortisol level suggested adrenal insufficiency. No specific findings were detected by bilateral adrenal angiography. 6 to 12?months later, abdominal CT showed decreased bilateral adrenal haemorrhage. This case illustrates the importance of prompt diagnosis and treatment of acute adrenal insufficiency, and shows sequential changes in the size of bilateral adrenal haemorrhage. Rapid corticosteroid replacement is important if acute adrenal insufficiency is suspected. In a case with unilateral adrenal haemorrhage, the possibility of additional adrenal haemorrhage on the opposite side should also be considered. PMID:23729712

  18. Safety of bilateral intravitreal injections delivered in a teaching institution.

    PubMed

    Chao, Daniel L; Gregori, Ninel Z; Khandji, Joyce; Goldhardt, Raquel

    2014-07-01

    Intravitreal injection is one of the most common in-office procedures performed in ophthalmic practices. In teaching institutions such as the Veterans Affairs (VA) Hospitals, patient care is delivered by physicians-in-training, while mastering intravitreal injection technique. Infectious endophthalmitis and visual loss are the most feared complications of intravitreal injections, especially in the context of recent outbreaks caused by contaminated compounded medications. Ophthalmologists and ophthalmic educators increasingly face the dilemma of timing as well as balancing the risks and benefits of bilateral treatments required by many patients. In this editorial, we discuss published reports of bilateral injections, summarize our experience with bilateral intravitreal injections in a teaching setting at the Miami VA Hospital and list our recommendations for minimizing the risk of infectious endophthalmitis. PMID:24815986

  19. Poor outcome associated with probable bilateral extracranial ICA vasospasm

    PubMed Central

    Sawa, Nobufusa Nobuhiro; Kataoka, Hiroshi; Ueno, Satoshi

    2013-01-01

    We describe a woman with bilateral extracranial internal carotid artery (ICA) vasospasm. Initial MRI of the brain showed multiple areas of high-signal intensity in the white matter of both the frontoparietal lobes. She was alert, and the muscle strength of the four extremities was moderately decreased. Cranial CT on day 3 showed increased numbers of low-density areas in both the anterior cerebral arteries (ACA) and middle cerebral arteries (MCA), accompanied by neurological deterioration. Cranial and cervical CT angiography on day 9 showed that all areas of both the ACA and MCA had become low density. Both ICAs were markedly narrowed along their entire length and tapered. On day 16, the patient died. Three patients with bilateral extracranial ICA spasms have been described previously. To our knowledge, this is the first time to document bilateral ICA spasm causing elongated narrowing of the carotid arteries, leading to an unfortunate outcome. PMID:23749861

  20. [Bilateral tuberculous epididymitis after intravesical Bacillus Calmette-Guerin therapy].

    PubMed

    Shigehara, Kazuyoshi; Kobori, Yoshitomo; Amano, Toshiyasu; Takemae, Katuro

    2005-12-01

    We describe a case of bilateral tuberculous epididymitis that occurred two weeks after intravesical Bacillus Calmette-Guerin (BCG) instillation. A 72-year-old man received transuretheral resection of bladder transitional cell carcinoma in November 2000. Although he had no recurrence for about 4 years, cystoscopy revealed small papillary tumors in the bladder in September 2004. A course of 8 weekly intravesical instillations of BCG was started. After the second BCG instillation (160 mg) he had bilateral painful scrotal swelling. Although he was administered isoniazid (INH) and rifampicin (RFP), scrotal swelling got worse. Right orchiectomy and left epididymectomy was performed in December 2004. Histological diagnosis was bilateral tuberculous epididymitis. Postoperatively, he was administered INH and RFP and had no recurrence for 3 months. PMID:16440736

  1. Marfan Syndrome

    MedlinePLUS

    ... that determine whether you have blue eyes or brown or whether you look like your mom or your dad. But sometimes genes can pass on some not-so-great things, too, like certain illnesses and diseases. In most cases, the gene change for Marfan syndrome runs in families, getting passed down to children ...

  2. Hypereosinophilic syndromes

    Microsoft Academic Search

    Florence E Roufosse; Michel Goldman; Elie Cogan

    2007-01-01

    Hypereosinophilic syndromes (HES) constitute a rare and heterogeneous group of disorders, defined as persistent and marked blood eosinophilia (> 1.5 × 109\\/L for more than six consecutive months) associated with evidence of eosinophil-induced organ damage, where other causes of hypereosinophilia such as allergic, parasitic, and malignant disorders have been excluded. Prevalence is unknown. HES occur most frequently in young to

  3. Aicardi Syndrome

    MedlinePLUS

    ... seen in boys born with an extra "X" chromosome. (Females have two X chromosomes, while males normally have an X and a Y chromosome.) The precise gene or genetic mechanism causing Aicardi syndrome is ... callosum ) that links the two halves of the brain (2) infantile spasms (a ...

  4. Exfoliation Syndrome

    Microsoft Academic Search

    Robert Ritch; Ursula Schlötzer-Schrehardt

    2001-01-01

    Exfoliation syndrome (XFS) is an age-related disease in which abnormal fibrillar extracellular material is produced and accumulates in many ocular tissues. Its ocular manifestations involve all of the structures of the anterior segment, as well as conjunctiva and orbital structures. Glaucoma occurs more commonly in eyes with XFS than in those without it; in fact, XFS has recently been recognized

  5. Wells syndrome

    PubMed Central

    Bansal, Manish; Rai, Tulika; Pandey, Shyam S.

    2012-01-01

    Wells syndrome or eosinophilic cellulitis is characterized clinically by an acute dermatitis resembling cellulitis and histopathologically by dermal eosinophilic infiltration. Various morphological presentations have been described. We report a 32-year-old female with recurrent, erythematous plaques on left forearm of 8 months duration, associated with mild itching that resolved leaving mild hyperpigmentation. PMID:23189251

  6. Rett Syndrome.

    ERIC Educational Resources Information Center

    Culbert, Linda A.

    This pamphlet reviews the historical process involved in initially recognizing Rett Syndrome as a specific disorder in girls. Its etiology is unknown, but studies have considered factors as hyperammonemia, a two-step mutation, a fragile X chromosome, metabolic disorder, environmental causation, dopamine deficiency, and an inactive X chromosome.…

  7. Rud's syndrome.

    PubMed

    Pavani, K; Reddy, B S N; Singh, B Amar

    2014-04-01

    Rud's syndrome is a rare autosomal recessive hereditary disorder characterized by congenital ichthyosis, epilepsy, dwarfism, sexual infantilism, polyneuritis, and macrocytic anemia. We report here an interesting case of this disorder in an 18-year-old girl for its rarity and academic interest. PMID:24860755

  8. Rud's syndrome

    PubMed Central

    Pavani, K.; Reddy, B. S. N.; Singh, B. Amar

    2014-01-01

    Rud's syndrome is a rare autosomal recessive hereditary disorder characterized by congenital ichthyosis, epilepsy, dwarfism, sexual infantilism, polyneuritis, and macrocytic anemia. We report here an interesting case of this disorder in an 18-year-old girl for its rarity and academic interest. PMID:24860755

  9. Sotos syndrome

    PubMed Central

    Baujat, Genevičve; Cormier-Daire, Valérie

    2007-01-01

    Sotos syndrome is an overgrowth condition characterized by cardinal features including excessive growth during childhood, macrocephaly, distinctive facial gestalt and various degrees of learning difficulty, and associated with variable minor features. The exact prevalence remains unknown but hundreds of cases have been reported. The diagnosis is usually suspected after birth because of excessive height and occipitofrontal circumference (OFC), advanced bone age, neonatal complications including hypotonia and feeding difficulties, and facial gestalt. Other inconstant clinical abnormalities include scoliosis, cardiac and genitourinary anomalies, seizures and brisk deep tendon reflexes. Variable delays in cognitive and motor development are also observed. The syndrome may also be associated with an increased risk of tumors. Mutations and deletions of the NSD1 gene (located at chromosome 5q35 and coding for a histone methyltransferase implicated in transcriptional regulation) are responsible for more than 75% of cases. FISH analysis, MLPA or multiplex quantitative PCR allow the detection of total/partial NSD1 deletions, and direct sequencing allows detection of NSD1 mutations. The large majority of NSD1 abnormalities occur de novo and there are very few familial cases. Although most cases are sporadic, several reports of autosomal dominant inheritance have been described. Germline mosaicism has never been reported and the recurrence risk for normal parents is very low (<1%). The main differential diagnoses are Weaver syndrome, Beckwith-Wiedeman syndrome, Fragile X syndrome, Simpson-Golabi-Behmel syndrome and 22qter deletion syndrome. Management is multidisciplinary. During the neonatal period, therapies are mostly symptomatic, including phototherapy in case of jaundice, treatment of the feeding difficulties and gastroesophageal reflux, and detection and treatment of hypoglycemia. General pediatric follow-up is important during the first years of life to allow detection and management of clinical complications such as scoliosis and febrile seizures. An adequate psychological and educational program with speech therapy and motor stimulation plays an important role in the global development of the patients. Final body height is difficult to predict but growth tends to normalize after puberty. PMID:17825104

  10. Recurrent bilateral varicose veins secondary to tricuspid regurgitation

    Microsoft Academic Search

    S. A. Badger; R. R. Makar; E. W. Chew; B. Lee

    Background  Varicose veins are a common condition. We present a case of recurrent veins due to tricuspid regurgitation.\\u000a \\u000a \\u000a \\u000a Case history  A 55-year-old female presented with large bilateral varicosities. On examination these were extensive and pulsatile in nature\\u000a over both legs. Three and 5 years previously she presented with similar signs and had undergone bilateral venous surgery including\\u000a Trendelenburg procedure. Past medical history included

  11. Bilateral muscular tinnitus due to myoclonus of extrinsic auricular muscles.

    PubMed

    Lee, Kijeong; Chang, Jiwon; Park, Sangheon; Im, Gi Jung; Choi, Hyung Joon; Kim, Jin Hwan; Kim, Hyung-Jong

    2015-04-01

    The muscular tinnitus due to an extrinsic auricular myoclonus is an extremely rare disorder which demonstrates a semirhythmic involuntary movement of the ear. We report a 33-year-old man with clicking tinnitus caused by focal myoclonic jerks of bilateral posterior auricularis muscle and bilateral temporalis muscle. This muscular tinnitus persisted except for when he was sleeping or breath holding. His symptom responded poorly to medical therapy but was controlled by botulinum toxin type A injection under electromyography monitoring with favorable outcome. Previous reports of this condition and possible therapeutic approaches are discussed. PMID:25466808

  12. Bilateral Sclerosing Stromal Ovarian Tumor in an Adolescent

    PubMed Central

    Naidu, Anjani; Chung, Betty; Simon, Mitchell; Marshall, Ian

    2015-01-01

    Sclerosing stromal tumor of the ovary is a rare, benign, sex cord stromal tumor occurring predominantly in younger women in the 2nd and 3rd decades of life. It typically presents unilaterally with only 2 previously reported cases of bilateral presentation. Common clinical presentations include pelvic or abdominal pain, a mass, or menstrual changes. Although occasionally presenting with hormonal manifestations, virilization as a result of androgen production by the tumor is rare. Here we present an extremely rare case of a sclerosing stromal ovarian tumor in a 14-year-old patient with bilateral presentation and with clinical and biochemical evidence of hyperandrogenemia.

  13. A rare entity in adults: Bilateral Hutch diverticulum with calculi.

    PubMed

    Telli, Onur; Guclu, Adil Gucal; Haciyev, Perviz; Burgu, Berk; Gogus, Cagatay

    2015-01-01

    Congenital bladder diverticulum (CBD) is a very uncommon entity in adults. CBD could be unilateral or bilateral and is caused by a congenital weakness in the bladder musculature. CBD is differentiated from the paraureteral or Hutch type of diverticula. A 42-year-old male presented with bilateral Hutch diverticulum and multiple diverticulum calculus on intravenous pyelography. Cystoscopy revealed bladder diverticulum just medial to the left ureteral orifice with multiple calculi; the patient successfully underwent endoscopic laser cystolithotripsy with resolution of his urinary tract infection. To the best of our knowledge, this is the first case report presenting stone formation of CBD in an adult. PMID:26029313

  14. Bilateral renal infarction: an uncommon presentation of fibromuscular dysplasia

    PubMed Central

    Ayach, Taha; Kazory, Amir

    2013-01-01

    While fibromuscular dysplasia (FMD) is an established cause of secondary hypertension, its association with renal infarction is less well recognized. We report a middle-aged man who presented with complaints of loin pain and severe hypertension. Computed tomography angiography of the abdomen revealed bilateral renal infarction with multiple short-segment arterial dissection compatible with FMD in the absence of systemic vasculitis and other risk factors for thromboembolic events. Bilateral renal infarction complicating FMD is extremely rare and has so far been reported only in a handful of cases. Physicians encountering cases of otherwise unexplained renal infarction/ischemia need to be aware of this complication.

  15. Conservative Management in Congenital Bilateral Upper Eyelid Eversion

    PubMed Central

    Nchifor, Alice; Ngwanou, Aronette Nana; Attha, Elisabeth; Ngounou, Faustin; Bella, Assumpta Lucienne; Ebana Mvogo, Côme

    2015-01-01

    Aim. To report the case of congenital bilateral upper eyelid eversion with severe chemosis that was successfully managed conservatively. Report. The patient was a six-hour-old male neonate with bilateral congenital upper eyelid eversion and severe chemosis, following uneventful delivery. Conservative management consisted of the application of antibiotic ointment and padding the exposed conjunctiva with 5% hypertonic saline-soaked gauze. The eyelids reverted spontaneously on day 3 and the condition was completely resolved by the third week. Conclusion. Congenital upper lid eversion is usually a benign condition which responds well to conservative treatment. Creating awareness amongst healthcare professionals is essential. PMID:25960903

  16. A rare entity in adults: Bilateral Hutch diverticulum with calculi

    PubMed Central

    Telli, Onur; Guclu, Adil Gucal; Haciyev, Perviz; Burgu, Berk; Gogus, Cagatay

    2015-01-01

    Congenital bladder diverticulum (CBD) is a very uncommon entity in adults. CBD could be unilateral or bilateral and is caused by a congenital weakness in the bladder musculature. CBD is differentiated from the paraureteral or Hutch type of diverticula. A 42-year-old male presented with bilateral Hutch diverticulum and multiple diverticulum calculus on intravenous pyelography. Cystoscopy revealed bladder diverticulum just medial to the left ureteral orifice with multiple calculi; the patient successfully underwent endoscopic laser cystolithotripsy with resolution of his urinary tract infection. To the best of our knowledge, this is the first case report presenting stone formation of CBD in an adult.

  17. Primary uterine osteosarcoma presenting synchronously with bilateral breast carcinomas.

    PubMed

    Powell, George; Barth, Laura; Todd, Richard; Ganesan, Raji

    2014-01-01

    Primary uterine sarcomas are infrequent neoplasms and most commonly leiomyosarcomas or endometrial stromal sarcomas. We report a rare case of primary uterine osteosarcoma discovered in a woman in her 60s following staging CT imaging for bilateral breast carcinomas. Examination of the subsequent hysterectomy specimen showed a tumour composed of malignant spindle cells and osteoclast-like giant cells associated with osteoid and neoplastic bone, in keeping with primary uterine osteosarcoma. Distinction of osteosarcoma from the more common carcinosarcoma is important due to the worse prognosis impacting on treatment decisions. In addition, synchronous presentation of this unusual tumour with bilateral breast carcinomas raises the possibility of a mutual genetic pathogenesis. PMID:24898994

  18. Spontaneous bilateral rod fracture of malleable penile prosthesis

    PubMed Central

    Bozkurt, Ibrahim Halil; Yonguc, Tarik; Arslan, Burak; Kozacioglu, Zafer; Degirmenci, Tansu; Polat, Salih; Minareci, Suleyman

    2014-01-01

    Mechanical failure with a malleable penile prosthesis is very rare. To the best of our knowledge, this is the first case reporting on a bilateral AMS 650 rod mechanical failure. We present a 50-year-old man with organic erectile dysfunction who experienced bilateral AMS 650 rod fracture after 14 years. The rod fracture of the left side was confirmed via X-ray preoperatively. The surgical exploration revealed a fracture of both rods. After the removal of both rods, we implanted a new malleable device during the same session. At the 6-month follow-up, the patient was satisfied with his prosthesis. PMID:25408816

  19. Bilateral macular injury from a green laser pointer

    PubMed Central

    Dirani, Ali; Chelala, Elias; Fadlallah, Ali; Antonios, Rafic; Cherfan, George

    2013-01-01

    We report the case of a 13-year-old boy who had a bilateral macular injury after playing with a green laser pointer for a duration of 1 minute. Clinical examination revealed a decrease in visual acuity and macular injury in both eyes, and imaging investigations revealed a bilateral macular lesion due to exposure to the laser pointer. At 3 months’ follow up, visual function had improved but remained partially impaired. This case emphasizes the importance of cautious and appropriate use of laser pointer devices because of the potential vision-threatening hazards induced by mishandling of these devices. PMID:24204114

  20. Migrated orbital silastic sheet implant mimicking bilateral sinusitis.

    PubMed

    Lee, Dong Hoon; Joo, Young Eun; Lim, Sang Chul

    2011-11-01

    We present a unique case of a migrated silastic sheet implant that was used during reconstruction of an orbital floor fracture, presenting as bilateral sinusitis. The patient had an orbital floor fracture that was repaired at a local hospital 20 years ago. An orbital silastic sheet implant perforated the bony nasal septum and migrated to the contralateral nasal cavity causing bilateral nasal symptoms. The silastic sheet was successfully removed during endoscopic sinus surgery. Therefore, ocular or nasal symptoms occurring in patients with a previous orbital wall fracture repair must be carefully evaluated clinically and radiologically with a suspicion of late complications, such as migration of the orbital implant. PMID:22075841

  1. Bilateral s-shaped kidneys: A rare congenital malformation

    PubMed Central

    Ranjan, Nikhil; Singh, Rana P.; Upadhyay, Rohit; Kumar, Vijoy

    2015-01-01

    A bilateral S-shaped kidney is a rare anomaly in which both the kidneys are in their normal position, in contrast to the commonly reported S-shaped fusion anomaly, in which the contralateral kidney crosses the midline to fuse with opposite kidney leaving the ipsilateral renal fossa empty. Here we present the diagnosis and management of a case of bilateral S-shaped renal anomaly with associated left pelviureteric junction obstruction and nonfunctioning kidney and right renal stones. Left kidney was managed by open nephrectomy and right kidney by PNL.

  2. Bilateral macular colobomata: Temporal dragging of optic disc

    PubMed Central

    Mathew, David J

    2015-01-01

    A 13-year-old male presented with decreased vision and squint from childhood. He had bilateral large colobomata at the macula in each eye, the one on the right being larger than the left. The disc was dragged temporally with straightening of the temporal retinal vessels. This is a case report of bilateral large macular coloboma and serves to report its association with a temporally dragged disc and straightened temporal retinal vessels. A dragged disc if present with a colobomatous defect at the macula may strengthen the case for diagnosis of macular coloboma and help exclude other differentials. PMID:26044479

  3. Hypertension associated with massive, bilateral, posture-dependent renal dysfunction.

    PubMed

    Clorius, J H; Schmidlin, P; Raptou, E; Huber, W; Georgi, P

    1981-07-01

    Hippurate function scintiscans were obtained in prone and standing positions in a group of 76 patients with concurrent hypertension and nephroptosis. TWelve of these patients had massive, bilateral disturbance of intrarenal hippurate transport in the standing position; hippurate transport was normal in the prone position. This pattern was present in only three of 120 normotensive patients with nephroptosis. To investigate the importance of nephroptosis, 87 other hypertensive patients were examined. Eighteen of these patients demonstrated posture-dependent tubular dysfunction, but only four had nephroptosis. The results suggest a direct relationship between bilateral posture-dependent tubular dysfunction and hypertension. PMID:7244230

  4. Bilateral absence of mental foramen in a living human subject.

    PubMed

    Lauhr, Géraldine; Coutant, Jean-Christophe; Normand, Eric; Laurenjoye, Mathieu; Ella, Bruno

    2015-05-01

    The mental foramen, through which the mental nerve emerges, is an important anatomic landmark in odonto-stomatology. Knowing its anatomic variations, according to the ethnic group or age, is essential when performing local anesthesia or implant and orthognathic surgeries. Besides the presence of a supernumerary foramen and variations in its location, numerous topographies have been described such as unilateral or bilateral triple foramina, hypoplasia or agenesis. The case reported here is extremely rare because it has been observed in a living and asymptomatic patient whose scan shows a bilateral absence of mental foramen. PMID:25062671

  5. Femoral-facial syndrome in an infant of a diabetic mother.

    PubMed

    Ahmed, Saleem; Alsaedi, Saad Abdullah; Al-Wassia, Heidi; Al-Aama, Jumana Yousef

    2015-01-01

    Femoral-facial syndrome (FFS) is a very rare multiple congenital anomaly syndrome. The authors describe a case of FFS in a 2-day-old infant of a diabetic mother. The phenotypic features include bilateral symmetrical femoral involvement with completely aplastic right-sided femur, severely hypoplastic left femur and unusual facial dysmorphic features without other skeleton/spinal and genitourinary anomalies. Cases of FFS need to be carefully evaluated because of the similarity between FFS and caudal dysgenesis, a condition frequently related to maternal diabetes and with other syndromes characterised by femoral hypoplasia and associated anomalies, which can pose a diagnostic challenge. PMID:26150620

  6. Why Metabolic Syndrome Matters

    MedlinePLUS

    Why Metabolic Syndrome Matters Updated:Jul 24,2014 Metabolic syndrome may be diagnosed when a patient has a cluster of ... Diabetes High Blood Pressure My Life Check Heart360® Metabolic Syndrome • Home • About Metabolic Syndrome • Why Metabolic Syndrome Matters • ...

  7. Bilateral Pheochromocytomas, Hemihyperplasia, and Subtle Somatic Mosaicism: The Importance of Detecting Low-Level Uniparental Disomy

    PubMed Central

    Kalish, Jennifer M.; Conlin, Laura K.; Mostoufi-Moab, Sogol; Wilkens, Alisha B.; Mulchandani, Surabhi; Zelley, Kristin; Kowalski, Megan; Bhatti, Tricia R.; Russo, Pierre; Mattei, Peter; Mackenzie, William G.; LiVolsi, Virginia; Nichols, Kim E.; Biegel, Jaclyn A.; Spinner, Nancy B.; Deardorff, Matthew A.

    2014-01-01

    We report on a patient with early onset pediatric bilateral pheochromocytomas caused by mosaic chromosome 11p15 paternal uniparental isodisomy (UPD). Hemihyperplasia of the arm was diagnosed in a 4-month-old female and clinical methylation testing for 11p15 in the blood was normal, with a reported detection threshold for mosaicism of 20%. She was subsequently diagnosed at 18 months with bilateral pheochromocytomas. Single-nucleotide polymorphism (SNP) array analysis of pheochromocytoma tissue demonstrated mosaic deletions of 8p12pter, 21q21.1qter, 22q11.23qter; commonly seen in pheochromocytomas. In addition, mosaic 11p15.3pter homozygosity was noted. Molecular testing for other causes of pheochromocytomas was normal, suggesting that 11p15 homozygosity was the primary event. Subsequent SNP array analysis of skin fibroblasts from the hyperplastic side demonstrated 5% mosaic paternal UPD for 11p15. We have subsequently used SNP array analysis to identify four patients with subtle hemihyperplasia with low-level mosaic UPD that was not detected by methylation analysis. Given the increased sensitivity of SNP array analysis to detect UPD along with the increased incidence of tumorigenesis in these UPD patients, we suggest that it has high utility in the clinical work-up of hemihyperplasia. The present case also suggests that 11p15 paternal UPD may be an under-detected mechanism of sporadic pheochromocytoma in the pediatric population. Furthermore, a review of the literature suggests that patients with 11p15 paternal UPD may present after 8 years of age with pheochromocytoma and raises the possibility that ultrasound screening could be considered beyond 8 years of age in this subset of hemihyperplasia and Beckwith–Wiedemann syndrome patients. PMID:23532898

  8. Reinnervation of Bilateral Posterior Cricoarytenoid Muscles Using the Left Phrenic Nerve in Patients with Bilateral Vocal Fold Paralysis

    PubMed Central

    Zheng, Hongliang; Chen, Donghui; Zhu, Minhui; Wang, Wei; Liu, Fei; Zhang, Caiyun

    2013-01-01

    Objective To evaluate the feasibility, effectiveness, and safety of reinnervation of the bilateral posterior cricoarytenoid (PCA) muscles using the left phrenic nerve in patients with bilateral vocal fold paralysis. Methods Forty-four patients with bilateral vocal fold paralysis who underwent reinnervation of the bilateral PCA muscles using the left phrenic nerve were enrolled in this study. Videostroboscopy, perceptual evaluation, acoustic analysis, maximum phonation time, pulmonary function testing, and laryngeal electromyography were performed preoperatively and postoperatively. Patients were followed-up for at least 1 year after surgery. Results Videostroboscopy showed that within 1 year after reinnervation, abductive movement could be observed in the left vocal folds of 87% of patients and the right vocal folds of 72% of patients. Abductive excursion on the left side was significantly larger than that on the right side (P < 0.05); most of the vocal function parameters were improved postoperatively compared with the preoperative parameters, albeit without a significant difference (P > 0.05). No patients developed immediate dyspnea after surgery, and the pulmonary function parameters recovered to normal reference value levels within 1 year. Postoperative laryngeal electromyography confirmed successful reinnervation of the bilateral PCA muscles. Eighty-seven percent of patients in this series were decannulated and did not show obvious dyspnea after physical activity. Those who were decannulated after subsequent arytenoidectomy were not included in calculating the success rate of decannulation. Conclusions Reinnervation of the bilateral PCA muscles using the left phrenic nerve can restore inspiratory vocal fold abduction to a physiologically satisfactory extent while preserving phonatory function at the preoperative level without evident morbidity. PMID:24098581

  9. Fluency Disorders in Genetic Syndromes

    ERIC Educational Resources Information Center

    Van Borsel, John; Tetnowski, John A.

    2007-01-01

    The characteristics of various genetic syndromes have included "stuttering" as a primary symptom associated with that syndrome. Specifically, Down syndrome, fragile X syndrome, Prader-Willi syndrome, Tourette syndrome, Neurofibromatosis type I, and Turner syndrome all list "stuttering" as a characteristic of that syndrome. An extensive review of…

  10. Mixed Markets in Bilateral Monopoly Stat-Math Unit

    E-print Network

    Bandyopadhyay, Antar

    does not offer a clear answer. In Latin America for example, Chile has privatized water utilities while water privatization in Brazil has been relatively limited. While British Gas and British Telecom (both Is there justification for any pattern in the sequencing of privatization of a public bilateral monopoly? To address

  11. Fulbright-Hays Seminars Abroad Program and Special Bilateral Projects

    ERIC Educational Resources Information Center

    Office of Postsecondary Education, US Department of Education, 2012

    2012-01-01

    The Fulbright-Hays Seminars Abroad Program and Special Bilateral Projects provides short-term study and travel seminars abroad for U.S. educators in the social sciences and humanities for the purpose of improving their understanding and knowledge of the people and culture of other countries. There are approximately 10 seminars with 16 participants…

  12. Simultaneous bilateral quadriceps tendon rupture in a uremic patient

    Microsoft Academic Search

    Yu-Cheng Pei; Po-Ching Hsieh; Li-Zen Huang; Cheng-Kuen Chiang

    2011-01-01

    Quadriceps is a part of extensor mechanism, and it is a strong muscle bundle for knee joint movement. It rarely ruptures in the general population. We present a case with simultaneous bilateral quadriceps tendon rupture and discuss the causes. A 45-year-old man had a history of end stage renal disease and received regular hemodialysis treatment for more than 12 years.

  13. Thoracoscopic resection of bilateral metastatic sarcomas causing spontaneous pneumothorax.

    PubMed

    Jones, D R; Tanguilig, G G; Graeber, G M

    1994-10-01

    Pulmonary metastases from soft tissue sarcomas may present with spontaneous pneumothorax. Metastatectomy via thoracotomy or median sternotomy has been the procedure of choice. We present a patient with bilateral spontaneous pneumothorax secondary to metastatic angiosarcoma where staged video-assisted thoracic surgery using the thoracoscope was used to successfully resect the tumors. PMID:7924513

  14. Bilateral Negotiation of a Meeting Point in a Maze: Demonstration

    E-print Network

    Paris-Sud XI, Université de

    Bilateral Negotiation of a Meeting Point in a Maze: Demonstration Fabien Delecroix, Maxime Morge agents are situated in a maze and the negotiation outcome is a cell where they will meet scenario where two agents are situated in a maze and the negotiation outcome is a cell where they will meet

  15. Bilateral renal vein thrombosis due to inapparent polycythaemia.

    PubMed

    Rapur, Ram; Gudithi, Swarnalatha; Adiraju, Krishna Prasad; Vangipurapu Rangacharlu, Srinivasasan; Venkata, Dakshinamurty Kaligotla

    2011-08-01

    Thromboses at unusual sites are characteristic of polycythaemia. We present a patient of bilateral renal vein thromboses due to polycythaemia that was inapparent. The diagnosis was confirmed by trilineage hyperplasia in bone marrow and JAK 2 V617F mutation in blood. PMID:25949491

  16. Dental pulpalgia contributing to bilateral preauricular pain and tinnitus.

    PubMed

    Wright, E F; Gullickson, D C

    1996-01-01

    The case of a patient with bilateral preauricular pain and tinnitus is reported. Minimal relief was obtained with traditional temporomandibular disorders therapy, and complete relief was obtained after endodontic therapy. Pulpal conditions that can refer pain are discussed, and recommendations are made to help practitioners identify a possible pulpal etiology for symptoms and tests of temporomandibular disorders. PMID:9133861

  17. Automatic symmetry plane estimation of bilateral objects in point clouds

    Microsoft Academic Search

    Benoît Combčs; Robin Hennessy; John Waddington; Neil Roberts; Sylvain Prima

    2008-01-01

    In this paper, the problem of estimating automatically the symmetry plane of bilateral objects (having perfect or imperfect mirror symmetry) in point clouds is reexamined. Classical methods, mostly based on the ICP algorithm, are shown to be limited and complicated by an inappropriate parameterization of the problem. First, we show how an ad- equate parameterization, used in an ICP-like scheme,

  18. Bilateral Cingulotomy and Anterior Capsulotomy Applied to Patients with Aggressiveness

    Microsoft Academic Search

    Fiacro Jiménez; Julián E. Soto; Francisco Velasco; Pablo Andrade; Juan J. Bustamante; Paulina Gómez; Ylián Ramírez; José D. Carrillo-Ruiz

    2012-01-01

    Objective: To perform a preliminary study on the effects and safety of bilateral cingulotomy and anterior capsulotomy in patients with aggressive behavior. Patients and Methods: Twenty-three psychiatric patients showing aggressive behavior refractory to conventional treatment were initially evaluated. The subjects were clinically selected using the Overt Aggression Scale (OAS) and the Global Assessment of Functioning Scale (GAF). Each case was

  19. Bilateral Macro–Micro Teleoperation Using Magnetic Levitation

    Microsoft Academic Search

    Moein Mehrtash; Naoaki Tsuda; Mir Behrad Khamesee

    2011-01-01

    This paper introduces a novel magnetic-haptic micro- manipulation platform with promising potential for extensive bio- logical and biomedical applications. The platform has three basic subsystems: a magnetic untethered microrobotic system, a haptic device, and a scaled bilateral teleoperation system. A mathematical force model of the magnetic propulsion mechanism is developed, and used to design PID controllers for magnetic actuation mech-

  20. Bilateral Cochlear Implants in Children: Localization Acuity Measured with Minimum

    E-print Network

    Litovsky, Ruth

    Bilateral Cochlear Implants in Children: Localization Acuity Measured with Minimum Audible Angle participated, 13 with BI cochlear implants (cochlear implant co- chlear implant), ranging in age from 3 to 16 yrs, and six with a hearing aid in the nonimplanted ear (cochlear implant hearing aid), ages 4 to 14

  1. Bilateral Effects of Unilateral Cochlear Implantation in Congenitally Deaf Cats

    E-print Network

    Ryugo, David K.

    Bilateral Effects of Unilateral Cochlear Implantation in Congenitally Deaf Cats Jahn N. O'Neil,1 system via cochlear implants would restore the endbulb synapses to their normal morphology. Three and 6-month-old con- genitally deaf cats received unilateral cochlear implants and were stimulated

  2. Sequential bilateral cochlear implantation in children B. Petersa,*, R. Litovskyb

    E-print Network

    Litovsky, Ruth

    Sequential bilateral cochlear implantation in children B. Petersa,*, R. Litovskyb , J. Lakea , A and the ability to localize the source of sounds. Patients who have undergone unilateral cochlear implantation the degree to which similar benefits are realized by children who receive a second cochlear implant after

  3. Bilateral Filtering-Based Optical Flow Estimation with Occlusion Detection

    Microsoft Academic Search

    Jiangjian Xiao; Hui Cheng; Harpreet S. Sawhney; Cen Rao; Michael A. Isnardi

    2006-01-01

    Using the variational approaches to estimate optical flow between two frames, the flow discontinuities between different motion fields are usually not distinguished even when an anisotropic diffusion operator is applied. In this pa- per, we propose a multi-cue driven adaptive bilateral filter to regularize the flow computation, which is able to achieve the smoothly varied optical flow field with highly

  4. Bayer bilateral denoising on TriMedia3270

    NASA Astrophysics Data System (ADS)

    Phelippeau, H.; Akil, M.; Dias Rodrigues, B.; Talbot, H.; Bara, S.

    2009-02-01

    Digital cameras are now commonly included in several digital devices such as mobile phones. They are present everywhere and have become the principal image capturing tool. Inherent to light and semiconductors properties, sensor noise [10] continues to be an important factor of image quality [12], especially in low light conditions. Removing the noise with mathematical solutions appears thus unavoidable to obtain an acceptable image quality. However, embedded devices are limited by processing capabilities and power consumption and thus cannot make use of the full range of complex mathematical noise removing solutions. The bilateral filter [6] appears to be an interesting compromise between implementation complexity and noise removing performances. Especially, the Bayer [5] bilateral filter proposed in [11] is well adapted for single sensor devices. In this paper, we simulate and optimize the Bayer bilateral filter execution on a common media-processor: the TM3270 [4] from the NXP Semiconductors TriMedia family. To do so we use the TriMedia Compilation System (TCS). We applied common optimization techniques (such as LUT, loop unrolling, convenient data type representation) as well as custom TriMedia operations. We finally propose a new Bayer bilateral filter formulation dedicated to the TM3270 architecture that yields an execution improvement of 99.6% compared to the naĂve version. This improvement results in real-time video processing at VGA resolution at the 350MHz clock rate.

  5. Hippocampus 2001 In Press Bilateral Hippocampal Pathology Impairs

    E-print Network

    Burgess, Neil

    -Khadem b John O'Keefe a a. The Institute of Cognitive Neuroscience and the Department of Anatomy, recognition, virtual reality #12;2 ABSTRACT A virtual reality environment was used to test memory performance bilateral hippocampal pathology caused by perinatal anoxia. He was allowed to explore a large-scale virtual

  6. EMG changes in rat hind limb muscles following bilateral deafferentation

    Microsoft Academic Search

    P. Hník; R. Vejsada; S. Kasicki

    1982-01-01

    Bilateral section of dorsal roots was performed in 9 adult rats in order to ascertain whether the tendency to extension, the appearance of spontaneous electromyographic (EMG) activity in extensor muscles and other symptoms of postdenervation hypersensitivity after unilateral deafferentation are not due to the sensory inflow from the contralateral limb. EMG activity from the soleus (SOL) and tibialis anterior (TA)

  7. Diffuse pulmonary fibrosis and bilateral pneumothoraces in systemic lupus erythematosus

    PubMed Central

    Richards, Anthony J.; Swinson, D. R.; Talbot, I. C.; Hamilton, E. B. D.

    1975-01-01

    A case of systemic lupus erythematosus (SLE) which developed extensive pulmonary fibrosis and bilateral pneumothoraces is reported. Other features of SLE were minimal. Diffuse fibrosing alveolitis in SLE is probably more common than has previously been thought. ImagesFig. 1Fig. 2Fig. 3Fig. 4Fig. 5 PMID:1208295

  8. Channel Incentives as Unilateral and Bilateral Governance Processes

    Microsoft Academic Search

    David I. Gilliland; Daniel C. Bello

    2001-01-01

    The authors investigate channel incentives as extra-contractual governance processes that maintain and extend marketing channel relationships. More specifically, instrumental incentives are monetary-based payments made by a manufacturer in a unilateral channel arrangement to motivate distributor compliance, while equity incentives are bilateral expectations of fair treatment that motivate both parties to continue to cooperate with one another. A model of the

  9. Osteochondroma of bilateral mandibular condyle: a case report

    PubMed Central

    Zhou, Qin; Yang, Chi; Chen, Min-Jie

    2015-01-01

    Osteochondroma represents the largest group of benign tumors of bone, which usually develops in long bones and relatively uncommon in the craniofacial region. The condyle and coronoid tip are the most common sites of occurrence in the mandible, but both sides of condyle involved has never been reported. Here, we describe a case of osteochondroma arising from the bilateral mandibular condyle. PMID:25932269

  10. Epileptic Features of Patients With Unilateral and Bilateral Schizencephaly

    Microsoft Academic Search

    Camila F. Lopes; Fernando Cendes; Ana Maria S. G. Piovesana; Fábio Torres; Iscia Lopes-Cendes; Maria Augusta Montenegro; Marilisa M. Guerreiro

    2006-01-01

    The extent of cortical maldevelopment might correlate with the severity of the clinical manifestation, such as cognitive delay or motor dysfunction. The objective of this study was to investigate the clinical features of epilepsy in patients with unilateral and bilateral schizencephaly. We studied 44 consecutive patients with schizencephaly diagnosed by magnetic resonance imaging (MRI). The epileptic features were analyzed in

  11. Friction compensation in energy-based bilateral telemanipulation

    Microsoft Academic Search

    Michel Franken; Sarthak Misra; Stefano Stramigioli

    2010-01-01

    In bilateral telemanipulation algorithms based on time-domain passivity, internal friction in the devices poses an additional energy drain. Based on a model of the friction, the dissipated energy can be estimated and reclaimed inside the energy balance of the control algorithm. As long as the estimate is conservative, passivity of the entire system is maintained. In this paper we consider

  12. Bilateral oligopoly in pollution permit markets - experimental evidence

    Technology Transfer Automated Retrieval System (TEKTRAN)

    We experimentally investigate behavior in a bilateral oligopoly using a supply function equilibria model (Klemper and Meyer 1989; Hendricks and McAfee 2010; Malueg and Yates 2009). We focus on the role that market size and the degree of firm heterogeneity have on the market equilibrium. Our results ...

  13. Meningococcal meningitis presenting with bilateral deafness and ataxia

    Microsoft Academic Search

    R. Sandyk; M. J. Brennan

    1984-01-01

    A 50-year-old man presented with bilateral deafness and ataxia of sudden onset and without constitutional symptoms or signs of meningeal irritation. He was subsequently proved to have meningococcal meningitis, and the deafness and ataxia resolved following appropriate antibiotic therapy.

  14. PRECISION OF SINGLE VERSUS BILATERAL HIP BONE MINERAL DENSITY SCANS

    Technology Transfer Automated Retrieval System (TEKTRAN)

    Dual-energy X-ray absorptiometry (DXA) is a precise, fast, and noninvasive method to measure bone mineral density (BMD) and body composition. Software that integrates the results of bilateral DXA hip scans has recently become available. The present study was undertaken to determine and compare the...

  15. Linear bilateral CMOS resistor for neural-type circuits

    Microsoft Academic Search

    L. Sellamit; S. K. Singh; R. W. Newcomb; G. Moon

    1997-01-01

    A previous CMOS bilateral linear resistor is analyzed and shown to be reducible from four to two transistors with improved linearity. This is developed for neural-type circuits to allow its use in emulating both excitatory and inhibitory voltage variable synapses. Simulation results using parameters of MOSIS transistors are presented to verify the theory.

  16. Bilateral pneumectomy (volume reduction) for chronic obstructive pulmonary disease

    Microsoft Academic Search

    J. D. Cooper; E. P. Trulock; A. N. Triantafillou; G. A. Patterson; M. S. Pohl; P. A. Deloney; R. S. Sundaresan; C. L. Roper

    1995-01-01

    We undertook surgical bilateral lung volume reduction in 20 patients with severe chronic obstructive pulmonary disease to relieve thoracic distention and improve respiratory mechanics. The operation, done through median sternotomy, involves excision of 20% to 30% of the volume of each lung. The most affected portions are excised with the use of a linear stapling device fitted with strips of

  17. Presentation to U.S.-Canada Bilateral Technical Working Group

    EPA Science Inventory

    DHS and EPA have collaborated in the development of a draft charter for Technical Working Group (TWG) to serve as the basis of negotiations of bilateral agreements with other countries. The TWG would provide a mechanism for sharing both response and R&D expertise and experience i...

  18. The influence of pedaling rate on bilateral asymmetry in cycling

    Microsoft Academic Search

    W Smak; R. R Neptune; M. L Hull

    1999-01-01

    The objectives of this study were to (1) determine whether bilateral asymmetry in cycling changed systematically with pedaling rate, (2) determine whether the dominant leg as identified by kicking contributed more to average power over a crank cycle than the other leg, and (3) determine whether the dominant leg asymmetry changed systematically with pedaling rate. To achieve these objectives, data

  19. Bilateral internuclear ophthalmoplegia after intrathecal chemotherapy and cranial irradiation

    SciTech Connect

    Lepore, F.E.; Nissenblatt, M.J.

    1981-12-01

    A 26-year-old man developed transient bilateral internuclear ophthalmoplegia with exotropia after cranial irradiation and intrathecal administration of methotrexate for lymphoma. Resolution of the ophthalmoplegia and the decrease in abnormally high levels of cerebrospinal fluid myelin basic protein after discontinuation of intrathecal medication suggested demyelination from chemotherapy and irradiation.

  20. Paraneoplastic syndromes

    SciTech Connect

    Weller, R.E.

    1994-03-01

    Paraneoplastic syndromes (PNS) comprise a diverse group of disorders that are associated with cancer but unrelated to the size, location, metastases, or physiologic activities of the mature tissue of origin. They are remote effects of tumors that may appear as signs, symptoms, or syndromes which can mimic other disease conditions encountered in veterinary medicine. Recognition of PNS is valuable for several reasons: the observed abnormalities may represent tumor cell markers and facilitate early diagnosis of the tumor; they may allow assessment of premalignant states; they may aid in the search metastases; they may help quantify and monitor response to therapy; and, they may provide insight into the study of malignant transformation and oncogene expression. This review will concentrate on the pathophysiology, diagnosis, and treatment of some of the common PNS encountered in veterinary medicine.

  1. Overtraining Syndrome

    PubMed Central

    Kreher, Jeffrey B.; Schwartz, Jennifer B.

    2012-01-01

    Context: Fatigue and underperformance are common in athletes. Understanding overtraining syndrome (OTS) is helpful in the evaluation, management, and education of athletes. Evidence Acquisition: Relevant articles in English were searched with OVID (1948-2011) and PubMed using the following keywords: overtraining syndrome, overtraining, overreaching, unexplained underperformance, staleness, pathophysiology, management, treatment, evaluation. Bibliographies were reviewed for additional resources. Results: OTS appears to be a maladapted response to excessive exercise without adequate rest, resulting in perturbations of multiple body systems (neurologic, endocrinologic, immunologic) coupled with mood changes. Many hypotheses of OTS pathogenesis are reviewed, and a clinical approach to athletes with possible OTS (including history, testing, and prevention) is presented. Conclusions: OTS remains a clinical diagnosis with arbitrary definitions per the European College of Sports Science’s position statement. History and, in most situations, limited serologies are helpful. However, much remains to be learned given that most past research has been on athletes with overreaching rather than OTS. PMID:23016079

  2. Tourette Syndrome (For Parents)

    MedlinePLUS

    ... help their child cope with the condition. About Tourette Syndrome Tourette syndrome (TS) is named for French ... people with TS. Back Continue Diagnosing and Treating Tourette Syndrome Pediatricians and family doctors may refer a ...

  3. Reye syndrome - resources

    MedlinePLUS

    Resources - Reye syndrome ... The following organizations are good resources for information on Reye Syndrome : National Reye's Syndrome Foundation, Inc. - www.reyessyndrome.org National Institute of Neurologic Disorders and Stroke - www. ...

  4. Dental Issues & Down Syndrome

    MedlinePLUS

    ... Associated Conditions » Dental Issues & Down Syndrome Dental Issues & Down Syndrome Dental care is important for everybody, but people ... is Different About the Teeth of People With Down Syndrome? Delayed Eruption The teeth of people with Down ...

  5. Heart and Down Syndrome

    MedlinePLUS

    ... Associated Conditions » The Heart & Down Syndrome The Heart & Down Syndrome Abnormalities of the cardiovascular system are common in ... the Most Common Heart Defects in Children With Down Syndrome? The most common defects are Atrioventricular Septal Defect ( ...

  6. What Causes Down Syndrome?

    MedlinePLUS

    ... Information Clinical Trials Resources and Publications What causes Down syndrome? Skip sharing on social media links Share this: ... Down Syndrome Registry? . Chromosomal Changes That Can Cause Down Syndrome Research shows that three types of chromosomal changes ...

  7. What Is Marfan Syndrome?

    MedlinePLUS

    ... Syndrome PDF Version Size: 62 KB Audio Version Time: 11:11 Size: 10.5 MB November 2014 What Is Marfan Syndrome? Fast Facts: An Easy-to-Read Series of Publications for the Public Marfan syndrome is ...

  8. Polycystic Ovary Syndrome

    MedlinePLUS

    ... Another condition that is associated with PCOS is metabolic syndrome . This syndrome contributes to both diabetes and heart ... the levels of glucose (sugar) in the blood. Metabolic Syndrome: Combination of factors that contribute to both diabetes ...

  9. Metabolic Syndrome (For Parents)

    MedlinePLUS

    ... this is a condition called metabolic syndrome . About Metabolic Syndrome Not to be confused with metabolic disease (which ... chemicals in the body fail to interact properly), metabolic syndrome describes a cluster of risk factors that put ...

  10. Fragile X Syndrome

    MedlinePLUS

    ... Was Just Figuring Out CGG Repeats! Donate | Print Fragile X Syndrome Fragile X syndrome (FXS) is a genetic condition ... health concerns associated with the condition. Features of Fragile X Syndrome in Males Read our Story The majority of ...

  11. Fragile X Syndrome Overview

    MedlinePLUS

    ... NICHD Research Information Clinical Trials Resources and Publications Fragile X Syndrome: Condition Information Skip sharing on social media links Share this: Page Content What is Fragile X syndrome? Fragile X syndrome is a genetic disorder that ...

  12. Carpal Tunnel Syndrome

    MedlinePLUS

    ... key nerve in the wrist. What is carpal tunnel syndrome? Carpal tunnel syndrome occurs when the median ... or traumatized. What are the symptoms of carpal tunnel syndrome? Symptoms usually start gradually, with frequent burning, ...

  13. Renal dysplasia\\/hypoplasia, Williams Syndrome phenotype and non-Hodgkin lymphoma in the same patient: only a coincidence?

    Microsoft Academic Search

    Adela Urisarri-Ruiz de Cortázar; Marta Gil Calvo; Manuel Vázquez Donsión; Gema Ariceta Iraola; Jose Miguel Couselo Sánchez

    2009-01-01

    Congenital renal anomalies, Williams Syndrome and non-Hodgkin lymphoma all occur separately at low incidence, so their simultaneous\\u000a presence in the same patient is exceptional. We present a young patient manifesting all three conditions. This child is a\\u000a boy with a Williams Syndrome phenotype who was diagnosed with severe chronic kidney disease secondary to bilateral renal dysplasia\\/hypoplasia.\\u000a Due to his small

  14. Foreign body reaction with delayed extrusion of ganciclovir implant in a patient with immune recovery vitritis syndrome

    Microsoft Academic Search

    Abdel Hakim Taguri; Baljean Dhillon; Stephen B Wharton; Ahmed Kamal

    2002-01-01

    PURPOSE: To report a case of delayed extrusion of primary ganciclovir implants in a patient with immune recovery vitritis syndrome.METHOD: Interventional case report. A 54-year-old HIV positive male patient with immune recovery vitritis syndrome had spontaneous extrusion of bilateral ganciclovir devices 4 years after primary implantation.RESULT: The extruded ganciclovir implants were removed from both eyes, and removal was complicated by

  15. Kallmann syndrome

    Microsoft Academic Search

    Catherine Dodé; Jean-Pierre Hardelin; J-P Hardelin

    2009-01-01

    The Kallmann syndrome (KS) combines hypogonadotropic hypogonadism (HH) with anosmia. This is a clinically and genetically heterogeneous disease. KAL1, encoding the extracellular glycoprotein anosmin-1, is responsible for the X chromosome-linked recessive form of the disease. Mutations in FGFR1 or FGF8, encoding fibroblast growth factor receptor-1 and fibroblast growth factor-8, respectively, underlie an autosomal dominant form with incomplete penetrance. Finally, mutations

  16. Hepatorenal syndrome.

    PubMed

    Turban, Sharon; Thuluvath, Paul J; Atta, Mohamed G

    2007-08-14

    Hepatorenal syndrome (HRS) is a "functional" and reversible form of renal failure that occurs in patients with advanced chronic liver disease. The distinctive hallmark feature of HRS is the intense renal vasoconstriction caused by interactions between systemic and portal hemodynamics. This results in activation of vasoconstrictors and suppression of vasodilators in the renal circulation. Epidemiology, pathophysiology, as well as current and emerging therapies of HRS are discussed in this review. PMID:17696221

  17. Startle syndromes.

    PubMed

    Bhidayasiri, Roongroj; Truong, Daniel D

    2011-01-01

    Startle refers to a sudden involuntary movement of the body in response to a surprising and unexpected stimulus. It is a fast twitch of facial and body muscles evoked by a sudden and intense tactile, visual, or acoustic stimulus. While startle can be considered to be a protective function against injury, startle syndromes are abnormal responses to startling events, consisting of three heterogeneous groups of disorders. The first is hyperekplexia, characterized by brisk and generalized startle in response to trivial stimulation. The major form of hereditary hyperekplexia has a genetic basis, frequently due to mutations in the ?1 subunit of the glycine receptor (GLRA1) on chromosome 5q. In the second group, normal startle induces complex but stereotyped motor and/or behavioral abnormalities lasting several seconds, termed as startle epilepsy. It usually occurs in the setting of severe brain damage, particularly perinatal hypoxia. The third group is characterized by nonhabituating hyperstartling, provoked by loud noises, sudden commands, or gestures. The intensity of startle response tends to increase with frequency of stimulation, which often leads to injury. Interestingly, its occurrence is restricted to certain social or ethnic groups in different parts of the world, such as jumping Frenchmen of Maine among Franco-Canadian lumberjack communities, and Latah in Southeast Asia. So far, no neurological abnormalities have been reported in association with these neuropsychiatric startle syndromes. In this chapter, the authors discuss the clinical presentation, physiology, and the neuronal basis of the normal human startle as well as different groups of abnormal startle syndromes. The aim is to provide an overview of hyperstartling with some diagnostic hints and the distinguishing features among these syndromes. PMID:21496599

  18. Antisynthetase syndrome

    Microsoft Academic Search

    Agathe Imbert-Masseau; Mohamed Hamidou; Christian Agard; Jean-Yves Grolleau; Pascal Chérin

    2003-01-01

    Seven autoantibodies directed against synthetases have been identified to date, the best known being anti-Jo1. Synthetases play a vital role in protein synthesis by catalyzing the acetylation of transfer RNAs (tRNAs). The most common form of antisynthetase syndrome is characterized by anti-Jo1 production, interstitial lung disease (ILD), inflammatory muscle disease, and, in many cases, fever, polyarthritis, Raynaud’s phenomenon, and thick

  19. Masquerade Syndromes

    Microsoft Academic Search

    Shouvik Saha; Elizabeth M. Graham

    The term “masquerade syndrome” was first used in 1967 to describe a case of conjunctival carcinoma that presented as chronic\\u000a conjunctivitis [1]. Since then, the term has been applied to range of disorders that may present as intraocular inflammation.\\u000a The disorders may be malignant or nonmalignant, but they share the common characteristic that intraocular inflammation is\\u000a not due to an

  20. Maffucci Syndrome

    Microsoft Academic Search

    Leida B. Rozeman; Yvonne M. Schrage; Judith V. M. G. Bovée; Pancras C. W. Hogendoorn

    Maffucci syndrome is characterized by the presence of multiple enchondromas, referred to as enchondromatosis, combined with\\u000a multiple haemangiomas and\\/or lymphangiomas, as described by (1881). Both lesions tend to have an unilateral predominance (Albregts and Rapini 1995). Enchondromas are benign cartilaginous neoplasms and occur mainly in the tubular bones of hands and feet. Their presence\\u000a in long bones can result in