نتایج جستجو برای: optic ataxia

تعداد نتایج: 62591  

Journal: :Brain : a journal of neurology 2011
Rik Vandenberghe

Neurological effects of high-dose idebenone in patients with Friedreich's ataxia: a rando-mized, placebo-controlled trial. et al. A randomized placebo-controlled trial of idebenone in Leber's hereditary optic neuropathy. Brain (this issue) 2011. Koilkonda RD, Guy J. Leber's hereditary optic neuropathy – gene therapy: from benchtop to bedside. Do idebenone and vitamin therapy shorten the time to...

Journal: :Neurology 2022

Objective To report an anti-metabotropic glutamate receptor 1 (mGluR1) encephalitis case present with cerebellar ataxia and a paraneoplastic retinopathy optic neuropathy anti-inositol 1,4,5-trisphosphate type (ITPR1) antibodies. Background Medusa-head antibodies refer to group of autoantibodies staining pattern resembling Gorgon's head, caused by binding IgG Purkinje cell somata dendrites in ti...

Accurately detection of retinal landmarks, like optic disc, is an important step in the computer aided diagnosis frameworks. This paper presents an efficient method for automatic detection of the optic disc’s center and estimating its boundary. The center and initial diameter of optic disc are estimated by employing an ANN classifier. The ANN classifier employs visual features of vessels and th...

Journal: :iranian journal of allergy, asthma and immunology 0
toshio miyawakimohammad hossein sanati behnaz bayat ahmad aleyasin hasti atashi shirazi anna isaian abolhassan farhoudi

ataxia-telangiectasia (at) is an autosomal recessive disorder involving cerebellar degeneration, immunodeficiency, radiation sensitivity and cancer predisposition. the atm gene on human chromosome 11q22.3 has recently been identified as the gene responsible for ataxia-telangiectasia (at). the gene mutated in at, which has been designated as the atm gene, encodes a large protein kinase with a pi...

Journal: :The New England journal of medicine 2013
David H Margolin Maria Kousi Yee-Ming Chan Elaine T Lim Jeremy D Schmahmann Marios Hadjivassiliou Janet E Hall Ibrahim Adam Andrew Dwyer Lacey Plummer Stephanie V Aldrin Julia O'Rourke Andrew Kirby Kasper Lage Aubrey Milunsky Jeff M Milunsky Jennifer Chan E Tessa Hedley-Whyte Mark J Daly Nicholas Katsanis Stephanie B Seminara

BACKGROUND The combination of ataxia and hypogonadism was first described more than a century ago, but its genetic basis has remained elusive. METHODS We performed whole-exome sequencing in a patient with ataxia and hypogonadotropic hypogonadism, followed by targeted sequencing of candidate genes in similarly affected patients. Neurologic and reproductive endocrine phenotypes were characteriz...

2017
Kendra A. Klein Alexis K. Warren Caroline R. Baumal Thomas R. Hedges

BACKGROUND Methanol toxicity poses a significant public health problem in developing countries, and in Southeast Asia, where the most common source of poisoning is via adulterated liquor in local drinks. Methanol toxicity can have devastating visual consequences and retinal specialists should be aware of the features of this toxic optic neuropathy. The authors report a case of severe systemic m...

Journal: :JAMA neurology 2013
Beenish Arif Kishore R Kumar Philip Seibler Franca Vulinovic Amara Fatima Susen Winkler Gudrun Nürnberg Holger Thiele Peter Nürnberg Ahmad Zeeshan Jamil Anne Brüggemann Ghazanfar Abbas Christine Klein Sadaf Naz Katja Lohmann

IMPORTANCE We sought to unravel the genetic cause in a consanguineous Pakistani family with a complex neurological phenotype. OBSERVATIONS Neurological and ophthalmological examination, including videotaping and fundoscopy, and genetic investigations, including homozygosity mapping and exome sequencing, were performed at the University of the Punjab and the University of Lübeck. Participants ...

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