نتایج جستجو برای: cmt1a

تعداد نتایج: 183  

Journal: :Annals of Clinical and Translational Neurology 2019

Journal: :Canadian Journal of Neurological Sciences / Journal Canadien des Sciences Neurologiques 2007

Journal: :Journal of Investigative Medicine High Impact Case Reports 2018

Journal: :JAMA neurology 2016
Kayla M D Cornett Manoj P Menezes Paula Bray Mark Halaki Rosemary R Shy Sabrina W Yum Timothy Estilow Isabella Moroni Maria Foscan Emanuela Pagliano Davide Pareyson Matilde Laurá Trupti Bhandari Francesco Muntoni Mary M Reilly Richard S Finkel Janet Sowden Katy J Eichinger David N Herrmann Michael E Shy Joshua Burns

IMPORTANCE Disease severity of childhood Charcot-Marie-Tooth disease (CMT) has not been extensively characterized, either within or between types of CMT to date. OBJECTIVE To assess the variability of disease severity in a large cohort of children and adolescents with CMT. DESIGN, SETTING, AND PARTICIPANTS A cross-sectional study was conducted among 520 children and adolescents aged 3 to 20...

Journal: :Journal of Neuropathology & Experimental Neurology 1998

2015
Jonas Mandel Viviane Bertrand Philippe Lehert Shahram Attarian Laurent Magy Joëlle Micallef Ilya Chumakov Catherine Scart-Grès Mickael Guedj Daniel Cohen

CMT1A is the most common inherited peripheral neuropathy. There is currently no approved treatment. We performed a meta-analysis including four randomized, double-blind, Placebo-controlled clinical trials to assess the disease progression after one year under Placebo, Ascorbic Acid (AA) or PXT3003, a combination of three repurposed drugs. We observed a weak deterioration in patients under Place...

Journal: :Current treatment options in neurology 2011
Angelo Schenone Lucilla Nobbio Margherita Monti Bragadin Giulia Ursino Marina Grandis

OPINION STATEMENT Inherited peripheral neuropathies are among the most common hereditary diseases of the nervous system. Charcot-Marie-Tooth (CMT) disease, also known from previous classifications as hereditary motor and sensory neuropathy (HMSN), is certainly the most common inherited neuropathy. In the past several years, various treatments for CMT have been proposed, although specific therap...

2004
E Nelis J Berciano N Verpoorten K Coen I Dierick V Van Gerwen O Combarros P De Jonghe V Timmerman

C harcot-Marie-Tooth (CMT) disease is a clinically and genetically heterogeneous group of disorders that involve the peripheral nervous system. It is characterised by progressive distal neurogenic muscular atrophy and weakness that initially affects the peroneal muscles and later the hands. Charcot-Marie-Tooth disease type 1 (CMT1), also called hereditary motor and sensory neuropathy type I (HM...

Journal: :Molecular human reproduction 1998
A De Vos K Sermon H Van de Velde H Joris M Vandervorst W Lissens G Mortier P De Sutter A Löfgren C Van Broeckhoven I Liebaers A Van Steirteghem

Charcot-Marie-Tooth (CMT) disease type 1A is an autosomal dominant peripheral neuropathy characterized by slow progressive distal muscle wasting and weakness, and decreased nerve conduction velocities. Most CMT1A cases (>98%) are caused by a duplication of a 1.5 Mb region on the short arm of chromosome 17 containing the PMP22 gene. A couple with a previous history of CMT followed by termination...

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