نتایج جستجو برای: ataxia oculomotor apraxia 1 aoa1

تعداد نتایج: 2770963  

2011
Tyler Mark Pierson David Adams Florian Bonn Paola Martinelli Praveen F. Cherukuri Jamie K. Teer Nancy F. Hansen Pedro Cruz James C. Mullikin for the NISC Comparative Sequencing Program Robert W. Blakesley Gretchen Golas Justin Kwan Anthony Sandler Karin Fuentes Fajardo Thomas Markello Cynthia Tifft Craig Blackstone Elena I. Rugarli Thomas Langer William A. Gahl Camilo Toro

We report an early onset spastic ataxia-neuropathy syndrome in two brothers of a consanguineous family characterized clinically by lower extremity spasticity, peripheral neuropathy, ptosis, oculomotor apraxia, dystonia, cerebellar atrophy, and progressive myoclonic epilepsy. Whole-exome sequencing identified a homozygous missense mutation (c.1847G>A; p.Y616C) in AFG3L2, encoding a subunit of an...

2012
Peter Klivényi Dezso Nemeth Tamas Sefcsik Karolina Janacsek Ildiko Hoffmann Gabor Peter Haden Zsuzsa Londe Laszlo Vecsei

BACKGROUND Ataxia with oculomotor apraxia type 2 (AOA2) is characterized by cerebellar atrophy, peripheral neuropathy, oculomotor apraxia, and elevated serum alpha-fetoprotein (AFP) levels. The disease is caused by a recessive mutation in the senataxin gene. Since it is a very rare cerebellar disorder, no detailed examination of cognitive functions in AOA2 has been published to date. The aim of...

Journal: :Neurology 2014
Alberto J Espay Scott R Allen

A 54-year-old woman developed acute hypertensive encephalopathy associated with acetaminophen-induced liver failure. Examination showed blindness with absence of horizontal and vertical volitional and reflex saccades (video on the Neurology® Web site at Neurology.org, first segment). MRI showed biparieto-occipital signal abnormalities consistent with the posterior reversible encephalopathy synd...

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