نتایج جستجو برای: hereditary ataxia

تعداد نتایج: 100227  

2015
Areej Elkamil Krisztina K. Johansen Jan Aasly

OBJECTIVE Ataxia with vitamin E deficiency (AVED) is a rare autosomal recessive neurological disorder which usually starts in childhood. The clinical presentation is very similar to Friedreich ataxia, most patients have progressive truncal and extremity ataxia, areflexia, positive Babinski sign, dysarthria and sensory neuropathy. METHODS We made an inquiry to our colleagues in Norway, we incl...

2015
Gerald Pfeffer Angela Pyle Helen Griffin Jack Miller Valerie Wilson Lisa Turnbull Katherine Fawcett David Sims Gail Eglon Marios Hadjivassiliou Rita Horvath Andrea Németh Patrick F. Chinnery

To an increasing extent, the desire to catalog and categorize neurologic disorders based on genotype– phenotype correlations is challenged by frequent departures from the syndromic categorizations that were commonplace in the pregenomic era. This challenge is not limited to the recent striking revelations concerning diseases attributable to noncoding, nonconventional mutations, such as repeat e...

Journal: :Journal of the Neurological Sciences 2018
Kunihiro Yoshida Satoshi Kuwabara Katsuya Nakamura Ryuta Abe Akira Matsushima Minako Beppu Yoshitaka Yamanaka Yuji Takahashi Hidenao Sasaki Hidehiro Mizusawa

Cortical cerebellar atrophy (CCA) and multiple system atrophy with predominant cerebellar ataxia (MSA-C) are the two major forms of adult-onset sporadic ataxia. Contrary to MSA-C, there are neither diagnostic criteria nor neuroimaging features pathognomonic for CCA. Therefore, it is assumed that the category of CCA in the Japanese national registry include heterogeneous cerebellar ataxic disord...

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