Restricted Diffusion in Vanishing White Matter

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Restricted diffusion in vanishing white matter.

OBJECTIVE To investigate the occurrence of restricted diffusion in vanishing white matter, the affected structures,the time of occurrence in the disease course, and the histopathologic correlate. DESIGN Retrospective observational study. PATIENTS Forty-six patients with vanishing white matter. SETTING VU University Medical Center. MAIN OUTCOME MEASURES We evaluated all available diffusi...

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Vanishing white matter disease 1 (VWM) is a puzzling leukoencephalopathy caused by mutations in any of the five genes encoding eukaryotic translation initiation factor 2B (eIF2B), an ubiquitously expressed protein complex with a crucial role in initiation of mRNA translation for virtually every protein in the human body. VWM is one of the most prevalent inherited childhood white matter disorder...

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Atypical presentation of vanishing white matter disease.

Dra. Lucia Maria da Costa Fontenelle – Rua Sorocaba 464 / 302 22271-110 Rio de Janeiro RJ Brasil. E-mail: [email protected] Leukoencephalopathy with vanishing white matter (VWM) has also been called childhood ataxia with central hypomyelination (CACH). VWM was described by van der Knaap et al. in nine children with a leukoencephalopathy of similar type according to clinical and MRI fi...

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ژورنال

عنوان ژورنال: Archives of Neurology

سال: 2012

ISSN: 0003-9942

DOI: 10.1001/archneurol.2011.1658