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White matter disorders with autosomal dominant heredity: a review with personal clinical case studies and their MRI findings

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Sundal C, Ekholm S, Andersen O. White matter disorders with autosomal dominant heredity: a review with personal clinical case studies and their MRI findings.

Acta Neurol Scand: 2010: 121: 328–337.

© 2009 The Authors Journal compilation © 2009 Blackwell Munksgaard. Background – 

Leukoencephalopathies are a heterogeneous group of severe encephalopathy syndromes with myelin, axonal or vascular pathology, typically with extensive white matter lesions on MRI T2-FSE and/or -FLAIR sequences. Objectives – 

This review is restricted to leukoencephalopathies with onset in adult age and a dominant inheritance. These diseases are generally severe and often lethal and present with an exacerbating or insidiously progressive course. Material and methods – 

The focus is on four syndromes with pure leukoencephalopathies, however, leukoencephalopathies with associated clinical features are included. Results – 

T2 weighted MR imaging often show features common for leukoencephalopathies, yet shows distinguishing features in transthyretin amyloidosis. Conclusion – 

The diagnosis within the group of leukoencephalopathies thus characterized by MRI relies mainly upon clinical and genetic analysis. The differential diagnosis against treatable leukoencephalopathies is increasingly relevant.
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Keywords: MRI; adult; autosomal dominant; white matter

Document Type: Research Article

Affiliations: 1: Department of Neurology, Sahlgrenska University Hospital, Gothenburg, Sweden 2: Department of Imaging Sciences, University of Rochester, Rochester, NY, USA

Publication date: 2010-05-01

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