This Article  • References  • Full text PDF  • Reply to article  • Send to a friend  • Save in My Folder  • Save to citation manager  • Permissions  Citing Articles  • Citation map  • Citing articles on HighWire  • Contact me when this article is cited  Related Content  • Similar articles in this journal  Social Bookmarking   What's this?

John D. Schwankhaus, MD; Nicholas Patronas, MD; Robert Dorwart, MD; Roswell Eldridge, MD; Sandra Schlesinger, MS; Henry McFarland, MD

Arch Neurol. 1988;45(9):1004-1008.

Abstract
• Five clinically affected and nine atrisk members of a kindred with an autosomal dominant adult-onset leukodystrophy simulating chronic progressive multiple sclerosis were studied with computed tomography (CT) and magnetic resonance imaging (MRI). Computed tomographic scans showed white matter lucencies occurring earliest and most prominently in the frontoparietal region. The lesions were nondiscrete, diffuse, and bilaterally symmetric. These changes were more clearly visualized as areas of increased signal intensity with T₂-weighted MRI. Magnetic resonance imaging also showed increased signal intensity in the brain stem, cerebellar white matter, or both of four patients. Both MRi and CT differentiated this entity from multiple sclerosis, but MRI was superior to CT in detailing the extent of white matter involvement.

Author Affiliations
From the Department of Neurology, Veterans Administration (VA) Medical Center, Washington, DC (Dr Schwankhaus); the Department of Radiology, Georgetown University Hospital, Washington, DC (Dr Patronas); the Department of Radiology, Medical Center Hospital of Vermont, Burlington (Dr Dorwart); the Neuroepidemiology Branch (Dr Eldridge) and the Neuroimmunology Branch (Dr McFarland), National Institute of Neurological and Communicative Disorders and Stroke, Bethesda, Md; and the Interinstitute Medical Genetics Program, Warren Grant Magnuson Clinical Center, Bethesda, Md (Ms Schlesinger).

Footnotes
Accepted for publication Feb 8, 1988.

Reprint requests to Department of Neurology, VA Medical Center, 50 Irving St NW, Washington, DC 20422 (Dr Schwankhaus).

CiteULike    Connotea    Del.icio.us    Digg    Reddit    Technorati     What's this?

THIS ARTICLE HAS BEEN CITED BY OTHER ARTICLES

MR Imaging Characteristics and Neuropathology of the Spinal Cord in Adult-Onset Autosomal Dominant Leukodystrophy with Autonomic Symptoms
Sundblom et al.
Am. J. Neuroradiol. 2009;30:328-335.
ABSTRACT | FULL TEXT  

MR characteristics and neuropathology in adult-onset autosomal dominant leukodystrophy with autonomic symptoms.
Melberg et al.
Am. J. Neuroradiol. 2006;27:904-911.
ABSTRACT | FULL TEXT  

Genetic localization of an autosomal dominant leukodystrophy mimicking chronic progressive multiple sclerosis to chromosome 5q31
Coffeen et al.
Hum Mol Genet 2000;9:787-793.
ABSTRACT | FULL TEXT  

Defining and Categorizing Leukoencephalopathies of Unknown Origin: MR Imaging Approach
van der Knaap et al.
Radiology 1999;213:121-133.
ABSTRACT | FULL TEXT  

Case 33-1995- Progressive neurologic deterioration with unusual findings on magnetic resonance imaging in a 43-year-old man treated for demyelinating disease
Ronthal and Vonsattel
NEJM 1995;333:1135-1143.
FULL TEXT  

Clinical and Pathological Features of an Autosomal Dominant, Adult-Onset Leukodystrophy Simulating Chronic Progressive Multiple Sclerosis
Schwankhaus et al.
Arch Neurol 1994;51:757-766.
ABSTRACT