Alexander disease: diagnosis with MR imaging.

Alexander disease: diagnosis with MR imaging. Research Abstract Details 

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Alexander disease: diagnosis with MR imaging. Abstract Text:

M S van der Knaap,S Naidu,S N Breiter,S Blaser,H Stroink,S Springer,J C Begeer,R van Coster,P G Barth,N H Thomas,J Valk,J M Powers,

BACKGROUND AND PURPOSE: To date, the demonstration of Rosenthal fibers on brain biopsy or autopsy specimens is considered a prerequisite for a definitive diagnosis of Alexander disease. We initiated a multiinstitutional survey of MR abnormalities in both presumed and confirmed cases of Alexander disease to assess the possibility of an MR-based diagnosis. METHODS: MR imaging studies in three patients with an autopsy-based diagnosis of Alexander disease were analyzed to define MR criteria for the diagnosis. These criteria were then applied to 217 children with leukoencephalopathy of unknown origin. RESULTS: Five MR imaging criteria were defined: extensive cerebral white matter changes with frontal predominance, a periventricular rim with high signal on T1-weighted images and low signal on T2-weighted images, abnormalities of basal ganglia and thalami, brain stem abnormalities, and contrast enhancement of particular gray and white matter structures. Four of the five criteria had to be met for an MR imaging-based diagnosis. In a retrospective analysis of the MR studies of the 217 patients, 19 were found who fulfilled these criteria. No other essentially new MR abnormalities were found in these patients. In four of the 19 patients, subsequent histologic confirmation was obtained. The clinical symptomatology was the same in the patients with and without histologic confirmation and correlated well with the MR abnormalities. MR abnormalities were in close agreement with the known histopathologic findings of Alexander disease. CONCLUSION: The defined criteria are sufficient for an in vivo MR imaging diagnosis of Alexander disease; only in atypical cases is a brain biopsy still necessary for a definitive diagnosis.

Alexander disease: diagnosis with MR imaging. Publishing Authors By Initials

MS van der Knaap,S Naidu,SN Breiter,S Blaser,H Stroink,S Springer,JC Begeer,R van Coster,PG Barth,NH Thomas,J Valk,JM Powers,

For similar nervous system: central nervous system: brain: prosencephalon: diencephalon: thalamus research abstracts see: nervous system: central nervous system: brain: prosencephalon: diencephalon: thalamus research

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Alexander disease: diagnosis with MR imaging. Journal Published:

PUBLICATION TYPE: Journal Article

Journal: AJNR. American journal of neuroradiology

VOLUME: 22

Page Numbers: 541-52

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ISSN: 0195-6108

DAY: 14

MONTH: Mar

YEAR: 2001

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LANGUAGE: eng

NlmUniqueID: 8003708

Alexander disease: diagnosis with MR imaging. Keywords Mesh Terms:

KEYWORDS: Thalamus

MESH TERMS: pathology

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Grant and Affiliation Information for Alexander disease: diagnosis with MR imaging.

AFFILIATION: Department of Child Neurology, Free University Hospital, Amsterdam, the Netherlands.

Country: United States

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MEDLINETA: AJNR Am J Neuroradiol

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