MRI diagnosis of Alexander disease

MRI diagnosis of Alexander disease

Alexander disease (fibrinoid leucodystrophy; originally described by Alexander in 1949) is a rare, fatal, nonfamilial leucoencephalopathy caused by astrocyte dysfunction characterised by missense mutation in the genes coding for glial fibrillary acidic protein(GFAP). It typically presents with front...

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Bibliographic Details
Main Authors: C G Muralidharan, R P S Tomar, R Aggarwal
Format: Article
Language:English
Published: AOSIS 2012-09-01
Series:South African Journal of Radiology
Subjects:
Macroencephaly, Leucoencephalopathy, Frontal preponderance, Periventricular rim, Cystic cavitation
Online Access:https://sajr.org.za/index.php/sajr/article/view/296
Description
Summary:Alexander disease (fibrinoid leucodystrophy; originally described by Alexander in 1949) is a rare, fatal, nonfamilial leucoencephalopathy caused by astrocyte dysfunction characterised by missense mutation in the genes coding for glial fibrillary acidic protein(GFAP). It typically presents with frontal preponderance of white matter abnormalities and macroencephaly. We report a case of leucoencephalopathy with macroencephaly that shows characteristic MRI features of Alexander disease.
ISSN:1027-202X
2078-6778

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