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Case Reports
. 2003 Sep;74(9):1345-7.
doi: 10.1136/jnnp.74.9.1345.

Hereditary diffuse leucoencephalopathy with spheroids

N Hancock  1 , M Poon, B Taylor, C McLean
Affiliations
Case Reports

Hereditary diffuse leucoencephalopathy with spheroids

N Hancock et al. J Neurol Neurosurg Psychiatry. 2003 Sep.

Abstract

Hereditary diffuse leucoencephalopathy with spheroids (HDLS) is a rare inherited progressive leucoencephalopathy characterised by giant neuroaxonal swellings (spheroids) within the CNS white matter. The case is reported of a 45 year old woman with a rapidly progressive fulminant illness course characterised by progressive cognitive decline with depressive features. A presumed dominant inheritance pattern was elicited. This report reviews the literature on HDLS and the relation of this disorder to other conditions with giant neuroaxonal swellings.

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