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J. Neurol. Neurosurg. Psychiatr. · Sep 2003
Case ReportsHereditary diffuse leucoencephalopathy with spheroids.
- N Hancock, M Poon, B Taylor, and C McLean.
- Department of General Internal Medicine, Royal Hobart Hospital, Tasmania, Australia. nicole.hancock@dhhs.tas.gov.au
- J. Neurol. Neurosurg. Psychiatr. 2003 Sep 1; 74 (9): 1345-7.
AbstractHereditary 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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