Hereditary diffuse leucoencephalopathy with spheroids.
case_report · Level V
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- Record sourced from PubMed, PMID 12933955.
- Also identified by PMC identifier 1738687.
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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.
Medical subject headings
- Brain Ischemia
- Cerebrovascular Disorders
- Cognition Disorders