Hereditary diffuse leucoencephalopathy with spheroids. Issue 9 (21st August 2003)

• Record Type: Journal Article
• Authors: Hancock, N; Poon, M; Taylor, B; McLean, C
• Is Part Of: Journal of neurology, neurosurgery and psychiatry. Volume 74:Issue 9(2003)
• Page Start: 1345
• Page End: 1347
• Publication Date: 2003-08-21
• Subjects: dementia -- hereditary -- leucoencephalopathy; Neurology -- Periodicals; Nervous system -- Surgery -- Periodicals; Psychiatry -- Periodicals; 616.8
• DOI: 10.1136/jnnp.74.9.1345
• Languages: English
• ISSNs: 0022-3050

Abstract

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.