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Hereditary diffuse leucoencephalopathy with spheroids.

N Hancock·M Poon·B Taylor·C McLean
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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.

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AtrophyBrainBrain IschemiaCerebrovascular DisordersCognition DisordersFemaleFrontal LobeHumansMagnetic Resonance ImagingMiddle AgedCase ReportsJournal Article

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N Hancock, M Poon, B Taylor, C McLean. 2003. Hereditary diffuse leucoencephalopathy with spheroids.. https://doi.org/10.1136/jnnp.74.9.1345

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