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Short report
Hereditary diffuse leucoencephalopathy with spheroids
  1. N Hancock1,
  2. M Poon2,
  3. B Taylor3,
  4. C McLean4
  1. 1Department of General Internal Medicine, Royal Hobart Hospital, Tasmania, Australia
  2. 2Department of Neurology, Western Hospital, Melbourne, Australia
  3. 3Department of Neurology, Royal Hobart Hospital
  4. 4The National Health and Medical Research Council Dementia Brain Bank, The University of Melbourne and The Alfred Hospital, Melbourne, Australia
  1. Correspondence to:
 Dr N Hancock, Department of General Internal Medicine, Royal Hobart Hospital, GPO Box 1061L, Hobart 7001, Tasmania, Australia; 
 nicole.hancock{at}dhhs.tas.gov.au

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.

  • dementia
  • hereditary
  • leucoencephalopathy

https://doi.org/10.1136/jnnp.74.9.1345

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