title: Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia can present as frontotemporal dementia syndrome.
abstract: OBJECTIVE
  We review the characteristics of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia(ALSP) and determine prevalence of behavioral variant of frontotemporal dementia (bvFTD) features in ALSP.
  
  
  METHODS
  Clinical and pathological information was abstracted from histopathologically confirmed ALSP cases identified by a systematic literature search. A new case of ALSP presenting as bvFTD was also described.
  
  
  RESULTS
  We retrieved 51 ALSP cases. Mean age of onset was 42.2 years. Mean disease duration was 6.2 years, with 24 cases lasting 4 years or fewer. Fourteen cases had 3 or more of the 6 key bvFTD features. White matter hyperintensities on T(2)-weighted MRI, motor symptoms, seizures and amnesia were common.
  
  
  CONCLUSIONS
  ALSP can underlie FTD syndrome, as well as rapidly progressive dementia.
authors: Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Ont., Canada.
authors: Janice C
authors: JC
authors: Wong
authors: Tiffany W
authors: TW
authors: Chow
authors: Lili-Naz
authors: LN
authors: Hazrati
mesh_terms: D001369:Axons / Q000473:pathology*; D003937:Diagnosis, Differential; D057180:Frontotemporal Dementia / Q000175:diagnosis* / Q000473:pathology / Q000523:psychology; D030342:Genetic Diseases, Inborn / Q000175:diagnosis / Q000473:pathology; D006801:Humans; D056784:Leukoencephalopathies / Q000175:diagnosis* / Q000235:genetics / Q000473:pathology / Q000523:psychology; D008297:Male; D008875:Middle Aged; D009457:Neuroglia / Q000473:pathology*; D010858:Pigmentation; D018874:Spheroids, Cellular / Q000473:pathology*