Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia can present as frontotemporal dementia syndrome

Dement Geriatr Cogn Disord. 2011;32(2):150-8. doi: 10.1159/000331422. Epub 2011 Oct 5.


Background/aims: 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.

Conclusion: ALSP can underlie FTD syndrome, as well as rapidly progressive dementia.

Publication types

  • Case Reports
  • Research Support, Non-U.S. Gov't
  • Review

MeSH terms

  • Axons / pathology*
  • Diagnosis, Differential
  • Frontotemporal Dementia / diagnosis*
  • Frontotemporal Dementia / pathology
  • Frontotemporal Dementia / psychology
  • Genetic Diseases, Inborn / diagnosis
  • Genetic Diseases, Inborn / pathology
  • Humans
  • Leukoencephalopathies / diagnosis*
  • Leukoencephalopathies / genetics
  • Leukoencephalopathies / pathology
  • Leukoencephalopathies / psychology
  • Male
  • Middle Aged
  • Neuroglia / pathology*
  • Pigmentation
  • Spheroids, Cellular / pathology*