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

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

Author information

1
Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Ont., Canada.

Abstract

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.

PMID:
21986056
DOI:
10.1159/000331422
[Indexed for MEDLINE]
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