In:
Dementia and Geriatric Cognitive Disorders, S. Karger AG, Vol. 32, No. 2 ( 2011), p. 150-158
Abstract:
〈 i 〉 Background/Aims: 〈 /i 〉 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. 〈 i 〉 Methods: 〈 /i 〉 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. 〈 i 〉 Results: 〈 /i 〉 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 〈 sub 〉 2 〈 /sub 〉 -weighted MRI, motor symptoms, seizures and amnesia were common. 〈 i 〉 Conclusion: 〈 /i 〉 ALSP can underlie FTD syndrome, as well as rapidly progressive dementia.
Type of Medium:
Online Resource
ISSN:
1420-8008
,
1421-9824
Language:
English
Publisher:
S. Karger AG
Publication Date:
2011
detail.hit.zdb_id:
1482186-2
