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  • Title: Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia can present as frontotemporal dementia syndrome.
    Author: Wong JC, Chow TW, Hazrati LN.
    Journal: Dement Geriatr Cogn Disord; 2011; 32(2):150-8. PubMed ID: 21986056.
    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.
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