Adult-onset leukoencephalopathy with axonal spheroids and pigmented glia can present as frontotemporal dementia syndrome.
作者： Janice C. Wong , Tiffany W. Chow , Lili-Naz Hazrati
DOI: 10.1159/000331422
关键词:
摘要: Background/Aims: We review the characteristics of adult-onset leukoencephalopathy with axonal spheroids and pigmented glia(ALSP) determine prevalence behavioral variant frontotemporal dementia (bvFTD) features in ALSP. Methods: Clinical pathological information was abstracted from histopathologically confirmed ALSP cases identified by a systematic literature search. A new case presenting as bvFTD also described. Results: retrieved 51 cases. Mean age onset 42.2 years. disease duration 6.2 years, 24 lasting 4 years or fewer. Fourteen had 3 more 6 key features. White matter hyperintensities on T2-weighted MRI, motor symptoms, seizures amnesia were common. Conclusion: can underlie FTD syndrome, well rapidly progressive dementia.
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