Tanz Centre for Research in Neurodegenerative Diseases, University of Toronto, Ont., Canada.
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.
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.
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.
ALSP can underlie FTD syndrome, as well as rapidly progressive dementia.
背景/目的：我们回顾了伴有轴索性球体和色素性神经胶质的成人起病脑白质病（ALSP）的特征，并确定了 ALSP 中行为变异型额颞叶痴呆（bvFTD）特征的患病率。
通过系统的文献检索，从组织病理学确诊的 ALSP 病例中提取临床和病理信息。还描述了一例表现为 bvFTD 的新的 ALSP 病例。
我们检索到 51 例 ALSP 病例。发病年龄平均为 42.2 岁。平均病程为 6.2 年，24 例病程为 4 年或更短。14 例有 6 个关键 bvFTD 特征中的 3 个或更多。T2 加权 MRI 上的脑白质高信号、运动症状、癫痫发作和遗忘症很常见。
ALSP 可引起 FTD 综合征，也可引起快速进展性痴呆。
