Supplementary Material for: Adult-Onset Leukoencephalopathy with Axonal Spheroids and Pigmented Glia Can Present as Frontotemporal Dementia Syndrome

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 T2-weighted MRI, motor symptoms, seizures and amnesia were common. Conclusion: ALSP can underlie FTD syndrome, as well as rapidly progressive dementia.

背景与研究目的：本研究旨在综述伴轴索球体与色素性胶质细胞的成人起病脑白质病（ALSP）的临床特征，并明确ALSP患者中额颞叶痴呆行为变异型（bvFTD）特征的患病率。研究方法：通过系统文献检索获取经组织病理学确诊的ALSP病例，提取其临床与病理资料；同时报告1例以bvFTD为首发表现的ALSP新病例。研究结果：本次检索共纳入51例ALSP病例，患者平均起病年龄为42.2岁，平均病程为6.2年，其中24例患者病程≤4年；14例患者具备6项核心bvFTD特征中的3项及以上。T2加权磁共振成像可见脑白质高信号，运动症状、癫痫发作及遗忘症较为常见。研究结论：ALSP可作为额颞叶痴呆（FTD）综合征及快速进展性痴呆的潜在病因。