Browne Lorna, Sweeney Brian J, Farrell Michael A
Department of Clinical Neurological Sciences, Beaumont Hospital, Dublin, Ireland.
Eur Neurol. 2003;50(2):85-90. doi: 10.1159/000072504.
A 54-year-old man with a 7-year history of early-onset, slowly progressive dementia and motor impairment characterised by diffuse, non-enhancing white matter signal change. Neuropathologic examination demonstrated subcortical pigmentation with neuroaxonal spheroid formation, profound axonal loss with secondary myelin degeneration and widespread betaA4 immunopositivity involving meningeal and subcortical vessels. There was relative sparing of brain stem and cerebellar white matter. The neocortex was normal. The appearances expand the spectrum of adult-onset neuroaxonal leucoencephalopathy with spheroids (NALS) and demonstrate that vascular betaA4 amyloid plays a key role in the white matter pathology of NALS.
一名54岁男性，有7年早发型、缓慢进展性痴呆和运动障碍病史，其特征为弥漫性、无强化的白质信号改变。神经病理学检查显示皮质下色素沉着伴神经轴突球状体形成，严重轴突丢失伴继发性髓鞘变性，以及脑膜和皮质下血管广泛的βA4免疫阳性。脑干和小脑白质相对 spared。新皮质正常。这些表现扩展了成人起病的伴有球状体的神经轴突白质脑病（NALS）的范围，并表明血管βA4淀粉样蛋白在NALS的白质病变中起关键作用。 （注：“spared”疑有误，可能是“spared”，推测意为“ spared”，即“ spared”的过去式或过去分词形式，意为“幸免、 spared”。但由于原文有此拼写错误，暂按原样翻译，准确内容需结合更准确的原文判断。）
