Ueki Y, Kohara N, Oga T, Fukuyama H, Akiguchi I, Kimura J, Shibasaki H
Department of Neurology, Faculty of Medicine, Kyoto University, Japan.
Acta Neurol Scand. 2000 Jul;102(1):60-4. doi: 10.1034/j.1600-0404.2000.102001060.x.
A case of membranous lipodystrophy (Nasu-Hakola disease; NHD) associated with palilalia was reported. A 38-year-old Japanese woman developed walking difficulty in her twenties. At age 35 she manifested neuropsychiatric symptoms characterized by euphoria, palilalia and dementia. A bone marrow biopsy showed periodic acid Schiff-positive membranous cystic lesions in the adipose tissue. Positron emission tomography with (18F)-2-fluoro-2-deoxy-D-glucose disclosed that regional cerebral glucose metabolism was decreased in the bilateral frontal white matter with mild hypometabolism in the thalamus and basal ganglia; all predominantly on the right. Taken together with the previous postmortem findings, it is postulated that frontal lobe hypofunction, predominantly in the right hemisphere, produced the unique neuropsychiatric symptoms in this patient.
报告了一例与复述症相关的膜性脂肪营养不良（那须-哈科拉病；NHD）病例。一名38岁的日本女性在二十多岁时出现行走困难。35岁时，她出现了以欣快、复述症和痴呆为特征的神经精神症状。骨髓活检显示脂肪组织中有高碘酸希夫阳性的膜性囊性病变。用（18F）-2-氟-2-脱氧-D-葡萄糖进行正电子发射断层扫描显示，双侧额叶白质区域脑葡萄糖代谢降低，丘脑和基底神经节有轻度代谢减低；均以右侧为主。结合之前的尸检结果，推测主要是右半球额叶功能减退导致了该患者独特的神经精神症状。
