Department of Neurology, Memory Clinic, Brugmann University Hospital [Vrije Universiteit Brussel (V.U.B.)], Belgium.
Alzheimer Dis Assoc Disord. 2012 Jan-Mar;26(1):96-9. doi: 10.1097/WAD.0b013e31821cc65d.
We describe an exceptional clinical picture, namely, cognitive impairment of the Alzheimer disease type in a man who later developed manifestations typical of amyotrophic lateral sclerosis and who was subsequently found to have adult polyglucosan body disease (APGBD) upon postmortem neuropathologic explorations. The combined occurrence of amyotrophic lateral sclerosis and cognitive impairment of the Alzheimer disease type in APGBD has not been reported before. This case also underlines the diverse clinical presentation of this rare clinicopathologic entity (namely APGBD) and highlights the importance of recognizing the unusual association of clinical features in making the diagnosis.
我们描述了一种特殊的临床表现，即一名男子出现阿尔茨海默病类型的认知障碍，随后出现典型的肌萎缩侧索硬化症表现，随后在死后神经病理学研究中发现患有成人多聚糖体病 (APGBD)。以前没有报道过 APGBD 同时发生肌萎缩侧索硬化症和阿尔茨海默病类型的认知障碍。该病例还强调了这种罕见临床病理实体（即 APGBD）的多样化临床表现，并强调了认识到不常见的临床特征关联对于做出诊断的重要性。
