Molloy Anna, Cassidy Eugene, Ryan Aisling, O' Toole Orna
Neurology Department, Cork University Hospital, Cork, Ireland.
BMJ Case Rep. 2011 Dec 1;2011:bcr0820114642. doi: 10.1136/bcr.08.2011.4642.
Voltage gated potassium channel antibodies (VGKC Abs) are known to cause three rare neurological syndromes- neuromyotonia, Morvan's syndrome and limbic encephalitis although an increasing array of other associated neurological symptoms are becoming recognised. The authors describe the case of a 60-year-old female who presented to the neurology clinic with an apparent early onset dementing process. She was noted to have both extrapyramidal and frontal release signs on examination and was admitted for further evaluation. Her dementia investigation including a neoplastic screen was negative except for VGKC antibody positivity. Her symptoms dramatically improved with commencement of immunosuppression. A non-paraneoplastic VGKC antibody associated dementia-like syndrome has rarely been described. The authors add to the few existing reports of what represents an important reversible cause of cognitive impairment.
电压门控钾通道抗体(VGKC抗体)已知可引发三种罕见的神经系统综合征——神经性肌强直、莫旺综合征和边缘叶脑炎,尽管越来越多的其他相关神经症状也逐渐得到认识。作者描述了一名60岁女性的病例,该患者到神经科门诊就诊,表现出明显的早期痴呆进程。检查发现她既有锥体外系症状又有额叶释放征,并因此住院接受进一步评估。除了VGKC抗体呈阳性外,她的痴呆相关检查包括肿瘤筛查均为阴性。免疫抑制治疗开始后,她的症状显著改善。非副肿瘤性VGKC抗体相关的类痴呆综合征鲜有报道。作者补充了为数不多的现有报告,这些报告指出了认知障碍的一个重要可逆病因。