Jacob S, Irani S R, Rajabally Y A, Grubneac A, Walters R J, Yazaki M, Clover L, Vincent A
Department of Clinical Neurology, Weatherall Institute of Molecular Medicine, John Radcliffe Hospital, Headington, Oxford OX3 9DS, UK.
J Neurol Neurosurg Psychiatry. 2008 Feb;79(2):202-4. doi: 10.1136/jnnp.2007.130039.
Voltage-gated potassium channel antibody (VGKC-Ab)-associated limbic encephalitis (LE) is a recently described syndrome that broadens the spectrum of immunotherapy-responsive central nervous system disorders. Limbic encephalitis is typically characterised by a sub-acute onset of disorientation, amnesia and seizures, but the clinical spectrum is not yet fully defined and the syndrome could be under-diagnosed. We here describe the clinical profile of four patients with VGKC-Ab-associated LE who had intermittent, episodic hypothermia. One of the patients also described a prodrome of severe neuropathic pain preceding the development of limbic symptoms. Both of these novel symptoms responded well to immunosuppressive therapy, with concurrent amelioration of amnesia/seizures.
电压门控钾通道抗体(VGKC-Ab)相关边缘叶脑炎(LE)是一种最近描述的综合征,它拓宽了免疫治疗反应性中枢神经系统疾病的范围。边缘叶脑炎的典型特征是亚急性起病的定向障碍、失忆和癫痫发作,但临床谱尚未完全明确,该综合征可能诊断不足。我们在此描述4例伴有间歇性发作性体温过低的VGKC-Ab相关LE患者的临床特征。其中1例患者还描述了在边缘叶症状出现之前有严重神经性疼痛的前驱症状。这两种新症状对免疫抑制治疗反应良好,同时失忆/癫痫发作也有所改善。