Hypothermia in VGKC antibody-associated limbic encephalitis

Hypothermia in VGKC antibody-associated limbic encephalitis
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DOI:
10.1136/jnnp.2007.130039
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发表时间:
2008-02-01
影响因子:
11
通讯作者:
Vincent, A.
Vincent, A.
中科院分区:
医学1区
文献类型:
--
作者:
Jacob, S.;Irani, S. R.;Vincent, A.

文献摘要

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电压门控钾通道抗体(VGKC-Ab)相关边缘脑炎(LE)是最近发现的一种综合征,拓宽了免疫治疗反应性中枢神经系统疾病的范围。边缘脑炎的典型特征是亚急性发作的定向障碍、健忘症和癫痫,但临床频谱尚未完全定义,该综合征可能未得到充分诊断。我们在这里描述了4例vgkc - ab相关LE患者的临床资料,这些患者患有间歇性、偶发性低体温。其中一名患者还描述了在边缘症状发展之前的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,同时健忘症/癫痫发作得到改善。
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.