背景：发作性毛发勃起（IP）是局灶性癫痫的一种罕见表现。自身免疫性边缘叶脑炎（LE）和恶性脑肿瘤是最常见的病因。
方法：我们选择了2004年至2020年在我们研究所诊断为LE并表现为IP的所有患者。我们对出现IP的LE患者进行了文献综述。
结果：在诊断为LE的15例患者中（13.3％），两个明显的IP作为突出的ictal特征。其中一人还患有四肢僵硬综合征。视频EEG记录了右侧颞区的发作性放电，并伴随着交感神经皮肤反应（SSR）记录。抗体检测显示GAD65抗体（Ab）的血清和CSF滴度升高，在这两种情况下。尽管结合了几种抗癫痫药物和一线和二线免疫疗法，经过2年和9年的随访，他们的临床结果不佳，分别。文献综述产生了13篇论文，报告了26例IPLE病例。LGI1Ab是最常见的相关（73.1％），其次是VGKC复合物（7.7％），GAD65（7.7%），NMDAr（3.8%），Ma2（3.8%）和Hu（3.8%）Ab。LGI1Ab的病例对免疫疗法表现出良好的反应。
结论：我们的LE队列中IP的患病率为13.3％，高于预期。根据文献综述,大多数病例与LGI1Ab相关,并对免疫治疗表现出良好的反应.有了我们案件的贡献，GAD65成为第二最常检测到的Ab，显示一个糟糕的结果。我们的发现扩大了IP相关Ab的范围，具有各自的预后意义。
BACKGROUND: Ictal piloerection (IP) is a rare manifestation of focal epilepsy. Autoimmune limbic encephalitis (LE) and malignant brain tumours are the most frequent recognized aetiologies.
METHODS: We selected all patients diagnosed with LE in our Institute from 2004 to 2020 and manifesting with IP. We performed a literature
review on LE patients presenting IP.
RESULTS: Of 15 patients diagnosed with LE (13.3%), two manifested IP as prominent ictal feature. One of them also had stiff-limb syndrome. Video-EEG documented ictal discharges from the right temporal regions with concomitant sympathetic skin response (SSR) recording. Antibody testing showed elevated serum and CSF titres of GAD65 antibodies (Ab), in both cases. Despite a combination of several anti-seizure medications and first- and second-line immunotherapy, they showed a poor clinical outcome after 2 and 9 years of follow-up, respectively. The literature
review yielded 13 papers reporting 26 LE cases with IP. LGI1 Ab were the most frequently associated (73.1%) followed by VGKC-complex (7.7%), GAD65 (7.7%), NMDAr (3.8%), Ma2 (3.8%) and Hu (3.8%) Ab. Cases with LGI1 Ab showed a good response to immunotherapy.
CONCLUSIONS: The prevalence of IP in our LE cohort was of 13.3%, higher than expected. According to the literature
review, most cases were associated with LGI1 Ab and showed a good response to immunotherapy. With the contribution of our cases, GAD65 emerged as the second most frequently detected Ab, showing a poor outcome. Our findings widen the spectrum of IP-associated Ab, with the respective prognostic implications.
