Department of Neurology, Institute of Neurological Sciences, Southern General Hospital, Glasgow, UK.
Scott Med J. 2009 Nov;54(4):27-31. doi: 10.1258/rsmsmj.54.4.27.
The syndrome of limbic encephalitis (LE) associated with antibodies against voltage-gated potassium channels (VGKC-LE) has recently been described. The number of published cases is however small. We therefore aimed to review all cases seen at our centre and compare with published cases.
Retrospective cases of VGKC-LE were identified using a questionnaire to Neurologists at the Southern General hospital, Glasgow, and by reviewing patients with a positive VGKC antibody test (2002-2007). Case-note review of identified cases and a literature review of all published cases of VGKC-LE were performed.
Seven cases were identified (four female, age range 51-81). Patients presented sub-acutely with seizures and anterograde memory loss. Five patients had medial temporal lobe change on cranial imaging. No paraneoplastic cases were identified. 5/7 patients made some improvement with immunotherapy. In 2006, 3/18 (17%) patients with a coded discharge of encephalitis were diagnosed with VGKC-LE. The literature review revealed 40 patients with VGKC-LE. Age, gender or VGKC level did not predict likelihood for a significant recovery. Patients treated < or =5 months of symptom onset with immunotherapy were more likely to make a significant recovery (83% vs. 45%, p=0.04).
VGKC-LE is being increasingly diagnosed and is best identified early and treated with immunotherapy to offer the greatest chance of recovery. This series and literature review expands the current published evidence in VGKC-LE.
电压门控钾通道(VGKC)相关抗体的边缘性脑炎(LE)综合征最近已被描述。然而,发表的病例数量较少。因此,我们旨在回顾我们中心所见的所有病例,并与已发表的病例进行比较。
通过向格拉斯哥南部总医院的神经科医生使用问卷以及通过检查 VGKC 抗体检测呈阳性的患者(2002-2007 年),确定了 VGKC-LE 的回顾性病例。对确定的病例进行病历回顾,并对所有已发表的 VGKC-LE 病例进行文献复习。
共发现 7 例病例(4 例女性,年龄 51-81 岁)。患者亚急性发作出现癫痫发作和顺行性记忆丧失。5 例患者颅成像有内侧颞叶改变。未发现副肿瘤病例。5/7 例患者经免疫治疗后有所改善。2006 年,18 例编码为脑炎的患者中有 3 例被诊断为 VGKC-LE。文献复习显示有 40 例 VGKC-LE 患者。年龄、性别或 VGKC 水平均不能预测是否有显著恢复。免疫治疗开始症状出现后 <或=5 个月的患者更有可能显著恢复(83%比 45%,p=0.04)。
VGKC-LE 的诊断越来越多,最好早期识别并进行免疫治疗,以提供最大的恢复机会。本系列和文献综述扩展了目前已发表的关于 VGKC-LE 的证据。