Department of Neurology, University Hospital Heidelberg, Im Neuenheimer Feld 400, 69120 Heidelberg, Germany.
J Neuroimmunol. 2013 Jun 15;259(1-2):88-91. doi: 10.1016/j.jneuroim.2013.04.004. Epub 2013 Apr 28.
Gamma-aminobutyric-acid B (GABA-B)-receptor encephalitis represents a novel entity among autoimmune CNS disorders. Most cases are characterised by limbic encephalitis.
A 63-year-old patient presented with acute vertigo, nausea and vomiting, facial palsy and dysarthria. He developed dysphagia, gait ataxia and, finally, respiratory failure. Antibodies to GABA-B receptors were positive and declined under treatment with intravenous methylprednisolone and plasma exchange, followed by clinical improvement and stabilisation. Broad tumour screening revealed oesophageal carcinoma.
The spectrum of neurological manifestations and tumours associated with the paraneoplastic variant of anti-GABA-B-receptor encephalitis may be broader than previously reported.
γ-氨基丁酸 B(GABA-B)受体脑炎是一种新型的自身免疫性中枢神经系统疾病。大多数病例以边缘性脑炎为特征。
一名 63 岁患者出现急性眩晕、恶心和呕吐、面瘫和构音障碍。他随后出现吞咽困难、步态共济失调,最终发展为呼吸衰竭。抗 GABA-B 受体抗体阳性,经静脉甲基泼尼松龙和血浆置换治疗后滴度下降,随后临床症状改善和稳定。广泛的肿瘤筛查发现食管癌。
与抗 GABA-B 受体脑炎的副肿瘤变异型相关的神经表现和肿瘤谱可能比之前报道的更广泛。