Dogan Onugoren Müjgan, Rauschka Helmut, Bien Christian G
Epilepsy Centre Bethel, Krankenhaus Mara, Bielefeld, Germany.
Department of Neurology, Sozialmedizinisches Zentrum Ost - Donauspital, Vienna, Austria.
J Neuroimmunol. 2014 Aug 15;273(1-2):115-6. doi: 10.1016/j.jneuroim.2014.05.011. Epub 2014 May 29.
Antibodies (abs) to the GABAB receptor have been recently found to be responsible for immune-mediated encephalitis with dominant seizures. They are in approximately 50% of cases associated with small-cell lung cancer (SCLC). GABAB receptors are mainly located in the hippocampus, thalamus and cerebellum in the presynaptic and postsynaptic regions of synapses. The main function of these receptors is to reduce activity states of neurons. In some instances, GABAB receptor abs in these patients were accompanied by other antibodies, among them VGCC abs (Lancaster et al., 2010, Boronat et al., 2011). VGCC abs cause paraneoplastic Lambert Eaton myasthenic syndrome (LEMS) by reduction of presynaptic VGCCs (Titulaer et al., 2011). In the domain of CNS disease, VGCC abs have been found in association with paraneoplastic cerebellar ataxia (Mason et al., 1997) and rarely and at low titres also in other paraneoplastic encephalopathies together with Hu abs (Lennon et al., 1995). It has been a long-standing debate if abs in paraneoplastic conditions associate rather with the neurological syndrome or the tumour. Here, we describe the conjoint occurrence of abs to the GABAB receptor and to the VGCC in a patient with SCLC presenting only symptoms of the peripheral nervous system giving another example of the latter hypothesis.
最近发现,针对GABAB受体的抗体(abs)可导致以癫痫发作为主的免疫介导性脑炎。在大约50%的病例中,它们与小细胞肺癌(SCLC)有关。GABAB受体主要位于海马体、丘脑和小脑突触的突触前和突触后区域。这些受体的主要功能是降低神经元的活动状态。在某些情况下,这些患者体内的GABAB受体抗体还伴有其他抗体,其中包括电压门控性钙通道抗体(VGCC abs,Lancaster等人,2010年;Boronat等人,2011年)。VGCC抗体通过减少突触前电压门控性钙通道导致副肿瘤性兰伯特-伊顿肌无力综合征(LEMS,Titulaer等人,2011年)。在中枢神经系统疾病领域,已发现VGCC抗体与副肿瘤性小脑共济失调有关(Mason等人,1997年),在其他副肿瘤性脑病中也很少见且滴度较低,同时伴有Hu抗体(Lennon等人,1995年)。关于副肿瘤性疾病中的抗体是更多地与神经综合征还是肿瘤相关,一直存在长期争论。在此,我们描述了一名仅表现出周围神经系统症状的小细胞肺癌患者同时出现GABAB受体抗体和VGCC抗体的情况,为后一种假设提供了另一个例子。