Clinical Research
Clinical analysis of 42 autoimmune epilepsy patients with autoantibody
Ruijuan Lyu, Xiaoqiu Shao, Tao Cui, Maomao Liu, Zhimei Li, Weixiong Shi, Chao Chen, Qun Wang
Published 2018-06-08
Cite as Chin J Neurol, 2018, 51(6): 444-450. DOI: 10.3760/cma.j.issn.1006-7876.2018.06.008
Abstract
ObjectiveTo analyze the characteristics of clinical manifestation, brain magnetic resonance imaging (MRI) and 18F-fluoro-deoxy-glucose positron emission tomography (FDG-PET), inflammatory cerebrospinal fluid (CSF), electroencephalography (EEG), and associated tumour in autoimmune epilepsy (AE) patients with different autoantibodies.
MethodsForty-two patients diagnosed as AE with different autoantibodies in Beijing Tiantan Hospital, Capital Medical University between May 2014 and May 2017 were recruited. The clinical manifestation, brain MRI and PET, CSF findings, EEG and biochemical examination of these patients were analyzed.
ResultsSpecific autoimmune antibodies were detected in 42 patients, including anti-amphiphysin in one patient, anti-contactin-associated protein 2 in two, anti-γ-aminobutyric acid-B receptor in six, anti-leucine-rich glioma inactivated 1(LGI1) in 24, anti-N-methyl-D-aspartate receptor (NMDAR) in nine. The case series of 42 patients had an average age of (49.9±14.5) years with a male to female ratio of 5∶1. Except anti-NMDAR associated AE, most patients (21/33) presented with the symptoms of limbic encephalitis including temporal lobe seizures, memory decline, personality and neuropsychiatric changes, mesial temporal lobe abnormality in MRI or FDG-PET, and CSF inflammation. The seizure semiologic characteristics included frequent seizure, short seizure duration and common secondarily generalized tonic-clonic seizures during sleeping. Faciobrachial dystonic seizures and hyponatremia were the special clinical manifestation of AE with anti-LGI1. AE patients with all kinds of antibodies presented as initial resistance to anti-epilepsy drugs (AEDs) and favorable outcome of immunosuppressive treatment in combination with AEDs.
ConclusionsAE patients with each type of antibody have the special clinical manifestation. Except anti-NMDAR associated AE, the seizure semiologic characteristics often present as frequent and short seizures. All AE patients present as drug refractory epilepsy initially. Seizures in AE patients can be well controlled by immunotherapy combined with AEDs.
Key words:
Epilepsy; Autoimmunity; Hyponatremia; Anticonvulsants; Immunotherapy
Contributor Information
Ruijuan Lyu
Department of Neurology, Beijing Tiantan Hospital, Capital Medical University, China National Clinical Research Center for Neurological Diseases, Beijing 100050, China
Xiaoqiu Shao
Tao Cui
Maomao Liu
Zhimei Li
Weixiong Shi
Chao Chen
Qun Wang
