Clinical characteristics of 12 patients with anti-leucine rich glioma inactivated protein 1 encephalitis / 中华神经科杂志

ABSTRACT

Objective To analyze the clinical features and prognosis of anti-leucine rich glioma inactivated protein 1 (LGI1) encephalitis.Methods Twelve encephalitis patients with anti-LGI1 antibodies were collected from the First Affiliated Hospital of Zhengzhou University from June 2015 to December 2016.The clinical manifestations,electroencephalogram,laboratory examination and imaging findings were summarized and the prognosis was observed.The modified Rankin Scale (mRS) was used for evaluation before and after treatment.Results The major clinical features included memory deficit (10/12),spatial disorientation (7/12),epilepsy with generalized tonic-clonic seizures (9/12),faciobrachial dystonic seizures (7/12),hyponatremia (5/12),mental and behavioral abnormalites (1/12),light sleep (1/12),increased sleep (3/12),aphasis (4/12),dysphagia,choking (2/12),headache (1/12),dizziness (2/12),fatigue (2/12),ataxia (2/12),bradycardia (3/12),urinary disorders (2/12),intestinal obstruction (1/12),diarrhea (1/12).Admission mRS score was found to be three in eight cases,four in four cases.The abnormal electroencephalogram was found in six cases,mainly manifested as focal or diffuse slow wave,some accompanied by epileptic wave.MRI scan of brain showed abnormal signals in four cases,mainly involved medial temporal lobe,hippocampus,basal ganglia,while one patient avoided MRI scan due to implantation of pacemaker.Two patients presented with pulmonary nodules,one case with positive thyroid antibody and increased rheumatoid factor.The follow-up after treatment showed no one died;mRS score was two in two cases,one in nine cases and zero in one case;the sequelae were memory deficit,increased sleep,faciobrachial dystonic seizures.Conclusions Anti-LGI1 encephalitis is a treatable disease,cardinal clinical features of which are seizures,cognitive disorders,hyponatremia.Immunotherapy can improve the symptoms of the disease significantly,and the prognosis is better comparatively.