Clinical Characteristica of 21 Cases Series with Anti-γ-aminobutyric Acid B Receptor Encephalitis / 中风与神经疾病杂志

@#Objective　To summarize the clinical characteristics，treatment and outcome of 21 patients with anti-γ-Aminobutyric acid-B receptor （GABA-B） encephalitis. Methods　The clinical data of inpatients with anti-GABABR encephalitis at Neurology Department of Beijing Youanmen Hospital from October 2012 to December 2019 were retrospectively collected. all patients were followed up by outpatient visits or telephone. The good prognosis was defined as a mRS of 0 to 2 points. The clinical feature，treatment and followed-up outcome were analyzed. Results　A total of 21 patients were included in this analysis，with a median age of 57 years （range：11～77 years） and 14 males. The major clinical manifestations included seizures （21/21），cognitive impairment （19/21），psychiatric symptoms （19/21），involuntary movement （7/21），speech impairment/silence （14/21），sleep disorders（13/21），disturbance of consciousness（13/21），involuntary movement（7/21），autonomic nervous dysfunction（6/21）and ataxia（1/21）. Lung cancer was detected in 9（9/21） patients. Electroencephalogram （EEG） showed mild to moderate abnormal changes in 11（11/14）cases. The head magnetic resonance imaging showed abnormal signals in 12（12/21）cases，with the most involved area in temporal lobe. All the 21 patients showed anti-GABABR positive in serum and cerebrospinal fluid. 17 patients received the first-line immunotherapy，except 3 patients showing no effect，all the other patients responded well to the treatment. During a median follow-up of 16（range：3～30 months），11 cases had good prognosis，9 cases died，and 4 cases had recurrent encephalitis. Conclusion　The cases series with anti-GABABR encephalitis and limbic encephalitis presentation were more common in middle-aged and elderly men. The main clinical symptoms were epilepsy and neuropsychiatric disorders，and most epilepsy were refractory epilepsy. The proportion of concomitant tumor in the patients with this disorder was high，especially lung cancer. Most of these patients responded well to immunotherapy and had a good prognosis，and the patients with lung cancer had a relatively poor prognosis.