Clinical characteristics and prognostic analysis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis in Northeast China

Abstract

ObjectiveTo investigate the clinical characteristics and prognosis of anti-gamma-aminobutyric acid-B (GABA-B) receptor encephalitis.MethodsThis retrospective study enrolled nineteen patients with anti-GABA-B receptor encephalitis. Clinical manifestations, radiological and electroencephalogram features, treatment and outcomes were collected and analyzed. The neurological function was evaluated according to the modified Rankin Scale (mRS).ResultsThere were eleven patients in the favorable-prognosis group (mRS ≤ 2) and eight patients in the poor-prognosis group (mRS > 2). In the favorable-prognosis group, clinical symptoms included memory deterioration (n = 10; 90.9%), epileptic seizures (n = 9; 81.8%), psychiatric disorders (n = 9; 81.8%), and conscious disturbance (n = 5; 45.5%); magnetic resonance imaging (MRI) indicated an involvement of the limbic system in three (27.3%) cases in this group. Lung cancer was detected in one patient (9.1%). After an average follow-up period of 11.7 months, four (36.4%) patients were cured, and seven (63.6%) patients showed significant improvements. In the poor-prognosis group, all patients presented with memory deterioration, epileptic seizures, psychiatric disorders, and conscious disturbance; five (62.5%) patients had convulsive status epilepticus, and five (62.5%) patients developed respiratory failure; MRI indicated an involvement of the limbic system in seven (87.5%) cases. Malignant tumors were detected in five (62.5%) patients. After an average follow-up period of 14.8 months, seven (87.5%) patients died and one (12.5%) patient remained dependent in daily life.ConclusionsThe clinical manifestations of anti-GABA-B receptor encephalitis include epileptic seizures, cognitive impairment and psychiatric disorders. Patients with convulsive status epilepticus or respiratory failure have poor outcomes. In anti-GABA-B receptor encephalitis, limbic system involvement is associated with a poor prognosis in and radiological examinations can reflect disease progression. Early diagnosis and appropriate treatment should be highlighted.

Clinical characteristics and long-term follow-up of seven cases of anti-GABABR encephalitis in patients of Han Chinese descent

“…However, only a few patients in our study had prodromal symptoms of infection; infection was not a trigger for the onset in most patients. Epilepsy is a prominent and critical clinical manifestation of anti-GABABR encephalitis, and epilepsy or refractory epilepsy is often the first symptom [6]. In a previous study by Guan et al, 17 of the 18 patients presented with new refractory epilepsy or status epilepticus [7], while all the patients in our study had epilepsy.…”

Anti‐LGI1, anti‐GABABR, and Anti‐CASPR2 encephalitides in Asia: A systematic review

“…17/18 were said to have presented with new-onset refractory seizures. Dysautonomia was usually reported in the form of dysrhythmia, and even diarrhea in one of the patients (Zhang et al, 2020).…”

Neuropsychiatric Disorders Due to Limbic Encephalitis: Immunologic Aspect

“…Co-expression of GABA-BR with other autoantibodies is relatively common [ 63 , 115 , 116 , 117 , 118 ]. Reported coexisting antibodies in GABA-BR encephalitis included potassium channel tetramerization domain containing 16 (KCTD16), GAD65, Hu, Ri, amphiphysin, SOX1, CV2/CRMP, 5N-type or P/Q-type voltage-gated calcium channels, or thyroid peroxidase [ 115 , 116 , 117 , 119 , 120 ]. Most of these cases were paraneoplastic.…”