Abstract
ObjectiveTo improve the diagnosis and treatment of anti-GABAB receptor (anti-GABABR) encephalitis and prevent misdiagnosis or non-diagnosis.MethodsWe retrospectively examined the chief clinical manifestations, auxiliary examination results, treatment strategies, treatment efficacy, and long-term follow-up results of seven consecutive patients with anti-GABABR encephalitis.ResultsEpileptic seizures were the first symptom in 100% of the patients; 85.7% had memory deficit in the hospital, 42.8% had residual symptoms of cognitive impairment at discharge, and 28.6% had cognitive impairment at the end of follow-up; 71.4% of the patients had psychosis in the hospital, 57.1% had residual symptoms of psychosis at discharge, and 14.3% still had psychosis at the end of follow-up. However, the clinical symptoms (psychiatric disorders, cognitive decline) and signs (consciousness disturbance) at onset and after follow-up were not significantly different (P > 0.05). In 71.4% of the patients, anti-GABABR antibody serum levels were higher than those in the cerebrospinal fluid (especially in patients with lung cancer). Magnetic resonance imaging in 71.4% of patients indicated that the marginal lobe demonstrated encephalitis lesions. The average modified Rankin Scale score (2.0 ± 2.31) at follow-up was significantly better than that (3.86 ± 0.90) at the time of admission (P < 0.05).ConclusionThe clinical characteristics of anti-GABABR encephalitis were refractory epilepsy, psychiatric disorders, and cognitive impairment. Multiple antiepileptic drugs are crucial for the treatment of intractable epilepsy. Clinicians should eliminate the possibility of small-cell lung cancer in patients with high anti-GABABR antibody levels. Early active immunotherapy is effective, and the long-term prognosis is good for patients without tumors.
Highlights
Anti-GABAB receptor encephalitis is a newly reported form of autoimmune encephalitis associated with anti-neuron surface antigen antibodies [1]
The rate of misdiagnosis is high, since only a few cases have been reported to date and the clinical manifestations and prognosis of antiGABABR encephalitis have yet to be investigated systematically [2]
All patients met the following diagnostic criteria [3] for antiGABABR encephalitis: (1) acute or subacute onset, progressive aggravation; (2) clinical symptoms in accordance with the characteristics of marginal encephalitis; (3) slightly elevated lymphocyte levels and/or normal white blood cell (WBC) count in the cerebrospinal fluid (CSF); (4) presence of antiGABABR antibodies in the serum and/or CSF; abnormal signals in the unilateral/bilateral medial temporal lobe or an absence of lesions on brain magnetic resonance imaging (MRI); and (5) abnormal electroencephalogram (EEG) findings
Summary
Anti-GABAB receptor (anti-GABABR) encephalitis is a newly reported form of autoimmune encephalitis associated with anti-neuron surface antigen antibodies [1]. The rate of misdiagnosis is high, since only a few cases have been reported to date and the clinical manifestations and prognosis of antiGABABR encephalitis have yet to be investigated systematically [2]. We analyzed the clinical manifestations, auxiliary examination results, treatment strategies, and long-term prognoses in seven consecutive patients with anti-GABABR encephalitis, to improve the diagnosis and treatment of antiGABABR encephalitis. The study included seven consecutive patients (women: 3, men: 4; age: 44.7 ± 14.09 years, range: 27–64 years) with anti-GABABR encephalitis, who were treated at the Department of Neurology at the First Affiliated Hospital of Guangxi Medical University (China). No patient had a relevant family history of genetic abnormalities, immunodeficiency history, or developmental abnormalities
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