Gozubatik-Celik Rabia Gokcen, Baykan Betul, Emekli Ahmed Serkan, Tuzun Erdem, Soysal Aysun, Uzunkopru Cihat, Ilhan Algın Demet, Comruk Erol, Şanlı Elif, Akkoyun Arıkan Fatma, Hanagası Hasmet, Kürtüncü Murat, Erturk Cetin Ozdem, Velioglu Sibel, Cerrahoglu Sirin Tuba, Yalaz Tekan Ulgen, Yayla Vildan, Cilingir Vedat, Yılmaz Vuslat, Basturk Ayhan Zeliha, Terzi Murat
Bakırköy Prof. Dr. Mazhar Osman Training and Research Hospital for Mental Health and Neurological Disorders, Department of Neurology, University of Health Sciences, Istanbul, Türkiye.
EMAR Medical Center, Department of Neurology, Istanbul, Türkiye.
Neurol Res. 2025 Jul;47(7):612-625. doi: 10.1080/01616412.2025.2495932. Epub 2025 May 2.
GABAB-R encephalitis is a rare recognized autoimmune disease. This study investigates the clinical and laboratory features, treatment response, prognosis, and malignancy associations in GABAB-R encephalitis.
We included consecutive encephalitis patients with GABAB-R autoantibodies and retrospectively analyzed their clinical data, neuroimaging, EEG findings, seizure characteristics, treatment responses, prognosis, and cancer presence. Prognosis was classified using the final Modified Rankin Score (mRS), with mRS > 2 indicating poor prognosis.
There were 17 patients with GABAB-R antibodies (12 males). The mean age at onset was 61.29 (range: 37-86), and the mean follow-up was 20.3 months (range: 6-60). The most common findings at onset were seizures, observed in 10 patients (58.8%), which increased to 13 patients (76.5%) during follow-up, psychiatric symptoms in 35.3%, and hyponatremia in 35.3%. Ten patients required intensive care unit (ICU) admission, and 11 patients had an underlying malignancy, predominantly lung cancer. Additionally, one patient had CASPR2 antibodies, and another had AMPA-R antibodies. Lesion probability map analysis revealed predominant involvement of the bilateral mesiotemporal regions. Patients with a final modified Rankin Scale Score greater than 2 ( = 10) exhibited a higher prevalence of psychiatric symptoms, ICU admission, and hyponatremia. Of the 12 patients on anti-seizure medications, only 8 achieved seizure-free status during follow-up. Those with a paraneoplastic etiology were more likely to present with psychiatric symptoms. Mortality, which occurred in 7 patients, was associated with persistent seizures (4/4 vs 3/10; = 0.015) and ICU admission (7/7 vs 3/10 = 0.010) Patients with both serum and CSF antibody positivity showed trends towards exhibiting higher rates of lung cancer and mortality.
Male gender and seizures are common in GABAB-R encephalitis, which also displays high malignancy and mortality rates. Remarkable prognostic factors include psychiatric symptoms, seizures, malignancy, and hyponatremia. 4(23%) of 17 patients with GABA-B receptor antibody encephalitis experience persistent seizures during follow-up.
GABAB-R 脑炎是一种罕见的已被认知的自身免疫性疾病。本研究调查了 GABAB-R 脑炎的临床和实验室特征、治疗反应、预后及与恶性肿瘤的关联。
我们纳入了连续的伴有 GABAB-R 自身抗体的脑炎患者，并回顾性分析了他们的临床资料、神经影像学、脑电图结果、癫痫发作特征、治疗反应、预后及是否存在癌症。使用最终的改良 Rankin 量表（mRS）对预后进行分类，mRS > 2 表明预后不良。
有 17 例伴有 GABAB-R 抗体的患者（12 例男性）。发病时的平均年龄为 61.29 岁（范围：37 - 86 岁），平均随访时间为 20.3 个月（范围：6 - 60 个月）。发病时最常见的表现为癫痫发作，10 例患者（58.8%）出现，随访期间增加至 13 例患者（76.5%），精神症状占 35.3%，低钠血症占 35.3%。10 例患者需要入住重症监护病房（ICU），11 例患者有潜在恶性肿瘤，主要为肺癌。此外，1 例患者有 CASPR2 抗体，另 1 例患者有 AMPA-R 抗体。病变概率图分析显示双侧颞叶内侧区域受累为主。最终改良 Rankin 量表评分大于 2（n = 10）的患者精神症状、入住 ICU 和低钠血症的发生率更高。在 12 例服用抗癫痫药物的患者中，随访期间只有 8 例达到无癫痫发作状态。副肿瘤病因的患者更易出现精神症状。7 例患者死亡，与持续性癫痫发作（4/4 对比 3/10；P = 0.015）和入住 ICU（7/7 对比 3/10；P = 0.010）相关。血清和脑脊液抗体均为阳性的患者显示出肺癌发生率和死亡率较高的趋势。
男性和癫痫发作在 GABAB-R 脑炎中较为常见，其还表现出高恶性率和死亡率。显著的预后因素包括精神症状、癫痫发作、恶性肿瘤和低钠血症。17 例 GABA-B 受体抗体脑炎患者中有 4 例（23%）在随访期间经历持续性癫痫发作。
