■对伴有抗两栖类蛋白抗体的自身免疫性脑炎的临床特征的分析。
■回顾性评估了使用抗两栖类蛋白抗体的脑炎患者的数据,包括人口统计,神经和实验室检查结果,成像,治疗,和预后预测。
■包括10名年龄在29至78岁(中位年龄52岁)之间的患者。男女比例为4:6。在9例患者中发现了边缘叶脑炎,而在7例患者中发现了癫痫发作。所有患者在血清中均显示抗两栖生理素抗体阳性,而1/9的CSF抗体阳性。脑电图检查结果异常,包括背景活动的减少,以及扩散慢波的存在,尖锐的波浪,尖峰和波浪。5例患者在MRI上表现出颞叶内侧T2信号增加的迹象,而PET在几个脑区表现出高代谢或低代谢变化。包括颞叶,海马体,基底神经节,额叶和顶叶皮质。10例患者中有9例接受了免疫治疗,有不同程度的改进。癫痫发作频率显著降低,最后随访时所有患者均无癫痫发作。
■使用抗两栖类蛋白抗体的自身免疫性脑炎具有多种临床表现。最常见的症状是边缘叶脑炎。虽然减轻癫痫发作可以相对容易地实现,许多病人患有精神病,认知,和睡眠后遗症。与先前报道的副肿瘤神经综合征相比,发现该疾病与癌症发病率较低有关。
An analysis of the clinical features of autoimmune encephalitis accompanied by anti-
amphiphysin antibodies.
The data of encephalitis patients with anti-
amphiphysin antibodies were retrospectively evaluated, including demographics, neurological and laboratory findings, imaging, treatment, and prognostic predictions.
Ten patients aged between 29 and 78 years (median age 52 years) were included. The male: female ratio was 4:6. Limbic encephalitis was found in nine patients while epileptic seizures were present in seven patients. All patients showed anti-
amphiphysin antibody positivity in sera while one ninth was positive for CSF antibody. The EEG findings were abnormal, including reductions in background activity, and the presence of diffuse slow waves, sharp waves, and spikes and waves. Five patients showed signs of increased T2 signals in the medial temporal lobe on MRI while PET showed either hyper- or hypo-metabolic changes in several brain regions, including the temporal lobe, hippocampus, basal ganglia, frontal and parietal cortices. Nine of ten patients were treated with immunotherapy, with improvements of varying degrees. There was a significant reduction in seizure frequency, and all patients were seizure-free at last follow-up.
Autoimmune encephalitis with anti-
amphiphysin antibodies has a variety of clinical manifestations. The most common symptom is limbic encephalitis. Although relief from seizures can be achieved relatively easily, many patients suffer psychiatric, cognitive, and sleep sequelae. The disease was found to be associated with a lower incidence of cancer than has been previously reported for paraneoplastic neurologic syndromes.