Suezumi Koki, Tagawa Asako, Ogawa Tomoko, Hashimoto Ritsuo, Otsuka Mieko, Kato Hiroyuki
Department of Neurology, International University of Health and Welfare Hospital.
Rinsho Shinkeigaku. 2018 Sep 28;58(9):560-564. doi: 10.5692/clinicalneurol.cn-001178. Epub 2018 Aug 31.
After a 34-year-old female developed a headache and high fever, she was diagnosed with aseptic meningitis. On admission, neurological examinations revealed cerebellar limb ataxia, horizontal gaze paretic nystagmus, and pyramidal tract signs. Laboratory tests showed hyponatremia (129 mEq/l). Five days after admission, convulsions in the upper limbs due to the severe hyponatremia (108 mEq/l) were noted. In addition, serum antidiuretic hormone levels were markedly increased to 18.5 pg/ml. Brain MRI showed multiple small inflammatory lesions in the subcortical cerebral white matter, thalamus, and around the third ventricular diencephalic regions. Pulse corticosteroid treatment promptly improved her symptoms. Although tests for serum anti-aquaporin 4, anti-myelin oligodendrocyte glycoprotein, and anti-voltage-gated potassium channel antibodies were negative, cerebrospinal fluid samples tested positive for anti-N-methyl-D-aspartate (NMDA) receptor antibodies. Oral prednisolone administration was continued, but she developed paresthesia in her upper and lower extremities and gaze-evoked nystagmus three months after the first attack. MRI showed that the previously observed high-intensity regions were decreased, but a new area of high intensity was observed in ventral regions through the lower midbrain to the pons. Because pulse corticosteroid treatment was again effective, we continued the oral prednisolone treatment. This case presented none of the characteristic symptoms of anti-NMDA receptor antibody encephalitis during the clinical course other than repeated demyelinating encephalitis and severe syndrome of inappropriate antidiuretic hormone secretion (SIADH). Additional clinical observations are needed to better understand the underlying pathology of the NMDA receptor antibodies in the cerebrospinal fluid in this case.
一名34岁女性出现头痛和高热后，被诊断为无菌性脑膜炎。入院时，神经系统检查发现小脑肢体共济失调、水平凝视麻痹性眼球震颤和锥体束征。实验室检查显示低钠血症（129 mEq/l）。入院五天后，因严重低钠血症（108 mEq/l）出现上肢惊厥。此外，血清抗利尿激素水平显著升高至18.5 pg/ml。脑部MRI显示皮质下脑白质、丘脑和第三脑室间脑区域周围有多个小炎症病灶。脉冲皮质类固醇治疗迅速改善了她的症状。尽管血清抗水通道蛋白4、抗髓鞘少突胶质细胞糖蛋白和抗电压门控钾通道抗体检测均为阴性，但脑脊液样本抗N-甲基-D-天冬氨酸（NMDA）受体抗体检测呈阳性。继续口服泼尼松龙，但首次发作三个月后她出现了上下肢感觉异常和凝视诱发性眼球震颤。MRI显示先前观察到的高强度区域减少，但在从下中脑到脑桥的腹侧区域观察到一个新的高强度区域。由于脉冲皮质类固醇治疗再次有效，我们继续口服泼尼松龙治疗。该病例在临床过程中除了反复出现脱髓鞘性脑炎和严重抗利尿激素分泌不当综合征（SIADH）外，没有出现抗NMDA受体抗体脑炎的特征性症状。需要进一步的临床观察，以更好地了解该病例脑脊液中NMDA受体抗体的潜在病理。
