目的扩大与多巴胺2受体(D2R)抗体相关的急性神经系统综合征的症状谱。
一名13岁的青春期男孩因下颌运动异常而被送入神经内科。初步评估包括血液实验室检查,胸部X线摄影术,脑部MRI,脑电图,和神经心理测验.收集血清和CSF样品用于免疫学研究。首次住院后随访患者18个月的临床转归。
阵发性下颌阵痛,眼睑痉挛,在有Tourette综合征和强迫症病史以及与D2R抗体相关的急性神经系统综合征的患者中观察到流涕。症状对静脉注射甲基强的松龙(IVMP)有反应,在泼尼松减少期间复发两次,and,最后,IVMP和IV免疫球蛋白联合治疗后改善。
认识到阵发性下颌阵风(可能伴有眼睑痉挛和流涕)并考虑这些发作与D2R抗体之间的关系将有助于免疫神经综合征的早期诊断和治疗。
To extend the symptomatic spectrum of acute neurologic syndrome associated with dopamine-2 receptor (D2R) antibodies.
A 13-year-old adolescent boy was admitted to the Neurology Department with abnormal jaw movements. The initial evaluation included laboratory examinations of blood, chest radiography, brain MRI, EEG, and neuropsychologic tests. Serum and CSF samples were collected for immunologic studies. The clinical outcome of the patient was followed up for 18 months after the first hospitalization.
Paroxysmal jaw clonus, blepharospasm, and sialorrhea were observed in the patient with a history of Tourette syndrome and obsessive-compulsive disease and with an acute neurologic syndrome associated with D2R antibodies. The symptoms responded to IV methylprednisolone (IVMP), relapsed twice during prednisone reduction, and, finally, improved after the combined treatment of IVMP and IV immunoglobulin.
Recognizing paroxysmal jaw clonus (possibly with blepharospasm and sialorrhea) and considering the relationship between these episodes and D2R antibodies will be helpful in the early diagnosis and treatment of immune neurologic syndromes.