A Young Man with Anti-NMDAR Encephalitis following Guillain-Barré Syndrome.

A Young Man with Anti-NMDAR Encephalitis following Guillain-Barré Syndrome.
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DOI:
10.1159/000323751
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发表时间:
2011-01-11
影响因子:
0.7
通讯作者:
Ikeda S
Ikeda S
中科院分区:
其他
文献类型:
--
作者:
Tojo K;Nitta K;Ishii W;Sekijima Y;Morita H;Takahashi Y;Tanaka K;Ikeda S

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一名 19 岁男子在流感样发作后出现快速进行性肌肉无力、四肢感觉迟钝和呼吸困难。神经传导研究显示,传导阻滞导致运动神经传导速度降低,无法诱发感觉神经动作电位。患者被诊断为吉兰-巴利综合征(GBS),接受2个周期的静脉注射免疫球蛋白(IVIg)治疗并辅以机械通气辅助。在病情恢复过程中,从入院第37天开始,他出现了几次精神运动性激越发作,入院第42天突然出现全身强直性惊厥。脑部 MRI 显示双侧丘脑和内侧颞叶存在高强度病变。通过持续输注硫喷妥钠(直到住院第 50 天)和机械通气(直到住院第 84 天)控制抽搐。静脉注射甲基强的松龙脉冲疗法(1,000 毫克/天)持续 3 天,然后添加地塞米松(16 毫克/天)。惊厥发作缓解后,出现明显的口舌运动障碍,MRI 可见双侧内侧颞叶明显萎缩。入院第92天,血清和脑脊液中的抗N-甲基-D-天冬氨酸受体(NMDAR)抗体呈阳性。抗 NMDAR 脑炎通常影响年轻女性,但也有少数男性病例报道。我们的男性患者的独特之处在于,其既往疾病是 GBS(一种感染后自身免疫性疾病),这表明抗 NMDAR 脑炎本身是由副感染性自身免疫机制引起的。
A 19-year-old man developed rapidly progressive muscle weakness and dysesthesia in the extremities, and dyspnea after a flu-like episode. Nerve conduction studies showed reduced motor nerve conduction velocities with conduction block, and sensory nerve action potentials could not be evoked. The patient was diagnosed as having Guillain-Barré syndrome (GBS), and was treated with 2 cycles of intravenous immunoglobulin (IVIg) therapy and was assisted by mechanical ventilation. During the recovery course of the illness, he experienced several attacks of psychomotor agitation from the 37th hospital day, and generalized tonic convulsive seizures suddenly developed on the 42nd hospital day. Brain MRI showed high-intensity lesions in the bilateral thalamus and medial temporal lobes. The convulsions were controlled by continuous thiopental infusion (until the 50th hospital day) and mechanical ventilation (until the 84th hospital day). Intravenous methylprednisolone pulse therapy (1,000 mg/day) for 3 days followed by dexamethasone (16 mg/day) was added. After relief of convulsive seizures, prominent orolingual dyskinesia appeared, and on MRI marked atrophy of the bilateral medial temporal lobes was seen. Anti-N-methyl-D-aspartate receptor (NMDAR) antibodies in serum and cerebrospinal fluid were positive on the 92nd hospital day. Anti-NMDAR encephalitis usually affects young females but a small number of male cases with this disease have been reported. Our male patient was unique in having GBS, a post-infectious autoimmune disease, as a preceding disease, suggesting that anti-NMDAR encephalitis itself is caused by a parainfectious autoimmune mechanism.
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