Akiyama Hisanao, Sasaki Rie, Hasegawa Yasuhiro
Department of Neurology, St. Marianna University School of Medicine, Kawasaki, Kanagawa, Japan.
Medicine (Baltimore). 2019 Jan;98(2):e14033. doi: 10.1097/MD.0000000000014033.
We report this 1st case because perampanel may be effective against the seizures and abnormal behavior that occur in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis.
The patient was a healthy 26-year-old woman who suddenly developed seizures and exhibited abnormal behavior.
NMDAR encephalitis was diagnosed based on positive NMDAR antibody on cerebrospinal fluid analysis.
Treatment with anticonvulsants and sedatives was started immediately, along with steroid pulse therapy and plasmapheresis, but these measures did not adequately control the repeated seizures and abnormal behavior. However, with the addition of oral perampanel, an α-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid (AMPA) receptor antagonist, the seizures and abnormal behavior promptly disappeared.
The patient was transferred to the rehabilitation hospital and returned to her job.
It appears that perampanel rapidly eliminated these clinical features by inhibiting inflow of abnormal glutamic acid and attenuating nerve hyperexcitability by acting as a selective and noncompetitive antagonist of AMPA receptors that had increased in the postsynaptic membrane due to anti-NMDAR encephalitis. To the best of our knowledge, there are no other reports showing that perampanel was effective against anti-NMDAR encephalitis. This case suggests that perampanel may be effective against the seizures and abnormal behavior that occur in anti-NMDAR encephalitis.
我们报告这首例病例,因为吡仑帕奈可能对抗N-甲基-D-天冬氨酸受体(NMDAR)脑炎中出现的癫痫发作和异常行为有效。
患者为一名26岁健康女性,突然出现癫痫发作并表现出异常行为。
根据脑脊液分析中NMDAR抗体呈阳性,诊断为NMDAR脑炎。
立即开始使用抗惊厥药和镇静剂进行治疗,同时进行类固醇脉冲疗法和血浆置换,但这些措施未能充分控制反复出现的癫痫发作和异常行为。然而,加用口服吡仑帕奈(一种α-氨基-3-羟基-5-甲基-4-异恶唑丙酸(AMPA)受体拮抗剂)后,癫痫发作和异常行为迅速消失。
患者被转至康复医院并重返工作岗位。
吡仑帕奈似乎通过抑制异常谷氨酸的流入,并作为突触后膜因抗NMDAR脑炎而增加的AMPA受体的选择性非竞争性拮抗剂,减弱神经兴奋性过高,从而迅速消除了这些临床特征。据我们所知,尚无其他报告表明吡仑帕奈对抗NMDAR脑炎有效。该病例提示吡仑帕奈可能对抗NMDAR脑炎中出现的癫痫发作和异常行为有效。