[Neuroleptic Intolerance and Residual Mutism in a Young Woman with Anti-N-Methyl-D-Aspartate Receptor (NMDAR) Encephalitis].

Q3 Medicine Brain and Nerve Pub Date : 2024-03-01 DOI:10.11477/mf.1416202598
Kazue Tajima, Toshio Fukutake
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Abstract

We report a case of anti-NMDAR encephalitis and residual mutism in a 23-year-old woman who presented with neuroleptic intolerance. Admission to our department for investigation of her abnormal behavior revealed cerebrospinal fluid (CSF) positivity for anti-NMDAR antibodies, and the patient underwent immunotherapy. However, generalized tonic seizures developed, requiring mechanical ventilation in the intensive care unit. Antipsychotic drugs were also administered for involuntary movements and insomnia. Thereafter, a malignant syndrome of severe hyperCKemia (Max: 191,120 IU/L) and shock developed, requiring resuscitation and three sessions of hemodialysis. Subsequent rituximab therapy led to improvement, except for mutism, which had newly developed during resuscitation. Seven months after initial admission, the patient was discharged with independent gait. However, her mutism still persists. Temporary mutism has been reported to occur in this type of encephalitis, albeit rarely. The fact that remission was not observed in this case may have been due to cerebellar infarction occurring during resuscitation, but the true cause remains unclear. Malignant syndrome or rhabdomyolysis, as seen in this patient, has also sometimes been reported in this form of encephalitis when antipsychotic agents, especially dopamine receptor blockers, have been administered. Therefore, such agents should be administered with caution in patients with anti-NMDAR encephalitis. (Received August 17, 2023; Accepted October 24, 2023; Published March 1, 2024).

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[一名患有抗 N-甲基-D-天冬氨酸受体(NMDAR)脑炎的年轻女性的神经抑制剂不耐受和残余缄默症]。
我们报告了一例抗 NMDAR 脑炎和残余缄默症病例,患者 23 岁,曾出现神经安定剂不耐受症状。入院后,我科对她的异常行为进行了调查,发现脑脊液(CSF)中抗 NMDAR 抗体呈阳性,患者接受了免疫治疗。然而,患者出现了全身强直性癫痫发作,需要在重症监护室进行机械通气。患者还因不自主运动和失眠服用了抗精神病药物。此后,出现了严重的高CK血症(最大值:191 120 IU/L)和休克的恶性综合征,需要进行复苏和三次血液透析。随后的利妥昔单抗治疗使病情有所好转,但在复苏期间新出现的缄默症除外。入院 7 个月后,患者出院时已能独立行走。然而,她的缄默症仍然存在。据报道,这种类型的脑炎也会出现暂时性缄默症,尽管这种情况很少见。本病例未见缓解的原因可能是在抢救过程中发生了小脑梗塞,但真正的原因仍不清楚。在使用抗精神病药物,尤其是多巴胺受体阻滞剂时,有时也会出现恶性综合征或横纹肌溶解症,如该患者所见。因此,抗 NMDAR 脑炎患者应慎用此类药物。(2023年8月17日收到;2023年10月24日接受;2024年3月1日发表)。
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Brain and Nerve
Brain and Nerve Medicine-Neurology (clinical)
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