Rituximab Used Successfully in the Treatment of Anti-NMDA Receptor Encephalitis

Ikeguchi, Ryotaro; Shibuya, Koichi; Akiyama, Shigeo; Hino, Shuji; Kubo, Hiromasa; Takeda, Takahiro; Shibata, Noriyuki; Yamamoto, Kenji

doi:10.2169/internalmedicine.51.6874

Cited by 25 publications

(15 citation statements)

References 26 publications

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“…Although several case reports have indicated that both rituximab and cyclophosphamide are quite effective alone (8-10), we administered these drugs in combination in the present case based on the recommendation of Dalmau et al (2), which resulted in a rapid clinical improvement in our patient. It is difficult to determine which regimen was more effective in this case, as previous reports have shown that both agents have an immediate effect on clinical symptoms (8,9). This immediate effect may result from the more selective suppression of the antibodysecreting function of B cells than that achieved with firstline immunotherapy.…”

Section: Discussionmentioning

confidence: 87%

Refractory Status Epilepticus Caused by Anti-NMDA Receptor Encephalitis that Markedly Improved Following Combination Therapy with Rituximab and Cyclophosphamide

et al. 2015

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We herein describe the case of a 48-year-old woman who presented with nonconvulsive status epilepticus refractory to antiepileptic drugs caused by anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis without any tumors. She developed nausea and psychiatric symptoms, followed by fever and an acute progressive disturbance of consciousness. On admission to our hospital, she presented with involuntary orofacial movements and central hypoventilation, and an electroencephalogram showed a generalized slow activity consistent with nonconvulsive status epilepticus. The patient's drug-resistant status epilepticus markedly improved following second-line immunotherapy with rituximab and cyclophosphamide. Physicians should consider the early initiation of second-line therapy in certain cases of anti-NMDAR encephalitis.

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Section: Discussionmentioning

confidence: 87%

Refractory Status Epilepticus Caused by Anti-NMDA Receptor Encephalitis that Markedly Improved Following Combination Therapy with Rituximab and Cyclophosphamide

et al. 2015

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“…A later report documented the condition in patients free of teratoma, and found that such patients may fare worse [4]. The anti-CD20 antibody rituximab has been used to successfully treat this condition [5], and has become the standard of care in refractory cases [6]. To our knowledge autopsy findings from a patient who fails to respond to rituximab therapy have not previously been reported.…”

Section: Introductionmentioning

confidence: 99%

Extra–central nervous system target for assessment and treatment in refractory anti–N-methyl-d-aspartate receptor encephalitis

Nauen

2017

Journal of Critical Care

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Anti-N-methyl-D-aspartate-type glutamate receptor autoimmune encephalitis can arise in the setting of ovarian teratoma and often responds to resection. When arising in the absence of tumor failure to respond to treatment may be more likely, and affected patients often require intensive care. To further understanding of mechanisms and potential management we present findings from an autopsy conducted on a young woman who died from refractory autoimmune encephalitis of this type. Rituximab was administered 70 days before death, and both 37 and 14 days before death CD19+ lymphocytes were only 0.1% of blood cells. 10 sessions of plasmapheresis were performed following rituximab treatment. Nonetheless, the auto-antibodies were present in serum 4 days before death, demonstrating ongoing antibody production. The hippocampus and medial temporal lobe demonstrated inflammation with T-cell and prominent microglial involvement, but no plasma cells or plasmablasts were found there, or anywhere in the brain, despite an extensive search. Examination of lymph node tissue identified many plasma cells along sinusoids. These findings demonstrate that the antibody-producing cells are long-lived and likely reside in lymphoid tissue. Awareness of continuing antibody production, the extra-CNS site, the indication for cytotoxic therapy, and the potential for biopsy assessment may lead to more effective treatment.

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“…For instance a woman with NMDARE, but without an associated tumor was treated with rituximab successful after conventional immunotherapy consisting of corticosteroids, IVIg, and plasma exchange resulted in little improvement. In her case, significant improvement was noted approximately one week after rituximab administration demonstrating the potential efficacy of these second line treatments in refractory [15].…”

Section: Case Presentationmentioning

confidence: 83%

Progression from Acute Psychosis to Seizures and Autonomic Instability in an Adolescent with Ovarian Mass

Wen¹

2017

Clin Med Rev Case Rep

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Anti-N-Methyl-D-Aspartate Receptor Encephalitis (NMDARE) is a complex clinical entity that is both diagnostically and therapeutically challenging. A 17-year-old girl presented to the emergency room with worsening anxiety, confusion, and acute psychosis. While in an inpatient psychiatric unit, she developed progressive catatonia with decreased responsiveness. After transfer to the pediatric inpatient unit, she became hyperreflexic with seizure-like activity consisting of back arching, left arm extension, and right arm flexion refractory to lorazepam. Electroencephalogram (EEG) monitoring was started on the first hospital day and within twenty-four hours showed extreme delta brush pattern and abnormal background activity. Cerebrospinal fluid analysis demonstrated pleocytosis with a lymphocytic predominance. Imaging studies were notable for a left ovarian mass and non-specific sulcal hyperintensity on magnetic resonance imaging of the brain. The ovarian mass was subsequently resected, and tumor pathology was consistent with a grade II immature teratoma. Serum and CSF NMDAR IgG antibody titers wereboth elevated to 1:320, which combined with extreme delta brush pattern on EEG confirmed the diagnosis of paraneoplastic NMDARE. Further treatment for the patient's NMDARE after tumor removal included intravenous immune globulin, high dose pulse steroids, plasmapheresis, and rituximab. The patient's hospital course was complicated by episodes of autonomic instability. Prevention of dysautonomic crises, agitation, and rigidity were managed with benzodiazepines, benztropine, and diphenhydramine. Antipyretics were given for unexplained fevers while multiple antihypertensive agents were given for episodic hypertension. Over several weeks, the patient demonstrated gradual neurocognitive improvement to near-baseline levels and was discharged to a rehabilitation facility for further recovery. NMDARE is a major cause of disease with both psychiatric and neurologic features.Symptomatic treatment can be challenging requiring a balance of pharmacologic therapies. In the pediatric population, NMDARE is an important diagnosis to consider in applicable cases since it is a highly treatable disease with an overall good prognosis after adequate treatment.

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Rituximab Used Successfully in the Treatment of Anti-NMDA Receptor Encephalitis

Cited by 25 publications

References 26 publications

Refractory Status Epilepticus Caused by Anti-NMDA Receptor Encephalitis that Markedly Improved Following Combination Therapy with Rituximab and Cyclophosphamide

Refractory Status Epilepticus Caused by Anti-NMDA Receptor Encephalitis that Markedly Improved Following Combination Therapy with Rituximab and Cyclophosphamide

Extra–central nervous system target for assessment and treatment in refractory anti–N-methyl-d-aspartate receptor encephalitis

Progression from Acute Psychosis to Seizures and Autonomic Instability in an Adolescent with Ovarian Mass

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