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

Abstract

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.

Seizure outcomes in patients with anti‐NMDARencephalitis: A follow‐up study

“…Of all seizure types, generalized seizure was the most common seizure type, which was consistent with the reported seizure types of the disease . Furthermore, data about the incidence of RSE and SRSE are lacking; however, some case studies reported the occurrence of RSE and SRSE in the acute phase of anti‐NMDAR encephalitis . In our cohort, 14.8% RSE and 10.2% SRSE occurred in the acute phase of this disease.…”

“…Most patients develop generalized tonic–clonic seizures, whereas some exhibit a complex partial type . Status epilepticus (SE) was also reported . In addition, anti‐NMDAR encephalitis is one of the most common forms of autoimmune encephalitis that is associated with SE …”

“…1,9,10 Status epilepticus (SE) was also reported. [11][12][13] In addition, anti-NMDAR encephalitis is one of the most common forms of autoimmune encephalitis that is associated with SE. 14,15 To date, some studies have described the prevalence of seizures and seizure types in patients at the acute phase of anti-NMDAR encephalitis, 1,3,6 although, as far as we know, studies that focused on seizures outcome in anti-NMDAR encephalitis patients are rare.…”

Anti-N-Methyl-D-aspartate Receptor Encephalitis: A Severe, Potentially Reversible Autoimmune Encephalitis

“…If tumors are found, the surgical resection should be taken into account [1, 98]. When diagnosis is delayed, or patients do not have a tumor, or the first-line immunotherapy fails, additional treatment with second-line immunotherapy is usually applied [5, 11], which includes cyclophosphamide, rituximab, azathioprine, mycophenolate mofetil, methotrexate, and so on [99, 100]. Supporting therapies play an important role, for example antiepileptic and antipsychotic treatment, respiratory and cardiac support, management of blood pressure and temperature, and prevention of deep venous thrombosis (DVT) and bedsore [46, 101].…”

Auto-immune anti-N-methyl-D-aspartate receptor (anti-NMDAR) encephalitis: three case reports

“…Status epilepticus can also be a manifestation of anti-NMDAR encephalitis. 16 Symptomatic treatment of movement disorders in anti-NMDAR encephalitis is challenging; antipsychotic medications, sedatives and sleep medications have been used to treat the symptoms with variable responses. It is recommended that anti-psychotic therapy be used for a short period to avoid extrapyramidal side-effects.…”