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Worth a Second Look: Successful Treatment of Persistent Anti-NMDA-r Encephalitis
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A6866 - Worth a Second Look: Successful Treatment of Persistent Anti-NMDA-r Encephalitis
Author Block: N. Wheeler, R. W. Weller, D. Golovyan, R. Patel; Indiana University School of Medicine, Indianapolis, IN, United States.
Introduction:Anti-N-methyl-D-aspartate receptor (NMDA-r) encephalitis is an autoimmune encephalitis. Patients typically present with a viral prodrome followed by neuropsychiatric symptoms, seizures, motor dysfunctions, depressed consciousness and autonomic dysfunction. The majority of patients are young females, and an ovarian teratoma is found in in over 50% of this population. Early treatment is critical for favorable neurological outcome. Early diagnosis requires a high index of suspicion as symptoms are nonspecific.
Case PresentationA 24-year-old woman with no prior medical problems presented to the emergency department with new onset psychosis. Metabolic and infectious evaluation and brain imaging was unrevealing, and a urine toxicology screen negative. While awaiting inpatient psychiatric placement she developed generalized tonic-clonic seizures. Despite antiepileptic treatment, she remained comatose and exhibited stereotyped movements and dystonia. EEG showed extreme delta brush raising suspicion anti-NMDAr encephalitis. Lumbar puncture was performed and abdominal and pelvic CT revealed a 1.4 cm teratoma in the right ovary. She underwent right laparoscopic salpingo-oophorectomy. She was started on high dose steroids and plasma exchange without response. Her cerebrospinal fluid anti-NMDAr antibody titer was 1:512. Additional immunosuppressive therapy with IVIG was administered, followed by treatment with rituximab. Despite treatment, she continued to have episodes of severe agitation with dyskinesia and dystonia requiring four-point restraints.
As her symptoms failed to improve, there was concern about a persistent source of autoantibodies. Repeat abdominal CT was obtained on hospital day 52 revealing a new teratoma in the left ovary, which was removed. CSF anti-NMDAr titers resulted at 1:640. The patient was treated again with plasma exchange and rituximab. She made a gradual neurologic recovery over the next two months.
DiscussionRecovery from anti-NMDAr encephalitis is variable, generally occurring between two weeks to two years after onset of symptoms. Patients treated with early tumor resection and medical therapy have a higher likelihood of neurological recovery. Our patient had prompt diagnosis, teratoma resection and aggressive immunotherapy, yet had a protracted clinical course with severe encephalitis symptoms due to a second teratoma. Teratomas are bilateral in up to 15% of cases and have been reported to be fast-growing. It is unknown what percentage of patients with prolonged recovery time have a second source of anti-NMDAr antibodies. If a patient with anti-NMDAr encephalitis fails to respond to appropriate treatment, repeat diagnostic studies should be done to look for other sources of paraneoplastic antibodies.
Author Block: N. Wheeler, R. W. Weller, D. Golovyan, R. Patel; Indiana University School of Medicine, Indianapolis, IN, United States.
Introduction:Anti-N-methyl-D-aspartate receptor (NMDA-r) encephalitis is an autoimmune encephalitis. Patients typically present with a viral prodrome followed by neuropsychiatric symptoms, seizures, motor dysfunctions, depressed consciousness and autonomic dysfunction. The majority of patients are young females, and an ovarian teratoma is found in in over 50% of this population. Early treatment is critical for favorable neurological outcome. Early diagnosis requires a high index of suspicion as symptoms are nonspecific.
Case PresentationA 24-year-old woman with no prior medical problems presented to the emergency department with new onset psychosis. Metabolic and infectious evaluation and brain imaging was unrevealing, and a urine toxicology screen negative. While awaiting inpatient psychiatric placement she developed generalized tonic-clonic seizures. Despite antiepileptic treatment, she remained comatose and exhibited stereotyped movements and dystonia. EEG showed extreme delta brush raising suspicion anti-NMDAr encephalitis. Lumbar puncture was performed and abdominal and pelvic CT revealed a 1.4 cm teratoma in the right ovary. She underwent right laparoscopic salpingo-oophorectomy. She was started on high dose steroids and plasma exchange without response. Her cerebrospinal fluid anti-NMDAr antibody titer was 1:512. Additional immunosuppressive therapy with IVIG was administered, followed by treatment with rituximab. Despite treatment, she continued to have episodes of severe agitation with dyskinesia and dystonia requiring four-point restraints.
As her symptoms failed to improve, there was concern about a persistent source of autoantibodies. Repeat abdominal CT was obtained on hospital day 52 revealing a new teratoma in the left ovary, which was removed. CSF anti-NMDAr titers resulted at 1:640. The patient was treated again with plasma exchange and rituximab. She made a gradual neurologic recovery over the next two months.
DiscussionRecovery from anti-NMDAr encephalitis is variable, generally occurring between two weeks to two years after onset of symptoms. Patients treated with early tumor resection and medical therapy have a higher likelihood of neurological recovery. Our patient had prompt diagnosis, teratoma resection and aggressive immunotherapy, yet had a protracted clinical course with severe encephalitis symptoms due to a second teratoma. Teratomas are bilateral in up to 15% of cases and have been reported to be fast-growing. It is unknown what percentage of patients with prolonged recovery time have a second source of anti-NMDAr antibodies. If a patient with anti-NMDAr encephalitis fails to respond to appropriate treatment, repeat diagnostic studies should be done to look for other sources of paraneoplastic antibodies.
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