A - 76The Role of Age in Early Functional Outcomes for Children Diagnosed with Anti-NMDA Receptor Encephalitis During Inpatient Rehabilitation

AbstractWe describe two cases of confirmed anti-NMDA receptor encephalitis; one patient initially presented with a clinical picture that resembled delirium and later appeared to present with a conversion reaction and the second patient presented with a first psychotic break followed by the clinical picture of neuroleptic malignant syndrome with catatonia. Neither patient had a previous history of psychiatric illness or recreational drug use. These cases illustrate the diagnostic and treatment challenges associated with this neuropsychiatric condition and underscore the role of psychosomatic medicine psychiatrists in diagnosing anti-NMDA receptor encephalitis.

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Long-term Functional Outcomes and Relapse of Anti-NMDA Receptor Encephalitis

Neurology Neuroimmunology & Neuroinflammation ◽

10.1212/nxi.0000000000000958 ◽

2021 ◽

Vol 8 (2) ◽

pp. e958

Author(s):

Xue Gong ◽

Chu Chen ◽

Xu Liu ◽

Jingfang Lin ◽

Aiqing Li ◽

...

Keyword(s):

Nmda Receptor ◽

Functional Outcomes ◽

Western China ◽

Residual Symptoms ◽

Delayed Treatment ◽

Nmda Receptor Encephalitis ◽

Disturbance Of Consciousness ◽

Related Risk ◽

Multiple Relapses

ObjectiveTo study the factors associated with relapse and functional outcomes in patients with anti-NMDA receptor encephalitis in Western China.MethodsThe Outcome of the anti-NMDA receptor Encephalitis Study in Western China was initiated in October 2011 to collect prospective observational data from consecutively enrolled patients with anti-NMDA receptor encephalitis.ResultsWe consecutively enrolled 244 patients (median age: 26 years, range: 9–78 years; females: 128 [52.45%]) between October 2011 and September 2019. Fatality occurred in 17 (6.96%) patients, and tumors were found in 38 (15.57%) patients. The median follow-up duration was 40 (6–96) months. Of these patients, 84.8% showed clinical improvements within 4 weeks after immunotherapy, with a median modified Rankin Scale of 2 (interquartile range [IQR]: 2–3), and 80.7% (median: 1, IQR: 0–2) and 85.7% (median: 0, IQR: 0–1) had substantial recovery (i.e., mild or no residual symptoms) at 12 and 24 months, respectively. The overall prognosis was still improving at 42 months after onset. Disturbance of consciousness during the first month was the only independent predictor (OR: 2.91, 95% CI: 1.27–6.65; p = 0.01) of a poor functional neurologic outcome. Overall, 15.9% of the patients had one or multiple relapses, with 82.0% experiencing the first relapse within 24 months and 76.9% experiencing relapses that were less severe than the initial episodes. Relapse-related risk factors included the female sex and delayed treatment (p < 0.05).ConclusionsMost patients achieved favorable long-term functional outcomes. Some patients experienced one or multiple relapses, especially female patients. Timely immunotherapy at onset may reduce the risk of relapse.

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POTENTIAL ROLE OF NMDA-RECEPTOR ANTIBODIES IN SCHIZOPHRENIA: OVERLAP AND DISTINCTION FROM NMDA-RECEPTOR ENCEPHALITIS

Schizophrenia Research ◽

10.1016/s0920-9964(14)70114-8 ◽

2014 ◽

Vol 153 ◽

pp. S34

Author(s):

Johann Steiner ◽

Hannelore Ehrenreich ◽

Souhel Najjar

Keyword(s):

Nmda Receptor ◽

Potential Role ◽

Nmda Receptor Encephalitis ◽

Receptor Antibodies

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Bortezomib for treatment of anti–NMDA receptor encephalitis in a pediatric patient refractory to conventional therapy

American Journal of Health-System Pharmacy ◽

10.1093/ajhp/zxaa415 ◽

2021 ◽

Vol 78 (5) ◽

pp. 395-400 ◽

Cited By ~ 1

Author(s):

Marroyln L Simmons ◽

Kimberly A Perez

Keyword(s):

Nmda Receptor ◽

Clinical Improvement ◽

Pediatric Patient ◽

Mental Status ◽

Clinical Status ◽

Inpatient Rehabilitation ◽

High Dose ◽

Nmda Receptor Encephalitis ◽

High Dose Methylprednisolone ◽

Receptor Antibodies

Abstract Purpose The case of a pediatric patient with treatment refractory anti–N-methyl-d-aspartate (NMDA) receptor encephalitis treated with the plasma cell–depleting therapy bortezomib is reported. Summary A 5-year-old female presented to the hospital with a 1-week history of altered mental status, agitation, and possible seizure-like activity. She was admitted to the hospital for suspected meningitis or meningoencephalitis and an extensive workup was completed, including sending blood and cerebrospinal fluid (CSF) for testing for NMDA receptor antibodies. While test results were pending, the patient was treated initially with intravenous immunoglobulin (IVIG) for 4 days followed by high-dose methylprednisolone for 5 days. The patient’s serum and CSF studies were positive for NMDA receptor antibodies, confirming the diagnosis of anti–NMDA receptor encephalitis. She was then treated with plasmapheresis therapy every other day for 5 treatments, without any clinical improvement. The patient then received rituximab once weekly for 6 weeks. Three weeks after completion of rituximab therapy, the patient was started on her first cycle of bortezomib therapy. She received a total of 6 cycles, with improvement in her clinical status beginning with the third cycle. Upon completion of 6 cycles, the patient’s mental status and level of functioning had greatly improved. She was discharged to an inpatient rehabilitation facility and ultimately able to return home to her family. Conclusion A 5-year-old female with anti–NMDA receptor encephalitis was successfully treated with bortezomib after having shown no clinical improvement during treatment with IVIG, high-dose methylprednisolone, plasmapheresis, and rituximab.

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