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Abstract Details

Driving Out of Coma: Treatment of Anti-NMDA Receptor Encephalitis with Bortezomib
Autoimmune Neurology
P2 - Poster Session 2 (8:00 AM-9:00 AM)
15-011
We report a severe case of anti-NMDA receptor encephalitis who failed standard treatments with immunosuppressive and B-cell depleting agents; however, responded well to Bortezomib, a plasma-cell depleting proteasome inhibitor.
Anti-NMDA receptor encephalitis is one of the autoimmune encephalitis syndromes, which usually presents with prodromal viral-like symptoms, and subsequent psychiatric symptoms, seizures, dyskinesias, cognitive dysfunction, and autonomic instability that may be life threatening. Immunosuppressive therapies are effective in most cases; however, they should be individualized based on the clinical response and anti-NMDA antibody titers.
N/A

A 43-year-old gentleman developed behavioral changes, psychiatric symptoms and seizures requiring intubation and mechanical ventilation in April 2016. Neurological exam was notable for a comatose state and orofacial dyskinesia. CSF showed WBC: 143 with 99% lymphocytes as well as protein: 72, anti-NMDA antibody titer: 1:320. Brain and spine MRI were unremarkable, malignancy work up was negative.

For two years, he underwent 5-day course of Dexamethasone, four sessions of plasma exchange, 5-day course of IVIG, four cycles of Cyclophosphamide and five infusions of Rituximab with no clinical improvement. In late 2017, repeat anti-NMDA CSF and serum titers were 1:80 and 1:640 respectively. He was treated with 5 cycles of Bortezomib after which his mental status dramatically improved, he was weaned off mechanical ventilation and gastrostomy tube. Maintenance treatment with monthly Rituximab was continued. Patient was able to independently drive to the UCI neuro-oncology clinic for his most recent visit.

Despite aggressive immunotherapies, severe cases of anti-NMDA receptor encephalitis may leave patients with life threatening autonomic dysfunction and seizures requiring prolonged ICU stays. Bortezomib, a plasma-cell depleting proteasome inhibitor, has shown promising results in cases of severe anti-NMDA receptor encephalitis and should be considered for select patients. 
Authors/Disclosures
Maryam H. Hosseini, MD
PRESENTER 		Dr. Hosseini has nothing to disclose.
Shadi Milani, DO Dr. Milani has nothing to disclose.
Xiao-Tang Kong, MD, PhD (UC Irvine) Dr. Kong has nothing to disclose.