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Clinical Manifestations of Anti-NMDAR Encephalitis: Western Canadian Case Series and Practical Recommendations for Treatment

Saeed Shahbeigi [Brain Mapping Research Centre, Shahid Beheshti University of Medical Sciences, Neuroscience Research Center, Iran University of Medical Sciences], Amir Ali Sepehry [Faculty of Medicine, Division of Neurology, University of British Columbia, Vancouver, Canada] , Joel Oger [Emeritus Professor of University of British Columbia]

Anti-NMDAR encephalitis is a life threatening yet treatable neuroimmune disease secondary to the presence of autoantibodies. Despite a growing body of literature, this disorder remains under-recognized, due to variable presentations, and limited clinicians familiarity with the condition. Methods: We describe three cases that were diagnosed at Vancouver General Hospital, Canada. Results: Case 1, a 23-year old Caucasian woman presented with low-grade fever, personality changes, and status epilepticus. She had a normal brain MRI and CSF markers. Anti-NMDAR (NR1) was positive in serum. She had bilateral ovarian teratomas. After oophorectomy and immunosuppressive treatments, the NMDAR antibodies became negative and her seizures completely disappeared. Case 2, following delivery, a 27-year Chinese woman rapidly developed sudden significant behavioural changes, and seizure. She had a teratoma and positive NMDAR (NR1) in the CSF and serum. Despite bilateral oophorectomy and receiving high doses of immunosuppression, she has developed severe sequelae. Case 3, a 32-year Philippino-Canadian female developed short-term memory problems, olfactory hallucinations, and orofacial dyskinesia for 2 years. After receiving steroids, PLEX and IVIG, she clinically improved markedly without any sequel. It is important to mention, on the paper we talk about the first and second line of therapy and interestingly a new treatment option called Bortizomib for refractory NMDAR patients to 1st and 2nd line of therapy as well. Conclusion: Based on our experience when the diagnosis of the anti-NMDAR encephalitis is made in the presence of normal brain MRI and CSF the prognosis is better

Keywords : NMDAR encephalitis; Autoimmune encephalitis; Paraneoplastic antibodies; Limbic encephalitis; Review; Case series


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