ANTI-NMDA RECEPTOR ANTIBODY ENCEPHALITIS: A CASE STUDY

We present the case of a 25 year old Hispanic female, who presented to the ED with status epilepticus. She has had frequent hospital admissions since her seizures started six months ago, described as tonic clonic jerking. Her episodes have been associated with receptive and expressive aphasia, changes in personality, aggression, and flat affect. Work up revealed a normal MRI, intermittent slowing on EEG (Fig. 1), and lymphocytic pleocytosis with 4+ oligoclonal bands. NMDA receptor antibody encephalitis was highly suspected. Intravenous Solumedrol was given, with minimal improvement. Serology sent came back positive for NMDA receptor antibodies. Anti-N-methyl-D-asparate receptor (NMDAR) antibody encephalitis is a paraneoplastic syndrome affecting younger women, characterized by psychiatric symptoms, autonomic instability, neurologic abnormalities, and tonic-clonic type of seizures. CSF usually reveals lymphocytic pleocytosis, and MRI findings are non-specific. Diagnosis is confirmed with serology. 1 A large percentage of patients diagnosed also present with a detectable tumor, the most common of which is ovarian teratoma. 2 Anti-NMDA receptor antibody encephalitis is very responsive to treatment. The first line of management includes steroids, immunoglobulins, and plasma exchange. Rituximab and cyclophosphamide have shown to improve outcome in refractory cases. 3 Purpose (a): To describe a patient with anti-NMDA Receptor Antibody Encephalitis, discuss the clinical features based on this case study, and explore the management options for this condition. Methods (b): This is a case study on a 25 year old Hispanic female with no history of childhood seizures, who presented to the ED with status epilepticus. She has had frequent hospital admissions since her seizures started six months ago, described as tonic clonic jerking. Her episodes have been associated with intermittent receptive and expressive aphasia, changes in personality, aggression, and flat affect. During these admissions, all of her work-up, including MRI, CT scan, and CSF analysis have been negative for causing seizures. Her EEG has showed seizure focus and slowing of waves during these episodes. She has been followed by an outpatient neurologist, who has placed her on several anti-seizure medications, including Depakote, Tegretol, and Zonegran. On her latest admission, she presented to the ED with partial complex status epilepticus, exhibited generalized tonic-clonic movements, with associated tongue biting and urinary incontinence. Two days prior, she had bouts of nausea and vomiting. She was given Ativan and Cerebryx, which eventually resolved seizure activity. Further work up later revealed a normal MRI, intermittent slowing on repeat EEG, and lymphocytic pleocytosis with 4+ oligoclonal bands. Results (c): NMDA receptor antibody encephalitis was highly suspected. She was given Intravenous Solumedrol, minimal improvement was noted. Abdominal and pelvic CT were negative any neoplastic disease, including ovarian teratoma. Serology sent later came back positive for NMDA receptor antibodies. Conclusions (d): Anti-NMDA receptor antibody diagnosis should be high suspected in a young person presenting with seizures, psychiatric symptoms, speech disturbance, orofacial dyskinesias, and autonomic instability. Work-up should include serum and CSF titers for antibodies to NMDA receptors (NR1/NR2), as well as an extensive screen for any neoplasitic diseases, most especially ovarian teratomas in females.