Prolonged Follow-up and CSF Antibody Titers in a Patient With Anti-NMDA Receptor Encephalitis

Abstract

Anti-NMDA receptor (NMDAR) encephalitis is a form of autoimmune encephalitis characterized by flulike prodromal symptoms, psychosis, memory problems, seizures, abnormal movements, and autonomic disturbances. We report a man with prolonged follow-up of symptoms and antibody titers during a 1-year hospital admission. An 18-year-old man was admitted to an outside hospital for hallucinations, jerky limb movements, and encephalopathy. Twelve days after admission, NMDAR antibodies were detected in the CSF. He was started on IV immunoglobulin (IVIg), methylprednisolone, propofol, phenytoin, and levetiracetam, and transferred to our center. On arrival, he was febrile, tachycardic, and ventilator-dependent. His pupils were reactive, but after sedation was weaned, he could not fix and follow, and he did not blink to threat. He had rhythmic orofacial movements characterized by chewing, blinking, and tongue thrusting. Muscle tone and reflexes were increased throughout, and he did not respond to painful stimulation. The patient was stuporous for nearly 12 months. He had complex movements of the face and slow rhythmic motions with the arms, hands, and fingers, as if conducting an orchestra. For 4 months, he had periods of sinus tachycardia, tachypnea, and hypertension that lasted for 10–20 minutes and partially responded to IV opiates, β-blockers, benzodiazepines, or hydration. EEG revealed a background of generalized slow and disorganized activity, but also intermittent low-voltage …