Abstracts

Rationale: Anti-NMDAR encephalitis is the most common type of paraneoplastic encephalitis. It occurs primarily in young women and is mostly associated with ovarian teratomas. The clinical picture typically appears by steps, starting with psychiatric symptoms, language disorders and seizures. Consciousness disturbances, dyskinesias, autonomic instability and coma then follow.Methods: We present a 20 years old patient with no relevant personal history, admitted at age 15 after partial seizures with secondary generalization and behavioral changes. CT scan was normal. Initial EEG showed slowing in the left hemisphere. CSF revealed pleocytosis (94 cells/l)- mononuclear cells predominance. It was followed by mutism and status epilepticus was diagnosed. He was transferred to ICU, where he remained 105 days given the EEG patterns, strange behavior and clinical resistance to multiple anti-epileptic drugs. All research was negative including anti-neuronal antibodies available at the time (anti-Ru, anti-Ri, anti-Yo ). Autoimmune encephalitis was considered and treatment with pulses of methylprednisolone and immunoglobulin proceeded. There was progressive clinical stabilization and recovery. He was aphasic at discharge and never regained previous school performance.Results: At 18, he was hospitalized again for episodes of loss of contact and behavioral changes (laughing, crying, agitation and aggression) that responded to midazolam with post-ictal aphasia. He was transferred to the ICU where he remained 15 days and was again treated with corticosteroids with overall improvement. EEG showed focal slowing in left frontal regions. Oligoclonal bands and positive anti-NMDAR antibodies were present in CSF confirming the initial clinical suspicion. In 2013 the patient had another relapse and it was decided to start immunosuppression with clinical improvement. Some weeks later there was a new episode with ICU admission and dependence on ventilation. The patient died of sepsis. Conclusions: The diagnosis of anti-NMDAR encephalitis depends on high clinical suspicion and EEG extreme delta brushes as early features, help to consider the diagnosis. Therapy should be started as early as possible, even empirically, to improve prognosis. However some patients continue to have relapses with important morbidity/mortality. Thus it is crucial to recognize which patients might benefit from earlier aggressive imunossupression.