Anti-NMDA-receptor encephalitis in a young woman: a diagnostic challenge

Abstract

A 42-year-old woman is admitted in the Psychiatric Department with signs of acute psychosis. She had orolinguofacial dyskinesias, episodes of bilateral rigidity, oculocephalogyric crisis and becomes stuporous. The cerebral MRI highlighted two supratentorial demyelination lesions. The CSF examination shows clear CSF with moderate pleocytosis and the predominance lymphocyte. Viral and bacterial cultures were negative. The patient is transferred to our Department of Neurology after 5 days and runs a fever. Also, orolinguofacial dyskinesias, hypersalivation, puppet’s eye syndrome, swallowing disorders, bilateral jerks osteotendinous reflexes, and coma state (GCS=5) are present. A second cerebral MRI was performed, which suggested two hypersignal lesions, T2, FLAIR and T1 hyposignal present, with no diffusion restriction, one being 0.9 cm, situated subcortically and to the right, the other of 0.5 cm being located on the left subcortical parietal, without gadolinium enhancement. The native pelvic CT scan marked out a 4.9 cm left annex cyst present. Anti-NMDAR antibodies were present in the CSF. As the patient was comatose, an interventional genital procedure is contraindicated. The patient was given iv Immunoglobulis, Methylprednisolone, Cyclophosphamide, anticonvulsives, and large spectrum antibiotics. She manifested multiple oculocephalogyric crisis, in both directions, bronchopneumonia, respiratory failure, requiring mechanical ventilatory support. The patient dies three weeks after the onset of the symptoms. The pathological examination revealed a left mature ovarian teratoma. The anti-NMDAR encephalitis is a life-threatening condition consisting of memory and psychiatric disorder, alteration of consciousness and hypoventilation, common associated with ovarian teratomas. The fast diagnosis and an early specific treatment can strongly influence the anti-NMDAR encephalitis’s natural evolution.

Keywords

• anti-n-methyl-d-aspartate-receptor (nmdar) encephalitis
• autoimmune encephalitis
• paraneoplastic syndrome
• ovarian teratoma