A severe anti-NMDA-receptor encephalitis case with extensive cortical and white matter changes, cerebral atrophy and communicating hydrocephalus

Abstract

A 21-year-old woman presented with a viral prodrome, abnormal behaviours, confusion and short-term memory loss, followed by status epilepticus that later evolved to orofacial dyskinesias, autonomic dysfunctions and hypoventilation requiring prolonged ventilator support and ICU admission. Cerebrospinal fluid (CSF) and serum analysis confirmed the presence of anti-NMDAR autoantibodies. A left salpingoopherectomy was performed on day 35 of admission revealing an immature ovarian teratoma. Following surgical and two courses of intravenous immunoglobulin therapy, her response remained poor. Initial brain magnetic resonance imaging (MRI) during the acute stage showed enlarged left hippocampus. Further MRI follow-up 13 weeks after admission showed unusual findings of extensive cortical and white matter changes, generalised cerebral atrophy, dilated ventricles and possible transependymal CSF seepage of communicating hydrocephalus. A ventriculo-peritoneal shunt was performed subsequently and she was discharged 6 months after admission without significant change in her clinical status. Follow-up 4 months later showed some improvement but patient remained severely disabled.