Subacute Onset of Psychosis with Epileptic Seizures and Dysautonomia: Think Autoimmunity!

Abstract

RG is a 23-year-old, previously healthy woman who recently completed her training to become a tracer. Eight weeks prior to her admission to our institution she experienced a series of generalized epileptic fits over a period of five days. Neurological examination and an extensive diagnostic workup, including cranial MR imaging (MRI) and lumbar puncture (LP), were unremarkable. Due to ongoing agitation and delusions, she had to be transferred to a secure psychiatric ward where she continued to experience epileptic fits and also episodes of fainting. After an interdisciplinary neurological-psychiatric discussion blood tests for anti-NMDA-NR1 antibodies were ordered, and returned positive twice for serum IgG (titer 1:100 and 1:320, respectively) seven weeks after onset and six weeks after psychiatric admission. A follow-up MRI revealed subtle T2 hyperintensities compatible with limbic encephalitis. On a repeat LP anti-NMDA-NR1 antibodies were also detected in the otherwise completely normal cerebrospinal fluid (CSF; 1:10; normal < 1:2). The electroencephalogram (EEG) showed generalized slowing without epileptic discharge patterns (figure). An ovarian teratoma was ruled out by repeated gynaecological examinations and by pelvic MRI. The patient was treated with five days of i.v. high-dose methylprednisolone (IVMP) followed by five days of i.v. immunoglobulins (IVIg, total of 100 g). Her condition improved steadily, and she was referred to a neuro-rehabilitation unit four weeks later. Also, an autonomic dysfunction was diagnosed by means of a pathological sympathetic skin reaction (SSR), which reverted to normal prior to discharge, concomitant with the cessation of faints. On follow-up seven months later she was near normal with only subtle mnestic abnormalities as assessed by neuropsychological testing, but there were no psychosis, further fits or faints. Importantly, she was off any anti-psychotic or anti-convulsive medication.