A 32-year-old woman presented subacutely with headache, neck pain and vomiting. There was haemorrhagic papilloedema and bilateral sixth nerve palsies, but no localising signs. MRI of the brain was normal and there was no evidence of venous sinus thrombosis. Lumbar puncture revealed an opening pressure of 39 cm water with 277 cells (96% lymphocytes), a CSF protein of 1543 mg/l, but normal glucose ratio. Extensive investigation failed to identify an underlying infective or neoplastic cause. Despite full courses of antimicrobial therapy she deteriorated and developed visual hallucinations and behavioural disturbance. Brain biopsy demonstrated lymphocytic infiltration of the cerebral cortex, but no malignant cells. High-dose steroid therapy resulted in sustained symptomatic improvement within days. Further lumbar puncture confirmed a corresponding improvement in both cell count and CSF protein although the intracranial pressure remained elevated at 42 cm water. NMDAR antibodies were subsequently detected in her serum. There was no associated ovarian teratoma. During the course of her illness we did not observe any seizures, movement disorders, autonomic dysfunction or other typical features reported in the literature. This appears to be a novel presentation of a recently described autoimmune encephalitis, thus further widening the phenotypic spectrum of this disorder.
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