Abstract Details

NMDAR-antibody encephalitis is the most common and apparently, the best defined of the autoimmune encephalitides. In adults, the usual prodrome is characterized by psychiatric and cognitive symptoms, subsequently seizures, and in some cases, movement disorders, mostly orofacial dyskinesias. Seizure semiology in NMDAR-antibody encephalitis is mostly generalized tonic-clonic and a predominantly epileptic presentation is more common amongst children. Faciobrachial dystonic seizures (FBDS) are not known in the setting of NMDAR-antibody encephalitis. Conversely, FBDS is pathognomonic of Anti Leucine-rich Glioma Inactivated 1 (LGI-1) encephalitis, which mostly occurs amongst middle-aged and elderly males. 

To report a case of N-methyl D-aspartate receptor (NMDAR)-antibody encephalitis in a young, gravid Nigerian woman who presented with faciobrachial dystonic seizures

Case report and a review of literature

(CASE SUMMARY): A 23-year-old Nigerian lady in her second trimester of pregnancy, presented with somewhat bizarre, frequent focal seizures involving the left upper and lower limbs, with dystonic posturing of the left upper limb, usually accompanied by a tonic head tilt to the left and twitching of the left side of the face. She subsequently developed intermittent fever and psychiatric symptoms. Her Brain MRI findings were in keeping with limbic encephalitis and her CSF was positive for NMDA receptor antibodies and negative for anti LGI-1. The ictal EEG was unremarkable. She responded to corticosteroid therapy, following antiepileptic drug trials, which were of no benefit.

Prompt recognition of FBDS, which is usually accompanied by an absence of ictal EEG epileptiform discharges and no response to antiepileptic drug therapy, is crucial in narrowing down differential diagnoses and early commencement of effective treatment, usually immunotherapy.