Dados do Trabalho

Título

Anti-NMDAR encephalitis: epileptic seizures versus movement disorders

Apresentação do caso

This is a 16-year-old male patient; in April 2022, he started with fever for 2 days, with no apparent focus; after 1 week evolved with falls and difficulty in gait, followed by generalized tonic-clonic seizures, delusions, psychomotor agitation and visual hallucinations. Admitted for investigation, there was mild pleocytosis in the CSF and signal alteration in the cerebral and cerebellar white matter. He underwent corticosteroid therapy for 5 days, being later transferred to a specialized service, where a screening for occult neoplasia and a panel of autoantibodies were performed. New brain MRI showed hypersignal in the right mesial temporal region, left optic tract, right parietal cortico-juxtacortical region and midbrain. Anti-NMDAR antibodies were detected in plasma and cerebrospinal fluid, leading to a diagnosis of autoimmune encephalitis (APE score: 12 points). He underwent to 5 plasmapheresis sessions and 5 infusions of human immunoglobulin (0.4 g/kg/day). During hospitalization, he evolved with stereotyped motor phenomena, present during wakefulness and mitigated during sleep, including: rhythmic palpebral twitching, facial dyskinesias, oromastigatory movements and dystonic postures involving the trunk and limbs. Video-EEGs (6 conventional and 1 prolonged) demonstrated the presence of marked diffuse encephalopathy and, eventually, of beta-delta complexes of diffuse projection (i.e., “extreme delta brushes”), these being more evident in wakefulness, however, not an unequivocal correlation was observed between the aforementioned clinical phenomena and ictal electroencephalographic alterations. At the moment, the patient is still using anti-seizure drugs and is waiting for the start of rituximab.

Discussão

Epileptic seizures and movement disorders are common findings in anti-NMDAR encephalitis, and the semiological distinction is often challenging; our patient presented an electrographic pattern typical of this condition, but without evidence of associated epileptiform activity.

Comentários finais

Caution is advocated in the management of motor alterations associated with autoimmune encephalitis, and the use of video-EEG is essential to clarify their nature.

Área

NEUROFISIOLOGIA CLÍNICA