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Título

Tetrabenazine use in treatment of movement disorders in anti-NMDAr encephalitis - a case report

RESUMO

Apresentação do Caso: 14-year-old female presented with acute behavioral changes, confusion, excessive worry and short-term memory impairment. Later on, she exhibited manual automatisms, fever and seizures. CSF exam showed 75 cells with lymphomonomorfic predominance and normal glucose. Intravenous acyclovir was prescribed. She was intubated after an episode of bronchoaspiration and still showed continuous ocular, facial and limb dyskinesias and dystonic posture, tachycardia and fever after sedation. She was diagnosed with encephalitis anti-NMDA based on typical clinical presentation and positive CSF antibodies. Brain MRI with mild hypersignal in FLAIR and contrast enhancement in encephalic cortical sulci. FDG-PET scan showed diffuse asymmetry of cerebral metabolic activity, with increased metabolism in the basal frontal cortex specially on the right hemisphere and marked deficit of metabolism in the bilateral occipital cortex. Electroencephalogram showed extreme delta brush pattern, without epileptic paroxysms. Tumor screening with pelvic MRI and full body pet scan was negative. Treatment with methylprednisolone 1g/daily for 5 days and intravenous immunoglobulin (IVIG) was initiated. Dyskinesias, dystonic posture and dysautonomia persisted. There was no symptomatic response to oral benzodiazepines, ketamine or clonidine relying on IV benzodiazepines. Initiation of tetrabenazine made significant improvement of movement disorders, which resurface upon drug half-time clearance. Discussão: Anti-NMDAr encephalitis is characterized by limbic encephalitis, fever, seizures and dysautonomia. Movement disorders are present in 75% of young children and 95% of adults, appearing as oral, facial or lingual dyskinesias, dystonia, chorea, athetosis, myorhythmia, opisthotonus, ballismus, blepharospasm and oculogyric crisis. Immunotherapy generally improves this movement disorders. Benzodiazepines, valproate, haloperidol, ketamine and propofol have been reported for symptomatic treatment for refractory cases. Tetrabenazine is a reversible inhibitor of the human vesicular monoamine transporter type 2 (VMAT-2) with scarce data about use in anti-NMDA encephalitis. The case reported presented time-related and dose-related improvement of dyskinesias and dystonia with tetrabenazine. Comentários Finais: Tetrabenazine is shown as a possible alternative symptomatic treatment to high doses of benzodiazepines with less adverse effects in movement disorders of anti-NMDAr encephalitis.

Palavras Chave

"Tetrabenazine" "Dysknesia" "Anti-N-Methyl-D-Aspartate Receptor Encephalitis"

Área

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