Bilateral globus pallidus internus deep brain stimulation in medication-refractory dystonia secondary to infantile acute disseminated encephalomyelitis: illustrative case

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BACKGROUND: Acute disseminated encephalomyelitis (ADEM) is a rare immune-mediated condition associated with a range of neurological sequelae, including dystonia. Deep brain stimulation (DBS) has been established as an effective therapy for various forms of primary and secondary dystonia; however, its use in dystonia secondary to ADEM remains scarcely reported. Here, the authors present a case of dystonia following an episode of ADEM in childhood unresponsive to bilateral DBS of the globus pallidus internus (GPi). OBSERVATIONS: The authors report the case of a 12-year-old patient presenting with medication-refractory generalized dystonia secondary to infantile ADEM. Following onset of ADEM at 22 months of age, the patient began exhibiting motor skill regression and progressive generalized dystonia. The dystonia remained refractory to numerous medications, including anticonvulsant, dopaminergic, anticholinergic, and muscle relaxant agents. The decision was made to perform bilateral GPi DBS. The Burke-Fahn-Marsden Dystonia Rating Scale-Motor was used to assess treatment outcomes before and after surgery. At 12 months postoperation, no significant improvements or adverse effects were observed. LESSONS: Age of onset and duration of dystonic symptoms must be considered when evaluating pediatric patients for DBS treatment of dystonia secondary to ADEM. https://thejns.org/doi/10.3171/CASE25979.

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