Objective: To study the implication of pallidal DBS in treatment of pediatric status dystonicus (SD).

Background: SD or dystonic storm is an emergency condition caused by exacerbation of dystonia with severe generalized or focal hyperkinetic movements, which requires urgent hospitalization. SD is more common in children and young adults, has variable course and outcome, is associated with a high level of life-threatening complications and mortality [1,2,3,4]. Pharmacological treatment of SD is often insufficient, and surgical procedures are used to save patients [5,6,7]

Method: We report 7 children with generalized dystonia and pharmacoresistant SD treated by GPi-DBS (age of dystonia onset 4.3±2.3, dystonia duration 4.1±2.2, age at surgery 8.3±2.8 years). In 5 patients genetic testing was performed, which revealed mutations in GNAO1(2), KMT2B(1), KIF1A(1), and PAK1(1) genes. SD duration prior to surgery comprised 1.5-6 months. 4 patients had relapsing SD course. Medication included benzodiazepines, trihexyphenidyl, baclofen, tetrabenazine, valproate, gabapentin, chlorpromazine, levodopa, fentanyl, dexmedetomidine, thiopental, botulinum toxin. 5 patients required staying in ICU. Follow-up ranged from 6 months to 2 years.

Results: Stimulation was initiated 1-6 days after implantation. Following GPi-DBS, SD was resolved in all cases during the first 2 weeks with subsequent amelioration of motor disturbances. In one patient with GNAO1-related dystonia, early postoperative period was complicated by development of seizures/status epilepticus. In 2 patients, SD relapse developed later in course. 2 patients had infectious complications in short-term or long-term follow-up, which necessitated removal of implant. In one of them rescue bilateral pallidotomy was performed. Patient with KIF1A-related dystonia died due to dystonia worsening and complications of SD relapse 2 years after surgery.

Conclusion: Status and prestatus distonicus require prompt recognition and timely management. GPi-DBS is effective in relieving of pharmacoresistant SD of various etiologies and reduces dystonia severity in the short term. In the long term, outcome varies individually and is characterized by a high incidence of complications and possible SD recurrence. Pallidotomy is an option for patients with limitations for DBS use. When SD is terminated, overall prognosis is determined by underlying condition (developmental disorder, concomitant neurological symptoms).

References: 1. Fasano A, Ricciardi L, Bentivoglio AR, Canavese C, Zorzi G, Petrovic I, Kresojevic N, Kostić VS, Svetel M, Kovacs N, Balas I, Roubertie A, Mishra D, Mariotti P, Temudo T, Nardocci N. Status dystonicus: predictors of outcome and progression patterns of underlying disease. Mov Disord. 2012;27(6):783-8. doi: 10.1002/mds.24981
2. Lumsden DE, Lundy C, Fairhurst C, Lin JP. Dystonia Severity Action Plan: a simple grading system for medical severity of status dystonicus and life-threatening dystonia. Dev Med Child Neurol. 2013;55(7):671-2. doi: 10.1111/dmcn.12108
3. Saini AG, Hassan I, Sharma K, Muralidharan J, Dhawan S, Saini L, Suthar R, Sahu J, Sankhyan N, Singhi P. Status Dystonicus in Children: A Cross-Sectional Study and Review of Literature. J Child Neurol. 2022;37(6):441-450. doi: 10.1177/08830738221081593
4. Iodice A, Pisani F. Status dystonicus: management and prevention in children at high risk. Acta Biomed. 2019;90(3):207-212. doi: 10.23750/abm.v90i3.7207
5. Vogt LM, Yan H, Santyr B, Breitbart S, Anderson M, Germann J, Lizarraga KJ, Hewitt AL, Fasano A, Ibrahim GM, Gorodetsky C. Deep Brain Stimulation for Refractory Status Dystonicus in Children: Multicenter Case Series and Systematic Review. Ann Neurol. 2023. doi: 10.1002/ana.26799
6. Garofalo M, Beudel M, Dijk JM, Bonouvrié LA, Buizer AI, Geytenbeek J, Prins RHN, Schuurman PR, van de Pol LA. Elective and Emergency Deep Brain Stimulation in Refractory Pediatric Monogenetic Movement Disorders Presenting with Dystonia: Current Practice Illustrated by Two Cases. Neuropediatrics. 2023;54(1):44-52. doi: 10.1055/a-1959-9088
7.Garg K, Singh M, Samala R, Rajan R, Gulati S, Goyal V. Bilateral pallidotomy for acquired or heredodegenerative generalized dystonia in children. Neurosurg Focus. 2022;53(4):E12. doi: 10.3171/2022.7.FOCUS22332

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MDS Abstracts - https://www.mdsabstracts.org/abstract/treatment-of-status-dystonicus-in-children-using-pallidal-deep-brain-stimulation/