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Deep brain stimulation in pediatric dystonia: a systematic review

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Abstract

While deep brain stimulation (DBS) treatment is relatively rare in children, it may have a role in dystonia to reduce motor symptoms and disability. Pediatric DBS studies are sparse and limited by small sample size, and thus, outcomes are poorly understood. Thus, we performed a systematic review of the literature including studies of DBS for pediatric (age < 21) dystonia. Patient demographics, disease causes and characteristics, motor scores, and disability scores were recorded at baseline and at last post-operative follow-up. We identified 19 studies reporting DBS outcomes in 76 children with dystonia. Age at surgery was 13.8 ± 3.9 (mean ± SD) years, and 58% of individuals were male. Post-operative follow-up duration was 2.8 ± 2.8 years. Sixty-eight percent of patients had primary dystonia (PD), of whom 56% had a pathological mutation in DYT1 (DYT1+). Across all patients, regardless of dystonia type, 43.8 ± 36% improvement was seen in Burke-Fahn-Marsden Dystonia Rating Scale (BFMDRS) motor (-M) scores after DBS, while 43.7 ± 31% improvement was observed in BFMDRS disability (-D) scores. Patients with PD were more likely to experience ≥ 50% improvement (56%) in BFMDRS-M scores compared to patients with secondary causes of dystonia (21%, p = 0.004). DYT1+ patients were more likely to achieve ≥ 50% improvement (65%) in BFMDRS-D than DTY1− individuals (29%, p = 0.02), although there was no difference in BFMDRS-M ≥ 50% improvement rates between DYT1+ (66%) or DYT1− (43%) children (p = 0.11). While DBS is less common in pediatric patients, individuals with severe dystonia may receive worthwhile benefit with neuromodulation treatment.

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Funding

A.T.H. is supported by the Vanderbilt University Medical Scientist Training Program (T32GM007347). D.J.E. receives support from the National Institutes of Health (NIH, R00 NS097618).

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Authors and Affiliations

  1. Department of Neurological Surgery, Vanderbilt University Medical Center, Nashville, TN, USA

    Andrew T. Hale, Meredith A. Monsour, Robert P. Naftel & Dario J. Englot

  2. Medical Scientist Training Program, Vanderbilt University School of Medicine, 2200 Pierce Ave. 610 Robinson Research Building, Nashville, TN, 37232, USA

    Andrew T. Hale

  3. Department of Neurosurgery, University of Utah, Salt Lake City, UT, USA

    John D. Rolston

  4. Division of Pediatric Neurosurgery, Monroe Carell Jr. Children’s Hospital of Vanderbilt University, Nashville, TN, USA

    Robert P. Naftel

  5. Vanderbilt Institute for Surgery and Engineering, Vanderbilt University Medical Center and Vanderbilt University, Nashville, TN, USA

    Dario J. Englot

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Hale, A.T., Monsour, M.A., Rolston, J.D. et al. Deep brain stimulation in pediatric dystonia: a systematic review. Neurosurg Rev 43, 873–880 (2020). https://doi.org/10.1007/s10143-018-1047-9

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