The Dystonia Society provided funding to the Evelina to re-assess the results of DBS for children with acquired dystonia using a new performance measure.

The above results established that early-onset secondary dystonia is both more common and causes more severe functional impairment than early-onset primary dystonia. However, the findings also offered some ground for optimism that, if children with acquired dystonia who have had a period of normal motor development can receive DBS early, at least a degree of symptom mitigation can be provided in some cases.

The Evelina also identified the qualitative finding that parents of children with dystonia reported important functional improvements after DBS even if the main rating scale assessing the severity of dystonia, the BFMDRS, did not show improvement. Given the severity of the disablement, such functional improvements can make a substantial difference to quality of life.

To test this, the Evelina evaluated the results of DBS using a different scale, the Canadian Occupational Performance Measure (COPM). The article “Evaluation of functional goal outcomes using the Canadian Occupational Performance Measure (COPM) following Deep Brain Stimulation (DBS) in childhood dystonia” was published in the “European Journal of Paediatric Neurology” in January 2014. It reported that:

  • The acquired dystonia group achieved significant improvement on the COPM at 6 months for performance and satisfaction scores. In the majority of acquired dystonias, the BFMDRS failed to demonstrate significant improvement.
  • This highlights the insensitivity of the existing scale.

The Evelina group continue to develop this important work which is part of process of intense review of treatment of early-onset dystonias by the Paediatric Movement Disorder Special Interest Group.