The Society provided funding to the Evelina Children’s hospital to review its records to assess the impact of childhood dystonia and the results of therapies provided on 2011. This research has now resulted in two important published papers.

The article “Proportion of life lived with dystonia inversely correlates with response to pallidal deep brain stimulation in both primary and secondary childhood dystonia” was published in “Developmental medicine and child neurology” in June 2013.  The article made two important points that are likely to improve the treatment of children with dystonia:

  • Deep brain stimulation (DBS) appears to decline in effectiveness for children with primary dystonia as the proportion of life lived with dystonia grows. Hitherto DBS had been considered a treatment of last resort and so was delayed while other therapies were tried. As a result, the effectiveness of DBS was reduced. There is therefore more urgency in providing DBS than had previously been understood.
  • DBS had been considered ineffective for children with secondary dystonia but the results suggest if it can be done early enough it may be effective. More work needs to be done in this area and the Society has provided additional funding to the Evelina (see Treatment of Secondary Dystonia below).

The article “The impact and prognosis for dystonia in childhood including dystonic cerebral palsy: a clinical and demographic tertiary cohort” was published in the Journal of Neurosurgery and Psychiatry in February 2014. The article clarified a number of points about early-onset dystonia:

  • Acquired dystonia (72% of cases) is far more common than primary dystonia (18%) of cases. Children with secondary had more serious functional impairment.
  • Spasticity was present as comorbidity in only 34% of cases – (N.b. it is sometimes said that dystonia caused by cerebral palsy rarely appears in isolation from spasticity. This establishes that is not the case and that dystonic cerebral palsy is a distinct condition).
  • In 60% of cases dystonia was still progressing at time of referral (median 4.8 years after onset). Previously it had been thought dystonia stabilised more quickly.
  • 20% of children with secondary dystonia had a period of normal motor development which improve the prognosis for treatment if timely referral is made.

These results help services offering neurosurgical interventions and health service planning agencies to understand the context and predicament of life with childhood dystonia.