ACQUIRED HEMIDYSTONIA IN CHILDHOOD - A CLINICAL AND NEURORADIOLOGICALSTUDY OF 13 PATIENTS

A retrospective study of 13 patients (4 males/9 females) with acquired hemidystonia in childhood is reported, The mean age of onset of hemid ystonia was 6.4 years (range 1-13.4 years); the mean duration of dysto nia at the time of last follow-up was 11.4 years (range 3.6-23 years). Hemidystonia was caused by ischemic infarction in 9 patients and was attributed to perinatal trauma in 1; in 4 of the 9 patients with strok e and in the remaining 3 patients laboratory investigations were sugge stive of primary antiphospholipid syndrome, Eleven of the 13 patients had delayed onset of dystonia: between 1 month and 8.9 years (mean 3.4 years), Ten patients had neuroradiological evidence of contralateral basal ganglia damage, A history of hemiparesis and evidence of striata l damage on CT or MRI were important risk factors for the development of dystonia, Response to medical treatment (trihexyphenidyl dose as hi gh as 40 mg daily) in 5 patients was disappointing; 4 of the 5 patient s who underwent functional stereotaxic operations were improved, but d ystonia was still present at the end of the follow-up. Our study provi des additional evidence that lesions of the striatum may induce dyston ia, supporting the theory of striato-pallido-thalamic disconnection. F urthermore, our results indicate that the occurrence of delayed dyston ia must be considered in the diagnostic approach to childhood-onset dy stonia.