Dystonia in Pediatric Huntington's Disease; Prominent and Possibly Painful

Le résumé fourni par la source

Pediatric Huntington's disease (PHD) is defined as affected with Huntington's disease under the age of 18. 1 Common clinical features at the presentation of PHD are cognitive impairment, behavioral changes, gait disorder, cerebellar signs and oral motor dysfunction. 1 Dystonia and pain have previously been reported as possibly common, but unrecognized features in PHD, which are not necessarily intertwined.2 Our two videos suggest that painful generalized dystonia can be a prominent feature in PHD (Videos 1 and 2).Dystonia can be paroxysmal (Video 2) and non-paroxysmal (Video 1).When correctly recognized, symptomatic treatment can improve quality of life of these patients.Unfortunately, in case 1 (Video 1) pain medication including opiates were not sufficient and ultimately, pain led to palliative sedation.In case 2 (Video 2), opiates were prescribed to improve the pain, with limited effect (Videos 1,2).

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