Dystonia Severity Action Plan: a simple grading system for medical severity of status dystonicus and life‐threatening dystonia

Gabapentin can significantly improve dystonia severity and quality of life in children

“…19 The newly defined Dystonia Severity Assessment Plan (DSAP) grading was also used ( Table 2). 21 This is a parent/carer/allied…”

Recognizing the Common Origins of Dystonia and the Development of Human Movement: A Manifesto of Unmet Needs in Isolated Childhood Dystonias

“…Dystonia, Parkinsonian rigidity, tics, chorea, and athetosis are always abolished by sleep (40, 41). The exploration of the influence of sleep should therefore be a focus of all dystonia management plans (42–44) and may be considered the “ poor man’s examination under anesthesia .”…”

“…A crucial use of sleep to “switch off dystonia” in SD can be a very valuable clinical strategy for managing this life-threatening clinical catastrophe, which most commonly occurs in adolescent males with secondary dystonia, often of CP origin (42, 43, 45). The disadvantage of conventional muscle relaxants is that they also provoke respiratory depression and muscle weakness leading to central hypoventilation, shallow breathing, or frank apnoea (42, 43), whereas the hypnotic approach, e.g., with clonidine, can switch off SD by inducing sleep while preserving muscle function and breathing (43).…”

“…The disadvantage of conventional muscle relaxants is that they also provoke respiratory depression and muscle weakness leading to central hypoventilation, shallow breathing, or frank apnoea (42, 43), whereas the hypnotic approach, e.g., with clonidine, can switch off SD by inducing sleep while preserving muscle function and breathing (43). …”

The impact and prognosis for dystonia in childhood including dystonic cerebral palsy: a clinical and demographic tertiary cohort study

“…The functional burden of dystonia, likelihood of worsening and lack of likely spontaneous remission in this selected cohort further serves to emphasise the importance of increasing interventional research efforts in the paediatric dystonia population to reduce comorbidities and improve the quality of life for children with dystonia. More work is required to recognise the severity of dystonia in children,29 obtain true population data on dystonia in children and especially to determine the quality of life of children with dystonia and the burden of care to parents and carers. Improved understanding and management plans for dystonia emergencies such as ‘brittle dystonia’ and status dystonicus30 in children will be helped by an overall understanding of the demographic impact of dystonia in children.…”