Background Estimation of calorie need in children with cerebral palsy is challenging; many have decreased energy requirements in line with increasing severity of gross motor impairment. However, those with dyskinesia may have higher calorie demand but there is limited evidence to quantify this. Gastrointestinal dysmotility is common in children with dystonia and dystonia is often exacerbated by gastrointestinal pain. A vicious cycle can result leading to severe dystonia, gastrointestinal failure and a poor nutritional state. We report a case of a child requiring total parental nutrition (TPN) that enabled estimation of calorie demand in poorly controlled dystonia.
Case history A 7 year old boy with 4 limb dystonic cerebral palsy (GMFCS V), secondary to extreme prematurity (23 weeks), presented with frequent episodes of status dystonicus and enteral feeding and medication intolerance associated with intercurrent illness and hip surgery. Weight gain had been limited for several years. Failure of conservative management, including jejunal feeding and sigmoid colectomy, led to a prolonged admission requiring TPN. As enteral baclofen was not tolerated, it was decided to insert an intrathecal baclofen (ITB) pump. Prior to this, dystonia management was very difficult needing high dose intravenous clonidine and diazepam. Following insertion, there was a dramatic improvement in dystonia control which had a profound effect on calorie demand. At the time of ITB insertion (week 8 of TPN, weight 23.55 kg) satisfactory weight gain had been achieved at 84 kcal/kg/day. Over the next 4 weeks, he gained 2.05 kg despite calorie reduction by 36% to 53 kcal/kg/day. This need continued until week 28 when it was decreased to 42 kcal/ kg/day (50% of initial requirement). After a further 9 months, he remains on 42 kcal/kg/day of home TPN. He has not yet reestablished enteral nutrition but dystonia remains well controlled with ITB (690 micrograms/day), PR diazepam 20 mg BD and enteral clonidine 25 micrograms at night.
Conclusion Calorie requirements in children with severe dystonia are high; recognising this is important to ensure optimal nutrition. When dystonia is complicated by gut dysmotility, providing calories, in addition to achieving dystonia control, can be a challenge; in this case ITB was an effective treatment.
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