Enuresis and urinary incontinence in children and adolescents with spinal muscular atrophy

Objective To assess the rate and type of urinary incontinence in a large sa mple of children and adolescents with spinal muscular atrophy (SMA), a gene tic disorder characterized by loss of motor function caused by anterior hor n degeneration. Patients, subjects and methods The study included 96 severely incapacitated patients with SMA (aged 6.0-18.11 years) who were examined in detail, incl uding a structured interview (Kinder-DIPS). the Child Behaviour Checklist ( CBCL) and a specific questionnaire for urinary incontinence. They were comp ared with two control groups of unaffected siblings and normal children. Results In all. 29%, of the patients were wet at night and/or during the da y; mostly younger children with SMA types I and II only were affected. The results of the interview were more reliable than the CBCL. The specific que stionnaire revealed a variety of possible functional and neurogenic forms o f wetting, including nocturnal enuresis, voiding postponement, dysfunctiona l voiding, stress, symptomatic (urinary tract infections, UTIs) and neuroge nic incontinence. Many patients were constipated, soiled or had UTIs. The r ate of behavioural problems was twice as high (32%) as normal (15%: CBCL). Conclusion Children with SMA have it high rate of urinary incontinence whic h is often overlooked, and not diagnosed and treated adequately. These prob lems should be addressed routinely by paediatricians in children referred t o paediatric urological specialists.