title: Behavioural problems in children and adolescents with spinal muscular atrophy and their siblings.
abstract: Spinal muscular atrophy (SMA) is a chronic illness characterized by loss of motor function. The aim of the study was to investigate behavioural adjustment in 96 children and adolescents with SMA (47 males, 49 females; mean age 11 years 2 months, range 6 to 18 years). Forty-five non-affected siblings (26 males, 19 females; mean age 11 years 6 months, range 6 to 18 years) and 59 normally developing children (33 males, 26 females; mean age 10 years 8 months, range 6 to 18 years) were recruited as control participants. Behavioural symptoms were measured with the Child Behaviour Checklist (CBCL) and disorders were assessed with a structured psychiatric interview (Kinder-DIPS). Of the patients with SMA, 12.5% fulfilled the criteria for an ICD-10 or DSM-IV diagnosis, with separation anxiety disorder being the most common diagnosis. The CBCL total score was in the clinical range for 11.5% of patients, 20% of the siblings, and 11.7% of the control children; the externalizing score rates were 2.1%, 22.2%, and 11.9% respectively; the internalizing score 18.9%, 24.4%, and 13.6% respectively. Comorbid psychopathology was not influenced by sex, IQ, nor severity of SMA, and only externalizing behaviour was correlated to age. In conclusion, children and adolescents with SMA are characterized by a low psychiatric comorbidity not different from control individuals. The group with the highest rate of behavioural problems and with the greatest need for intervention were the non-affected siblings who had a two- to threefold higher rate of behavioural problems than the normative population.
authors.affiliation: Department of Child and Adolescent Psychiatry, University of Cologne, Germany. laufersweiler-plass.c@debitel.net
authors.forename: C
authors.initials: C
authors.lastname: Laufersweiler-Plass
authors.forename: S
authors.initials: S
authors.lastname: Rudnik-Schöneborn
authors.forename: K
authors.initials: K
authors.lastname: Zerres
authors.forename: M
authors.initials: M
authors.lastname: Backes
authors.forename: G
authors.initials: G
authors.lastname: Lehmkuhl
authors.forename: A
authors.initials: A
authors.lastname: von Gontard
mesh_terms: D000293:Adolescent; D000367:Age Factors; D000704:Analysis of Variance; D016022:Case-Control Studies; D002648:Child; D002653:Child Behavior Disorders / Q000175:diagnosis / Q000453:epidemiology / Q000209:etiology* / Q000523:psychology; D015897:Comorbidity; D039721:Diagnostic and Statistical Manual of Mental Disorders; D005260:Female; D005858:Germany / Q000453:epidemiology; D006801:Humans; D007361:Intelligence Tests; D007389:Internal-External Control; D007406:Interview, Psychological; D008297:Male; D009134:Muscular Atrophy, Spinal / Q000150:complications*; D010290:Parents / Q000523:psychology; D012307:Risk Factors; D012494:Sampling Studies; D012720:Severity of Illness Index; D035781:Siblings