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Behavioural problems in children and adolescents with spinal muscular atrophy and their siblings
Author(s) -
LaufersweilerPlass C,
RudnikSchöneborn S,
Zerres K,
Backes M,
Lehmkuhl G,
Gontard A
Publication year - 2003
Publication title -
developmental medicine and child neurology
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.658
H-Index - 143
eISSN - 1469-8749
pISSN - 0012-1622
DOI - 10.1111/j.1469-8749.2003.tb00858.x
Subject(s) - cbcl , sma* , child behavior checklist , psychopathology , anxiety , comorbidity , medicine , pediatrics , psychology , psychiatry , combinatorics , mathematics
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.

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