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Behavioural problems in children and adolescents with spinal muscular atrophy and their siblings
C Laufersweiler-Plass1, S Rudnik-Schöneborn, K Zerres
1Department of Child and Adolescent Psychiatry, University of Cologne, Germany. laufersweiler-plass.c@debitel.net
Insights
Children with spinal muscular atrophy (SMA) show low psychiatric comorbidity, similar to typically developing peers. However, their unaffected siblings exhibit significantly higher rates of behavioral problems, indicating a greater need for support.
Area of Science:
- Pediatric Neurology
- Child Psychiatry
- Behavioral Science
Background:
- Spinal muscular atrophy (SMA) is a chronic neuromuscular disorder impacting motor function.
- Understanding the psychological and behavioral adjustment in children with SMA is crucial for comprehensive care.
- Previous research has not fully elucidated the behavioral profiles of children with SMA and their siblings.
Purpose of the Study:
- To investigate behavioral adjustment and psychiatric comorbidity in children and adolescents with SMA.
- To compare behavioral outcomes between children with SMA, their unaffected siblings, and typically developing controls.
- To identify factors influencing psychopathology in the SMA population.
Main Methods:
- A cross-sectional study involving 96 children/adolescents with SMA, 45 unaffected siblings, and 59 typically developing controls.
- Behavioral symptoms assessed using the Child Behaviour Checklist (CBCL).
- Psychiatric disorders diagnosed via structured interview (Kinder-DIPS) based on ICD-10/DSM-IV criteria.
Main Results:
- 12.5% of SMA patients met criteria for a psychiatric diagnosis, most commonly separation anxiety disorder.
- CBCL scores indicated clinical ranges for 11.5% of SMA patients, 20% of siblings, and 11.7% of controls.
- Unaffected siblings showed a 2-3 fold higher rate of behavioral problems compared to the normative population, particularly in externalizing and internalizing behaviors.
Conclusions:
- Children and adolescents with SMA exhibit low psychiatric comorbidity, comparable to control groups.
- Unaffected siblings of children with SMA represent a vulnerable group with a higher prevalence of behavioral issues.
- Targeted psychological support and intervention may be warranted for siblings of children with SMA.
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.