Behavior Problems and Family Stress Associated with Fragile X Syndrome (FXS) and Spinal Muscular Atrophy (SMA) in German Boys

Children with mental retardation or chronic physical illness can be very challenging for families. To compare behavioral problems and family stresses associated with mental retardation, chronic illness, and the absence of both retardation and illness, a team of German researchers used the CBCL, a structured psychiatric interview, and measures of parental stress, coping, and social support (Von Gontard et al., 2002).

The children included 49 boys with FXS, which is the second most common genetic form of mental retardation; 46 boys with SMA, which is a relatively common genetically caused condition characterized by normal intelligence but loss of motor function, muscular atrophy, and degeneration of the anterior horn cells; and 32 normal control boys. Von Gontard et al. found that 89.8% of the FXS boys had CBCL Total Problems scores in the borderline or clinical range, compared to 21.7% of the SMA boys and 15.7% of the normal control boys. On the Internalizing scale, 63.3% of the FXS boys obtained borderline or clinical scores, compared to 34.8% of the SMA boys and 21.9% of the normal control boys.

On the Externalizing scale, the percentages were 67.3% for the FXS boys and 11.8% for both other groups. In all comparisons, borderline and clinical range problem scores were significantly more common among FXS boys than among the other two groups, which did not differ significantly from each other. Structured diagnostic interviews with the boys’ parents identified disorders in 81.6% of the FXS boys, compared to 10.9% of the SMA boys.

In all three groups, CBCL problem scores correlated significantly with family stress measured by the Questionnaire on Resources and Stress. Based on the much higher rate of behavioral and emotional problems reported for FXS than SMA boys and the significant correlations between CBCL scores and family stress in each group, the authors concluded “that families with mentally retarded children are in even greater need of help than those of children with severe chronic illness/physical handicap” (p. 955).

Reference: Von Gontard, A., Backes, M., Laufersweller-Plass, C., Wendland, C., Lehmkuhl, G., Zerres, K., & Rudnik-Schoneborn, S. (2002). Psychopathology and familial stress-comparison of boys with Fragile X syndrome and Spinal Muscular Atrophy. Journal of Child Psychology and Psychiatry, 43, 949-957.