Children With SMA Have Increased Anxiety, Depression

New study findings show that internalized problems such as anxiety and depression were found at higher levels in children and infants who had a diagnosis of spinal muscular atrophy (SMA).

Symptoms of anxiety and depression were increased in infants and young children who had a diagnosis of spinal muscular atrophy (SMA), according to new study findings in European Journal of Paedriatric Neurology.

This study aimed to evaluate the behavior of children with a diagnosis of SMA who are younger than 5 years compared with children who had other chronic nonneurological illnesses.

The researchers used a cross-sectional study design that was performed at a tertiary care teaching hospital in North India. Children with genetically proven SMA who were aged 3 months to 5 years were enrolled from August 2019 to November 2021; 15 children were aged 2 to 5 years. All children were classified into types 1 (n = 11), 2 (n = 19), and 3 (n = 5). Control groups of healthy children (n = 35) and children with nephrotic syndrome (n = 15) also were included.

All children had their medical history assessed before the study via physical and neurological evaluations. The researchers used the Infant Behavior Questionnaire-revised (IBQ-R) and Child Behavior Check List for infants and older children, respectively, to assess their behavior. The developmental profile-3 (DP3) and Malin’s intelligence scale for Indian children were used for development quotient assessment in the control groups.

The researchers found that development measures that were obtained through the DP3 were all lower in the group with SMA, with statistically significant differences only in the mean (SD) physical (18.4 [6.5] in SMA group vs 97.6 [5.1] in control) and adaptive (25.7 [8.6] in SMA group vs 93.8 [4.9] in control group) behavior scores. Children with SMA also had significantly higher scores for distress to limitations (5.1 [2.6] vs 3.8 [1.4]), fear (4.9 [2.1] vs 3.8 [1.7]), sadness (4.8 [2.3] vs 2.1 [0.9]), and falling reactivity (4.1 [1.9] vs 2.8 [1.2]) in the IBQ-R compared with the healthy control group.

The internalizing problem scores and stress problem scores in older children with a diagnosis of SMA were higher compared with the control groups. The emotionally reactive (7.1 [3.8] vs 4.6 [2.8]) and anxious (7.4 [3.9] vs 3.8 [2.1]) domains also had higher scores. Adjustment for socioemotional, cognitive, and communication domains in the DP3 found that the difference were still statistically significant.

Children with types 1 and 2 SMA and aged 2 to 5 years had a better behavioral profile compared with children with type 3 SMA.

There were some limitations to this study. There was a small sample size of children with SMA, and a lack of highly sensitive and objective measures was noted. Most participants came from financially challenging households, and none of the children included were receiving drug treatment.

The researchers concluded that infants and young children who had a diagnosis of SMA had an increase in internalizing problems such as anxiety and depression.


Panda PK, Rmachandran A, Verma PK, Sharawat IK. Behavioral problems in infants and young children with spinal muscular atrophy and their siblings: a cross-sectional study. Eur J Paediatr Neurol. 2023;42:47-52. doi:10.1016/j.ejpn.2022.12.006

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