Smartphone measures motor and respiratory function in spinal muscular atrophy.

Investigating and following the motor function in children with SMA is challenging. In this issue of Neuromuscular Disorders, Perumal et al. (2023) describes how the smartphone sensor can be used to assess the respiratory and upper limb function in comparison to physical outcome measures currently in use. Eight out of nine items were closely in accord.

Sleep and social-emotional problems in preschool-age children with developmental delay.

Sleep problems and social-emotional (SE) problems are common in preschooler children and may be affected by culture. However, little is known about the link between sleep and social-emotional problems in non-Western countries. This study aims to compare sleep problems and their association with SE problems in Turkish children with either typical development (TD) or neurodevelopmental delay (NDD). In this case-control study, children with NDD (n = 126) and children with TD (n = 102) were included. Parents completed the Children's Sleep Habits Questionnaire (CSHQ), and the Ages and Stages Questionnaire: Social-Emotional (ASQ:SE). More than half of the TD and NDD groups had sleep problems when assessed by CSHQ (54.4%, 72.2%, respectively). The correlation between CSHQ and ASQ:SE scores in the NDD and TD groups was r = 0.44 and r = 0.352, respectively (both p < 0.001). Children who slept less than 9 h had lower ASQ:SE scores in the NDD (p = 0.003) and TD group (p = 0.023). In the TD group, those who slept after 23:01 h had lower ASQ:SE scores compared to early sleepers (p = 0.04). Multivariate analysis revealed associations between SE problems and male gender, lower family income, bedtime resistance, and shorter sleep duration in the NDD group. In the TD group, male gender, sleep onset delay, sleep duration of CSHQ subscale were found to be associated with SE problems. Sleep problems were identified in more than half of all preschooler children, regardless of developmental delay, and were associated with increased SE problems. Interventions to correct sleep problems may have a positive impact on children's emotional development.

Concurrence of juvenile idiopathic arthritis and primary demyelinating disease in a young child.

CASE REPORT: The association of juvenile idiopathic arthritis (JIA) and primary demyelinating disease of central nervous system (CNS) in the same patient is rare. Here we present a 10-year-old girl formerly diagnosed with JIA who presented with acute total vision loss. Magnetic resonance imaging of the brain and spinal cord showed bilateral optic neuritis and T2 hyperintense lesions in the brain, cerebellum and cervical spinal cord, some of them gadolinium-enhancing. Oligoclonal bands were present in the cerebrospinal fluid. Visual evoked potentials were prolonged. Aquaporin-4 antibodies were negative. The patient was treated with methylprednisolone 30 mg/kg daily for five days, resulting in improvement in vision and gait. This first demyelinating event in this patient with JIA with clinical and paraclinical features meeting the 2017 MS diagnostic criteria supports a possible predisposition to autoimmune disorders. CONCLUSION: The concurrence of JIA and multiple sclerosis (MS) has been reported in only two adult cases and not in the pediatric population. While JIA and MS are two distinct chronic inflammatory diseases, immunogenetic predisposition and common environmental triggers might be involved.