Preliminary Study of Neurodevelopmental Outcomes and Parenting Stress in Pediatric Mitochondrial Disease

Publication date: Available online 2 February 2017
Source:Pediatric Neurology
Author(s): Soyong Eom, Young-Mock Lee
PurposeLittle is known regarding the neuropsychological profiles of pediatric patients with mitochondrial diseases or their parents, which are crucial for improving quality of life (QOL) for both patients and parents. The present study aimed to delineate neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the preliminary investigation of adequate intervention methods, better prognoses, and improved QOL for both patients and parents.MethodsSeventy children diagnosed with mitochondrial diseases were neuropsychologically evaluated. Neurocognitive (development, intelligence) and psychological (behavior, daily living function, maternal depression, parenting stress) functions were analyzed. Clinical variables including first symptom, epileptic classification, organ involvement, lactic acidosis, brain magnetic resonance imaging findings, muscle pathology, biochemical enzyme assay results, and syndromic diagnosis of mitochondrial diseases were also reviewed.ResultsPre-diagnostic assessments indicated that cognitive and psychomotor development were significantly delayed. Group mean full scale IQ (intelligence quotient) scores indicated mild levels of intellectual disability, borderline levels of verbal IQ impairment, and mild levels of intellectual disability on performance IQ. Many children exhibited clinically significant levels of behavior problems, while mothers of children with mitochondrial diseases exhibited significant increases in parenting stress relative to mothers of healthy children. Furthermore, 65% of mothers exhibited significant levels of depression. Early onset of first symptom, diffuse brain atrophy, and drug-resistant epilepsy negatively influenced neurodevelopmental and adaptive functions.ConclusionBetter understanding of functional levels and profiles of neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the pre-diagnostic period is essential for adequate support and QOL of children with mitochondrial diseases and their parents.



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