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

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

Eom S; Lee Y M

Pediatric Neurology

2017

emotion; child behavior; priority journal; intellectual impairment/dm [Disease Management]; prognosis; preschool child; parental stress; human; article; child; female; male; quality of life; clinical article; daily life activity; disease severity; aggression; intelligence quotient; childhood disease/dm [Disease Management]; comorbidity; Leigh disease/dm [Disease Management]; MELAS syndrome/dm [Disease Management]; nervous system development; problem behavior/dm [Disease Management]; anxiety disorder/dm [Disease Management]; attention disturbance/dm [Disease Management]; brain atrophy/dm [Disease Management]; delinquency; depression/dm [Disease Management]; drug resistant epilepsy/dm [Disease Management]; drug resistant epilepsy/dr [Drug Resistance]; intelligence; lactic acidosis/dm [Disease Management]; muscle disease/dm [Disease Management]; neuroimaging; nuclear magnetic resonance imaging; postnatal depression/dm [Disease Management]; psychomotor development; sleep disorder/dm [Disease Management]; social problem; somatization/dm [Disease Management]; thinking impairment/dm [Disease Management]; behavioral problems; Leigh syndrome; mitochondrial disorders; MELAS syndrome; trajectory; characteristics

Background Little is known regarding the neuropsychological profiles of pediatric patients with mitochondrial diseases or their parents, information that is crucial for improving the quality of life (QOL) for both patients and parents. We 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. Methods Seventy 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 the 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. Results Prediagnostic assessments indicated that cognitive and psychomotor developments were significantly delayed. Group mean full scale intelligence quotient (IQ) 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 behavioral problems, whereas 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 the first symptoms, diffuse brain atrophy, and drug-resistant epilepsy negatively influenced neurodevelopmental and adaptive functions. Conclusion Better understanding of the functional levels and profiles of neurodevelopment and psychological comorbidity in children with mitochondrial diseases in the prediagnostic period is essential for adequate support and QOL of children with mitochondrial diseases and their parents.

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