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Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy
09:18

Measurements of Motor Function and Other Clinical Outcome Parameters in Ambulant Children with Duchenne Muscular Dystrophy

Published on: January 12, 2019

10.9K

Cognitive profile in Duchenne muscular dystrophy boys without intellectual disability: The role of executive

R Battini1, D Chieffo2, S Bulgheroni3

  • 1Pediatric Neurology Unit, Fondazione Policlinico Gemelli, and Nemo Center, UCSC Rome, Italy; Department of Developmental Neuroscience, IRCCS Stella Maris, Calambrone (Pisa), Italy.

Neuromuscular Disorders : NMD
|January 7, 2018
PubMed

Insights

Cognitive function, particularly executive functions like problem-solving and working memory, can be impaired in boys with Duchenne muscular dystrophy, even without intellectual disability.

Area of Science:

  • Neurology
  • Pediatrics
  • Cognitive Science

Background:

  • Duchenne muscular dystrophy (DMD) is a genetic disorder primarily affecting muscle strength.
  • Cognitive function, including executive functions, has been an understudied area in DMD, especially in individuals without intellectual disability.

Purpose of the Study:

  • To assess cognitive function profiles in boys with Duchenne muscular dystrophy.
  • To investigate potential impairments in executive functions within this cohort.

Main Methods:

  • Prospective observational study involving 40 boys with Duchenne muscular dystrophy (age 6-11.5 years).
  • Assessment using the Wechsler Intelligence Scale and a battery of tests for working memory and executive functions (inhibition, switching, problem-solving, planning).

Main Results:

  • Impairments were frequently observed in specific cognitive functions.
  • Affected areas included multitasking, problem-solving, inhibition, and working memory crucial for goal-directed behavior.

Conclusions:

  • Cognitive function deficits can occur in boys with Duchenne muscular dystrophy, irrespective of intellectual disability.
  • Executive functions appear to play a significant role in the cognitive impairments associated with Duchenne muscular dystrophy.

Keywords:
CerebellumDMDExecutive functionsNeuropsychological profileWorking memory

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