Cognitive and behavioral functioning in two neurogenetic disorders; how different are these aspects in Duchenne muscular dystrophy and Neurofibromatosis type 1?

The presence of neurocognitive and behavioral problems are common features in various neurogenetic disorders. In Duchenne muscular dystrophy (DMD), these problems have been linked to mutations along the dystrophin gene affecting different brain dystrophin isoforms. However, comparable cognitive and behavioral problems have been found in Neurofibromatosis type 1 (NF1).

Can IQ predict parent-reported behavioral and emotional problems in children with neurological deficiencies?

Objective: The aim of the current study was to investigate whether total intelligence scores (FSIQ) and/or a discrepancy in intelligence can predict behavioral or emotional problems in children with neurological deficiencies.

Method: The population consists of children with neurological deficiencies (N = 610, ranging from 6 to 17 years), referred due to concerns on the (educational) development of the child to a tertiary outpatient clinic. All children were tested with the Dutch Wechsler Intelligence Scale for Children - third edition (WISC-III-NL).