Evaluation of narrative abilities in patients suffering from Duchenne Muscular Dystrophy.
The present work investigated cognitive, linguistic and narrative abilities in a group of children suffering from Duchenne Muscular Dystrophy, an allelic X-linked recessive disorder caused by mutations in the gene encoding dystrophin. The patients showed mildly reduced IQ with lower Verbal than Performance Intelligence Quotient and were mildly affected in visual attention and short-term memory processing. At the linguistic assessment, neither receptive (word comprehension) nor expressive (naming tasks and fluency) lexical abilities were impaired. However, their narratives were qualitatively inferior with respect to those produced by a group of typically developing children. Their speech samples were characterized by the presence of fewer verbs and complete sentences. It is suggested that the reduced production of complete sentences is due to a selective problem in verb argument structure generation. Since the lack of dystrophin is assumed to produce effects on the maturation of the cerebellum, whose involvement has been recently suggested in verb and syntactic processing, these findings may lend indirect support to the hypothesis of a cerebellar-cortical circuit specialized in verb and sentence production.
Marini
A
A
University of Udine, Udine, Italy. andrea.marini@uniud.it
Lorusso
M L
ML
D'Angelo
M G
MG
Civati
F
F
Turconi
A C
AC
Fabbro
F
F
Bresolin
N
N
D002648:Child; D003072:Cognition Disorders / Q000209:etiology; D005260:Female; D006801:Humans; D007360:Intelligence; D007361:Intelligence Tests; D007802:Language; D007806:Language Disorders / Q000209:etiology*; D008297:Male; D020388:Muscular Dystrophy, Duchenne / Q000150:complications*; D009483:Neuropsychological Tests