Introduction:

One third of children with Duchenne muscular dystrophy (DMD) have IQ (Intelligence Quotient) below 70, with a specific profile of cognitive affection and behavioural problems. There is paucity of reports from developing countries.

Objectives:

Assessment of cognitive and behavioural impairment in DMD children and its predictors and relevance.

Materials and methods:

Steroid naive DMD children were assessed by Malins Intelligence Scale (Indian adaptation of Weschlers intelligence scale) and also underwent MRI Brain with MRS and EEG.

Results and discussion:

Twenty children fulfilling inclusion and exclusion were enrolled between March 2010 to February 2012. Mean age at enrolment was 9.52 years. The mean IQ was 78.3 (SD - 9.01) which is statistically significant compared to population mean (p < 0.001).  Twenty percent had mild mental retardation, 50% had borderline intelligence and 30% had average intelligence (DSM IV criteria). Mean performance IQ was higher than verbal IQ. MRS showed reduced NAA in cerebellum which has not been described previously in literature. There is also increase in the incidence of cognitive impairment in children with deletion towards 3’ end of the gene. There was more than expected association of behavioural problems, learning difficulties, sleep disorders and EEG abnormalities in children with DMD.

Conclusion:

DMD children have high incidence of cognitive and behavioural impairment which is under recognized and has specific profile of affection. Cognitive assessment and therapy needs to be a part of integral approach in management.  This area needs further research to elucidate the pathophysiology of the disease.