Neuropsychological profile of adult patients with Duchenne Muscular Dystrophy

NATALIA SIERRA; LILIA MESA; ALBERTO DUBROVSKY; PABLO SOJO; TERESA TORRALVA; MARÍA ROCA; FERNANDO CHLOACA; LAURA PIRRA; FACUNDO MANES

Toronto

Encuentro; 62nd AAN 2010 Annual Meeting; 2010

American Academy of Neurology

OBJECTIVE: To analyze the cognitive profile of adult patients with normal IQ diagnosed with Duchenne Muscular Dystrophy (DMD). BACKGROUND: Research on muscular dystrophies has focused extensively on the impact of peripheral neural structures affected by the degenerative nature of the disease. However, investigating the cognitive impairment in these patients may contribute to the understanding of the pathophysiological changes occurring on the central nervous system (CNS) and its relationship with peripheral structures. DESIGN/METHODS: Ten patients with diagnosis of DMD aged between 17 and 28 years were assessed with a general comprehensive cognitive battery as well as a specific executive battery. All patients had within normal IQ scores. RESULTS: Tasks associated with a motor component such as the Complex Rey Figure (z= -2,02) and TMT-A (z= -2,31) and TMT-B (z=-2,47) were impaired. Performance deficits were also found on tasks of verbal inhibitory control (z=-3,02) with normal scores on reading speed (z=-0,69) and color naming (z=-0,81) on the Stroop task. Decreased scores were observed for tasks of theory of mind (z=-1,19) and decision-making (Iowa Gambling Task), although performance on the latter did not correlate significantly with theory of mind or verbal inhibitory control scores (both p > .05). CONCLUSIONS/RELEVANCE: These results are in accordance with previous studied (e.g. Hinton et al, 2007) and suggest that normal IQ patients with DMD present theory of mind and verbal inhibitory control deficits. Our results also revealed decision-making impairments, although apparently not associated with theory of mind and executive deficits.