Résumé :
|
OBJECTIVES: The aim of this study was to identify apathy levels, and to discuss the association of this condition with mood disturbances and neuropsychological manifestations in patients with Myotonic Dystrophy Type-1 (DM1).METHOD: we assessed 37 adults with juvenile (n=20) and "adult-classic" form (n=17) of DM1, 19 patients with Facio-scapulo-humeral dystrophy (FSHD) and 20 healthy people on their functional abilities (Boston functional scale), apathy (LARS), depression (MINI, MADRS), global cognitive status (MMSE) and executive functions (BREF). Healthy subjects were matched with DM1 subjects on age and scholarship level; whereas patients with FSHD were significantly older (p<0.05) and more disabled (p<0.05) than the DM1 patients group. MAIN RESULTS: MMSE mean scores were significantly decreased in DM1 (m=27.63) compared to FSHD (m=28.9; p<0.05) and healthy subjects (m=29.3; p<0.001). Global scores of apathy were significantly higher in DM1 than in FSHD (p<0.01) and in the healthy people group (p<0.0001). In the DM1 patients group: 15/37 (41%) patients met the criteria of significant apathy; 11/37 (30%) showed a tendency to apathy, and 11/37 (30%) had no apathy. 9/37 (24%) DM1 patients met the criteria of a current major depressive syndrome (MDS); 6/37 (16%) were both apathetic and depressed. There was no statistical correlation between apathy and depression scores, whereas there were significant correlations between apathy and global intellectual status (MMSE scores, p= 0.028), as well as with verbal fluencies (BREF subtest) scores (p= 0.009). CONCLUSION: in DM1, apathy is highly prevalent and independent from depression. These results suggest that a systematic evaluation of apathy in DM1 would be helpful in order to distinguish apathy from depression and, thus, to better understand the lack of motivation and initiative seen in many adult DM1 patients. Furthermore, the correlations with neuropsychological tests suggest the involvement of the central nervous system in the aetiology of apathy in DM1. Further researches (including cerebral imagery) could be necessary to demonstrate this hypothesis.
|