Cognitive and psychological profile of a Tunisian population of limb girdle muscular dystrophy

This study assesses the cognitive functioning of a Tunisian population suff ering from limb girdle muscular dystrophy (LGMD). The population was random ly selected from patients referred to the outpatient clinic of the National Institute of Neurology of Tunis. The only criterion for inclusion was the absolute proof of this type of disease through clinical assessments, an imm unohistochemical muscle study (dystrophin positive) and linkage study. Over a 2-year period, 16 cases were identified. Cognitive function was measured by individual IQ testing using a language-free, motor-reduced and cultural -free intelligence test. This test relies on adequate visual discrimination and spatial relationship skills; therefore, a test to appraise perceptual skills was administered to rule out potential deficiencies in these abiliti es. The results showed that this population was functioning well within nor mal limits of cognitive abilities (IQ of 102 +/- 3 mean +/- SEM). Additiona lly, the study looked upon the emotional adjustment of this population. Fou r projective cards were administered (eliciting themes of family confrontat ion, school attitude, dependence/anxiety and conflicts with parents/depress ion), Clinical indicators were recorded for each card. The findings were co mpared with a similar population in terms of demographic characteristics an d referred to an outpatient psychology clinic for emotional and behavioral difficulties. The results showed that the LGMD population had a higher numb er of clinical indicators and a specific profile characterized by low self- esteem with feelings of sadness and internalized culpability. (C) 1999 Else vier Science B.V. All rights reserved.