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

This study assesses the cognitive functioning of a Tunisian population suffering from limb girdle muscular dystrophy (LGMD). The population was randomly 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 immunohistochemical 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 abilities. The results showed that this population was functioning well within normal limits of cognitive abilities (IQ of 102 +/- 3 mean +/- SEM). Additionally, the study looked upon the emotional adjustment of this population. Four projective cards were administered (eliciting themes of family confrontation, school attitude, dependence/anxiety and conflicts with parents/depression). Clinical indicators were recorded for each card. The findings were compared with a similar population in terms of demographic characteristics and referred to an outpatient psychology clinic for emotional and behavioral difficulties. The results showed that the LGMD population had a higher number of clinical indicators and a specific profile characterized by low self-esteem with feelings of sadness and internalized culpability.