The findings of this study suggest that the personality pattern of patients with DM is related to their disease. Their personality disorders are not attributable to their adjustment to a disabling condition. By contrast, among the patients with DM, the high incidence of avoidant personality, a phenotype poorly represented in the general population, supports the idea that it is the expression of a primary phenomenon related to a genetic mutation.

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Objective-To evaluate psychopathological disturbances in patients with myotonic dystrophy (MD) and compare patients with MD to both patients with facioscapulohumeral dystrophy (FSHD) and healthy control subjects. Methods-A semistructured interview was used to determine DSM III-R criteria for major depressive episodes, dysthymic episodes, and generalised anxiety. The Montgomery and Asberg and the Hamilton depressive scales, the Covi and Tyrer anxiety scales, the Abrams and Taylor scale for emotional blunting, and the depressive mood scale were all used in the study. Subjects were also asked to complete questionnaires for physical and social anhedonia. Results-Fifteen patients with MD, 11 patients with FSHD, and 14 healthy subjects were studied. Patients with MD were not more depressed or anxious than healthy controls. Patients with FSHD were the most depressed and most anxious. However, patients with MD had significantly lower scores for expressiveness and significantly higher scores for anhedonia than the other two groups. Conclusion-Patients with MD did not present significant depressive or anxious symptomatology but rather an emotional deficit. This emotional deficit may be an adaptive reaction to the threatening implications of the disease, or the eVect of the CNS lesions which occur with MD, or both. (J Neurol Neurosurg Psychiatry 1998;65:353-356)