Psychopathological and emotional deficits in myotonic dystrophy

OBJECTIVE—To evaluatepsychopathological disturbances in patients with myotonic dystrophy(MD) and compare patients with MD to both patients withfacioscapulohumeral dystrophy (FSHD) and healthy control subjects.
METHODS—Asemistructured interview was used to determine DSM III-R criteria formajor depressive episodes, dysthymic episodes, and generalised anxiety.The Montgomery and Asberg and the Hamilton depressive scales, the Coviand Tyrer anxiety scales, the Abrams and Taylor scale for emotionalblunting, and the depressive mood scale were all used in the study.Subjects were also asked to complete questionnaires for physical andsocial anhedonia.
RESULTS—Fifteenpatients with MD, 11 patients with FSHD, and 14 healthy subjects werestudied. Patients with MD were not more depressed or anxious thanhealthy controls. Patients with FSHD were the most depressed and mostanxious. However, patients with MD had significantly lower scores forexpressiveness and significantly higher scores for anhedonia than theother two groups.
CONCLUSION—Patientswith MD did not present significant depressive or anxioussymptomatology but rather an emotional deficit. This emotional deficitmay be an adaptive reaction to the threatening implications of thedisease, or the effect of the CNS lesions which occur with MD, or both.