Psychopathological and emotional deficits in myotonic dystrophy

Abstract

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)

Brain 1H magnetic resonance spectroscopic differences in myotonic dystrophy type 2 and type 1

“…Ratings of depression (Beck Depression Inventory Score) 4 were not significantly different between the three groups, which accords with earlier studies of DM1 patients. 5 All DM1 and DM2 patients had percussion or grip myotonia and showed myotonic discharges in the electromyogram. DM2 patients especially complained of pain and fluctuating stiffness, and had a moderate degree of muscle impairment, which was not significantly different from the DM1 patients: MRC megascore 139 Ϯ 5 vs. 140 Ϯ 5, respectively.…”

Influence of Relatives on Fatigue Experienced by Patients with Facioscapulohumeral Dystrophy, Myotonic Dystrophy and HMSN-I

“…In spouses living together we found more disagreement in marital satisfaction within MD couples than within FSHD and HMSN couples. Possibly, disease-specific problems associated with a multisystem disorder like MD, such as apathy, lack of expressiveness, and inability to anticipate pleasure, contribute to more marital dissatisfaction [19, 20]. We found that the relatives of patients with MD had difficulty in estimating the level of fatigue experienced by the patient.…”

Retentissement des troubles cognitifs sur la vie sociale, professionnelle et émotionnelle des patients dans la forme adulte de la dystrophie myotonique de type I

“…Il a d'ailleurs été montré que les patients atteints de DM1 présentent un profil de personnalité spécifique caractérisé par une baisse de l'expressivité émotionnelle. Ce déficit émotionnel peut être consécutif à une réaction d'adaptation à la maladie et/ou au retentissement de l'atteinte des fonctions cognitives [8].…”