Depression in Myotonic Dystrophy type 1: clinical and neuronal correlates
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RESEARCH
Depression in Myotonic Dystrophy type 1: clinical and neuronal correlates Research
Stefan Winblad*1,2, Christer Jensen3, Jan-Eric Månsson4, Lena Samuelsson5 and Christopher Lindberg1,4
Abstract Background: This study was designed to investigate the prevalence and correlates of depression in Myotonic dystrophy type 1 (DM1). Methods: Thirty-one patients with DM1 and 47 subjects in a clinical contrast group, consisting of other neuromuscular disorders, including Spinal muscular atrophy, Limb girdle muscle atrophy and Facioscapulohumeral dystrophy, completed Beck Depression Inventory (BDI). We aimed to establish whether different factors associated with DM1 correlated with ratings in the BDI. Results: Signs of a clinical depression were prevalent in 32% of the patients with DM1, which was comparable with ratings in the clinical contrast group. The depressive condition was mild to moderate in both groups. In DM1, a longer duration of clinical symptoms was associated with lower scores on the BDI and higher educational levels were correlated with higher scores on depression. We also found a negative association with brain white matter lesions. Conclusions: Findings indicate significantly more DM1 patients than normative collectives showing signs of a clinical depression. The depressive condition is however mild to moderate and data indicate that the need for intervention is at hand preferentially early during the disease process. Background Depression is an important health issue because of its high lifetime prevalence and association with substantial disability [1]. An increased risk of having major depression is associated with chronic disease, neurological and neuromuscular disorder and a comorbid state of depression has been found to incrementally worsen health [1-3]. Without treatment, depression has a tendency to assume a chronic course, be recurrent and over time to be associated with increasing disability [1]. Consequently, the possible comorbidity of depression with chronic disease is important to acknowledge. Myotonic dystrophy type 1 (DM1) is a progressive, dominantly inherited, multisystem disease caused by an expanded and unstable trinucleotide CTG repeat localized to the 3' untranslated region of the dystrophia myotonica protein kinase (DMPK) gene on chromosome 19q13.3 [4]. The expansion of CTG repeats causes muscle * Correspondence: [email protected] 1
Neuromuscular Centre, Department of Neurology, Sahlgrenska University Hospital, Göteborg, Sweden
wasting, myotonia, heart conduction defect, lens cataract, endocrine dysfunction and brain abnormalities through different molecular mechanisms [5]. A growing body of evidence shows that a RNA gain-of-function mechanism plays a major role in the disease development but the process explaining neurocognitive dysfunction is however more uncertain [6-8]. It is known that DM1 is a disorder associated with neuropsychological deficits, including reduced attention, speed, visuoconstructive and executive abilities [9]. Accompanying brain
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