Depression in Myotonic Dystrophy type 1: clinical and neuronal correlates

Abstract

BackgroundThis study was designed to investigate the prevalence and correlates of depression in Myotonic dystrophy type 1 (DM1).MethodsThirty-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.ResultsSigns 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.ConclusionsFindings 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.

Evaluation of CNS involvement in myotonic dystrophy type 1 and type 2 by transcranial sonography

“…Such white and grey matter alterations do not seem to be depictable by means of transcranial sonography. On the other hand, clinical symptoms and signs seem to indicate preferential involvement of distinct regions, since attention deficits, daytime sleepiness, depression and restless legs are common features in DM1 and DM2 [12,13,32]. With regard to the correlation of brain morphological alterations to these CNS syndromes, MRI studies revealed inconsistent findings [1][2][3].…”

“…Depressive symptoms are frequent in DMs [1,12]. The cause of depression in DMs is still unclear, as some authors explain depression as a psychological reaction to the chronic disease, and others as a direct consequence of structural CNS alterations [41,42].…”

“…This is of particular interest since different features like depression, excessive daytime sleepiness (EDS) and RLS occur frequently in DM 1 and also in DM2 [11][12][13]. In DM1, alterations of hypocretin release or missplicing of the hypocretin receptor 1 could be excluded and the pathophysiology of EDS is unclear so far [14].Interestingly, in an immunhistochemical study loss of serotonin-containing neurons in the raphe nucleus of DM-patients could be related to hypersomnia [15].…”

“…TCS has become a reliable and sensitive diagnostic tool in the evaluation of extrapyramidal movement disorders, ataxias, and in many neurological disorders with psychiatric symptoms as well as in restless legs syndrome (RLS) [6][7][8][9][10][11][12][13][14][15][16][17][18][19]. This is of particular interest since different features like depression, excessive daytime sleepiness (EDS) and RLS occur frequently in DM 1 and also in DM2 [11][12][13].…”

The use of an online support group for neuromuscular disorders: a thematic analysis of message postings

“…(10) For others, personal factors such as denying their symptoms or feeling too proud to seek medical advice influenced their delay in getting their symptoms checked out: I am a relatively private person when it comes to medical problems so I didn't really say to anyone about it. (11) Delays in having symptoms recognised or taken seriously by medical professionals and in accessing specialist referrals were also commonly discussed:…”