Intellectual Functions and Personality in Subjects with Noncongenital Myotonic Muscular Dystrophy

Abstract

Mental retardation and personality disorders are commonly described among the symptoms of myotonic dystrophy. Nevertheless, this tendency is not supported by systematic studies performed on large samples, whose results are controversial. We studied the cognitive functions and personalities of a group of 28 patients, in whom myotonic dystrophy had commenced in juvenile or adult life. The severity of the disease was variable, but all subjects were self-sufficient. Only 7.1% of subjects showed low intelligence with deterioration of perceptual-motor functions. This was not correlated with the severity of their disease. Women had a substantially lower mean Wechsler-Bellevue score than men. The personality function study of the entire group showed no change of psychiatric relevance but did present a depressive attitude with marked somatic concern and difficulties in establishing relationships in social life.

Psychological characteristics of patients with myotonic dystrophy type 1

“…The fact that no specific pattern of psychological characteristic was found in the entire DM1 patient group contrasts previous reports . This may be due to the fact that the present study assessed a large heterogeneous cohort which consequently hid individual variability.…”

“…While behavioral particularities in DM1 were first described by Steinert at the beginning of the 20th century, it is only in the 1980s that personality characteristics became a matter of interest. Previous studies reported heterogeneous results; some reported avoidant or schizotypal personality traits, whereas others mainly noted bodily concerns such as somatization and hypochondria bringing patients to seek medical attention . Moreover, personality disorders have been reported in 20–60% of patients with DM1 .…”

“…Moreover, personality disorders have been reported in 20–60% of patients with DM1 . Depressive symptoms, such as low self‐esteem and motivation, are also encountered . In clinical settings, disturbed personality and psychosocial adjustment may represent a burden as they can lead to unsuccessful therapeutic compliance, in addition to limiting everyday functioning and quality of life of patients .…”

“…Most previous studies had small sample sizes, potentially limiting the relevance of their findings . Some exclusively considered individuals with mild muscular involvement or lower repeat sizes , while others did not take disease severity into account .…”

Cerebral involvement in myotonic dystrophies

“…Whether patients with DM1 have specific personality patterns is debatable. 7,12,26,33,72 It is common clinical experience that patients with DM1 have characteristic personalities: they tend to be either obsessive in their healthrelated care, continuously consulting their referring physician, or avoidant and passive in their attitudes toward health care. In an initial study by Bird et al, 7 one third of a small series of patients had prominent abnormalities, but these were considered to be the natural consequence of their motor and cognitive impairment.…”