Cognitive functioning and autonomy of patients with Duchenne muscular dystrophy

Paulina Anikiej , Arkadiusz Mański , Mariola Bidzan


Background: Cognitive problems and a deepening dependence on one’s immediate environment inherently accompany Duchenne Muscular Dystrophy (DMD). The disease is progressive, and the size of the dystrophin gene determines the extraordinary complexity of the causes of this disease at the genetic and molecular level. The aim of the study was to characterize the cognitive problems and the extent of independence of patients with genetically confirmed DMD. An attempt was also made to reconstruct the patient's life history in three periods: before the appearance of the first symptoms, during the search for a diagnosis and after confirming the diagnosis of DMD. Methods: The study group consisted of male patients between 10 and 13 years of age (N = 14). The Diagnosis of Cognitive Functions Battery - PU1 and an experimental tool for studying autonomy were used. Information on the condition of the attention, memory and executive functions of patients was obtained. The study of autonomy measurably supplemented knowledge in terms of the degree of the patients’ dependence on the environment with regard to everyday functioning. Results: The best functioning component of attention in the examined patients is orientation (o) (13 patients achieved average results in this aspect). Selectivity (s) turned out to be the weakest component, as only five patients obtained average results (the others obtained low results) in this aspect. Autonomy results (AU) indicate group diversity and inter-subject variability in the disease progression (subjects scored from 6 to 47 points). Conclusions: The clinical picture of the disease is not homogeneous. Patients, despite their similar age, differ in the progression of the disease and the resulting effects. This induces the need for an individual approach to each patient and the preparation of a unique set of therapeutic interactions for each of them.
Author Paulina Anikiej (FSS / IPsych / DCPN)
Paulina Anikiej,,
- Division of Clinical Psychology and Neuropsychology
, Arkadiusz Mański (FSS / IPsych / DCPN)
Arkadiusz Mański,,
- Division of Clinical Psychology and Neuropsychology
, Mariola Bidzan (FSS / IPsych / DCPN)
Mariola Bidzan,,
- Division of Clinical Psychology and Neuropsychology
Journal seriesActa Neuropsychologica, ISSN 1730-7503, e-ISSN 2084-4298, (B 12 pkt)
Issue year2018
Publication size in sheets0.5
Keywords in Englishrare disease, attention, memory, executive functions
ASJC Classification3202 Applied Psychology; 3206 Neuropsychology and Physiological Psychology
Languageen angielski
LicenseJournal (articles only); published final; Other open licence; with publication
Score (nominal)12
Score sourcejournalList
ScoreMinisterial score = 12.0, 29-01-2020, ArticleFromJournal
Publication indicators WoS Citations = 0; Scopus SNIP (Source Normalised Impact per Paper): 2018 = 0.448
Citation count*
Share Share

Get link to the record

* presented citation count is obtained through Internet information analysis and it is close to the number calculated by the Publish or Perish system.
Are you sure?