Cognitive functioning and autonomy of patients with Duchenne muscular dystrophy

Paulina Anikiej , Arkadiusz Mański , Mariola Bidzan

Abstract

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.
Autor Paulina Anikiej (WNS / IPsych / ZPKiN)
Paulina Anikiej
- Zakład Psychologii Klinicznej i Neuropsychologii
, Arkadiusz Mański (WNS / IPsych / ZPKiN)
Arkadiusz Mański
- Zakład Psychologii Klinicznej i Neuropsychologii
, Mariola Bidzan (WNS / IPsych / ZPKiN)
Mariola Bidzan
- Zakład Psychologii Klinicznej i Neuropsychologii
Tytuł czasopisma/seriiActa Neuropsychologica, ISSN 1730-7503, e-ISSN 2084-4298, (B 12 pkt)
Rok wydania2018
Tom16
Nr2
Paginacja157-166
Objętość publikacji w arkuszach wydawniczych0.5
Słowa kluczowe w języku angielskimrare disease, attention, memory, executive functions
Klasyfikacja ASJC3202 Applied Psychology; 3206 Neuropsychology and Physiological Psychology
URL https://actaneuropsychologica.com/resources/html/article/details?id=170922
Języken angielski
LicencjaCzasopismo (tylko dla artykułów); licence.documentVersion.FINAL_PUBLISHED; Inna otwarta licencja; w dniu opublikowania
Punktacja (całkowita)12
PunktacjaPunktacja MNiSW = 12.0, 24-07-2019, ArticleFromJournal
Wskaźniki publikacji Scopus SNIP (Source Normalised Impact per Paper): 2017 = 0.513
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