Ausbildung und berufliche Qualifikation von Erwachsenen mit Myotonen Dystrophien – eine fehlgeleitete Wahrnehmung durch die Facies myopathica?

Stahl, K.; Wenninger, S.; Schüller, A.; Montagnese, F. und Schoser, B. (2016): Ausbildung und berufliche Qualifikation von Erwachsenen mit Myotonen Dystrophien – eine fehlgeleitete Wahrnehmung durch die Facies myopathica? In: Fortschritte der Neurologie, Psychiatrie, Bd. 84, Nr. 4: S. 211-216

Abstract

Background: Myotonic dystrophies types 1 and 2 (DM1/DM2) are the most frequent inherited progressive, segmental progeroid, multisystemic neuromuscular diseases in adulthood. The executive impairment is one of the key disease features. The myopathic face triggers the general perception of DM1 patients being associated with a low educational level. Methods: We used a standardized questionnaire to evaluate educational levels in adults with genetically confirmed DM1 and DM2 in comparison to data of the general population. Investigated topics included the level of education, e.g. the highest university degree aquired. Results: Out of a total cohort of 546 DMpatients, 125 DM1 and 156 DM2 patients (51%) participated in this study. There was no statistically significant difference between the two collectives as far as high school levels are concerned. 50.4% of DM1 and 48.3% of DM2 patients obtained the higher education entrance qualification compared to 29.6% of the normal German population. However, there were significant differences between the two collectives in spelling problems (DM1 cohort: p=0.039), difficulty in mental arithmetic (p=0.043), and classification of patients with learning difficulties (p=0.012). Discussion: Misled by a myopathic face, many physicians associate myotonic dystrophy with cognitive deficiency. Based on our study, the minimal deviation between DM1 and DM2 and the normal German population indicates that the multisystemic disease does not significantly influence the maximum attainable level of education in adults with DM1. Conclusion: In summary, physicians should be aware that the general educational levels are rather normal in patients with myotonic dystrophy type 1 and rethink their perception of DM1 patients.

• Dokumententyp: Zeitschriftenartikel
• Fakultät: Medizin
• Themengebiete: 600 Technik, Medizin, angewandte Wissenschaften > 610 Medizin und Gesundheit
• ISSN: 0720-4299
• Sprache: Deutsch