[Cognitive impairment in myotonic dystrophy type 1 (Steinert's disease)]

Abstract

Traditionally, it has been recognized that patients with myotonic dystrophy type 1 (MD-1) - also known as Steinert disease -, they show a specific behaviour, not including those who suffer from mental or neurodevelopmental diseases. The neurological substrate of this behaviour is described. The aim of this text has two purposes. The first intention is that clinical staff, when faced with a patient with MD-1, always consider the cognitive aspects of the disease. On the other hand, it is intended to combat preconceived ideas about the particular behaviour of these patients.

Keywords: Anosognosia; Cognitive functioning; Disexecutive syndrome; Distrofia miotónica tipo 1; Educative level; Enfermedad de Steinert; Funcionamiento cognitivo; Myotonic dystrophy type 1; Nivel educativo; Steinert's disease; Síndrome disejecutivo.