Rosado Bartolomé A, Puertas Martín V, Domínguez González C, Ramos Miranda M
Medicina de Familia y Comunitaria, Grupo de Trabajo de Neurología SEMERGEN, Centro de Salud Mar Báltico, Madrid, España.
Facultad de Educación, Universidad Internacional de la Rioja (UNIR), Logroño, La Rioja, España.
Semergen. 2022 Apr;48(3):208-213. doi: 10.1016/j.semerg.2022.01.013. Epub 2022 Mar 12.
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.
传统上,人们已经认识到1型强直性肌营养不良症(MD-1)患者——也被称为斯坦纳特病——会表现出一种特定行为,但不包括患有精神或神经发育疾病的患者。文中描述了这种行为的神经学基础。本文的目的有两个。第一个目的是让临床工作人员在面对MD-1患者时,始终考虑到该疾病的认知方面。另一方面,旨在消除对这些患者特定行为的先入之见。