Clinical Guide for the Diagnosis and Follow-up of Myotonic Dystrophy Type 1, MD1 or Steinert’s Disease
Neurología
Gutiérrez Gutiérrez, G., Díaz-Manera, J., et al. (2020).
Neurología, 35(3), 185-206.
This guideline provides recommendations on the diagnosis and management of myotonic dystrophy type 1 (MD1) or Steinert's disease. This summary highlights recommendations relevant to an SLP's scope of practice.
Sociedad Española de Neurología (Spain)
Spanish Society of Endocrinology and Nutrition (SEEN, Spain); Familial Cardiomyopathies Group of the Spanish Society of Cardiology (SEC, Spain)
Recommendations regarding the assessment of dysphagia in patients with MD1 or Steinert's disease are as follows:<br />
<ul>
<li>Patients should be monitored for symptoms of dysphagia to solids, liquids, or mixed textures at least once per year. </li>
<li>The 10-item Eating Assessment Tool (EAT-10) should be administered annually.</li>
<li>Patients scoring a 3 or more on the EAT-10 should also undergo a volumeviscosity swallow test, fiber-optic endoscopic evaluation of swallowing, or video fluoroscopy, depending on the equipment available.</li>
</ul>
Recommendations regarding the treatment of dysphagia in patients with MD1 or Steinert's disease are as follows:<br />
<ul>
<li>If indicated, thickeners should be used if the ability to swallow liquids is affected; and mixtures of different textures should be avoided.</li>
<li>Soft or blended foods should be given if the ability to swallow solid food is affected. Patients should avoid the foods that present the greatest risk to them. "Guided physiotherapy with a speech therapist may improve this symptom" (p. 198).</li>
<li>"As in other neurological diseases or cases of dysphagia, a nasogastric tube or gastrostomy (depending on disease progression time) are advised in cases of severe dysphagia in which it is not possible to implement specific recommendations" (p. 198).</li>
</ul>
