Illness-associated muscle weakness in dystroglycanopathies.

ABSTRACT:

Objective

To describe the phenomenon of acute illness-associated weakness (AIAW) in patients with dystroglycanopathy (DG), determine the frequency of this phenomenon in DGs, and compare it to the frequency in Duchenne-Becker muscular dystrophy (DBMD).

Methods

Patients enrolled in a DG natural history study provided medical history, including major illnesses or hospitalizations, at enrollment and annually. We noted a recurring syndrome of profound transient weakness in the setting of febrile illness. To determine the frequency of this phenomenon in the DG cohort and compare it to a cohort with another membrane-related muscular dystrophy, DBMD, we surveyed patients (e-survey tool), collecting demographics and information about episodes of sudden progression of weakness and eve

SUBMITTER: Carlson CR 

PROVIDER: S-EPMC5719925 | biostudies-literature | 2017 Dec

REPOSITORIES: biostudies-literature