Premium
Myotonic Dystrophies type 1 and 2: anesthetic care
Author(s) -
Veyckemans Francis,
Scholtes JeanLouis
Publication year - 2013
Publication title -
pediatric anesthesia
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 0.704
H-Index - 82
eISSN - 1460-9592
pISSN - 1155-5645
DOI - 10.1111/pan.12120
Subject(s) - myotonic dystrophy , medicine , myotonia , disease , anesthetic , anesthesia
Summary Myotonic dystrophy is classified as one of the myotonic syndromes although myotonia is only a minor characteristic of it. It is, in fact, also a multisystem disease with cardiac, digestive, ocular, and endocrine abnormalities. Two subgroups are currently identified with many similarities: DM 1 refers to classic dystrophia myotonica (Steinert disease), while DM 2, formerly called proximal myotonic myopathy has a later onset. The congenital form is present only in DM 1. The genetic causes of DM 1 and 2 are different but end up in a similar way of altering RNA m processing and splicing of other genes. The anesthetic risk is increased in case of DM 1 type. This review summarizes current knowledge concerning the pathophysiology and anesthetic management of this disease in children and adults.