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Myotonic dystrophy type 2 with focal asymmetric muscle weakness and no electrical myotonia
Author(s) -
Milone Margherita,
Batish Sat D.,
Daube Jasper R.
Publication year - 2009
Publication title -
muscle and nerve
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 1.025
H-Index - 145
eISSN - 1097-4598
pISSN - 0148-639X
DOI - 10.1002/mus.21150
Subject(s) - myotonic dystrophy , myotonia , muscle biopsy , weakness , electromyography , medicine , motor unit , myopathy , creatine kinase , muscle weakness , fibrillation , physical medicine and rehabilitation , proximal muscle weakness , muscular dystrophy , cardiology , anatomy , biopsy , atrial fibrillation
Genetically proven myotonic dystrophy type 2 (DM2) was found in a 61‐year‐old woman with creatine kinase (CK) elevation and only isolated weakness of one triceps. There was no clinical or electrical myotonia. Electromyography (EMG) showed only scattered fibrillation potentials and short duration motor unit potentials. Muscle biopsy showed nonspecific myopathic features and highly atrophic fibers with nuclear clumps. DM2 should be considered in patients with focal proximal weakness and abnormal EMG without myotonic discharges. Muscle Nerve 39: 383–385, 2009