Myotonic dystrophy type 2 with focal asymmetric muscle weakness and no electrical myotonia

Margherita Milone; Sat D. Batish; Jasper R. Daube

doi:10.1002/mus.21150

Myotonic dystrophy type 2 with focal asymmetric muscle weakness and no electrical myotonia

Margherita Milone, Sat D. Batish, Jasper R. Daube

Neurology

Research output: Contribution to journal › Article › peer-review

17 Scopus citations

Abstract

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.

Original language	English (US)
Pages (from-to)	383-385
Number of pages	3
Journal	Muscle and Nerve
Volume	39
Issue number	3
DOIs	https://doi.org/10.1002/mus.21150
State	Published - Mar 2009

Keywords

Focal weakness
Myotonia
Myotonic dystrophy type 2

ASJC Scopus subject areas

Physiology
Clinical Neurology
Cellular and Molecular Neuroscience
Physiology (medical)

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10.1002/mus.21150

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abstract = "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.",

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N2 - 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.

AB - 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.

KW - Focal weakness

KW - Myotonia

KW - Myotonic dystrophy type 2

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Myotonic dystrophy type 2 with focal asymmetric muscle weakness and no electrical myotonia

Abstract

Keywords

ASJC Scopus subject areas

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