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Neurology. 2013 Jun 11; 80(24): e251.
PMCID: PMC3721102
PMID: 23751923

Teaching Video NeuroImages: Trapezius myotonia percussion sign in myotonic dystrophy type 2

Associated Data

Supplementary Materials

Abstract

Myotonic dystrophy type 2 (DM2) is an autosomal dominant disorder with proximal weakness, muscle pain, and early-onset cataracts.1 In comparison with myotonic dystrophy type 1 (DM1), myotonia is less symptomatic, more proximal, and harder to detect during clinical and electrodiagnostic testing.2 Here we document the presence of trapezius myotonia in patients with DM2 (video on the Neurology® Web site at www.neurology.org). In our experience, similar proximal percussion does not produce as marked a response in DM1 or nondystrophic myotonic disorders. This sign demonstrates a mechanism to test for proximal myotonia, and in at-risk patients, may be suggestive of an underlying diagnosis of DM2.

Myotonic dystrophy type 2 (DM2) is an autosomal dominant disorder with proximal weakness, muscle pain, and early-onset cataracts.1 In comparison with myotonic dystrophy type 1 (DM1), myotonia is less symptomatic, more proximal, and harder to detect during clinical and electrodiagnostic testing.2 Here we document the presence of trapezius myotonia in patients with DM2 (video on the Neurology® Web site at www.neurology.org). In our experience, similar proximal percussion does not produce as marked a response in DM1 or nondystrophic myotonic disorders. This sign demonstrates a mechanism to test for proximal myotonia, and in at-risk patients may be suggestive of an underlying diagnosis of DM2.

Supplementary Material

Footnotes

Supplemental data at www.neurology.org

AUTHOR CONTRIBUTIONS

Nicholas E. Johnson: drafting manuscript, data collection. Chad R. Heatwole: revision of manuscript, design of project.

STUDY FUNDING

No targeted funding reported.

DISCLOSURE

N. Johnson reports no disclosures. C. Heatwole receives support through the National Institute of Arthritis and Musculoskeletal and Skin Disorders (1K23AR055947). Go to Neurology.org for full disclosures.

REFERENCES

1. Day JW, Ricker K, Jacobsen JF, et al. Myotonic dystrophy type 2: molecular, diagnostic and clinical spectrum. Neurology 2003;60:657–664 [PubMed] [Google Scholar]
2. Logigian EL, Ciafaloni E, Quinn LC, et al. Severity, type, and distribution of myotonic discharges are different in type 1 and type 2 myotonic dystrophy. Muscle Nerve 2007;35:479–485 [PubMed] [Google Scholar]

Articles from Neurology are provided here courtesy of American Academy of Neurology