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Bedside Myotonia
Availability
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Classification
Supplemental - Highly Recommended: Myotonic Dystrophy (DM)
Recommended for QOL, functional status, therapy
Short Description of Instrument
Myotonia will be sought on physical examination using the following techniques: 1. Eye closure myotonia. The participant will be instructed to close the eyes tightly for 3 seconds, and then to open the eyes. This test will be performed 5 times. Eye closure myotonia is present if there is difficulty fully opening the eyelids. 2. Hand-grip myotonia. The participant will be instructed to forcefully close the fingers in a fist for 3 seconds, and then rapidly open the fist. This test will be performed 5 times. Hand-grip myotonia is present if the fingers cannot be immediately extended.
Comments/Special Instructions
Administration Time: 5 minutes
Scoring and Psychometric Properties
Scoring: Eye closure myotonia and hand-grip myotonia are scored by seconds before the subject can either fully open their eyes or their hand. 5 trials are performed.
 
Psychometric Properties: Not applicable.
References
Key References:
Trip J, Drost G, Ginjaar HB, Nieman FH, van der Kooi AJ, de Visser M, van Engelen BG, Faber CG. Redefining the clinical phenotypes of non-dystrophic myotonic syndromes. J Neurol Neurosurg Psychiatry. 2009 Jun;80(6):647-52.
 
Trip J, Drost G, van Engelen BG, Faber CG. Drug treatment for myotonia. Cochrane Database Syst Rev. 2006 Jan 25;2006(1):CD004762. Drug treatment for myotonia - PMC (nih.gov)
 
Additional References:
Heatwole CR, Statland JM, Logigian EL. The diagnosis and treatment of myotonic disorders. Muscle Nerve. 2013 May;47(5):632-48.
 
Johnson NE, Heatwole CR. Myotonic dystrophy: from bench to bedside. Semin Neurol. 2012 Jul;32(3):246-54.
 
Document last updated October 2024