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Egen Klassifikation Scale Version 2 (EK2)
Freely available but must contact creator/copyright holder: Birgit F Steffersen at
Supplemental - Highly recommended: Congenital Muscular Dystrophy specifically for studies involving adolescents and adults with CMD.
Short Description of Instrument
The Egen Klassifikation (EK) scale was designed to measure functional ability in non-ambulant DMD. It examined activities and abilities such as transfers, trunk mobility, wheelchair use, bed mobility, cough, well-being, and the original scale of 1–10 items showed good validity and reliability using traditional methods of psychometric analysis. The scale was extended in 2008 by adding seven items to capture functional ability specifically related to feeding, bulbar issues and distal hand function. At the same time items 1–10 were revised to update the scale in relation to advances in equipment and respiratory management.
Construct measured: Activities of daily living - measure of functional ability for non ambulatory individuals
Disease specific measure for non ambulatory individuals with DMD and SMA. Used to plan & evaluate effect of interventions.
Questionnaire of 10 questions, each has 4 responses on level of difficulty in performing a task. Questions assess patient's ability to use wheelchair, transfer from wheelchair, ability to stand, balance on wheelchair, move arms, use hands/arms for eating, turn in bed, cough, and speak, as well as their physical well being.
Score: 0 is better; 30 is worse.
Equipment needs: Pen and paper only.
Time to administer: Approximately 10 minutes.
Validated in English
EK of 21 or higher predicted high risk for introduction to noninvasive ventilation in DMD patients.
Limitation: not validated for CMD
Advantage: may still be applicable to non-ambulatory patients.
Brunherotti MA, Sobreira C, Rodrigues-Junior AL, de Assis MR, Terra Filho J, & Baddini Martinez JA. (2007). Correlations of Egen Klassifikation and Barthel Index scores with pulmonary function parameters in Duchenne muscular dystrophy. Heart Lung, 36(2), 132-139.
Connolly AM, Malkus EC, Mendell JR, Flanigan KM, Mille, JP, Schierbecker JR, . . . Florence JM. (2015). Outcome reliability in non-Ambulatory Boys/Men with duchenne muscular dystrophy. Muscle Nerve, 51(4), 522-532.
Jeppesen J, Green A, Steffensen BF, Rahbek J. (2003). The Duchenne muscular dystrophy population in Denmark, 1977-2001: prevalence, incidence and survival in relation to the introduction of ventilator use. Neuromuscul Disord, 13(10), 804-812.
Mayhew AG, Cano SJ, Scott E, Eagle M, Bushby K, Manzur A, Muntoni F. (2013). Detecting meaningful change using the North Star Ambulatory Assessment in Duchenne muscular dystrophy. Dev Med Child Neurol, 55(11), 1046-1052.
Mayhew AG, Eagle M, Steffenson B. (2012). 17th International Congress of The World Muscle Society: S.P.6 Exploratory Rasch analysis of the EK2 scale used in a population of Duchenne muscular dystrophy (DMD). Neuromusc Disorders, 22(9-10), 877.
Rahbek J, Werge B, Madsen A, Marquardt J, Steffensen BF, Jeppesen J. (2005). Adult life with Duchenne muscular dystrophy: observations among an emerging and unforeseen patient population. Pediatr Rehabil, 8(1), 17-28.
Steffensen B, Hyde S, Lyager S, Mattsson E. (2001). Validity of the EK scale: a functional assessment of non-ambulatory individuals with Duchenne muscular dystrophy or spinal muscular atrophy. Physiother Res Int, 6(3), 119-134.
Steffensen BF, Lyager S, Werge B, Rahbek J, Mattsson E. (2002). Physical capacity in non-ambulatory people with Duchenne muscular dystrophy or spinal muscular atrophy: a longitudinal study. Dev Med Child Neurol, 44(9), 623-632.
Werlauff U, Fynbo Steffensen B. (2014). The applicability of four clinical methods to evaluate arm and hand function in all stages of spinal muscular atrophy type II. Disabil Rehabil, 36(25), 2120-2126.
Werlauff U, Vissing J, Steffensen BF. (2012). Change in muscle strength over time in spinal muscular atrophy types II and III. A long-term follow-up study. Neuromuscul Disord, 22(12), 1069-1074.


Document last updated July 2018