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Neuromuscul Disord. 2018 Dec;28(12):996-1002. doi: 10.1016/j.nmd.2018.09.007. Epub 2018 Sep 27.

Isokinetic assessment of trunk muscles in facioscapulohumeral muscular dystrophy type 1 patients.

Author information

1
Hôpital Rothschild, Service de Reeducation Neuro-orthopédique, 5 Rue Santerre 75012 Paris, France. Electronic address: mpr.julien.esnault@gmail.com.
2
Hôpital Rothschild, Service de Reeducation Neuro-orthopédique, 5 Rue Santerre 75012 Paris, France.
3
Hôpital Pitié-Salpêtrière, Institut de Myologie, 47-83 Boulevard de l'Hôpital 75013 Paris, France.

Abstract

Facioscapulohumeral muscular dystrophy type 1 is the third most common inherited myopathy. Its severity is proportionate to the loss of microsatellite D4Z4 repetitions, which are below 10. Patients suffer from weakness in facial muscles, shoulder girdles and ankle dorsiflexors. Trunk impairment is reported in few studies. To assess correlation between D4Z4 number of repetitions in facioscapulohumeral muscular dystrophy type 1 patients and trunk extensors and flexors isokinetic peak torque, 48 patients with southern Blot confirmed facioscapulohumeral muscular dystrophy type 1 were enrolled to perform clinical evaluation (Ricci's Clinical Severity Scoring, Berg Balance Scale, Functional Reach Test, timed up-and-go test, six-minute walk test, functional independence measure) and trunk isokinetic assessment. Trunk extensors and flexors isokinetic peak torque at 60°/sec were significantly correlated with number of D4Z4 microsatellite repetitions, sex, weight and age-independent (r = 0.391 [0.121; 0.662], p < 0.006 and r = 0.334 [0.028; 0.641], p < 0.033, respectively). Ricci's Clinical Severity Scoring was significantly correlated to trunk extensors isokinetic peak torque at 60°/sec, sex and weight-independent (r = -0.743 [-0.938; -0.548], p < 0.0001). This study demonstrates moderate correlation between pathologic compression of D4Z4 microsatellite array and trunk extensors isokinetic strength among facioscapulohumeral muscular dystrophy type I patients.

KEYWORDS:

Clinical severity scoring; Facioscapulohumeral muscular dystrophy type 1; Muscular dystrophy; Trunk isokinetic assessment; isokinetic dynamometer; neuromuscular disease

PMID:
30415787
DOI:
10.1016/j.nmd.2018.09.007
[Indexed for MEDLINE]

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