Characterizing the face in facioscapulohumeral muscular dystrophy
| Autor: | Karlien Mul, George W. Padberg, C. H. G. Beurskens, S. Knuijt, B.G.M. van Engelen, Thomas J.J. Maal, T. G. J. Loonen, Jeffrey Statland, Nicol C. Voermans, Corinne G.C. Horlings, Sanne C. C. Vincenten |
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| Přispěvatelé: | Oral and Maxillofacial Surgery |
| Jazyk: | angličtina |
| Rok vydání: | 2021 |
| Předmět: |
Male
0301 basic medicine medicine.medical_specialty Neurology Fleiss' kappa Facial weakness Severity of Illness Index Outcome measures 03 medical and health sciences 0302 clinical medicine Disease severity Facioscapulohumeral muscular dystrophy medicine Humans In patient Longitudinal Studies Neuroradiology Original Communication business.industry Disorders of movement Donders Center for Medical Neuroscience [Radboudumc 3] medicine.disease Muscular Dystrophy Facioscapulohumeral Reconstructive and regenerative medicine Radboud Institute for Health Sciences [Radboudumc 10] 030104 developmental biology Face Physical therapy Female Neurology (clinical) medicine.symptom business 030217 neurology & neurosurgery |
| Zdroj: | Journal of neurology, 268(4), 1342-1350. D. Steinkopff-Verlag Journal of Neurology, 268, 1342-1350 Journal of Neurology, 268, 4, pp. 1342-1350 Journal of Neurology |
| ISSN: | 0340-5354 |
| Popis: | ObjectiveTo evaluate facial weakness in patients with FSHD to better define clinical signs, and pilot a facial weakness severity score.Methods87 FSHD patients and 55 controls were video recorded while performing seven facial tasks. The videos were assessed by three independent examiners to compile an overview of signs of facial weakness. Next, videos were semi-quantitatively assessed using a newly developed 4-point facial weakness score (FWS). This score was evaluated and correlated to other FSHD disease characteristics.ResultsPatients had lower scores on the total FWS than controls (mean score 43 ± 28, range 4–118, vs 14 ± 9, range 0–35,p p ConclusionThis study provides an overview of the clinical spectrum of facial weakness and its relation to other disease characteristics. The 4-point scale we introduced to grade the severity of facial weakness enables correlation of facial weakness to disease characteristics, but is not suited as clinical outcome measure for longitudinal studies. |
| Databáze: | OpenAIRE |
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