Karlien Mul1, Kiera N Berggren1, Mattie Y Sills1, Ayla McCalley1, Baziel G M van Engelen1, Nicholas E Johnson1, Jeffrey M Statland2. 1. From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands. 2. From the Department of Neurology (K.M., M.Y.S., A.M., J.M.S.), University of Kansas Medical Center, Kansas City; Department of Neurology (K.N.B., N.E.J.), Virginia Commonwealth University, Richmond; and Department of Neurology (K.M., B.G.M.v.E.), Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, the Netherlands. jstatland@kumc.edu.
Abstract
OBJECTIVE: This study explores the use of quantitative data on strength and fatigability of orofacial muscles in patients with facioscapulohumeral muscular dystrophy (FSHD) and assesses the frequency of swallowing and communication difficulties and their relationship to orofacial muscle involvement. METHODS: We included 43 patients with FSHD and 35 healthy controls and used the Iowa Oral Performance Instrument (IOPI) to obtain quantitative measurements of strength and endurance of lip compression, cheek (buccodental) compression, and tongue elevation. For the assessment of swallowing and communication difficulties, we used the dysphagia-specific quality of life (SWAL-QOL) and Communicative Participation Item Bank questionnaires. RESULTS: Cheek compression strength was reduced in patients with FSHD compared to healthy controls. Dysphagia and difficulty with verbal communication were reported by 25% and 35% of patients, respectively, and correlated to cheek compression strength and endurance and to anterior tongue elevation endurance. Prolonged cheek compression or anterior tongue elevation endurance (decreased fatigability) made swallowing or speech problems less likely to occur. CONCLUSION: Cheek compression strength is the most sensitive IOPI measure for orofacial weakness in FSHD. Orofacial weakness contributes to dysphagia and speech difficulties in FSHD, which are both common, though generally mild. Higher endurance of orofacial muscles was associated with a lower chance of dysphagia or speech problems. More research is required for further refinement of the pattern of facial muscle involvement in FSHD and to provide new insights for improvement of speech and language therapy.
OBJECTIVE: This study explores the use of quantitative data on strength and fatigability of orofacial muscles in patients with facioscapulohumeral muscular dystrophy (FSHD) and assesses the frequency of swallowing and communication difficulties and their relationship to orofacial muscle involvement. METHODS: We included 43 patients with FSHD and 35 healthy controls and used the Iowa Oral Performance Instrument (IOPI) to obtain quantitative measurements of strength and endurance of lip compression, cheek (buccodental) compression, and tongue elevation. For the assessment of swallowing and communication difficulties, we used the dysphagia-specific quality of life (SWAL-QOL) and Communicative Participation Item Bank questionnaires. RESULTS: Cheek compression strength was reduced in patients with FSHD compared to healthy controls. Dysphagia and difficulty with verbal communication were reported by 25% and 35% of patients, respectively, and correlated to cheek compression strength and endurance and to anterior tongue elevation endurance. Prolonged cheek compression or anterior tongue elevation endurance (decreased fatigability) made swallowing or speech problems less likely to occur. CONCLUSION: Cheek compression strength is the most sensitive IOPI measure for orofacial weakness in FSHD. Orofacial weakness contributes to dysphagia and speech difficulties in FSHD, which are both common, though generally mild. Higher endurance of orofacial muscles was associated with a lower chance of dysphagia or speech problems. More research is required for further refinement of the pattern of facial muscle involvement in FSHD and to provide new insights for improvement of speech and language therapy.
Authors: Jildou N Dijkstra; Rianne J M Goselink; Nens van Alfen; Imelda J M de Groot; Maaike Pelsma; Nienke van der Stoep; Thomas Theelen; Baziel G M van Engelen; Nicol C Voermans; Corrie E Erasmus Journal: Neurology Date: 2021-10-21 Impact factor: 9.910
Authors: T G J Loonen; C G C Horlings; S C C Vincenten; C H G Beurskens; S Knuijt; G W A M Padberg; J M Statland; N C Voermans; T J J Maal; B G M van Engelen; K Mul Journal: J Neurol Date: 2020-10-28 Impact factor: 4.849