Treatment Approaches for Altered Facial Expression: A Systematic Review in Facioscapulohumeral Muscular Dystrophy and Other Neurological Diseases

Author:

Rasing Nathaniël B.1,van de Geest-Buit Willianne A.1,Chan On Ying A.2,Mul Karlien1,Lanser Anke3,van Engelen Baziel G.M.1,Erasmus Corrie E.4,Fischer Agneta H.5,Ingels Koen J.A.O.6,Post Bart1,Siemann Ietske7,Groothuis Jan T.8,Voermans Nicol C.1

Affiliation:

1. Department of Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands

2. Information Specialist, Medical Library, Radboud University, Nijmegen, The Netherlands

3. Patient Representative and Chairman FSHD Advocacy Group, Patient Organization for Muscular Disease Spierziekten Nederland, Baarn, The Netherlands

4. Department of Paediatric Neurology, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Amalia Children’s Hospital, Nijmegen, The Netherlands

5. Department of Psychology, Social Psychology, University of Amsterdam, Amsterdam, the Netherlands

6. Department of Otorhinolaryngology, Radboud University Medical Center, Nijmegen, The Netherlands

7. Department of Medical Psychology, Radboud Institute for Health Sciences, Radboud University Medical Center, Nijmegen, The Netherlands

8. Department of Rehabilitation, Donders Institute for Brain, Cognition and Behaviour, Radboud University Medical Center, Nijmegen, The Netherlands

Abstract

Background: Facial weakness is a key feature of facioscapulohumeral muscular dystrophy (FSHD) and may lead to altered facial expression and subsequent psychosocial impairment. There is no cure and supportive treatments focus on optimizing physical fitness and compensation of functional disabilities. Objective: We hypothesize that symptomatic treatment options and psychosocial interventions for other neurological diseases with altered facial expression could be applicable to FSHD. Therefore, the aim of this review is to collect symptomatic treatment approaches that target facial muscle function and psychosocial interventions in various neurological diseases with altered facial expression in order to discuss the applicability to FSHD. Methods: A systematic search was performed. Selected studies had to include FSHD, Bell’s palsy, Moebius syndrome, myotonic dystrophy type 1, or Parkinson’s disease and treatment options which target altered facial expression. Data was extracted for study and patients’ characteristics, outcome assessment tools, treatment, outcome of facial expression and or psychosocial functioning. Results: Forty studies met the inclusion criteria, of which only three studies included FSHD patients exclusively. Most, twenty-one, studies were performed in patients with Bell’s palsy. Studies included twelve different therapy categories and results were assessed with different outcomes measures. Conclusions: Five therapy categories were considered applicable to FSHD: training of (non-verbal) communication compensation strategies, speech training, physical therapy, conference attendance, and smile restoration surgery. Further research is needed to establish the effect of these therapies in FSHD. We recommend to include outcome measures in these studies that cover at least cosmetic, functional, communication, and quality of life domains.

Publisher

IOS Press

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