2019, Article / Letter to editor (Disability and Rehabilitation, vol. 41, iss. 16, (2019), pp. 1898-1905)Objective: The impact of difficulties with eating and drinking in adolescents and young adults with cerebral palsy is unknown. The purpose of this study is to find out which difficulties adolescents and young adults with cerebral palsy experience with eating and drinking in daily life and how they deal with these difficulties. The study also explores how they think about themselves with respect to eating and what does or does not help regarding social participation. Method: We collected the data from ten participants with spastic cerebral palsy (aged 15-23 years) living in the Netherlands. We used a qualitative study design with a conventional content analysis. Semi-structured in-depth interviews were used to identify meaningful factors related to eating and drinking difficulties. We coded relevant phrases from each interview and clustered and synthesized them into categories. Result: We derived four categories from the transcripts: (I) perceived eating and drinking difficulties (e.g., not managing to eat all food textures and/or choking); (II) challenges in physical and social context (e.g., accessibility of restaurants, menu supply, and/or needing assistance or not); (III) dealing with eating and drinking difficulties (e.g., adaptation, food avoidance, and/or giving up); (IV) Negative feelings about their eating and drinking (e.g., shame, frustration, fear for choking, and/or concerns about the future). One striking finding was that most participants had not recently received either monitoring or intervention for their feeding skills. Conclusion: This study shows that adolescents and young adults with cerebral palsy experience many restrictions in eating and drinking situations leading to negative feelings and lower participation levels, while little attention is directed towards these difficulties. Regular multidisciplinary rehabilitation programs should include evaluation, advice, and intervention regarding eating and drinking ability in order to increase social participation and self-management.
2012, Article / Letter to editor (Journal of Neurology, vol. 260, iss. 5, (2012), pp. 1295-1303)Dysphagia is reported in advanced stages of Duchenne muscular dystrophy (DMD). The population of DMD is changing due to an increasing survival. We aimed to describe the dysphagia in consecutive stages and to assess the underlying mechanisms of dysphagia in DMD, in order to develop mechanism based recommendations for safe swallowing. In this cross-sectional study, participants were divided into: early and late ambulatory stage (AS, n = 6), early non-ambulatory stage (ENAS, n = 7), and late non-ambulatory stage (LNAS, n = 11). Quantitative oral muscle ultrasound was performed to quantify echo intensity. Swallowing was assessed with a video fluoroscopic swallow study, surface electromyography (sEMG) of the submental muscle group and tongue pressure. Differences in outcome parameters among the three DMD stages were tested with analysis of variance. Oral muscles related to swallowing were progressively affected, starting in the AS with the geniohyoid muscle. Tongue (pseudo) hypertrophy was found in 70 % of patients in the ENAS and LNAS. Oral phase problems and post-swallow residue were observed, mostly in the LNAS with solid food. sEMG and tongue pressure data of swallowing solid food revealed the lowest sEMG amplitude, the longest duration and lowest tongue pressure in the LNAS. In case of swallowing problems in DMD, based on the disturbed mechanisms of swallowing, it is suggested to (1) adjust meals in terms of less solid food, and (2) drink water after meals to clear the oropharyngeal area.
2012, Article / Letter to editor (Muscle and Nerve, vol. 46, iss. 1, (2012), pp. 31-37)The purpose of this study was to assess the feasibility of quantitative muscle ultrasound (QMUS) to visualize oral muscles and to establish normative data for muscle thickness and echo intensity of submental and tongue muscles in healthy children and young adults. The data were compared with those of 5 patients with Duchenne muscular dystrophy (DMD). Ultrasound images from the suprahyoid region and from the surface of the tongue were made in 53 healthy subjects aged 5 to 30 years. All measurements were feasible in all subjects and patients with good reproducibility except for the mylohyoid muscle. Muscle thickness depended on height, and echo intensity depended on weight. Our findings suggest gradual involvement of oral muscles in DMD. QMUS in oral muscles is feasible in healthy children, adults and patients with DMD. These data show that it is possible to differentiate between healthy persons and patients with DMD.
2011, Article / Letter to editor (Movement Disorders, vol. 26, iss. 9, (2011), pp. 1670-1676)Drooling is an incapacitating feature of Parkinsons disease. Better pathophysiological insights are needed to improve treatment. In this study, we tested the hypothesis that the cause of drooling is multifactorial. We examined 15 patients with Parkinsons disease with distinct diurnal saliva loss ("droolers") and 15 patients with Parkinsons disease without drooling complaints ("nondroolers"). We evaluated all factors that could potentially contribute to drooling: swallowing capacity (maximum volume), functional swallowing (assessed with the dysphagia subscale of the Therapy Outcome Measures for rehabilitation specialists), unintentional mouth opening due to hypomimia (Unified Parkinsons Disease Rating Scale item), posture (quantified from sagittal photographs), and nose-breathing ability. We also quantified the frequency of spontaneous swallowing during 45 minutes of quiet sitting, using polygraphy. Droolers had more advanced Parkinsons disease than nondroolers (Unified Parkinsons Disease Rating Scale motor score 31 vs 22; P=.014). Droolers also scored significantly worse on all recorded variables except for nose breathing. Swallowing frequency tended to be higher, possibly to compensate for less efficient swallowing. Logistic regression with adjustment for age and disease severity showed that hypomimia correlated best with drooling. Linear regression with hypomimia as the dependent variable identified disease severity, dysphagia, and male sex as significant explanatory factors. Drooling in Parkinsons disease results from multiple risk factors, with hypomimia being the most prominent. When monitored, patients appear to compensate by increasing their swallowing frequency, much like the increased cadence that is used to compensate for stepping akinesia. These findings can provide a rationale for behavioral approaches to treat drooling.