Follow-up of newborns treated with extracorporeal membrane oxygenation: a nationwide evaluation at 5 years of age

INTRODUCTION: Extracorporeal membrane oxygenation (ECMO) is a supportive cardiopulmonary bypass technique for babies with acute reversible cardiorespiratory failure. We assessed morbidity in ECMO survivors at the age of five years, when they start primary school and major decisions for their school careers must be made. METHODS: Five-year-old neonatal venoarterial-ECMO survivors from the two designated ECMO centres in The Netherlands (Erasmus MC – Sophia Children's Hospital in Rotterdam, and University Medical Center Nijmegen) were assessed within the framework of an extensive follow-up programme. The protocol included medical assessment, neuromotor assessment, and psychological assessment by means of parent and teacher questionnaires. RESULTS: Seventeen of the 98 children included in the analysis (17%) were found to have neurological deficits. Six of those 17 (6% of the total) showed major disability. Two of those six children had a chromosomal abnormality. Three were mentally retarded and profoundly impaired. The sixth child had a right-sided hemiplegia. These six children did not undergo neuromotor assessment. Twenty-four of the remaining 92 children (26%) showed motor difficulties: 15% actually had a motor problem and 11% were at risk for this. Cognitive delay was identified in 11 children (14%). The mean IQ score was within the normal range (IQ = 100.5). CONCLUSION: Neonatal ECMO in The Netherlands was found to be associated with considerable morbidity at five years of age. It appeared feasible to have as many as 87% of survivors participate in follow-up assessment, due to cooperation between two centres and small travelling distances. Objective evaluation of the long-term morbidity associated with the application of this highly invasive technology in the immediate neonatal period requires an interdisciplinary follow-up programme with nationwide consensus on timing and actual testing protocol

Meet and eat, an interdisciplinary group intervention for patients with myotonic dystrophy about healthy nutrition, meal preparation, and consumption: a feasibility study

Purpose: To develop and evaluate an interdisciplinary group intervention for patients with myotonic dystrophy regarding healthy nutrition, meal preparation, and consumption, called Meet and Eat. Materials and methods: A design-based approach was used, exploring experiences and needs of patients with myotonic dystrophy and their next of kin. This resulted in a 4-week interdisciplinary outpatient group intervention to increase awareness and change behavior. In a mixed-method feasibility study this intervention was evaluated with participants and facilitators. Results: Participants reported on social impact of difficulties with eating and drinking (speaking, swallowing, and social embarrassment) and a variety of complaints affecting meal preparation and consumption. Patients and family members expressed the wish to learn from others. After delivery of the intervention, self-reported outcome measures demonstrated improvement on eating and drinking issues in daily life. The qualitative evaluation showed increased awareness and appreciation of the following design principles: peer support, multidisciplinary approach, active involvement of patient with myotonic dystrophy and their next of kin by using personal goals and motivational interviewing. Conclusion: Participation in Meet and Eat seemed to increase insight in patients’ condition and ways of management. However, a longer self-management life style program is recommended to change behavior.Implications for rehabilitation Patients with myotonic dystrophy experience physical, cognitive and psychosocial problems to manage healthy nutrition, meal preparation, and consumption; This multidisciplinary group intervention Meet and Eat in patients with myotonic dystrophy appears to be feasible and seems to result in increased insight and management strategies; Peer support, multidisciplinary approach, personal goals, and involvement of family were key factors; To change eating and drinking behavior, a 4-week intervention is too short

Language Impairment in Cerebellar Ataxia

Background: Several studies have suggested that language impairment can be observed in patients with cerebellar pathology. The aim of this study was to investigate language performance in patients with spinocerebellar ataxia type 6 (SCA6). Methods: We assessed speech and language in 29 SCA6 patients with standardized linquistic tests and correlated this with the severity of ataxia, as quantified by the Scale of Assessment and Rating of Ataxia. Results: Individual patients show mild-to-moderate linguistic impairment. Linguistic abnormalities were most distinct on the writing and comprehension subtests. A strong correlation between severity of ataxia and linguistic performance was consistently found. Conclusions: This study confirms the occurrence of linguistic impairments in patients with cerebellar degenerative diseases, such as SCA6. The relation between linguistic abnormalities and severity of ataxia provides further evidence for a role of the cerebellum in linguistic processing. (C) 2014 International Parkinson and Movement Disorder Societ

Dysarthria and dysphagia are highly prevalent among various types of neuromuscular diseases

Purpose: Patients with a neuromuscular disease (NMD) can present with dysarthria and/or dysphagia. Literature regarding prevalence rates of dysarthria and dysphagia is scarce. The purpose of this study was to determine prevalence rates, severity and co-presence of dysarthria and dysphagia in adult patients with NMD. Method: Two groups of adult patients with NMD were included: 102 consecutive outpatients (the "unselected cohort") and 118 consecutive patients who were referred for multidisciplinary assessment (the "selected cohort"). An experienced speech-language pathologist examined each patient in detail. Results: The pooled prevalence of dysarthria was 46% (95% CI: 36.5-55.9) and 62% (95% CI: 53.3-70.8) in the unselected and selected cohorts, respectively. The pooled prevalence of dysphagia was 36% (95% CI: 27.1-45.7) and 58% (95% CI: 49.4-67.2) in the unselected and selected cohorts, respectively. There was a modest but significant association between the presence of dysarthria and dysphagia (r(s) 0.40; p <0.01). Although the dysphagia was generally mild, dysarthria was moderate to severe in 15% of the dysarthric patients. Conclusion: The prevalence rates of dysarthria and dysphagia among patients with various types of NMD are high. Physicians should therefore be aware of this prevalence and consider referring NMD patients to a speech-language pathologist