The Non-speech Oral Movement Assessment Children (NOMAC).

INTRODUCTION: Examination of oral movements is often part of an assessment undertaken by a speech and language therapist (SLT). Until now there have been no specific instruments or tests with reference values for typically developing children in Dutch that exclusively evaluate non-speech oral movements in young children. Therefore, a non-speech oral-motor observation list was designed to attempt to bridge this gap: The Non-speech Oral Movement Assessment Children (NOMAC). The aim of this study was to evaluate the psychometric properties of the NOMAC in terms of inter-rater reliability and its' construct validity. In addition, we aimed to collect reference values for the non-speech oral movements in children. METHODS: Data from typically developing Dutch children aged 2 to 8 years were collected. Inter-rater reliability was studied by estimating the intra-class correlation coefficient (ICC). Construct validity was investigated by assessing the effect of age group and gender on the mean execution score per item (general linear model). To present normative data the percentage of the children performing a normal oral - motor execution was calculated. RESULTS: The study includes a total of 318 children, divided into 9 age groups. The inter-rater reliability shows a [sufficient] to [good] ICC for most items. A significant effect of the factor age group for almost all items was seen, confirming a robust construct validity. Normative data are presented with the percentage of the children performing a normal oral movement execution. CONCLUSION: Non-speech oral movements can be assessed with the NOMAC in children between 2 to 8 years old and can be compared with values obtained from a normative group. It should be used as part of a clinical feeding and speech assessment. Despite the fact that current insights indicate that oral- motor training has no value for improving mastication, swallowing and speech, it is important to know the status of non-speech oral motor capabilities. With this assessment a complete profile of the child's oral-motor abilities can be achieved, supportive for clinical decision making in SLT.

Best practice recommendations for speech-language pathology in children with neuromuscular disorders: A Delphi-based consensus study.

PURPOSE: Speech-language pathology (SLP) is considered an essential intervention due to the high prevalence of dysphagia and dysarthria in paediatric neuromuscular disorders (pNMD). Evidence-based guidelines for SLP in pNMD are missing and children could be deprived the best of care. This study aimed to achieve consensus and present best practice recommendations on SLP intervention in pNMD. METHOD: A modified Delphi technique was used with a panel of experienced Dutch speech-language pathologists. In two online survey rounds and a face-to-face consensus meeting, the SLP experts proposed intervention items for cases of four types of pNMD (congenital myopathy, Duchenne muscular dystrophy, myotonic dystrophy type 1, and spinal muscular atrophy type 2), covering symptoms of dysphagia, dysarthria, drooling, and oral hygiene problems. They rated the level of agreement. RESULT: Intervention items that achieved consensus were incorporated into best practice recommendations. These recommendations cover six core intervention components (wait and see, explanation and advice, training and treatment, aids and adjustments, referral to other disciplines, and monitoring) suitable for the described symptoms. CONCLUSION: Insight into treatment options is essential to facilitate speech-language pathologists in clinical decision-making. The current study led to best practice recommendations for speech-language pathologists working within the field of pNMD.

Validity and reliability of visual assessment of orofacial muscle ultrasound images using a modified Heckmatt scale.

INTRODUCTION/AIMS: Orofacial muscle ultrasound images can be evaluated quantitatively or using a visual grading system. Quantitative muscle ultrasound (QMUS) is currently the most sensitive technique to detect pathology, but can be time-consuming. The aim of this study was to investigate the validity and reliability of two visual grading systems (the original Heckmatt scale or a modified 3-point version) for the optimal grading of orofacial muscle images. METHODS: A retrospective, comparative, reliability and validity study was performed. Ultrasound images of the digastric, geniohyoid, masseter, temporalis muscles, and intrinsic muscles of the tongue of healthy participants and of patients (suspected of) having a neuromuscular disease were included. QMUS was used as the "gold standard." Two expert raters and one inexperienced rater rated all ultrasound images using both visual grading systems. RESULTS: A total of 511 ultrasound images were included. Criterion validity showed Spearman rho correlation coefficients of >0.59. Construct validity analysis showed strong to very strong associations between the visual grading systems and mastication and/or swallowing. Inter- and intrarater reliability of the original Heckmatt scale and the modified scale were good and comparable. Rater experience had a beneficial effect on the interrater reliability of both scales. DISCUSSION: Both the original Heckmatt and the modified Heckmatt scale are valid and reliable tools for the visual grading of orofacial ultrasound images. The modified Heckmatt scale, with only three grades and including an "uncertain" category, is considered easier to use in clinical practice.

Dysphagia limit in children with cerebral palsy aged 4 to 12 years.

AIM: To assess the dysphagia limit in children with cerebral palsy (CP) according to Eating and Drinking Ability Classification System (EDACS) level, sex, and age compared to typically developing children. METHOD: Seventy-seven children with CP (54 males, 23 females; mean age 7y 6mo, SD 2y 2mo, age range 4-12y) were assessed with the Maximum Volume Water Swallow Test. Median dysphagia limit in the CP group was compared with data of typically developing children. RESULTS: The dysphagia limit of children with CP differed significantly (p<0.001) from typically developing children. The latter showed a threefold higher median dysphagia limit (22mL) compared to children with CP in EDACS level I (7mL). The higher the EDACS level, the lower the dysphagia limit in children with CP. EDACS level explained 55% of the variance in the dysphagia limit of the CP group. INTERPRETATION: Where children with CP in EDACS levels IV and V showed that their capacity met the level of their performance, children in EDACS level I had the ability to perform a maximum capacity task, but still had a threefold lower median dysphagia limit than typically developing children. Establishment of the dysphagia limit should be part of general swallowing assessment in children with CP.

Validation of the pediatric Radboud Dysarthria Assessment.

PURPOSE: The Radboud Dysarthria Assessment (RDA) was published in 2014. Adaptation into a pediatric version (p-RDA) was required because of relevant differences between children and adults. The purpose of this study was to assess the feasibility of the p-RDA and to test intra-rater and inter-rater reliability as well as the validity of the two severity scales (function and activity level). METHODS: Video recordings were made of 35 participants with (suspected) dysarthria (age 4 to 17 years) while being assessed using the p-RDA. Intra-rater reliability was assessed by one, and inter-rater reliability by two experiments using the Intraclass Correlation Coefficient (ICC). Validity of the severity scales was tested by correlating the consensus scores with the independently rated scores on four communication scales, three mobility scales, and one self-care scale using Spearman correlation coefficients (rs). RESULTS: The assessment was applicable for 89% of the tested sample, with good intra-rater and inter-rater reliability (ICC = 0.88-0.98 and 0.83-0.93). The p-RDA severity scales (function and activity level) correlated from substantially to strongly with the communication scales (rs = 0.69-0.82 and 0.77-0.92) and self-care scale (rs = 0.76-0.71) and correlated substantially with the mobility scales (rs = 0.49-0.60). CONCLUSION: The feasibility, reliability and validity of the p-RDA are sufficient for clinical use.

Maximum repetition rate in a large cross-sectional sample of typically developing Dutch-speaking children.

Purpose: The current study aims to provide normative data for the maximum repetition rate (MRR) development of Dutch-speaking children based on a large cross-sectional study using a standardised protocol.Method: A group of 1014 typically developing children aged 3;0 to 6;11 years performed the MRR task of the Computer Articulation Instrument (CAI). The number of syllables per second was calculated for mono-, bi-, and trisyllabic sequences (MRR-pa, MRR-ta, MRR-ka, MRR-pata, MRR-taka, MRR-pataka). A two-way mixed ANOVA was conducted to compare the effects of age and gender on MRR scores in different MRR sequences.Result: The data analysis showed that overall MRR scores were affected by age group, gender and MRR sequence. For all MRR sequences the MRR increased significantly with age. MRR-pa was the fastest sequence, followed by respectively MRR-ta, MRR-pata, MRR-taka, MRR-ka and MRR-pataka. Overall MRR scores were higher for boys than for girls, for all MRR sequences.Conclusion: This study presents normative data of MRR of Dutch-speaking children aged 3;0 to 6;11 years. These norms might be useful in clinical practice to differentiate children with speech sound disorders from typically developing children. More research on this topic is necessary. It is also suggested to collect normative data for other individual languages, using the same protocol.

Speech sound development in typically developing 2-7-year-old Dutch-speaking children: A normative cross-sectional study.

BACKGROUND: Dutch is a West-Germanic language spoken natively by around 24 million speakers. Although studies on typical Dutch speech sound development have been conducted, norms for phonetic and phonological characteristics of typical development in a large sample with a sufficient age range are lacking. AIM: To give a detailed description of the speech sound development of typically developing Dutch-speaking children from 2 to 7 years. METHODS & PROCEDURES: A total of 1503 typically developing children evenly distributed across the age range of 2;0-6;11 years participated in this normative cross-sectional study. The picture-naming task of the Computer Articulation Instrument (CAI) was used to collect speech samples. Speech development was described in terms of (1) percentage consonants correct-revised (PCC-R) and percentage vowels correct (PVC); (2) consonant, vowel and syllabic structure inventories; (3) degrees of complexity (phonemic feature hierarchy); and (4) phonological processes. OUTCOMES & RESULTS: A two-way mixed analysis of variance (ANOVA) confirmed a significant increase in the number of PCC-R and PVC between the ages of 2;0 and 6;11 years (p < 0.001). The consonant inventory was found to be complete at 3;7 years of age for the syllable-initial consonants, with the exception of the voiced fricatives /v/ and /z/, and the liquid /r/. All syllable-final consonants were acquired before age 4;4 years. At age 3;4 years, all children had acquired a complete vowel inventory, and at age 4;7 years they produced most syllable structures correctly, albeit that the syllable structure CCVCC was still developing. All phonological contrasts were produced correctly at 3;8 years of age. Children in the younger age groups used more phonological simplification processes than the older children, and by age 4;4 years, all had disappeared, except for the initial cluster reduction from three to two consonants and the final cluster reduction from two to one consonant. CONCLUSIONS & IMPLICATIONS: This paper describes a large normative cross-sectional study of Dutch speech sound development which, in clinical practice, can help Dutch speech-language pathologists to differentiate children with delayed or disordered speech development from typically developing children. What this paper adds What is already known on this subject In recent years many studies have been conducted worldwide to investigate speech sound development in different languages, including several that explored the typical speech sound development of Dutch-speaking children, but none of these latter studies explored both phonetic and phonological progress within a comprehensive age range and a large sample that is representative of the Dutch population. What this study adds to existing knowledge This study serves to fill this gap by providing normative cross-sectional results obtained in 1503 typically developing Dutch-speaking children aged between 2;0 and 6;11 years on informative parameters of speech development: PCC-R and PVC, consonant, vowel and syllabic structure inventories, degrees of complexity (phonemic feature hierarchy), and phonological simplification processes. What are the potential or actual clinical implications of this work? The detailed description of typical Dutch speech sound development provides speech-language pathologists with pertinent information to determine whether a child's speech development progresses typically or is delayed or disordered.

Young children with Noonan syndrome: evaluation of feeding problems.

Noonan syndrome (NS) is a common genetic syndrome with a high variety in phenotype. Even though genetic testing is possible, NS is still a clinical diagnosis. Feeding problems are often present in infancy. We investigated the feeding status of 108 patients with clinically and genetically confirmed NS. Only patients with a documented feeding status before the age of 6 were included. A distinction was made between patients with early onset feeding problems (< 1 year) and children with late onset feeding problems (> 1 year). Seventy-one of 108 patients had feeding problems, of which 40 patients required tube feeding. Children with a genetic mutation other than PTPN11 and SOS1 had significantly more feeding problems in the first year. Fifty-two of all 108 patients experienced early onset feeding problems, of which 33 required tube feeding. A strong decrease in prevalence of feeding problems was found after the first year of life. Fifteen children developed feeding problems later in life, of which 7 required tube feeding.Conclusion: Feeding problems occur frequently in children with NS, especially in children with NS based on genetic mutations other than PTPN11 and SOS1. Feeding problems develop most often in infancy and decrease with age. What is Known: • Young children with Noonan syndrome may have transient feeding problems. • Most of them will need tube feeding. What is New: • This is the first study of feeding problems in patients with clinically and genetically proven Noonan syndrome. • Feeding problems most often develop in infancy and resolve between the age of 1 and 2.

Dysphagia and Dysarthria in Children with Neuromuscular Diseases, a Prevalence Study.

BACKGROUND: Dysphagia and dysarthria are frequently described in pediatric neuromuscular diseases (pNMD). The consequences can be substantial: failure to thrive, malnutrition, aspiration pneumonia, or communication problems. Early detection and identification of risk factors and etiology support preventing complications and morbidity, including impact on quality of life. Information about the prevalence of dysphagia and dysarthria in pNMD is scarce. OBJECTIVE: To describe the pooled prevalence of dysphagia and dysarthria in pNMD in the Netherlands. In addition, we describe the prevalence of dysphagia and dysarthria each, and the prevalence of chewing (oral) and swallowing problems per diagnostic group, based on their anatomic origin. METHODS: Data were collected from 295 children (mean age 11;0 years, range 2;6-18;0) with pNMD in 12 hospitals and rehabilitation centers in the Netherlands. A speech language therapist established whether dysphagia and dysarthria were present or not. RESULTS: In almost all the 14 diagnostic groups of pNMD, dysphagia and dysarthria were present. Pooled overall prevalence of dysphagia and dysarthria was 47.2% and 31.5%, respectively. Of 114 children with dysphagia, 90.0% had chewing problems, 43.0% showed swallowing problems and 33.3% showed both chewing and swallowing problems. CONCLUSIONS: The overall pooled prevalence of dysphagia and dysarthria was high in the population of pNMD. It can be argued that periodic monitoring of dysphagia and dysarthria and early referral to a speech language therapist should be a necessity from the start of the diagnosis in the whole pNMD population.

Orofacial muscles may be affected in early stages of Becker muscular dystrophy: A preliminary study.

BACKGROUND: Dysphagia is reported in patients with Duchenne or Becker muscular dystrophy. Our clinical experience suggests that, compared with Duchenne patients, impaired mastication and swallowing occur early in Becker patients relative to their skeletal muscle involvement. The aim of this study was to assess dysphagia in Duchenne and Becker patients in relation to ambulatory capacity. METHODS: In patients in the early ambulatory stage, clinical symptoms, quantitative muscle ultrasound of the orofacial muscles, and maximum bite force were assessed. The 6-Minute Walk Test (6MWT) was used to measure ambulatory capacity. RESULTS: Eleven Duchenne and 11 Becker patients were included. Although Becker patients had a greater 6MWT distance than Duchenne patients, the occurrence of mastication and swallowing difficulties was similar. The temporalis muscle was significantly thicker in Becker patients. CONCLUSIONS: Clinicians should be aware of dysphagia in both groups, even when ambulation is still well preserved.

Profiling Speech Sound Disorders for Clinical Validation of the Computer Articulation Instrument.

Purpose The current article presents data from 2 studies on clinical groups of children referred for speech assessment. The aims of these studies are to validate the Computer Articulation Instrument (CAI) with the known-group validation method and to determine the differential diagnostic power of the resulting speech profiles. Method Study 1 examined known-group validity by comparing the scores of 93 children diagnosed with speech-language difficulties on the picture naming (PN) task of the CAI with intelligibility judgments given by speech-language pathologists. In Study 2, the speech profiles of 41 children diagnosed with speech sound disorders (SSDs), consisting of 4-6 factor scores extracted from the 4 tasks of the CAI, namely, PN, nonword imitation (NWI), word and nonword repetition, and maximum repetition rate (MRR), were validated against clinical judgments of severity of the SSD given by speech-language pathologists. Results In Study 1, a repeated-measures analysis of variance revealed a significant effect of intelligibility level on the PN performance of the CAI and there were highly significant correlations between intelligibility and PN performance in the expected direction. Neither intelligibility level nor PN performance was related to nonverbal intelligence and language scores. The analysis of variance and a series of t tests in Study 2 revealed significant differences between the moderate and severe groups for the CAI factors based on PN and NWI and the bisyllabic and trisyllabic sequences of MRR, but not for the factor word and nonword proportion of whole-word variability based on word and nonword repetition, and the monosyllabic sequences of MRR. These results suggest that, especially, the tasks PN, NWI, and the bisyllabic and trisyllabic sequences of MRR are most sensitive for diagnosing SSDs. Conclusions The findings of these 2 studies support the known-group validity of the CAI. Together with the results of a previous study of our group on reliability and validity ( van Haaften et al., 2019 ), we can conclude that the CAI is a reliable and valid tool for assessment of children with SSDs.

The Psychometric Evaluation of a Speech Production Test Battery for Children: The Reliability and Validity of the Computer Articulation Instrument.

Purpose The aims of this study were to assess the reliability and validity of the Computer Articulation Instrument (CAI), a speech production test battery assessing phonological and speech motor skills in 4 tasks: (1) picture naming, (2) nonword imitation, (3) word and nonword repetition, and (4) maximum repetition rate (MRR). Method Normative data were collected in 1,524 typically developing Dutch-speaking children (aged between 2;0 and 7;0 [years;months]). Parameters were extracted on segmental and syllabic accuracy (Tasks 1 and 2), consistency (Task 3), and syllables per second (Task 4). Interrater reliability and test-retest reliability were analyzed using subgroups of the normative sample and studied by estimating intraclass correlation coefficients (ICCs). Construct validity was investigated by determining age-related changes of test results and factor analyses of the extracted speech measures. Results ICCs for interrater reliability ranged from sufficient to good, except for percentage of vowels correct of picture naming and nonword imitation and for the MRRs for bisyllabic and trisyllabic items. The ICCs for test-retest reliability were sufficient (picture naming, nonword imitation) to insufficient (word and nonword repetition, MRR) due to larger-than-expected normal development and learning effects. Continuous norms showed developmental patterns for all CAI parameters. The factor analyses revealed 5 meaningful factors: all picture-naming parameters, the segmental parameters of nonword imitation, the syllabic structure parameters of nonword imitation, (non)word repetition consistency, and all MRR parameters. Conclusion Its overall sufficient to good psychometric properties indicate that the CAI is a reliable and valid instrument for the assessment of typical and delayed speech development in Dutch children in the ages of 2-7 years.

"Everyone sees you sitting there struggling with your food": experiences of adolescents and young adults with cerebral palsy.

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. Implications for Rehabilitation Adolescents and young adults with cerebral palsy experience difficulties with eating, drinking, and swallowing, and they encounter difficulties in participating in mealtimes with family and friends. Although adolescents and young adults with cerebral palsy rated their eating and drinking abilities as reasonable, they reported feelings of shame, frustration, fear, distress, and concerns for the future or unknown environments. Regular multidisciplinary involvement with eating and drinking is needed for purposes of evaluation, advice, and intervention throughout the life course, adjusted to living conditions and the latest evidence. Adolescents and young adults with cerebral palsy showed limited initiative in asking for personal assistance in eating and drinking activities.

International standardisation of the test of masticating and swallowing solids in children.

The Test of Masticating and Swallowing Solids (TOMASS) is a validated assessment tool measuring the efficiency of solid bolus intake by four quantitative parameters: discrete bites, masticatory cycles, swallows and time to ingest a single cracker. A normative database for adults (20-80+ years) has previously been established. The objective of this study was to investigate the applicability and reliability of the TOMASS in children and adolescents (TOMASS-C) and to establish the normative database for this younger population. We collected data from 638 participants (male: 311, female: 327) in five age groups (4-18 years) with five different but very similar test crackers in four countries. Significant effects of bolus type (cracker), age group and gender on the TOMASS parameters were identified, requiring stratification of the TOMASS-C database by these variables. Intra-rater reliability was excellent (ICC > 0.94) for all parameters; inter-rater reliability was moderate for "number of swallows" (ICC = 0.54), good for "bites" (ICC = 0.78) and "time" (ICC = 0.82), and excellent for "masticatory cycles" (ICC = 0.96). The "Test of Masticating and Swallowing Solids in Children (TOMASS-C)" was identified to be a reliable diagnostic tool for the comprehensive measurement of discrete oral stage components of solid bolus ingestion, standardised by a large normative database that covers age groups from preschoolers to young adults. While differences between gender groups were less pronounced than in the adult population, previous results relating to changes in masticatory and swallowing as a function of age are confirmed by our data.

The Test of Masticating and Swallowing Solids (TOMASS): reliability, validity and international normative data.

BACKGROUND: Clinical swallowing assessment is largely limited to qualitative assessment of behavioural observations. There are limited quantitative data that can be compared with a healthy population for identification of impairment. The Test of Masticating and Swallowing Solids (TOMASS) was developed as a quantitative assessment of solid bolus ingestion. AIMS: This research programme investigated test development indices and established normative data for the TOMASS to support translation to clinical dysphagia assessment. METHODS & PROCEDURES: A total of 228 healthy adults (ages 20-80+ years) stratified by age and sex participated in one or more of four consecutive studies evaluating test-retest and interrater reliability and validity to instrumental assessment. For each study the test required participants to ingest a commercially available cracker with instructions to 'eat this as quickly as is comfortably possible'. Further averaged measures were derived including the number of masticatory cycles and swallows per bite, and time per bite, masticatory cycle and swallow. Initial analyses identified significant differences on salient measures between two commercially available crackers that are nearly identical in shape, size and ingredients, suggesting the need for separate normative samples for specific regional products. Additional analyses on a single cracker identified that the TOMASS was sensitive at detecting changes in performance based on age and sex. Test-retest reliability across days and interrater reliability between clinicians was high, as was validation of observational measures to instrumental correlates of the same behaviours. Therefore, normative data are provided for the TOMASS from a minimum of 80 healthy controls, stratified by age and sex, for each of seven commercially available crackers from broad regions worldwide. OUTCOMES & RESULTS: Analyses on a single cracker identified Arnott's Salada, and that TOMASS measures were sensitive for detecting changes in performance based on age and sex. Interrater and test-retest reliability across days were high, as was validation of observational measures to instrumental correlates of the same behaviours. Significant differences were identified between two commercially available crackers, nearly identical in shape, size and ingredients, thus normative samples for specific regional products were required. Normative data were then acquired for the TOMASS from a minimum of 80 healthy controls, stratified by age and sex, for each of seven commercially available crackers from broad regions worldwide. CONCLUSIONS & IMPLICATIONS: The TOMASS is presented as a valid, reliable and broadly normed clinical assessment of solid bolus ingestion. Clinical application may help identify dysphagic patients at bedside and provide a non-invasive, but sensitive, measure of functional change in swallowing.

Pediatric feeding and swallowing rehabilitation: An overview.

Children with neurological disabilities frequently have problems with feeding and swallowing. Such problems have a significant impact on the health and well-being of these children and their families. The primary aims in the rehabilitation of pediatric feeding and swallowing disorders are focused on supporting growth, nutrition and hydration, the development of feeding activities, and ensuring safe swallowing with the aim of preventing choking and aspiration pneumonia. Pediatric feeding and swallowing disorders can be divided into four groups: transient, developmental, chronic or progressive.This article provides an overview of the available literature about the rehabilitation of feeding and swallowing disorders in infants and children. Principles of motor control, motor learning and neuroplasticity are discussed for the four groups of children with feeding and swallowing disorders.

Detecting fasciculations in cranial nerve innervated muscles with ultrasound in amyotrophic lateral sclerosis.

INTRODUCTION: Cranial muscle fasciculations may be difficult to detect in amyotrophic lateral sclerosis (ALS). Ultrasound (US) detection of fasciculations in these muscles may have clinical utility. METHODS: Patients with suspected ALS were prospectively enrolled. Nerve conduction studies, needle electromyography (EMG), and US examination of cranial muscles were performed. Controls were examined by US only. Fasciculations were counted and scored for each muscle after 10 or 30 seconds. RESULTS: There were 84 patients with ALS. Fasciculations were most frequently found in the genioglossus muscle. Overall, detection rates by US and EMG were similar, but US was more likely to detect frequent fasciculations. Fasciculations were rare in controls, seen in 7 of 1,090 (0.6%) muscles. No control had > 5 fasciculations in any muscle. DISCUSSION: Fasciculations were frequently detected in cranial muscles of patients with ALS. US was found to be a sensitive method, and was not impaired by factors such as anxiety and the inability of the patient to relax. Muscle Nerve 56: 1072-1076, 2017.

Ultrasound of oral and masticatory muscles: Why every neuromuscular swallow team should have an ultrasound machine.

Patients with neuromuscular disorders often present with swallowing difficulties due to oral phase problems and pharyngeal residue after swallow. It is important to assess the underlying pathology and cause of the swallowing disturbance in this patient group, such as dystrophic changes in oral and masticatory muscles. This allows for more patient-tailored recommendations, for example optimal compensation strategies to maintain function for longer. Ultrasound can show structural changes caused by dystrophy or denervation of muscles, detect involuntary movements such as fasciculations, and provide dynamic video images of tongue motion during swallowing attempts. This article, based on the authors' extensive experience with ultrasound in neuromuscular disease, explains the concepts of oral muscle ultrasound and its proven value in assessing neuromuscular mastication and swallowing problems. As a patient-friendly and portable technique, we advocate its use as a standard tool for analyzing neuromuscular dysphagia. Clin. Anat. 30:183-193, 2017. © 2017 Wiley Periodicals, Inc.

Dysphagia in children with repaired oesophageal atresia.

UNLABELLED: Dysphagia is a common problem in children with repaired oesophageal atresia (OA). Abnormalities in the oropharyngeal and oesophageal phase have hardly been studied. The aims of this study were to assess the prevalence of dysphagia in children with repaired OA and to identify and differentiate oral and pharyngeal dysphagia based on videofluoroscopic swallow study (VFSS) findings in a limited number of children in this cohort. Medical records of 111 patients, born between January 1996 and July 2013 and treated at the Radboudumc Amalia Children's Hospital, were retrospectively reviewed. The prevalence of dysphagia was determined by the objective and modified Functional Oral Intake Scale (FOIS) in four age groups. The first performed VFSS of 12 children was structurally assessed. The prevalence of dysphagia was 61 of 111 patients (55 %) in age group <1 year. In age group 1-4, 5-11 and 12-18 years, the prevalence of dysphagia decreased from 54 of 106 (51 %) patients to 11 of 64 (17 %) and 5 of 24 (21 %) patients. The 12 VFSS's reviews revealed oral dysphagia in 36 % and pharyngeal dysphagia in 75 %. CONCLUSIONS: This study highlights dysphagia as an important problem in different age groups of children with repaired OA. Furthermore, our study shows the presence of oropharyngeal dysphagia in this population. This study emphasizes the need to standardize the use of objective dysphagia scales, like the modified FOIS, to provide a careful follow-up of children with repaired OA. WHAT IS KNOWN: • Prevalence of dysphagia in children with repaired oesophageal atresia varies widely (ranges from 45 to 70 %) in literature. • Oral, pharyngeal and oesophageal dysphagia require different treatment approaches. What is New: • We determined dysphagia based on functional oral intake and provide an overview of change in dysphagia prevalence and severity over time in children with repaired OA. • Our study shows that dysphagia, including oropharyngeal dysphagia, is highly prevalent in young children with repaired OA and improves with time.