Cerebral palsy, a neurological disease of the young developing brain, is accompanied by a plethora of side effects. As an extension of general poverty of motor control, those with cerebral palsy show deficient hand function. Patients also present with various cognitive deficits, including reduced intellectual ability, and linguistic deficits, such as impaired language comprehension. There are concomitant sensory deficits, especially concerning tactile sensibility. Additionally, people with cerebral palsy often have impairments related to oral processes, such as swallowing. Other comorbid conditions include mental retardation and epilepsy.
ORAL IMPAIRMENTS
Cerebral palsy sufferers tend to have comorbid oral impairments (aside from trouble speaking). Dysphagia (trouble swallowing) is extremely prevalent among cerebral palsy sufferers and often leads to drooling. It also makes eating quite difficult; patients who exhibit dysphagia tend to be fairly inefficient eaters, taking a long time to complete meals and sometimes requiring soft or mashed-up foods. Such feeding problems put the cerebral palsied at risk for undernourishment which usually results in poor health. The extent of oral impairments is most often related to the severity of general motor impairments.
1. Dysphagia in children with severe generalized cerebral palsy and intellectual disability
This study assessed the clinical indicators and severity of dysphagia in a representative sample of children with severe generalized cerebral palsy and intellectual disability. A total of 166 children (85 males, 81 females) with Gross Motor Function Classification System Level IV or V and IQ<55 were recruited from 54 daycare centres. Mean age was 9 years 4 months (range 2 y 1 mo-19 y 1 mo). Clinically apparent presence and severity of dysphagia were assessed with a standardized mealtime observation, the Dysphagia Disorders Survey (DDS), and a dysphagia severity scale. Additional measures were parental report on feeding problems and mealtime duration. Of all 166 participating children, 1% had no dysphagia, 8% mild dysphagia, 76% moderate to severe dysphagia, and 15% profound dysphagia (receiving nil by mouth), resulting in a prevalence of dysphagia of 99%. Dysphagia was positively related to severity of motor impairment, and, surprisingly, to a higher weight for height. Low frequency of parent-reported feeding problems indicated that actual severity of dysphagia tended to be underestimated by parents. Proactive identification of dysphagia is warranted in this population, and feasible using a structured mealtime observation. Children with problems in the pharyngeal and esophageal phases, apparent on the DDS, should be referred for appropriate clinical evaluation of swallowing function.
[Calis, E. A., Veugelers, R., Sheppard, J. J., Tibboel, D., Evenhuis, H. M. & Penning, C. (2008). Dysphagia in children with severe generalized cerebral palsy and intellectual disability. Developmental Medicine and Child Neurology, 50(8), 625-30.]
2. Drooling, saliva production, and swallowing in cerebral palsy
Fourteen participants (six females, eight males) ranging in age from 7 years 11 months to 18 years 2 months (mean 11y 7mo) with a confirmed diagnosis of spastic cerebral palsy (CP) were included in the study. Participants included those who drooled (CP+, n=14); age- and sex-matched children with spastic CP who were dry to mild and never to infrequent droolers (CP-, n=14) as well as typically developing peers (CTRL, n=14) served as controls. Frequency of swallowing was measured by using simultaneous cervical ausculation and videotaping of the head and neck. Saliva production was measured with the Saxon test, a simple gauze-chewing procedure. In addition, Pediatric Evaluation of Disability Inventory (PEDI), Test of Nonverbal Intelligence-3 (TONI-3), dysarthria severity scale, and Gross Motor Function Classification System (GMFCS) scores were obtained for each participant. Both groups of participants with CP tended to swallow less frequently than typically developing participants and tended to produce less saliva than typically developing controls; however, these differences were not statistically significant. No correlation was found between amount of saliva produced and amount drooled (r=0.245). An analysis of variance (ANOVA) conducted on the PEDI functional skills mean scores indicated significant differences between the three groups (F(2,39)=23.522,p<0.0001). Likewise, an ANOVA conducted on the TONI-3 scores revealed statistically significant differences between the three groups (F(2,39)=31.761, p<0.0001). A Spearman's rho correlation indicated that GMFCS scores were not significantly correlated with drooling severity (Spearman's rho correlation=0.3951,p=0.037). Drooling severity was found to be positively correlated with dysarthria severity (Spearman's rho correlation=0.82,p<0.0001). These findings suggest that drooling in patients with CP is related to swallowing difficulties rather than hypersalivation.
[Senner, J. E., Logemann, J., Zecker, S. & Gaebler-Spira, D. (2004). Drooling, saliva production, and swallowing in cerebral palsy. Developmental Medicine and Child Neurology, 46(12), 801-6.]
3. Prevalence of drooling in children with cerebral palsy attending special schools
Prevalence of drooling in children with cerebral palsy (CP) and its severity across dental age (i.e. children with primary, mixed, and permanent dentitions) was investigated. One hundred and sixty children and adolescents (91 males, 69 females; mean age 10 years 10 months, SD 4 years 2 months; range 4 to 18 years) with CP who attended special schools were involved in this study. Sixty eight per cent of participants had spastic CP with the majority (45%) diagnosed as having quadriplegia. Presence or absence of drooling was determined by direct observation of the participants. Other data, such as age, sex, learning disability, and type of CP were collected using questionnaires. Results showed 93 of 160 children (58%) with CP had a drooling condition and of these 53 (33%) had severe drooling. It was found that the degree of drooling decreased as the child's dental age increased. Prevalence of drooling in children with primary dentition was found to be 75% as compared with 43% of those with permanent dentition, although this difference was not statistically significant. It was concluded that any invasive treatment to reduce drooling should, perhaps, be postponed until the child's oromusculature has matured.
[Tahmassebi, J. F. & Curzon, M. E. (2003). Prevalence of drooling in children with cerebral palsy attending special schools. Developmental Medicine and Child Neurology, 45(9), 613-7.]
4. Objective measures of dysphagia complexity in children related to suckle feeding histories, gestational ages, and classification of their cerebral palsy
Data collected during the routine assessment of 117 dysphagic children with cerebral palsy have been related to both suckle feeding histories and gestational ages and to the classification of cerebral palsy. In addition, a concurrent survey involving 281 children with cerebral palsy in special schools was undertaken which revealed that the sample of referred children appeared to be a true representation of a wider population of dysphagic children with cerebral palsy. A Feeding Difficulty Symptom Score (FDSS) describes the severity of swallowing symptoms reported. A numerical Dysphagia Complexity Index (DCI) quantifies numerically the neurological complexity of the swallowing difficulty. The FDSS correlates closely with the DCI. Twenty-seven percent of mothers of the children who were referred for advice on their present swallowing difficulties stated that they recalled no suckle feeding problems. However, there was no difference in the severity of present swallowing difficulties between those infants who suckle fed well and those who experienced severe difficulties. Those referred children with cerebral palsy born at term exhibited more complex later swallowing problems and were more likely to be classified as athetoid than those born preterm.
[Selley, W. G., Parrott, L. C., Lethbridge, P. C., Flack, F. C., Johnston, K. J., Foumeny, M. A. & Tripp, J. H. (2001). Objective measures of dysphagia complexity in children related to suckle feeding histories, gestational ages, and classification of their cerebral palsy. Dysphagia, 16(3), 200-7.]
5. Prevalence of feeding problems and oral motor dysfunction in children with cerebral palsy: A community survey
OBJECTIVE: To determine the prevalence and nature of feeding difficulties and oral motor dysfunction among a representative sample of 49 children with cerebral palsy (12 to 72 months of age). STUDY DESIGN: A population survey was undertaken by means of a combination of interview and home observational measures. RESULTS: Sucking (57%) and swallowing (38%) problems in the first 12 months of life were common, and 80% had been fed nonorally on at least one occasion. More than 90% had clinically significant oral motor dysfunction. One in three (36.2%) was severely impaired and therefore at high risk of chronic undernourishment. There was a substantial discrepancy between the lengthy duration of mealtimes reported by mothers and those actually observed in the home (mean, 19 minutes 21 seconds; range, 5 minutes 21 seconds to 41 minutes 39 seconds). In 60% of the children, severe feeding problems preceded the diagnosis of cerebral palsy. CONCLUSIONS: Using a standardized assessment of oral motor function, we found the majority of children to have clinically significant oral motor dysfunction. Contrary to maternal report, mealtimes were relatively brief, and this, combined with the severity of oral motor dysfunction, made it difficult for some children to achieve a satisfactory nutritional intake. The study illustrates the importance of observing feeding, preferably in the home.
[Reilly, S., Skuse, D. & Poblete, X. (1996). Prevalence of feeding problems and oral motor dysfunction in children with cerebral palsy: A community survey. Journal of Pediatrics, 129(6), 877-82.]
6. Classification of eating impairments based on eating efficiency in children with cerebral palsy
Eating impairments in children with cerebral palsy (CP) may vary widely from mild to severe. Accurate diagnosis of the severity of eating has been hampered by the lack of a classification system which would permit stratification of this wide range of problems into mild, moderate, and severe. We propose such a classification system based on measures of (1) growth: weight, height, and skinfold thickness and (2) eating skills: eating efficiency and oral-motor skills. One hundred children, 54 boys and 46 girls from 2 to 16 years, who had various degrees of CP and eating impairments, were studied. Classification, treatment effectiveness, as well as the implications for growth monitoring, based on this classification system are discussed.
[Gisel, E. G. & Alphonce, E. (1995). Classification of eating impairments based on eating efficiency in children with cerebral palsy. Dysphagia, 10(4), 268-74.]
7. Feeding problems in children with cerebral palsy
OBJECTIVE: To determine the magnitude and extent of feeding problems in children with cerebral palsy (CP) and to evaluate the effectiveness of nutritional interventions. DESIGN: Prospective hospital based interventional study. METHOD: Children with cerebral palsy of either sex were enrolled randomly and their parents were interviewed for their perception about feeding problems, nutritional status and for their views about the expected outcome of feeding problems. Each case was assessed for feeding problems based on Gisel and Patrick feeding skill score; for nutritional status by measurement of weight, skinfold thickness (at biceps, triceps, suprailiac and subscapular), mid arm circumference and caloric intake; neurologically for type and severity of cerebral palsy and for developmental age by Gasell s developmental scale. Equal number of age and sex matched controls were included for comparison of nutritional status and developmental quotient. Various rehabilitation procedures were applied and their response was observed in the followup ranging from 3-10 months. RESULTS: One hundred children (76 boys and 24 girls) with cerebral palsy of mean age 2.5 years (range 1 to 9 years) and mean developmental age of 7.6 months (range 1 to 36 months) were included in the study. Oral motor dysfunction (OMD) was found in all cases and in each category. Spastic quadriplegic cerebral palsy (SQCP) and hypotonic patients had significantly poor feeding skill score (p < 0.001). Mean duration of feeding session was 31.5 minutes (range 10-60 minutes). Main food of children with cerebral palsy consisted of liquid and semisolid diet. Children with poor OMD were unable to take solid food. Cases with seizures had significantly more feeding problems than those without seizures (p < 0.001). Parental awareness about feeding problems of their children was significantly low and they overestimated the nutritional status of their children. Anthropometric indicators were significantly lower than controls (p < 0.001). Spastic quadreparesis, hypotonia and poor feeding skill score had negative effect on nutritional status. Thirty per cent parents of cerebral palsy patients were pessimistic about the possibility of any improvement in feeding problems. After nutritional rehabilitation, good improvement was seen in feeding problems, OMD and nutritional status. CONCLUSION: Nutritional status of children with cerebral palsy is poor due to summation of several factors. Therefore, they should be thoroughly assessed for feeding problems and nutritional status in order to start timely nutritional rehabilitation which can significantly improve their nutritional status and quality of life.
[Gangil, A., Patwari, A. K., Aneja, B., Ahuja, B. & Anand, V. K. (2001). Feeding problems in children with cerebral palsy. Indian Pediatrics, 38(8), 839-46.]
8. Feeding dysfunction is associated with poor growth and health status in children with cerebral palsy
OBJECTIVE: To describe parent-reported feeding dysfunction and its association with health and nutritional status in children with cerebral palsy. DESIGN: Anthropometry was measured and z scores calculated. The Child Health Questionnaire was used to assess health status, and a categorical scale (none to severe) was used to classify subjects according to severity of feeding dysfunction. SUBJECTS: 230 children (9.7+/-4.6 years; 59% boys) with moderate to severe cerebral palsy were recruited from 6 centers in the United States and Canada. STATISTICAL ANALYSES: Descriptive statistics, the Kruskal-Wallis and Pearson chi2 tests. RESULTS: Severity of feeding dysfunction was strongly associated with indicators of poor health and nutritional status. The mean weight z scores were -1.7, -2.5, -3.3, and -1.8 among children with none, mild, moderate, or severe (largely tube-fed) feeding dysfunction, respectively (P= .003). Similar results were observed for height z score (P=.008), triceps z score (P=.03), and poor Global Health score (part of the Child Health Questionnaire) (P<.001). Subjects who were tube fed were taller (P=.014) and had greater body fat stores (triceps z score, P=.001) than orally fed subjects with similar motor impairment. For subjects exclusively fed by mouth, a dose-response relationship was observed between feeding dysfunction severity and poor nutritional status. Subjects with only mild feeding dysfunction had reduced triceps z score (-0.9) compared with those with no feeding problems (-0.3). CONCLUSION: For children with moderate to severe cerebral palsy, feeding dysfunction is a common problem associated with poor health and nutritional status. Even children with only mild feeding dysfunction, requiring chopped or mashed foods, may be at risk for poor nutritional status. Parental report of feeding dysfunction with a structured questionnaire may be useful in screening children for nutritional risk.
[Fung, E. B., Samson-Fang, L., Stallings, V. A., Conaway, M., Liptak, G., Henderson, R. C., Worley, G., O’Donnell, M., Calvert, R., Rosenbaum P., Chumlea, W. & Stevenson, R. D. (2002). Feeding dysfunction is associated with poor growth and health status in children with cerebral palsy. Journal of the American Dietetic Association, 102(3), 361-73.]
9. Videofluoroscopic assessment of dysphagia in children with severe spastic cerebral palsy
Very little has been published about the characteristics and sequelae of dysphagia in children with neurological impairment. The swallowing difficulties encountered by children with spastic cerebral palsy are particularly debilitating and potentially lethal. However, aggressive evaluation and management of their feeding is typically deferred until they are medically or nutritionally compromised. Reports of the use of videofluoroscopy to analyze the swallowing patterns and presence or absence of aspiration in such children are rare. This paper describes the histories and analyzes the videofluorographic swallow studies of 22 patients with the primary diagnosis of severe spastic cerebral palsy. The ages of the subjects ranged from 7 months to 19 years. All had severe dysphagia and were slow, inefficient eaters. Fifteen patients (68.2%) demonstrated significant silent aspiration during their swallow study. Analysis of specific features of their swallowing patterns indicated that decreased or poorly coordinated pharyngeal motility was predictive of silent aspiration. Moderately to severely impaired oral-motor coordination was indicative of severity of feeding complications. Our data suggest that early diagnostic workup, including baseline and comparative videofluoroscopic swallow studies, could be helpful in managing the feeding difficulties in these children and preventing chronic aspiration, malnutrition, and unpleasant lengthy mealtimes.
[Mirrett, P. L., Riski, J. E., Glascott, J. & Johnson, V. (1994). Videofluoroscopic assessment of dysphagia in children with severe spastic cerebral palsy. Dysphagia, 9(3), 174-9.]
10. Characteristics of dysphagia in children with cerebral palsy
Videofluoroscopic modified barium swallow (VMBS) examinations may provide clinically relevant information regarding deglutition in children with cerebral palsy and dysphagia. A retrospective review of clinical evaluations and VMBS studies on 90 consecutive children with cerebral palsy and dysphagia was completed. Most children were referred because of concerns regarding airway protection during oral feedings. Most children had multiple disabilities and 93% were nonambulatory. The majority of children were totally dependent for oral feedings (80%). Oral and pharyngeal phase abnormalities were present in almost all patients. Abnormalities of deglutition were observed only while swallowing specific food textures in the majority of patients. Aspiration of specific food textures was significantly more common than aspiration of all food textures (p < 0.0001). Finally, aspiration was silent in 97% of the patients. VMBS studies can provide clinicians with valuable information regarding the most appropriate food textures and rates of oral feeding for children with cerebral palsy and dysphagia.
[Rogers, B., Arvedson, J., Buck, G., Smart, P. & Msall, M. (1994). Characteristics of dysphagia in children with cerebral palsy. Dysphagia, 9(1), 69-73.]
11. Swallowing disorders in a population of children with cerebral palsy
One of the disabilities in patients with cerebral palsy (CP) is dysphagia. To establish the prevalence of dysphagia in a population of children with CP, and to determine if any factors are related to dysphagia, we studied 56 CP patients, 5-21 years, enrolled in a primary school for the disabled. Fifteen patients (27%) had either radiographic or clinical evidence of dysphagia. These 15 patients were compared to the remaining 41 patients without dysphagia. Using data obtained from chart review and interviews with speech pathologists, several factors that contributed to dysphagia were found. These included: bite reflexes, slowness of oral intake, poor trunk control, inability to feed independently, anticonvulsant medication, coughing with meals, choking, and pneumonia. We also noted trends in the following factors: presence of tongue thrusting, presence of drooling, severity of CP, poor head control, severity of mental retardation, seizures, and speech disorders. Factors not related to the presence of dysphagia include: subject age, cause of CP, and type of CP. Early, aggressive work-up and identification in CP patients with the risk factors outlined above can reduce the associated pulmonary complications.
[Waterman, E. T., Koltai, P. J., Downey, J. C. & Cacace, A. T. (1992). Swallowing disorders in a population of children with cerebral palsy. International Journal of Pediatric Otorhinolaryngology, 24(1), 63-71.]