[Early education for children with Prader-Willi syndrome]. / Préconisations d'education précoce pour les enfants porteurs d'un syndrome de Prader-Willi.

MATERIAL AND METHOD: A cohort of 21 children (under 42 months of age) with Prader-Willi syndrome were followed in a longitudinal way by the referral center team with evaluation of language and cognitive function (every six months). RESULTS: Disorders seen between birth and the second year are primarily related to hypotonia with oropharyngeal dysphagia and impact on the pre-lingual phase of communication. After 2 years the psychomotor delay and the disorders of language acquisition effect development. When compared to patients who had treatment after 4 years and with data published in the literature, those patients with an early diagnosis and early treatment using kinesitherapy and logopedy showed faster linguistic acquisition. However articulation disorders still remain. CONCLUSION AND RECOMMENDATIONS: This work highlights the importance of early readaptation associating kinesitherapy, logopedy and psychomotoricity which can improve the cognitive phenotype of these children.

[Validation of a self assessment for speech disorders (Phonation Handicap Index)]. / Validation d'un questionnaire d'auto-evaluation de la parole (Parole Handicap Index).

OBJECTIVE: The aim of this work is the psychometric validation of a self assessment questionnaire about speech in dysarthria. MATERIALS AND METHODS: A prospective study was performed on 91 persons to analyze reliability and validity of this new questionnaire. It is composed of 25 items subdivided in 3 domains: Physical, functional and emotional. We first compared 17 normal speakers to 21 dysarthric patients. Then we analysed the intra-individual variability with 53 patients who completed the questionnaire twice with two weeks interval between the completion. The statistical analysis verified internal consistency of each item, intra-subject reliability was analyzed through Pearson test and clinical validity was calculated through the non parametric Mann Whitney test. RESULTS: The internal consistency reliability was correct (Cronbach's alpha > 0.9). It appeared a statically significant difference between normal speakers and dysarthric patients (p < 0.05). The correlations to the handicap and severity felt were fair ensured us of the content's validity. However the absence of difference for 5 items drove us to eliminate them. Also, analysis the test / retest by the correlation matrix allowed to delete 5 other questions. The obtained total score was 0,861. As for the validity of contents, the correlations in the handicap and in the degree of severity felt by the dysarthric patients were satisfactory. CONCLUSION: The results of this study allows to resume the validation of this questionnaire, its short form of 15 items is particularly adapted to dysarthric patients. It now remains to test its reliability with the medical evolution of the patients. We propose to name it "Phonation Handicap Index".

[Study of the predictive value of detection tests for silent aspirations]. / Etude de la valeur prédictive des tests de détection des fausses routes silencieuses.

UNLABELLED: Screening for aspiration in patients with swallowing disorders is important in preventing complications. The tests used in this regard are insufficient due to silent aspiration relating to abnormal protective reflexes in many patients with swallowing problems. OBJECTIVE: The aim of this study is to determine the predictive values of simple tests in screening for silent aspiration. MATERIAL AND METHOD: The reference test used was videofluoroscopic examination on swallowing. In the presence of aspiration (FR+) the presence (ME+) or not (ME-) of a cough of throat clearing was noted. The tests being studied were a nasal test with isotonic saline and swallowing according to a set time. RESULTS: For screening for aspiration the presence of a "wet voice" was considered to be a sign of reduced protective reflexes. 1) During the nasal test, the results are 100% for the positive predictive value (VPp) and 83.3% for the negative predictive value (VPn); 2) These results are respectively 84.6% and 35.9% during the swallowing test. Regarding screening for silent aspiration, 1) during the nasal test, the results are 62.5% for the positive predictive value (VPp) and 36.3% for the negative predictive value (VPn); 2) These results are respectively 54.5% and 26.6% during the swallowing test. CONCLUSION: This preliminary study points out the lack of predictive value of the nasal test and the swallow test for the silent aspirations. However the results could be useful for other researchers developing other tests in this area.

[Positioning in the oropharyngeal dysphagia]. / Positionnement dans les dysphagies oropharyngées.

Sitting body posture and the movements for the feeding gesture are interdependent and can worsen an oropharyngeal dysphagia. Their management will have a direct impact on the patient's handicap and will be able to decrease the complications. Clinical process aiming at placing a person having postural disorders, positioning will ensure a base adapted to the person, comfortable, allowing mobility and independence. It requires a methodical accompaniment: evaluation, test, validation, control of adapted equipment for the patient and gesture readjustment.

[Management of swallowing disorders after brain injuries in adults]. / Prise en charge des troubles de déglutition après lésions cérébrales dans un centre de réveil pour adultes.

The management of swallowing disorders after brain injury must be soon as well. The physiopathological analysis and the organization of the therapeutic project of these patients require the intervention of an interdisciplinary team. Dysphagia falls under a complex clinical context associating impairments of cognition, communication and behavioural control. The management associates speech therapist, caregivers, otolaryngolologist, phoniatrician, physiotherapist and nutritional therapist without forgetting the family circle. The fluctuations of consciousness and concentration of our patients brings us to constantly readjusting and rehabilitating the strategies of feeding. Obstacles with their evolution towards a normal feeding are akinesia, limits of motor functions, impairements of cognition and behavioural control. In the located lesions swallow recovers can be fast, instead of in severe brain-injury the challenge is to ensure safe and adequate nutrition, using a variety of strategies depending on the presenting symptoms. The purpose of this article is to relate our experience beside patients with an acute or recent cerbrovascular event.