Development and initial validation of a screening tool for visual ability/performance of people with polyhandicap.

BACKGROUND: Visual impairments are common in people with polyhandicap although they are poorly assessed. However, evaluation of the visual abilities of these people is critical to determining treatment for impairments. OBJECTIVES: To develop and validate an easy-to-use visual-behavioural scale for assessing the visual abilities of people with polyhandicap. METHODS: The development of the Visual Assessment for People with Polyhandicap (VA-PLH) involved 2 steps: i) construction of the scale and ii) field validation. Participant selection criteria were aged > 3 years, age at onset of cerebral lesion < 3 years, a combination of motor impairment and profound intellectual impairment associated with restricted mobility (Gross Motor Function Classification System levels [GMFCS] III, IV or V), and everyday life dependence (Functional Independency Measure [FIM] <55). Vision assessment by both an orthoptist and an ophthalmologist was the reference against which were analysed the items of the scale completed by local health care workers. Acceptability, validity, and reliability were analysed. RESULTS: Amongst the 232 participants included, 217 had a complete assessment, and 33% were < 18 years of age. Ocular abnormalities were reported in 83% of participants. Visual ability was altered or insufficient in 60% of participants. The final version of the VA-PLH included 3 items related to visual reaction (Area Under Curve Receiver Operating Characteristic = 0.83). Participants were considered at-risk if they had at ≥ 1 of 3 signs present (sensitivity 83% and specificity 73%). The scale's reliability was satisfactory CONCLUSION: The VA-PLH scale provides an easy-to-use, reliable and valid measure of visual status for people with polyhandicap and may be used both in clinical practice and clinical research. In addition, this study provides an overview of the diversity of visual impairments in a large population of people with polyhandicap, showing that most experience visual challenges.

Impact of severe polyhandicap cared for at home on French informal caregivers' burden: a cross-sectional study.

OBJECTIVES: Polyhandicap (PLH), defined by a combination of profound intellectual impairment and serious motor deficits, is a severe condition with complex disabilities. In France, care of the large majority of PLH individuals is managed in specialised rehabilitation centres or residential facilities, but some of PLH individuals are cared for at home. The aims of this study were to assess the self-perceived burden among informal caregivers of PLH individuals and to identify potential determinants of this burden. DESIGN: Cross-sectional study (Neuropaediatric Department, Trousseau Hospital, Paris, France). SETTINGS: PLH children were recruited from a specialised paediatric/neurological department. PARTICIPANTS: The selection criteria of caregivers were age above 18 years and being the PLH individual's next of kin. OUTCOMES MEASURES: From March 2015 to December 2016, data were collected from the caregivers, including sociodemographical data, health status, psycho-behavioural data (quality of life, mood disorders and coping) and self-perceived burden. In addition, the health status of the PLH individual was collected. Relationships between the burden scores and potential determinants were tested (correlations coefficients, Mann-Whitney tests, generalised estimating equations models). RESULTS: Eighty-four children were eligible; 77 families returned their questionnaire. The informal caregivers of PLH children experienced a high level of perceived burden (scores ranged from 55±20 to 81±12). Eighty per cent of them had more than 5 hours of daily caregiving and 51% of them had to get up more than twice during the night. The main factors associated with caregiver burden were age, financial issues, health status, daily care and coping strategies. The patients' health status was not associated with caregiver burden. CONCLUSIONS: Some of the caregiver burden determinants might be modifiable. These findings should help healthcare workers and health-decision makers implement specific and appropriate interventions. TRIAL REGISTRATION NUMBER: NCT02400528.

Valproic acid-induced encephalopathy in very long course treated patients.

PRIMARY OBJECTIVE: In patients receiving valproate (VPA) treatment, valproate induced-encephalopathy (VIE) is among the most serious adverse side-effects and hyperammonaemia is a frequent and well-recognized event. This report evaluates adult patients receiving VPA for several years. RESEARCH DESIGN: This study was conducted in adult patients receiving VPA for several years. Many studies have evaluated blood ammonia levels and VPA-related encephalopathy in patients whose treatment was initiated for a few months. Most studies were conducted in children. METHODS AND PROCEDURES: This study looked at retrospectively occurrences of VIE in 63 patients who were receiving VPA for a minimum duration of at least 2 years. At the beginning of the study basal ammonaemia was measured for all patients. MAIN OUTCOMES AND RESULTS: This study reports a rather frequent onset of VIE. Long duration of VPA treatment was not correlated with encephalopathy onset. In seven cases temporary administration of lactulose alone was effective and VPA was continued. CONCLUSIONS: This study confirms that, under VPA treatment, hyperammonaemia is a current event; 25.4% patients presented isolated elevated ammonaemia when receiving VPA for very long durations. VPA therapy should be monitored closely and clinicians must be warned of VPA encephalopathy signs.