Background: It is thought that physical health conditions start at a young age in people with profound intellectual and multiple disabilities (PIMD). Knowledge regarding the prevalence, associations and development of these physical health conditions could be used for purposes of prevention as well as appropriate care and support but is currently lacking. Objective: The aim of this study is to gain insight into the prevalence of physical health conditions and associations between these conditions in young children with PIMD. Methods: The study used cross-sectional data related to the physical health conditions of children with PIMD (n = 51, aged between 12 and 61 months). Data were collected in Belgium and in the Netherlands through a checklist filled in by primary caregiver(s). Physical health conditions were classified into categories by the 10th revision of the International Classification of Diseases and Related Health Problems (ICD-10) system. The number of physical health conditions and associations between them were analysed. The analysis focused on prevalence rates and associations represented by odds ratios (p < 0.05). A graphical model was estimated to represent dependencies and conditional dependencies between physical health conditions. Results: We found a mean of 3.8 (range 1–8, SD 1.9) physical health conditions per child. Most of the physical health conditions were found in the ICD-10 chapter ‘Nervous System’, with hypotonia as the most frequent at 70.6%. Five significant large associations were found between spasticity–contractures (OR 9.54); circulatory system–contractures (OR 7.50); scoliosis–contractures (OR 10.25); hearing impairments–skin problems (OR 58.20) and obstipation–hypotonia (OR 19.98). Conclusion: This study shows that at a young age, multiple physical health conditions are present in children with PIMD. In addition, we found five associations between physical health conditions.
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Background: Infantile hereditary proximal spinal muscular atrophy (SMA) type 1 is characterized by onset in the first 6 months of life and severe and progressive muscle weakness. Dysphagia is a common complication but has not been studied in detail. Objective: To study feeding and swallowing problems in infants with SMA type 1, and to explore the relation between these problems and functional motor scores. Methods: We prospectively included 16 infants with SMA type 1 between September 2016 and October 2018. Eleven infants received palliative care and five infants best supportive care in combination with nusinersen. We compiled and used an observation list with feeding related issues and observed feeding sessions during inpatient and outpatient visits. The Children’s Hospital of Philadelphia Infant Test of Neuromuscular Disorders (CHOP INTEND) was used as a measure of motor function. Results: All infants in the palliative care group (median onset of disease 14 days (range 1–56); median inclusion in the study 52 days (range 16–252) demonstrated symptoms of fatigue during feeding and unsafe swallowing. Symptoms were short nursing sessions (10–15 minutes), and not being able to finish the recommended feeding volumes (72%); increased frequency of feeding sessions (55%); coughing when drinking or eating (91%), and wet breathing during and after feeding (64%).Two out of five infants in the nusinersen group (median onset of disease 38 days (range 21–90); inclusion in the study at 63 days (range 3–218) were clinically pre-symptomatic at the start of treatment. The other three infants showed symptoms of fatigue and unsafe swallowing at inclusion in the study. These symptoms initially decreased after the start of the treatment, but (re)appeared in all five infants between the ages of 8 to 12 months, requiring the start tube of feeding. In the same period motor function scores significantly improved (median increase CHOP INTEND 16 points). Conclusion: Impaired feeding and swallowing remain important complications in infants with SMA type 1 after the start of nusinersen. Improvement of motor function does not imply similar gains in bulbar function.
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BACKGROUND: Paratonia is a progressive motor problem that is observed in individuals with dementia and is not a well-known phenomenon. This study explores the development and risk factors of paratonia in moderate stage dementia patients.METHODS: A multi-center, longitudinal, one-year follow-up cohort study was performed. Patients with an established diagnosis of dementia, with a score of 6 or lower on the Global Deterioration Scale (GDS) were included. The participants were assessed using the Paratonia Assessment Instrument (PAI), the Timed Up and GO test, the Qualidem, the Global Deterioration Scale (Reisberg et al., 1982) and the Mini-mental State Examination. Information about each patient's diagnosis of dementia, comorbidities and use of medication were obtained from the participant's medical file. The PAI was assessed every three months, the other variables at baseline and after 12 months. Cross-tabulation χ2 and logistic regression tests were used for the statistical analyses.RESULTS: Baseline measures were assessed in the 204 participants - 111 (54%) female and 93 (46%) male, with a mean age of 79.8 years (56-97). Seventy-one patients (34.8%) were diagnosed with paratonia at baseline, and 51 patients developed paratonia over one year. The highest hazard ratio (3.1) for developing paratonia within one year was observed in the vascular dementia group. The logistic regression analysis revealed that the presence of diabetes mellitus (OR = 10.7) was significantly related to the development of paratonia (Wald χ2 p-value < 0.01).CONCLUSIONS: Diabetes mellitus and likely vascular damage are risk factors for the development of paratonia.
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