BackgroundThe natural history of clinical symptoms in the spinocerebellar ataxias (SCA)s has been well characterised. However there is little longitudinal data comparing cognitive changes in the most common SCA subtypes over time. The present study provides a preliminary longitudinal characterisation of the clinical and cognitive profiles in patients with SCA1, SCA2, SCA3, SCA6 and SCA7, with the aim of elucidating the role of the cerebellum in cognition.Methods13 patients with different SCAs all caused by CAG repeat expansion (SCA1, n = 2; SCA2, n = 2; SCA3, n = 2; SCA6, n = 4; and SCA7, n = 3) completed a comprehensive battery of cognitive and mood assessments at two time points, a mean of 7.35 years apart. All patients were evaluated clinically using the Scale for the Rating and Assessment of Ataxia (SARA) and the Inventory of Non-Ataxia Signs (INAS). Patients underwent structural MRI imaging at follow-up.ResultsClinical scale scores increased in all patients over time, most prominently in the SCA1 (SARA) and SCA3 (INAS) groups. New impairments on neuropsychological tests were most commonly observed with executive functions, speed, attention, visual memory and Theory of Mind. Results suggest possible differences in cognitive decline in SCA subtypes, with the most rapid cognitive decline observed in the SCA1 patients, and the least in the SCA6 patients, congruent with observed patterns of motor deterioration. Minimal changes in mood were observed, and MRI measures of atrophy did not correlate with cognitive decline.ConclusionAs well as increasing physical impairment, cognitive decline over time appears to be a distinct aspect of the SCA phenotype, in keeping with the cerebellar cognitive-affective syndrome. Our data suggest a trend of cognitive decline that is different for each SCA subtype, and for the majority is related to the severity of cerebellar motor impairment.Electronic supplementary materialThe online version of this article (doi:10.1186/s13023-016-0447-6) contains supplementary material, which is available to authorized users.
IntroductionThere is a consistent body of evidence supporting the role of cognitive functions, particularly executive function, in the elderly and in neurological conditions which become more frequent with ageing. The aim of our study was to assess the role of different domains of cognitive functions to predict balance and fall risk in a sample of adults with various neurological conditions in a rehabilitation setting.MethodsThis was a prospective, cohort study conducted in a single centre in the UK. 114 participants consecutively admitted to a Neuro-Rehabilitation Unit were prospectively assessed for fall accidents. Baseline assessment included a measure of balance (Berg Balance Scale) and a battery of standard cognitive tests measuring executive function, speed of information processing, verbal and visual memory, visual perception and intellectual function. The outcomes of interest were the risk of becoming a faller, balance and fall rate.ResultsTwo tests of executive function were significantly associated with fall risk, the Stroop Colour Word Test (IRR 1.01, 95% CI 1.00–1.03) and the number of errors on part B of the Trail Making Test (IRR 1.23, 95% CI 1.03–1.49). Composite scores of executive function, speed of information processing and visual memory domains resulted in 2 to 3 times increased likelihood of having better balance (OR 2.74 95% CI 1.08 to 6.94, OR 2.72 95% CI 1.16 to 6.36 and OR 2.44 95% CI 1.11 to 5.35 respectively).ConclusionsOur results show that specific subcomponents of executive functions are able to predict fall risk, while a more global cognitive dysfunction is associated with poorer balance.
Young patients with impaired gait and balance or medium to severe motor disability appear to be at increased risk of falling. Patients who are relatively independent and still participating in challenging activities have an increased exposure to fall-risk. Walking aids, wheelchair characteristics and environmental hazards are significant environmental risk factors. These risk factors should be monitored closely in the young neurological population to help prevent falls.
The first 2 weeks of admission is a high risk time for fallers, in particular those who become walkers or are cognitively impaired. Prevention policies should be put in place based on fall characteristics. Implications for Rehabilitation The ICF is a valuable instrument for describing subject and environmental factors during a fall-event. Falls are frequent events but do not usually cause serious injuries during inpatient rehabilitation. There is an increased fall risk for subjects with cognitive impairments or those relearning how to walk.
Aim Self‐harm is common among adolescents and young adults and carries increased risk of suicide and other negative outcomes, yet only a small proportion will seek professional help. General practitioners (GPs) are a potentially valuable source of help as most young people visit their GP regularly for other reasons. The primary aim of this research was to explore GPs' empathy and attitudes towards young people who self‐harm and how these are related to GP specialist training. Method GPs completed questionnaires regarding their training, levels of empathy, perceived knowledge and attitudes towards young people who self‐harm. The cross‐sectional design included a random sample of 178 GPs and 47 GPs‐in‐training in the Republic of Ireland. Bootstrapped mediation analysis using structural equation modelling (SEM) was applied to explore the pattern of relationships among GP‐training, empathy, perceived knowledge of self‐harm and attitudes towards youth who self‐harm. Results The SEM results revealed that the model had a very good fit to the data. Empathy was the strongest predictor of attitudes towards self‐harm even if GPs had received youth mental health training and fully mediated the effect of youth mental health training on perceived knowledge of self‐harm. Specialized training in self‐harm was a stronger predictor of perceived knowledge than empathy but had no association with negative attitudes. Conclusions The findings emphasize positive outcomes associated with GP training and highlight potential differences in the effects associated with specific vs general training in youth mental health. These differences may be used to inform the design and implementation of continuing professional development.
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