Background: It is suggested that community based neurorehabilitation can provide benefits to both patients and health care providers in the shape of improved clinical outcomes and lower costs relative to inpatient care in tertiary centres. National guidelines within the UK have also called for care to be delivered ‘closer to home’. However, there is very little research on community based models of care for complex neurological patients and little evidence of how these models can deliver improved outcomes. Content: This article describes the model of care employed by Central Surrey Health and presents data from an audit of clinical records to support its effectiveness. Conclusions: This article has shown that the Neurorehabilitation Pathways Team (NRPT) achieve important clinical improvements for our patients whether treated as inpatients or in the community. As a group of experienced senior clinicians the NRPT accurately predict the likely clinical outcomes for each patient (90–97%) and give health care purchasers accurate predictions of the care pathway each patient will follow (84%). This provides a model of care which meets national guidelines and is potentially delivering both good clinical outcomes and cost savings for the health service compared to the former and more common model of care used in the UK.
Depression is common among people with multiple sclerosis (MS). Research suggests that treatments for depression are effective in this population, though few patients appear to access these treatments. Our objectives were to consider whether persons with MS and significant depressive symptoms, prompted to seek treatment by letter, did so, what professionals they consulted, and the benefits of any treatment obtained. A total of 495 individuals with MS (401 female; 94 male), aged 22-65 years (mean: 45.8 years), were surveyed in three phases at yearly intervals. Significant depressive symptoms were found over the three phases (50-60.2%). Despite a high prevalence of depressive symptoms, few participants sought treatment, even though prompted to do so. Where treatment was sought, general practitioners remained the principal group consulted. Contrary to previous reports of the efficacy of treatment in clinical trials, no strong support for this was found. Future research needs to consider why the majority of people with MS do not seek treatment for depression, and why interventions, which are not clinical trials, seem to lack effectiveness. Multiple Sclerosis 2007; 13: 632-635. http://msj.sagepub.com
A group of 111 patients with Huntington's disease (HD) underwent a minimum of three annual neuropsychiatric assessments, using the Problem Behaviors Assessment for Huntington's Disease (PBA-HD). Longitudinal prevalence of neuropsychiatric symptoms was notably higher than baseline prevalence, suggesting that previous studies may have underestimated the extent of this clinical problem. Moreover, apathy, irritability, and depression were each associated with distinct longitudinal profiles. Apathy progressed over time and across disease stages. Irritability also increased significantly, but only in early stages of HD. Depression did not increase significantly at any stage of disease. The neuropsychiatric syndrome of apathy appears to be intrinsic to the evolution and progression of HD.
Age at onset of diagnostic motor manifestations in Huntington disease (HD) is strongly correlated with an expanded CAG trinucleotide repeat. The length of the normal CAG repeat allele has been reported also to influence age at onset, in interaction with the expanded allele. Due to profound implications for disease mechanism and modification, we tested whether the normal allele, interaction between the expanded and normal alleles, or presence of a second expanded allele affects age at onset of HD motor signs.
Methods:
We modeled natural log-transformed age at onset as a function of CAG repeat lengths of expanded and normal alleles and their interaction by linear regression.
Results:
An apparently significant effect of interaction on age at motor onset among 4,068 subjects was dependent on a single outlier data point. A rigorous statistical analysis with a well-behaved dataset that conformed to the fundamental assumptions of linear regression (e.g., constant variance and normally distributed error) revealed significance only for the expanded CAG repeat, with no effect of the normal CAG repeat. Ten subjects with 2 expanded alleles showed an age at motor onset consistent with the length of the larger expanded allele.
Conclusions:
Normal allele CAG length, interaction between expanded and normal alleles, and presence of a second expanded allele do not influence age at onset of motor manifestations, indicating that the rate of HD pathogenesis leading to motor diagnosis is determined by a completely dominant action of the longest expanded allele and as yet unidentified genetic or environmental factors. Neurology® 2012;78:690–695
There is controversy about the clinical consequences of intermediate alleles (IAs) in Huntington disease (HD). The main objective of this study was to establish the clinical manifestations of IA carriers for a prospective, international, European HD registry.We assessed a cohort of participants at risk with <36 CAG repeats of the huntingtin (HTT) gene. Outcome measures were the Unified Huntington's Disease Rating Scale (UHDRS) motor, cognitive, and behavior domains, Total Functional Capacity (TFC), and quality of life (Short Form-36 [SF-36]). This cohort was subdivided into IA carriers (27-35 CAG) and controls (<27 CAG) and younger vs older participants. IA carriers and controls were compared for sociodemographic, environmental, and outcome measures. We used regression analysis to estimate the association of age and CAG repeats on the UHDRS scores.Of 12,190 participants, 657 (5.38%) with <36 CAG repeats were identified: 76 IA carriers (11.56%) and 581 controls (88.44%). After correcting for multiple comparisons, at baseline, we found no significant differences between IA carriers and controls for total UHDRS motor, SF-36, behavioral, cognitive, or TFC scores. However, older participants with IAs had higher chorea scores compared to controls (p = 0.001). Linear regression analysis showed that aging was the most contributing factor to increased UHDRS motor scores (p = 0.002). On the other hand, 1-year follow-up data analysis showed IA carriers had greater cognitive decline compared to controls (p = 0.002).Although aging worsened the UHDRS scores independently of the genetic status, IAs might confer a late-onset abnormal motor and cognitive phenotype. These results might have important implications for genetic counseling.NCT01590589.
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