Health-related quality of life and supportive care in patients with rare long-term neurological conditions.

PURPOSE: Rare long-term neurological conditions (rLTNCs) may have significant impact on patients' health-related quality of life (HRQL); however, evidence is sparse. We assessed HRQL and access to supportive care in patients with rLTNCs. METHODS: Survey of patients with rare rLTNCs (motor neurone disease, Huntington's disease, cerebellar ataxia, progressive supranuclear palsy, multiple system atrophy, Charcot-Marie-Tooth disease and postpolio syndrome) to assess current access to health and social care, and HRQL using the Euroqol EQ-5D. RESULTS: A total of 266 participants with rLTNCs completed the survey. The HRQL of patients is substantially reduced compared to the general population. Many patients reported pain, were anxious or depressed and experienced problems with mobility, self-care and usual activities (mean EQ-5D index scores ranged from 0.2 to 0.44). Although some patients have accessed rehabilitative services, results suggest care coordination could be improved. CONCLUSIONS: Rare long-term neurological conditions have a significant impact on HRQL. Many patients with rLTNCs do not seem to be accessing the level of health and social care services that could improve their HRQL.

Huntington's disease: current epidemiology and pharmacological management in UK primary care.

BACKGROUND: Recent debate suggests Huntington's disease (HD) may be more prevalent than previously reported. In addition, relatively little is known about current disease management. This study aims to provide epidemiological data and describe the pharmacological management of HD in the United Kingdom. METHODS: A primary care research database was accessed to identify incident and prevalent HD cases between January 1, 2004, and December 31, 2008. Patients with Read codes denoting a definite diagnosis or possible diagnosis, and undiagnosed patients with a positive family history were identified. A subset of patients with a definite diagnosis and prescribed medication indicating symptom onset was also identified. Epidemiological data were estimated. Pharmacological prescriptions to HD patients from 2004 to 2008 were identified, and prescription frequencies were grouped according to the British National Formulary categories. RESULTS: HD incidence estimates ranged from 0.44 to 0.78 per 100,000 person-years, and HD prevalence ranged from 5.96 to 6.54 per 100,000 of the population. Forty-four percent of pharmacological prescriptions targeted the central nervous system. Nearly half of the HD patients were prescribed antidepressants, and over 40% were prescribed analgesics. CONCLUSIONS: Although prevalence estimates fell short of figures suggested in recent debate, it is feasible that the true prevalence may be much higher than previously reported. Pharmacological management appears to rely heavily on central nervous system drugs and nutrition support. Many of these drugs are prescribed to HD patients for reasons other than the medication's primary use. Further work is required to evaluate the impact of alternative management strategies, such as therapist intervention, counselling, and organisation support, on the patients' quality of life.

A systematic review of the incidence and prevalence of long-term neurological conditions in the UK.

BACKGROUND: Updated, robust estimates of the incidence and prevalence of rare long-term neurological conditions in the UK are not available. Global estimates may be misrepresentative as disease aetiology may vary by location. OBJECTIVES: To systematically review the incidence and prevalence of long-term neurological conditions in the UK since 1988. SEARCH STRATEGY: Medline (January 1988 to January 2009), Embase (January 1988 to January 2009), CINAHL (January 1988 to January 2009) and Cochrane CENTRAL databases. SELECTION CRITERIA: UK population-based incidence/prevalence studies of long-term neurological conditions since 1988. Exclusion criteria included inappropriate diagnoses and incomprehensive case ascertainment. DATA COLLECTION AND ANALYSIS: Articles were included based on the selection criteria. Data were extracted from articles with ranges of incidence and prevalence reported. MAIN RESULTS: Eight studies met the criteria (3 on motor neurone disease; 4 on Huntington's disease; 1 on progressive supranuclear palsy). The incidence of motor neurone disease ranged from 1.06 to 2.4/100,000 person-years. The prevalence ranged from 4.02 to 4.91/100,000. The prevalence of Huntington's disease ranged from 4.0 to 9.94/100,000. The prevalence of progressive supranuclear palsy ranged from 3.1 to 6.5/100,000. CONCLUSIONS: The review updates the incidence/prevalence of long-term neurological conditions. Future epidemiological studies must incorporate comprehensive case ascertainment methods and strict diagnostic criteria.

Effects of a physiotherapy and occupational therapy intervention on mobility and activity in care home residents: a cluster randomised controlled trial.

OBJECTIVE: To compare the clinical effectiveness of a programme of physiotherapy and occupational therapy with standard care in care home residents who have mobility limitations and are dependent in performing activities of daily living. DESIGN: Cluster randomised controlled trial, with random allocation at the level of care home. SETTING: Care homes within the NHS South Birmingham primary care trust and the NHS Birmingham East and North primary care trust that had more than five beds and provided for people in the care categories "physical disability" and "older people." PARTICIPANTS: Care home residents with mobility limitations, limitations in activities of daily living (as screened by the Barthel index), and not receiving end of life care were eligible to take part in the study. INTERVENTION: A targeted three month occupational therapy and physiotherapy programme. MAIN OUTCOME MEASURES: Scores on the Barthel index and the Rivermead mobility index. RESULTS: 24 of 77 nursing and residential homes that catered for residents with mobility limitations and dependency for activities of daily living were selected for study: 12 were randomly allocated to the intervention arm (128 residents, mean age 86 years) and 12 to the control arm (121 residents, mean age 84 years). Participants were evaluated by independent assessors blind to study arm allocation before randomisation (0 months), three months after randomisation (at the end of the treatment period for patients who received the intervention), and again at six months after randomisation. After adjusting for home effect and baseline characteristics, no significant differences were found in mean Barthel index scores at six months post-randomisation between treatment arms (mean effect 0.08, 95% confidence interval -1.14 to 1.30; P=0.90), across assessments (-0.01, -0.63 to 0.60; P=0.96), or in the interaction between assessment and intervention (0.42, -0.48 to 1.32; P=0.36). Similarly, no significant differences were found in the mean Rivermead mobility index scores between treatment arms (0.62, -0.51 to 1.76; P=0.28), across assessments (-0.15, -0.65 to 0.35; P=0.55), or interaction (0.71, -0.02 to 1.44; P=0.06). CONCLUSIONS: The three month occupational therapy and physiotherapy programme had no significant effect on mobility and independence. On the other hand, the variation in residents' functional ability, the prevalence of cognitive impairment, and the prevalence of depression were considerably higher in this sample than expected on the basis of previous work. Further research to clarify the efficacy of occupational therapy and physiotherapy is required if access to therapy services is to be recommended in this population. TRIAL REGISTRATION: ISRCTN79859980.

Rehabilitation interventions for foot drop in neuromuscular disease.

BACKGROUND: "Foot drop" or "Floppy foot drop" is the term commonly used to describe weakness or contracture of the muscles around the ankle joint. It may arise from many neuromuscular diseases. OBJECTIVES: To conduct a systematic review of randomised trials for the treatment of foot drop resulting from neuromuscular disease. SEARCH STRATEGY: In this update, we searched the Cochrane Neuromuscular Disease Group Trials Register (April 2009), MEDLINE (January 1966 to April 24 2009), EMBASE January 1980 to April 24 2009), CINAHL (January 1982 to May 6 2009), AMED (January 1985 to April 24 2009), the British Nursing Index (January 1985 to January 2008) and Royal College of Nursing Journal of Databases (January 1985 to January 2008). SELECTION CRITERIA: Randomised and quasi-randomised trials of physical, orthotic and surgical treatments for foot drop resulting from lower motor neuron or muscle disease and related contractures were included. People with primary joint disease were excluded. Interventions included a 'wait and see' approach, physiotherapy, orthoses, surgery and pharmacological therapy. The primary outcome measure was quantified ability to walk whilst secondary outcome measures included range of movement, dorsiflexor torque and strength, measures of activity and participation, quality of life and adverse effects. DATA COLLECTION AND ANALYSIS: Methodological quality was evaluated by two authors using the van Tulder criteria. Four studies with a total of n = 152 participants were included in the update to the original review. Heterogeneity of the studies precluded pooling the data. MAIN RESULTS: Early surgery did not significantly affect walking speed in a trial including 20 children with Duchenne muscular dystrophy. Both groups deteriorated during the 12 months follow-up. After one year, the mean difference (MD) of the 28 feet walking time was 0.00 seconds (95% confidence interval (CI) -0.83 to 0.83) and the MD of the 150 feet walking time was -2.88 seconds, favouring the control group (95% CI -8.18 to 2.42). Night splinting of the ankle did not significantly affect muscle force or range of movement about the ankle in a trial of 26 participants with Charcot-Marie-Tooth disease. Improvements were observed in both the splinting and control groups. In a trial of 26 participants with Charcot-Marie-Tooth disease and 28 participants with myotonic dystrophy, 24 weeks of strength training significantly improved six-metre timed walk in the Charcot-Marie-Tooth group compared to the control group (MD 0.70 seconds, favouring strength training, 95% CI 0.23 to 1.17), but not in the myotonic dystrophy group (MD -0.20 seconds, favouring the control group, 95% CI -0.79 to 0.39). No significant differences were observed for the 50 metre timed walk in the Charcot-Marie-Tooth disease group (MD 1.90 seconds, favouring the training group, 95% CI -0.29 to 4.09) or the myotonic dystrophy group (MD -0.80 seconds, favouring the control group, 95% CI -5.29 to 3.69). In a trial of 65 participants with facioscapulohumeral muscular dystrophy, 26 weeks of strength training did not significantly affect ankle strength. After one year, the mean difference in maximum voluntary isometric contraction was -0.43 kg, favouring the control group (95%CI -2.49 to 1.63) and the mean difference in dynamic strength was 0.44 kg, favouring the training group (95%CI -0.89 to 1.77). AUTHORS' CONCLUSIONS: Only one study, involving people with Charcot-Marie-Tooth disease, demonstrated a statistically significant positive effect of strength training. No effect of strength training was found in people with either myotonic dystrophy or facioscapulohumeral muscular dystrophy. Surgery had no significant effect in children with Duchenne muscular dystrophy and night splinting of the ankle had no significant effect in people with Charcot-Marie-Tooth disease. More evidence generated by methodologically sound trials is required.

An investigation into the utility of the Stroke Aphasic Depression Questionnaire (SADQ) in care home settings.

OBJECTIVE: To assess the utility of the Stroke Aphasia Depression Scale (SADQ) to identify emotional distress among individuals living in a care home setting. DESIGN: A prospective comparison of the SADQ and the depression subsection of the Hospital Anxiety and Depression Scale (HADS). SETTING: Care homes in Oxfordshire. PARTICIPANTS: Eighty-two care home residents without major communication or cognitive problems. MEASURES: Participants completed the HADS. The SADQ was completed on behalf of each resident by his or her principal carer. RESULTS: Spearman's rank correlation between the two measures was statistically significant (r = 0.447, P < 0.01). Receiver operator characteristics plots revealed an optimal cut-off point of 14 on the SADQ which gave sensitivity and specificity of 77% and 78% respectively. CONCLUSION: The SADQ, an observational measure, can identify emotional distress in non-aphasic patients in a care home setting. It would be reasonable to use it with people with communication and cognitive problems who are unable to take part in conventional assessments.