Background:Studies of physical therapy and multidisciplinary rehabilitation programs for Huntington’s disease (HD) have shown improvements in gait function, balance, and physical quality of life. There is a gap in the literature on effects of cognitive interventions and the potential to improve cognitive performance.Objective:To assess changes in cognitive performance among patients with early to middle stage HD as secondary analyses from a one-year multidisciplinary rehabilitation program. The program included cognitive stimulation as a non-specific cognitive intervention in addition to physical interventions.Methods:A one-year rehabilitation program that included comprehensive neuropsychological assessments was completed by 31 out 37 participants with early to middle stages of HD. Socio-demographic and clinical information was recorded. A battery of neuropsychological tests was used to measure cognitive functions before and after the intervention. Descriptive statistics was used for sample characteristics. Paired sample t-tests and nonparametric Wilcoxon Signed ranked tests were used to compare cognitive measures at both time points.Results:Scores on the Symbol Digit Modalities Test (SDMT) were significantly lower post intervention. There were no significant differences in all other measures. Scores on the Stroop color naming and California Verbal Learning Test-II (CVLT-II) long-term delayed recall tasks showed tendencies towards lower scores post intervention.Conclusions:An intensive multidisciplinary rehabilitation program for patients with HD was generally well tolerated and feasible, with no indication of negative effects on cognition. Neuropsychological measures overall remained stable following an intensive multidisciplinary rehabilitation program, however continued progression of cognitive impairment was evident on the SDMT, suggesting that disease progression is not halted. Randomized controlled trials are needed to verify these findings.

BAKGRUnnFysisk inaktivitet er en økende utfordring, og inaktiviteten øker i alle alders grupper. Samfunnet vårt har de siste tiårene blitt tilrettelagt for inaktivitet, og man skal i dag vaere bevisst for å få fysisk aktivitet som del av hverdagen. En økning av den fysiske aktiviteten er ett av de tiltak som vil ha størst positiv effekt på folkehelsen. Regelmessig fysisk aktivitet representerer et stort forebyggingspotensiale i forhold til de dominerende sykdomsgruppene. Å få økt bruk av fysisk aktivitet i forebyggende arbeid er derfor vesentlig. Her må helsesektoren ta sitt ansvar, men like viktig er andre sektorer som NAV, oppvekst-og utdanning, areal-og transport, arbeidslivet osv.Videre er det godt dokumentert at bruk av fysisk aktivitet er svaert effektiv i behandling av en rekke sykdommer og tilstander. I en del tilfeller kan fysisk aktivitet erstatte legemidler og i andre redusere behovet for medisiner.Denne rapporten har til hensikt å bidra med nødvendig kunnskap for at dette skal bli en realitet. «Aktivitetshåndboken -fysisk aktivitet i forebygging og behandling» er tenkt som et verktøy i arbeidet med å tilrettelegge for og fremme fysisk aktivitet. Hel setjenesten er en sentral målgruppe, men også andre sektorer vil forhåpentligvis ha stor nytte av boken. Foruten å oppsummere kunnskap om effekt av fysisk aktivitet i forebygging og behandling, og presentere anbefalinger på individ-og gruppenivå, har boken stor fokus på hvordan få til atferdsendring.Helsetjenesten har et gunstig utgangspunkt for å fremme økt fysiske aktivi teten i befolkningen. Dels på grunn av det faktum at en stor andel av befolknin gen årlig er i kontakt med helsetjenesten, men også fordi det er stor troverdighet til den informasjonen helsetjenesten formidler. Helsetjenesten har også kontakt med de grupper i samfunnet som er mest inaktive.Økt fysisk aktivitet gir mange positive ringvirkninger. Forskning viser at per soner som blir fysisk aktive, ofte blir mer bevisst i forhold til andre levevaner også. Dette ser man spesielt i forhold til røykevaner og kosthold.Aktivitetshåndboken er et resultat av samarbeid mellom norske og svenske fagfolk. Boken bygger på anbefalingsmanualen FYSS som Statens Folkhälsoin stitut utga i 2004, og med det utgangspunktet har forfatterne foretatt nye selv stendige søk innenfor aktuelt tema. Hvilke databaser som er benyttet og hvilke typer studier man har inkludert, vil derfor variere noe. Helsedirektoratet har i samråd med Nasjonalt råd for fysisk aktivitet valgt ut de norske forfatterne. Aktivitetshåndboken Fysisk aktivitet i forebygging og behandlingVi vil rekke en stor takk til Statens Folkhälsoinstitut og Yrkesföreningen för fysisk aktivitet for at vi i samarbeid har utviklet denne boken. Jeg vil også rekke benytte anledningen til å takke de norske forfatterne for konstruktivt og posi tivt samarbeid. Avslutningsvis retter jeg er saerskilt takk til prof. dr. med Roald Bahr, som har vaert redaktør for dette omfattende arbeidet.Vi håper at Aktivitetshåndboken vil vaere et nyttig verktøy for deg -enten du b...

Background Huntington’s disease is a complex neurodegenerative hereditary disease with symptoms in all domains of a person’s functioning. It begins after a healthy start in life and leads through the relentless progression over many years to complete care dependency and finally death. To date, the disease is incurable. The long progressive complex nature of the disease demands multiple disciplines for treatment and care of patient and family. These health care providers need inter- and multidisciplinary collaboration to persevere and be efficacious in this devastating disease trajectory. Discussion The position paper outlines current knowledge and experience alongside the experience and consensus of a recognised group of HD multidisciplinary experts. Additionally the patient’s voice is clear and calls for health care providers with a holistic view on patient and family. Building long-term trust is a cornerstone of the network around the patient. This paper describes a managed care network comprising all the needed professionals and services. In the health care system, the role of a central coordinator or case manager is of key importance but lacks an appropriate guideline. Other disciplines currently without guidelines are general practitioners, nurses, psychologists, and social workers. Guidelines for neurologists, psychiatrists, geneticists, occupational therapists, speech and language therapists, physiotherapists, dieticians, and dentists are being discussed. Apart from all these profession-specific guidelines, distinctive inter- and multidisciplinary collaboration requirements must be met. Conclusions and recommendations The complex nature of Huntington's disease demands multidisciplinary treatment and care endorsed by international regulations and the lay association. Available guidelines as reviewed in this paper should be used, made available by a central body, and updated every 3–5 years. Time needs to be invested in developing missing guidelines but the lack of this ‘proof’ should not prevent the ‘doing’ of good care.

Background: Primary periodic paralysis (PPP) are rare inherited neuromuscular disorders including Hypokalemic periodic paralysis (HypoPP), Hyperkalemic periodic paralysis (HyperPP) and Andersen-Tawil syndrome (ATS) characterised by attacks of weakness or paralysis of skeletal muscles. Limited effective pharmacological treatments are available, and avoidance of lifestyle related triggers seems important. Objective: Our aim was to search and assess the scientific literature for information on trigger factors related to nutrition and physical activity in PPP. Methods: We searched Ovid Medline and Embase database for scientific papers published between January 1, 1990, to January 31, 2020. Results: We did not identify published observation or intervention studies evaluating effect of lifestyle changes on attacks. Current knowledge is based on case-reports, expert opinions, and retrospective case studies with inadequate methods for description of nutrition and physical activity. In HypoPP, high carbohydrate and salt intake, over-eating, alcohol, dehydration, hard physical activity, and rest after exercise are frequently reported triggers. Regarding HyperPP, fasting, intake of potassium, alcohol, cold foods or beverages, physical activity, and rest after exercise are frequently reported triggers. No nutrition related triggers are reported regarding ATS, exercise can however induce ventricular arrhythmias. Conclusions: Our results support that dietary intake and physical activity may play a role in causing paralytic attacks in PPP, although the current scientific evidence is weak. To provide good evidence-based patient care, several lifestyle aspects need to be further assessed and described.

BackgroundResearch suggests that physical activity is associated with improved quality of life and motor function in individuals affected by Huntington’s disease (HD). Few studies have assessed the level of physical activity in the general population of people with HD.AimsThis study aimed to describe the self-reported levels of physical activity in individuals with early to mid-stage HD, and to investigate how physical activity is associated with health-related quality of life (HRQoL) and life satisfaction (LS). Methods42 patients in stages I-III living in South-East Norway were included in a cross-sectional study. Physical activity, including sedentary behaviour were recorded using the short form of the International Physical Activity Questionnaire (IPAQ-SF). We assessed HRQoL using the EQ-5D Visual Analogue Scale and LS using the Satisfaction with life scale (SWLS). Socio-demographic and clinical characteristics were also collected. Descriptive and multivariate analyses were performed.Results6 (14%) participants reported high, 21 (50%) medium and 15 (36%) low levels of physical activity. Multivariate regression analyses revealed that physical activity levels (β value = 5.2, p = 0.047) and apathy (β value = −6.5, p = 0.003) were strongest associated with LS and sedentary behaviour (β value = −2.7, p = 0.030), depression (β value = −12.8, p = 0.042) and apathy (β value = −17.5, p = 0.008) were strongest associated with HRQoL.ConclusionsPhysical activity and apathy may impact HRQoL and life satisfaction in individuals with HD. Sedentary behaviour seems to be an important variable on its own, and should be subject for further research.