Abstract

Since studies on Huntington's disease (HD) mice models have shown a positive impact of environmental enrichment on the performance of affected animals and on disease progression, research in the field of rehabilitative therapies for HD patients has been stimulated and encouraged. A pilot project on intensive multidisciplinary rehabilitation was started more than 10 years ago in Italy. This experience was the first to achieve a quantitative, as well as qualitative, assessment of the effect of rehabilitation therapy in HD patients in the short–medium term over a 2 year period. At present we are completing the data collection of 10 years of rehabilitation experience (from 2000 to 2009) involving about 100 patients, Shoulson stage I–III, with no current severe psychiatric comorbidity or advanced dementia, who underwent a minimum of 1 up to 24 consecutive treatments. The treatment programme included physiotherapy, occupational therapy, cognitive rehabilitation, respiratory exercises and speech therapy. The treatment was timed on 3 weeks of inpatient intensive treatment to be repeated up to three times a year. A standard clinical assessment was performed at the beginning of each admission using: Zung Scale (depression), Mini-Mental State Examination (cognition), Barthel Index (ADL). Tinetti Scale (balance and gait) and Physical Performance Test-PPT (performance on specific tasks) were used to asses motor and functional performance at the beginning and end of each admission. First exploration of outcomes in groups with 1 (n=85), 1–2 (n=68), …, 1–13 (n=12) treatments showed that each 3 week period of intensive rehabilitation treatment had highly positive short term effects on the Tinetti Scale and PPT (p<0.001). A decrease in performance was very small and became significant only after a 2 year period (n=35). Treatment effect was on average 3.6 and 3.9 for Tinetti and PPT, respectively, while the decrease from two consecutive admissions (means 5.95 months after) was on average 0.2 and 0.5. This decline seems inferior to normal decline, suggesting a positive effect of the treatment effect. This is confirmed by the Barthel Index which showed a decrease of 2.2/year. Cognition capacity remained stable over the 5 year period while for depression scores a moderately decrease was apparent.