Effectiveness of intensive neurorehabilitation in patients with Huntington's disease

Huntington's disease (HD) is a neurodegenerative disorder characterized by motor, cognitive, and behavioral impairments that differ in their presentation and progression across subjects. Studies validating the effectiveness of intensive neurorehabilitation such as a strategy to reducing functional impairments and to improving motor capacities in HD patients are limited and heterogeneous.

To design and test an intensive multifunctional neurorehabilitative protocol in symptomatic patients with HD in the attempt to limit the progression of neurological deficits and to preserve and maintain independence in the activities of daily living.

Case series.

Rehabilitation nursing home.

Thirty-four patients (12 men and 22 women) with HD.

Three-week in-hospital intensive multifunctional neurorehabilitation. The evaluation of patients was performed before and at the end of the 3-week neurorehabilitative treatment by the Barthel Index (BI) and the Total Functional Capacity Scale (TFCS) assessing independence in the activities of daily living, by the Physical Performance Test (PPT) assessing motor performances on functional tasks, and by the Tinetti Scale (TS) assessing balance and gait. A telephone follow-up interview evaluating individual autonomy by the BI was scheduled 3 months after discharge in order to evaluate the short-term results.

We found a significant increase (P<0.001) of the mean scores of BI, TS, PPT and TFCS in all patients at the end of the 3-week in-hospital intensive multifunctional neurorehabilitation with respect to the score values obtained before rehabilitative treatment. The differences of BI, TS, PPT and TFCS scores (Δ scores) observed in HD patients assuming tetrabenazine and in patients not assuming the drug, before and after rehabilitation, were not statistically different. The improvement in independence in the activities of daily living evaluated by BI vanished 3 months after discharge (P<0.05).

Rehabilitative treatment in HD patients needs to be multifunctional and continuous to improve or maintain motor performances and functional independence.

Despite Huntington's disease is a progressive and incurable disease intensive neurorehabilitation lessens patients' disability and improves their quality of life ameliorating autonomy and delaying the progression of motor dysfunction.