Objective: The Behavior Observation Scale Huntington (BOSH) was developed to obtain a detailed description of the manifestation of Huntington disease (HD) in the final stages.
Background: The Unified Huntington's Disease Rating Scale (UHDRS), developed to assess Huntington patients' clinical capacities, does not differentiate adequately in later stages of the disease. A scale easy to administer by nursing personnel for progression of the disease in later stages was needed.
Method: Two pilot questionnaires preceded the final version of the BOSH. Observers administered the final version twice independently on 91 patients in 4 nursing homes.
Results: The BOSH contains 32 items in 3 subscales: 1) activities of daily living (ADL), 2) social-cognitive functioning, and 3) mental rigidity and aggression. Internal and interrater reliabilities were between 0.83 and 0.95. Rigidity and aggression become more frequent as the disease progresses; later on, this behavior diminishes. Social-cognitive capabilities deteriorate more rapidly in later stages.
Conclusions: This study sought to develop a scale for assessing behaviors and individual differences in later stages of HD. The findings support the use of the BOSH in identifying these behaviors and differences.