Nature and development of Huntington disease in a nursing home population: The Behavior Observation Scale Huntington (BOSH).
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.


METHODS
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.
Timman
Reinier
R
Leiden University Medical Center, Department of Neurology, Leiden, The Netherlands. r.timman@erasmusmc.nl
Claus
Hans
H
Slingerland
Henk
H
van der Schalk
Marijanne
M
Demeulenaere
Saskia
S
Roos
Raymund A C
RA
Tibben
Aad
A
D000203:Activities of Daily Living; D000375:Aging / Q000523:psychology; D001519:Behavior; D018450:Disease Progression; D005260:Female; D006801:Humans; D006816:Huntington Disease / Q000523:psychology*; D008297:Male; D008875:Middle Aged; D015233:Models, Statistical; D009483:Neuropsychological Tests / Q000592:standards*; D009735:Nursing Homes; D025341:Principal Component Analysis; D012727:Sex Characteristics; D011795:Surveys and Questionnaires