Ten-year rate of longitudinal change in neurocognitive and motor function in prediagnosis Huntington disease.

Longitudinal studies of neurocognitive function in prediagnosis Huntington disease (pre-HD) have been few, and duration of follow-up has been brief. In this study, 155 individuals at-risk for HD completed a battery of cognitive and motor tasks at two study visits approximately 10 years apart. Participants were classified as: (1) at-risk, without the CAG expansion (healthy controls, NC; n = 112), or (2) CAG expanded (CAG+; n = 43). To examine the rate of decline at different stages of the pre-HD period, participants in the CAG+ group were further characterized as converters (i.e., individuals who developed manifest HD over the course of the study; n = 21) or nonconverters (n = 22), and their performances were compared. The CAG+ group exhibited faster rates of neurocognitive decline over the course of the study, relative to the NC group. In addition, more rapid decline was associated with closer proximity to estimated age of disease onset in the CAG+ group. Faster rates of motor and psychomotor decline were observed in the CAG+ converter group, relative to the nonconverters. These findings suggest that neurocognitive decline in pre-HD, particularly in motor and psychomotor domains, begins insidiously and accelerates as individuals approach disease onset.

Development and exploratory analysis of the Neurorehabilitation Program Styles Survey.

OBJECTIVE: To develop a survey instrument that assesses implementation of key components of outpatient neurorehabilitation programs and test the capacity of this instrument to differentiate between rehabilitation approaches. DESIGN: The Neurorehabilitation Program Styles Survey (NPSS) was administered to 18 outpatient facilities: 10 specialized and 8 discipline-specific outpatient neurorehabilitation programs. Scores were compared between types of programs using independent samples t tests. RESULTS: The NPSS showed good reliability and contrasted groups validity, significantly differentiating between types of programs. CONCLUSIONS: The NPSS holds considerable promise as a tool for distinguishing among different types of brain injury programs, and for assessing the differential effectiveness of specialized versus discipline-specific outpatient brain rehabilitation programs. Future research on the NPSS will assess the stability of the instrument over time, its content validity, and capacity to differentiate the full continuum of neurorehabilitation programs.