Measuring Functional Status in Huntington's Disease

Abstract

Background Neuropsychiatric and cognitive symptoms account for substantial morbidity in Huntington's disease (HD), but their impact on functional status may not be captured using the Total Functional Capacity (TFC) scale. The objective of this study was to assess the impact of motor, cognitive, and neuropsychiatric symptoms on functional status in persons with HD, comparing two instruments. Methods Multiple regression analyses assessed the relationship between neuropsychiatric, cognitive, and motor symptoms and functional status as measured using TFC and Adult Functional Adaptive Behavior (AFAB) scales. Results Greater burden of neuropsychiatric (P = 0.017), cognitive (P = 0.001), and motor (P = 0.001) symptoms was associated with greater impairments to functional status as measured by the AFAB scale. Only motor symptoms were associated with TFC scores (P = 0.002). The 3 symptom domains explained more of the variance in AFAB than TFC scores (P = 0.016). Conclusions TFC may have limited applicability, particularly in early‐stage HD patients, as a measure of functional status. The AFAB scale can be used in HD studies as a more holistic measure of functional status. © 2020 International Parkinson and Movement Disorder Society

Factors Influencing the Total Functional Capacity Score as a Critical Endpoint in Huntington’s Disease Research

“…Hence, our analysis does not support an influence of distinct cognitive and motor aspects on the TFC. Thus, we cannot confirm the independent effects of depression and apathy or cognitive impairments on the TFC as earlier described [29,30]. 4.3.…”

“…After reviewing 14 PubMed-published articles, Sellers et al found some evidence for depression and apathy being associated with decreased functional capacities in Huntington's disease [29]. As a conflicting result, the influence of cognitive burden and neuropsychiatric symptoms was not confirmed by a study by Gibson et al in early-stage HD [30]. A more recently published review concluded that further investigations are necessary to gain a better understanding of the mechanisms caused by CAG-repeat-dependent changes occurring alongside different HD phenotypes and symptoms [31].…”

Estudio de modificadores ambientales epigenéticos asociados a la progresión de la Enfermedad de Huntington, y su relación con la apatía. Aplicaciones socioeducativas prácticas para la terapia ocupacional

“…La gravedad de los síntomas motores se evaluó mediante la escala Unificada de la Enfermedad de Huntington (UHDRS)(69) y el estadio de la enfermedad se obtuvo a partir de las puntuaciones de la Capacidad Funcional Total (TFC) (70).El comportamiento motor y del sueño en términos de actividad física dentro y fuera de casa y las evaluaciones del sueño en casa se midieron utilizando un acelerómetro triaxial (ActiGraph GT3X). El dispositivo se fijó de forma segura a la cintura y los datos se registraron durante siete días.…”

“…Para medir la gravedad de la enfermedad se administraron herramientas estandarizadas de evaluación de la EH, como: UHDRS(69) y TFC (70). La UHDRS incluye evaluación motora, conductual y cognitiva; mientras que la TFC cuantifica la capacidad del paciente de realizar AVDS básicas e instrumentales.…”