The Addenbrooke’s Cognitive Examination-Revised accurately detects cognitive decline in Huntington’s disease

Abstract

Cognitive features, which begin before manifestation of the motor features, are an integral part of Huntington's disease and profoundly affect quality of life. A number of neuropsychological batteries have been used to assess this aspect of the condition, many of which are difficult to administer and time consuming, especially in advanced disease. We, therefore, investigated a simple and practical way to monitor cognition using the Addenbrooke's Cognitive Examination-Revised (ACE-R) in 126 manifest Huntington's disease patients, 28 premanifest gene carriers and 21 controls. Using this test, we demonstrated a selective decrease in phonemic, but not semantic, fluency in premanifest participants Cognitive decline in manifest Huntington's disease varied according to disease severity with extensive cognitive decline observed in early-stage Huntington's disease patients, indicating that this would be an optimal stage for interventions designed to halt cognitive decline, and lesser changes in the advanced cases. We next examined cognitive performance in patients prescribed antidopaminergic drugs as these drugs are known to decrease cognition when administered to healthy volunteers. We paradoxically found that these drugs may be beneficial, as early-stage Huntington's disease participants in receipt of them had improved attention and Mini-Mental State Examination scores. In conclusion, this is the first study to test the usefulness of the ACE-R in a Huntington's disease population and demonstrates that this is a brief, inexpensive and practical way to measure global cognitive performance in clinical practice with potential use in clinical trials.

Cerebellar Integrity in the Amyotrophic Lateral Sclerosis - Frontotemporal Dementia Continuum

“…The ACE-R is a test that detects early cognitive impairment with 94% sensitivity and 89% specificity [33] and has been well validated across various neurodegenerative diseases [34]–[37]. The participant works through a battery of items designed to reveal levels of functioning across five subscales: attention & orientation, memory, fluency, language and visuospatial cognition.…”

Rating Apathy in Huntington's Disease: Patients and Companions Agree

“…Only the total motor score, functional assessment (FA) and total functional capacity (TFC) sections of the UHDRS were used. Manifest patients were staged according to previously published criteria based on the Total Functional Capacity (TFC) score: scores of between 11 and 13 were classified as early disease, between 7 and 10 as moderate disease and scores of 6 and less as late disease [30].…”

Reduced striato‐cortical and inhibitory transcallosal connectivity in the motor circuit of Huntington's disease patients

“…Manifest HDGEC were defined as carriers of the genetic mutation with ≥ 36 repeats with a diagnostic confidence score (DCS) of 4 on the Unified Huntington's Disease Rating Scale (UHDRS) (Huntington Study Group, ), which corresponds to a confidence ≥ 99% that the motor abnormalities are due to HD. According to criteria based on the Total Functional Capacity (TFC) score [Begeti et al, ], 17 of the manifest HDGEC were at the early stage of the disease (TFC: M = 12.4, SD = 0.6), whereas 4 of them were at moderate stage of the disease (TFC: M = 9.8, SD = 0.4). Premanifest HDGEC were defined as carriers of the genetic mutation with a DCS < 4 on the UHDRS.…”