Abstract
Apathy is one of the most common psychiatric symptoms experienced by patients with Huntington’s disease (HD). It appears early, progresses with the disease course and has been shown to contribute significantly to caregiver burden. However, what is understood by apathy in HD is not clearly defined nor the underlying mechanisms responsible for it. In this review, we discuss the concept of apathy in the context of HD and propose that a consensus regarding its conceptualisation and subsequently its diagnostic criteria would significantly benefit the field. In order to undertake this work, we began by reviewing the existing literature on the definition and assessment of apathy in HD, its underlying neurobiological basis and its relationship to other related features such as abulia, anhedonia and alexithymia. In the context of HD, apathy could be described by a loss of or diminished motivation, emotion and goal-directed behaviour that is not best explained by motor or social constraints of the disease. However, there is an urgent need to better understand the characteristics of apathy specifically in HD, how they evolve across the disease course and how they relate to central dopaminergic pathways. Only by undertaking such work can we hope to better understand this early and disabling aspect of HD.
Highlights
Huntington’s disease is a rare autosomal-dominant neurodegenerative disorder caused by the expansion of a CAG triplet in the huntingtin gene, affecting approximately 2.71 people per 100,000 worldwide [1]
We examined work that sought to define the underlying neurobiological substrates for apathy, borrowing from other related conditions such as Parkinson’s disease (PD) to propose avenues for future investigation
There is an extensive list of psychometric scales published to assess apathy [46,62,63], for the purpose of this review we have focused on those which have been published using Huntington’s disease (HD) patients
Summary
Huntington’s disease is a rare autosomal-dominant neurodegenerative disorder caused by the expansion of a CAG triplet in the huntingtin gene, affecting approximately 2.71 people per 100,000 worldwide [1]. It is a fatal condition that typically develops between the ages of 30 and 50 years old [2,3] and progresses over a 20 year period [4] During this time, patients experience a range of problems including a movement disorder [5], cognitive dysfunction and neuropsychiatric symptoms [6]. Apathy is considered a significant problem by patients and caregivers alike who both rank it as one of the three most impactful features of the disease [19] This is apparent from clinical observation and empirical evidence where apathy is negatively associated with patient’s quality of life [24]. Emotion as evidenced by at least 1 of the
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