Abstract
Parkinson's disease-related pain has increasingly been investigated in research studies. Still, only a few studies have addressed the prevalence and clinical characteristics of pain in neurodegenerative disorders with atypical parkinsonism. The existing evidence, although scarce, suggests that, similarly as in Parkinson's disease, individuals with neurodegenerative diseases with atypical parkinsonism might be predisposed to the development of persistent pain. Today, as the global population is aging and we face an epidemic of neurodegenerative disorders, under-treated pain is taking a great toll on an ever-rising number of people. Here, we provide an up-to-date review of the current knowledge on the prevalence of pain, its clinical features, and findings from experimental studies that might signpost altered pain processing in the most prevalent neurodegenerative disorders with atypical parkinsonism: multiple system atrophy, progressive supranuclear palsy, corticobasal syndrome, frontotemporal dementia, and dementia with Lewy bodies. Finally, we point out the current gaps and unmet needs that future research studies should focus on. Large-scale, high-quality clinical trials, coupled with pre-clinical research, are urgently needed to reveal the exact pathophysiological mechanisms underpinning heightened pain and pave the path for mechanistically-driven analgesic interventions to be developed, ultimately leading to an improvement in the quality of life of individuals with neurodegenerative disorders.
Highlights
While Parkinson’s disease (PD) related pain has increasingly received attention and been researched, only a few studies have addressed the prevalence and clinical characteristics of pain in neurodegenerative disorders with atypical parkinsonism [1, 2]
We provide an up-to-date, systematic review of the current knowledge on the experience of pain, a still largely under-researched realm, in the most prevalent neurodegenerative disorders with atypical parkinsonism: multiple system atrophy, progressive supranuclear palsy, cortico-basal syndrome, frontotemporal dementia and dementia with Lewy Bodies
Multiple system atrophy (MSA) is a neurodegenerative disease neuropathologically characterized by α-synuclein– positive oligodendroglial cytoplasmic inclusions and neurodegenerative changes in striatonigral and olivopontocerebellar structures. It may present with predominant parkinsonism (MSA-P) or predominant cerebellar features (MSA-C), in addition to varying autonomic and pyramidal symptoms [22]
Summary
While Parkinson’s disease (PD) related pain has increasingly received attention and been researched, only a few studies have addressed the prevalence and clinical characteristics of pain in neurodegenerative disorders with atypical parkinsonism [1, 2]. The existing evidence suggests that in those disorders, as in PD, pain may be highly prevalent and might have a substantial adverse effect on the quality of life [3,4,5,6]. We provide an up-to-date, systematic review of the current knowledge on the experience of pain, a still largely under-researched realm, in the most prevalent neurodegenerative disorders with atypical parkinsonism: multiple system atrophy, progressive supranuclear palsy, cortico-basal syndrome, frontotemporal dementia and dementia with Lewy Bodies. Focusing on the prevalence of pain, its clinical characteristics, and objective findings that might signpost altered pain processing, we point out the current gaps and unmet needs that should be a center of attention for future research studies (Summarized in Fig. 1 and Table 1, Ref. [7,8,9,10,11,12,13,14,15,16,17,18,19,20])
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