It is remarkable that studies focusing on the prevalence and the burden of pain in patients with Huntington disease (HD) are scarce. This may lead to inadequate recognition of pain and hence lack of treatment, eventually affecting the quality of life. The aim of this review is to investigate the prevalence of pain and its burden in HD by performing a systematic literature search. In February 2018, a systematic search was performed in the electronic databases of Pubmed, Embase, Cinahl, Cochrane, and PsycINFO. Studies focusing on patients with juvenile HD were excluded. All other types of study were included without language restrictions. In total, 2234 articles were identified, 15 of which met the inclusion criteria and provided information on 2578 patients with HD. The sample-weighted prevalence of pain was 41.3% (95% confidence interval: 36%-46%). The pain burden, which was measured with the SF-36, is significantly less compared with that in the general population. The sample-weighted mean score on the SF-36 was 84 (95% confidence interval: 81-86), where a score of 100 represents the lowest symptom burden. The results demonstrate that pain could be an important nonmotor symptom in patients with HD, and there are indications that the pain burden could be diminished because of HD. Larger and high-quality prospective cohort and clinical studies are required to confirm these findings. In the meantime, awareness about pain and its burden in patients with HD is warranted in clinical practice.
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