Prevalence and burden of pain across the entire spectrum of Huntington's disease.

BACKGROUND: Pain is an important symptom in Huntington's disease (HD), however, not systematically studied and understood. The objective of the current study is to assess the prevalence of pain, pain interference in daily activities, painful conditions, analgesic use and the severity of the pain burden across different disease stages and 'Age at symptom Onset' groups. Additionally, the association between pain and disease burden was investigated. METHODS: A cross-sectional analysis was conducted within two large data sets, which included different types of pain scales. Multivariable logistic regression analyses and analyses of variance were performed to compare the pain levels with those in the general population. The analyses were adjusted for sex and age. Locally Estimated Scatterplot Smoothing was used to test the association between pain and the HD pathology score: a measure of disease burden. RESULTS: The mean prevalence of pain in the HD population was 40% and for pain interference around 35% in both data sets. Patients in the early, middle and late stage of HD experience more pain burden compared with what is reported in patients with chronic pain (p<0.01). A positive and significant association was demonstrated between pain and disease burden. Patients in late stage HD with pain use significantly less analgesics compared with the general population (5% vs 13%, respectively (p<0.01)). CONCLUSIONS: Pain is a prevalent and important symptom in HD. Severe pain burden in the HD population is present and positively associated with disease burden. Risk for undertreatment with analgesics is nevertheless present. Awareness of pain in HD needs to be increased, both clinically and scientifically.

[Pain and neurocognitive disorders: current state of the art and remaining challenges]. / Pijn en neurocognitieve stoornissen: stand van zaken en de weg nog te gaan.

The detection and treatment of pain is hampered by cognitive disorders. In this review we discuss the epidemiology of pain in cognitive disorders, and elaborate further on the current state of the art on pain in these populations. We will specifically highlight current gaps and recommendations for the future for the following knowledge domains: 1) Biology and neuropathology,  2) Assessment and evaluation, 3) Treatment and management , and 4) Contextual factors such as the organization and education.  We identify the following knowledge gaps: 1) (Biology): Do pain experience and pain expressions change in different types of cognitive disorders, and how? 2) (Assessment): How to recognize, evaluate and assess pain, in case the self-report of pain is no longer reliable? 3) (Treatment): Which treatment possibilities are effective? How can we organize this in an interdisciplinary way? And how to monitor this? How can we ensure proper implementation of pain assessment and treatment in clinical practice? Specifically with regard to non-pharmacological treatment, how can we communicate observations from different disciplines, family members and clinicians to improve the detection of pain as well as treatment monitoring/evaluation? 4) (Contextual): How can we increase knowledge and skills on pain in cognitive impairment within educational training?

The prevalence of pain in Huntington's disease in a large worldwide cohort.

INTRODUCTION: Pain could be an unknown non-motor symptom in Huntington's Disease (HD). The aim is therefore, to study the prevalence of pain interference, painful conditions and analgesic use across the different stages of HD and compare these levels to non-HD gene mutation carriers. METHODS: A cross-sectional analysis of the Enroll-HD study was conducted in premanifest, manifest HD gene mutation carriers (n = 3989 and n = 7,485, respectively) and in non-HD gene mutation carriers (n = 3719). To investigate group differences, multivariable logistic regression analysis was performed with pairwise comparisons. RESULTS: In the HD mutation carriers, the overall prevalence of pain interference was 34% (95% CI 31%-35%), of painful conditions 17% (95% CI 15%-19%) and analgesic use 13% (95% CI 11%-15%). Compared to non-mutation carriers, the prevalence of pain interference was significantly higher in the middle stage of HD (33% [95% CI 31%-35%] vs 42% [95% CI 39%-45%], P = 0,02), whereas the prevalence of painful conditions was significant lower in the late and middle stage of HD (17% [95% CI 16%-18%] vs 12% [95% CI 10%-14%], 15% [95% CI 13%-17%], P < 0,01]. No significant group difference was present in analgesic use. CONCLUSIONS: The prevalence of pain interference increases as HD progresses, however, the prevalence of painful conditions and analgesics do not increase accordingly. Further studies are necessary to investigate the aetiology of pain in HD and the risk for undertreatment of pain.

[Psychological and psychiatric care for hereditary brain disorders]. / Psychologische en psychiatrische zorg bij erfelijke hersenziekten.

Hereditary neurological disorders are characterised by a combination of neurocognitive, neuropsychiatric and somatic symptoms, with average onset in mid-adulthood. Offspring of gene carriers is at risk of developing the disorder. Patients, partners and family members may present with various psychological problems that engender several care demands throughout life. For physicians and other medical professionals who occasionally come across these disorders it may be difficult to find adequate care. We describe two patients to illustrate the importance of specialised, multidisciplinary psychological and psychiatric care. First, we present a 43-year-old female patient with Huntington's disease who has had long-time psychiatric problems with a strong negative impact on her life and that of her spouse and children. Second, we present a 31-year-old female gene carrier of CADASIL who has psychological problems that are leading to uncertainty about whether the disease process has started.

The prevalence and the burden of pain in patients with Huntington disease: a systematic review and meta-analysis.

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.