Brain Sciences

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Special Issue Editors

Dr. Marianna Delussi

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Guest Editor

Applied Neurophysiology and Pain Unit, Basic Medical Sciences, Neurosciences and Sense Organs Department "Aldo Moro" University, Bari, Italy
Interests: neuropsychology; psychophysiology and signal analysis in neurodegenerative disease
Special Issues, Collections and Topics in MDPI journals

Prof. Dr. Elena Salvatore

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Co-Guest Editor

Università degli Studi di Napoli Federico II, Naples, Italy
Interests: neuroimaging; neurodegenerative disease

Dr. Giulia Paparella

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Co-Guest Editor

IRCCS Neuromed, Pozzilli, IS, Italy
Interests: movement disorders; clinical neurophysiology; motor control; kinematic analysis of movement

Special Issue Information

Dear Colleagues,

Huntington’s disease (HD) is a progressive neurodegenerative illness with involuntary movements, cognitive decline, and varying degrees of behavioral and psychiatric dysfunction. Pain may be a minor problem compared with other patient’s symptoms; nevertheless, it could play a key role in the quality of life of affected individuals. The scientific literature shows contingent neurophysiological and neuroanatomical evidence regarding the progressive decrease in the prevalence of pain in HD progression and a scarcity of standardized neuropsychological assessment tools for measuring the pain variable in these patients. This may lead to inadequate recognition of pain and hence lack of treatment. Few and often conflicting studies have examined this topic. Given the relevance of pain in neurodegenerative disorders, the comprehensive understanding of mechanisms and predisposing factors, the application and validation of specific scales and new specific therapeutic trials are needed. The main purpose of this Special Issue is to collect studies that can shed light on the prevalence and mechanisms of pain in HD. As a common feature, the studies will rely upon theories in cognitive processing, and evidence from neuroanatomical and neurophysiological patterns to allow a better understanding of how pain works in HD. This Special Issue can also be read as an invitation to the scientific community interested in HD to share common study designs in order to collect data in large databases studies with reliable sample size. Authors are invited to submit relevant original research articles, as well as opinion and review papers. This topic has the ultimate goal to open the doors of the guidelines for assessment and treatment of pain in HD.

Dr. Marianna Delussi
Prof. Dr. Elena Salvatore
Dr. Giulia Paparella
Guest Editors

Manuscript Submission Information

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Keywords

Huntington Disease
pain
neuroanatomy
neuropsychology
psychophysiology
cognitive processing
pain assessment
pain treatment

Published Papers (2 papers)

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Research

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13 pages, 2776 KiB

Open AccessArticle

Lower Prevalence of Chronic Pain in Manifest Huntington’s Disease: A Pilot Observational Study

by Marianna Delussi, Vittorio Sciruicchio, Paolo Taurisano, Francesca Morgante, Elena Salvatore, Isabella Pia Ferrara, Livio Clemente, Chiara Sorbera and Marina de Tommaso

Brain Sci. 2022, 12(5), 676; https://doi.org/10.3390/brainsci12050676 - 21 May 2022

Cited by 2 | Viewed by 1884

Abstract

Pain is a minor problem compared with other Huntington Disease (HD) symptoms. Nevertheless, in HD it is poorly recognized and underestimated. So far, no study evaluated the presence of chronic pain in HD. The aim of this pilot study was to evaluate the presence and features of chronic pain in a cohort of HD gene carriers. An observational cross-sectional study was conducted in a cohort of HD gene carriers compared to not gene carriers (n.134 HD subjects, n.74 not gene mutation carriers). A specific pain interview, alongside a neurological, cognitive and behavioural examination, was performed in order to classify the type of pain, subjective intensity. A significant prevalence of “no Pain” in HD was found, which tended to increase with HD progression and a reduced frequency of pain in the last 3 months. A clear difference was found between manifest and premanifest HD in terms of intensity of pain, which did not change significantly with HD progression; however, a tendency emerges to a progressive reduction. No significant group difference was present in analgesic use, type and the site of pain. These findings could support a lower prevalence of chronic pain in manifest HD. Prevalence and intensity of chronic pain seem directly influenced by the process of neurodegeneration rather than by an incorrect cognitive and emotional functioning. Full article

(This article belongs to the Special Issue Huntington's Disease and Pain: Clinical Outcome, Cognitive Processing, Neurophysiological Pattern and Neuroanatomical Basis)

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Review

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26 pages, 2644 KiB

Open AccessEditor’s ChoiceReview

Pathogenesis of Huntington’s Disease: An Emphasis on Molecular Pathways and Prevention by Natural Remedies

by Zainab Irfan, Sofia Khanam, Varnita Karmakar, Sayeed Mohammed Firdous, Bothaina Samih Ismail Abou El Khier, Ilyas Khan, Muneeb U. Rehman and Andleeb Khan

Brain Sci. 2022, 12(10), 1389; https://doi.org/10.3390/brainsci12101389 - 14 Oct 2022

Cited by 14 | Viewed by 3235

Abstract

Background: Huntington’s disease is an inherited autosomal dominant trait neuro-degenerative disorder caused by changes (mutations) of a gene called huntingtin (htt) that is located on the short arm (p) of chromosome 4, CAG expansion mutation. It is characterized by unusual movements, cognitive and psychiatric disorders. Objective: This review was undertaken to apprehend biological pathways of Huntington’s disease (HD) pathogenesis and its management by nature-derived products. Natural products can be lucrative for the management of HD as it shows protection against HD in pre-clinical trials. Advanced research is still required to assess the therapeutic effectiveness of the known organic products and their isolated compounds in HD experimental models. Summary: Degeneration of neurons in Huntington’s disease is distinguished by progressive loss of motor coordination and muscle function. This is due to the expansion of CAG trinucleotide in the first exon of the htt gene responsible for neuronal death and neuronal network degeneration in the brain. It is believed that the factors such as molecular genetics, oxidative stress, excitotoxicity, mitochondrial dysfunction, neuroglia dysfunction, protein aggregation, and altered UPS leads to HD. The defensive effect of the natural product provides therapeutic efficacy against HD. Recent reports on natural drugs have enlightened the protective role against HD via antioxidant, anti-inflammatory, antiapoptotic, and neurofunctional regulation. Full article

(This article belongs to the Special Issue Huntington's Disease and Pain: Clinical Outcome, Cognitive Processing, Neurophysiological Pattern and Neuroanatomical Basis)

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