Olfactory Dysfunction in Huntington's Disease

J Huntingtons Dis. 2021;10(4):413-422. doi: 10.3233/JHD-210497.

Abstract

Olfactory dysfunction is a common symptom in patients with neurodegenerative disorders, including Huntington's disease (HD). Understanding its pathophysiology is important in establishing a preventive and therapeutic plan. In this literature review, we cover the physiology of olfaction, its role in neurodegeneration, and its characteristics in patients with HD. In the general population, olfactory dysfunction is present in 3.8-5.8%and the prevalence increases significantly in those older than 80 years. For HD, data regarding prevalence rates are lacking and the scales used have been inconsistent or have been restructured due to concerns about cross-cultural understanding. Pathogenic huntingtin deposits have been found in the olfactory bulb of individuals with HD, although no studies have correlated this with the grade of olfactory impairment. Olfactory dysfunction is present in both premanifest and manifest patients with HD, showing a progressive decline over time with more severe deficits at advanced stages. No specific treatment for olfactory impairment in HD has been proposed; identifying and avoiding potential medications that cause olfactory dysfunction, as well as general safety recommendations remain the basis of the therapeutic strategy.

Keywords: Huntington’s disease; neurodegenerative diseases; olfaction disorders; smell.

Publication types

  • Review

MeSH terms

  • Humans
  • Huntington Disease* / complications
  • Neurodegenerative Diseases*
  • Olfaction Disorders* / epidemiology
  • Olfaction Disorders* / etiology
  • Smell