scholarly journals Olfactory Dysfunction in Huntington’s Disease

2021 ◽  
pp. 1-10
Author(s):  
Jorge Patino ◽  
Nicholas E. Karagas ◽  
Shivika Chandra ◽  
Nivedita Thakur ◽  
Erin Furr Stimming
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Therapeutic Strategy ◽  
Specific Treatment ◽  
Olfactory Dysfunction ◽  
Cross Cultural ◽  
Common Symptom ◽  
Progressive Decline ◽  
Advanced Stages ◽  
Olfactory Impairment

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.

Sensory- and memory-mediated olfactory dysfunction in Huntington's disease

1995 ◽  
Vol 1 (3) ◽  
pp. 281-290 ◽  
Author(s):  
Steven Nordin ◽  
Jane S. Paulsen ◽  
Claire Murphy
Keyword(s):  
Recognition Memory ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Olfactory Dysfunction ◽  
Detection Sensitivity ◽  
Intensity Discrimination ◽  
Olfactory Sensitivity ◽  
Short Term ◽  
Odor Recognition ◽  
Gender And Education

AbstractNeuropathology in Huntington's disease (HD) known to project to areas that process olfactory information raises the questions of which olfactory function, if any, is most affected in HD, and how to explain such dysfunction in terms of olfactory sensitivity and cognition. These questions were studied by comparing HD patients and controls (matched for age, gender, and education) on absolute detection, intensity discrimination, quality discrimination, short-term recognition memory, and lexical- and picture-based identification for odor. Taste or vision were used as comparison modalities. The results suggest that whereas odor-recognition memory is not affected in patients with HD, these patients have impaired olfactory functioning with respect to absolute detection, intensity discrimination, quality discrimination, and identification. The three latter impairments were significantly explained by poor detection sensitivity. Odor identification was the function most affected. (JINS, 1995, I, 281–290.)

scholarly journals Huntington’s Disease: Recent Advances in Diagnosis and Management

1995 ◽  
Vol 22 (1) ◽  
pp. 5-12 ◽  
Author(s):  
Sarah Furtado ◽  
Oksana Suchowersky
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Trinucleotide Repeat ◽  
Age Of Onset ◽  
Specific Treatment ◽  
Disease Process ◽  
Disease Diagnosis ◽  
Repeat Sequence ◽  
The Central Nervous System ◽  
Degenerative Disorder

ABSTRACT:Huntington’s Disease (HD) is a progressive degenerative disorder of the central nervous system inherited as an autosomal dominant trait. Clinically, the disorder is characterized by choreoathetosis (with age of onset typically in the late thirties or early forties) and neuropsychiatric disturbance. The striatum is particularly vulnerable to the degenerative disease process, with selective loss of medium spiny neurons and decreased levels of associated neurotransmitters, including substance P, GABA, met-enkephalin and dynorphin. Although the underlying pathophysiology is unknown, recent theories concerning pathogenesis have involved mitochondrial abnormalities and excitotoxin-mediated damage. The gene for HD has recently been discovered and characterized as an unstable CAG trinucleotide repeat sequence on the short arm of chromosome 4 (now known as IT 15). The direct test now available for the HD gene has facilitated disease diagnosis, particularly for those with unclear family history or chorea of uncertain origin; presymptomatic testing is also available. Management of affected individuals is unsatisfactory as only symptomatic control is available. However, as the effect of the genetic abnormality may soon be known, specific treatment of the disorder may become available in the near future.

scholarly journals PND59 Cross-Cultural Validation of the Huntington'S Disease Quality of Life Battery for Carers in France and Italy

2012 ◽  
Vol 15 (7) ◽  
pp. A556
Author(s):  
J. Dorey ◽  
A. Aubeeluck ◽  
F. Squitieri ◽  
E. Clay ◽  
E.J.N. Stupple ◽  
...  
Keyword(s):  
Quality Of Life ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Cross Cultural ◽  
Cultural Validation

Activating mitochondrial regulator PGC-1α expression by astrocytic NGF is a therapeutic strategy for Huntington's disease

2012 ◽  
Vol 63 (4) ◽  
pp. 719-732 ◽  
Author(s):  
Li-Wen Chen ◽  
Lin-Yea Horng ◽  
Chia-Ling Wu ◽  
Hui-Ching Sung ◽  
Rong-Tsun Wu
Keyword(s):  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Therapeutic Strategy

Case Studies in Huntington's Disease: Music Therapy Assessment and Treatment in the Early to Advanced Stages

1995 ◽  
Vol 9 (2) ◽  
pp. 13-19 ◽  
Author(s):  
Wendy Magee
Keyword(s):  
Music Therapy ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Case Studies ◽  
Assessment Tool ◽  
Neurological Impairment ◽  
Therapy Assessment ◽  
Group Settings ◽  
Assessment And Treatment ◽  
Advanced Stages

The purpose of this paper is to describe the different stages of Huntington's Disease (HD) and to present methods of music therapy assessment and treatment specifically related to the varying levels of abilities seen in the early to advanced stages of this illness. Whilst these methods are described here in relation to HD, they are also useful when working with clients with other forms of cognitive or neurological impairment. Music therapy, unlike other treatments, provides an active outlet for a person living with HD even during the most advanced stages of the illness. Participation in music therapy can be facilitated for those people who are unable to take part in any other form of activity, thus providing a valuable assessment tool for other disciplines working with the HD client. This paper will present a progressive picture of life for the HD client. Using case studies, it will suggest different music therapy treatment approaches for the varying stages of the illness. Special consideration will be given to assessment when working with clients experiencing cognitive degeneration, and methods for optimising clients' remaining skills within individual and group settings.

Juvenile Huntington's Disease

1991 ◽  
Vol 8 (2) ◽  
pp. 149-153 ◽  
Author(s):  
Brian O'Shea ◽  
Jane Falvey
Keyword(s):  
Positron Emission Tomography ◽  
Huntington's Disease ◽  
Huntington’S Disease ◽  
Racial Differences ◽  
Genetic Research ◽  
Specific Treatment ◽  
Predictive Testing ◽  
Emission Tomography ◽  
Positron Emission ◽  
Juvenile Huntington’S Disease

AbstractA significant minority of cases of Huntington's disease commence before the age of 20 years and these juvenile cases are more likely to demonstrate paternal transmission and a clinical picture dominated by rigidity. Genetic research and positron emission tomography may make early diagnosis easier but predictive testing in childhood is replete with serious problems. There is no specific treatment for this fatal disorder. Juvenile Huntington's disease has a similar pathology and biochemistry to the adult condition despite a shorter course and certain clinical differences, such as rigidity and convulsions. Racial differences are explored.

Sign in / Sign up

Export Citation Format

Share Document