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The psychopharmacology of Huntington disease

Petersén, Åsa LU and Weydt, Patrick (2019) 165. p.179-189
Abstract
Huntington disease (HD) is a hereditary neurodegenerative disorder caused by an expanded cytosine–adenine–guanine triplet repeat in the huntingtin gene. The current diagnosis is based on the presence of typical motor signs in combination with a positive gene test. The motor onset of the disease is usually between 30 and 50 years of age, and the disease then progresses over around 20 more years. Nonmotor symptoms and signs such as cognitive decline, metabolic dysfunction, sleep disturbances, as well as psychiatric symptoms are common and can occur many years before motor onset. Psychiatric symptoms include irritability, apathy, depression, anxiety, and OCD. Although there exist no disease-modifying treatment, available pharmacologic drugs... (More)
Huntington disease (HD) is a hereditary neurodegenerative disorder caused by an expanded cytosine–adenine–guanine triplet repeat in the huntingtin gene. The current diagnosis is based on the presence of typical motor signs in combination with a positive gene test. The motor onset of the disease is usually between 30 and 50 years of age, and the disease then progresses over around 20 more years. Nonmotor symptoms and signs such as cognitive decline, metabolic dysfunction, sleep disturbances, as well as psychiatric symptoms are common and can occur many years before motor onset. Psychiatric symptoms include irritability, apathy, depression, anxiety, and OCD. Although there exist no disease-modifying treatment, available pharmacologic drugs often offer significant symptom relief and improve quality of life. Today, there are only two drugs that are approved by the US Food and Drug Association for the treatment of HD. These are the dopamine-depleting drugs tetrabenazine and deutetrabenazine that both target motor symptoms. The current status of best clinical practice for HD is based on expert opinions as well as evidence and/or experience of treating similar symptoms in other conditions. In this chapter, we provide an overview of the complex clinical manifestations of HD and the commonly used psychopharmacologic treatments. (Less)
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author
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organization
publishing date
type
Chapter in Book/Report/Conference proceeding
publication status
published
subject
host publication
Handbook of Clinical Neurology : Psychopharmacology of Neurologic Disease - Psychopharmacology of Neurologic Disease
editor
Reus, V and Lindqvist, D
volume
165
pages
179 - 189
external identifiers
  • pmid:31727211
  • scopus:85074704375
DOI
10.1016/B978-0-444-64012-3.00010-1
language
English
LU publication?
yes
id
1493510b-d6e8-460f-8d76-cc3c51aea2ff
date added to LUP
2020-04-29 12:15:52
date last changed
2022-05-12 02:04:55
@inbook{1493510b-d6e8-460f-8d76-cc3c51aea2ff,
  abstract     = {{Huntington disease (HD) is a hereditary neurodegenerative disorder caused by an expanded cytosine–adenine–guanine triplet repeat in the huntingtin gene. The current diagnosis is based on the presence of typical motor signs in combination with a positive gene test. The motor onset of the disease is usually between 30 and 50 years of age, and the disease then progresses over around 20 more years. Nonmotor symptoms and signs such as cognitive decline, metabolic dysfunction, sleep disturbances, as well as psychiatric symptoms are common and can occur many years before motor onset. Psychiatric symptoms include irritability, apathy, depression, anxiety, and OCD. Although there exist no disease-modifying treatment, available pharmacologic drugs often offer significant symptom relief and improve quality of life. Today, there are only two drugs that are approved by the US Food and Drug Association for the treatment of HD. These are the dopamine-depleting drugs tetrabenazine and deutetrabenazine that both target motor symptoms. The current status of best clinical practice for HD is based on expert opinions as well as evidence and/or experience of treating similar symptoms in other conditions. In this chapter, we provide an overview of the complex clinical manifestations of HD and the commonly used psychopharmacologic treatments.}},
  author       = {{Petersén, Åsa and Weydt, Patrick}},
  booktitle    = {{Handbook of Clinical Neurology : Psychopharmacology of Neurologic Disease}},
  editor       = {{Reus, V and Lindqvist, D}},
  language     = {{eng}},
  pages        = {{179--189}},
  title        = {{The psychopharmacology of Huntington disease}},
  url          = {{http://dx.doi.org/10.1016/B978-0-444-64012-3.00010-1}},
  doi          = {{10.1016/B978-0-444-64012-3.00010-1}},
  volume       = {{165}},
  year         = {{2019}},
}