loading...| Preferred Name | Huntingtons disease | |
| Synonyms |
Huntington's Huntington Chronic Progressive Hereditary Chorea Huntington's disease Huntington's Chorea Chronic Progressive Hereditary Chorea (Huntington) Huntington disease |
|
| Definitions |
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. |
|
| ID |
http://uri.neuinfo.org/nif/nifstd/birnlex_12500 |
|
| acronym |
HD |
|
| alternative label |
Huntington's Huntington Chronic Progressive Hereditary Chorea Huntington's disease Huntington's Chorea Chronic Progressive Hereditary Chorea (Huntington) Huntington disease |
|
| createdDate |
2007-10-05 |
|
| definingCitation |
Adams et al., Principles of Neurology, 6th ed, pp1060-4 |
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| definingCitationURI |
http://www.ninds.nih.gov/disorders/huntington/huntington.htm |
|
| definition |
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. |
|
| editorial note | ||
| externalSourceId |
D006816 _8.3_2.10 |
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| hasBirnlexCurator | ||
| hasCurationStatus | ||
| hasDefinitionSource | ||
| hasExternalSource | ||
| label |
Huntingtons disease |
|
| MeshUid |
D006816 |
|
| modifiedDate |
April 11, 2009 |
|
| nifID |
_8.3_2.10 |
|
| note |
A familial disorder inherited as an autosomal dominant trait and characterized by the onset of progressive CHOREA and DEMENTIA in the fourth or fifth decade of life. Common initial manifestations include paranoia; poor impulse control; DEPRESSION; HALLUCINATIONS; and DELUSIONS. Eventually intellectual impairment; loss of fine motor control; ATHETOSIS; and diffuse chorea involving axial and limb musculature develops, leading to a vegetative state within 10-15 years of disease onset. The juvenile variant has a more fulminant course including SEIZURES; ATAXIA; dementia; and chorea. |
|
| preferred label |
Huntingtons disease |
|
| Resource Identifier |
D006816 _8.3_2.10 |
|
| synonym |
Huntington's Huntington Chronic Progressive Hereditary Chorea Huntington's disease Huntington's Chorea Chronic Progressive Hereditary Chorea (Huntington) Huntington disease |
|
| subClassOf |