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 |
|
createdDate |
2007-10-05 |
|
definingCitation |
Adams et al., Principles of Neurology, 6th ed, pp1060-4 |
|
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. |
|
hasBirnlexCurator | ||
hasCurationStatus | ||
hasDefinitionSource | ||
hasExternalSource | ||
label |
Huntingtons disease |
|
MeshUid |
D006816 |
|
modifiedDate |
April 11, 2009 |
|
nifID |
_8.3_2.10 |
|
preferred label |
Huntingtons disease |
|
synonym |
Huntington's Huntington Chronic Progressive Hereditary Chorea Huntington's disease Huntington's Chorea Chronic Progressive Hereditary Chorea (Huntington) Huntington disease |
|
subClassOf |