Biomaterial Database

Late onset Huntington Disease: clinical and genetic characteristics of 34 cases. 2009

Hillary Lipe, and Thomas Bird

Geriatric Research and Clinical Center, VA Puget Sound Health Care System, Seattle, WA 98108, USA.

We performed a retrospective observational study of thirty-four persons with late onset of Huntington Disease (HD) (onset range 60-79 years). CAG trinucleotide expansion size ranged from 38-44 repeats. Even at this late age a significant negative correlation (r=-0.421, p<0.05) was found between the length of repeat and age of onset. Important characteristics of these older subjects were: (1)Most (68%) were the first in the family to have a diagnosis of HD, (2) Motor problems were the initial symptoms at onset, (3) Disability increased and varied from mild to severe (4) Disease duration was somewhat shorter (12 years) than that reported for mid-life onset, (5) Death was often related to diseases of old age, such as cancer and cerebrovascular disease, (6) Serious falls were a major risk and (7) Global dementia may be associated with coincident Alzheimer disease. Recognizing these characteristics will help physicians and other health care providers better identify and follow the late onset presentation of this disease.

UI	MeSH Term	Description	Entries
D008297	Male		Males
D005260	Female		Females
D006801	Humans	Members of the species Homo sapiens.	Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D006816	Huntington Disease	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. (From Adams et al., Principles of Neurology, 6th ed, pp1060-4)	Huntington Chorea,Juvenile Huntington Disease,Akinetic-Rigid Variant of Huntington Disease,Chorea, Chronic Progressive Hereditary (Huntington),Chronic Progressive Hereditary Chorea (Huntington),Huntington Chronic Progressive Hereditary Chorea,Huntington Disease, Akinetic-Rigid Variant,Huntington Disease, Juvenile,Huntington Disease, Juvenile-Onset,Huntington Disease, Late Onset,Huntington's Chorea,Huntington's Disease,Juvenile-Onset Huntington Disease,Late-Onset Huntington Disease,Progressive Chorea, Chronic Hereditary (Huntington),Progressive Chorea, Hereditary, Chronic (Huntington),Akinetic Rigid Variant of Huntington Disease,Chorea, Huntington,Chorea, Huntington's,Huntington Disease, Akinetic Rigid Variant,Huntington Disease, Juvenile Onset,Huntington Disease, Late-Onset,Juvenile Onset Huntington Disease,Late Onset Huntington Disease
D000203	Activities of Daily Living	The performance of the basic activities of self care, such as dressing, ambulation, or eating.	ADL,Chronic Limitation of Activity,Limitation of Activity, Chronic,Activities, Daily Living,Activity, Daily Living,Daily Living Activities,Daily Living Activity,Living Activities, Daily,Living Activity, Daily
D000368	Aged	A person 65 years of age or older. For a person older than 79 years, AGED, 80 AND OVER is available.	Elderly
D000369	Aged, 80 and over	Persons 80 years of age and older.	Oldest Old
D012720	Severity of Illness Index	Levels within a diagnostic group which are established by various measurement criteria applied to the seriousness of a patient's disorder.	Illness Index Severities,Illness Index Severity
D017668	Age of Onset	The age, developmental stage, or period of life at which a disease or the initial symptoms or manifestations of a disease appear in an individual.	Age-at-Onset,Age at Onset,Onset Age
D018911	Trinucleotide Repeats	Microsatellite repeats consisting of three nucleotides dispersed in the euchromatic arms of chromosomes.	Triplet Repeats,Repeat, Trinucleotide,Repeat, Triplet,Repeats, Trinucleotide,Repeats, Triplet,Trinucleotide Repeat,Triplet Repeat