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Start free with EleplanHuntington disease
ORPHA:399Disease
Also called Huntington chorea
What it is
Huntington disease (HD) is a rare neurodegenerative disorder of the central nervous system characterized by unwanted choreatic movements, behavioral and psychiatric disturbances and dementia.
Key facts
- Prevalence
- 1-9 / 100 000
- Age of onset
- Adolescent, Adult, Childhood, Elderly
- Inheritance
- Autosomal dominant
- Classified as
- Disease
Prevalence figures describe populations, not individuals, and are often estimates from a single study. Where the figure is an incidence or a birth prevalence, it says so.
Signs and symptoms
Common30–79%
30- Abnormality of eye movement
- Abnormality of the sense of smell
- Abnormal libido
- Aggressive behavior
- Agitation
- Anxiety
- Apathy
- Bradykinesia
- Bradyphrenia
- Clumsiness
- Compulsive behaviors
- Delusion
- Depression
- Disinhibition
- Dystonia
- Gait disturbance
- Gait imbalance
- Generalized muscle weakness
- Hallucinations
- Hostility
- Hypokinesia
- Involuntary movements
- Irritability
- Memory impairment
- Myoclonus
- Poor fine motor coordination
- Seizure
- Speech articulation difficulties
- Staring gaze
- Weight loss
Sometimes5–29%
19- Abnormal cerebral white matter morphology
- Abnormality of cholesterol metabolism
- Alcoholism
- Babinski sign
- Caudate atrophy
- Cerebral atrophy
- Choking episodes
- Clonus
and 11 more in this range
These ranges are how often a sign appears across everyone diagnosed, not a prediction for one person — most people have some of these and not others. Orphanet also records findings that are specifically absent in this disease; those are in the data but not listed here.
Genes
1 modifying gene — variants that can change how the disease behaves, not cause it
ICD-10 codes
ICD-10 has no distinct code for most rare diseases, so many are filed under the nearest available one. A shared code identifies the group, not this disease — the ORPHAcode above is what identifies it.
Related health topics
Reached through the ICD-10 categories this disease is filed under, so these describe the broader group rather than this disease specifically.
Cross-references
Orphadata Science: Free access data from Orphanet. © INSERM 1999. Data version 2026-06-23. Available under CC BY 4.0. This is a reference, not medical advice.
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