Rare Medical News
Advertisement
Spotlight On
Huntington disease
Huntington disease is a progressive neurologic disorder that causes uncontrolled movements, emotional problems, and loss of cognition
Prevalence
1-9 / 10 000
3,310-29,790
US Estimated
5,135-46,215
Europe Estimated
Age of Onset
ICD-10
G10
Inheritance
Autosomal dominant
Autosomal recessive
Mitochondrial/Multigenic
X-linked dominant
X-linked recessive
Rare View
Huntington's Disease is a progressive, inherited neurodegenerative disorder caused by a mutation in the HTT gene, which leads to the production of an abnormal form of the huntingtin protein. This faulty protein gradually damages neurons, especially in the basal ganglia—a brain region involved in movement, emotion, and cognition.
5 Facts you should know
FACT
The most common age of onset of the signs and symptoms is 35 to 44 years
FACT
Typical symptoms include uncontrolled movements, loss of intellectual abilities, and emotional and psychiatric problems
FACT
It is also a basal ganglia disease causing a hyperkinetic movement disorder known as chorea
FACT
Chorea is characterized by brief, semi-directed, irregular movements that are not repetitive or rhythmic but appear to flow from one muscle to the next
FACT
Chorea is the defining symptom of Huntington's, and one of the most impactful
Interest over time
Common signs & symptoms
Behavioral disturbances
Clumsiness
Moodiness
Irritability
Dystonia
Involuntary movements
Trouble with balance and walking
Weight loss
Current treatments
Unfortunately, there is currently no cure for Huntington disease (HD). The current goal of treatment is to slow down the course of the disease and help affected people function for as long and as comfortably as possible.[3]
Current treatment strategies involve the use of various medications to treat specific symptoms such as abnormal movements and behaviors. Depression and suicide are more common among affected people, so caregivers should monitor for associated symptoms and seek help if necessary. As symptoms of the disease worsen, affected people need more assistance, supervision, and care.
Deutetrabenazine(Brand name: Austedo)
Manufactured by Teva Branded Pharmaceutical Products R&D, Inc.
Tetrabenazine(Brand name: Xenazine®)
Manufactured by Lundbeck Inc. FDA-approved indication.