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Huntington’s Disease : Dance is in the Gene!

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Probably our biggest fear is the loss of cognition towards oneself, their families and friends. But Huntington’s Disease does exactly that. It makes one incapable of self.

The Backdrop

Huntington’s Disease is poorly understood. But, from what we have gathered so far, this disease encapsulates the brain. It is a rare form of progressive brain disorder. Additionally, it affects movements and can manifest into psychological disorders.

HuntingTon's Disease - brain

Huntington’s Disease is typically inherited. The disease begins in the early thirties or forties. Among the three groups of symptoms, the one which appears first greatly describes the disease progression.

Causes

One inherited gene causes Huntington’s Disease. The gene is autosomal dominant. This mean,s only one copy of the gene is needed for expression.

Huntington's Disease - Dominant

Scientists refer to this as the HTT gene. This gene involves a segment called the CAG nucleotide repeat. Normally, CAG occurs 10-35 times. But in individuals with Huntington’s Disease, the repeat may occur up to 120 times.

Huntington's Disease- CAG

The increase in the CAG segments leads to the increased production of the huntingtin protein. This protein is toxic to neurons. The death and damage to these neurons in certain specific parts of the brain underlie the signs and symptoms of the disease.

Huntington’s Disease Symptoms

Huntington's Disease - Symptoms

  1. Movement

    Also known as Chorea. This type of movement is involuntary and can result in untoward events.

    • Involuntary jerking movements
    • Slow, painful muscle movements
    • Abnormal eye movements
    • Abnormal gait
    • Difficulty with speech
  2. Cognition

    • Difficulty in understanding and prioritizing tasks
    • Lack of impulse control resulting in outbursts and distorted speech
    • Lack of awareness
    • Unable to identify people
    • Slow/Difficulting in finding words
    • Difficulty in understanding new concepts
  3. Psychiatric Disorders

    People with Huntington’s Disease tend to be depressed easily. Depression is a distinguishing feature of HD. Signs and Symptoms include

    • Insomnia
    • Anhedonia
    • Fatigue and Demotivation
    • Frequent contemplation of Death or suicide.
  4. Physical changes

    • Seizures
    • Tremors
    • Changes in fine motor skill

 

With these being said, it is safe to assume that Huntington’s Disease is a rare occurrence. However, the number of cases seem to be on the rise. Prevention is low and slow. Doctors prescribe Genetic Counselling to individuals who have a family history of Huntington’s Disease.

Supportive care is the mainstay for the diagnosed individuals. The most dangerous aspect of HD is the associated depression. This can lead a patient towards eventual suicide. However, Doctors don’t assume Huntington’s as a killer disease. Given the low rate of incidence and the usual late onset, Huntington’s might be better screened in the future.

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