Huntington disease and other choreas

Huntington's disease is a progressive, inherited movement disorder that typically appears in your 30s or 40s; it causes the progressive degeneration of the nerve cells in your brain and has an impact on functional abilities. It usually results in movement, cognitive and psychiatric disorders:
  • The movement disorders associated with Huntington’s disease involve involuntary movements and a loss of control in voluntary actions: 
    • Involuntary jerking or writhing movements; this is the chorea component
    • Rigid or contracted muscles (dystonia)
    • Slow or abnormal eye movements
    • Irregular gait and abnormal posture and balance
    • Difficulty with speech or swallowing
  • Cognitive impairments are also a component of Huntington’s disease: 
    • Difficulty in organizing or focusing on tasks
    • Tendency to get stuck on a thought, concept, behavior or action
    • Lack of impulse control 
    • Lack of self-awareness 
    • Difficulty remembering the word you want to use
    • Difficulty learning new information
  • Changes and damages to the brain cause psychiatric disorders too: 
    • Alternating bouts of depression and mania
    • Irritability
    • Social withdrawal
    • Insomnia
    • Thoughts of death and suicide
    • Obsession and compulsion

People  with Huntington’s disease often experience weight loss.

A chorea is an abnormal involuntary movement disorder known as a dyskinesia; choreas are caused by overactivity of the neurotransmitter dopamine in the areas of the brain that control movement. Choreas may be inherited (such as in Huntington’s disease), occur because of illness (such as Syndenham’s chorea as a complication of rheumatic fever), be induced by drugs (such as levodopa, anticonvulsives and antipsychotics) or caused by metabolic and endocrine disorders (hyperthyroidism) or vascular incidents (stroke).

A chorea is characterized by short bursts of irregular contractions that are not repetitive or rhythmic; the movements seem to flow from one muscle to the next. These movements are frequently accompanied by twisting and writhing motions.  

Other choreas include Sydenham's chorea, a form of chorea contracted from the bacteria Streptococcus and growing increasingly rare; chorea gravidarum, chorea symptoms triggered by bacteria, use of oral contraceptives or previous acute rheumatic fever that appear during pregnancy and then disappear after delivery; and choreas acquired from other sources such as drugs or medications, metabolic conditions and Wilson’s disease (an accumulation of copper in the body).

Diagnosis  of Huntington’s Disease 
If our team suspects Huntington’s disease or a chorea, we will ask you several sets of questions, give you a general physical exam, review your family medical history and put you through some neurological and psychiatric tests.

The neurological exams will test your motor functions, reflexes, muscle tone and strength, coordination and balance, sense of touch, vision and eye movements and hearing. The psychiatric exams will try to determine your mental state and mood, behaviors and patterns, judgment, coping skills and evidence of substance abuse, as well as your memory, ability to reason, mental agility, language skills and spatial reasoning.

Our team may order brain-imaging tests to assess the structure or function of your brain; these may include an MRI  or CT scan. The images can reveal structural changes at the sites of the brain that is normally affected by the disease or they may rule out other conditions with similar symptoms.

Treatment Options for Huntington’s Disease
Although there is no treatment that can change the progression of Huntington’s disease, medications can reduce the symptoms of movement and psychiatric disorders. We can recommend occupational, speech and physical therapy as ways of coping with the effects of Huntington’s disease.

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