Huntington's Chorea Disease

BASICS

DESCRIPTION
Huntington's chorea is an inherited disease characterized by dementia and spasmodic muscle movement. It has a gradual onset and slow progression. Symptoms usually do not develop until after 30 years of age. By the time the disease is diagnosed, the patient has usually had children and passed the disease to another generation.
SIGNS AND SYMPTOMS
  • Spasmodic, involuntary movement of the limb and facial muscles
  • Difficulty swallowing
  • Difficulty speaking
  • Impaired memory, judgment
  • Intellectual decline
  • Emotional disturbances, mood swings
  • Depression, anxiety, mania
  • Delusions
  • Agitation, aggression
  • Urinary incontinence
  • Bowel incontinence
  • Weight loss
  • Difficulty walking
  • Unsteadiness
  • Hyperkinesia
  • Abnormal eye movements
  • Facial twitching
  • Apathy, withdrawal
  • Dementia
  • Rigidity
  • Mania
  • Hallucinations
  • Delusions
  • Paranoia
  • Impulsiveness
CAUSES
Hereditary genetic defect
RISK FACTORS
Family history

DIAGNOSIS

WHAT THE DOCTOR LOOKS FOR
  • The doctor will perform a physical examination to identify the signs and symptoms of Huntington's chorea.
  • The doctor may take a detailed family medical history.
TESTS AND PROCEDURES
  • Blood tests
  • Genetic tests
  • Special imaging procedures may be used to assist in diagnosis, including computed tomography (CT scan), magnetic resonance imaging (MRI), or positron emission tomography (PET).

TREATMENT

GENERAL MEASURES
  • Huntington's chorea is managed in the outpatient setting.
  • Genetic counseling should be considered.
  • Electroconvulsive therapy (ECT) for drug-resistant depression may be considered.
  • Speech and occupational therapy may be of benefit.
ACTIVITY

Full activity as long as possible

DIET

No special diet, but soft diet with liquid supplements may be needed.

FOLLOW UP

PREVENTION/AVOIDANCE

Genetic counseling

POSSIBLE COMPLICATIONS

Haloperidol (Haldol)

WHAT TO EXPECT

The outcome is poor; disease leads to progressive impairment and is fatal within 20 years.

MISCELLANEOUS

PREGNANCY

Usually does not occur until after puberty

OTHER NOTES

N/A

Clinical Investigations

ROLE OF HOMOEOPATHY

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