Huntington's Disease

Alternative names: Huntington's chorea

Huntington's disease (formerly Huntington's chorea) is a rare, inherited illness named after American neurologist George Huntington.  It is often mistakenly labeled as "schizophrenia" because of its progression of mental decline.  A chorea is a nervous disorder marked by involuntary movements of the body and face and lack of coordination of the limbs.  Even when involuntary movements appear, they may be mistaken for drug side-effects.

Incidence; Causes and Development; Contributing Risk Factors

Huntington's disease usually appears in midlife, when people are at their prime, aged 30-40.

Huntington's is caused by an error in a section of DNA called the huntingtin gene, which contains the instructions for making a protein (huntingtin) that is vital for brain development.  This error corrupts huntingtin and turns it into a killer of brain cells.

Having a family history of Huntington's disease is a significant risk factor.

Signs and Symptoms

Early symptoms can include irritability, eccentricity and psychosis.  Further signs include paranoia, obstinacy, indifference, euphoria, and violence.  The disorder results in complete mental deterioration.

Treatment and Prevention

It was reported in December, 2017 by a research team at University College London that the defect that causes Huntington's disease has been corrected in patients for the first time, offering hope that it can at last be stopped.  An experimental drug, injected into spinal fluid of 46 trial patients, safely lowered levels of toxic proteins in the brain by silencing the huntingtin gene and stopping production of damaged huntingtin protein.

Several years previously, a Dr. Abram Hoffer claimed to have successfully treated two cases with a nutritional regime.

Prognosis

Patients usually die 10-20 years after symptoms start.