Huntington’s disease (HD) results from genetically programmed degeneration of brain cells, called neurons, in certain areas of the brain. This degeneration causes uncontrolled movements, loss of intellectual faculties, and emotional disturbance. HD is a familial disease, passed from parent to child through a mutation in the normal gene.
One of its earliest names was chorea, which, as in “choreography,” is the Greek word for dance. The term chorea describes how people affected with the disorder writhe, twist, and turn in a constant, uncontrollable dance-like motion. Later, other descriptive names evolved. “Hereditary chorea” emphasizes how the disease is passed from parent to child. “Chronic progressive chorea” stresses how symptoms of the disease worsen over time. Today, physicians commonly use the simple term Huntington’s disease to describe this highly complex disorder that causes untold suffering for thousands of families.
Each child of an HD parent has a 50-50 chance of inheriting the HD gene. If a child does not inherit the HD gene, he or she will not develop the disease and cannot pass it to subsequent generations. A person who inherits the HD gene will sooner or later develop the disease. Whether one child inherits the gene has no bearing on whether others will or will not inherit the gene.
Some early symptoms of HD are mood swings, depression, irritability or trouble driving, learning new things, remembering a fact, or making a decision. As the disease progresses, concentration on intellectual tasks becomes increasingly difficult and the patient may have difficulty feeding himself or herself and swallowing. The rate of disease progression and the age of onset vary from person to person.
A genetic test, coupled with a complete medical history and neurological and laboratory tests, help physician’s diagnose HD. Presymptomic testing is available for individuals who are at risk for carrying the HD gene. In 1 – 3% of individuals with HD, no family history of HD can be found.
Conditions that suggest Huntington's Disease
Schizophrenia
Huntington’s chorea, a rare, inherited illness is commonly labeled as schizophrenia because of its progression of mental decline. Even when involuntary movements appear, they may be mistaken for drug side effects.
Huntington's disease
Counter Indicators
Huntington's disease
Risk factors for Huntington's Disease
Family history of Huntingtons's
Counter Indicators
No family history of Huntingtons's
Huntington's Disease can lead to
Schizophrenia
Huntington’s chorea, a rare, inherited illness is commonly labeled as schizophrenia because of its progression of mental decline. Even when involuntary movements appear, they may be mistaken for drug side effects.
Recommendations for Huntington's Disease
Glutamine
Please see the link between Alzheimer’s and Glutamine.
Fish Oil / Krill
Huntington’s disease is a devastating illness caused by over-expression of the protein huntingtin. The mechanism of the damage is unknown. There is evidence of increased membrane breakdown and oxidative damage to neurons. This may be associated with excess activity of phospholipase (PL) A2.
Eicosapenaenoic acid (EPA) is an inhibitor of PLA2, A randomised, placebo-controlled double-blind trial of 2gm per day of the 97% pure ethyl ester of EPA, also known as LAX-101, was performed over a 6 month period in patients with end-stage Huntington’s disease.
The overall condition of each patient was assessed on the Unified Huntinngton’s Disease Rating Scale (UHDRS) and the brain of each patient at the beginning and end of the study was assessed by magnetic resonance imaging (MRI). Eight patients entered the study but one died prior to randomisation.
At the end of the study, four patients were rated as deteriorated and three as improved. The four patients who deteriorated were on placebo while the three who improved were on ethyl EPA.
All patients had MRI scans at the beginning and end of the study but, because of the movement disorders, the before and after pairs were evaluable in only four patients. In the two patients on placebo, ventricular size had increased, indicating disease progression. In contrast, in the two patients on ethyl-EPA ventricular size decreased consistent with some recovery of brain mass.
Ethyl-EPA is the first treatment shown to be beneficial in Huntington’s disease in a placebo-controlled study. Whether fish oil, which contains EPA, would be beneficial or not remains to be seen.
Vitamin E
Dr. Abram Hoffer has treated an advanced case of Huntington’s Chorea and returned him to apparent normalcy with large amounts of vitamin E (4,800IU per day) along with niacin.
Key
| Weak or unproven link |
| Strong or generally accepted link |
| Proven definite or direct link |
| Strongly counter-indicative |
| Very strongly or absolutely counter-indicative |
| Likely to help |
| Highly recommended |
| May have adverse consequences |
Glossary
Chorea
Chorea is seen as rapid, purposeless, involuntary movements in the extremities and the face. Chorea is a neurologic syndrome that may appear several months after infection with Streptococcus Type A, the agent causing rheumatic fever..
Chronic
Usually Chronic illness: Illness extending over a long period of time.
Schizophrenia
Any of a group of psychotic disorders usually characterized by withdrawal from reality, illogical patterns of thinking, delusions, and hallucinations, and accompanied in varying degrees by other emotional, behavioral, or intellectual disturbances. Schizophrenia is associated with dopamine imbalances in the brain and defects of the frontal lobe and is caused by genetic, other biological, and psychosocial factors.