Huntington’s diseaseBy

Colten ApplebyKristian Nee

Nick Reynoso

Please check the notes section for additional information

Mrs. GMPeriod 82/8/11

Woody Guthrie

Here is a short video on Woody Guthrie, a famous American musician who wrote famous songs such as “This Land is Your Land”. He suffered Huntington’s Disease in his early adult life.

http://player.discoveryeducation.com/index.cfm?guidAssetId=295F0B2E-5F28-49C1-86BA-36A797D21285&blnFromSearch=1&productcode=US

Definition

Taber’s Cyclopedia Medical Dictionary defines Huntington’s Chorea as “an inherited disease of the central nervous system which usually has its onset between 30 to 50 years of age. The patient has progressive dementia with bizarre involuntary movements characteristics of chorea. The disease slowly progressives and death is usually due to an intercurrent* infection.”

The Numbers

• It is estimated that 5 per 100,000 people of European ancestry have HD. The condition appears to be less common in other populations, such as African Americans.

• In United States alone, about 30,000 people have Huntington's disease; about 1 in every 10,000 people is thought to have the condition.

History

• American doctor George Huntington first described the disorder in 1872.

• It is a genetic defect on Chromosome #4.

Diagnosis and Tests

Most important is an accurate medical history of the patient.

Complete physical including neurological exam.Further testing includes Head Cat Scan, Head

MRI scan, and PET scan of the brain which will show brain shrinkage. DNA marker studies are done to determine if you are the carrier of the disease.

Symptoms

• Uncoordinated movements• Loss of muscle control• Personality changes• Loss of learning • Dementia• Slurred speech• Swallowing and eating difficulties

Causes•Huntington’s disease is a dominant gene, meaning you have it or you don’t. Huntington’s disease can not skip a generation like recessive genes can.•If one of your parents has it and your other parent doesn’t have it you have a 50% chance of getting it. If you don’t have the gene and your spouse doesn’t have the gene, all of your child and grandchildren will be fine.

Treatment• Treatment for HD is aimed at controlling symptons not

curing the disease.• At this moment in time there is no known cure for HD.• The U.S. food and drug administration has approved

Tetrabenazine (Xenazine). • Tetrabenazine helps to reduce involuntary movements

by increasing dopamine in the brain.• This drug usually is given once a day to start, and may

gradually increase to up to three times a day.• Due to bad side affects physicians often prescribe the

lowest possible dose. *

Treatment

The treatment for Huntington's disease isthe use of drugs such as:• Antidepressants • Antipsychotics• Mood-stabilizers • Botulinum toxinsThese will not cure Huntington's Disease but will

slow or stop the jerking of limbs, mood swings, and depression.

•  • "Huntington's Disease Symptoms, Causes, and Treatment on MedicineNet.com." MedicineNet.

Web. 07 Feb. 2011. <http://www.medicinenet.com/huntington_disease/article.htm>.•  • Lang, A. "Huntington's Disease: MedlinePlus Medical Encyclopedia." National Library of

Medicine - National Institutes of Health. Web. 07 Feb. 2011. <http://www.nlm.nih.gov/medlineplus/ency/article/000770.htm>.

•  • Staff, Mayo Clinic. "Huntington's Disease - MayoClinic.com." Mayo Clinic. 8 May 2009. Web. 07

Feb. 2011. <http://www.mayoclinic.com/health/huntingtons-disease/DS00401>.•  • The End Of Woody's Life: Looking Back At His Body Of Work. Discovery Education. 1999.

Web. 07 Feb. 2011. <http://player.discoveryeducation.com/index.cfm?guidAssetId=295F0B2E-5F28-49C1-86BA-36A797D21285>.

•  • "What Is Huntington’s Disease?" Science Museum London. Web. 07 Feb. 2011.

<http://www.sciencemuseum.org.uk/WhoAmI/FindOutMore/Yourgenes/Whatcausesgeneticconditions/Whatisdominantinheritance/WhatisHuntingtonsdisease.aspx>.