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What is Huntington's Disease?
Huntington's Disease is a neurological disorder that causes the brain cells to break down over time. HD is described as having ALS, Parkinson's and Alzheimer's disease simultaneously. Every individual has the HD protein in their DNA, but individuals who specifically have an excessive amount of repeats, 39 and above, carry the faulty gene and will develop symptoms at some point in life, typically between the ages of 30 and 40. Each child of a parent with HD has a 50/50 chance of inheriting the disease.
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Symptoms include: involuntary jerking or movements (chorea), muscle problems, slow or abnormal eye movements, slurred speech, memory issues, balance issues, swallowing issues, weight loss, depression, anxiety, OCD.
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Huntington's Disease affects 41,000 Americans directly while another 200,000 are genetically “at risk” of inheriting the faulty gene that causes the disease.
History of HD
Huntington's Disease was first recognized as an inherited disorder in 1872 when a 22-year-old American doctor, George Huntington, wrote a paper called On Chorea. His paper was later published in the Medical and Surgical Reporter of Philadelphia and the disorder he described became known as Huntington's Chorea. In 1993, the genetic test for HD was created and now at-risk patients were able to test their DNA for the faulty gene. One of the first, and most famous, individuals who suffered from Huntington's was American singer-songwriter, Woody Guthrie. Guthrie died of complications of Huntington's disease on October 3, 1967.
George Huntington. Via Wikipedia
JHD: Juvenile Huntington's Disease
Juvenile Huntington's disease (JHD) is a less common, early-onset form of Huntington's disease that begins in childhood or adolescence, typically present in someone under the age of 20. Juvenile HD only accounts for only 5-10% of overall HD cases. It is a progressive disorder that causes the breakdown of brain cells in certain areas of the brain. Juvenile Huntington's disease has a rapid disease progression once symptoms present.
Symptoms of Juvenile HD include losing the ability to walk, impaired speech, difficulty swallowing, seizures, uncontrolled movement (chorea), depression, OCD, anxiety. Many JHD patients experience changes to their mood and have difficulty managing their behavior.
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Shelby Lentz, President and Founder of Champions for HD, and her sister, Breanna, who was diagnosed with JHD.