More About CJD, Huntington's Disease, Korsakoff Syndrome and Parkinson's Disease

Posted by McKenna Burr on Sep 17, 2015

In Alzheimer's & Dementia, Main

This article below shares more details about different types of dementia. To explore other types of dementia, Click here.

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4. Creutzfeldt- Jakob Disease (CJD) is a fatal brain disorder that affects people and other certain mammals. This occurs with a person whose prion protein begins folding into an abnormal three-dimensional shape. The mis-folded prion protein destroys brain cells which leads to rapid decline in thinking, reasoning, confusion, involuntary muscle movements and difficulty walking. CJD is a type of dementia that gets worse unusually fast.

Prion Protein is found throughout the entire body. The specific function is not yet known for the prion protein but scientists do know that the protein starts to fold into an abnormal three-dimensional shape in the brain. These misfolded proteins eventually start to destroy brain cells in a domino effect.

CJD is so rare that it occurs about 1 in 1 million people annually worldwide which breaks down to about 300 new cases each year in the United States. There is no single test yet to diagnose CJD. The following tests may help to determine if a person has CJD. There is no known specific cause for CJD but there is an estimated 90 percent of those diagnosed with sporadic CJD die within one year. Those affected lose their ability to speak or move and they require full-time daily care.

There are different types of CJD, one being Sporadic CJD. This is made up of 85% of CJD cases. This usually occurs between ages 60 and 65. Familial CJD is another form of this disease. This is when a person inherits the CJD gene from the parent. With this being a dominant gene, so people with this gene will develop this disorder. This covers about 10-15% of cases. The third type is called Infectious CJD, this is caused by an exposure of an external source of abnormal prion. The two most common outside sources are Medical procedures and Meat or other cattle products.

There is no treatment for CJD yet but these tests below can help determine a diagnosis.

– Electroencephalogram (EEG): This test will measure the brain’s pattern of electrical activity.

– Lumbar Puncture (Spinal Tap): This will test the cerebrospinal fluid for the presence of certain proteins.

– Brain Magnetic Resonance Imaging (MRI): This will help detect certain brain changes that are consistent with CJD.

CJD progresses rapidly and those that are affected by the disease lose their ability to move or speak. They will get to the point where they need to seek 24-hour medical help to assist with their daily tasks. An estimated 90% of people diagnosed with sporadic CJD will die within one year. If you know someone who is struggling with this disease, just help assist them with their needs and be there emotionally for them so they know they have your support.

5. Huntington’s Disease is a brain disorder caused by a single defective gene. This defect in this gene is dominant so anyone who inherits it from a parent with Huntington’s disease will eventually develop the disease. This will cause involuntary movements, depression and mood changes, severe decline in thinking, and reasoning skills. About 30,000 Americans have Huntington’s disease which breaks down to 1 in every 10,000 Americans. Scientists are still working on trying to understand the normal function of the Huntingtin protein.

Symptoms of Huntington’s Disease Include:

– Uncontrolled movement of the arms, legs, head, upper body and face.

– Alterations in mood, especially depression, anxiety, and anger.

– Decline in thinking, organization, concentration and memory.

– Possible Obsessive- Compulsive Disorder, repeating same question over and over.

– Symptoms usually develop between ages 30- 50 years old. People diagnosed can live up to 15 to 25 years.

As of right now, there are no known ways to slow down the progress of Huntington's disease but a group of experts joined together to create a first-line strategy for Huntington's. The Chorea (Involuntary movements) can first be treated with an atypical anti-psychotic drug that is approved by the FDA. For a persons irritability, they are able to try a selective serotonin re-uptake inhibitor, type of antidepressant if it is not severe.

6. Korsakoff Syndrome is a chronic memory disorder that is caused by a lack of thiamine. Thiamine helps the brain cells to produce energy from sugar which will help you function properly. If your levels are too low then the brain cells have a hard time functioning which leads to the body not acting accordingly. This is caused by a misuse of alcohol, chronic infections, poor nutrition, AIDS, Cancers and other conditions.

Korsakoff is sometimes followed by an episode of Wernicke Encephalopathy. This is where an acute brain reaction caused by a severe lack of thiamine. Since this can follow of episode of Wernicke Encephalopathy, it is also know as Wernicke- Korsakoff Syndrome.

Symptoms of Korsakoff Syndrome:

- Problems learning new information.

- Difficulty remembering recent events.

- Thinking and Social Skills are usually unaffected.

- A person may make up information they don't remember and believe their lies.

Diagnosis is a clinical diagnosis done by a Doctor, who uses their best judgement after learning about the persons symptoms. When having thiamine deficiency, it causes disruptions in the brain that destroy brain cells and cause microscopic bleeds in the brain. If Korsakoff Syndrome is left untreated, it can cause death up to 20% of cases. This is a disease that can be prevented by those who do not abuse alcohol.

7. Parkinson's Disease Dementia is there is some deterioration in thinking and reasoning. This deterioration happens in the part of the brain that plays a key role in movement. As the damage throughout the brain spreads, their mental functions like memory, making good judgments and breaking down tasks into simple steps. Parkinson's disease is estimated to affect almost 2% of those over the age of 65, that breaks down to 1 million Americans suffering from Parkinson's disease. Up to 50 to 80% of those with Parkinson's disease will also experience the dementia side of this disease.Black-and-white image of senior female being embraced by her husband

Early signs of Parkinson's Disease Dementia include:

- Tremors

- Shakiness

- Muscle stiffness

- Stooped Posture

- Shuffling Steps

For diagnosing Parkinson's dementia, there are a combination of tests that can be completed. Once a person is diagnosed with Parkinson's, the dementia symptoms don't begin to appear until a year or more. If someone is diagnosed with Parkinson's then they are at a greater risk for developing dementia. Since the Parkinson's disease dementia destroys the brain cells, the disease and dementia eventually become worse over time. Since there are no treatments to prevent the damage of the brain, Doctors just work on helping manage the symptoms.

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