Dementia: Part III

Wernicke-Korsakoff Syndrome
Wernicke-Korsakoff Syndrome is named for Carl Wernicke and Sergei Korsakoff, the two doctors who first identified it. Wernicke's Encephalopathy and Korsakoff Syndrome are two stages of the same disorder. Symptoms of Wernicke's appear first and as they wear off Korsakoff Syndrome symptoms begin to develop. Damage caused to the brain is due to a lack of Vitamin B1 (Thiamine) and often results from chronic, severe alcoholism. Alcohol breaks down thiamine in the body and does not allow it to absorb properly. Other causes include eating disorders, malnutrition, abnormally high thyroid levels, dialysis (used to treat the kidneys) and diuretic (used to treat congestive heart failure) therapies that last for an extended period of time, and the consequences of some forms of chemotherapy. The most common symptoms of this disease include confusion, hallucinations, short-term memory loss, and permanent gaps in memory. Other symptoms include loss of muscle coordination, unusual eye movements, the incapacity to develop new memories, and confabulation (making up stories). There is no known cure for this disease. The goal of treatment is to control and alleviate symptoms and to prevent the disease from worsening.

Pick's Disease
Pick's Disease is a comparatively rare, degenerative brain disease that was first described by Arnold Pick in 1892. It is marked by a shrinking of the brain cells due to protein build-up in the neurons (nerve cells). It is similar to Alzheimer's Disease and affects the frontal and temporal lobes of the brain. Usually occurring in those between the ages of 50-60 it is the most common form of the Fronto-Temporal dementias and accounts for about 1% - 5% of all dementias. Scientists believe that there may be a genetic component involved in the cause of Pick's Disease but most family members are not affected. The foremost symptoms of this disease are severe behavioral and personality changes, resulting in a decline in social skills and in the ability to carry out daily living activities. Other symptoms include problems with speech and articulation, memory loss, emotional indifference, and lack of empathy. There is no known cure for this disease. Treatment for Pick's Disease is generally the same as that for Alzheimer's, the goal being to manage symptoms through medications and to increase the patient's quality of life.

Creutzfeldt-Jakob Disease
Creutzfeldt-Jakob Disease, commonly referred to as CJD, is also mistakenly known as Mad Cow Disease. It is a degenerative neurological disorder that is caused by a virus that inhibits the brains ability to function normally and can be fatal. It is believed to have a genetic component and affects those aged 40 - 60. It is a rare disease that affects about one in one million people and develops very rapidly, usually over a period of a few months. Symptoms of this disease include difficulty with concentration and attention, vision and coordination, impairments in speech, memory loss and confusion, muscle rigidity and spasms, and problems with appetite. Occasionally there are some visual and non-visual hallucinations associated with this disease. CJD can be transmitted through medical equipment that is infected or during procedures where sterile technique is not employed properly. There is currently no treatment that can control or cure CJD. Some medications, such as steroids, interferon, antiviral agents, and antibiotics, are available to help alleviate the symptoms of this disease. The goal of treatment is to make the patient as comfortable as possible.

Huntington's Disease
Huntington's Disease, also known as Huntington's Chorea, is a genetically inherited disorder. If one parent has it there is a 50% chance that it will be passed on to their child. It is a progressive condition that affects an individual's cognition, behavior, and movement. Huntington's is a disorder in which the neurons (nerve cells) in the brain waste away causing symptoms such as memory loss (this becomes more severe as the disease progresses), problems with coordination and equilibrium (balance), impaired judgment, depression and mood swings, difficulty with speech and articulation (especially slurred speech), uncontrollable movements, gait disturbances (trouble walking), and in some cases delusions and hallucinations. There is no cure for Huntington's Disease but there are medications available to help manage symptoms.

Sources

Schnider, Armin. The Confabulating Mind: How the Brain Creates Reality. New York, NY: Oxford UP, USA; 1 Edition, 2008.

Miller, Bruce L., and Bradley F. Boeve. The Behavioral Neurology of Dementia (Cambridge Medicine). Cambridge, MA: Cambridge UP; 1 Edition, 2009.

Larner, Andrew J. A Dictionary of Neurological Signs. New York, NY: Springer; 2nd Edition, 2005.

ICON. The Official Patient's Sourcebook on Pick's Disease. San Diego, CA: ICON Health Publications, 2002.

Neary, David, Julie Snowden, and David M. Mann. Fronto-Temporal Lobar Degenertaion. Maryland Heights, MO: Churchill Livingstone, 1996.

Stonebrook, Michael J. Creutzfeldt-Jakob Disease: New Research. Hauppauge, NY: Nova Biomedical Books, 2006.

Bates, Gillian, Peter Harper, and Leslie Jones. Huntington's Disease (Oxford Monographs on Medical Genetics, 45). New York, NY: Oxford UP, USA; 3rd Edition, 2002.