Saturday, April 25, 2009

Alzheimer's Disease

Common Name: Alzheimer's Disease, AD, Senile dementia/Alzheimer's type (SDAT)

Age of Onset: The prevalence of Alzheimer's disease increases with age. AD is most prevalent in people older than 60 years old. However there are a few forms of familial early-onset Alzheimer’s disease can appear as early as in the victim's 30s, but this represents a subgroup of the less than 10% of all familial cases of Alzheimer’s disease.

Duration: Patients can live for many years with Alzheimer's and 20 years or more from the time of diagnosis. After the onset of symptoms, the average length of time for the victim is 4 - 8 years.

Males/Females/Equal: Although AD affects both men and women, studies have shown that Alzheimer's is significantly higher in women than in men.

Particular Ethnic Group: Alzheimer's Disease does not affect a certain ethnic or racial group, and more evidence would have to be collected and obtained in order to make such otherwise conclusions. Studies however do show that African Americans are more likely to develop Alzheimer's than Caucasians, but additional studies have shown that the quality of education and socioeconomic factors that affect a person's access to an education could explain this discrepancy.

Cause: Alzheimer's Disease does not have a singular, proven, determined cause. Instead, the theory that scientists believe is that it may take a combination of genetic, lifestyle and environmental factors to trigger the onset of such diverse symptoms. While the causes of AD are not understood, the physiology of AD and its effect on brain tissue is clear. Alzheimer's disease damages and kills brain cells in 2 ways:
  1. Plaques- clumps of a normally harmless protein called beta-amyloid may interfere with communication between brain cells. Evidence suggests that the abnormal processing of beta-amyloid protein, maybe a significant cause of Alzheimer's Disease.
  2. Tangles- the internal support structure for brain cells depends on the normal functioning of a protein named tau. In people with Alzheimer's, threads of tau protein undergo alterations that cause them to become twisted---which could lead to the neuron deterioration.
Symptoms: Although Alzheimer's is a progressive disease and affects individuals at different rates, the symptoms grows intolerably worse over time, such as:
  • gradual loss of short-term memory
  • anxiety, suspiciousness, agitation
  • confusion
  • difficulties with activities of daily living, such as dressing and bathing
  • difficulty recognizing family and friends
  • forgetting how to use simple, ordinary things like a fork or brush or pencil
  • inability to recognize objects
  • social withdrawn
  • loss of appetite, weight loss
  • routinely misplace things, by placing them in illogical places
  • loss of bladder or bowel control
  • problems finding or speaking the right word
  • loss of speech
  • repetitive speaking or actions
  • sleep disturbances
  • total dependence on a caregiver
  • wandering and pacing
Prognosis (progression of the disease): Although "neurofibrillary tangles" (twisted fragments of protein within nerve cells that clog up the cell), "neuritic plaques" (abnormal clusters of dead and dying nerve cells, other brain cells, and protein), and "senile plaques" (areas where products of dying nerve cells have accumulated around protein) are found in the brains of most elderly people, victims suffering from Alzheimer's have many more of these changes present on their brain masses. These tangles and plagues lead to the destruction of neurons, which leads to a decrease in neurotransmitters; thus upsetting the brain's critical balance of neurotransmitters. By causing both structural and chemical problems in the brain, AD appears to disconnect areas of the brain that normally work together. These disconnections lead to the numerous mental and behavioral symptoms associated with AD; ultimately most AD victims die of infection.

Treatment Options (Rx): As of right now there is no cure for Alzheimer's disease. Doctors will sometimes choose the approach of treating and lessening Alzheimer's many symptoms, such as anxiety, depression, wandering, sleeplessness, and agitation, by prescribing drugs to improve these signs and symptoms. Doctors also often times prescribe one of only two varieties of medications to slow the cognitive decline with Alzheimer's Disease.
  1. Cholinesterase inhibitors: medications that improve the levels of neurotransmitters in the brain, which includes donepezil (Aricept), rivastigmine (Exelon) and galantamine (Razadyne). These medications do not work for everyone, for 50% show no improvement. Some patient quit taking these drugs because of the side effects, which include diarrhea, nausea and vomiting.
  2. Memantine (Namenda): protects brain cells from damage caused by the chemical messenger glutamate and sometimes used with a cholinesterase inhibitor. Memantine's most common side effect is dizziness, but other side effects include increased agitation and delusional behavior.

Doctors also have to take into account the family of the victim with AD because modes of caregiving affect the entire family. Relaxation is an important approach and aim for the caregiver, and exercises such as massages, walks, breathing techniques, and calming music can improve sleep, ease anxiety, and improve the victim's overall behavior.

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Wednesday, April 22, 2009

Amyotrophic Lateral Sclerosis

Proper Name: Amyotrophic Lateral Sclerosis

Common Name: ALS, Lou Gehrig's Disease

Age of Onset: ALS may occur from as early on as teenage years to the late 80s; however, the peak age at onset occurs from 55-75 years, with a mean age of 62 years at diagnosis.

Duration: Amotrophic Lateral Sclerosis is a terminal, progressive neurotransmitter disease that once diagnosed, since there is no cure, death is the final result. ALS affects people in many different ways, for example:

-50% of all victims live 3 years or more,

-25% live 5 years or more, and

-10% live up to 10 years or more

Males/Females/Equal: ALS is slightly more common in males than females, which is represented in this ratio of 1.5-2.0 males: 1 female. This ratio, however, diminishes with increasing age where the number of men and women suffering from ALS is nearly equivalent.

Particular Ethnic Group: Amyotrophic Lateral Sclerosis exhibits zero proven ethnic, racial prevalences on the mortality charts. ALS however according to ALS CARE Database shows that "93% of the victims were Caucisan". Although ALS has no patterned relationship to a singular, particular, more-affected ethnic group, according to the Mayo Clinic, "people living in Guam, West New Guinea and parts of Japan have an increased risk of developing ALS. Dietary factors may be to blame." There is some evidence that just by serving in the military, people have an increased chance of suffering from ALS.

Cause: The cause of ALS is yet unknown, but new technological advances allow the disease to be further researched and explained. ALS affects its victims in many ways: some patients experience a slow, deteriorating journey, while others may experience episodes of the disease and then go into remission. Possible causes of ALS include:

  1. "Free radicals. The inherited form of ALS often involves a mutation in a gene responsible for producing a strong antioxidant enzyme that protects your cells from damage caused by free radicals — the byproducts of oxygen metabolism.

  2. Glutamate. People who have ALS typically have higher than normal levels of glutamate, a chemical messenger in the brain, in their spinal fluid. Too much glutamate is known to be toxic to some nerve cells.

  3. Autoimmune responses. Sometimes, a person's immune system begins attacking some of his or her body's own normal cells, and scientists have speculated that such antibodies may trigger the process that results in ALS."
Although the specific cause of Lou Gehrig's Disease is unknown, scientific observation in recent years has yielded a wealth of knowledge as far as the physiology of the disease goes. A few diseases that can evolve into ALS are:

- Progressive bulbar palsy
- Progressive Muscular Atrophy
- Primary Lateral Sclerosis
- Adult-onset Spinal Muscular Atrophy

Symptoms: Since ALS is a terminal, progressive neurodegenerative disease that affects nerve cells in the brain and the spinal cord; the disease exhibits early symptoms such as:

  • increasing muscle weakness, especially involving the arms, legs, and ankles, leading to decreased movement
  • slurring of speech
  • trouble swallowing
  • hand weakness
  • clumsiness
  • muscle twitching in arms, shoulders, and/or tongue
  • muscle cramps
  • difficulty lifting the front part of your foot and toes (footdrop)

As ALS progresses throughout the body, the patient is left paralyzed and often times loses the capability of chewing, swallowing, speaking and breathing.

Prognosis (progression of the disease): The progression of ALS begins with the motor neurons in the body deteriorating. These motor neurons make voluntary muscular movement possible as they branch from the brain to the spinal cord to the skeletal muscles, which allow us to walk and talk. When the motor neurons die, the ability of the brain to initiate, control, and send impulses for muscle movement is lost. Once the muscle fibers are no longer stimulated because of the dead motor neurons and the scarred, hardened (sclerosis) spinal cord, the muscle fibers begin to atrophy and become smaller because they no longer have any function. Loss of muscular movement leads to the more severe symptoms such as paralysis and inability to eat, breathe, and talk; even though the victim's intellectual ability remains intact throughout the disease. The ultimate fatality behind ALS triggers from the victim's inability to breathe any longer---respiratory failure.

Treatment Options (Rx): Since there is no cure to Amyotropic Lateral Sclerosis, the only treatment used is a medication called Rilutek, which slows the progression of ALS. Rilutek is in fact the first and only drug approved to treat ALS by the FDA, as it decreases the levels of glutamate in the brain.

Victims of ALS often times get the option of therapy to help decrease the effects of ALS in addition to the Rilutek in their prescribed medication.

  • Physical therapy for low-impact exercises to maintain muscle strength and range of motion as long as possible, helps the victim preserve a sense of independence.
  • Occupational therapy to help the victim be accustomed to a brace, walker or wheelchair and may be able to suggest devices such as ramps that make it easier for them to get around.
  • Speech therapy to help teach techniques to make the victim's speech more clearly understood. Later in the disease, a speech therapist can recommend devices such as speech synthesizers and computers that may help the victim communicate.

Links:

Sunday, December 14, 2008

Osteitis Deformans

Common Name: Paget's Disease

Age of Onset: Paget's Disease is most commonly found in people after they reach 50 years of age, and it is rarely diagnosed those younger than 20 years.

Duration: Paget's Disease is a chronic disease, that has no cure. Although it does not have a cure, Paget's Disease can be treated to minimize the disease's painful effects.

Males/Females/Equal: Paget's Disease is more commonly found in males than in females, the male-to-female ratio about 1.8:1.

Particular Ethnic Group: Paget's disease is more commonly found in the northern European ancestry and British migrant population in countries such as Great Britain, the United States, Australia, and New Zealand. It is particularly common in England. The disease is rare in Asia, Africa, Scandinavia, India, and Japan.

Cause: In Paget's disease, both osteoclasts and osteoblasts become overactive in some areas of bone, and the rate at which bone is broken down and rebuilt in these areas increases. These overactive areas enlarge and become structurally abnormal and weak.

The exact cause of Paget's Disease has not been pin-pointed. Probable causes of Paget's Disease include genetics, the environment, viral infection, inflammation, elevated parathyoid hormone levels, idiopathic hypoparathyroidism, autoimmune, connective tissue, and vascular disorders.

Symptoms: Paget's Disease affects everyone differently, and most often the patient will present zero symptoms. Paget's disease commonly affects the skull, the spine, the pelvis, and the bones of the limbs. The disease may affect a few specific areas of the body or it may be widespread. The symptoms of Paget's Disease will depend on the affected body part.

Some symptoms of Paget's Disease may include:

  • Pain in the affected bones (maybe constant, aching, deep, and severe at night)
  • Damaged cartilage lining the joints near the affected bones, which may lead to osteoarthritis (OA)
  • Severe pain from nerve compression, caused by the enlarged bones compressing the spinal cord. Pressure on a nerve can also cause numbness, tingling, weakness, hearing loss and double vision.
  • Warmth in your skin over the affected area
  • Neurological problems, such as hearing loss, headache and rarely, vision loss
  • Bone deformities, such as bowlegs and enlarged head size
  • Fractures

Prognosis (describe the progression of the disease): Paget's Disease causes the osteoclasts to become more active than osteoblasts, and so the bone remodeling and bone growth becomes off balance with more bone absorption than normal. The osteoblasts try to keep up by making new bone, but they overreact and make excess bone. The new-formed bone as a result is abnormally large, weak, brittle, and deformed. The patient's bones and joints then are prone to fractures, bowing, deformities, arthritis, inflammation, and nerve compression.

Treatment Options (Rx): Treatment to Paget's Disease varies greatly. If the patient has no symptoms, then treatment will not be adminstered. However treatment greatly depends on the area that is affected, if a high-risk bone is affected by Paget's (the skull or spine), then the doctor will recommend treatment to prevent OA, heart failure, and sarcoma. Treatment will alleviate some of the pain and halt bone damage due to Paget's, in some cases even cause remission of the disease. The different forms of treatment include:

  • Bone-regulating medication: A.) Bisphosphonate (risedronate: Actonel, alendronate: Fosamax, and pamidronate: Aredia, and zoledronic acid: Reclast), used to treat osteoporosis and to increase bone density, or B.) Calcitonin (Miacalcin), the naturally occuring hormone for calcium regulation and bone metabolism.
  • Arthritis medication, to reduce pain or inflammation: A.) Nonsteroidal anti-inflammatory drugs (NSAIDs), which reduce the pain and inflammatory effects of arthritis or B.) Acetaminophen (like Tylenol), to reduce the arthritis pain.
  • Surgery may be required to help fractures heal, to replace joints damaged by severe arthritis or to realign deformed bones. If Paget's disease affects your spine or your skull, you may need surgery to reduce pressure on nerves and prevent serious complications.
  • Daily diet with 1000-1500 mg of calcium and 400 U of vitamin D.
  • Exercise to increase skeletal strength and health
  • Rehabilitation programs, which include physical therapy, modalities, occupational therapy, and speech therapy

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Wednesday, October 22, 2008

Acute febrile neutrophilic dermatosis (Sweet Syndrome)

Proper Name: Acute febrile neutrophilic dermatosis

Common Name: Sweet Syndrome, Sweet disease, Sweet's syndrome, Sweet's disease, neutrophilic dermatitis, granulocyte colony-stimulating factor, G-CSF

Age Onset: Although infants and older-aged adults can develop Sweet Syndrome, it most commonly affects women between the ages of 30 and 50.

Duration: Lesions from Sweet Syndrome may last anywhere from a few days to over four years, and reoccurance is common.

Males/Females/Equal: Females are more prone to have Sweet Syndrome than males.

Particular Ethnic Group: Sweet Syndrome is very rare and has no known ethnic inclinations.

Cause: Exact cause is not known, but it can also develop early in the onset of cancer. Since Sweet Syndrome cannot be targeted for a specific cause, the syndrome can sometimes be an immune response:
  • to an upper respiratory tract infection (such as a chest infection or strep throat),
  • to blood disorders (esp. acute myelogenous leukemia),
  • to inflammatory bowel disease (such as ulcerative colitis or Crohn's disease),
  • to bowel or breast cancer,
  • to pregnancy,
  • to Rheumatoid arthritis,
  • to vaccination, and
  • to Certain medications (such as nonsteroidal anti-inflammatory drugs (NSAIDs)).

Symptoms:

  • distinctive skin lesions, that develop typically to this pattern: 1st.) a series of small red bumps appear suddenly on your back, neck, arms and face, often after a fever or upper respiratory infection, 2nd.) the bumps grow quickly in size, spreading into clusters called plaques that may be a centimeter in diameter or larger, 3rd.) the eruptions that are tender or painful, may develop blisters, pustules or even ulcers, and 4th.) lesions may persist for weeks to months and disappear on their own, without medication; with medical treatment the lesions coud be gone in a few days.
  • Moderate to high fever
  • Pink eye (conjunctivitis) or sore eyes
  • Tiredness
  • Aching joints and headache
  • Mouth ulcers
Prognosis (describe the progression of the disease): Sweet Syndrome begins as a series of small red bumps appearing suddenly on your back, neck, arms and face, often after a fever or upper respiratory infection. These bumps grow rapidly in size, spreading into clusters called plaques that may be a centimeter in diameter or larger. The eruptions are tender or painful and may develop blisters, pustules or even ulcers. Since Sweet Syndrome is commonly but not always accompanied by a more serious disease, the spread of the serious disease is also a progession of Sweet Syndrome.

Treatment Options (Rx):
  1. If the lesions are left untreated and Sweet syndrome is not associated with another condition, the lesions may disappear within 1 to 3 months.
  2. Medications can improve the skin lesions in a couple days to the worst cases within 1 to 4 weeks. Doctors usually prescribe systemic corticosteroids (prednisone or prednisolone) to treat Sweet syndrome because these oral anti-inflammatory medications reduce redness, itching, swelling and allergic reactions. But long-term use of corticosteroids can cause serious side effects, including an increased risk of hard-to-treat infections, osteoporosis, eye problems and diabetes. Topical corticosteroids may be used to provide immediate relief of swelling. In addition, your doctor may suggest that you take nonsteroidal anti-inflammatory drugs (NSAIDs) such as aspirin, ibuprofen or naproxen sodium to reduce inflammation and relieve other signs and symptoms, such as fever and headache. It is not unheard of for doctors to continue the treatment for Sweet Syndrome because it can reoccur.
  3. Avoid injury to the skin and wear protective clothing.
  4. Apply sunscreen with a sun protection factor (SPF) of 15 or greater before heading outdoors.

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