Part II Guillain-Barré syndrome GBS

Understanding how nerve damage occurs:

Various ideas have been proposed to explain how GBS develops. One explanation is known as the “molecular mimicry/innocent bystander” theory. According to this explanation, molecules on some nerves are very similar to or mimic molecules on some microorganisms. When those microbes infect someone, the immune system correctly attacks them. And if the microbe and myelin look similar, the immune system makes a mistake and attacks the myelin.

Different mechanisms may explain how the molecular mimicry concept may work.  When Guillain-Barré syndrome is preceded by a viral or bacterial infection, it is possible that the infecting agent has changed the chemical structure of some nerves. The immune system treats these nerves as foreign bodies and mistakenly attacks them. It is also possible that the virus makes the immune system itself less discriminating and no longer able to recognize its own nerves. Some parts of the immune system—special white blood cells called lymphocytes and macrophages—perceive myelin as a foreign body and attack it. Specialized white blood cells called T lymphocytes (from the thymus gland) cooperate with B lymphocytes (that originate in bone marrow) to produce antibodies against the person’s own myelin and damage it.

In some forms of GBS, antibodies made by the person to fight a Campylobacter jejuni bacterial infection attack axons in the motor nerves. This causes acute motor axonal neuropathy, which is a variant of GBS that includes acute paralysis and a loss of reflexes without sensory loss. Campylobacter infections can be caused by ingesting contaminated food or from other exposures. The infected person’s body then makes antibodies against Campylobacter. Some Campylobacter molecules resemble molecules in the person’s nerve axons, so when the person’s antibodies fight the Campylobacter bacteria they also attack the look-alike axons. This slows nerve conduction and causes paralysis. Scientists are investigating various GBS subtypes to find why the immune system reacts abnormally in this syndrome and other autoimmune diseases.

Guillian Barre Syndrome (GBS) is associated with cranial nerve involvement. Commonest cranial nerves involved were the facial and bulbar (IXth and Xth)  .Guillian Barre Syndrome (GBS) is associated with cranial nerve involvement. Most common cranial nerves involved are the facial and bulbar (IXth and Xth). Involvement of twelfth cranial nerve is rare in GBS.  Your hypoglossal nerve is the 12th cranial nerve which is responsible for the movement of most of the muscles in your tongue. It starts in the medulla oblongata and moves down into the jaw, where it reaches the tongue.

What disorders are related to GBS?

Guillain-Barré syndrome is one of several disorders involving weakness due to peripheral nerve damage caused by the person’s immune system. While GBS comes on rapidly over days to weeks, and the person usually recovers, other disorders develop slowly and can linger or recur.

The most common type of GBS seen in the United States is acute inflammatory demyelinating polyneuropathy (AIDP). In AIDP, the immune response damages the myelin coating and interferes with the transmission of nerve signals. In two other types of Guillain-Barré syndrome, acute motor axonal neuropathy (AMAN) and acute motor-sensory axonal neuropathy (AMSAN), the axons themselves are damaged by the immune response.

Miller-Fisher syndrome is a rare, acquired nerve disease that is a variant of Guillain-Barré syndrome. It is characterized by abnormal muscle coordination with poor balance and clumsy walking, weakness or paralysis of the eye muscles, and absence of the tendon reflexes. Like GBS, symptoms may follow a viral illness. Additional symptoms include generalized muscle weakness and respiratory failure. Most individuals with Miller Fisher syndrome have a unique antibody that characterizes the disorder.

Related peripheral nerve disorders with slow onset and persisting or recurrent symptoms include chronic inflammatory demyelinating polyneuropathy (CIDP) and multifocal motor neuropathy. CIDP features weakness that can recur, repeatedly, over the course of years. Multifocal motor neuropathy typically affects many different muscles in a small part of a limb or limbs. Usually the symptoms are more severe on one side of the body.

Stay tune for Part III on GBS regarding how its diagnosed and the Rx including rehab.

QUOTE FOR THURSDAY:

GuillainBarré syndrome is a rare but serious autoimmune disorder in which the immune system attacks healthy nerve cells in your peripheral nervous system (PNS). It involves certain cranial nerves.  This leads to weakness, numbness, and tingling, and can eventually cause paralysis.”

MAYO CLINIC

QUOTE FOR WEDNESDAY:

“Don’t wait for winter health problems. Be proactive about your health this winter!
-Get a flu shot, wash your hands, and cover your mouth when you cough or sneeze.
-Stay active, maintain a healthy diet, and be sure to get enough sleep each night.
-Don’t wait for winter health problems. Schedule a wellness visit with your primary care physician.
-Talk to your doctor when you experience symptoms. You don’t have to wait for your symptoms to get “bad enough” before seeking treatment or advice.”
Medical Associates of Northwest Arkansas

Common winter problems!

 

   

Asthma

Asthma is one of the most common respiratory illnesses in the United States, and one common trigger becomes more prevalent in winter: freezing temperatures.

If you have asthma and intend on spending time outdoors in the elements, cover your nose and mouth with a scarf or face warmer to keep from breathing in the cold, dry air. Consider exercising indoors, such as at a gym.

Catching a virus

While the cold weather doesn’t have a direct effect on your chances of contracting a virus, it does force people indoors with centralized heating. The close proximity of many human beings can allow viruses to spread with ease.

According to The National Institutes of Health (NIH), dry winter air allows the flu virus to survive and transmit itself more easily. Also, research suggests that flu virus’ coating becomes tougher at temperatures close to freezing, making them easier to transmit in the winter.

Washing your hands is one of the simplest and most important ways to avoid getting a cold or the flu. Germs are on all surfaces, from door knobs to elevator buttons, and you may be exposing your body to those microbes if you rub your eyes or put your fingers in your nose or mouth without proper hand washing. Also, she recommends to get the flu vaccine. The Centers for Disease Control recommends the flu vaccine for persons six months of age and older.

Weight gain

Exercising daily may already be a struggle, and adding another obstacle in the form of cold and unruly weather can really extinguish your motivation. The numbers on the scale can tend to creep up during reduced exercise isn’t helped by the rich holiday foods and gatherings that involve large meals.

To best combat weight gain, make a conscious effort to get at least 30 minutes of exercisesuch as an indoor exercise class to outsmart the elements—at least several times per week. Also, be sure to not binge on the holiday sweets that may be around; leave the cookies for Santa.

Dry skin

Cold weather and low humidity during the winter can dehydrate your skin and leave it looking dull. The Mayo clinic states “Remember to drink at least 64 ounces of water every day to keep your skin supple and to flush out toxins.   Health benefits about water is that water is your body’s principal chemical component and makes up about 60 percent of your body weight. Your body depends on water to survive.

Every cell, tissue and organ in your body needs water to work properly. For example, water:

  • Gets rid of wastes through urination, perspiration and bowel movements
  • Keeps your temperature normal
  • Lubricates and cushions joints
  • Protects sensitive tissues

Lack of water can lead to dehydration!”  This condition can cause many health problems if left untreated.

Also treat your skin gently. Avoid strong soap, shave carefully, use warm water instead of hot water and pat your skin dry rather than rub it dry. Hot water and strong soaps can remove oil that your skin needs to stay healthy.

Seasonal depression

The winter blues can have a real effect on people and can cause some seasonal depression. In fact, the “winter blues” are described as a milder version of seasonal affective disorder (SAD).

To some extent, these mood changes can be attributed to the decreased sunlight and daylight hours. However, for some people, depression can be triggered because of the loss of a loved one during the holidays or the lack of social engagement that can occur because of bad weather.

In order to beat the winter blues, make an effort to get natural sunlight during the day and limit your days spent entirely indoors. Also, be sure that you’re getting proper amount of sleep—about eight hours for most adults.

QUOTE FOR TUESDAY:

”The signs and symptoms of Cushing syndrome can vary depending on the levels of excess cortisol.  Cortisol also helps your body respond to stress and regulates the way you convert (metabolize) proteins, carbohydrates and fats in your diet into usable energy. However, when the level of cortisol is too high in your body, you may develop Cushing syndrome with its complications.”

NIH National Institute of Diabetes and Digestive and Kidney Diseases (U.S. Department of Health and Human Services)

Part II What is Cushing’s Syndrome

 

Complications can include:

-Diabetes (High or Low blood glucose levels)

-Enlargement of pituitary tumor and other complications from the tumor growth

-Fractures due to osteoporosis which are common in older people

-High blood pressure which could be life threatening

-Kidney stones from the increase in cortisol and other chemicals filtered through the kidneys

-Serious infections which could lead to further secondary infections

How Cushing’s syndrome is diagnosed and treated?

Diagnosis is established on a survey of the patient’s medical history including somatic examination and laboratory tests.

Frequently x-ray exams of the adrenal or pituitary glands are beneficial for locating tumors. These tests assist to find out if excess levels of cortisol are exhibited and why.

Radiologic imaging: direct visualization of the endocrine glands. Imaging tests reveal the size and shape of the pituitary and adrenal glands and help determine if a tumor is present.  The most common imaging tests are the computerized tomography (CT) scan and magnetic resonance imaging (MRI).  A CT scan produces a series of x-ray pictures giving a cross-sectional image of a body part.  MRI also produces images of internal organs but without exposing patients to ionizing radiation.

Imaging procedures are used to find a tumor after a diagnosis has been made. Imaging is not used to make the diagnosis of Cushing’s syndrome because benign tumors are commonly found in the pituitary and adrenal glands.  These tumors, sometimes called incidentalomas, do not produce hormones in quantities that are harmful.  They are not removed unless blood tests show they are a cause of symptoms or they are unusually large.  Conversely, pituitary tumors may not be detectable by imaging in almost half of people who ultimately need pituitary surgery for Cushing’s syndrome.

24-hour urinary free cortisol level. In this test, a person’s urine is collected several times over a 24-hour period and tested for cortisol.  Levels higher than 50 to 100 micrograms a day for an adult suggest Cushing’s syndrome.  Low-dose dexamethasone suppression test (LDDST). In the LDDST, a person is given a low dose of dexamethasone, a synthetic glucocorticoid, by mouth every 6 hours for 2 days.  Urine is collected before dexamethasone is administered and several times on each day of the test.  A modified LDDST uses a onetime overnight dose.

The normal upper limit varies in different laboratories, depending on which measurement technique is used.

Midnight plasma cortisol and late-night salivary cortisol measurements. The midnight plasma cortisol test measures cortisol concentrations in the blood.

Dexamethasone-corticotropin-releasing hormone (CRH) test. Some people have high cortisol levels but do not develop the progressive effects of Cushing’s syndrome, such as muscle weakness, fractures, and thinning of the skin.  These people may have pseudo-Cushing’s syndrome, a condition sometimes found in people who have depression or anxiety disorders, drink excess alcohol, have poorly controlled diabetes, or are severely obese.  Pseudo-Cushing’s does not have the same long-term effects on health as Cushing’s syndrome and does not require treatment directed at the endocrine glands.

Treatment of Cushings Syndrome:

Treatment of cushings syndrome is by castigation of the under lying cause. Treatments for Cushing’s syndrome are contrived to pass your body’s cortisol production to normal. By indurate, or even distinctly lowering cortisol levels, you’ll feel evident improvements in your signs and symptoms. Left untreated, however, Cushing’s syndrome can finally induce to death. The treatment choice depend on the cause. For example:

*If a tumour in an adrenal gland is the reason, an operation to withdraw it will cure the condition.

*For adrenal hyperplasia, both adrenal glands may require to be withdraw. You will then require to take lifelong replacement therapy of several adrenal hormones.

*Other tumours in the body that produce ‘ectopic’ ACTH may be able to be removed, depending on the kind of tumour, where it is, etc.

*Medication to block the production or consequence of cortisol may be a choice.

Ending line treatments for Cushing syndrome are designed to lower the high level of cortisol in your body. The best treatment for you depends on the cause of the syndrome. Treatment options include:

  • Reducing corticosteroid use. If the cause of Cushing syndrome is long-term use of corticosteroid medications, your doctor may be able to keep your Cushing signs and symptoms under control by reducing the dosage of the drug over a period of time, while still adequately managing your asthma, arthritis or other condition. For many of these medical problems, your doctor can prescribe noncorticosteroid drugs, which will allow him or her to reduce the dosage or eliminate the use of corticosteroids altogether.

Don’t reduce the dose of corticosteroid drugs or stop taking them on your own. Do so only under your doctor’s supervision.

Abruptly discontinuing these medications could lead to deficient cortisol levels. Slowly tapering off corticosteroid drugs allows your body to resume normal cortisol production.

  • If the cause of Cushing syndrome is a tumor, your doctor may recommend complete surgical removal. Pituitary tumors are typically removed by a neurosurgeon, who may perform the procedure through your nose. If a tumor is present in the adrenal glands, lungs or pancreas, the surgeon can remove it through a standard operation or in some cases by using minimally invasive surgical techniques, with smaller incisions.

After the operation, you’ll need to take cortisol replacement medications to provide your body with the correct amount of cortisol. In most cases, you’ll eventually experience a return of normal adrenal hormone production, and your doctor can taper off the replacement drugs.

However, this process can take up to a year or longer. In some instances, people with Cushing syndrome never experience a resumption of normal adrenal function; they then need lifelong replacement therapy.

  • Radiation therapy. If the surgeon can’t totally remove a pituitary tumor, he or she will usually prescribe radiation therapy to be used in conjunction with the operation. Additionally, radiation may be used for people who aren’t suitable candidates for surgery.

Radiation can be given in small doses over a six-week period, or with a technique called stereotactic radiosurgery (Gamma Knife surgery). In the latter procedure, administered as a single treatment, a large dose of radiation is delivered to the tumor, and the radiation exposure to surrounding tissues is minimized.

  • Medications can be used to control cortisol production when surgery and radiation don’t work. Medications may also be used before surgery in people who have become very sick with Cushing syndrome. Doctors may recommend drug therapy before surgery to improve signs and symptoms and minimize surgical risk.

Medications to control excessive production of cortisol at the adrenal gland include ketoconazole (Nizoral), mitotane (Lysodren) and metyrapone (Metopirone).

Mifepristone (Korlym) is approved for people with Cushing syndrome who have type 2 diabetes or glucose intolerance. Mifepristone does not decrease cortisol production, but it blocks the effect of cortisol on your tissues.

Side effects from these medications may include fatigue, nausea, vomiting, headaches, muscle aches, high blood pressure, low potassium and swelling. Some have more serious side effects, such as neurological side effects and liver toxicity.

The newest medication for Cushing’s is pasireotide (Signifor), and it works by decreasing ACTH production from a pituitary tumor. This medication is given as an injection twice daily. It’s recommended if pituitary surgery is unsuccessful or cannot be done.

Side effects are fairly common, and may include diarrhea, nausea, high blood sugar, headache, abdominal pain and fatigue.

In some cases, the tumor or its treatment will cause other hormones produced by the pituitary or adrenal gland to become deficient and your doctor will recommend hormone replacement medications.

If none of these treatment options are appropriate or effective, your doctor may recommend surgical removal of your adrenal glands (bilateral adrenalectomy). This procedure will cure excess production of cortisol, but will require lifelong replacement medications.

Complications are rare but include:

Bruising on your thigh

Ear pain during the procedure

Very rarely, neurological problems

QUOTE FOR MONDAY:

“Cushing’s syndrome is a hormonal disorder caused by prolonged exposure of the body’s tissues to high levels of the hormone cortisol.”

NIH National Institute of Diabetes and Digestive and Kidney Diseases (U.S. Department of Health and Human Services)

Part I What is Cushing’s Syndrome

 

Cushing’s syndrome describes the signs and symptoms associated with prolonged exposure to inappropriately high levels of the hormone cortisol. This can be caused by taking glucocorticoid drugs, or diseases that result in excess cortisol, adrenocorticotropic hormone (ACTH), or CRH levels.

Cushing’s syndrome appears when the body’s tissues are display to immoderate levels of cortisol for long periods of time.

There are two types of the disease and they are known as exogenous and endogenous.  Exogenous Cushing syndrome is caused by something outside of the body, like when hormones are given to a patient during a RX for another condition.

Endogenous is caused by natural causing problems within the body.  Endogenous is likely to be hereditary and not caused by an outside force like a steroid complex.

Causes of Cushing Syndrome

The most common cause of Cushing’s syndrome is exogenous administration of glucocorticoids prescribed by a health care practitioner to treat other diseases (called iatrogenic Cushing’s syndrome). This can be an effect of corticosteroid treatment of a variety of disorders such as asthma and rheumatoid arthritis, or in immunosuppression after an organ transplant.

Administration of synthetic ACTH(adrenocorticotropichormone) is also possible, but ACTH is less often prescribed due to cost and lesser utility. Although rare, Cushing’s syndrome can also be due to the use of medroxyprogesterone   In this form of Cushing’s, the adrenal glands atrophy due to lack of stimulation by ACTH, since glucocorticoids downregulate production of ACTH. Cushing syndrome in childhood usually results from use of glucocorticoid medication.

Endogenous Cushing’s syndrome results from some derangement of the body’s own system of secreting cortisol. Normally, ACTH is released from the pituitary gland when necessary to stimulate the release of cortisol from the adrenal glands.

In pituitary Cushing’s, a benign pituitary adenoma secretes ACTH. This is also known as Cushing’s disease and is responsible for 70% of endogenous Cushing’s syndrome.

In adrenal Cushing’s, excess cortisol is produced by adrenal gland tumors, hyperplastic adrenal glands, or adrenal glands with nodular adrenal hyperplasia.

Tumors outside the normal pituitary-adrenal system can produce ACTH (occasionally with CRH) that affects the adrenal glands. This etiology is called ectopic or paraneoplastic Cushing’s disease and is seen in diseases like small cell lung cancer.

Finally, rare cases of CRH-secreting tumors (without ACTH secretion) have been reported, which stimulates pituitary ACTH production.

Pseudo-Cushing’s syndrome

 Elevated levels of total cortisol can also be due to estrogen found in oral contraceptive pills that contain a mixture of estrogen and progesterone, leading to Pseudo-Cushing’s syndrome. Estrogen can cause an increase of cortisol-binding globulin and thereby cause the total cortisol level to be elevated. However, the total free cortisol, which is the active hormone in the body, as measured by a 24 hour urine collection for urinary free cortisol, is normal.

Epidemiology

Iatrogenic Cushing’s syndrome (caused by treatment with corticosteroids) is the most common form of Cushing’s syndrome.

Symptoms of Cushings Syndrome

Symptom of cushings syndrome include fat deposits close to the face neck and trunk; weariness; muscular weakness; salt and water retention; acne; leisurely bruising; menstlruall irregularities; and signs (in women) of virilisation, such as increase of the voice, commute in body, shape, loss of scalp hair, and extend in facial and body hair. Complications of cushings syndrome include advanced blood pressure, The symptoms and signs of cushings syndrome induced by a chronic redundant of corticosteroid hormones in the blood. The redundant may be acquired by a tumour of the outer part (cortex) of the adrenal gland, or may be referable to over inspiration of the adrenal glands by a tumour the pituitary gland.

Many children and teenagers with Cushing’s syndrome will exhibit various symptoms of the following:

-extreme weight gain

-growth retardation

-missed periods in teenage girls

-excess hair growth

-acne

-reddish-blue streaks on the skin

-high blood pressure

-tiredness and weakness

-either very early or late puberty

Adults with the disease may also have symptoms of intense weight gain, redundant hair growth, high blood pressure, and skin difficulties. In addition, they may show:

-muscle and bone weakness

-moodiness, irritability, or depression

-sleep disturbances

-high blood sugar

-menstrual disorders in women and diminished fertility in men

 

 

 

 

 

QUOTE FOR THE WEEKEND:

“Treatments for coronary heart disease include heart-healthy lifestyle changes, medicines, medical procedures and surgery, and cardiac rehabilitation.”

National Heart, Lung and Blood Institute

Part III Know what Coronary Artery Disease treatments are!

Surgery inserting a stent

Treatments of Coronary Artery Disease!

1.) Lifestyle changes

Making a commitment to the following healthy lifestyle changes can go a long way toward promoting healthier arteries:

  • Quit smoking.
  • Eat healthy foods.
  • Exercise regularly.
  • Lose excess weight.
  • Reduce stress.

2.) Drugs

Various drugs can be used to treat coronary artery disease, including:

  • Cholesterol-modifying medications. By decreasing the amount of cholesterol in the blood, especially low-density lipoprotein (LDL, or the “bad”) cholesterol, these drugs decrease the primary material that deposits on the coronary arteries. Your doctor can choose from a range of medications, including statins, niacin, fibrates and bile acid sequestrants.
  • Your doctor may recommend taking a daily aspirin or other blood thinner. This can reduce the tendency of your blood to clot, which may help prevent obstruction of your coronary arteries.

If you’ve had a heart attack, aspirin can help prevent future attacks. There are some cases where aspirin isn’t appropriate, such as if you have a bleeding disorder or you’re already taking another blood thinner, so ask your doctor before starting to take aspirin.

  • Beta blockers. These drugs slow your heart rate and decrease your blood pressure, which decreases your heart’s demand for oxygen. If you’ve had a heart attack, beta blockers reduce the risk of future attacks.
  • Nitroglycerin. Nitroglycerin tablets, sprays and patches can control chest pain by temporarily dilating your coronary arteries and reducing your heart’s demand for blood.
  • Angiotensin-converting enzyme (ACE) inhibitors and angiotensin II receptor blockers (ARBs). These similar drugs decrease blood pressure and may help prevent progression of coronary artery disease.

3.) Procedures to restore and improve blood flow

Coronary artery stent

Surgical procedure in which one or more blocked coronary arteries are bypassed by a blood vessel graft to restore normal blood flow to the heart. These graft usually come from the patient’s own arteries and veins located in the chest (thoracic), leg (saphenous) or arm (radial). The graft goes around the blocked artery (or arteries) to create new pathways for blood to flow to the heart.

Sometimes more aggressive treatment is needed. Here are some options:

  • Angioplasty and stent placement (percutaneous coronary revascularization). Your doctor inserts a long, thin tube (catheter) into the narrowed part of your artery. A wire with a deflated balloon is passed through the catheter to the narrowed area. The balloon is then inflated, compressing the deposits against your artery walls.

A stent is often left in the artery to help keep the artery open. Some stents slowly release medication to help keep the artery open.

  • Coronary artery bypass surgery. A surgeon creates a graft to bypass blocked coronary arteries using a vessel from another part of your body. This allows blood to flow around the blocked or narrowed coronary artery as described at the top of this article. Because this requires open-heart surgery, it’s most often reserved for cases of multiple narrowed coronary arteries.

Alternative medicine

Omega-3 fatty acids are a type of unsaturated fatty acid that’s thought to reduce inflammation throughout the body, a contributing factor to coronary artery disease. However, recent studies have not shown them to be beneficial. More research is needed.

  • Fish and fish oil. Fish and fish oil are the most effective sources of omega-3 fatty acids. Fatty fish — such as salmon, herring and light canned tuna — contain the most omega-3 fatty acids and, therefore, the most benefit. Fish oil supplements may offer benefit, but the evidence is strongest for eating fish.
  • Flax and flaxseed oil. Flax and flaxseed oil also contain beneficial omega-3 fatty acids, though studies have not found these sources to be as effective as fish. The shell on raw flaxseeds also contains soluble fiber, which can help lower blood cholesterol.
  • Other dietary sources of omega-3 fatty acids. Other dietary sources of omega-3 fatty acids include canola oil, soybeans and soybean oil. These foods contain smaller amounts of omega-3 fatty acids than do fish and fish oil, and evidence for their benefit to heart health isn’t as strong.