Sinco Mark

GUILLAIN BARRE SYNDROME A CASE PRESENTATION

disorder wherein the body’s own immune system attacks the peripheral nerves that convey sensory information (e.g., pain, temperature) from the body to the brain and motor (i.e., movement) signals from the brain to the body. It is a rare condition occurring once in every 100,000 people per year but it is one of the leading causes of non-trauma induced paralysis in the world. Exactly what triggers Guillain-Barre syndrome is unknown and it may occur at any age but is most common among young adults and the elderly. The condition is often preceded by either a viral or bacterial People GBS usually have muscle tract. weakness infectionwith of the lungs or gastrointestinal Signs or of paralysis that starts have in thealready legs and feet. It progresses up the original infection disappeared before the toward theof arms and head. Sometimes the weakness symptoms Guillain-Barre begin. starts in the arms and moves downward. Occasionally, it starts in the arms and legs at the same time. The spread from feet to head can occur within 24 to 72 hours but can take longer. Mild cases of Guillain-Barre syndrome may present only with ataxia (difficulty in walking and balancing), whereas severe cases may present with difficulty in respiration due to paralysis of the respiratory muscles and cranial nerves.

Treatment for Guillain-Barre Syndrome consists of supportive care, ventilatory management (in about one third of patients), and specific therapy with intravenous immunoglobulin or plasmapheresis. However, each case of Guillain-Barre' Syndrome is different. It is important to realize that the complications and therefore treatments of Our patient is Mr. C, a seventeen old first year Guillain-Barre' syndrome are notyear predictable. For high the school student of Loreto National High School. He was most part, treatments are highly individualized. admitted last September 25, 2009 at the Caraga Regional Hospital after being referred by the Loreto District Hospital. His chief complaint upon admission was sudden onset of weakness on both legs. Guillain-Barre Syndrome is a serious disorder requiring prompt medical attention which can even be a medical emergency. However, most Filipinos have not even heard of such our a condition. Yes, it to is present a rare condition, Hence, group decided this case but withmore the and moreintention Filipinos are by it. earnest to struck acquire sufficient and accurate information about the disorder for us to be able to play our part on the prevention of the said disorder through information dissemination. As future nurses, we understand the indispensable role of health promotion in

Guillain-Barré syndrome (GBS) causes progressive muscle weakness and paralysis (the complete inability to use a particular muscle or muscle group), which develops over days or up to four weeks, and lasts several weeks or even months. The classic scenario in GBS involves a patient who has just recovered from a typical, seemingly uncomplicated Description viral infection. Symptoms of muscle weakness appear one to four weeks later. The most common preceding infections are cytomegalovirus, herpes, Epstein-Barr virus, and viral hepatitis. A gastrointestinal infection with the bacteria Campylobacter jejuni is also common and may cause a severe type of GBS from which it is particularly difficult to recover. About 5% of GBS patients have a surgical procedure as a preceding event. Patients with lymphoma, systemic lupus erythematosus, or AIDS have a higher than normal risk of GBS. Other GBS patients have recently received an immunization, while still others have no known preceding event. In 1976–77, there was a vastly increased number of GBS cases among people who had been recently vaccinated against the Swine flu. The reason for this phenomenon has never been identified, and no other flu vaccine has caused such

GBS can be divided into types on the basis of the type of destruction caused. If the myelin sheath (described below, see figure 1) insulating an axon is damaged or destroyed, the nerve signals through the axon are disrupted or slowed down, causing symptoms such as abnormal sensations and weakness. This inflammation is the demyelinising type, In the axonal type, the nerve axon itself is destroyed in a and the process is called primary demyelination. process called secondary demyelination. This is said to occur in patients who experience a very violent inflammation phase. If the axon dies, the nerve signal is blocked, and cannot be transmitted further. This causes weakness and paralysis in the body area controlled by the The nerve. axonal type occurs most frequently after preceding diarrhoea. It may be responsible for a less favourable prognosis (outcome), as axons regenerate after a long delay compared to the myelin sheath, which heals faster. There are however some kinds of axonal types that have a more favourable prognosis

Peripheral nerves and spinal roots are the major sites of demyelination, but cranial nerves may The mixed type destroys both axons and myelin. Long-term paralysis in some GBS patients is thought to be caused by permanent damage to both axons and myelin sheaths. also be involved.

struction of nerve insulation

The immune system is responsible for the production of ne system's attack on the peripheral nerve cells starts a chain re special proteins, the antibodies or immunoglobulins (Ig), as part of the body's normal defence mechanism. These antibodies are produced in reaction to the presence of antigens, or introduced particles in the body, such as various bacteria and vira. Antibodies match specific antigens, and when the two come in contact, they bind together and a number of destructive reactions occur. In GBS patients, antibodies are somehow produced against myelin. They circulate in the blood seeking myelin, which is found in nerve cells.

Nerve cells have long, thin extensions called axons, that transmit signals between nerve cells. Some axons are surrounded by a myelin sheath, a little like electrical cables are surrounded by plastic. The myelin sheath insulates and protects the nerve cells. It also increases both the speed and the distance over which nerve signals can be transmitted. For example, signals from the brain to muscles are transmitted at a speed of over 50 km/h!

Fig.1: Longitudinal section of an axon and its myelin sheath. The axon is part of nerve cell 1, stretching toward nerve cell 2. The myelin sheath resembles tape, wrapped around the axon in several layers. Myelin does not cover the axon in an uninterrupted tube, like an electrical cable. Instead, it resembles long beads on a string, with space between the beads (see figure above). The spaces are known as Ranvier Nodes. Axons are uncovered between these nodes of Ranvier and are

The nerve signals transmitted are also delayed a little at the nodes, and the more nodes there are, the slower the signal becomes. This fact is important when understanding recovery - increased numbers of Ranvier The antibodies produced in the GBS nodesmyelin-attacking may be produced during nerve recovery, slowing patient circulate in the blood and eventually find myelin. signal transmission. They attack and destroy it with the help of white blood cells, producing inflammation in the nerves. The inflamed cells in turn secrete chemicals that affect the Schwann cells. These cells produce the fatty materials required to produce myelin. Affecting Schwann cells reduces myelin production, and some of them may even die, further reducing myelin production, while at the same time the As the attack the nerve network is existing myelinprogresses, is destroyed byperipheral the antibodies. gradually destroyed. Motor, sensory or autonomic nerves are attacked, signals are slowed down, blocked or If the signal transmission speed motor nerve is disrupted and the patient's bodyof is aaffected accordingly. reduced, the patient experiences weakness in the body area controlled by the nerve. If the signal speed is reduced further, or blocked, the patient can become paralysed. Similarly, attacks on sensory or autonomic nerves result in disturbances of the

from the body, and these may be disrupted. This results in parts of the body feeling numb, as well as strange sensations of pain, tingling, and pins and needles. Signals to and from the arms and legs must travel furthest from the brain and spinal cord, and are therefore most susceptible to a barrage of disturbances while en route. This is why hands and feet are usually the first body areas that display GBS symptoms.

ack on the peripheral nerves

All the nerves in the human body, with the exception of the brain and spinal cord, belong to the peripheral group of nerves. i.e. the peripheral nervous system comprises most of the cranial nerves and the spinal nerves (sensory, motor, autonomic, and mixed). The peripheral nerves transmit signals from the brain and spinal cord to, and from, the muscles, organs and skin. Depending on their function, the nerves can be classified as motor, sensory and autonomous (involuntary) peripheral nerves.

When the immune system malfunctions temporarily and GBS sets in, an attack is launched on the peripheral nerves, damaging them. This causes sensory disturbances, progressive weakening and/or acute paralysis. Metaphorically speaking, the nervous system short-circuits. Exactly how the nerves are damaged is described below. Only the peripheral nervous system is affected, which is why GBS is also known as "peripheral neuropathy".

Causes and symptoms

The cause of the weakness and paralysis of GBS is the loss of myelin, which is the material that coats nerve cells (the loss of myelin is called demyelination). Myelin is an insulating substance which is wrapped around nerves in the body, serving to speed conduction of nerve impulses. Without myelin, nerve conduction slows or stops. GBS has a short, severe course. It causes inflammation and destruction of the myelin sheath, and it disturbs multiple nerves. Therefore, it is considered an acute inflammatory demyelinating polyneuropathy.

unknown, although it is thought that the underlying problem is autoimmune in nature. An autoimmune disorder is one in which the body's immune system, trained to fight against such foreign invaders as viruses and bacteria, somehow becomes improperly programmed. The immune system becomes confused, and is not able to distinguish between foreign invaders and the body itself. Elements of the immune system are unleashed against The symptoms of GBS consist of muscle weakness areasfirst of the body, resulting in damage and destruction. (legs first, reason, then arms, then face), accompanied by prickly, For some in the case of GBS, the myelin sheath tingling Symptoms affect both appears sensations to become (paresthesias). a target for the body's own immune sides of the body simultaneously, a characteristic that system. helps distinguish GBS from other causes of weakness and paresthesias. Normal reflexes are first diminished, then lost. The weakness eventually affects all the voluntary muscles, resulting in paralysis. When those muscles necessary for breathing become paralyzed, the patient must be placed on a mechanical ventilator which takes over the function of breathing. This occurs about 30% of the time. Very severely ill GBS patients may have complications stemming from other nervous system abnormalities which can result in problems with fluid balance in the body, severely fluctuating blood pressure,

Diagnosis of GBS is made by looking for a particular

Diagnosis cluster of symptoms (progressively worse muscle

weakness and then paralysis), and by examining the fluid that bathes the brain and spinal canal through cerebrospinal fluid (CSF) analysis. This fluid is obtained by inserting a needle into the lower back (lumbar region). When examined in a laboratory, the CSF of a GBS patient will reveal a greaterthan-normal quantity of protein, with normal numbers of white blood cells and a normal amount of sugar. Electrodiagnostic studies may show slowing or block of conduction in nerve endings in parts of the body other than the brain. Minor abnormalities will be present in 90% of patients. Diagnostic tests Lumbar Puncture A needle is inserted in between the bones of the lower back into the fluid around the spinal cord. A small amount of spinal fluid is taken and the protein level is tested. In GBS, the protein level is usually increased.

Electromyogram (EMG or Electromyography)

A thin-needle electrode is inserted into the muscle to be tested and electrodes are placed on the skin over peripheral nerves. This test helps to determine if the peripheral nerves are not communicating between the brains and muscles in the body.

Nerve Conduction Study (NCS)

Two electrodes are taped to the skin in the affected area of the patient's body. A small shock is then passed through to measure the electrical impulses from one electrode to the other. This test records how long it takes for the electrical impulse to travel from one electrode to another. If the impulse is slow, it suggests nerve damage There is no direct treatment for GBS. Instead, treatments Treatment are used that support the patient with the disabilities caused by the disease. The progress of paralysis must be carefully monitored, in order to provide mechanical assistance for breathing if it becomes necessary. Careful attention must also be paid to the amount of fluid the patient is taking in by drinking and eliminating by urinating. Blood pressure, heart rate, and heart rhythm

A procedure called plasmapheresis, performed early in the course of GBS, has been shown to shorten the course and severity of GBS. Plasmapheresis consists of withdrawing the patient's blood, passing it through an instrument that separates the different types of blood cells, and returning all the cellular components (red and white blood cells and platelets) along with either donor plasma or a manufactured replacement solution. This is thought to rid the blood of the substances that are attacking the It has also been shown that the use of high doses of patient's myelin. immunoglobulin given intravenously (by drip through a needle in a vein) may be just as helpful as plasmapheresis. Immunoglobulin is a substance naturally manufactured by the body's immune system in response to various threats. It is interesting to note that corticosteroid medications (such as prednisone), often the mainstay of anti-autoimmune disease treatment, are not only unhelpful, but may in fact be harmful to patients with GBS.

Prognosis About 85% of GBS patients make reasonably good recoveries. However, 30% of adult patients, and a greater percentage of children, never fully regain their previous level of muscle strength. Some of these patients suffer from residual weakness, others from permanent paralysis. About 10% of GBS patients begin to improve, then suffer a relapse. These patients suffer chronic GBS symptoms. About 5% of all GBS patients die, most from cardiac rhythm disturbances.

Prevention Because so little is known about what causes GBS to develop, there are no known methods of prevention.