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Introduction Some of the neurological disorders are very rare but if once happened becomes chaos. Now mega star of the millennium Mr. Amitabh Bacchan had suffered from a very rare disease in 1980s called as Myesthenia gravis. synonyms

MG Myasthenia Gravis Pseudoparalytica Erb-Goldflam syndrome

Review the anatomy and physiolog y.

Anatomy and physiology of brain The brain is divided into the cerebrum, diencephalons, brain stem, and cerebellum. Cerebrum The largest and most obvious portion of the brain is the cerebrum, which is divided by a deep longitudinal fissure into two cerebral hemispheres. The two hemispheres are two separate entities but are connected by an arching band of white fibers, called the corpus callosum that provides a communication pathway between the two Lecture cum discussion

halves. Each cerebral hemisphere is divided into five lobes, four of which have the same name as the bone over them: the fontal lobe, the parietal lobe, the occipital lobe, and the temporal lobe. A fifth lobe, the insula or Island of Reil, lies deep within the lateral sulcus.

Lecture cum discussion.

Diencephalon The diencephalons is centrally located and is nearly surrounded by the cerebral hemispheres. It includes the thalamus, hypothalamus, and epithalamus. The thalamus, about 80 percent of the diencephalons, consists of two oval masses of gray matter that serve as relay stations for sensory impulses, except for

the sense of smell, going to the cerebral cortex. The hypothalamus is a small region below the thalamus, which plays a key role in maintaining homeostasis because it regulates many visceral activities.

Brain Stem The brain stem is the region between the diencephalons and the spinal cord. It consists of three parts: midbrain, pons, and medulla oblongata. The midbrain is the most superior portion of the brain stem. The pons is the bulging middle portion of the brain stem. This region primarily consists of nerve fibers that form conduction tracts between the higher brain centers and spinal cord. The medulla oblongata, or simply medulla, extends inferiorly from the pons. It is continuous with the spinal cord at the foramen magnum. All the ascending (sensory) and descending (motor) nerve fibers connecting the brain and spinal cord pass through the medulla. Cerebellum The cerebellum, the second largest portion of the brain, is located below the occipital lobes of the cerebrum. Three paired bundles of myelinated nerve fibers, called cerebellar peduncles, form communication pathways between the cerebellum and other parts of

the central nervous system. Ventricles and Cerebrospinal Fluid A series of interconnected, fluid-filled cavities are found within the brain. These cavities are the ventricles of the brain, and the fluid is cerebrospinal fluid (CSF). Spinal Cord The spinal cord extends from the foramen magnum at the base of the skull to the level of the first lumbar vertebra. The cord is continuous with the medulla oblongata at the foramen magnum. Like the brain, the spinal cord is surrounded by bone, meninges, and cerebrospinal fluid. The spinal cord has two main functions:

Lecture cum discussion

Define the key terms.

Serving as a conduction pathway for impulses going to and from the brain. Sensory impulses travel to the brain on ascending tracts in the cord. Motor impulses travel on descending tracts. Serving as a reflex center. The reflex arc is the functional unit of the nervous system. Reflexes are responses to stimuli that do not require conscious thought and consequently, they occur more quickly than reactions that require thought processes.

To discuss the incidence rate.

Definition Myasthenia gravis (MG) is a chronic disease characterised by fluctuating levels of muscle weakness. Lecture cum discussion

Incidence It is uncommon, affecting only 15 per 100,000 individuals, and although it can be a very serious condition, 90 per cent of patients live a near-normal life while continuing medical treatment. Classification The most widely accepted classification of myasthenia gravis is the Myasthenia Gravis Foundation of America Clinical Classification:

Class I: Any eye muscle weakness, possible ptosis, no other evidence of muscle weakness elsewhere Class II: Eye muscle weakness of any severity, mild weakness of other muscles
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Class IIa: Predominantly limb or axial muscles Class IIb: Predominantly bulbar and/or respiratory muscles

Class III: Eye muscle weakness of any severity, moderate weakness of other muscles
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Class IIIa: Predominantly limb or axial muscles

Class IIIb: Predominantly bulbar and/or respiratory muscles

List the causes.

Class IV: Eye muscle weakness of any severity, severe weakness of other muscles
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Class IVa: Predominantly limb or axial muscles Class IVb: Predominantly bulbar and/or respiratory muscles (Can also include feeding tube without intubation)

Class V: Intubation needed to maintain airway

Explain the pathophys iology .

Causes and risk factors it is an autoimmune disorder, in which weakness is caused by circulating antibodies that block acetylcholine receptors at the postsynaptic neuromuscular junction,[1] inhibiting the excitatory effects of the neurotransmitter acetylcholine on nicotinic receptors throughout neuromuscular junctions. Myasthenia is treated medically with cholinesterase inhibitors or immunosuppressants, and, in selected cases, thymectomy.

Pathophysiology MG is the result of a selective overactivity of the body's immune system in which patients produce antibodies against

Enumerate the signs and symptoms .

a particular target in their own body. Antibodies are the specialised types of protein made by the immune system in response, normally, to intrusion of a foreign body such as a virus. In MG however the protein being attacked is a normal component of the body, which is therefore being attacked by its own immune system. This is what is termed an 'autoimmune disease'. The target is a protein called the acetylcholine receptor (AChR), which is found at the junction between the nerve and the muscle (the neuromuscular junction) In order for a nerve impulse to reach a muscle fibre and cause the muscle to contract, a minute packet of a signalling chemical, acetylcholine, must be released from the end of the nerve and cross the gap to the muscle membrane. Here the acetylcholine molecule fits in to the receptor of the muscle like a key fits in a lock, and the signal is passed: a process known as neuromuscluar transmission. Under normal circumstances this process works very efficiently, but in MG, antibodies against the AChR reduce the efficiency of neuromuscular transmission, and as a result the person experiences muscle weakness particularly when they repeatedly try to use the same muscle. Although we now understand how antibodies to the AChR cause muscle weakness, we do not know why patients with MG develop these autoantibodies. Very rarely an individual can be born with a genetic

abnormality that causes a disorder that resembles MG (congenital myasthenia).

Signs and symptoms Muscle weakness caused by myasthenia gravis worsens as the affected muscle is used repeatedly. Since symptoms typically improve with rest. However, the symptoms of myasthenia gravis tend to progress over time, usually reaching their worst within a few years after the onset of the disease. Although myasthenia gravis can affect any of the muscles that you control voluntarily, certain muscle groups are more commonly affected than others. Eye muscles In more than half the people who develop myasthenia gravis, their first signs and symptoms involve eye problems, such as: Drooping of one or both eyelids (ptosis) Double vision (diplopia), which may be horizontal or vertical Face and throat muscles In about 15 percent of people with myasthenia gravis, the first symptoms involve face and throat muscles, which can cause difficulties with: Altered speaking. Your speech may be very soft or may sound nasal, depending upon which muscles have been affected. Difficulty swallowing. You may choke very easily, which makes it difficult to eat, drink or take pills. In some cases, liquids you're trying to swallow may come out your nose.

Lecture cum discussion

Discuss the diagnostic evaluation

Problems chewing. The muscles used for chewing may wear out halfway through a meal, particularly if you've been eating something hard to chew, such as steak. Limited facial expressions. Family members may note that you've "lost your smile" if the muscles that control your facial expressions are affected. Neck and limb muscles Myasthenia gravis can cause weakness in your neck, arms and legs, but this usually happens in conjunction with muscle weakness in other parts of your body such as your eyes, face or throat. The disorder usually affects arms more often than legs. However, if it affects your legs, you may waddle when you walk. If your neck is weak, it may be hard to hold your head erect. Factors that can worsen myasthenia gravis Fatigue Illness Stress Extreme heat Some medications such as beta blockers, calcium channel blockers, quinine and some antibiotics Diagnostic evaluation Patient History. Physical especially Neurologic examination,

Lecture cum discussion

Tests and diagnosis The key sign that points to the possibility of myasthenia gravis is muscle weakness that improves with rest. Tests to help confirm the diagnosis may include: Edrophonium test Injection of the chemical edrophonium (Tensilon) may result in a sudden, although temporary, improvement in your muscle strength an indication that you may have myasthenia gravis. Edrophonium acts to block an enzyme that breaks down acetylcholine, the chemical that transmits signals from your nerve endings to your muscle receptor sites. Blood analysis A blood test may reveal the presence of abnormal antibodies that Lecture disrupt the receptor sites where nerve impulses signal your muscles cum to move. discussion Repetitive nerve stimulation This is a type of nerve conduction study, in which electrodes are attached to skin over the muscles to be tested. Small pulses of electricity are sent through the electrodes to measure the nerve's ability to send a signal to muscle. Single-fiber electromyography (EMG)

Explain the treatment .

Electromyography (EMG) measures the electrical activity traveling between brain and your muscle. It involves inserting a fine wire electrode through your skin and into a muscle. In single-fiber EMGs, Lecture cum a single muscle fiber is tested. discussion Imaging scans CT scan or an MRI to see if there's a tumor or other abnormality in your thymus. Treatment:- There is no cure for myasthenia gravis, but treatment can help relieve signs and symptoms such as weakness of arm or leg muscles, double vision, drooping eyelids, and difficulties with speech, chewing, swallowing and breathing. Medications

Cholinesterase inhibitors. Drugs such as pyridostigmine (Mestinon) enhance communication between nerves and muscles. These drugs don't cure the underlying problem, but they do improve muscle contraction and muscle strength. Possible side effects may include gastrointestinal upset, excessive salivation and tearing, and frequent urination. Corticosteroids. These types of drugs inhibit the immune system, limiting antibody production. Prolonged use of corticosteroids, however, can lead to serious side effects, such as bone thinning, weight gain, diabetes, increased risk of some infections, and an increase and redistribution of body fat.

Immunosuppressants. Your doctor may also prescribe other medications that alter your immune system, such as azathioprine (Imuran), cyclosporine (Sandimmune, Neoral) or mycophenolate (CellCept). Side effects of immunosuppressants can be serious and may include increased risk of infection, liver damage, infertility and increased risk of cancer.

Therapy

Plasmapheresis This procedure uses a filtering process similar to dialysis. Blood is routed through a machine that removes the antibodies that are blocking transmission of signals from your nerve endings to your muscles' receptor sites. However, the beneficial effects usually last only a few weeks. Repeated treatments can lead to difficulty gaining access to a vein, which may require implanting a catheter, a long, flexible tube, into your chest. Another risk associated with plasmapheresis is a drop in blood pressure. Bleeding occasionally occurs because of the medications used to keep the blood from clotting during the procedure. It's also possible to develop an allergic reaction to the solutions used to replace the plasma or to the sterilizing agents used for the tubing. Intravenous immune globulin. This therapy provides body with normal antibodies, which alters immune system response. It has a lower risk of side effects than do plasmapheresis and immune-suppressing therapy, but it can take a week or two to start working, and the benefits usually last no more than a

month or two. Side effects, which usually are mild, may include chills, dizziness, headaches and fluid retention. Explain the nursing manageme nt is detail. Surgery About 15 percent of the people who have myasthenia gravis have a tumor in their thymus, a gland under the breastbone that is involved with the immune system. need to have thymus removed. For people with myasthenia gravis who don't have a tumor in the thymus, it's unclear whether the potential benefit of removing the thymus outweighs the risks of surgery. Surgery is not recommended by most doctors if:

Your symptoms are mild Your symptoms involve only your eyes You're older than 60

Lecture cum discussion

Nursing interventions

Pain Management: Alleviation of pain or a reduction in pain to a level of comfort that is acceptable to the patient Analgesic Administration: Use of pharmacologic agents to reduce or eliminate pain Environmental Management: Comfort: Manipulation of the patients surroundings for promotion of optimal comfort Anxiety Reduction: Minimizing apprehension, dread,

foreboding, or uneasiness related to an unidentified source or anticipated danger Provision of a modified environment for the patient who is experiencing a chronic confusional state Calming Technique: Reducing anxiety in patient experiencing acute distress Delusion Management: Promoting the comfort, safety, and reality orientation of a patient experiencing false, fixed beliefs that have little or no basis in reality Environmental Management: Safety: Manipulation of the Lecture cum patients surroundings for therapeutic benefit Temperature Regulation: Attaining and/or maintaining body discussion temperature within a normal range Fever Treatment: Management of a patient with hyperpyrexia caused by non-environmental factors Nutrition Management: Assisting with or providing a balanced dietary intake of foods and fluids Weight Gain Assistance: Facilitating gain of body weight. Eating Disorders Management: Prevention and treatment of severe diet restrictions and over exercising or binging and purging of foods and fluids Respiratory Monitoring: Collection and analysis of patient data to ensure airway patency and adequate gas exchange Oxygen Therapy: Administration of oxygen and monitoring of its effectiveness Airway Management: Facilitation of patency of air passages Exercise Therapy: Use of active or passive body movement to

maintain or restore flexibility; use of specific activity or exercise protocols to enhance or restore controlled body movement Fluid Monitoring: Collection and analysis of patient data to regulate fluid balance Hemodynamic Regulation: Optimization of heart rate, preload, afterload, and contractility Lecture Skin Surveillance: Collection and analysis of patient data to cum maintain skin and mucous membrane integrity discussion Pessure Management: Minimizing pressure to body parts Pressure Ulcer Prevention: Prevention of pressure ulcers for a patient at high risk for developing them.

Prepare a nursing process for the patient.

Nursing diagnosis

Acute pain Anxiety Disturbed thought processes Hyperthermia Imbalanced nutrition: Less than body requirements Impaired gas exchange Impaired physical mobility Risk for deficient fluid volume Risk for impaired skin integrity. Nursing interventions

1. Listen to the patients concerns and answer the questions

Discuss the prognosis. Enlist th complicati ons

honestly. 2. Administer medications on time and at evenly spaced intervals, as ordered, to prevent relapses. 3. Plan exercise, meals, patient care, and activities to make the most of energy peaks. 4. When swallowing is difficult, give semi-solid foods instead of liquids to lessen the risk of choking. 5. After severe exacerbations, try to increase social activity as soon as possible. 6. Establish accurate neurologic and respiratory baseline. 7. Stay alert for signs of impending myesthenic crisis such as increased muscle weakness and difficulty talking or chewing. 8. Help the patient plan daily activities to coincide with energy peaks. 9. Stress the need for frequent rest periods. 10.If surgery is scheduled, provide perioperative teaching. Prognosis The prognosis of MG has improved significantly with the introduction of immunosuppressive therapy. The majority of patients become symptom free if they take adequate doses of steroids. However, most patients do have to remain on tablets for an indefinite period as the symptoms generally return if they stop the medication. Complications Complications of myasthenia gravis are treatable, but some can be life-threatening.

Myasthenic crisis Myasthenic crisis is a life-threatening condition, which occurs when the muscles that control breathing become too weak to do their jobs. Emergency treatment is needed to provide mechanical assistance with breathing. Medications and blood-filtering therapies help people recover from myasthenic crisis, so they can again breathe on their own. Thymus tumors About 15 percent of the people who have myasthenia gravis have a tumor in their thymus, a gland under the breastbone that is involved with the immune system. Most of these tumors are noncancerous. Other disorders People who have myasthenia gravis are also more likely to have the following problems: Underactive or overactive thyroid. The thyroid gland, which is in the neck, secretes hormones that regulate your metabolism. If your thyroid is underactive, your body uses energy more slowly. An overactive thyroid makes your body use energy too quickly. Pernicious anemia. This is a condition in which vitamin B-12 is not properly absorbed from the diet, leading to anemia or neurological problems.

Conclusion. So, here we can conclude by saying that as brain is the regulating center of the body and infection can lead to deadly complications. Hence, prompt treatement and wise decision on time is very important for the patien. So as a nurse we should anticipate in providing quality nursing care to a patient in the clinical practice.

REFERENCES 1. B.D. Chaurasias (2004). Human Anatomy, 4th edition CBS publisher and distributer NEW DELHI; volume -1; Pp.265272. 2. Brunner & Suddarth (2009), Text book of Medical Surgical Nursing : 11th edition Wolters kluwer, Lippincott Williams & Wilkins, Volume-1; Pp.723-739. 3. Joycee M. Black ,Jane Hokinson, Howks (2005), Medical surgical nursing: 7th edition Philadelphia : W.B. Saunders. 4. Lewis, Heilkemper, Dirksen, OBrien, Buchers (2007), Medical Surgical Nursing , 7th edition Mosby Elsevier ; Pp. 543-55 5. Lippincott (2006), Mannual of Nursing Practice, 8 th edition JP Indian edition Lippincott Williams & wilkins ; Pp-216-219