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DISEASES DEGENERATIVE

MULTIPLE SCLEROSIS
Alternative names MS Multiple sclerosis is an autoimmune disease that affects the central nervous system (the brain and spinal cord). Causes, incidence, and risk factors   Multiple sclerosis (MS) usually affects woman more than men. The disorder most commonly begins between ages 20 and 40, but can strike at any age. The exact cause is not known, but MS is believed to result from damage to the myelin sheath, the protective material which surrounds nerve cells. It is a progressive disease, meaning the damage gets worse over time. Inflammation destroys the myelin, leaving multiple areas of scar tissue (sclerosis). The inflammation occurs when the body's own immune cells attack the nervous system. The inflammation causes nerve impulses to slow down or become blocked, leading to the symptoms of MS. Repeated episodes, or flare ups, of inflammation can occur along any area of the brain and spinal cord. Symptoms vary because the location and extent of each attack varies. Usually episodes that last days, weeks, or months alternate with times of reduced or no symptoms (remission). Recurrence (relapse) is common although non-stop progression without periods of remission may also occur.

Researchers are not sure what triggers an attack. Patients with MS typically have a higher number of immune cells than a healthy person, which suggests that an immune response might play a role. The most common theories point to a virus or genetic defect, or a combination of both. There also appears to be a genetic link to the disease. MS is more likely to occur in northern Europe, the northern United States, southern Australia, and New Zealand than in other areas. Geographic studies indicate there may be an environmental factor involved. People with a family history of MS and those who live in a geographical area with a higher incidence rate for MS have a higher risk of the disease. Symptom

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weakness of one or more extremities paralysis of one or more extremities tremor of one or more extremities muscle spasticity (uncontrollable spasm of muscle groups) muscle atrophy movement, dysfunctional - slowly progressive; beginning in the legs numbness or abnormal sensation in any area tingling facial pain extremity pain loss of vision -- usually affects one eye at a time double vision eye discomfort

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uncontrollable rapid eye movements eye symptoms worsen on movement of the eyes decreased coordination loss of balance decreased ability to control small or intricate movements walking/gait abnormalities muscle spasms (especially in the legs) dizziness vertigo urinary hesitancy, difficult to begin urinating strong urge to urinate (urinary urgency) frequent need to urinate (urinary frequency) incontinence (leakage of urine, loss of control over urination) decreased memory decreased spontaneity decreased judgment loss of ability to think abstractly loss of ability to generalize depression decreased attention span slurred speech difficulty speaking or understanding speech fatigue, tired easily

The nerves that branch off the (CNS) provide messages to the muscles and organs for normal function. When there is CNS damage, the function of these organs and tissues

Additional symptoms that may be associated with this disease:

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constipation hearing loss positive Babinski's reflex

Note: Symptoms may vary with each attack. They may last days to months, then reduce or disappear, then recur periodically. With each recurrence, the symptoms are different as new areas are affected. Fever can trigger or worsen attacks, as can hot baths, sun exposure, and stress. Signs and tests • • Symptoms of MS may mimic many other neurologic disorders. Diagnosis is made by ruling out other conditions. A history of at least two attacks separated by a period of reduced or no symptoms may indicate one pattern of attack/remission seen in MS (known as relapsing-remitting pattern). If there are observable decreases in any functions of the central nervous system (such as abnormal reflexes), the diagnosis of MS may be suspected. Examination by the health care provider may show focal neurologic deficits (localized decreases in function). This may include decreased or abnormal sensation, decreased ability to move a part of the body, speech or vision changes, or other loss of neurologic functions. The type of neurologic deficits usually indicates the location of the damage to the nerves. Eye examination may show abnormal pupil responses, changes in the visual fields or eye movements, nystagmus (rapid eye movements) triggered by movement of the eye, decreased

visual acuity, or abnormal findings on a fundoscopy (an examination of the internal structures of the eye). Tests that indicate or confirm multiple sclerosis include:

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head MRI scan that shows scarring or a new lesion spine MRI scan that shows scarring or a new lesion lumbar puncture (spinal tap) CSF oligoclonal banding CSF IgG index

Treatment There is no known cure for multiple sclerosis at this time. However, there are promising therapies that may slow the disease. The goal of treatment is to control symptoms and maintain a normal quality of life. Types of treatment include:

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Immune modulators. Patients with a relapsing-remitting course of the disease are often placed on an immune modulating therapy. This requires injection under the skin or in the muscle once or several times a week. It may be in the form of interferon (such as Avonex or Betaseron) or another medicine called glatiramer acetate (Copaxone). They are all similar in their effectiveness and the decision on which to use depends on concerns about particular side effects. Steroids. Steroids are given to decrease the severity of attacks when they occur. These shut the immune system down to stop cells from causing inflammation. Lioresal (Baclofen), tizanidine (Zanaflex), or a benzodiazepine may be used to reduce muscle spasticity. Cholinergic medications to reduce urinary problems. Antidepressants for mood or behavior symptoms. Amantadine for fatigue. Physical therapy, speech therapy, occupational therapy, and support groups can help improve the person's outlook, reduce depression, maximize function, and improve coping skills. Exercise. A planned exercise program early in the course of the disorder can help maintain muscle tone.

Nursing Management:      Eye patch for diplopia Diet: well balanced diet, high in fiber to prevent constipation Avoid hot baths. Heat increases weakness Speech theraoy A healthy lifestyle is encouraged, including good general nutrition. Adequate rest and relaxation can help maintain energy levels. Attempts should be made to avoid fatigue, stress, temperature extremes, and illness to reduce factors that may trigger an MS attack.

Expectations (prognosis)


The outcome is variable and unpredictable. Although the disorder is chronic and incurable, life expectancy can be normal or nearly so. Most people with MS continue to walk and function at work with minimal disability for 20 or more years. The factors felt to best predict a relatively benign course are female gender, young age at onset (less than 30 years), infrequent attacks, a relapsing-remitting pattern, and low burden of disease on imaging studies. The amount of disability and discomfort varies with the severity and frequency of attacks and the part of the central nervous system affected by each attack. Commonly, there is initially a return to normal or near-normal function between attacks. As the disorder progresses, there is progressive loss of function with less improvement between attacks.

Complications

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progressive disability urinary tract infections side effects of medications used to treat the disorder

PARKINSON’S DISEASE
Paralysis agitans; Shaking palsy
Parkinson's disease is a disorder of the brain characterized by shaking (tremor) and difficulty with walking, movement, and coordination. The disease is associated with damage to a part of the brain that is involved with movement. Causes, incidence, and risk factors   It affects both men and women and is one of the most common neurologic disorders of the elderly. Males/ Females; 50- 60 years In some cases the disease occurs within families, especially when it affects young people. Most late onset cases have no known cause. The term "parkinsonism" refers to any condition that involves a combination of the types of changes in movement seen in Parkinson's disease, which is the most common cause of these symptoms. Parkinsonism may be caused by other disorders (secondary parkinsonism) or by external factors like certain medications used to treat schizophrenia.

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Parkinson's disease is caused by progressive deterioration of the nerve cells of the part of the brain that controls muscle movement. Dopamine, which is one of the substances used by cells to transmit impulses, is normally produced in this area. Deterioration of this area of the brain reduces the amount of dopamine available to the body. Insufficient dopamine disturbs the balance between dopamine and other transmitters, such as acetylcholine. Without dopamine, the nerve cells cannot properly transmit messages, and this results in the loss of muscle function. The exact reason that the cells of the brain deteriorate is unknown. The disorder may affect one or both sides of the body, with varying degrees of loss of function. In addition to the loss of muscle control, some people with Parkinson's disease become severely depressed. This may be due to loss of dopamine in certain brain areas involved with pleasure and mood. Lack of dopamine can also affect motivation and the ability to initiate voluntary movements. Although early loss of mental capacities is uncommon, with severe Parkinson's, the person may exhibit overall mental deterioration (including dementia and hallucinations). Dementia can also be a side effect of some of the medications used to treat the disorder. Although rare, when Parkinson's is found in children, it appears to be due to decreased sensitivity of the nerves to dopamine rather than deterioration of the area of the brain that produces dopamine.


Symptoms

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Muscle rigidity o Stiffness o Difficulty bending arms or legs Unstable, stooped, or slumped-over posture Loss of balance Gait (walking pattern) changes Shuffling walk Slow movements Difficulty initiating any voluntary movement o Difficulty beginning to walk o Difficulty getting up from a chair Small steps followed by the need to run to maintain balance Freezing of movement when the movement is stopped, inability to resume movement Muscle aches and pains (myalgia) Shaking, tremors (varying degrees, may not be present) o Characteristically occur at rest, may occur at any time o May become severe enough to interfere with activities o May be worse when tired, excited, or stressed o Finger-thumb rubbing (pill-rolling tremor) may be present Changes in facial expression o Reduced ability to show facial expressions o "Mask" appearance to face o Staring o May be unable to close mouth o Reduced rate of blinking Voice or speech changes o Slow speech o Low volume o Monotone o Difficulty speaking Loss of fine motor skills o Difficulty writing, may be small and illegible

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Difficulty eating Difficulty with any activity that requires small movements Uncontrolled, slow movement Frequent falls Decline in intellectual function (may occur, can be severe) A variety of gastrointestinal symptoms, mainly constipation.

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Note: Initial symptoms may be mild and nonspecific, for instance, a mild tremor or a slight feeling that one leg or foot is stiff and dragging. Additional symptoms that may be associated with this disease:

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Depression Confusion Dementia Seborrhea (oily skin) Loss of muscle function or feeling Muscle atrophy Memory loss Drooling Anxiety, stress, and tension

Signs and tests The health care provider may be able to diagnose Parkinson's disease based on the symptoms and physical examination. However, the symptoms may be difficult to assess, particularly in the elderly. For example, the tremor may not appear when the person is sitting quietly with arms in the lap. The posture changes may be similar to osteoporosis or other changes associated with aging. Lack of facial expression may be a sign of depression. An examination may show "cogwheel" rigidity (jerky, stiff movements), tremors of the Parkinson's type, and difficulty initiating or completing voluntary movements. Reflexes are essentially normal. Tests are not usually specific for Parkinson's, but they may be required to rule out other disorders that cause similar symptoms. See also essential tremor. Treatment   There is no known cure for Parkinson's disease. Treatment is aimed at controlling the symptoms. Treatment must be tailored to the individual. Medications control symptoms primarily by increasing the levels of dopamine in the brain. The type of medication, the dose, the amount of time between doses, or the combination of medications used may need to be adjusted as symptoms change. Many of the medications can cause severe side effects, so monitoring and follow-up by the health care provider is important. Deprenyl may provide some improvement to mildly affected patients. Amantadine or anticholinergic medications may be used to reduce early or mild tremors. Levodopa is a medication that the body converts to a brain transmitter called dopamine. It may be used to increase the body's supply of dopamine, which may improve movement and balance. Carbidopa is a medication that reduces the side effects of levodopa and makes it work better.

Additional medications to help reduce symptoms or control side effects of primary treatment medications include antihistamines, antidepressants, dopamine agonists, monoamine oxidase inhibitors (MAOIs), and others.

Nursing Management  Good general nutrition and health are important. Exercise should continue, with the level of activity adjusted to meet the changing energy levels that may occur. Regular rest periods and avoidance of stress are recommended, because fatigue or stress can make symptoms worse. Physical therapy, speech therapy, and occupational therapy may help promote function and independence. Simple aids, such as railings or banisters placed in commonly used areas of the house or special eating utensils, may be of great benefit to the person experiencing difficulties with daily living activities. Social workers or other counseling services may help the patient cope with the disorder and with obtaining assistance, such as Meals-on-Wheels, as appropriate. Experimental or less common treatments may be recommended. For example, surgery to destroy tissues responsible for tremors may reduce symptoms in some people. Transplantation of adrenal gland tissue to the brain has been attempted, with variable results.

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Expectations (prognosis) Untreated, the disorder progresses to total disability, often accompanied by general deterioration of all brain functions, and may lead to an early death. Treated, the disorder impairs people in varying ways. Most people respond to some extent to medications. The extent of symptom relief, and how long this control of symptoms lasts, is highly variable. The side effects of medications may be severe. Complications

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Varying degrees of disability Difficulty swallowing or eating Difficulty performing daily activities Injuries from falls Side effects of medications :

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Involuntary movements Nausea and vomiting Dizziness Changes in alertness, behavior or mood Severe confusion or disorientation Delusional behavior Hallucinations

HUNTINGTON’S DISEASE
Alternative names Huntington chorea Definition Huntington's disease is an inherited condition characterized by abnormal body movements, dementia, and psychiatric problems. Causes, incidence, and risk factors


Huntington's disease is a progressive disorder involving wasting (degeneration) of nerve cells in the brain. The disorder was first described in 1872 by George Huntington, an American physician. Huntington's disease is inherited as a single faulty gene on chromosome #4. There is a part of the gene that is repeated in multiple copies. The greater the number of repeats, the more likely it is that the person will develop symptoms and the greater the chance they will occur at a younger age. The disease may occur earlier and more severely in each succeeding affected generation because the number of repeats can increase. Every child of a parent with the disorder has a 50% chance of inheriting Huntington's Disease. Symptoms do not usually appear until adulthood, typically between ages 35 and 50. Again, because onset depends on the number of repeats found in the gene, the disease may appear in younger people. In children it may resemble Parkinson's disease with rigidity, slow movements, and tremor. There is progressive loss of mental function, including personality changes, and loss of cognitive functions such as judgment, and speech. Abnormal facial and body movements develop, including quick jerking movements. (The term chorea means "dance" and refers to the typical movements that develop.)

Symptoms

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behavior changes o irritability, moodiness o restlessness, fidgeting o antisocial behaviors o psychosis, paranoia, hallucinations facial movements, grimaces need to turn head to shift the gaze progressive dementia o loss of memory o loss of judgment o speech changes o loss of other functions (calculating, etc.) o personality changes o disorientation or confusion unsteady gait

progressive development of abnormal (choreiform) movements o quick, sudden, jerking movements of arms, legs, face, trunk (movement, unpredictable - jerky) o movement, uncontrolled - slow

Additional symptoms that may be associated with this disease:

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speech impairment anxiety, stress, and tension difficulty swallowing

In children:

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slow movements tremor rigidity

Signs and tests Examination may indicate progressive dementia and choreiform (chorea-like) movements. Reflexes may be abnormal. The gait is often "prancing" and wide. Speech may be hesitant or enunciation poor. A head CT scan may show atrophy (loss of tissue), especially of deep brain (caudate) structures, or other abnormalities. Other tests that may indicate Huntington's disease include:

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head MRI scan PET (isotope) scan of the brain DNA marker studies (gene studies that indicate the tendency to develop this disorder) may be available

Treatment  There is no cure for Huntington's disease, and there is no known way to stop progression of the disorder. Treatment is aimed at slowing progression and maximizing ability to function for as long as possible. Medications vary depending on the symptoms. Dopamine blockers such as haloperidol or phenothiazine medications may reduce abnormal behaviors and movements. Reserpine . Drugs like Tetrabenazine and Amantadine are used to try to control extra movements.

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Nursing Management  Psychiatric illness, depression and suicide are common in Huntington's Disease. It is important for the caretaker and the physicians who care for a person with Huntington's Disease to monitor for symptoms and treat accordingly.

Symptomatic treatment for the dementia is similar to that used for any organic brain syndrome. Initially, reminders and aids may improve memory function. There is a progressive need for assistance and supervision, and 24-hour care may eventually be required. Introduce remotivation therapy, and provide a more stimulating environment.

Expectations (prognosis) Huntington's disease causes progressive disability. The disorder is usually fatal within 15 to 20 years. The cause of death is often infection, although suicide is also common. It is important to realize that not all patients with Huntington's Disease progress at the same pace and are equally affected. The number of repeats may determine severity. People with few repeats may have mild abnormal movements later in life and slow disease progression, whereas those with many repeats may be severely affected at a young age. Complications

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loss of ability to care for self loss of ability to interact injuries to self or others increased risk of infections depression

AMYOTROPHIC LATERAL SCLEROSIS
Alternative names Lou Gehrig's disease; ALS Definition Amyotrophic lateral sclerosis, or ALS, is a disease of the motor nerve cells in the brain and spinal cord. It causes progressive loss of motor control. Causes, incidence, and risk factors  Amyotrophic Lateral Sclerosis is characterized by progressive loss of motor nerves in the spinal cord and brain. In about 10% of cases, ALS is caused by a genetic defect. In other cases, the cause of the nerve deterioration is unknown. ALS affects approximately 1 out of every 100,000 people. Except for having a family member affected with the hereditary form of the disease, there are no known risk factors.

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Symptoms  Symptoms usually do not develop until after age 50. Progressive loss of muscle strength and coordination eventually interfere with the ability to perform routine activities, such as going up steps, getting out of a chair, or swallowing. Occasionally, breathing or swallowing muscles may be the first to be affected. As the disease progresses, more muscle groups are affected and patients become progressively incapacitated. There is no effect on the ability to think or reason.

Symptoms include:

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muscle weakness, decrease in muscle strength and coordination o gradual onset o progressively worsens o commonly involves one limb initially (such as the hand) o progresses to difficulty lifting, climbing stairs, and walking paralysis muscle cramps voice changes, hoarseness speech impairment, slow or abnormal speech pattern difficulty swallowing, gags or chokes easily difficulty breathing (increasing effort required to breathe) head drop due to weak spinal and neck muscles

Additional symptoms that may be associated with this disease:

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muscle contractions muscle spasms muscle atrophy ankle, feet and leg swelling weight loss drooling

Signs and tests A neuromuscular examination indicates weakness, often beginning in one limb or area (such as shoulders or hips). There may be muscle tremors, spasms, twitching, or muscle atrophy (loss of tissue). Atrophy and twitching of the tongue are common. The gait may be stiff or clumsy. Reflexes may be abnormal, including loss of the gag reflex. Some patients have "emotional incontinence" in which it is hard to control crying or laughing.

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An EMG indicates that the motor nerves are not functioning, yet the sensory nerves are normal. A head CT or MRI of head may be done to rule out other conditions. If there is a family history, a genetic test may be performed. A breathing test may be given to see if respiratory muscles are affected. Blood tests can exclude other conditions that may cause similar symptoms.

Treatment and Nursing Management   There is no known cure for ALS. Riluzole may prolong life, but does not reverse or stop disease progression. Promote effective airway clearance.


Treatment is aimed at controlling the symptoms. Baclofen or diazepam may be used to control spasticity that interferes with activities of daily living. Trihexyphenidyl or amitriptyline may be prescribed for people with impaired ability to swallow saliva. Physical therapy, rehabilitation, use of appliances (such as braces or a wheelchair) or orthopedic intervention may be required to maximize muscle function and general health. Choking is common and there may be an early need for placement of a tube into the stomach for feeding (gastrostomy). A referral to an otolaryngologist may be advised. A nutritionist is helpful to prevent weight loss, especially for patients with limitied ability to swallow. The use of devices to assist in breathing includes machines that are only used at night as well as mechanical ventilation. Patients should discuss their wishes regarding artificial ventilation with their doctors Administer oxygen and ventilatory support as needed; suction as needed. Monitor cardiac stats and VS. Monitor fluid status and offer nutritional support. Initiate physical therapy. Auscultate breathe sounds. Encourage client to administer own ADL as long as possible. Monitor bowel and bladder function. Turn every 2 hours as needed; encourage ambulation; avoid strenuous exercise and plan rest periods. Encourage client and family to ventilate feelings. Involve client in a support group. Client education should include avoiding complications, importance of out-patient care, needs of physical mobility, use of assistive devices.

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Expectations (prognosis) There is progressive loss of ability to function or care for oneself. Death often occurs within 3 to 5 years of diagnosis, although there are some rare cases of patients living decades. Complications

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Loss of ability to care for self Inhaling food or fluid Pneumonia Respiratory failure (see adult respiratory distress syndrome) Skin breakdown (pressure sores) Weight loss

MYASTHENIA GRAVIS
Myasthenia gravis is a neuromuscular disorder characterized by variable weakness of voluntary muscles, which often improves with rest and worsens with activity. The condition is caused by an abnormal immune response. Causes, incidence, and risk factors  ↑ in women; young adults

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↓ acetylcholine secretion by motor end plate The cause of autoimmune disorders such as myasthenia gravis is unknown. In some cases, myasthenia gravis may be associated with tumors of the thymus (an organ of the immune system). Patients with myasthenia gavis have a higher risk of having other autoimmune disorders like thyrotoxicosis, rheumatoid arthritis, and systemic lupus erythematosus. Neuromuscular d/o that results in the failure to transmit nerve impulses at MYONEURAL JUNCTION

Symptoms

Vision changes: o Double vision o Difficulty maintaining steady gaze o Eyelid drooping Patients with generalized disease may also have: o Swallowing difficulty, frequent gagging or choking o Weakness or paralysis (may worsen with exertion later in the day) o Muscles that function best after rest o Drooping head o Difficulty climbing stairs o Difficulty lifting objects o Need to use hands to rise from sitting positions o Difficulty talking o Difficulty chewing

Additional symptoms that may be associated with this disease:

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Hoarseness or changing voice Fatigue Facial paralysis Drooling Breathing difficulty

Signs and tests Examination may be normal or may show muscle weakness that progressively worsens as the muscle is used. In many patients the eye muscles are affected first. Reflexes and sensation are normal. Weakness may affect the arms, legs, breathing or swallowing muscles and any other muscle group.

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Standard EMG results are usually normal. Repetitive stimulation (type of nerve conduction studies) are more sensitive. Single-fiber EMG is even more sensitive. Acetylcholine receptor antibodies may be present in the blood. A Tensilon test is positive in some cases but must be interpreted carefully by an experienced doctor. Baseline muscle strength is evaluated. After Tensilon (edrophonium, a medication that blocks the action of the enzyme that breaks down the transmitter acetylcholine) is given, muscle function may improve.

Treatment   There is no known cure for myasthenia gravis. However, treatment may result in prolonged periods of remission. Lifestyle adjustments may enable continuation of many activities. Activity should be planned to allow scheduled rest periods. An eye patch may be recommended if double vision is bothersome. Stress and excessive heat exposure should be avoided because they can worsen symptoms. Some medications, such as neostigmine or pyridostigmine, improve the communication between the nerve and the muscle. Prednisone and other medications that suppress the immune response (such as azathioprine or cyclosporine) may be used if symptoms are severe and there is inadequate response to other medications. Plasmapheresis, a technique in which blood plasma containing antibodies against the body is removed from the body and replaced with fluids (donated antibody-free plasma or other intravenous fluids), may reduce symptoms temporarily and is often used to optimize conditions before surgery. Surgical removal of the thymus (thymectomy) may result in permanent remission or less need for medicines. There are several medications that may make symptoms worse and should be avoided. Therefore, it is always important to check with your doctor about the safety of a medication before taking it. Crisis situations, where muscle weakness involves the breathing muscles, may occur. These attacks seldom last longer than a few weeks. Hospitalization and assistance with breathing may be required during these attacks. Often plasmapheresis is used to help end the crisis.

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Nursing Management        Assess swallowing/ gag reflex before feeding the patient Maintain effective breathing patent airway clearance Administer medications 20- 30 mins a.c. to prevent aspiration. Administer medications at precise time. To prevent respiratory distress w/c may cause death. Monitor client response and side effects to medications Start meal w/ cold beverage. To improve ability to swallow. Avoid exposure to infection. Exacerbations of the disease may be triggered.

Expectations (prognosis)  There is no cure, but long-term remission is possible. There may be minimal restriction on activity in many cases. Patients that only have eye symptoms (ocular myasthenia gravis), may progress to have generalized myasthenia over time. Pregnancy is possible for a woman with myasthenia gravis but should be closely supervised. The baby may be temporarily weak and require medications for a few weeks after birth but usually does not develop the disorder.

Complications

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Restrictions on lifestyle (possible) Side effects of medications (see the specific medication) Complications of surgery Myasthenic crisis (breathing difficulty), may be life threatening

GUILLAIN- BARRE SYNDROME
Alternative names Landry-Guillain-Barre syndrome; Acute idiopathic polyneuritis; Infectious polyneuritis; Acute inflammatory polyneuropathy Definition Guillain-Barre syndrome is a disorder caused by nerve inflammation involving progressive muscle weakness or paralysis, which often follows an infectious illness. Causes, incidence, and risk factors

Guillain-Barre syndrome is an acute type of nerve inflammation. The inflammation damages portions of the nerve cell, resulting in muscle weakness or paralysis and sensory loss. The damage usually includes loss of the myelin sheath of the nerve (demyelination), which slows the conduction of impulses through the nerve. The damage can also cause kill the axon part of the nerve cell (denervation), which stops nerve function entirely. Without the axon, messages cannot be transferred from one nerve cell to another. The exact cause of this disorder is unknown. It may occur at any age but is most common in people of both sexes between the ages 30 and 50. It often follows a minor infection, usually a respiratory (lung) infection or gastrointestinal (gut) infection. Frequently, signs of the original infection have disappeared before the signs of Guillain-Barre begin. Guillain-Barre syndrome may occur in association with viral infections like mononucleosis, AIDS, and herpes simplex, or after infections with bacteria (such as mycoplasma), and some types of diarrhea. Sometimes Guillain-Barre occurs following surgery or vaccinations (such as rabies and swine flu vaccines) or in association with other medical problems such as systemic lupus erythematosus or Hodgkin's disease.

Symptoms  The disorder progresses rapidly (from days to a few weeks), causing weakness or paralysis equally on both sides of the body. As weakness starts in the legs and then spreads to the arms, it is referred to as ascending paralysis. At the same time, patients may notice tingling, foot or hand pain, and clumsiness. As paralysis worsens, the patient may require assisted ventilation in order to breathe. The beginning phase of the illness is a rapid worsening that may take only a few hours to reach the most severe symptoms. These severe symptoms may last up to 3 weeks. This is followed by a plateau phase of no changes, then a recovery phase of improvement over days to 6 months or longer.

Typical symptoms include the following:

Muscle weakness or paralysis (the most common symptom) or uncoordinated movements: o Weakness begins in the feet and legs and may progress upward to the arms and cranial (head) nerves o May progress rapidly over 24 to 72 hours o May begin in the arms and progress downward o May occur in the arms and legs at the same time o May occur in the cranial nerves only o May not occur (mild cases)

Sensation changes o Numbness, decreased sensation o Tenderness or muscle pain (may be a cramp-like pain) o Usually accompanies or precedes muscle weakness o May not occur at all

Additional symptoms that may be associated with this disease:

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Blurred vision Difficulty moving face muscles Complications Clumsiness and falling Breathing difficulty (respiratory failure) Aspiration of food or fluids into the lungs Pneumonia Increased risk of infections Deep vein thrombosis Permanent loss of movement of an area Contractures of joints or other deformity

Palpitations (sensation of feeling heartbeat) • Muscle contractions

Symptoms indicating an emergency:

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Difficulty swallowing Drooling Difficulty breathing Breathing, absent temporarily Inability to take a deep breath Fainting

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Signs and tests   A history of increasing muscle weakness and paralysis may indicate Guillain-Barre syndrome, especially if there was a recent illness. Neurological examination shows muscle weakness and may indicate dysfunction of the control of involuntary (autonomic) body functions such as blood pressure and heart rate. Sensory loss may be minimal, even when sensory changes are present. There may be evidence of decreased breathing ability caused by paralysis of the breathing muscles, and a decrease in deep tendon reflexes in the arms and legs. An NCV (nerve conduction velocity) shows demyelination. An EMG (a test of electrical activity in muscles) shows lack of nervous stimulation. A CSF (cerebrospinal fluid) examination may be abnormal, showing increase in protein without increase in white blood cell count. An ECG may show abnormalities in some cases.

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Treatment  In the past, before treatment was available, many patients recovered completely with time. However, symptoms may be severe during the course of the disorder, and hospitalization is usually required to support breathing function and prevent complications and to provide physical therapy. Plasmaphoresis may decrease the severity of the symptoms and facilitate a more rapid recovery. In this procedure, blood plasma, which contains antibodies, is removed from the body and replaced with intravenous fluids or antibody-free donated plasma. Intravenous immune globulin (IVIg) is equally effective in reducing the severity and duration of the symptoms.

Other treatments are directed at preventing complications such as choking during feeding (through positioning or use of a feeding tube), blood clots (through positioning and sometimes blood thinners), intermittent bladder catheterization, and others. Pain is treated aggressively with anti-inflammatory agents and narcotics if needed.

Nursing Management:   Expectations No specific therapy; asymptomatic, supportive care of paralyzed, immobilized patient (prognosis) ROM Almost all cases  Prevent complications of immobility (95%) survive and  Protect immobile extremities with the use of the majority recover  1) Air mattress  2) Elbows and heel protection completely. Mild  Elevate extremities to prevent dependent edema weakness may  Use antiembolic compression devices blockings persist for some Skin care people. The Respiratory care Care is supportive with attention to function of all systems, especiallyoutcome is most respiratory and cardiac likely to be very Administer chest physiotherapy and pulmonary hygiene measures good when Monitor bowel and bladder function /Turn and position in good alignment

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