Chapter 69+70: ALS, MS, & MG (Module 21/Unit 4)
Thymectomy
A ______________ is a surgical procedure that can produce antigen-specific immunosuppression and result in clinical improvement of myasthenia gravis. This surgery is frequently performed in patients younger than 65 years who have had myasthenia gravis diagnosed within the past 3 years. It is the only treatment that can result in complete remission, which occurs in approximately 35% of patients. Improvement may take several months to several years after surgery to occur. A course of preoperative TPE decreases the time needed for postoperative mechanical ventilation. After the surgery, the patient is monitored in the ICU, with special attention to respiratory function. The patient is weaned from mechanical ventilation after thorough respiratory assessment.
Cholinergic crisis
A ______________ may be caused by overmedication with cholinesterase inhibitors in patient's with myasthenia gravis. However, this is rare. Symptoms include: salivation, lacrimation, urination, defecation, hypotension, bronchospasm, and severe bradycardia.
Myasthenic crisis
A ________________ is an exacerbation of myasthenia gravis characterized by severe generalized muscle weakness, respiratory and bulbar weakness that may result in respiratory failure. This may result from disease exacerbation or a specific precipitating event. The most common precipitator is respiratory infection; others include medication change, surgery, pregnancy, and medications that exacerbate myasthenia.
Edrophonium (Tensilon)
A common test to diagnose myasthenia gravis is the acetylcholinesterase inhibitor test. It is performed by administering __________________ intravenously. 30 seconds after the injection, facial muscle weakness and ptosis should resolve for about 5 minutes. Immediate improvement in muscle strength after administration of this agent represents a positive test and usually confirms the diagnosis. Atropine should be available to control the potential side effects of this medication which include bradycardia, asystole, bronchoconstriction, and cramping.
Acetylcholinesterase inhibitor test (Tensilon test)
A common test used to diagnose mysathenia gravis is the _________________________. It is performed by administering edrophonium chloride (Tensilon) intravenously. 30 seconds after the injection, facial muscle weakness and ptosis should resolve for about 5 minutes. Immediate improvement in muscle strength after administration of this agent represents a positive test and usually confirms the diagnosis. Atropine should be available to control the potential side effects of this medication which include bradycardia, asystole, bronchoconstriction, and cramping.
Myasthenia gravis
A number of medications are contraindicated for patients with ______________ because they exacerbate symptoms. Procaine (Novocaine) should be avoided, and the patient's dentist should be informed of the diagnosis of _____________.
Myasthenia gravis
Another diagnostic test used for patients with _____________ who have cardiac conditions or asthma (which the Tensilon test is contraindicated) this test consists of an ice pack held over the patient's eyes for one minute , the ptosis should temporarily resolve in a patient with _________________.
Gabapentin
Ataxia is a chronic problem most resistant to treatment in patients with MS. Medications used to treat this include beta blockers (propanolol), the anticonvulsant agent __________; and benzodiazepines (e.g., clonazepam).
Myasthenia gravis
Because ____________ is a chronic disease and most patients are seen on an outpatient basis, much of the nursing care focuses on patient and family education. Educational topics include: medication management, energy conservation, strategies to help with ocular manifestations, and prevention, and management of complications. Medication management is a crucial component of ongoing care. Understanding the actions of the medications and taking them on a schedule is emphasized, as well as the consequences of delaying medication. The nurse should educate the patient of signs and symptoms of myasthenic and cholinergic crisis.
No
Does a cure exist for ALS?
Amantadine
Fatigue in MS that interferes with ADLs may be treated with ___________.
Atropine
For patients being administered the acetylcholinesterase inhibitor test to aid in the diagnosis of myasthenia gravis with edrophonium chloride (an AChesterase inhibitor) the possible side effects of lingering acetylcholine can attribute to bradycardia, asystole, bronchoconstriction, sweating and cramping. Thus, the antidote ______________ should be available to control and reverse these effects.
Myasthenic crisis
For patients with a ____________ assessment strategies and supportive measures include the following: - ABGs, serum electrolytes, I&O, and daily weight are monitored. - If the patient cannot swallow, enteral feedings may be prescribed. - Sedative and tranquilizing agents are avoided because they aggravate hypoxia and hypercapnia, and can cause respiratory and cardiac depression.
Immunomodulating drugs
If pyridostigmine does not improve muscle strength and control fatigue in patients with myasthenia gravis , the next agents used are ____________________. The goal of this is to reduce the production of the antibodies. Corticosteroids suppress the patient's immune response, decreasing the amount of antibody production, and this correlates with clinical improvement.
ALS
In about 25% of patients with ______________ weakness starts in the muscles supplied by the cranial nerves and difficulty in talking, swallowing, and ultimately breathing occurs. When the patient ingests liquids, soft palate and upper esophageal weakness cause the liquid to be regurgitated through the nose. If bulbar muscles are impaired, speaking and swallowing are progressively difficult, and aspiration becomes a risk. The voice assumes a nasal sound and articulation becomes so disrupted that speech is unintelligible.
Choking and aspiration
In myasthenia gravis, laryngeal involvement produces dysphonia and dysphagia which increases the risk of _________ and ___________.
Diplopia and ptosis
In myasthenia gravis, the initial clinical manifestations are due to the effects of the ocular muscles, which result in ____________ (double vision) and _________ (eyelid drooping) being a common finding.
Generalized weakness
In patient's with myasthenia gravis _______________________ affects all extremities and intercostal muscles, resulting in decreasing vital capacity and respiratory failure. This disease is purely a motor disorder with no effect on sensation or coordination.
Heat; cold
In patients with MS exposure to _________ may increase fatigue and muscle weakness. Exposure to extreme ___________ may increase spasticity.
Ataxia
In patients with MS involvement of the cerebellum or basal ganglia can produce ____________ , which is impaired coordination of movements and tremor. This may also cause emotional lability and euphoria.
Vision
In patients with myasthenia gravis impaired __________ results from ptosis of one or both eyelids, decreased eye movement, or double vision (diplopia). To prevent corneal damage when the eyelids do not close completely, the patient is instructed to tape the eyes closed for short intervals and to regularly instill artificial tears. Patching one eye can also help with double vision.
Cholinesterase medications
Maintenance of stable blood levels of __________ is imperative to stabilize muscle strength. Therefore, the _______________ must be given on time. Any delay in administration of these medications may exacerbate muscle weakness and make it impossible for the patient to swallow the medications.
Myasthenia gravis
Management of _____________ is directed at improving function and reducing and removing circulating antibodies. Therapeutic modalities include: - Acetylcholinesterase inhibitor medications - Immunosuppressive therapy - Intravenous immune globulin (IVIG) - Therapeutic plasma exchange (TPE) - Thymectomy However, there is no cure the treatments do not stop the production of acetylcholine receptor antibodies.
CNS
Multiple sclerosis is an autoimmune demyelinating disorder affecting the ____________________ (which part of the nervous system? PNS or CNS)
Presence of CNS plaques on MRI; IgG banding in CSF
Multiple sclerosis is diagnosed based upon which diagnostic findings? The second answer indicates that an immune system abnormality is occurring.
Respiratory failure
Neuromuscular _____________ is the critical complication in a myasthenic and cholinergic crisis. Respiratory muscle and bulbar weakness combine to cause respiratory compromise. Weak respiratory muscles do not support inhalation. An inadequate cough and gag reflex caused by bulbar weakness, result in poor airway clearance. A downward trend of two respiratory function tests, the negative inspiratory force and vital capacity, is the first clinical sign of respiratory compromise. ET intubation and mechanical ventilation may be needed. Cholinesterase inhibitors are stopped when __________ occurs and are gradually restarted after the patient demonstrates improvement.
Multiple sclerosis
No cure exists for _______________, so an individual treatment program is indicated to relieve the patient's symptoms and provide continuing support, particularly for the patients with cognitive changes. The goals of treatment are to delay the progression, manage chronic symptoms, and treat acute exacerbations. Common symptoms requiring intervention include ataxia, bladder dysfunction, depression, fatigue, and spasticity.
Multiple Sclerosis
Nursing assessment for patients with ___________________ address neurological deficits, secondary complications, and the impact of the disease on the patient and family. The patient's mobility and balance are observed to determine whether there is a risk of falling. The patient is assessed for weakness, spasticity, visual impairment, incontinence, and disorders of swallowing and speech.
Minimize effects of immobility
Nursing interventions in patients with MS in order to ______________________ are because the decreased physical activity that often occurs with MS leads to complications associated with immobility including pressure ulcers, expiratory muscle weakness, and accumulation of bronchial secretions. Measures to prevent such complications include assessing and maintaining skin integrity, and having the patient utilize coughing and deep-breathing exercises.
Enhance bowel and bladder control
Nursing interventions in patients with MS in order to ______________________ is because of the multitude of bladder symptoms. The patient with urinary frequency, urgency, or incontinence should have a bedpan or urinal readily available. A voiding schedule is set up (every 1.5 to 2 hours initially) The nurse should encourage the patient to take prescribed medications for bladder spasticity. Also, intermittent catheterization has been successful in maintaining bladder control in patients with MS. Bowel problems include constipation, fecal impaction, and incontinence. Adequate fluids, dietary fiber, and bowel training programs are frequently effective in solving these problems.
Prevent injury
Nursing interventions in patients with MS in order to ______________________ is due to possible motor dysfunction that causes problems of incoordination and clumsiness, or if ataxia is apparent, the patient is at risk for falls. To overcome this disability, the patient is instructed to walk with the feet apart to widen the base of support and to increase stability. If loss of position sense occurs, the patient is instructed to watch the feet while walking. If incoordination is a problem and tremor of the upper extremities is apparent, weighted bracelets or wrist cuffs are helpful.
Minimizing spasticity and contractures
Nursing interventions in patients with MS in order to ______________________ is utilized because muscle spasticity is common and in its later stages is characterized by severe adductor spasm of the hips with flexor spasm of the hips and knees. Without relief fibrous contractures of these joints occur. Warm packs may be beneficial, but hot baths should be avoided because the risk of burn injury secondary to sensory loss and increasing symptoms that may occur with elevation of the body temperature. Daily exercises for muscle stretching are prescribed to minimize joint contractures. Special attention is given to the hamstrings, gastrocnemius muscles, hip adductors, biceps, wrists, and finger flexors.
Communication and swallowing
Nursing interventions in patients with MS in order to enhance ______________________ are because some patients may have impaired effects from cranial nerves that control mechanisms of speech and swallowing, which lead to dysarthrias and dysphagia. A speech-language pathologist evaluates speech and swallowing and instructs the family and healthcare team about strategies. This places the patient at risk for aspiration. To reduce this risk, the nurse should implement strategies such as having a suction apparatus available, ensuring careful feeding, confirming correct food and liquid consistency, and positioning properly for eating.
Vision
Nursing interventions in patients with MS in order to help with ______________________ are because the cranial nerves affecting vision may be implicated in MS. An eye patch or covered eyeglass lens may be used to block the visual impulses of one eye if the patient has diplopia. Prism glasses may be helpful for patients who are confined to bed and have difficulty reading in the supine position.
Nutrition
Nursing interventions in patients with MS in order to promote ______________________ is owed to the fact that many patients with MS are overweight or have obesity. Contributing factors include the use of corticosteroids for exacerbation of symptoms and mobility impairments as a result of the disease. Interventions to promote healthy eating and weight reduction need to take into account that fatigue and mobility impairments are barriers to nutritional behaviors. The nurse should also educate to avoid alcohol and cigarette smoking.
Activity and rest
Nursing interventions in patients with MS in order to promote ______________________ is utilized to encourage the patient to work and exercise to a point just short of fatigue. Very strenuous exercise is not advisable, because it raises the body temperature and may aggravate the symptoms. The patient is advised to take frequent, short rest periods, preferably lying down.
Thymus
Of the people with myasthenia gravis about 75% either have __________ hyperplasia, or a _________ tumor. The ____________ is believed to be the site of the antibody production.
MG
Other diagnostic tests to aid in the diagnosis of __________ include: - Several blood test for ACh antibodies. - Repetitive nerve stimulation (RNS) demonstrates a decrease in successive action potentials. - Electromyography detects a delay or failure of neuromuscular transmission and is about 99% sensitive in confirming the diagnosis. - The thymus gland, a site of acetylcholine receptor antibody production may be enlarged and may be identified by MRI scan.
MS
Researchers reported that patients with _________ have higher rates of depression, arthritis, diabetes, coronary artery disease, migraine headaches, and cancer. Additional complications include UTIs, constipation, pressure ulcers, contracture deformities, dependent pedal edema, pneumonia, and osteoporosis.
Myasthenic crisis
Respiratory distress and varying degrees of dysphagia, dyarthria (difficulty speaking), eyelid ptosis, diplopia, and prominent muscle weakness are symptoms of __________________. This patient is placed in an ICU for constant monitoring because of the associated intense and sudden fluctuations of the clinical condition. Providing ventilatory assistance takes precedence in the immediate management of the patient with ___________. Ongoing assessment for respiratory failure is essential. The nurse assesses the respiratory rate, depth, and breath sounds and monitors pulmonary function parameters (vital capacity and negative inspiratory force) to detect pulmonary problems and mechanical ventilation may be needed. If abdominal, intercostal, and pharyngeal muscles are severely weak, the patient cannot cough, deep breath, or clear secretions.
ALS
Risk factors that have been identified for ___________ include: - Smoking - Viral infections - Autoimmune disease - Environmental exposure to toxins.
True
T/F: Postural drainage should not be performed for 30 minutes or longer after eating.
Thymus gland
The ___________ a site of acetylcholine receptor antibody production may be enlarged and may be identified by MRI scan.
20-50 years
The age of onset for multiple sclerosis is what?
Lower motor neuron
The characteristics of Myasthenia Gravis is due to what type of motor neuron dysfunction? Upper or lower? Hint: Weakness and muscle fatigue
ALS
The clinical manifestations of ________ depend on the location of the affected neurons, however the chief symptoms are: - Fatigue - Progressive muscle weakness - Cramps - Fasciculations (twitching) - Lack of coordination. Spasticity is usually present, and deep tendon stretch reflexes become brisk and overactive. Usually, the function of the anal and bladder sphincters remain intact.
MS
The clinical manifestations of ___________ may assume many different patters, which may be mild or severe and reflect the location of the lesion (plaque). The main symptoms most commonly reported are: - Fatigue (affects most with this and is often the most disabling symptom) - Depression - Weakness - Numbness - Difficulty in coordination - Loss of balance - Spasticity - Pain (due to lesions) - Visual disturbances due to lesions may include blurred vision, diplopia, scotoma (patchy blindness) and total blindness. Primary _______ may result in quadriparesis, cognitive dysfunction, visual lose, and brainstem syndromes.
MS
The diagnosis of ______________ is based on the presence of multiple plaques in the CNS observed with an MRI Additional studies augment the diagnosis such as electrophoresis of CSF that identifies the presence of oligoclonal banding (several bands of immunoglobulin G bonded together, indicating an immune system abnormality).
Myasthenia gravis
The initial clinical manifestation of _____________ in 80% of patients involve the ocular muscles. Diplopia and ptosis (drooping of the eyelids) are common. Many patients also experience weakness of the muscles of the face and throat (bulbar symptoms) and generalized weakness. Weakness of the facial muscles result in a bland facial expression. Laryngeal involvement produces dysphonia (voice impairment) and dysphagia which increases the risk of choking and aspiration.
Death of motor neurons due to glutamate
The leading etiological theory of ALS is what? This results in cell injury and neuronal degeneration and atrophy and death of motor neurons.
ALS
The main focus of medical and nursing management for ___________ is to maintain or improve function, well-being, and quality of life. Riluzole (Rilutek) is a glutamate antagonist that has been shown to prolong survival for people with __________ for about 3 to 6 months. The action is not clear, but its pharmacological properties suggest a neuroprotective effect in the early stages. Baclofen (lioresal) and dantrolene sodium or diazepam may be useful for patients troubled by spasticity, which causes pain and interferes with self care. Modafinil may be used for fatigue. Additional medicines may be added to manage the pain, depresion, drooling, and constipation.
MS
The major goals for the patient with ____________ may include - Promotion of physical mobility - Avoidance of injury - Achievement of bladder and bowel continence - Promotion of speech and swallowing mechanisms - Improvement of cognitive function - Development of coping strengths - Improved home maintenance management - Adaption to sexual dysfunction.
Respiratory infection
The most common precipitator for a myasthenic crisis is _______________. However, medication change, surgery, pregnancy, and medications that exacerbate myasthenia.
ALS
The most common reasons for hospitalization for patients with _______________ are dehydration, malnutrition, pneumonia, and respiratory failure. Mechanical ventilation (using negative pressure) is an option as well as non-invasive ventilators. If the patient is experiencing aspiration and swallowing difficulties they may require enteral feeding.
Myasthenia crisis
The nurse should educate the patient on factors that may exacerbate symptoms of myasthenia gravis and cause a ______________ should be avoided such as: - Emotional stress - Infections (particularly respiratory) - Vigorous physical activity. - Certain medications (provocaine) - High environmental temperatures.
MS
The pathophysiology of ____________ includes autoimmunity infiltration in the CNS that leads to immune system attack and inflammation that destroys the myelin (insulates the axon and speeds up conduction of impulses) and the oligodendroglial cells that produce the myelin in the CNS. This interrupts the flow of nerve impulses and results in a variety of manifestations depending on the nerves affected. Demyelinated axons are scattered irregularly throughout the CNS. The areas most commonly effected are the optic nerves, chiasm, and tracts; cerebrum; brainstem; cerebellum; and spinal cord. The axons themselves begin to degenerate; resulting in permanent and irreversible damage.
Mysathenia gravis
The pathophysiology of _______________ is described as follows: Normally, a chemical impulse precipitates the release of acetylcholine from the nerves at the myoneural junction. The acetylcholine attaches to receptor sites on the motor endplate and stimulates muscle contraction. Continuous binding of acetylcholine to the receptor site is required for muscle contraction to be sustained. In this disease, antibodies directed at acetylcholine receptor sites impair transmission of impulses across the myoneural junction. Therefore, fewer receptors are available for stimulation, resulting in voluntary muscle weakness that escalates with continued activity. These antibodies are found in 80-90% of people with this disease. Of the people with this 75% either have thymic hyperplasia, or a thymic tumor, and the thymus gland is believed to be the site of the antibody production.
ALS
The prognosis of _________ is generally based on the area of CNS involvement and the speed at which the disease progresses. Eventually, respiratory function is compromised. Death usually occurs as a result of infection, respiratory insufficiency, or aspiration.
Aspiration
To minimize the risk of ___________ in patients with myasthenia gravis, mealtimes should coincide with the peak effects of anticholinesterase medications. In addition, rest before mealtimes is encouraged to reduce muscle fatigue. The patient is advised to sit upright during meals, with the neck slightly flexed to facilitate swallowing. Eating larger meals in the morning and smaller meals of the evening is another good strategy. If choking occurs frequently, the patient should be evaluated by a speech therapist, and suction should be available at home.
Autoimmune disease resulting in demyelination of the CNS
What is the pathophysiology of Multiple Sclerosis?
Antibodies at acetylcholine receptors (autoimmune reaction)
What is the pathophysiology of myasthenia gravis? Therefore, fewer receptors are available for stimulation, resulting in voluntary muscle weakness that escalates with continued activity. These antibodies are found in 80-90% of people with myasthenia gravis.
Severe generalized muscle weakness, respiratory/bulbar weakness, respiratory failure
What manifestations occur as a result of a myasthenic crisis?
Cardiac conditions and asthma
What type of patients should not have the acetylcholinesterase inhibitor test (Tensilon test) for a diagnosis of Myasthenia Gravis?
Multiple sclerosis
Which disorder between ALS, MG, and MS would be most likely to experience tinnitus?
Multiple sclerosis
Which disorder is the following symptoms classic for? - Fatigue - Diplopia - Spasticity - Ataxia
Sedatives
_________ and tranquilizing agents are avoided in patients with myasthenia gravis/myasthenic crisis because they aggravate hypoxia and hypercapnia, and can cause respiratory and cardiac depression.
Bulbar weakness
__________ is weakness in muscles with innervation from brainstem, this is seen in myasthenia gravis and can result in trouble speaking, and swallowing.
Dysphonia
___________ is voice impairment or altered voice production.
Procaine (Novocain)
___________ should be avoided in patients with myasthenia gravis, and the patient's dentist should be informed of the diagnosis of myasthenia gravis.
Spasticity
____________ is a symptom that occurs in 90% of patients with MS, most often in the lower extremities and can include the loss of abdominal reflexes. This results from the involvement in the main motor pathways (pyramidal tracts) of the spinal cord. Some degree of cognitive changes (e.g., memory loss, decreased concentration) occurs in about half of the patients.
Hemiplegia
____________ is paralysis of one side of the body, or part of it, due to an injury in the motor area of the brain.
Ptosis
_____________ is drooping of the eyelids.
Ataxia
_____________ is impaired coordination of movement during voluntary movement.
Hemiparesis
_____________ is weakness on one side of the body, or part of it, due to an injury in the motor area of the brain.
Benzodiazepines and dantrolene
______________ and __________ may also be used to treat spasticity in patient's with MS.
Secondary progressive MS
______________ is a clinical course of MS that is a progression from the initial relapse-remitting type but is characterized by periods of variability with occasional relapses and minor remissions.
Prion
______________ is a pathogen smaller than a virus that is resistant to sterilization procedures.
Disease modifying therapies
______________ is a type of pharmacological therapy for the treatment of MS that helps reduce frequency of relapse, the duration of relapse, and the number and size of plaques observed on MRI in the RR forms of MS but are NOT effective in the primary progressive type. - Interferon beta may be given. Side effects include flu-like symptoms which cause a majority of patients to discontinue therapy. For optimal results these should be started early in the disease. - Glatiramer acetate also reduces the rate of relapse in the RR course. This may take 6 months for evidence. - IV methylprednisolone is the key agent in treating acute relapse in the RR course, and shortens the duration of relapse, but has not been found to have long-term effect.
Multiple sclerosis (MS)
______________ is an immune-mediated, progressive demyelinating disease of the CNS. This can occur at any age, but the age of peak onset is between 25 and 35 years of age; affecting women more frequently than men. The cause is ongoing research, but autoimmune activity results in demyelination. This is considered to have many risks, including genetic factors (HLA); viruses that trigger the autoimmune response; smoking, lack of vitamin D exposure, and exposure to the Epstein-Barr virus.
Pain
______________ is another common symptom of MS that can contribute to social isolation. Lesions on the sensory pathways cause _________. Additional sensory manifestations with this include paresthesias, dysethesias, and proprioceptions. Many people with MS need daily analgesic medications such as opioids, anticonvulsants, or antidepressants.
ALS
______________ is diagnosed on the basis of signs and symptoms, because no clinical or laboratory tests are specific to this disease. Electromyography and muscle biopsy studies to the affected muscles indicate reduction in the number of functioning motor units. A MRI scan may show high signal sensitivity in the corticospinal tracts; which differentiates ________ from multifocal motor neuropathy.
Diplopia
______________ is double vision.
IV methylprednisolone
______________ is the key agent in treating acute relapse in the RR course, and shortens the duration of relapse, but has not been found to have long-term effect.
ALS
______________ most commonly occurs between 40 and 60 years of age and affects all social, racial, and ethnic backgrounds with men being affected in slightly higher rates than women. The majority of cases arise sporadically. About 30% of patients with this live for 5 years after diagnosis, with 10 to 20% surviving up to 10 years and 5% surviving greater than 20 years.
IVIG
_______________ can be used to treat exacerbations of myasthenia gravis, however, in selected patients it is used long term. This involves the administration of pooled human gamma-globulin and improvement occurs in a few days. The effects of ____________ typically last only 28 days after infusion. Complications: Headache, migraine exacerbation, aseptic meningitis, and flu-like symptoms.
Riluzole (Rilutek)
_______________ is a glutamate antagonist that has been shown to prolong survival for people with __________ for about 3 to 6 months. The action is not clear, but its pharmacological properties suggest a neuroprotective effect in the early stages.
Fatigue
_______________ is a symptom of MS that affects most people with this disease. This is often the most disabling symptom. Heat, depression, anemia, deconditioning, and medication may contribute to _________________. Avoiding hot temperatures, effective treatment of depression and anemia, and a change in medication as well as OT/PT may help manage ____________.
Cytotoxic medications
________________ are used to treat myasthenia gravis if there is inadequate response to steroids. Azathioprine (Imuran) inhibits T lymphocytes and B-cell proliferation and reduces acetylcholine receptor antibody levels. Therapeutic effects may not be evident for 3 to 12 months. Leukopenia and hepatotoxicity are serious effects, so monthly evaluation of liver enzymes and white blood cell counts are necessary.
Baclofen
________________ is a gamma-aminobutyric acid agonist and is the medication of choice for treating spasticity in patients with MS. It can be given orally or intrathecal injection for severe spasticity.
Relapsing-remitting MS
________________ is a type of clinical progression of patients with MS that includes clearly acute attacks with full recovery. However, residual deficits may occur and accumulate overtime, contributing to functional decline. Over time, most patients with this course of MS progress to a secondary progressive course, in which disease progression occurs with or without relapses.
Pyridostigmine (Mestinon)
________________ is an acetylcholinesterase inhibitor medication that is first line therapy for treatment of myasthenia gravis. MOA: Provides symptomatic relief by inhibiting the breakdown of acetylcholine, and increasing the relative concentration of available acetylcholine at the neuromuscular junction. The dosage is usually gradually increased to a daily maximum and given in divided doses (4x daily). Adverse effects: Diarrhea, abdominal cramps, excessive saliva. This drug tends to have fewer side effects than other anticholinesterase medications.
Progressive relapsing ms
_________________ is a clinical course of MS that is characterized by relapses with continuous disabling progression between exacerbations (least common) In other words, the progression from onset is presented with clear relapses without recovery.
Amyotrophic lateral sclerosis (ALS)
_________________ is a disease of unknown cause in which there is a loss of motor neurons in the anterior horns of the spinal cord and the motor nuclei of the lower brainstem. It is often referred to as Lou Gehrig disease, after the famous baseball player who suffered from this. As motor neuron cells die, the muscle fibers that they supply undergo atrophic changes. This degeneration may occur in both the upper and lower motor neuron systems. The leading theory held by researchers is that overexcitation of nerve cells by the neurotransmitter glutamate results in cell injury and neuronal degeneration. Risk factors that have been identified are smoking, viral infection, autoimmune disease, and environmental exposure to toxins. However, the exact cause is still unknown and requires further exploration.
Therapeutic plasma exchange (plasmapheresis)
_________________ is a technique, formerly referred to as plasmapheresis, and is used to treat exacerbations of myasthenia gravis. The patient's plasma and plasma components are removed through a centrally placed large-bore double lumen catheter. The blood cells and antibody-containing plasma are separated, after which the cells and the plasma substitute are reinfused. Temporary reduction in the level of circulating antibodies is provided with this. A typical course consists of daily or alternate-day treatment. Symptoms improve in 75% of patients, however, improvement only lasts a few weeks after treatment is completed.
Primary progressive MS
_________________ is a type of clinical course of MS where the disease's disabling symptoms steadily increase, with rare plateaus and temporary minor involvement. This may result in quadriparesis, cognitive dysfunction, visual loss, and brainstem syndromes.
Older adults
_________________ who have a diagnosis of MS have specific physical and psychosocial challenges. They may have chronic health problems for which they may be taking additional medications that could interact with medications prescribed for MS. They must be monitored closely for adverse and toxic effects of MS medications and for osteoporosis.
Myasthenia Gravis
___________________ is an autoimmune disorder affecting the myoneural junction , and is characterized by varying degrees of weakness of the voluntary muscles. More women are affected than men, and is common in the 20's and 30's, however, after age 50 the gender distribution is more equal.
