Multiple Sclerosis Chapter 59

Multiple Sclerosis Chapter 59
Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Copyright © 2014 by Mosby, an imprint of Elsevier Inc.
Multiple Sclerosis Chronic, progressive, degenerative disorder of the central nervous system (CNS) Characterized by segmental demyelination of nerve fibers of the brain and spinal cord Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Multiple Sclerosis Onset between 20 and 50 years of age Although it can occur in young teens and much older adults Women are affected 2-3 times more than men. More prevalent in temperate climates MS is 5 times more prevalent in temperate climates (between 45 and 65 degrees of latitude), such as those found in the northern United States, Canada, and Europe, as compared with tropical regions. Migration from one geographic area to another may alter a person’s risk of developing MS. Immigrants and their descendants tend to take on the risk level (either higher or lower) of the area to which they move. The change in risk may not appear immediately. For example, African Americans (in the United States) have a prevalence rate that is 40% that of European Americans whereas Africans (in Africa) are thought to have a prevalence rate of approximately 1% that of European Americans. The variations in incidence of MS suggest that geography, ethnicity, and other factors interact in a very complex way to cause MS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Etiology and Pathophysiology
Cause is unknown. Related to infectious, immunologic, and genetic factors Multiple genes confer susceptibility to multiple sclerosis (MS). Research suggests that it is unlikely MS is related to a single cause. Researchers believe the disease develops in a genetically susceptible person as a result of environmental exposure, like an infection. Multiple genes are believed to be involved in the inherited susceptibility to MS and first-, second-, and third-degree relatives of patients with MS are at an increased risk. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Association between precipitating factors and MS is controversial. Infection, smoking, physical injury, emotional stress, excessive fatigue, pregnancy, and a poorer state of health The role of precipitating factors such as exposure to pathogenic agents in the etiology of MS is controversial. It is possible that their association with MS is random and that there is no cause-and-effect relationship. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Three pathologic processes characterize MS Chronic inflammation Demyelination Gliosis in the CNS Primary neuropathologic condition is an autoimmune process orchestrated by activated T cells. This process may be triggered initially by an environmental factor or virus in genetically susceptible individuals. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Activated T cells migrate to CNS, causing blood-brain disruption. Likely the initial event in the development of MS Subsequent antigen-antibody reaction leads to demyelination of axons. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Case Study iStockphoto/Thinkstock 35-year-old L.S. presents to clinic after experiencing intermittent generalized weakness and blurred vision. She also complains of feeling an “electric shock” down her spine and chronic fatigue. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Case Study All blood tests for L.S. are normal. MRI reveals small plaques throughout brain. A diagnosis of multiple sclerosis is made. What possible triggers could be related to her MS? iStockphoto/Thinkstock Possible triggers are infection (especially upper respiratory and urinary tract infection), trauma, immunization, delivery after pregnancy, stress, and change in climate. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Pathogenesis of MS A, Normal nerve cell with myelin sheath. B, Normal axon. C, Myelin breakdown. D, Myelin totally disrupted; axon not functioning. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Initially, attacks cause damage to myelin sheaths of neurons in brain and spinal cord. Nerve fiber is not affected. Patient may complain of noticeable impairment of function. Transmission of nerve impulses still occurs, though transmission is slowed. At this point, the myelin can regenerate, and the symptoms will disappear. Therefore the patient experiences a remission. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

With ongoing inflammation, myelin loses ability to regenerate. Nerve impulse transmission is disrupted without myelin. {See next slide for figure.} As ongoing inflammation occurs, the nearby oligodendrocytes are affected, and the myelin loses the ability to regenerate. Eventually damage occurs to the underlying axon. Nerve impulse transmission is disrupted, resulting in the permanent loss of nerve function (Fig. 59-3, D). As inflammation subsides, glial scar tissue replaces the damaged tissue, leading to the formation of hard, sclerotic plaques. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Chronic Multiple Sclerosis
Demyelination plaque (P) at gray-white junction and adjacent partially remyelinated shadow plaque (V). These plaques are found throughout the white matter of the CNS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Clinical Manifestations
Common manifestations of MS include motor, sensory, cerebellar, and emotional problems. Vague symptoms occur intermittently over months and years. Disease may not be diagnosed until long after onset of the first symptom. The intermittent nature of the symptoms often dissuade the patient from seeking medical attention. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Characterized by Chronic, progressive deterioration in some Remissions and exacerbations in others Progressive deterioration in neurologic function with repeated exacerbations Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Classification scheme with four primary patterns of MS Relapsing-remitting Primary-progressive Secondary-progressive Progression-relapsing Since the disease process has a spotty distribution in the CNS, the clinical manifestations will vary with each patient according to the areas of the CNS involved. Some patients have severe, long-lasting symptoms early in the course of the disease. Others may experience only occasional and mild symptoms for several years after onset. See Table for more information. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Common manifestations of MS Motor problems Sensory problems Cerebellar problems Emotional problems {These are expanded in upcoming slides.} Average life expectancy after the onset of symptoms is more than 25 years. Death usually occurs as the result of infectious complications (e.g., pneumonia) of immobility or because of an unrelated disease. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Motor manifestations Weakness or paralysis of limbs, trunk, and head Diplopia Scanning speech Spasticity of muscles Muscles are chronically affected. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Sensory manifestations Numbness and tingling Blurred vision Vertigo and tinnitus ↓ hearing Chronic neuropathic pain Lhermitte’s sign Patients with MS experience a variety of sensory abnormalities. In addition to those listed above, other paresthiesias, patchy blindness (scotomas). Radicular (nerve root) pains may be present, particularly in the low thoracic and abdominal regions. Lhermitte’s sign is a transient sensory symptom described as an electric shock radiating down the spine or into the limbs with flexion of the neck. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Cerebellar manifestations Nystagmus Ataxia Dysarthria Dysphagia Fatigue Cerebellar signs include nystagmus, ataxia, dysarthria, and dysphagia. Severe fatigue is present in many MS patients and causes significant disability for some patients. The fatigue is usually associated with increased energy needs, heat, humidity, deconditioning, depression, and medication side effects. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Bowel and bladder functions may be impaired. Constipation Spastic bladder Small capacity for urine results in incontinence. Flaccid bladder Large capacity for urine and no sensation to urinate Spastic bladder is accompanied by urinary urgency and frequency and results in dribbling or incontinence. Generally, urinary retention occurs with flaccid bladder, but urgency and frequency may also occur with this type of lesion. Another urinary problem is a combination of the previous two problems. Urinary problems cannot be adequately diagnosed and treated unless urodynamic studies are done. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Sexual dysfunction can occur in MS. Erectile dysfunction Decreased libido Difficulty with orgasmic response Painful intercourse Decreased lubrication Diminished sensation can prevent a normal sexual response in both sexes. The emotional effects of chronic illness and the loss of self-esteem also contribute to loss of sexual response. Some women with MS who become pregnant experience remission or an improvement in their symptoms during the gestation period. The hormonal changes associated with pregnancy appear to affect the immune system. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Cognitive manifestations Difficulty with Short-term memory attention Attention Information processing Planning Visual perception Word finding General intellect, including long-term memory, conversational skills, and reading comprehension, remains unchanged and intact. In only about 5% to 10% of MS patients, the cognitive changes are so severe that they significantly impair the person's ability to carry out activities of daily living. Most of the time, cognitive difficulties occur later in the course of the disease. However, they can occur much earlier in the disease process, and occasionally they are present with the onset of MS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Emotional manifestations Anger Depression Euphoria Physical and emotional trauma, fatigue, and infection may aggravate or trigger signs and symptoms. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Case Study iStockphoto/Thinkstock What do you say to someone who has just been given a diagnosis of MS for which there is no cure? First just listen – allow them time to adjust. Remain neutral no matter how they react – it’s not about you. Help them grieve. Finally, offer information, education, and resources to help them live as optimally as possible. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Diagnostic Studies No definitive diagnostic test for MS Based primarily on history, clinical manifestations, and results of certain diagnostic tests MRI of brain and spinal cord may show the presence of plaques, inflammation, atrophy, and tissue breakdown and destruction. Because there is no definitive diagnostic test for MS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Diagnostic Studies Cerebral spinal fluid (CSF) analysis ↑ in immunoglobulin G Presence of oligoclonal banding Evoked potential responses are often delayed in persons with MS because of decreased nerve conduction from the eye and the ear to the brain. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Diagnostic Studies For a diagnosis of MS Evidence of at least 2 inflammatory demyelinating lesions in at least 2 different locations within the CNS Damage or an attack occurring at different times (usually >1 month apart) All other possible diagnoses must have been ruled out. MRI may be helpful because sclerotic plaques as small as 3 to 4 mm in diameter can be detected. Characteristic white-matter lesions scattered through the brain or spinal cord are evident on such a scan. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Collaborative Care Drug Therapy
Currently there is no cure for MS. Collaborative care is aimed at treating the disease process and providing symptomatic relief. Therapy is tailored specifically to the disease pattern and manifestations that the patient is experiencing. See Tables and for more information. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Collaborative Care Immunosuppressive Therapy
Drug therapy used to slow the progression of disease includes the use of immunosuppressants, immunomodulators, and adrenocorticotropic hormone. The initial treatment of MS is the use of immunomodulator drugs to modify the disease progression and prevent relapses. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Collaborative Care Immunomodulators
Immunomodulator drugs are used initially to modify the disease progression and prevent relapses. Interferon  (Betaseron, Extavia, Avonex, Rebif) Glatiramer acetate (Copaxone) Teriflunomide (Aubagio) Interferon β-1b (Betaseron) and glatiramer acetate (Copaxone), which are given subcutaneously every other day, and interferon β-1a (Avonex), which is given intramuscularly (IM). Another formulation of interferon β-1a is Rebif, which is administered subcutaneously 3 times weekly. Teriflunomide (Aubagio) is an immunomodulatory agent with antiinflammatory properties. The exact mechanism of action is unknown but may involve a reduction in the number of activated lymphocytes in the CNS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

For more active and aggressive forms of MS IV natalizumab (Tysabri) IV mitoxantrone (Novantrone) Dimethyl fumarate (Tecfidera) Natalizumab is a monoclonal antibody and is given monthly when patients have had an inadequate response to other drugs. Mitoxantrone, an antineoplastic medication, has serious effects, including cardiotoxicity, leukemia, and infertility. Dimethyl fumarate (Tecfidera) provides a new approach to treating MS by activating the Nrf2 pathway. This pathway provides a way for cells in the body to defend themselves against the inflammation and oxidative stress caused by MS. Dimethyl fumarate is used to treat relapsing-remitting MS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Corticosteroids (methylprednisolone, prednisone) Helpful in treating acute exacerbations by reducing edema and acute inflammation at the site of demyelination However, these drugs do not affect the ultimate outcome or degree of residual neurologic impairment from the exacerbation. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Collaborative Care Other Drug Therapy
Muscle relaxants CNS stimulants Anticholinergics Tricyclic antidepressants Selective potassium channel blocker Antiseizure drugs Muscle relaxants are used for spasticity. Amantadine (Symmetrel) and CNS stimulants (pemoline [Cylert], methylphenidate [Ritalin], and modafinil [Provigil]) are used to treat fatigue. Anticholinergics are used to treat bladder symptoms. Tricyclic antidepressants and antiseizure drugs are used for chronic pain syndromes. Dalfampridine (Ampyra) is used to improve walking speed in MS patients. It is a selective potassium channel blocker and improves nerve conduction in damaged nerve segments. It should not be used in patients with a history of seizure disorders or moderate to severe kidney disease. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Collaborative Care Other interventions may be required in the treatment of spasticity. Surgery Dorsal column electrical stimulation Intrathecal baclofen pump These therapies treat spasticity. Tremors that become unmanageable with drugs are sometimes treated by thalamotomy or deep brain stimulation. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Collaborative Care Physical therapy Relieve spasticity. Improve coordination. Train patient to substitute unaffected muscles for impaired muscles. Neurologic dysfunction sometimes improves with physical and speech therapies. Exercise improves daily functioning for patients with MS who are not experiencing an exacerbation. {See next slide for figure of water therapy.} Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Water Therapy Water therapy provides exercise and recreation for the patient with a chronic neurologic disease. Water gives buoyancy to the body and allows the patient to perform activities that would normally be impossible because it gives the patient greater control over the body. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Case Study iStockphoto/Thinkstock In general, what do you know about the drug therapies available for L.S., and what can they offer? Drug therapy may give her relief of her symptoms but will not cure the disease. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Nursing Assessment Subjective Data Health history Viral infections or vaccinations Residence in cold or temperate climates Physical and emotional stress Medications Elimination problems Weight loss, dysphagia Subjective and objective data that should be obtained from a patient with MS are presented in Table Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Nursing Assessment Health history Muscle weakness or fatigue, tingling or numbness, muscle spasms Blurred or lost vision, diplopia, vertigo, tinnitus Decreased libido, impotence Anger, depression, euphoria, isolation Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Case Study What are some of the possible problems that L.S. may develop as the disease progresses? How can you help anticipate and adjust to these problems? iStockphoto/Thinkstock Spasticity, constipation, bladder changes, fatigue, cognitive/emotional changes, and neurologic changes. Assist her through the grieving process. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Planning Maximize neuromuscular function. Maintain independence in activities of daily living for as long as possible. Manage disabling fatigue. Optimize psychosocial well-being. Adjust to the illness. ↓ factors that precipitate exacerbations Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Nursing Implementation
Help patient identify triggers and develop ways to avoid them or minimize their effects. Reassure patient during diagnostic phase. Assist patient in dealing with anxiety and grief caused by diagnosis. Exacerbations of MS are triggered by infection (especially upper respiratory and urinary tract infection), trauma, immunization, delivery after pregnancy, stress, and change in climate. Each person responds differently to these triggers. During the diagnostic phase the patient needs reassurance that, even though there is a tentative diagnosis of MS, certain diagnostic studies must be done to rule out other neurologic disorders. Assist the patient in dealing with the anxiety caused by a diagnosis of a disabling illness. The patient with recently diagnosed MS may need assistance with the grieving process. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

During acute exacerbation, prevent major complications of immobility. Focus teaching on building general resistance to illness. Avoiding fatigue, extremes of hot and cold, exposure to infection During an acute exacerbation, the patient may be immobile and confined to bed. The focus of nursing intervention at this phase is to prevent major complications of immobility, such as respiratory and urinary tract infections and pressure ulcers. This involves avoiding exposure to cold climates and to people who are sick, as well as vigorous and early treatment of infection when it does occur. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Teach patient Good balance of exercise and rest Minimize caffeine intake Nutritious, well-balanced meals Increase roughage if constipated Treatment regimen Management of medications Patients should know their treatment regimens, drug side effects, how to watch for and manage side effects, and drug interactions with over-the-counter medications. The patient should consult a health care provider before taking nonprescription drugs. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Teach Self-catheterization if necessary Adequate intake of fiber to aid in regular bowel habits Emotional adjustments Lifestyle changes Bladder control is a major problem for many patients with MS. Although anticholinergics may be beneficial for some patients to decrease spasticity, you may need to teach others self-catheterization. Bowel problems, particularly constipation, occur frequently in patients with MS. Increasing the dietary fiber intake may help some patients achieve regularity in bowel habits. The patient with MS and the caregiver need to make many emotional adjustments because of the unpredictability of the disease, the need to change lifestyles, and the challenge of avoiding or decreasing precipitating factors. The National Multiple Sclerosis Society and its local chapters can offer a variety of services to meet the needs of patients with MS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Evaluation Expected outcomes Maintain or improve muscle strength and mobility. Use assistive devices appropriately for ambulation and mobility. Maintain urinary continence. Make decisions about lifestyle modifications to manage MS. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Audience Response Question Interferon β-1b (Betaseron) has been prescribed for a patient who has been diagnosed with relapsing-remitting multiple sclerosis. Which statement, if made by the patient, indicates that additional teaching is needed? “I must rotate injection sites with each dose.” “I should report depression or suicidal thoughts.” “I will reduce my sodium intake to prevent edema.” “It is important to avoid direct sunlight and use sunscreen.” Answer: C Rationale: Interferon β-1b (Betaseron) is an immunomodulator drug (and not a corticosteroid). The drug is given subcutaneously every other day. Patient teaching should include rotate injection sites with each dose; assess for depression, suicidal ideation; wear sunscreen and protective clothing while exposed to sun; know that flu-like symptoms are common following initiation of therapy. Copyright © 2014 by Mosby, an imprint of Elsevier Inc.

Multiple Sclerosis Chapter 59

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