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Degenerative Neurologic Disorders Week 1 Co pyright © 2023 Els evier, Inc. 1 Objectives Interpret subjective and objective data from a neurologic assessment. Discuss the etiology, clinical manifestations, and diagnostic studies related to Guillain-Barré and de...

Degenerative Neurologic Disorders Week 1 Co pyright © 2023 Els evier, Inc. 1 Objectives Interpret subjective and objective data from a neurologic assessment. Discuss the etiology, clinical manifestations, and diagnostic studies related to Guillain-Barré and degenerative neurologic disorders Parkinson’s disease, multiple sclerosis, amyotrophic lateral sclerosis, and myasthenia gravis. Assess clients for complications related to neurologic diagnostic tests and treatments. Cop yri gh t © 2023 Els evier , Inc. 2 Objectives Assess the impact of degenerative neurologic disorders on physical and psychological well-being. Implement and evaluate a collaborative plan of care, including drug therapy, for clients with Guillain-Barré and degenerative neurologic disorders. Develop a teaching plan to educate clients, families, and the community about the care of clients with degenerative neurologic disorders, including community resources. Cop yri gh t © 2023 Els evier , Inc. 3 Nervous System Cop yri gh t © 2023 Els evier , Inc. 4  Anatomy Review Neuron structure Divisions of the nervous system o Central (CNS) o Peripheral (PNS) o Autonomic (ANS) Functions Cop yri gh t © 2023 Els evier , Inc. The human nervous system is responsible for the control and integration of the body’s many activities. The nervous system can be divided into the central nervous system and the peripheral nervous system. The central nervous system (CNS) consists of the brain, spinal cord, and cranial nerves I and II. The peripheral nervous system (PNS) consists of cranial nerves III to XII, spinal nerves, and the peripheral components of the autonomic nervous system (ANS). Neurons are the primary functional unit of the nervous system. Although neurons come in many shapes and sizes, they share three characteristics: (1) excitability, or the ability to generate a nerve impulse; (2) conductivity, or the ability to transmit an impulse; and (3) influence, or the ability to influence other neurons, muscle cells, or glandular cells by transmitting nerve impulses to them. 5  Gerontologic Considerations CNS o Loss of neurons o Enlargement of ventricles o Decreased brain weight, cerebral blood flow, CSF PNS o Decrease in nerve conduction o Decrease in coordinated neuromuscular activity Decrease in memory, sensory, neuromuscular functions Cop yri gh t © 2023 Els evier , Inc. In the PNS, degenerative changes in myelin cause a decrease in nerve conduction. Coordinated neuromuscular activity, such as the maintenance of blood pressure in response to changing from a lying to a standing position, is altered with aging. As a result, older adults are more likely to experience orthostatic hypotension. Similarly, coordination of neuromuscular activity to maintain body temperature is also less efficient with aging. Older adults are less able to adapt to extremes in environmental temperature and are more vulnerable to both hypothermia and hyperthermia. Additional relevant changes associated with aging include decreases in memory, vision, hearing, taste, smell, vibration and position sense, muscle strength, and reaction time. Sensory changes, including decreases in taste and smell perception, may result in decreased dietary intake in the older adult. Reduced hearing and vision can result in perceptual confusion. Problems with balance and coordination can put the older adult at risk for falls and subsequent fractures. 6 Multiple Sclerosis Cop yri gh t © 2023 Els evier , Inc. 7  Multiple Sclerosis (MS) Chronic, progressive, immune- mediated disease of the CNS o Segmental demyelination of nerve fibers Contributing factors o Age: young adults (20-50 yrs) o Gender: women o Geography o Genetic predisposition Cop yri gh t © 2023 Els evier , Inc. Characterized by remissions and exacerbations Initially, attacks cause damage to myelin sheaths of neurons in brain and spinal cord. Nerve fiber is not affected. Transmission of nerve impulses still occurs, though transmission is slowed. Patient may complain of noticeable impairment of function. At this point, the myelin can regenerate, and the symptoms will disappear. Therefore, the patient experiences a remission. 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. Research suggests that it is unlikely MS is related to a single cause. Onset between 20-50 years of age Women 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 8  degrees of latitude), such as those found in the northern United States, Canada, and Europe, as compared with tropical regions 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. IMAGE A, Normal nerve cell with myelin sheath. B, Normal axon. C, Myelin breakdown. D, Myelin totally disrupted; axon not functioning. 8  Clinical Manifestations Insidious and gradual onset Motor problems o Spasticity and/or weakness o Scanning speech, dysarthria o Intentional tremors Sensory problems o Visual disturbances o Decreased hearing, tinnitus o Paresthesia o Chronic neuropathic pain Cerebellar problems o Nystagmus o Dysphagia o Ataxia Cop yri gh t © 2023 Els evier , Inc. Vague symptoms occur intermittently over months and years. The intermittent nature of the symptoms often dissuade the patient from seeking medical attention. Disease may not be diagnosed until long after onset of the first symptom. Neuropathic pain 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. 9  Clinical Manifestations Cognitive manifestations o Difficulty with short-term memory, attention, information processing, planning, word finding Bowel and bladder problems o Constipation o Spastic or flaccid bladder Sexual dysfunction Emotional lability, depression Fatigue Cop yri gh t © 2023 Els evier , Inc. 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. 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. Sexual dysfunction Erectile dysfunction Decreased libido Difficulty with orgasmic response Painful intercourse Decreased lubrication Severe fatigue is present in many MS patients and causes significant disability for some patients. 10 Diagnostic Studies No definitive diagnostic test for MS MRI of brain may show sclerotic plaques in CNS MS diagnosis o Evidence of at least 2 inflammatory demyelinating lesions in at least 2 different CNS locations o Damage or an attack occurring at different times (usually > 1 month apart) o All other possible diagnoses ruled out Cop yri gh t © 2023 Els evier , Inc. 11  Collaborative Care Currently, there is no cure for MS. Drug Therapy / Examples o Immunosuppressants - Corticosteroids (prednisone, methylprednisolone, dexamethasone) o Immunomodulators - Interferon beta-1a and 1b - Glatiramer acetate o Antispasmodics - Baclofen - Dantrolene o Antiseizure - Carbamazepine - Gabapentin Cop yri gh t © 2023 Els evier , Inc. Drug therapy to relieve symptoms and delay disease progression. Treatment goals: 1. Relieve symptoms. 2. Decrease severity and frequency of relapse. Drug therapy does not affect ultimate outcome. Corticosteroids are given during exacerbation. Are helpful in treating acute exacerbations by reducing edema and acute inflammation at the site of demyelination. Monitor for infection, hypervolemia, hypernatremia, hypokalemia, hyperglycemia, GI bleed, and personality changes. Immunomodulators may act more selectively by targeting only specific portions of the immune system and therefore pose a lower risk of complications related to immune dysfunction. (ATI and Harding et al. gave different frequencies. Use ATI for testing.) Interferon β-1a Avonex IM once a week or Interferon β-1a Rebif subcutaneously 3x/week. 12  Interferon β-1b Betaseron subcutaneous either daily or 3x/week with 48 hours between injection. Antispasmodics for for spasticity and tremors. Intrathecal baclofen pump Observe for increased weakness. Monitor for liver damage with dantrolene. Antiseizure for paresthesia. 12  Collaborative Care Drug Therapy / Examples o Anticholinergics - Oxybutynin - Tolterodine - Propantheline o Laxatives Stool softener - Docusate sodium Bulk-forming - Psyllium o Antidepressants - Amitriptyline - Sertraline - Fluoxetine Cop yri gh t © 2023 Els evier , Inc. Anticholinergics are used to treat bladder symptoms. Psyllium for constipation. It comes as a powder, granules, capsule, liquid, and wafer to take by mouth. It usually is taken one to three times daily. Take psyllium exactly as directed. Mix powder and granules with 8 ounces of a pleasant tasting liquid, such as fruit juice, right before use. Chew wafers thoroughly. Drink at least 8 ounces of liquid when you take it to prevent side effects. Antidepressants and antiseizure medications are used for depression and chronic pain syndrome. 13  Collaborative Care Drug Therapy / Examples o Phosphodiesterase inhibitor - Sildenafil o Adamantanes and CNS - Amantadine stimulants - Methylphenidate - Modafinil o K channel blocker - Dalfampridine + Cop yri gh t © 2023 Els evier , Inc. Sildenafil treats erectile dysfunction by increasing blood flow to the penis during sexual stimulation. This increased blood flow can cause an erection. Amantadine and methylphenidate (Ritalin) are used to treat fatigue. 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. 14  Collaborative Care Thalamotomy or deep brain stimulation for tremors unmanageable with drugs Nursing interventions o Promote independence, rest and activity, and well- balanced meals. ▪ Encourage fiber. o Prevent injury and avoid stressors. o Teach self-injection and self-catheterization. o Refer to OT, PT, speech pathologist, and community resources. Cop yri gh t © 2023 Els evier , Inc. Promote independence and maintain an active, normal lifestyle as possible. Exercise improves daily functioning for patients with MS who are not experiencing an exacerbation. 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. Encourage fiber to prevent constipation. Institute fall prevention measures. Avoid stressors because they can precipitate an exacerbation. Fatigue and infection (especially upper respiratory and urinary tract) are stressors. Trauma, immunization, delivery after pregnancy, and change in climate are other stressors. Each person responds differently to these triggers. Teach self-catheterization. Bladder control is a major problem for many patients with MS. 15 Consult the health care team. Physical therapy relieves spasticity, improves coordination, and trains patient to substitute unaffected muscles for impaired muscles. 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. 15 Parkinson’s Disease Cop yri gh t © 2023 Els evier , Inc. 16  Parkinson’s Disease (PD) Chronic, progressive neurologic disorder Degeneration of dopamine-producing neurons in the basal ganglia and depletion of dopamine Diagnosis increases with age More common in men Exact cause unknown Cop yri gh t © 2023 Els evier , Inc. Disease of basal ganglia Characterized by Slowing down in the initiation and execution of movement Increased muscle tone Tremor at rest Gait disturbance Affects about 2% of people over age 60. As many as 15% of those diagnosed with PD are younger than age 50. Exact cause of PD is unknown. Possibly a result of a complex interplay between environmental factors and the person’s genetic makeup Family history Exposure to toxins may trigger disease. Drug-induced Parkinsonism – e.g., metoclopramide, lithium, haloperidol, and chlorpromazine therapy. Parkinsonism can also be seen following the use of illicit drugs, including amphetamine and methamphetamine. Other causes of Parkinsonism include hydrocephalus, multiple sclerosis, 17  encephalitis, infections, stroke, tumor, Huntington’s disease, and trauma. Lewy bodies, unusual clumps of protein, are found in the brains of patients with PD. It is not known what causes these bodies to form, but their presence indicates abnormal functioning of the brain. Lewy body dementia is discussed in Chapter 60. 17  Clinical Manifestations Insidious and gradual onset Bradykinesia with rigidity Resting tremor Postural and gait disturbances Expressionless, fixed gaze; masklike Drooling and slurred speech Depression Cop yri gh t © 2023 Els evier , Inc. Classic triad of PD 1. Tremor is often the first sign. It is so minimal initially that only the patient may notice it. Hand tremor is most common. It can involve the diaphragm, tongue, lips, and jaw but rarely causes shaking of the head. It is more prominent at rest and aggravated by emotional stress or increased concentration. It is described as pill rolling because thumb and forefinger appear to move in rotary fashion. This tremor can affect handwriting, causing it to trail off, particularly toward the ends of words. 2. Rigidity - Rigidity is the increased resistance to passive motion when limbs go through their range of motion. It is caused by sustained muscle contraction and consequently elicits the following: Complaint of soreness; feeling tired and achy; and pain in the head, upper body, spine, or legs. Another consequence of rigidity is slowness of movement because it inhibits the alternating of contraction and relaxation in opposing muscle groups. Rigidity is typified by a jerky quality when the joint is moved. 18  Rigidity is similar to intermittent catches in the movement of a cogwheel. 3. Bradykinesia (slowing down in initiation and execution of movement) is evident in the loss of automatic movements, such as blinking of the eyelids, swinging of the arms while walking, swallowing of saliva, and self-expression with facial and hand movements. This accounts for the stooped posture, masked face (deadpan expression), drooling of saliva, and shuffling gait (festination) that are characteristic of a person with this disease. Beginning stages may involve only mild tremor, slight limp, or decreased arm swing. Later stages may have shuffling, propulsive gait with arms flexed, and loss of postural reflexes. Some have slight speech changes. 18  Clinical Manifestations Nonmotor symptoms o Depression o Anxiety o Pain o Constipation o Fatigue o Sleep problems o Short-term memory loss and impairment - Dementia in 70% of the clients Complications o Dysphagia o General debilitation o Orthostatic hypotension Cop yri gh t © 2023 Els evier , Inc. Sleep problems Difficulty staying asleep Restless sleep Nightmares Drowsiness during the day Dementia is associated with an increase in mortality. Dysphagia may result in malnutrition and aspiration. General debilitation may lead to pneumonia, UTIs, and skin breakdown. Orthostatic hypotension may occur and could result in falls and injuries. 19 Diagnostic Studies No specific tests for PD Diagnosis based on history and clinical features o At least two of the three characteristics of the classic triad (tremor, rigidity, bradykinesia) Cop yri gh t © 2023 Els evier , Inc. 20  Collaborative Care Currently, there is no cure for PD. Drug Therapy / Examples o Dopaminergic agent - Levodopa/carbidopa o Dopamine agonist - Bromocriptine - Apomorphine o MAO-B inhibitors - Rasagiline - Selegiline o COMT inhibitors - Entacapone - Tolcapone Cop yri gh t © 2023 Els evier , Inc. Collaborative management is aimed at relieving symptoms. Antiparkinsonian drugs either Enhance or release supply of DA (dopaminergic). Antagonize or block the effects of overactive cholinergic neurons in the striatum (anticholinergic). Use of one drug is preferred due to to fewer side effects and dosages are easier to adjust. Combination therapy is often required as disease progresses. Excessive dopaminergic drugs can lead to paradoxical intoxication (aggravation instead of relief of symptoms). Levodopa/carbidopa The net result of the combination of levodopa and carbidopa is that more levodopa reaches the brain, and therefore less drug is needed. It is very effective for the management of akinetic symptoms. Tremor and rigidity may also respond to this drug. Effects may be delayed for several weeks to months. Levodopa has many side effects and drug interactions. Prolonged use can often result in dyskinesias and “off/on” periods when the medication will unpredictably 21  start or stop working. Teach patient/caregiver to report: uncontrolled movement of face, eyelid, mouth, tongue, arms, hands, legs mental changes palpitations severe N/V difficulty urinating Effectiveness of levodopa/carbidopa could wear off after a few years of therapy. Therefore, some initiate therapy with a DA receptor agonist instead (e.g., bromocriptine). Anticholinergic drugs decrease the activity of Ach and provide balance between cholinergic and dopaminergic actions. Bromocriptine Patient may become dizzy or faint due to orthostatic hypotension, especially after first dose. Notify physician immediately if severe H/A develops that does not let up or continues to get worse. Apomorphine subcutaneous injection to treat hypomobility. Within 3-5 years of treatment, patients experience episodes of hypomobility. Hypomobility manifests in inability to rise from a chair, to speak, or to walk. Episodes can occur toward the end of a dosing interval with standard medications (so-called end-of-dose wearing off) or at unpredictable times (spontaneous “on/off”). Apomorphine must not be taken with the antiemetics in the serotonin (5- HT3) receptor antagonist class (e.g., ondansetron [Zofran]), because the combination of apomorphine and these drugs can lead to very low blood pressure and loss of consciousness. Monoamine oxidase type B (MAO-B) inhibitors, selegiline and rasagiline, may be combined with Sinemet. Increase dopamine levels. Reduce wearing-off phenomenon when administered concurrently with levodopa. Note that severe interactions can occur when these meds are administered with sympathomimetics, meperidine, and fluoxetine. Avoid foods high in tyramine which can cause hypertensive crisis. Catechol O-methyltransferase (COMT) inhibitors decrease the breakdown of levodopa. Monitor for dyskinesia/hyperkinesia when used with levodopa. 21  Assess for diarrhea. Note that dark urine is a normal finding. 21  Collaborative Care Drug Therapy / Examples o Anticholinergic - Benztropine o Antihistamine - Diphenhydramine o Antiviral - Amantadine o Antidepressant - Amitriptyline o Anticholinesterase inhibitor - Donepezil Cop yri gh t © 2023 Els evier , Inc. Anticholinergics decrease the activity of Ach. Antihistamines with anticholinergic or β-adrenergic blockers to manage tremors Amantadine – exact mechanism of action is unknown, but the drug promotes the effect of DA. Donepezil (Aricept) is used to treat mild to moderate Parkinson’s dementia. 22  Collaborative Care Deep brain stimulation Nursing interventions o Promote physical exercise and well-balanced diet. o Encourage independence. o Prevent complications. o Assist client and caregiver with lifestyle adjustments. o Provide referrals. Cop yri gh t © 2023 Els evier , Inc. DBS has been shown to improve motor function and reduce dyskinesia and medication usage. Device is programmed to deliver specific current to targeted brain location. IMAGE A deep brain stimulator can be used to treat the tremors and uncontrolled movements in Parkinson’s disease. Electrodes are surgically placed in the brain and connected to a neurostimulator (pacemaker device) in the chest. Focus of teaching and nursing care: Maximize neurologic function and maintain good health. Maintain independence in ADLs for as long as possible. Avoid complications such as contractures and falls. Optimize psychosocial wellbeing. Limit the consequences from decreased mobility. Specific exercises to strengthen muscles involved with speaking and swallowing A physical therapist may be consulted to design a personal exercise program aimed at strengthening and stretching specific muscles. 23  Assistive devices to support patient. Promote well-balanced diet. Malnutrition and constipation can be serious consequences. Patients with dysphagia and bradykinesia need food that is easily chewed and swallowed. Small meals to avoid fatigue. Ample time for eating to avoid frustration. Adequate roughage and fruit to avoid constipation. Levodopa can be impaired by protein and vitamin B6 ingestion. Some patients are advised to limit their protein intake to the evening meal to decrease this problem and to consult with their health care provider regarding possible supplementation of vitamin B6 in their multivitamins and fortified cereals. Encourage independence. Simplify clothing – e.g., slip on shoes, velco hook-and-loop fasteners or zippers instead of buttons and hooks. Schedule med so that peak action occurs at mealtime. Avoid complications such as contractures and falls. Teach diaphgramatic and abdominal exercises to avoid respiratory complications. Teach specific exercises to strengthen muscles involved with speaking and swallowing. Teach strategies to improve bowel and bladder function. 23  Case Study: Client with Parkinson’s Disease and Hip Fracture Cop yri gh t © 2023 Els evier , Inc. Patient with Parkinson’s disease and hip fracture https://coursewareobjects.elsevier.com/objects/elr/Lewis/medsurg9e/casestudies/m ain.php?ch=59a 24 Amyotrophic Lateral Sclerosis Cop yri gh t © 2023 Els evier , Inc. 25  Amyotrophic Lateral Sclerosis (ALS) Also known as Lou Gehrig’s disease Unknown etiology Upper and lower motor neurons affected o Axonal degeneration, demyelination, and glial proliferation and scarring resulting in progressive weakness, muscle wasting, spasticity, and paralysis Onset between 40-70 years More common in men than women Cop yri gh t © 2023 Els evier , Inc. Beginning in one area of the body, motor weakness and deterioration spread until the entire body is involved including the ability to talk, swallow and breathe. As a result of loss of lower motor neurons (LMNs) found in the spinal cord and brainstem, the muscles to which they connect weaken, atrophy and die. Loss or death of upper motor neurons found in the brain breaks their connections with LMNs and spasticity occurs in the muscles. Death typically occurs due to respiratory failure. Patient remains cognitively intact while wasting away. 26  Clinical Manifestations Typical symptoms o Dysarthria o Dysphagia o Muscle weakness Fasciculations, cramping Overactive deep tendon reflex Fatigue Depression, anxiety Drooling, difficulty clearing airway, eventual respiratory compromise Cop yri gh t © 2023 Els evier , Inc. Fasciculations (twitching) Complications R/T musculoskeletal system: Paralysis, loss of verbal communication, aspiration R/T respiratory system: Pneumonia, eventual respiratory failure R/T nutrition: Malnutrition R/T emotional effects: Depression 27  Diagnostic Studies No specific diagnostic test o Diagnosis via clinical findings and ruling out other causes Electromyography (EMG) Muscle biopsy Pulmonary function test (PFT) Cop yri gh t © 2023 Els evier , Inc. EMG demonstrates fibrillations and fasciculations of the muscles. Muscle biopsy may be performed to determine cause of muscle disease other than ALS. PFT shows decreased vital capacity (< 2L). 28  Collaborative Care Currently, there is no cure for ALS. Drug therapy o Rilutek to slow disease progression ▪ Take on an empty stomach. ▪ Monitor liver enzymes. o Other medications to provide symptom relief Cop yri gh t © 2023 Els evier , Inc. Rilutek slows progression of ALS. It decreases the amount of glutamate (an excitatory neurotransmitter) in the brain. Other medications Baclofen, dantrolene, diazepam for spasticity Anticholinergic for excessive drooling Analgesic for pain Laxative for constipation Antidepressant for depression 29  Collaborative Care Nursing interventions o Ventilatory support o Nutritional support o Prevention of complications o Pain management o Psychosocial support and community referrals Cop yri gh t © 2023 Els evier , Inc. Supportive care Identify signs of respiratory insufficiency. Decrease aspiration risk. Support nutritional needs. Provide pain relief. Support cognitive and emotional functions. Facilitate communication/alternate communication methods. Provide diversional activities. Provide patient/caregiver teaching. Client/caregiver teaching: Suctioning Preventing complications R/T immobility Establishing a bladder and bowel routine Reporting signs of infection Much consideration is given to psychosocial concerns. Discuss advance directive. Hospice referral may be recommended for palliative care. 30 Myasthenia Gravis Cop yri gh t © 2023 Els evier , Inc. 31  Myasthenia Gravis (MG) Autoimmune disease o Antibodies damage acetylcholine (Ach) receptor sites leading to impaired transmission at the myoneural junction Thymic tumor in 15% of patients Equal incidence in men and women Peak onset between 50-70 years Cop yri gh t © 2023 Els evier , Inc. MG may take many forms – from mild disturbances of the ocular muscles to a rapidly developing generalized weakness that may lead to death from respiratory failure. 32 Clinical Manifestations Muscle weakness o Increased with activity and improved with rest o Ocular palsy, ptosis, diplopia o Dysphonia o Dysphagia o Loss of bowel and bladder control o Fatigue o Muscle wasting o Progressive deterioration, particularly the respiratory system Cop yri gh t © 2023 Els evier , Inc. 33  Types of Crisis Myasthenic Crisis Cholinergic Crisis Undermedication Overmedication Respiratory distress Respiratory distress Hypertension Hypotension Ptosis, diplopia Increased GI motility Dysarthria, dysphagia Hypersecretion Symptoms improve Symptoms worsen or do during Tensilon test not improve during Tensilon test Cop yri gh t © 2023 Els evier , Inc. Exacerbation can cause myasthenic crisis Factors contributing to exacerbations Infection Pregnancy Stress, emotional distress Fatigue Increases in body temperature Inconsistency with medication administration Lifestyle adaptations – e.g., avoiding crowds, heat (sauna, hot tubs, sunbathing), emotional extremes, factors in exacerbation (infection, stress, hard physical exercise, sedatives) 34  Diagnostic Studies Edrophonium chloride (Tensilon) o Antidote: atropine Serum Ach receptor antibodies Electromyography (EMG) MRI of the thymus gland Cop yri gh t © 2023 Els evier , Inc. Immediate improvement of symptoms that last 5 min after IV injection of Tensilon is a positive test and diagnostic of MG. Tensilon testing may also be sued to determine whether increasing weakness in previously diagnosed MG patient is due to cholinergic crisis (too much cholinesterase inhibitor drugs) or a mysthenic crisis (too little cholinestrate inhibitor drugs). In cholinergic crisis, muscle tone does not improve after giving Tensilon. Instead, weakness may increase and fasciculations (muscle twitching) may be seen around the eyes and face. Bradycardia is an adverse effect. AChR antibodies are elevated in patients with MG. Electromyography may show decreased response to repeated stimulation of hand muscles indicative of muscle fatigue. 35  Collaborative Care Drug Therapy / Examples Anticholinesterase - Pyridostigmine - Atropine Immunosuppressants - Corticosteroid - Azathioprine Caution with drugs that increase weakness Other Therapy Thymectomy Intravenous immunoglobulin (IVIG) Plasmapheresis Cop yri gh t © 2023 Els evier , Inc. Pyridostigmine is first-line therapy. Be sure to give on time to maintain blood levels and, thus, improve muscle strength. Give 45-60 min before a meal to avoid aspiration, especially if patient’s muscles of facial expression, chewing, and speech are affected. Antibiotics, such as neomycin and certain tetracyclines, impair transmitter release and increase myasthenic symptoms. Many drugs are contraindicated or must be used with caution in patients with MG. For example, anesthetics, antidysrhythmics, antipsychotics, barbiturates, sedatives, opioids, muscle relaxants, tranquilizers because they may increase patient’s weakness. Thymectomy is usually performed early in the disease. Those who have surgery within 2 yrs of the onset of myasthenic symptoms show the most improvement. Pyridostigmine may be given with a small sip of water o keep the patient stable during and after surgery. If steroids have been used, they are given before surgery and tapered during post-op period. 36 IV IgG’s exact action is unknown but is probably related to decrease in antibody production. Infuse IVIG slowly when started and monitor for S/E. Minor side effects: Chills, mild fever, H/A, myalgia Major complications: Anaphylaxis, aseptic meningitis, acute renal failure Plasmapheresis may be used before and after surgery to decrease circulating AChR antibodies more quickly. It is also indicated for patients in myasthenic crisis. It can yield short-term improvement in symptoms. Post-procedure care: Observe for hypovolemia. Observe access site for bleeding and signs of infection. Monitor electrolytes. Hypocalcemia (due to anticoagulant citrate dextrose binds with calcium): Circumoral and distal extremity paresthesias, Chvostek’s and Trousseau’s signs → IV calcium gluconate Hypokalemia: Cardiac dysrhythmias and leg cramps 36  Collaborative Care Nursing interventions o Maintain patent airway. o Offer small, frequent, high-calorie meals. o Provide eye care. o Plan activities early in the day. o Provide emotional support. o Refer to speech and physical therapy and community resources. Cop yri gh t © 2023 Els evier , Inc. Prevent aspiration. Because of respiratory muscle involvement, many of these patients have an increased risk for lung infections. Keep suction and manual ventilation equipment at the bedside. Give med on time. Offer meal during the peak time for medications. Head flexed forward. Foods with thickened consistency. Instill artificial tears and/or tape eye shut at intervals as prescribed. Balance energy conservation and mobility. Teach about factors that contribute to exacerbation and actions to take if an exacerbation occurs. Weakness of speech and facial muscles often results in dysarthric (slurred) and nasal speech. It may be difficult for maysthenic patients to make their speech understood by others. In collaboration with speech-language pathologist, determine patient’s ability to communicate. Instruct the patient to speak slowly 37 Guillain-Barré Syndrome Cop yri gh t © 2023 Els evier , Inc. 38  Guillain-Barré Syndrome (GBS) Acute, rapidly progressing form of polyneuritis Ascending, symmetric paralysis that usually affects the cranial nerves and the PNS Equal incidence in men and women Higher incidence in Euro-Americans Most cases are preceded by an infection (respiratory or gastrointestinal) Cop yri gh t © 2023 Els evier , Inc. Campylobacter jejuni gastroenteritis 39  Clinical Manifestations Ascending weakness progressing to paralysis and leading to acute respiratory failure Hyporeflexia Loss of bowel and bladder control Sensory - Paresthesia and pain Cranial nerve - Diplopia, dysarthria, dysphagia Autonomic - Bradycardia, labile BP - Dysrhythmia Cop yri gh t © 2023 Els evier , Inc. Recovery takes several months to 2 years 40  Diagnostic Studies Clinical signs Cerebrospinal fluid study EMG and nerve conduction velocity test Evoked potentials (EPs) MRI brain to rule out multiple sclerosis Cop yri gh t © 2023 Els evier , Inc. CSF analysis Elevated proteins due to release of plasma proteins from inflammation, degeneration and damage to nerve roots). EMG and nerve conduction studies show reduced nerve conduction velocity. EPSs demonstrate demyelinating neuropathy. MRI brain to rule out multiple sclerosis. 41  Collaborative Care IVIG Plasmapheresis Nursing interventions o Ventilatory support o Nutritional support o Prevention of complications of immobility o Pain management o Psychosocial support and community resources Cop yri gh t © 2023 Els evier , Inc. Prevent aspiration and provide parenteral supplementation as needed. Complications of immobility include atelectasis, pneumonia, DVT, UTI, pressure injury. 42  Clinical Application Cop yri gh t © 2023 Els evier , Inc. Joyce M. Black 43 Clinical Application Student groups (2 groups) 1. Multiple sclerosis -Create a care plan for the NANDAs listed. Include at least 4 interventions and a rationale for each intervention. Impaired urinary elimination Constipation Fatigue Impaired physical mobility Cop yri gh t © 2023 Els evier , Inc. 44  Clinical Application Student groups (3 groups) 2. Parkinson’s disease -Create a client/caregiver education guide with the indicated minimum teaching points for the following: Facilitating communication (10) Preventing aspiration (5) Avoiding development of contractures (5) Promoting safety (10) Cop yri gh t © 2023 Els evier , Inc. Numbers in parenthesis indicate minimum number of teaching points. 45 Clinical Application Student groups (2 groups) 3. Guidelines for meeting the needs of the family of the client with degenerative neurologic disorder -Provide the indicated minimum interventions for the following: Identification of client’s new health problems (3) Coping positively with stress, including dealing with role change (10) Decisional conflict related to making health care decisions for loved one (3) Cop yri gh t © 2023 Els evier , Inc. 46  Questions? Cop yri gh t © 2023 Els evier , Inc. Joyce M. Black 47  References Assessment Technologies Institute. (2019). Content mastery series review module: RN adult medical surgical nursing (11th ed.). https://student.atitesting.com/OpenPage Assessment Technologies Institute. (2019). Content mastery series review module: RN pharmacology for nursing (8th ed.) https://student.atitesting.com/OpenPage Harding, M. M., Kwong, J., Roberts, D., Hagler, D., & Reinisch, C. (2020). Lewis’ medical-surgical nursing: Assessment and management of clinical problems (11th ed.). Elsevier. Cop yri gh t © 2023 Els evier , Inc. 48