Chapter #44

Restless Legs Syndrome

(possibly genetic) dysfunction of Dopamine exacerbated by caffeine, alcohol, smoking, weight, or deficiencies such as iron or Mg++ although also commonly associated with Peripheral/Central nerve damage in the legs & spinal cord, also known as "Night Walkers"

Stages of Guillain-Barré Syndrome

1. Acute 2. Plateau 3. Recovery

Myasthenic Crisis

characterized by an increased pulse, increased respiration, rise in BP, incontinence, decreased UOP, absence of a cough + swallow reflex, and improvement of symptoms with a Tensilon Test interventions = medications will be restarted gradually as well as at lower doses (withhold cholinesterase-inhibiting drugs)

Three days later, the patient is stable and the plan is to discharge her to home, where her mother will provide care for her. Which preparations are essential before discharge? Select all that apply. A. Arrange for special equipment in the home. B. Arrange for home modifications, such as a ramp. C. Teach the mother how to provide physical therapy. D. Teach family members how to use special equipment. E. Arrange for the patient to go to a rehabilitation facility before going home.

A. Arrange for special equipment in the home. B. Arrange for home modifications, such as a ramp. D. Teach family members how to use special equipment. special equipment + home modifications need to be completed, and the family needs to know how to use the special equipment while in-home visits can be arranged for PT & OT

The home health nurse is visiting a client with myasthenia gravis and is discussing methods to minimize the risk of aspiration during meals related to decreased muscle strength. Which suggestions should the nurse give to the client? Select all that apply. A. Chew food thoroughly. B. Cut food into very small pieces. C. Sit straight up in the chair while eating. D. Lift the head while swallowing liquids. E. Swallow when the chin is tipped slightly downward to the chest.

A. Chew food thoroughly. B. Cut food into very small pieces. C. Sit straight up in the chair while eating. E. Swallow when the chin is tipped slightly downward to the chest.

What other medical conditions would the nurse expect to see in a patient with restless leg syndrome? A. Diabetes and kidney failure B. Myasthenia gravis and decreased vision C. Trigeminal neuralgia and facial paralysis D. Peripheral vascular disease and multiple sclerosis

A. Diabetes and kidney failure

During morning care, the patient is able to brush her teeth, wash her face, and brush her hair. She becomes fatigued after performing these actions. What is the appropriate nursing action? A. Provide assistance in completing the patient's morning care. B. Let her rest for 15 minutes and then continue self morning care. C. Document that the patient refuses morning care because of fatigue. D. Encourage her to continue with her own morning care to increase her strength.

A. Provide assistance in completing the patient's morning care. muscle weakness increases when the patient is fatigued which then limits her mobility and ability to participate in activities, provide assistance as necessary to prevent the patient from becoming fatigued

Later that day, the patient asks the nurse about activities she can do after her recovery. Which activity will the nurse discourage? A. Sunbathing B. Wearing sunglasses C. Walking in her neighborhood D. Taking medication as directed

A. Sunbathing patients should avoid getting overheated

MG Diagnostics

monitor excess TH, serum protein electrophoresis evaluates autoimmune disorders that can be associated, ACh Receptor Antibodies/Muscle-Specific Kinase (NOT definitive), x-rays of the thymus, and repetitive nerve stimulation

Tensilon Crisis

caused by testing to result in cardiac dysthymia or even though rare, cardiac arrest

Complications of Plasmapheresis: Fluid Shift or Depletion

monitor fluid status and vital signs during treatment and at least twice int he first hour after treatment

What is the ANTIDOTE to a Tensilon Crisis?

Atropine Sulfate is the antidote as it can dry secretions (consider mucous plugs)

The nurse is caring for a patient with a diagnosis of Bell's palsy. The nurse understands that for a patient with Bell's palsy the symptoms are the most severe during which time period after beginning? A. 12 hours after onset B. 48 hours after onset C. 96 hours after onset D. 1 to 2 weeks after onset

B. 48 hours after onset symptoms usually begin suddenly and reach their peak within 48 hours

During the evening shift, the nurse notes that the patient is having difficulty mobilizing secretions. Which interventions should be implemented for this problem? Select all that apply. A. Plasmapheresis B. Chest physiotherapy C .Coughing and deep breathing D. Oxygen at 2 L per nasal cannula E. Oropharyngeal suctioning as needed

B. Chest physiotherapy C. Coughing and deep breathing E. Oropharyngeal suctioning as needed the patient may need oxygen, but SaO2 & PaO2 should be assessed before this is applied

Facial Paralysis/Bell's Palsy

CN V, VII, & VIII involvement seen 48 hours to five days with a "sagging" appearance requiring eye protection such as manually closing the eyelid at determined times of the day, artificial tear use, and eye patches at night patient education = eat on the unaffected side, most recover within a few weeks to months, and some patients can experience residual weakness

Edrophonium/Tensilon Testing

Cholinesterase Inhibitor (Anticholinesterase) which slows the heart action, lowers blood pressure, and increases secretion.; used to distinguish between a Cholinergic v. Myasthenia Crisis as they appear the same at first (Prostigmin is also used)

What health history question will give the nurse the most information when evaluating a patient for Guillain-Barré syndrome (GBS)? A. "Did you get a flu vaccine in the past year?" B. "Has anyone else in your family ever had GBS?" C. "Have you ever been exposed to Epstein-Barr virus?" D. "Have you had a respiratory virus in the past 2 weeks?"

D. "Have you had a respiratory virus in the past 2 weeks?" GBS usually occurs a few days or weeks after the patient has had symptoms of a respiratory or GI viral infection (or surgery); vaccinations may increase the risk and this disorder can develop over the course of hours, days, or up to 3-4 weeks

Complications of Plasmapheresis: Citrate-Induced Hypocalcemia

monitor patient electrolytes before and after therapy while communicating any abnormal results to the PHCP, anticipate Ca++ Replacement Therapy

Which two learned about conditions have a similar intervention of eye patch use for diplopia?

MS & MG

Tensilon Testing (Cholinergic)

NO muscle tone improvement as there is already available ACh for muscle contraction (ex. twitching can occur around the eyes + face) crisis occurring from OVERMEDICATION

Guillain-Barré Syndrome Diagnostics (Lumbar Puncture)

a patient with GBS will have CSF with an increased protein level due to the release of inflammatory plasma proteins, myelin breakdown, and nerve root damage = DISTINGUISHING SIGN

Myasthenia Gravis

an acquired autoimmune disease characterized by muscle weakness and is related to the abnormal growth of the Thymus Gland; this condition can occur in many forms from mild disturbances of cranial + peripheral motor neurons to a rapidly developing, generalized weakness that can result in death from respiratory failure (distortion of ACh receptors = NO muscle contraction) = ONE OF THE MOST TREATABLE NEURO DISEASES (medication may be an immunosuppressant)

Respiratory Assessment for the Patient with Guillain-Barré

assessment shied be performed frequently (ex. 1-2 hours), intubation may be needed including supplemental O2, consider chest physiotherapy, hypoxia can lead to dysrhythmias (CARDIAC MONITOR), manage hypotension with a fluid bolus + medications (ex. Beta-Blockers OR Dopamine), and Atropine can be administered for bradycardia

Cholinergic Crisis

characterized by life-threatening bradycardia & bronchospasm, flaccid paralysis, hypersecretions (ex. saliva, tears, sweat), N/V/D, abdominal cramping, blurred vision (miosis), exacerbation of muscle weakness, and symptoms worsen with a Tensilon Test interventions = maintain respiratory function, withhold any Anticholinesterase medications if on a ventilator which would increase secretions, administer ATROPINE

Trigeminal Neuralgia

characterized by unilateral, sudden intense facial spasms from a familial connection initiated from light touch, change in facial expression, or chewing PAIN MANAGEMENT as a priority

Complications of Plasmapheresis: Access Site

complications at the plasmapheresis access site may include bleeding, phlebitis, infection, or clotting INTERVENTIONS = anchor the tubing securely during treatment, minimize patient agitation if present, assess the site after treatment cessation, and assess the site at regular intervals for appearance, dressing, thrill palpation, bruit auscultation; immediately report the loss of a thrill or bruit, uncontrolled or large-volume bleeding, presence of redness (especially along the venous pathway), drainage, or swelling

Facial Paralysis/Bell's Palsy: Drug Therapy

corticosteroids and antivirals (ex. Acyclovir)

CASE STUDY: A 20-year-old female college student presents at the university health clinic with manifestations that include fatigue, muscle aches, numbness and tingling, double vision, and difficulty swallowing. The patient is transferred to an acute care facility with a diagnosis of r/o myasthenia gravis (MG). Which diagnostic tests would the nurse expect to be performed?

diagnostic tests include Thyroid Function, Serum Protein Electrophoresis (immunologic disorders), ACh Receptor Antibodies, X-Ray & CT of the Thymus, Tensilon Testing, and Electromyography (EMG)

Guillain-Barré Syndrome Diagnostics

diagnostics may include a lumbar puncture, CBC to monitor for leukocytosis (present early then returns to normal), electrophysiological studies to demonstrate demyelinating neuropathy, electromyographic studies to assess peripheral nerve function, CT/MRI for "ruling out," and ABGs as the patient is experiencing weakness

Restless Legs Syndrome: Drug Therapy (ex. Mirapex)

dopamine agonist which may have a sleepiness side effect, also including AEDs or Melatonin for those that experience insomnia

Complications of Plasmapheresis: Sensitivity Reaction (ex. anaphylaxis)

follow policy for safe, effective administration of blood products such as FFP including the potential with incorrect crossmatch or administration

Complications of Plasmapheresis: Infection Risk

for patients with immunoglobulin depletion, make sure to assess + document vital signs (especially temperature 3x daily) and report any signs or symptoms of infection, fever, or abnormal vital signs

Complications of Plasmapheresis: Urticarial Reactions

for these reactions from proteins in replacement fluids, obtain an order and administer prophylactic Diphenhydramine/Benadryl or a corticosteroid when urticaria occurred with a previous exchange

Trigeminal Neuralgia Management

included AEDs (ex. Tegretol + Neurontin), Lyrica for nerve pain relief, muscle relaxants (ex. Baclofen), Percutaneous Sterotactic Rhizotomy, or a MIS Microvascular Decompression (usually requires a craniotomy)

Guillain-Barré Syndrome: Autonomic Manifestations

includes a labile blood pressure, cardiac dysrhythmias, and tachycardia

Signs & Symptoms of Guillain-Barré

includes a sudden onset of muscle weakness & pain, cranial nerve involvement, autonomic dysfunction, loss of reflexes in the arms/legs, paralysis, numbness, diplopia, and poorly controlled BP (hypotension) NOT affected = cerebral function or pupillary signs

Guillain-Barré Syndrome: Motor Manifestations Affecting Mobility

includes ascending symmetric muscle weakness (flaccid paralysis without atrophy), decreased or absent DTRs, incontinence, ataxia, and respiratory compromise = dyspnea, diminished breath sounds, decreased tidal volume, reduced SpO2 or vital capacity, and respiratory failure

Restless Legs Syndrome Signs & Symptoms

includes burning, "crawling" sensations, and paresthesias resulting in an irresistible urge to move relief = walking, stretching, moderate exercise, warm math

Post-Op Assessment

includes monitoring for headache, cranial dysfunction, bleeding, and EOM

Stages of Guillain-Barré Syndrome: Acute

lasts 1-4 weeks

Stages of Guillain-Barré Syndrome: Recovery

lasts over 4-6 months or up to two years and is associated with re-myelination + axonal regeneration, BUT some patients may still experience deficit (NOT recovering completely)

Stages of Guillain-Barré Syndrome: Plateau

lasts several days up to two weeks

Guillain-Barré Syndrome: Cranial Nerve Manifestations

manifestations include facial weakness,s dysphagia, diplopia, and difficulty speaking

Guillain-Barré Syndrome: Sensory Perception Manifestations

manifestations of sensory perception includes paresthesias and painful cramping

Overall Nursing Interventions for the Patient with Myasthenia Gravis

maximize patient independence, plan activities following medication administration, promote self-care with rest, inter professional management, speak slowly with the "repeat back" method, use "YES/NO questions," promote adequate fluid intake, calorie counts, I&O, daily weights Pre-Albumin/Albumin levels (may require tube feeding), diplopia patch, and artificial tears/lubricant gel as the patient might NOT be able to close their eyes

Exacerbation of Myasthenia Gravis & Patient Education

may include infection, stress, surgery, physical exercise, sedatives, enemas, strong cathartics, overheating, crowds, overeating, changes in sleep habits, and emotional extremes education = assertive devices, medication compliance, crises signs & symptoms, Medical Alert Bracelet

Initial Phase of Guillain-Barré Syndrome

monitor the patient closely for signs of respiratory distress (ex. dyspnea, air hunger, adventitious breath sounds, decreased SpO2, cyanosis), respiratory rate, rhythm, and depth every 1-2 hours; collaborate with the RT to check vital capacity every 2-4 hours, auscultate the lungs every four hours, monitor the ability to cough + swallow, and assess cognitive status (especially in older adults) as a decline in mental status may indicate hypoxia

Myasthenia Gravis: Motor Manifestations Affecting Mobility

motor manifestations include progressive proximal muscle weakness which worsens with repetitive use (improves with rest), poor posture, ocular palsies, ptosis, diplopia, respiratory compromise, incontinence, and fatigue

Improving Nutrition in Patients with Myasthenia Gravis

nutrition improvement involves assessment of the patient's gag reflex, assessment of the ability to chew + swallow, providing frequent oral hygiene, working with the interdisciplinary team to provide meals the patient can eat plus enjoy, cut food into small bites or request soft/edentulous diet, encourage the patient to eat slowly, observe the patient for choking/aspiration or nasal regurgitation, provide high-calorie snacks or supplements (ex. pudding), elevate HOB during meals as well as 30-60 minutes after, consider thickening liquids to avoid choking/aspiration, monitor caloric + food intake, weigh the patient daily, monitor Serum Pre-Albumin levels, and administer Anticholinesterase Drugs as prescribed (usually 45-60 minutes BEFORE meals)

Atropine & Risk for Aspiration

observe the patient carefully as secretions can be thickened by the drug, causing more difficulty with airway clearance and possibly the development of mucus plugs; unless complications (ex. pneumonia or aspiration) develop, the patient in a Cholinergic Crisis improves rapidly AND DO NOT give to patients on mechanical ventilation

Cholinesterase Inhibitor Drugs (ex. Pyridostigmine)

patients should be taught to eat their meals 45 minutes to an hour AFTER taking this medication in order to avoid aspiration; Mg++, Morphine, Neomycin, hypnotics, or sedatives should also be avoided because they may increase the patient's weakness

Pyridostigmine

prescribed to enhance neuromuscular impulse transmission by preventing the decrease of ACh, expect day-by-day variations of dosing depending on patient symptoms

Guillain-Barré + Cranial Nerve VII

screening involves facial expressions (ex. smiling or frowning) along with CN V, XI, XI, VII coordinated with swallowing

Myasthenia Gravis: Sensory Perception Manifestations

sensory manifestations include muscle achiness, paresthesias, and a decreased sense of smell + taste

GBS Drug Therapy (Immunoglobulin) Side Effects

side effects may include chills, mild fever, headache, myalgia, anaphylaxis, and acute renal failure so transfusions are administered SLOWLY as these side effects are extreme

Physical Assessment of the Patient with Myasthenia Gravis

signs & symptoms include an insidious/slow onset of fatigue, weakness worsening with repetitive use, mild disturbances to death with respiratory failure, more common in men, eye muscle involvement (ex. ptosis + diplopia), dysphagia, weak voice, issues with facial expression, chewing/speech difficulty, inadequate nutrition, and incontinence EOM = assess CN VI

Myasthenic Crisis v. Cholinergic Crisis

similar features of both types of crises includes apprehension, restlessness, dyspnea/respiratory failure, dysphagia, and generalized weakness

Demyelinating Guillain-Barré Syndrome (Ascending Paralysis)

symptoms of temporary paralysis that typically begin in the legs and spread to the arms along with the upper body as it affects the peripheral nerves; paralysis can increase in intensity until the muscles are unable to be used at all whereas the patient becomes completely immobile --> mechanical ventilation (reverse healing)

Percutaneous Stereotactic Rhizotomy

teach patients to avoid rubbing the eye on the affected side because the protective mechanism of pain will NO longer warn of injury, instructing them to inspect the eye daily for redness or irritation (as well as report any change or blurred vision); also stress the importance of regular dental examinations because the absence of pain may NOT warn the patient of potential problems = nerve sparing with NO facial droop

Complications of Plasmapheresis: Catheter-Related Trauma

teach the patient rationale for and how to monitor access site due to the risk for trauma to skin + blood vessels from large-bare needles for access

Drug Therapy Education for the Patient with Myasthenia Gravis

teach the patient to keep prescribed drugs plus a glass of water at the bedside if they're weak in the morning, wear a watch with an alar function (or beeper) to remind them to take their medications, post the drug schedule so others know it, plan strenuous activities during drug peak, keep a secure supply of drugs in the car or at work, and check with the PHCP before using any OTCs

Thymectomy

these patients are typically intubated in the ICU with post-op chest tubes and should be monitored for signs & symptoms of a Pneumothorax + Hemothorax

Risk Factors of Guillain-Barré Syndrome

this syndrome commonly affects men more, especially in those age 55+ and related to a history of acute illness such as bacterial infections, influenza, Epstein-Barre, Zika, trauma, surgery, or immunization

Plasmapharesis

treatment method which removes circulating antibodies assumed to cause disease, patient will then replace own plasma or be transfused with albumin

Complications of Plasmapheresis

treatment-related complications can include citrate-induced hypocalcemia, urticarial reactions from proteins in replacement fluids, infection risk due to immunoglobulin depletion, fluid shift or depletion, sensitivity reaction with FFP, catheter-related trauma (access site issues), or clotting

Guillain-Barré Syndrome

uncommon acute inflammatory disorder affecting axons and/or the myelin sheath of the Peripheral Nervous System to cause impaired mobility and sensory perception as a result of altered immunity = progression motor weakness + sensory abnormalities

Tensilon Testing (Myasthenic)

within 30-60 seconds after Tensilon injection, most MYASTHENIC patients show a (temporary) muscle tone improvement which lasts 4-5 minutes since ACh is now available in the patient body for muscle contraction crisis occurring from under medication

Factors Precipitating/Worsening Myasthenia Gravis

worsening factors include RA, alcohol, hormonal changes, stress, infection, seasonal temperature changes, heat, surgery, enemas, and various drugs = cathartics/laxatives, antidysrhythmics, Beta-Blockers, ahminoglycosides or antibiotics, anti-rheumatic drugs, antispasmodics, antihistamines, opioids, Phenytoin/Dilantin, antidepressants (ex. Tricyclics)