Unit 3

Management of Clients with Degenerative Neurological Disorders Gordons Functional Health Pattern Activity and Exercise Unit objectives : 1. Differentiate among the infectious disorders of the nervous system according to causes, manifestations, medical care, and nursing management. 2. Describe the pathophysiology, clinical manifestations, and medical and nursing management of Multiple Sclerosis, Parkinsons, Myasthenia Gravis, and Guillain-Barr syndrome.

3. Use the nursing process as a framework for care of patients with Multiple Sclerosis, Myasthenia Gravis, Parkinsons, and Guillain-Barr syndrome.

Unit 3
Do Case Studies from Critical Thinking Book Before Class!
1st CS on pg:363 Multiple Sclerosis 2nd CS on pg: 345 Parkinsons 3rd CS on pg:353 Amytrophic Lateral Sclerosis (ALS) 4th CS on pg:341 Guillain-Barre Syndrome REQUIRED READING: Smeltzer: Chapters 64 & 65

Multiple Sclerosis
Chronic, progressive degenerative disease affecting the myelin sheath of the white matter of the brain & spinal cord
Myelin sheath is highly conductive fatty material that surrounds the axon and speeds the nerve impulses; essential for normal conduction of nerve impulses

Plaques form on myelin sheath causing inflammation, edema, & demyelination, eventually scarring nerve transmission becomes erratic, and slows down Age onset: 20 -40 yrs; affects women 2:1; whites are affected more often than Hispanics, blacks or Asians. Ds more prevalent in colder climates: North America & Northern Europe.

Multiple Sclerosis
Etiology:
Exact cause unknown Theories: some evidence suggests that an infective agent causes a predisposition to MS (an immunogenetic-viral ds)
Multiple genes are involved (one found so far HLA gene complex on chromosome 6)

Precipitating factors: Infection, pregnancy, physical activity, emotional stress.

Multiple Sclerosis
Clinical Manifestations:
Hx; vague and unrelated symptoms early period of MS, varies from person-person Weakness or tingling sensation (parasthesias) of one or more extremity - weakness & fatigue are the most common symptom. Difficulty with coordination and balance that is due to cerebellar involvement spastic weakness, ataxia, tremors, sensation to temperature, foot dragging, staggering, or loss of balance Vision loss from optic neuritis - 70% experience involuntary, rhythmic movements of eyes (nystagmus) Bowel & bladder dysfunction due to spinal cord involvement (hesitancy, frequency, loss of sensation, incontinence & retention); constipation. Symptoms worsening after tasking hot bath or shower (Uhthoffs sign) Exacerbation and remissions.

Multiple Sclerosis
Diagnostic tests: no single test reliable in diagnosing MS.
CSF eval: presence of oligoclonal banding presence of IgG antibody in CSF MRI of brain & spinal cord for presence of MS plaques.

Multiple Sclerosis
Nursing Diagnosis:
Impaired physical mobility related to fatigue & weakness Activity intolerance r/t weakness, dizziness, and unsteady gait Self-esteem disturbance r/t loss of health & lifestyle changes Nursing Management 1. Promotes physical mobility activity and rest no vigorous physical exercise frequent rest periods walking and gait exercises minimize spasticity and contractures warm packs, daily muscle stretching activities: swimming, stationary bike, progressive wt bearing Minimize effects of immobility; skin integrity; cough and deep breathing exercises. 2. Prevent injury walk with feet wide apart, environment awareness and modification, gait training. Use of assistive devices walker, cane etc. 3. Promote bladder & bowel control - Urinal/bedpan readily available, po fluids intake schedule/voiding schedule, increase fiber in diet, intermittent self-catheterization 4. Improve sensory and cognitive function: Vision eye patch for diplopia; prism glasses for reading; talking books Speech slurred, low volume, problems with phonation speech therapist cognitive & emotional responses forgetfulness, easily distracted, emotionally labile, social activities; hobbies. 5. Development of coping strengths education about diseases process; stress relief; network of services social, speech, PT, psychological, homemaker/meal on wheels 6. Improve self care assistive devices, raised toilet seat, shower bench, reached tongs, decrease physical and emotional stress, decrease exposure of extreme temperatures 7. Adapting to sexual dysfunction counseling, plan sexual activity, willingness to experiment.

Multiple Sclerosis
Tx: for acute relapses
IV or oral corticosteroids
Methylprednisolone, followed by oral Prednisone taper Azathioprine (Imuran) Cyclophosphamide (Cytoxan)

Tx: treating exacerbations

Interferon B (Betaseron) used for ambulatory pts. [genetically engineered complex protein w both antiviral and immunoregulatory properties which reduce the # of exacerbations

 Symptomatic treatment: pharmacologic tx of the symptoms Bladder dysfunction: oxybutynin (ditropan), propantheline, urecholine, Constipation: psyllium hydromucilloid, bisacodyl Fatigue: amantadine, modafinil Muscle spascity: baclofen diazepam, dantrolene Tremors: propranolol, thenobarbial, clonazepam Trigeminal neuralgia: carbamazepine, phenytoin, amitriptyline Dysesthesia: TENS transcutaneous electrical nerve stimulation

Multiple Sclerosis

 Collaborative Tx:

Multiple Sclerosis

PT if pt needs instruction on assistive devices, or learn muscle tone to remain active; conduct ROM exercise For vision impairment teach to scan the environment, remove sources of injury. For urological control drink 1500 cc q day of fluids and void q 3 hrs. if urine is retained teach intermittent catherization & clean technique
Neurogenic bladder (most common in MS)
Refers to several bladder dysfunctions caused by lesions of central & peripheral nervous system: 5 types Uninhibited Sensory paralytic (detrusor muscle hyperreflexia) Motor paralytic (detrusor muscle areflexia) Autonomous reflex

Multiple Sclerosis
Tx for constipation
High-fiber diet, bulk laxatives, stool softners, fluids > 2000 cc/ day
Laxatives & enemas should be avoided lead to dependency Bowel program should be performed every other day
45 mins after largest meal (gastrocolic reflex) give suppository

Keep environment cool no hot baths, plan activity at peak energy level
Drugs: amantadine (symmetrel) modafinil (Provigil) may alleviate fatigue

Require assistance w ADLs (w/c or canes)

ADL nursing aides, assistive devices raised toilet seat, reacher tongs

Fear of loss of independence & fear of disability

Depressive episodes (short anti-depressent drug therapy)

Multiple Sclerosis
Knowledge Deficit
About MS, its unpredictable course, (remissions & exacerbations), the role of stress, National Multiple Sclerosis Society excellent resource for education & support

Discharge & Home Health Care

Medications: be sure pt understands meds dosage, route, action, & SE write them out

Bowel & bladder elimination routine Worsening of conditions telephone access & support network Prevention avoid stress, fatigue and being over headed stimulate exacerbations

Multiple Sclerosis
Evaluation: the pt with MS will:
Develop a realistic daily schedule that allows for adequate rest Achieve an activity level appropriate for the extent of disability Achieve bowel & bladder function Demonstrate self-catheterization if prescribed Verbalize and understanding of required lifestyle changes, eg: ways to manage emotional stress, maintain a nutritious diet, avoid infection, etc

Parkinsons Disease
A progressively degenerative neurological disorder affecting the brain centers (substantia nigra and basal ganglia) responsible for control & regulation of movement Occurs in 1% of pop. Over 50, affects men > women Pathophysiology: depletion of dopamine levels in the basal ganglia of the mid brain, which is where dopamine is produced & stored.
Dopamine promotes smooth, purposeful movements of motor function Dopamine depletion impairment of the extrapyramidal tracts w loss of movement coordination

Parkinsons Disease
Cause: unknown
Some heredity causes Secondary iatrogenic PD is drug or chemical related
Dopamine depleting drugs: reserpine. Phenothiazine, metoclopromide, butyrophenones (droperidol & haloperidol)

Clinical manifestations: 3 cardinal signs

Bradykinesia / akinesia slowness of movement or complete or partial loss of movement; difficulty w balance involuntary tremors course, rest tremor of the fingers & thumb ( pill-rolling movement) of one hand, occurs during rest, & intensifies w stress, fatigue, cold, disappears during sleep, the tremor can occur in tongue, lip, jaw, chin; eventually spreads to the foot on the same side

Parkinsons Disease
Progressive muscle rigidity to antagonistic muscle groups, causing resistance to both extension & flexion
Flexion contractures develop in the neck, trunk, elbows, knees & hip Face expressionless, mask-like appearance, drooling & tearing ability Propulsive gait

Speech: high-pitched monotone voice & parroting the speech of others Hypothalmic dysfunction: or perspiration, heat intolerance, seborrhea, & oil production Psychosocial : PD does not affect intellectual ability
20% of pts will end up having dementia (Alzheimers ds)

Parkinsons Disease
Diagnostic Evaluation: no specific dx tests History: progresses thru stages
Mild unilateral dysfunction Mild bilateral dysfunction, expressionless face & gait changes dysfunction w walking, initiating movements, and maintaining equilibrium Severe disability- difficulty in walking, & maintaining balance & steady propulsion, rigidity Invalid bed rest

Question
Which of the following is not a cardinal sign of Parkinsons disease? a. Tremor b. Dementia c. Bradykinesia d. Rigidity

Parkinsons Disease Drug therapy

Goal is to enhance dopamine transmission Dopaminergics
Levodopa is to provide dopamine to the basal ganglia, is a synthetic metabolic precursor to dopamine. Dopamine itself can not be used because it can not cross the blood brain barrier. Given in combination w Sinemet (carbidopa) to allow more levodopa to reach the brain and it prevents peripheral metabolism of levodopa; beneficial first few years; on & off reactions;
Sinemet
most common drug (carbidopa-levodopa) SE = Orthostatic hypotension, nausea, hallucinations, dyskinesia Nsg considerations: monitor B/P, use TED hose to venous return, monitor for urinary retention.

PD drug therapy
Dopaminergics
Symmetrel (amantadine)
Action causes release of dopamine in CNS Indications rigidity, bradykinesia SE dizziness, ataxia, insomnia, leg edema Nsg considerations monitor for postural hypotension, do not administer at bed time

PD - drug therapy
Anticholinergic to block the release of acetylcholine (balance between dopamine & acetylcholine) and to block the excitatory effects of the cholinergic system
Artane (trihexphenidyl) Cogentin (benztropine) Parsidol (ethopropazine)
Indications: tremor, rigidity, drooling SE: dry mouth, constipation, blurred vision, confusion, hallucinations Nursing Indications: usually contraindicated in acute-angle glaucoma, & tachycardia, monitor pulse & B/P during dosage adjustments, administer w meals, do not withdraw meds sudden

 Antihistamines:
Benadryl

PD drug therapy

Indications: tremor, rigidity, insomnia SE: dry mouth, lethargy, confusion Nsg considerations: use w caution in pts w seizures, hypertension, hyperthyroidism, renal ds, diabetes, administer w meals or antacids.

Dopamine agonists
Parlodel (bromocriptine)
Action: activates dopamine receptor in the CNS, helpful for tx of on-off reactions Indications: fluctuation of manifestations, dyskinesia, dystonia SE: Hallucinations, mental fogginess, orthostatic hypotension, confusion Nsg considerations: monitor B/P & mental status

 COMT inhibitors [catechol-O-methyltransferase}

Tolcapone
Action: enhance effect of dopamine Indications: adjuvant treatment SE: diarrhea, liver enzymes Nsg: monitor LE

PD drug therapy

MAO inhibators
Deprenyl (selegiline)
Action: inhibitmonoamine oxidase B, an enzyme that converts chemical byproducts in the brain into neurotoxins that prevent substantia nigra cell death Indications: adjuvant tx SE: nausea, dizziness, confusion, hallucinations, dry mouth Nsg: monitor for levodopa SE, Selegiline may effects of levodopa

Antidepressants

Parkinsonian Crisis
Sudden or inadvertent withdrawal of anti-PK drugs or emotional trauma, pts experiencing severe exacerbation of tremor, rigidity and bradykinesia, along w acute anxiety, sweating, tachycardia.
Interventions: respiratory & cardiac support, subdued lighting, mild barbiturates, anti PK drugs

PD nursing management
Improve mobility
Exercise & stretch regularly (first thing in morning)
Encourage daily ROM to avoid rigidity & contractures

Enhance walking walk erect, watch horizon, wide-based gait, heel-toe gait, long strides. Use cane or walker prevent falls

Improve hydration & nutrition

Maintain fluid intake 2 L/24 hrs Monitor weight & ability to chew & swallow
Upright position to chew & swallow, offer small freq. meals, soft foods & thick cold foods supplemental puddings Prevent aspiration think thru the steps of swallowing, keep lips closed, keep teeth together, chew, finish one bite before another

PD nursing management
Improve bowel elimination
Stool softners, mild laxatives, regular routine, fiber, raised toilet seat

Enhance self-care
Extra time needed to perform ADLs, use of side rails, overhead trapeze. Reinforce occupational & physical therapy Sleep on firm mattress, prevent neck contractures

Improve communication
Speech therapy speak slowly, use board, mechanical voice synthesizer

PD nursing management
Support coping abilities
Feel embarrassed, depressed, lonely, bored
Pd progresses, more rigidity & unresponsive to verbal stimuli, treat w dignity, do not ignore clients

Client to be active participant in EO Explore feelings Education Services: American Parkinsons Disease Foundation

Myasthenia Gravis
Autoimmune disorder affecting the myoneural junction

Antibodies directed at acetylcholine at the myoneural junction impair transmission of impulses

Manifestations
Myasthenia gravis is a motor disorder Initially, symptoms involve ocular muscles, causing conditions such as diplopia and ptosis Weakness of facial muscles, swallowing and voice impairment (dysphonia), generalized weakness

Myasthenia Gravis (cont.)

Normal ACh receptor site

ACh receptor site in myasthenia gravis

Medical Management
Pharmacologic therapy
Cholinesterase inhibitor: pyrostigmine bromide (Mestinon)
See Chart 64-4

Immunomodulating therapy

Plasmapheresis Thymectomy

Myasthenic Crisis
Result of disease exacerbation or a precipitating event, most commonly a respiratory infection Severe generalized muscle weakness with respiratory and bulbar weakness Patient may develop respiratory compromise failure

Cholinergic Crisis
Caused by overmedication with cholinesterase inhibitors Severe muscle weakness with respiratory and bulbar weakness Patent may develop respiratory compromise and failure

Management of Myasthenic Crisis

Patient instruction in signs and symptoms of myasthenic crisis and cholinergic crisis Ensuring adequate ventilation; intubation and mechanical ventilation may be needed Assessment and supportive measures include:
Ensure airway and respiratory support Take ABGs, serum electrolytes, I&O, and daily weight If patient cannot swallow, nasogastric feeding may be required Avoid sedatives and tranquilizers

MG - management
No cure Drug therapy: two groups of medications
Short-acting anticholinesterase compounds
Mestinon (pyridostigmine) Prostigmin (neostigmine)

Corticosteriods
Prednisone = assists in reducing the levels of serum Ach receptor antibodies, steroids may temporary worsen the symptoms followed by improvement

Immunosuppressive therapy: the level of circulating Ach receptor antibodies

Imuran (azaathioprine) Sandimmune (cycloporine)

Question
A patient is undergoing a Tensilon test to diagnose myasthenia gravis. Which of the following medications should be available to control the side effects of Tensilon? a. Lidocaine b. Atropine c. Mestinon d. Copaxone

MG Nursing Management
Improve resp function Increase physical mobility Improve communication Provide eye care Prevent aspiration

Nursing ProcessThe Care of the Patient With Myasthenia Gravis

Focus on patient and family teaching Provide medication teaching and management Implement energy conservation measures Implement strategies to help with ocular manifestations Prevent and/or manage complications and avoid crisis Implement measures to reduce risk of aspiration Avoid stress, infections, vigorous physical activity, some medications, and high environmental temperatures

Amyotrophic Lateral Sclerosis (ALS)

Lou Gehrigs disease
Loss of motor neurons in the anterior horn of the spinal cord and loss of motor nuclei in the brain stem cause progressive weakness and atrophy of the muscles of the extremities and trunk; weakness of the bulbar muscles impairs swallowing and talking; and respiratory function is also impaired

 Course of ds- death from pneumonia in 3-5 yrs. Diagnosis: clinical s&s; EMG changes, muscle biopsies, CSF analysis, CT, & MRI Nursing Management is focused on the tx of symptoms & rehabilitation measures
Nutrition: Airway management:

Amyotrophic Lateral Sclerosis (ALS)

Guillain-Barr Syndrome
Autoimmune disorder with acute attack of peripheral nerve myelin Rapid demyelination may produce respiratory failure and autonomic nervous system dysfunction with CV instability Most often follows a viral infection Manifestations are variable and include weakness, paralysis, paresthesias, pain, diminished or absent reflexes starting with the lower extremities and progressing upward, bulbar weakness, cranial nerve symptoms, tachycardia, bradycardia, hypertension, or hypotension

Guillain Barre Syndrome

Also known as acute demyelinating polyneuropathy or acute idiopathic polyneuritis, results in a temporary, flaccid paralysis lasting 4-8 weeks Medical emergency, 80% will recover The syndrome is preceded by a resp. or GI, viral infection 1 to 3 weeks prior to the onset of paralysis.
Autoimmune hypothesis Campylobacter jejuni is the organism most assoc.

Affects both men & women equally & of all ages.

Guillain-Barr Syndrome (cont.)

Medical management
Requires intensive care management with continuous monitoring and respiratory support Plasmapheresis and IVIG are used to reduce circulating antibodies

Recovery rates vary but most patients recover completely

Nursing ProcessAssessment of the Patient With Guillain-Barr Syndrome

Conduct ongoing assessment with emphasis on early detection of lifethreatening complications of respiratory failure, cardiac dysrhythmias, and deep vein thrombosis Monitor for changes in vital capacity and negative inspiratory force Assess VS frequently/continuously including continuous monitoring of ECG Encourage patient and family coping

Interventions
Enhance physical mobility and prevent DVT Support limbs in a functional position Perform passive ROM at least twice daily Initiate position changes at least every 2 hours Provide elastic compression hose and/or sequential compression boots Provide adequate hydration Administer IV and parenteral nutrition as prescribed Carefully assess swallowing and gag reflex and take measures to prevent aspiration Develop a plan for communication individualized to patient needs Decrease fear and anxiety Provide information and support Provide referral to support group Implement relaxation measures Maintain positive attitude and atmosphere to promote a sense of well-being Implement diversional activities

Interventions (cont.)
Develop a plan for communication individualized to patient needs Decrease fear and anxiety
Provide information and support Provide referral to support group Implement relaxation measures Maintain positive attitude and atmosphere to promote a sense of well-being Implement diversional activities