NURSING CARE OF CLIENTS WITH NEUROLOGIC DISORDERS

OUTLINE

Autoimmune Disorders

Multiple Sclerosis (MS) Myasthenia Gravis (MG) Guillen-Barr syndrome (GBS)

Degenerative disorders
Parkinsons Disease Huntingtons Disease Amyotrophic Lateral Sclerosis (ALS)

MULTIPLE SCLEROSIS

MULTIPLE SCLEROSIS
Chronic demyelinating disease of the CNS associated with abnormal immune response to environmental factor

MULTIPLE SCLEROSIS
Periods

of exacerbations and remissions Progression of disease with increasing loss of function Incidence is highest in young adults (20 40); onset between 20 50 Affects females more than males More common in temperate climates Occurs mainly in Caucasians

MANIFESTATIONS
Fatigue Optic

nerve involvement: blurred vision, haziness nystagmus, dysarthria,cognitive dysfunctions, vertigo, deafness Weakness, numbness in leg(s), spastic paresis, bladder and bowel dysfunction ataxia Spasticity Blindness

STRESS AGGRAVATES SYMPTOMS.

COLLABORATIVE CARE

Focus is on retaining optimum functioning and limiting disability

DIAGNOSTIC TESTS
Neurological exam, careful history Lumbar puncture with CSF analysis: increased number of T lymphocytes; elevated level of immunoglobulin G (IgG) Cerebral, spinal optic nerve MRI: shows multifocal lesions Evoked response testing of visual, auditory, somatosensory impulses show delayed conduction CT scan shows density of white matter or plaque formation

NO CURE EXISTS FOR MS

MEDICATIONS

Biologic response modifiers

Interferon beta-1a Interferon beta-1b Glatiramer acetate

Glucocorticosteroids Immunosuppressants

azathioprine (Imuran) cyclophosphamide (Cytoxan) methotrexate diazepam

Muscle relaxants to treat muscle spasms

Medications to deal with bladder problems: anticholinergics or cholinergics depending on problem experienced by client

NURSING DIAGNOSES
Self

care deficit Impaired physical mobility Risk for injury Impaired urinary and bowel elimination Impaired verbal communication Risk for aspiration Disturbed thought processes Ineffective individual coping Potential for sexual dysfunction

NURSING CARE
Monitor

motor movements for interference with ADLs Encourage activity balanced with rest periods Assess cognitive function Explain:

Bladder training Positioning Avoid temperature extremes Medication compliance Avoid STRESS.

HEALTH PROMOTION
Client needs to develop strategies to deal with fatigue, exacerbations Prevention of respiratory and urinary tract infections

HOME CARE
Education Referral to support group and resources Referral to home health agencies when condition requires

MYASTHENIA GRAVIS (MG)

MYASTHENIA GRAVIS (MG)

Chronic autoimmune neuromuscular disorder affecting the neuromuscular joint

MYASTHENIA GRAVIS (MG)

characterized by fatigue and severe weakness of skeletal muscles Occurs with remissions and exacerbations Occurs more frequently in females, with onset between ages 20 30

MANIFESTATIONS
Seen in the muscles that are affected: Ptosis (drooping of eyelids), diplopia (double vision) Weakness in mouth muscles resulting in dysarthria and dysplagia Weak voice, smile appears as snarl Head juts forward Muscles are weak but DTRs are normal Weakness and fatigue exacerbated by stress, fever, overexertion, exposure to heat; improved with rest

MG is purely a MOTOR disorder with no effect on sensation or coordination

COMPLICATIONS
Pneumonia Myasthenic Crisis Sudden exacerbation of motor weakness putting client at risk for respiratory failure and aspiration Manifestations: tachycardia, tachypnea, respiratory distress, dysphasia

COMPLICATIONS
Cholinergic Crisis Occurs with overdosage of medications (anticholinesterase drugs) used to treat MG Develops GI symptoms, severe muscle weakness, vertigo and respiratory distress Both crises often require ventilation assistance

DIAGNOSTIC TESTS
Physical examination and history Tensilon Test: edrophonium chloride (Tensilon) administered and client with myasthenia will show significant improvement lasting 5 minutes EMG: reduced action potential Antiacetylcholine receptor antibody serum levels: increased in 80% MG clients; used to follow course of treatment Serum assay of circulating acetylcholine receptor antibodies: if increased, is diagnostic of MG

MEDICATIONS

Anticholinesterase medications

Pyridostigmine bromide (Mestinon)

Immunsuppression medications including glucocorticoids

Cyclosporineor azathioprine (Imuran)

SURGERY
Thymectomy is recommended in clients <60 Remission occurs in 40 % of clients, but may take several years to occur

PLASMAPHERESIS
Used to remove antibodies Often done before planned surgery, or when respiratory involvement has occurred

NURSING CARE
Teaching interventions to deal with fatigue Importance of following medication therapy

NURSING DIAGNOSES
Ineffective Airway Clearance Impaired Swallowing: plan to take medication to assist with chewing activity

HOME CARE
Avoid fatigue and stress Plan for future with treatment options Keep medications available Carry medical identification Referral to support group, community resources

GUILLAIN-BARR SYNDROME (GBS)

GUILLAIN-BARR SYNDROME (GBS)

Acute autoimmune inflammatory demyelinating disorder of peripheral nervous system characterized by acute onset of ascending motor paralysis

GUILLAIN-BARR SYNDROME (GBS)

Cause is unknown but precipitating events include GI or respiratory infection, surgery, or viral immunizations 80 90% of clients have spontaneous recovery with little or no disabilities 4 6% mortality rate, and up to 10% have permanent disabling weakness 20 % require mechanical ventilation due to respiratory involvement

MANIFESTATIONS

Most clients have symmetric weakness beginning in lower extremities Ascends body to include upper extremities, torso, and cranial nerves Sensory involvement causes severe pain, paresthesia and numbness Paralysis of intercostals and diaphragmatic muscle Autonomic nervous system involvement: blood pressure fluctuations, cardiac dysrhythmias, paralytic ileus, urinary retention Weakness usually plateaus or starts to improve in the fourth week with slow return of muscle strength

DIAGNOSTIC TESTS
diagnosis made thorough history and clinical examination; there is no specific test CSF analysis: increased protein EMG: decrease nerve conduction Pulmonary function test reflect degree of respiratory involvement

MEDICATIONS
supportive and prophylactic care Antibiotics Morphine for pain control Anticoagulation to prevent thromboembolic complications

MEDICAL MANAGEMENT
Tracheostomy Plasmapheresis Enteral feeding IVIG

NURSING DIAGNOSES
Ineffective breathing pattern Impaired bed and physical mobility Imbalanced nutrition Acute Pain Risk for Impaired Skin Integrity Impaired Communication Fear

NURSING CARE
Maintain respiratory function Enhancing physical mobility Providing adequate nutrition Improving communication Decreasing fear and anxiety

HOME CARE
Clients will usually require hospitalization, rehabilitation, and eventually discharge to home Client and family will need support; support groups

DEGENERATIVE DISORDERS

PARKINSONS DISEASE

PARKINSONS DISEASE
Associated with decreased levels of dopamine resulting from destruction of pigmented neuronal cells in the substantia nigra in the basal ganglia.

PARKINSONS DISEASE
characterized by tremor at rest, muscle rigidity and akinesia (poor movement); cause unknown Affects older adults mostly, mean age 60 with males more often than females Parkinson-like syndrome can occur with some medications, encephalitis, toxins; these are usually reversible

MANIFESTATIONS
Tremor at rest with pill rolling motion of thumb and fingers Worsens with stress and anxiety Progressive impairment affecting ability to write and eat Rigidity Involuntary contraction of skeletal muscles Cogwheel rigidity: jerky motion

MANIFESTATIONS
Akinesia Slowed or delayed movement that affects chewing, speaking, eating May freeze: loss of voluntary movement Bradykinesia: slowed movement Posture instability Involuntary flexion of head and shoulders, stooped leaning forward position Equilibrium problems causing falls, and short, accelerated steps Shuffling gait

MANIFESTATIONS
Autonomic nervous system Constipation and urinary hesitation or frequency Orthostatic hypotension, dizziness with position change Eczema, seborrhea Depression and dementia; confusion, disorientation, memory loss, slowed thinking Inability to change position while sleeping, sleep disturbance Mask-like face Dysphonia

COMPLICATIONS
Impaired

communication

Falls
Infection

related to immobility and pneumonia Malnutrition related to dysphagia Skin breakdown Depression and isolation

PROGNOSIS
Slow progressive degeneration Eventual debilitation

DIAGNOSTIC TESTS
No specific test for disease Drug screens to determine medications or toxins causing parkinsonism EEG: slowed and disorganized pattern

MEDICATIONS
Antiparkinsonian-

Levodopa (Larodopa) antiviral therapy- amantadine (Symmetrel) anticholinergics- benztropine mesylate (Cogentin) Bromocriptine (Parlodel) pergolide (Permax) inhibit dopamine breakdown MAOI- selegiline (Eldepryl) Antihistamine- diphenhydramine hydrochloride (Benadryl) Medications may lose their efficacy; response to drugs fluctuates: on-off effect

TREATMENTS
Deep brain stimulation Stereotactic procedures Pallidotomy: destruction of involved tissue thalamotomy: destroys specific tissue involved in tremor

NURSING DIAGNOSES
Impaired Physical Mobility Impaired Verbal Communication Impaired Nutrition: Less than body requirements Self care deficit Constipation Disturbed Sleep Patterns Ineffective coping

NURSING CARE
Improve mobility Enhance self care activities Improving bowel elimination Improving nutrition Enhancing swallowing Improving communication supporting coping abilities

HOME CARE
Medication education Adaptation of home environment Gait training and exercises Nutritional teaching

HUNTINGTONS DISEASE (CHOREA)

HUNTINGTONS DISEASE (CHOREA)

Progressive, degenerative inherited neurologic disease characterized by increasing dementia and chorea

HUNTINGTONS DISEASE (CHOREA)

Cause unknown Autosomal dominant genetic disorder No cure Usually asymptomatic until age of 30 40 a significant reduction (volume and activity) of acetylcholine

MANIFESTATIONS
Abnormal

movement and progressive dementia Early signs are personality change with severe depression, memory loss; mood swings, signs of dementia Increasing restlessness, worsened by environmental stimuli and emotional stress; arms and face and entire body develops choreiform movements, lurching gait; difficulty swallowing, chewing, speaking Slow progressive debilitation and total dependence Death usually results from aspiration pneumonia or another infectious process

DIAGNOSTIC TESTS
Genetic testing of blood CT scan shows cerebral atrophy

MEDICATIONS
Antipsychotic (phenothiazines and butyrophenones) to block dopamine receptors Antidepressants

NURSING DIAGNOSES
Risk for injury Risk for Aspiration Imbalanced Nutrition: Less than body requirements Impaired Skin Integrity Impaired Verbal Communication Disturbed thought processes

NURSING CARE
Very challenging: physiological, psychosocial and ethical problems Genetic counseling

HOME CARE

Referral to agencies to assist client and family, support group and organization

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

LOU GEHRIGS DISEASE

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Disease of unknown cause in which there is a loss of motor neurons in the anterior horns of the spinal cord and the motor nuclei in the lower brain stem.

AMYOTROPHIC LATERAL SCLEROSIS (ALS)

Progressive, degenerative neurologic disease characterized by weakness and wasting of muscles without sensory or cognitive changes Several types of disease including a familial type onset is usually between age of 40 60 higher incidence in males at earlier ages but equally post menopause Physiologic problems involve swallowing, managing secretions, communication, respiratory muscle dysfunction Death usually occurs in 2 5 years due to respiratory failure

MANIFESTATIONS

Initial: spastic, weak muscles with increased DTRs; muscle flaccidity, paresis, paralysis, atrophy; clients note muscle weakness and fasciculations; muscles weaken, atrophy; client complains of progressive fatigue; usually involves hands, shoulders, upper arms, and then legs Atrophy of tongue and facial muscles result in dysphagia and dysarthria; emotional lability and loss of control occur 50% of clients die within 2 5 years of diagnosis, often from respiratory failure or aspiration pneumonia

DIAGNOSTIC TEST
Testing

rules out other conditions that may mimic early ALS such as hyperthyroidism, compression of spinal cord, infections, neoplasms EMG to differentiate neuropathy from myopathy Muscle biopsy shows atrophy and loss of muscle fiber Serum creatine kinase if elevated (nonspecific) Pulmonary function tests: to determine degree of respiratory involvement

MEDICATIONS
Rilutek

(Riluzole) antiglutamate

Prescribed

to slow muscle degeneration Requires monitoring of liver function, blood count, chemistries, alkaline phosphatase

NURSING DIAGNOSES
Risk for Disuse Syndrome Ineffective Breathing Pattern: may require mechanical ventilation and tracheostomy

NURSING CARE
Help client and family deal with current health problems Plan for future needs including inability to communicate

HOME CARE

Education regarding disease, community resources for health care assistance and dealing with disabilities

CREUTZFELDT-JAKOB
DISEASE

(CJD, spongiform encephalopathy)

CREUTZFELDT-JAKOB DISEASE

Rapid progressive degenerative neurologic disease causing brain degeneration without inflammation

DESCRIPTION
Transmissible

and progressively fatal Caused by prion protein: transmission of prion is through direct contamination with infected neural tissue Variant form of CJD is mad cow disease: believed transmitted by consumption of beef contaminated with bovine form of disease Pathophysiology: spongiform degeneration of gray matter of brain

No definitive treatment. Outcome is fatal.

MANIFESTATIONS
Onset: memory changes, exaggerated startle reflex, sleep disturbances Rapid deterioration in motor, sensory, language function Confusion progresses to dementia Terminal states: clients are comatose with decorticate and decerebrate posturing

DIAGNOSTIC TESTS
Clinical pictures, suggestive changes on EEG and CT scan Similar to Alzheimers in early stages Final diagnosis made on postmortem exam

NURSING CARE
Use of standard precautions with blood and body fluids Support and assistance to client and family

TRIGEMINAL
NEURALGIA

(tic douloureux)

DESCRIPTION
Chronic disease of trigeminal nerve (cranial nerve V) causing severe facial pain The maxillary and mandibular divisions of nerve are effected Occurs more often in middle and older adults, females more than males Cause is unknown

MANIFESTATIONS
Severe

facial pain occurring for brief seconds to minutes hundreds of times a day, several times a year Usually occurs unilaterally in area of mouth and rises toward ear and eye Wincing or grimacing in response to the pain Trigger areas on the face may initiate the pain Sensory contact or eating, swallowing, talking may set off the pain Often there is spontaneous remission after years, and then condition recurs with dull ache in between pain episodes

DIAGNOSIS

by physical assessment

MEDICATIONS
Anticonvulsants carbamazepine (Tegretol) phenytoin (Dilantin) gabapentin (Neurotin)

SURGERY
Intractable pain may be treated by severing the nerve root: rhizotomy Client may have lost facial sensation and have loss of corneal reflex

NURSING CARE
Teaching client self-management of pain Maintaining nutrition Preventing injury

BELLS PALSY

DESCRIPTION
Disorder of seventh cranial nerve and causes unilateral facial paralysis Occurs between age of 20 60 equally in males and females Cause unknown, but thought to be related to herpes virus

MANIFESTATIONS
Numbness, stiffness noticed first Later face appears asymmetric: side of face droops; unable to close eye, wrinkle forehead or pucker lips on one side Lower facial muscles are pulled to one side; appears as if a stroke

PROGNOSIS
Majority of person recover fully in few weeks to months Some persons have residual paralysis

DIAGNOSIS

based on physical examination

COLLABORATIVE CARE
Corticosteroids are prescribed in some cases but use has been questioned Treatment is supportive

NURSING CARE
Teaching client self-care: prevent injury and maintain nutrition Use of artificial tears, wearing eye patch or taping eye shut at night; wearing sunglasses Soft diet that can be chewed easily, small frequent meals