Transcript neuromuscular stressors

NEUROMUSCULAR
DYSFUNCTIONS
Joanna Shedd, MS, CNS, RN
LAUGHTER IS THE BEST MEDICINE
LAUGHTER IS THE BEST MEDICINE
CENTRAL NERVOUS SYSTEM
Central Nervous System
– Multiple Sclerosis
– Parkinson’s Disease
– Amyotrophic Lateral
Sclerosis
PERIPHERAL NERVOUS SYTEM
Peripheral Nervous
System
– Myasthenia Gravis
– Guillain-Barre Syndrome
FUNCTIONS
Motor
– Weakness
– Paralysis
– Tremors
Sensory
– Numbness
– Tingling
– Pain
FUNCTIONS
Mental processes
– Confusion
– Coma
Autonomic function
– Regulates smooth
muscle
MULTIPLE SCLEROSIS
Destruction of myelin
sheath of brain & spinal
cord
Lack of myelin causes
slowing or blockage of
conduction
MULTIPLE SCLEROSIS
Demyelinated areas are
scattered throughout
CNS
Can be both motor or
sensory
PATHOPHYSIOLOGY
Characterized by remissions and exacerbations
Disease progression differs from person to
person
Benign: Limited disability, slow progression
Exacerbating: Remitting
Chronic relapsing: Most common
Chronic progressive: No remission
STAGES
Acute
– Demyelinated areas
remyelinate
– Heal over with sclerotic or
fibrous tissue
Chronic
– Nerve fibers degenerate
– Disabilities become
permanent
ETIOLOGY
Basically unknown,
multiple theories
Autoimmune response
triggered by virus
Delayed hypersensitivity
response
Family history
Affects women more
than men
SIGNS & SYMPTOMS
E: eye, optic neuritis
A: ataxia, muscular incoordination
S: speech, slurred, slow
T: tremors, intentional tremor
E: emotional, mood swings, depression
R: rectal, bowel and/or bladder problems
EXACERBATION
Stress
Fatigue
Overheating
Weather
DIAGNOSTICS
Thorough history
and physical exam
Cerebrospinal Fluid
– Increase Tlymphocytes
– IgG (oligoclonal
banding)
DIAGNOSTICS
Magnetic Resonance
Imaging:
– visualize plaques and
lesions
DIAGNOSTICS
Evoked response tests
– Electroencephalogram
(EEG)
– Visual Evoked
Response (VER)
– Brainstem Auditory
Evoked Response
(BAER)
GOALS
Avoid or shorten
exacerbations
Maintain independence
Decrease complications
of immobility
MEDICATIONS
Interferon beta: 1B
Interferon beta: 1A
Steroids: acute
exacerbations
Immunosuppressants
Muscle relaxants
INTERVENTIONS
Plasmapheresis in
combination with
medications
Physical Therapy
Occupational Therapy
Patient education
– Prevention
– Protection
PARKINSON’S DISEASE
Imbalance of
acetylcholine and
dopamine
Acetylcholine: excitatory
Dopamine: inhibitory
PARKINSON’S DISEASE
Degeneration in
substantia nigra
Causes:
– Trauma
– Viral disease
– Aging
Degeneration
accelerated, cause not
known
Increase excitatory
SIGNS & SYMPTOMS
T: tremor, resting tremor
R: rigidity, muscle
stiffness
A: akinesia, slowness of
movement
P: postural instability,
stooped posture
SIGNS & SYMPTOMS
Mask-like facial
expression
Shuffling gait
Turns like a statue
Speech difficulties: soft,
slurred, monotonous
SIGNS & SYMPTOMS
General weakness
Autonomic problems
“Pill rolling”
Slowness of thought
Memory impairment
DIAGNOSTICS
History
Physical exam
Response to antiParkinson medications
MEDICATIONS
Levodopa/Carbidopa
(Sinemet)
Levoadpa is converted
into dopamine by DOPA
decarboxylase
Carbidopa prevents
Levodopa from
converting to dopamine
outside the brain
MEDICATIONS
Allows more dopamine to
pass blood brain barrier
Helps decrease side
effects in the periphery
Increases concentration
of dopamine in the brain
SIDE EFFECTS
Nausea/Vomiting
Sedation
Hallucinations, psychosis
Dyskinesia: involuntary
wiggly movements
Unsustained or
unpredictable response
(motor fluctuations)
SIDE EFFECTS
Postural hypotension
Anxiety
Depression
Mood swings
Cardiac arrhythmias
Athetoid movements
CAUTIONS
Relieves most signs &
symptoms
On/Off response
– sudden involuntary
inability to initiate
movement
CAUTIONS
Less effective with
prolonged use
Drug holiday
Hospitalize during drug
holiday
Adjunctive therapy with
Amantadine
MEDICATIONS
Dopamine agonists
– mimic dopamine effect
MAO-B inhibitors
– slow progression
Antiviral agent
– Amantadine
Anticholinergics
– decrease effects of
acetylcholine
SURGERY
Pallidotomy
– Cauterize global pallidus
in the basal ganglia
– Interrupts nerve
pathways
– Decreases abnormal
brain over activity
SURGERY
Adrenal medullary tissue
transplants
– Poor results
– Transplanted tissue was
necrotic
– Fetal brain cell tissue
transplants
REHABILITATION
Physical therapy
Occupational therapy
Speech therapy
Nutrition
High fowlers while eating
Consistency and
temperature of food
AMYOTROPHIC LATERAL
SCLEROSIS
Lou Gehrig’s disease
Motor neuron
dysfunction
Progressive,
degenerative
demyelinating disorder of
motor neurons in the
brain and spinal cord
Both upper and lower
AMYOTROPHIC LATERAL
SCLEROSIS
Motor disease
NO sensory loss
Mind intact
CAUSES
DNA abnormality
Viral or autoimmune
Metabolic or endocrine
abnormality
Over stimulation of
glutamate
SIGNS & SYMPTOMS
Disease of middle age
Fatigue, muscle weakness
Progresses to paralysis
Fasciculation, twitching,
spasticity
Atrophy r/t nerve cell death
Starts in hands and
spreads to arms then legs
SIGNS & SYMPTOMS
Difficulty swallowing,
speaking
Drooling due to affected
head and neck muscles
SIGNS & SYMPTOMS
Difficulty breathing
– affects diaphragm and
intercostal muscles
Death is usually from
pneumonia or respiratory
failure
DIAGNOSTICS
History
Physical exam
Rule out other diseases
EMG
– shows specific changes
– loss of nerve supply to
the muscle
INTERVENTIONS
No cure
Riluzole (Rilutek)
– May help prevent injury to
motor neuron
– Monitor liver function
tests
Supportive care
50% die within 5 years
SUPPORTIVE CARE
Keep independent as
long as possible
Prevent complications of
immobility
Comfort care
Conserve energy
SUPPORTIVE CARE
Psychological support for
patient and family
Education regarding
home care:
– Gastrostomy tube feeding
– Ventilator
– Communication devices
MEDICATIONS
Antispasmodics
– Baclofen (Lioresal)
– Zanaflex (Tizanadien)
– Spasticity interferes with
functional ability
MYASTHENIA GRAVIS
Peripheral nervous
system
Autoimmune disease
that causes a decrease
in acetylcholine
receptors
MYASTHENIA GRAVIS
Muscle receives weak
stimulation, so unable to
fully contract
Muscle fatigues quickly
After repeated
stimulation, acetylcholine
steadily decreases
Muscles do not contract
at all
SIGNS & SYMPTOMS
Classic sign: fluctuating weakness
Affects voluntary muscles
Onset insidious, progresses slowly
Remissions and exacerbations
Aggravated by infections, trauma, surgery,
stress, change in medication regime
Death usually r/t respiratory complications
SIGNS & SYMPTOMS
Ocular
– Ptosis
– Diplopia
Facial
– Flat expression
– Open mouth
Speech
– voice fades with talking
SIGNS & SYMPTOMS
Difficulty chewing or
swallowing
Risk for aspiration
Affects upper extremities
before lower
Proximal extremities
more than distal
Usually no sensory loss
DIAGNOSTICS
Tensilon test
– Short-acting
anticholinesterase drug
– Inactivates
cholinesterase
– Positive test: dramatic
improvement after IV
injection
DIAGNOSTICS
EMG
– Measures degree of
muscle response to
stimulation
Anti-ach receptor antibody
– 80% accurate
Pulmonary function test
– Assess degree of
pulmonary involvement
MEDICATIONS
Short-acting
anticholinesterase drugs
– Increase Ach at all cholinergic
receptors
– GI tract irritation
– Improves signs & symptoms,
but is not a cure
– Take on time
– If late, decrease in function
MEDICATIONS
Steroids &
Immunosuppressants
– Decrease auto-antibody
production
– Steroid precautions
SURGERY
Thymectomy
– Removal of thymus gland
– 80% with myasthenia
gravis have enlarged
thymus
– Improves signs &
symptoms
– In 40%, complete
remission occurred
INTERVENTIONS
Pace activities
– Plan rest periods, assist
as needed
– Assess respiratory status
COMPLICATIONS
Myasthenic crisis: exacerbation or progression of
disease
Cholinergic crisis: Overmedication with
anticholinesterase drugs (less common)
Signs & symptoms: generalized weakness, respiratory
failure, requiring ventilator support
Diagnosis: Tensilon test
– Will improve myasthenic crisis
– Will worsen cholinergic crisis
GUILLAIN-BARRE SYNDROME
Inflammatory disease
process
Demyelination of
peripheral nerves
Autoimmune response
stimulated by viral illness
GUILLAIN-BARRE SYNDROME
Primarily motor affected
Only one of 5 diseases
processes that is NOT
chronic
Acute demyelinating
polyneuropathy
DIAGNOSTIC
No influenza vaccine if
history of GB
No definitive test
CSF protein
– initially decrease followed
by rapid, large increase
EMG
– slowed conduction
velocity
SIGNS & SYMPTOMS
Acute Stage
Paresthesia
Pain
Stiff
Weakness
Rapidly progressive
paralysis
Ascending paralysis
SIGNS & SYMPTOMS
Acute Stage
Can progress to total
paralysis
May need ventilator
support
Cranial nerves affected
– Cannot chew, swallow,
talk, close eyes
SIGNS & SYMPTOMS
Acute Stage
Autonomic dysfunction
– Tachycarida or
bradycardia
– Cardiac arrthymias
– Fluctuating BP
– Urinary retention
No change in LOC or
thought processes
SIGNS & SYMPTOMS
Plateau/Stabilizing stage
– Several days to weeks
Recovery stage
– 6 months to 2 years
– Often resolves
completely
– Residual weakness
– Complications of
immobility
INTERVENTIONS
Supportive care
Careful monitoring during
acute stage
May need ventilator,
temporary pacemaker,
tube feedings
Prevent infection and
complications of immobility
No medications specific for
disease process
INTERVENTIONS
Exercise limited in acute
stage
Rehab stage
– gentle, passive ROM
Psychological support
– Frightened, anxious
– Paralyzed, but mentally
alert
Plasmapheresis
A client with numbness and tingling is
tentatively diagnosed with Guillain-Barre. An
important assessment for this client will be?
A.
B.
C.
D.
Discomfort and pain on movement
Breathing and tidal volume
Metabolic and nutritional state
Urine output and fluid intake
What scale is used to measure
neurological function?
A.
B.
C.
D.
Bathroom scale
Braden scale
Glascow coma scale
Pain scale
Which of the following would be included
when providing discharge teaching to a
client with multiple sclerosis?
A.
B.
C.
D.
“Avoid taking daytime naps”
“Avoid taking hot baths and showers.”
“Limit your fruit and vegetable intake.”
“Restrict fluids to 1 liter a day.”
A client is undergoing testing to confirm a
diagnosis of Myasthenia gravis. What drug
will most likely be given to test this
disorder?
A.
B.
C.
D.
Atenolol (Tenormin)
Dopamine
Kefzol (Ancef)
Tensilon (Edrophonium)
A client has Parkinson’s disease is on a
thickened liquid diet and needs assistance
with feedings. What position should the client
be to prevent complications?
A. High Fowler’s
B. HOB up 20 degrees
C. Flat on back
D. Trendelenberg
What intervention would inappropriate
when caring for client with ALS?
A. Active ROM exercises
B. Airway maintained, especially on a vent
C. Give Interferon IM daily using Z-track
method
D. Talk with client as you are doing
procedures