Myasthenia Gravis

 Disease of the neuromuscular junction

characterized by fluctuating weakness of
certain skeletal muscle groups.
Myasthenia Gravis(MG)
 Acetycholine (ACh) is an important
neurotransmitter that stimulates muscle
tissue to contract.
 MG is an autoimmune disease in which
antibodies are formed against ACh and a
reduction in ACh receptor sites at the
neuromuscular junction.

Pathophysiology
 Loss of muscle strength.
 There is no single cause identified, however,
thymic tumors and viral infections have been
found in a certain number of patients.
Clinical manifestations
 Primary s/s= easy fatigability of skeletal
muscle during activity.
 Muscles involved: eyes and eyelids, chewing,
swallowing, speaking, and breathing.
 Fluctuating weakness: usually strong in the
a.m., progressively weaker with activity.
Clinical Manifestations
 90% of patients have eye involvement
 Facial mobility may be impaired
 Muscles of limb and trunk less often affected.
 No sensory or reflex loss; muscle atrophy is
rare.
Clinical manifestations
 Variable course
 May be precipitated by emotional stress,
pregnancy, menses, secondary illness,
trauma, temperature extremes, hypokalemia,
ingestion of drugs with neuromuscular
blocking agents, surgery.
Complications
 Aspiration, respiratory insufficiency, and
respiratory infection
 Acute exacerbation called myasthenic crisis.
 The opposite of this is a cholinergic crisis and
results from overdose of cholinergic drugs.
Diagnostic studies
 Assessment:
 Have pt look up for 2-3 minutes; if MG, patient will have
increased droop of eyelids.
 EMG may show muscle fatigue
 Tensilon test- in MG reveal improved muscle contractility
after IV anticholinesterase agent edrophonium chloride
(tensilon)
 Also diagnosis cholinergic crisis- muscle weakness gets
worse
 Keep atropine on hand to counteract effects of tensilon
Therapeutic management
 Anticholinesterase inhibitors- prevents
anticholinestersase from breaking down ACh; helps
neurotransmission. Monitor dose!
 Mestinon, Prostigmine

Corticosteroids- decrease immune response

Prednisone
Plasmapheresis- removes ACh antibodies and
short-term improvement.
Nursing Management
 See handout!
Amyotrophic Lateral Sclerosis
(ALS or Lou Gehrig’s disease)

ALS
 Rare progressive disease characterized by
loss of motor neurons.
 Onset between 40 and 70 years of age
 Twice as many men as women
Motor neurons degenerate

ALS
 Motor neurons degenerate and leave dead tissue
which cannot send signals from brain to muscles
 Weakness of upper extremities, dysarthria,
dysphagia
 Muscle wasting
 Death from respiratory infection DT compromised
respiratory function; usually w/in 2-6 years.
ALS
 No cure
 No definitive test; diagnosis made through
physical exam and series of tests which rule
out other diseases which look like ALS.
 EMG and nerve conduction studies may be
done.
 Patient still cognitively intact!
 Drug Therapy
 Riluzole (Rilutek)- delays progression of ALS.
 Decreases amount of glutamate (an excitatory
neurotransmitter) in the brain.
 May delay need for trach and death by a few
months.
Nursing care
 Support cognitive and emotional functions
 Facilitate communication
 Provide diversional activities (read)
 Help family and pt with advanced planning
(hospice)
 Provide companionship
Trigeminal neuralgia

Trigeminal neuralgia
 Also called tic douloureax
 Uncommon cranial nerve disorder
 More common in women @ 50-60 years of
age
 Trigeminal nerve is 5th cranial nerve (CNV)
 And has both motor and sensory branches;
mostly maxillary and mandibular branches
involved.
Trigeminal neuralgia

Trigeminal Neuralgia
 Initiating pathologic events include:
 nerve compression by tortuous arteries of the
posterior fossa blood vessels
 demyelinating plaques
 herpes virus infection
 infection of teeth and jaw
 a brainstem infarct
Clinical manifestations
 Abrupt onset with excruciating pain!!
 Pain described as burning, knifelike, or
lightinglike shock in the lips, upper or lower
gums, cheek, forehead, or side of the nose.
 Patient may twitch, grimace, frequent blinking
and tearing of eye (tic) may occur.
Clinical manifestations
 Attacks may be brief (2 or 3 minutes)
 Unilateral
 Episodes may be initiated by triggering
mechanism of light cutaneous stimulation as
a specific point (trigger zone) along nerve
branches.
Precipitating stimuli
 Chewing, brushing teeth, hot or cold blast of
air on the face, washing the face, yawning, or
talking.
 Patient may eat improperly, neglect hygiene
practices, wear cloth over face, withdraw
from interaction with others.
Diagnostic studies
 Need to rule out other neurological causes of
facial and cephalic pain.
 CT scan will rule out brain lesions, vascular
malformations. LP and MRI will r/o MS.
 There is no specific diagnostic test for TN.
Drug Therapy
 Antiseizure meds may prevent and acute
attack or promote remission-mechanism
unknown.
 Carbamazeprine (Tegretol)…most common
 Phenytoin (Dilantin)
 Valproate (Depakene)
Drug Therapy
 Carbamazeprine has side effects:
 Bone marrow suppression leading to blood
abnormalities (CBC counts needed)
 Pain relief not permanent
Conservative Therapy
 Nerve block
 Biofeedback
Surgery
 Percutaneous radiofrequency rhizotomy
(electrocoagulation)- placing a needle into the
trigeminal nerve to destroy the area by
radiofrequency currents.
 May lose corneal reflex
 Easily performed; minimal risk
 Pain relieved, but face is numb
 (Table 57-2, Lewis, 1715)
Microvascular decompression
 Most common surgical procedure
 Blood vessels that are compressing the nerve
are displaced and repositioned. This relieves
pain without residual sensory loss.
 Long-term success rate
 Safe without residual sequelae
 Recurrence occurs in 30% of patients within
6 years
Glycerol rhizotomy
 Injecting glycerol near the root of the nerve
 Safer and fewer risks that percutaneous
types.
Nursing Interventions
 See handout

 Pay special attention to nursing

implementation section in Lewis (1716-1717).
Bell’s Palsy

Bell’s Palsy
 Characterized by:
 Peripheral facial paralysis
 Acute benign cranial polyneuritis

Acute disorder characterized by a disruption of the

motor branches of cranial nerve VII on one side of
the face. (in absence of stroke)
Bell’s Palsy
 Can affect any age  Reactivation causes
group, though more edema, inflammation,
common from 20-60. ischemia, and eventual
 Etiology unknown; demyelination of the
though reactivated nerve, creating pain
herpes simplex may be and alteration in motor
involved. and sensory function.
Clinical manifestations
 Benign, with 85% of 
people recovering in 6
months-remaining 15%
have some asymmetry
of facial muscles
Clinical manifestations
 Often accompanied by an outbreak of herpes
vesicles in or around the ear.
 Pain around or behind the ear
 Fever, tinnitus, hearing deficits
 Flaccidity of the affected side of the face with
drooping of the mouth accompanied by
drooling DT paralysis of the facial nerve
(motor branches)
Clinical manifestations
 Inability to close the eyelids, with an upward
movement of the eyeball when closure is attempted;
lower lid may turn out
 Wide palpebral fissure (opening between eyelids)
 Flattening of the nasolabial fold
 Inability to smile, frown, or whistle
 Unilateral loss of taste
 Altered chewing ability; loss of or excessive tearing
Complications
 Psychological withdrawal DT changes in
appearance,malnutrition or dehydration,
mucous membrane trauma, corneal abrasion,
muscle stretching, and facial spasms and
contractures.
Diagnostic Studies
 Diagnosis made on basis of symptoms in the
absence of other causes of paralysis such as
stroke.
 No definitive test
 EMG may determine nerve excitability or
absence
Therapeutic Management
 Corticosteroids- drug of choice
 Prednisone may be started immediately!
 Best if initiated before paralysis is complete
 Taper off over 2 weeks
 Decrease edema and pain
Analgesics may be needed for pain
Antivirals : Acyclovir (Zovirax) and Famvir because
HSV is implicated in 70% of cases.
See Lewis 1719-1720- Nursing Implementation
Guillain-Barre’

Guillain-Barre’ Syndrome
 Post-infectious polyneuropathy; ascending
polyneuropathic paralysis

 An acute, rapidly progressing and potentially

fatal form of polyneuritis
Guillain-Barre’ Syndrome
 Affects the peripheral nervous system

Guillain-Barre’
 T-cell sensitization occurs which causes loss
of myelin which disrupts nerve impulses
 Loss of myelin, edema and inflammation of
the affected nerves, causes a loss of
neurotransmission to the periphery.
 85% of patients recover with supportive care.
Pathophysiology
 Etiology unknown
 May be cell-mediated immunological reaction
directed at the peripheral nerves
 Frequently preceded by viral infection,
trauma, surgery or other immune system
stimulation.
Myelin Sheath

Clinical Manifestations
 Usually develop 1 to 3 weeks after URI or GI
infection
 Weakness of lower extremities (symmetrically)
 Parathesia (numbness and tingling), followed by
paralysis
 Hypotonia and areflexia (absence of reflexes)
 Pain in the form of muscles cramps or
hyperesthesias (worse at night).
Clinical manifestations
 Autonomic nervous system dysfunction results from
alterations in sympathetic and parasympathetic
nervous systems.
 Results in respiratory muscle paralysis, hypotension,
hypertension, bradycardia, heart block, asystole.
 Involvement of lower brainstem leads to facial and
eye weakness
Complications
 Most serious is respiratory failure.
 How do we manage?

Diagnostic studies
 Based on history and physical
 EMG and nerve conduction studies will be
abnormal
Therapeutic management
 Ventilator support!
 Plasmapheresis used within the first 2 weeks
of onset. If treated within the first 2 weeks,
LOS of morbidity is reduced. After three
weeks, plasmapharesis no benefit.
 IV immunoglobin
 Nutritional support (TF, TPN, Diet)
Nursing management
 See handout!

 Read Nursing Implementation: Lewis, 1607-

1608.
Reye Syndrome

Reye Syndrome (RS)
 Etiology obscure, but most cases follow a
common viral illness such as flu or chicken
pox.
 Characterized by cerebral edema and fatty
changes in the liver.
 W & W 1806-1807
RS-Pathophysiology
 Mitochondrial insult by several viruses, drugs,
exogenous toxins, and genetic factors
 Elevated ammonia levels
 Definitive dx made by liver biopsy
 Symptomatology range from lethargy to
comatose states due to liver dysfunction
Staging Criteria
 Box 37-8, Wong page 1806!
RS Nursing management
 Early assessment, diagnosis, treatment!
 Do not use aspirin to treat fever in children
with flu or varicella!
 Not as common as in the past due to
adequate labeling of meds and patient
education.
 Better diagnosing of children with viral and
metabolic illnesses.
Clinical manifestations
 Manifested by encephalopathy and coma!
 Changes in consciousness! (cerebral edema)
Lethargy to comatose!
 Vomiting (ICP)
Nursing considerations
 Monitor for and treat ICP
 Most deaths due to swelling due to delayed
treatment
 Monitor I & O
 Observe for complications of liver failure
(Bleeding from impaired coagulation)
Cerebral palsy (CP)

CP
 W & W 1966-1975
 Non-specific term that include disorders
characterized by early onset and impaired
movement and posture.
 Non-progressive and may include perceptual
problems, language deficits, and intellectual
involvement.
CP


Incidence
 Most common physical disability of childhood.
 Incidence has increased since the 60’s,
maybe due to improved survival of VLBW
infants.
Etiology
 Variety of perinatal, prenatal, and postnatal factors
contribute, either singly or multifactorily to CP.
 Commonly thought to be due to birth asphyxia; now
known to be due to existing prenatal brain
abnormalities.
 Premature delivery is the single most important
determinant of CP.
 In 24% of cases, no cause is found.
TABLE 40-1 Causes of CP
 Time (% of cases)  Causes
 Prenatal (44%)  Teratogens,
 First trimester chromosomal
abnormalities, genetic
syndromes, brain
malformations
 Second trimester  Intrauterine infections,
problems in
fetal/placental
functioning
Causes of CP
 Time (% of cases)  Causes
 Labor and delivery  Preeclampsia,
(19%) complications of labor
and delivery
 Perinatal (8%)  Sepsis/CNS infection,
asphyxia, prematurity
 Childhood (5%)  Meningitis, traumatic
brain injury, toxins

 Not obvious (24%)

Clinical Classification of CP
 Table 40-2, Page 1967.
 Spastic-hypertonicity with poor posture
control
 Dyskinetic/athetoid- abnormal involuntary
movement/slow wormlike writhing
 Ataxic- wide-based gait
 Mixed-type/dystonic- combination of
spasticity and athetosis
Clinical manifestations
 Delayed gross motor development
 A universal manifestation of CP
 The discrepancy between motor ability and
expected achievement tends to increase as
growth advances.
 Delayed development of ability to balance slows
milestones
 Delay in all motor accomplishments
Clinical Manifestations
 Abnormal motor performance
 Preferential unilateral hand use may be apparent
at 6 months.
 Hemiplegia, abnormal crawling or asymmetrical
crawl; spasticity may cause child to walk and
stand on toes
 dyskinetic CP or uncoordinated or involuntary
movements (writhing tongue, fingers, and toes;
facial grimacing), poor sucking and feeding,
persistent tongue thrust; head staggering, tremor
on reaching, truncal ataxia.
Alterations in muscle tone
 Increased or decreased resistance to passive
movement (abnormal muscle tone).
 Opisthotonic postures or exaggerated back arching,
feel stiff on dressing.
 Difficulty diapering due to spastic hip adductor
muscles and lower extremities
 When pulled to a sitting position, child may extend
the entire body and be rigid at hip and knee. This is
an early sign of spasticity.
Abnormal postures
 Children with spastic CP have abnormal posture at
rest or when position is changed
 Infantile lying prone may have hip higher than trunk
with legs and arms drawn in.
 Persistent infantile resting and sleeping position is a
sign of spasticity.
 Hemiparetic child may rest with affected arm
adducted and held against torso, with the elbow
pronated and slightly flexed and the hand closed.
Reflex Abnormalities
 Persistence of primitive infantile reflexes (one
of the earliest signs of CP)
 Tonic neck reflex
 Hyperactivity or moro, plantar, palmar grasp

Hyperreflexia, ankle clonus, stretch reflexes can be

elicited from any muscle group.
Associated disabilities and
problems
 Intellectual impairment
 70% w/in normal limits; wide range
 Tests should be carried out over a period of time.

 Children with athetosis and ataxia more intelligent.

Speech difficulties (not a sign or MR)- child has motor and

sensory defects
ADHD- (may occur)-poor attention span, marked distractibility,
hyperactive behavior
ASSOCIATED DISABILITIES
Seizures- generalized tonic-clonic;more in
postnatally acquired hemiplegia
Drooling- may occur and lead to wet clothing/skin
irritation
Feeding- alterations in muscle tone lead to
difficulties chewing, swallowing, talking, etc.
Address nutritional concerns.
Coughing, choking may lead to aspiration.
Altered respiratory patterns may lead to inadequate
gas exchange.
Motor Impairment
 Orthopedic complications
 Unilateral or bilateral hip dislocations, scoliosis, joint
contractures due to unbalanced muscle tone.
Decreased Mobility
 difficulties with toileting may lead to constipation

 Difficult chewing bulky foods may lead to constipation

 May need stool softeners or laxatives

Associated Problems
 Dental carries
 Improper dental hygiene
 congenital enamel defects (hyperplasia of
primary teeth)
 high carbohydrate intake and retention
 Dietary balance with poor nutritional intake
 Inadequate fluoride
 Difficulty in mouth closure and drooling
 Spastic or clonic movements cause gagging or
biting on toothbrush
Associated Problems
 Malocclusion in 90% of children
 Oral hypersensitivity causes resistance to
good hygiene
 Gingivitis is secondary to poor hygiene
 Dental health further complicated by anti-
seizure meds
Associated problems
 Nystagmus and amblyopia common
 May need surgery or corrective lenses
 May be due to sensoneural involvement
 Infants lying flat too long may have otitis media
which may leads to conductive hearing loss
Diagnostic Studies
 Physical Assessment
 Observe LBW, preterm, and those with low
Apgar scores at 5 minutes.
 Observe infants who have seizures,
intracranial hemorrhage, metabolic
disturbances
DX studies
 Since control of movement does not occur
until late infancy, dx may not be confirmed
until after 6 months of age.
 See Box 4-4, page 1968 for warning signs
WARNING SIGNS
 Physical Signs
 poor head control after 3 months
 stiff or rigid arms/legs, arching back, floppy
or limp posture
 Cannot sit up without support by 8 months
 Uses only one side of the body or only the
arms to crawl
Warning Signs
 Behavioral Signs
 Extreme irritability or crying
 Failure to smile by 3 months
 Feeding difficulties
 Persistent gagging or choking when fed
 After 6 months of age, tongue pushes soft food
out of the mouth.
Therapeutic management
 Box 40-5, Wong 1970.
 PHYSICAL THERAPY
 Most commonly used treatments.
 Goal is good skeletal alignment for the spastic
child.
 For the child with athetosis, training in purposeful
acts, even in the face of involuntary motion
 Maximum development of proprioceptive sense
for the child with ataxia.
 Orthotic devices (braces, splints, casting).
OCCUPATIONAL THERAPY
 Training in ADL’s along developmental lines.
 Sitting to walking; feeding to cooking.
 Important to incorporate play into program
 Adaptive equipment (utensils for functional
use, i.e., eating, writing), computers, etc.
Speech/Language therapy
 Early speech training by speech/language
pathologist !
 Before child develops poor habits
 Advice parents to follow directions of therapist
 May need to force child to use tongue/lips in
eating
Special Education
 Determined by child’s needs
 Early intervention programs
 Individualized Education Program (IEP)
 Specialized learning programs and support
services in schools
 Socialization to promote self-concept
development
Surgical Intervention
 Reserved for child who does not respond to
conservative therapy!
 Or whose spasticity causes progressive
deformities
Orthopedic surgery
 correct contractures or spastic deformities

 provide stability for uncontrolled joint

 provide balanced muscle power

Surgical Therapy
 Tendon-lengthening procedures (heel-cord)
 Release of spastic wrist flexor muscles
 Correction of hip-adductor muscle spasticity
or contracture to improve locomotion
 Surgery is for improved function rather than
cosmetic reasons and is followed by PT.
Medication Therapy
 Little usefulness
 Anti-anxiety agents may relieve excessive
motion and tension (child with athetosis)
 Skeletal muscle relaxants ( methocarbamol
(Tobaxin), dantrolene (Dantrium), Baclofen, may
be used short-term for older children and
adolescents.
 Diazepam (Valium) for older children and
adolescents, may relieve stiffness and ease
motion
Medications
 Local nerve blocks to motor points of a
muscle with a neurolytic agent (phenol
solution) may relieve spasticity.
 Botulism toxin (Botox) used to paralyze
certain muscles.
 Pain
 Secondary conditions (seizures, bowel and
bladder problems, lung complications).
Service Coordination
 Case Management!
 Important for collaboration of all health
professionals, services, therapies!
 Child needs support!
 Family needs support!