1. Myasthenia Gravis
Disease of the neuromuscular junction characterized by fluctuating weakness of certain skeletal muscle groups.

2. 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.

4. 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.

5. 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.

6. 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.

7. 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.

8. 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.

9. 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

10. 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.

11. Nursing Management
See handout!

12. Amyotrophic Lateral Sclerosis (ALS or Lou Gehrig’s disease)

13. ALS Rare progressive disease characterized by loss of motor neurons.
Onset between 40 and 70 years of age
Twice as many men as women

14. Motor neurons degenerate

15. 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.

16. 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!

17. 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.

18. Nursing care Support cognitive and emotional functions
Facilitate communication
Provide diversional activities (read)
Help family and pt with advanced planning (hospice)
Provide companionship

19. Trigeminal neuralgia

20. Trigeminal neuralgia Also called tic douloureax
Uncommon cranial nerve disorder
More common in years of age
Trigeminal nerve is 5th cranial nerve (CNV)
And has both motor and sensory branches; mostly maxillary and mandibular branches involved.

21. Trigeminal neuralgia

22. 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

23. 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.

24. 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.

25. 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.

26. 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.

27. Drug Therapy
Antiseizure meds may prevent and acute attack or promote remission-mechanism unknown.
Carbamazeprine (Tegretol)…most common
Phenytoin (Dilantin)
Valproate (Depakene)

28. Drug Therapy Carbamazeprine has side effects:
Bone marrow suppression leading to blood abnormalities (CBC counts needed)
Pain relief not permanent

29. Conservative Therapy
Nerve block
Biofeedback

30. 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)

31. 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

32. Glycerol rhizotomy Injecting glycerol near the root of the nerve
Safer and fewer risks that percutaneous types.

33. Nursing Interventions
See handout
Pay special attention to nursing implementation section in Lewis ( ).

34. Bell’s Palsy
                                                

35. 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)

36. Bell’s Palsy Can affect any age group, though more common from 20-60.
Etiology unknown; though reactivated herpes simplex may be involved.
Reactivation causes edema, inflammation, ischemia, and eventual demyelination of the nerve, creating pain and alteration in motor and sensory function.

37. Clinical manifestations
Benign, with 85% of people recovering in 6 months-remaining 15% have some asymmetry of facial muscles

38. 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)

39. 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

40. Complications
Psychological withdrawal DT changes in appearance,malnutrition or dehydration, mucous membrane trauma, corneal abrasion, muscle stretching, and facial spasms and contractures.

41. 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

42. 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 Nursing Implementation

43. Guillain-Barre’

44. Guillain-Barre’ Syndrome
Post-infectious polyneuropathy; ascending polyneuropathic paralysis
An acute, rapidly progressing and potentially fatal form of polyneuritis

45. Guillain-Barre’ Syndrome
Affects the peripheral nervous system

47. 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.

48. 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.

49. Myelin Sheath

50. 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).

51. 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

52. Complications
Most serious is respiratory failure.
How do we manage?

53. Diagnostic studies Based on history and physical
EMG and nerve conduction studies will be abnormal

54. 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)

55. Nursing management See handout!
Read Nursing Implementation: Lewis,

56. Reye Syndrome

57. 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

59. 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

60. Staging Criteria
Box 37-8, Wong page 1806!

61. 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.

62. Clinical manifestations
Manifested by encephalopathy and coma!
Changes in consciousness! (cerebral edema) Lethargy to comatose!
Vomiting (ICP)

63. 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)

64. Cerebral palsy (CP)

65. CP
W & W
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.

66. CP

67. Incidence Most common physical disability of childhood.
Incidence has increased since the 60’s, maybe due to improved survival of VLBW infants.

68. 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.

69. TABLE 40-1 Causes of CP Time (% of cases) Prenatal (44%) Causes
First trimester
Second trimester
Causes
Teratogens, chromosomal abnormalities, genetic syndromes, brain malformations
Intrauterine infections, problems in fetal/placental functioning

71. Causes of CP Time (% of cases) Labor and delivery (19%) Perinatal (8%)
Childhood (5%)
Not obvious (24%)
Causes
Preeclampsia, complications of labor and delivery
Sepsis/CNS infection, asphyxia, prematurity
Meningitis, traumatic brain injury, toxins

72. 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

74. 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

76. 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.

79. 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.

81. 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.

83. 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.

84. 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

86. 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.

87. 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

88. 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

89. 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

90. 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

91. 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

92. 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

93. 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

94. 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.

95. 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).

97. 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.

99. 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

100. 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

101. 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

102. 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.

103. 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

104. 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).

105. Service Coordination Case Management!
Important for collaboration of all health professionals, services, therapies!
Child needs support!
Family needs support!