ch 34 neuromuscular dysfunction

Question 1

early onset and impaired movement and posture

Answer 1

cerebral palsy

Question 2

causes cerebral palsy

Answer 2

-prenatal/postnatal infection
-prenatal/postnatal hypoxia/asphyxia
-*preterm birth of LBW
-anoxia

Question 3

types cerebral palsy

Answer 3

-spastic
-athetoid/dyskinetic
-ataxic
-mixed

Question 4

type cerebral palsy: characterized by persistent primitive reflexes

Answer 4

spastic

Question 5

diplegia

Answer 5

all extremities affected (CP)

Question 6

tetraplegia

Answer 6

all extremities + trunk, mouth, pharynx, and tongue affected (CP)

Question 7

type CP:
-involuntary irregular movements
-slow twisting movements trunk/extremities

Answer 7

dyskinetic

Question 8

type CP:
-wide based gait
-rapid repetitive movements
-disintegration of movement of upper extremities when child reaches for objects

Answer 8

ataxia

Question 9

possible early behavioral signs of CP

Answer 9

-excessive irritability
-no smiling by 3 mo
-feeding difficulty

Question 10

possible early motor signs of CP

Answer 10

-poor head control after 3 mo
-stiff/rigid limbs
-arching back/pushing away when being held
-floppy tone
-unable to sit w/o support at 8 mo
-clenched fists after 3 mo

Question 11

general S+S CP

Answer 11

-delayed gross motor development
-abnormal motor performance
-alterations motor tone
-abnormal postures
-reflex abnormalities
-associated disabilities

Question 12

meds to decrease spasticity in CP (4)

Answer 12

-bolulinum toxin (botox)
-baclofen (oral/implanted pump)
-dantrolene sodium (dantrium)
-diazepam (valium)

Question 13

children more likely to have neural tube defects

Answer 13

-girls
-hispanic

Question 14

med taken during pregnancy to prevent NTDs

Answer 14

folic acid

Question 15

most serious NTD
-incompatible with life
-many stillborn
-no resuscitation efforts
-comfort family

Answer 15

anencephaly

Question 16

types spina bifida

Answer 16

-oculta (not visible externally)
-cystica (saclike visible protrusion)
-meningocele (contains meninges and spinal fluid but not neural elements)
-myelomeningocele (contains meninges, spinal fluid, and neural elements)

Question 17

possible causes myelomeningocele

Answer 17

-drugs
-radiation
-maternal malnutrition
-chemicals
-genetic predisposition

Question 18

S+S myelomeningocele

Answer 18

*hydrocephalus
-obvious defect
-urine dribbling/overflow incontinence
-poor anal sphincter tone
-orthopedic deformities

Question 19

what can be tested prenatally to determine presence of spina bifida

Answer 19

alpha fetoprotein

Question 20

nursing interventions with spina bifida

Answer 20

-placement on stomach
-catheterization
-neuro checks before and after surgery
-*high prevalence of latex allergy (latex free environment)

Question 21

nursing consideration for kids with hypotonia

Answer 21

-resp care
-positioning

Question 22

S+S werdnig-hoffmann disease spinal muscular atrophy (type 1)

Answer 22

-onset within 6 mo of birth
-inactivity
-FTT

Question 23

S+S intermediate spinal muscular atrophy (type 2)

Answer 23

-onset within 18 mos of birth
-generalized weakness

Question 24

causes guillain barre syndrome (GBS)

Answer 24

-vaccines
-nonspecific viral illness

Question 25

S+S guillain barre syndrome

Answer 25

-paralysis 10 days after nonspecific viral illness -rapidly ascending paralysis -muscle tenderness, weakness -paresthesia -breathing difficulties and resp failure -loss of reflexes (including gag reflex*) -frequent urinary incontinence/retention and constipation

Question 26

Dx GBS

Answer 26

-CSF - increased protein concentration -physical findings (symmetric paralysis)

Question 27

Tx GBS

Answer 27

-supportive care -possible intubation if resp failure -steroids, IV immunoglobulin, plasmapheresis -heparin -gabapentin

Question 28

how often do you get tetanus shot

Answer 28

q10 years with injury

Question 29

S+S tetanus

Answer 29

-incubation period 3-10 days -muscle rigidity involving masseter and neck muscles -difficulty swallowing -rigid abdominal and limb muscles -resp: atelectasis, pneumonia, resp arrest -rapid HR, sweating

Question 30

4 requirements for developing lockjaw in tetanus

Answer 30

-presence of tetanus spores or vegetative forms of the bacillus -injury to tissues -wound conditions that encourage multiplication of organism -susceptible host (not vaccinated)

Question 31

Tx tetanus

Answer 31

-prevent with vaccine or antitoxin after exposure -Tx of contaminated wounds -monitor fluid and electrolytes -ICU admit for resp support -muscle relaxants -sedatives -pancuronium (pavulon) aids with intubation

Question 32

infant sources of botulism

Answer 32

honey light or dark corn syrup

Question 33

S+S botulism in children

Answer 33

-abrupt appearance of symptoms (12-36 hrs) -weakness, dizziness -headache -diplopia -speech difficulties -vomiting -progressive resp paralysis

Question 34

Tx botulism

Answer 34

-IV botulism immune globulin (babies get "babyBIG") -early Tx -resp support

Question 35

S+S botulism in infants

Answer 35

-constipation* -generalized weakness -loss of head control -feeding difficulty -weak cry -hypotonia -diminished gag reflex

Question 36

kids more likely to have myasthenia gravis

Answer 36

girls

Question 37

S+S myasthenia gravis

Answer 37

-progressive muscle weakness -difficulty swallowing, chewing, speaking -ptosis -diplopia

Question 38

Tx myasthenia gravis

Answer 38

-IvIG -prostigmine or neostigmine

Question 39

common onset age duchenne muscular dystrophy

Answer 39

3-5 yo

Question 40

what inheritance pattern is duchenne muscular dystrophy

Answer 40

-x linked -1/3 fresh mutations

Question 41

S+S duchenne MD

Answer 41

-waddling gait -frequent falls -*gower sign (using hands on legs to stand) -enlarged muscles (pseudohypertrophy) -mild to moderate mental impairment -loss independent ambulation by 9-12 yo -eventual death resp/cardiac failure

Question 42

Dx duchenne MD

Answer 42

-elevated serum CPK and AST -EMG -muscle biopsy

Question 43

Tx duchenne MD

Answer 43

-maintain function in muscles as long as possible -bracing -ROM -ADLs -surgical release contractures prn -genetic counseling

Question 44

meds to treat reyes syndrome

Answer 44

-corticosteroids -seizure meds -mannitol -vit k

Question 45

cushings triad signs with increased ICP

Answer 45

-increased SBP -widened pulse pressure -bradycardia -irregular respirations