congenital and pediatric neurological disorders Flashcards

1
Q

neural tube closes…

A

15-28 days post conception

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2
Q

90% of neural tube defects are…

A

spina bifida and anencephaly

  • 1 in 5000 live births
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3
Q

anencephaly

A

both cerebral hemispheres absent

  • incompatible with life, many stillborn
  • if born alive, given comfort measures, DNR
  • most pts know this in advance
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4
Q

spina bifida (in general)

A

failure of closure of neural tube

NORMAL INTELLECT should be assumed in the absence of significant hydrocephalus

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5
Q

spina bifida occulta

A

failure of vertebrae to close WITHOUT protrusion of spinal cord or meninges
- not visible externally

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6
Q

spina bifida occulta: cutaneous manifestations

A

may or may not have skin depression, tuft of hair

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7
Q

spina bifida occulta: neuromuscular disturbances

A

most often asymptomatic

- may have foot weakness, bowel/bladder sphincter issues

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8
Q

spina bifida cystica

A

visible defect with saclike protrusion

- meningocele or myelomeningocele

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9
Q

meningocele

A

rare, contains meninges and CSF only

form of spina bifida cystica

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10
Q

myelomeningocele

A

most common, contains meninges, CSF, spinal cord, and/or nerve roots

form of spina bifida cystica

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11
Q

spina bifida risks

A
  • previous pregnancies with neural tube defects
  • folic acid deficiency
  • girls > boys
  • caucasian > african american
  • diabetes in mom
  • obesity
  • anticonvulsants
  • maternal heat exposure (hot tubs!)
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12
Q

folic acid

A

big deal!

  • aap recommended daily intake: 0.4 mg
  • preconceptual folate supplement may decrease incidence of ntd by 50-70%
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13
Q

folic acid for previous ntd*

A

increase to 4.0mg

  • 1 month before pregnancy through 1st trimester
  • under provider supervision!
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14
Q

prenatal screening detection (4)

A
  • ultrasound (16-18 weeks gestation)
    • accurate for dates NOT defects
  • alpha-fetoprotein (afp)
  • fetal mri
  • amniocentesis (14-17 weeks)
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15
Q

myelomeningocele: degree of neuro impairment:

A

usually loss of sensation and complete/partial paralysis

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16
Q

hydrocephalus

A

increased accumulation of csf within ventricles of brain; acquired vs congenital

  • most frequent anomaly associated with spina bifida
  • monitor for s/s of increased intracranial pressure (icp)
  • may not be apparent until after birth and after primary closure of defect*
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17
Q

myelomeningocele: effect on bladder

A

decreased nerve supply affects both sphincter and detrusor

- can cause constant dribbling or lead to overflow incontinence

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18
Q

myelomeningocele: effect on anal sphincter tone

A

lack of bowel control

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19
Q

myelomeningocele: orthopaedic abnormalities

A

dislocated hips, club feet

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20
Q

myelomeningocele: spinal deformities

A

scoliosis (more profound vs scoliotic patient with closed neural tube), kyphosis

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21
Q

spina bifida management: initial care*

A
  • PREVENTION OF INFECTION!
  • sac exposed to air
  • monitor closely
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22
Q

spina bifida management: neuro assessment

A
  • daily head circumference
  • assess fontanels for tension, bulging
  • check for associated anomalies
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23
Q

anterior fontanel closes

A

15-18 mo

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24
Q

posterior fontanel closes

A

1 month

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25
spina bifida management: early closure of defect
- within 24-72 hours after birth - increased chance of most favorable outcome -- decreases morbidity/mortality especially from serious infection - decreases trauma to tissues - decreases stretching of nerve root
26
spina bifida care: gu functioning
- urinary diversion | - often need to be intermittently catheterized on regular schedule: parents taught, child taught around 6 yo
27
spina bifida care: bowel control
diet modification, regular toilet habits = decreases constipation and impaction
28
acquired hydrocephalus
infection, neoplasm, hemorrhage
29
communicating hydrocephalus
impaired absorption
30
non-communicating hydrocephalus
impaired/obstructed flow of csf through ventricle system
31
hydrocephalus: clinical manifestations in infancy
CUSHING'S TRIAD - bulging fontanel - dilated scalp veins - head growth abnormal rate - frontal bossing - sunsetting eyes - irritable, lethargic, change in loc, paradoxical cry, high pitched "neuro" cry, poor feeder
32
cushing's triad*
classic s/s increased icp - increased blood pressure, widened pulse pressure - bradycardia - abnormal or decreased RR look at vitals TREND!
33
hydrocephalus: clinical manifestations in child
- irritable, lethargic, confused, apathetic - headache on awakening: improves after emesis or sitting up - ataxia - CUSHING'S TRIAD
34
hydrocephalus: diagnosis
antenatal: fetal us @ 14 weeks - increasing head circumference - associated neurologic signs - primary diagnostic tools: CT, MRI
35
hydrocephalus: shunt procedure (2)
provides primary drainage of csf from ventricles to extracranial compartment, usually peritoneum - ventriculoperitoneal shunt (VP) - ventriculoatrial shunt (VA)
36
ventriculoperitoneal shunt (VP)
surgical management of hydrocephalus - most common
37
ventriculoatrial shunt (VA)
surgical management of hydrocephalus - rare
38
VP shunt complications: infection
MOST SERIOUS! greatest risk 1-2 months after placement, may be sepsis or wound infection
39
VP shunt complications: infection - s/s
shunt malfunction due to increased icp, fever, wound/shunt inflammation, abdominal pain
40
VP shunt complications: infection - treatment
IV antibiotics for 7-10 days | - often requires removal of shunt and placement of extraventricular drainage system (EVD)
41
extraventricular drainage system
possible procedure necessary after IV antibiotics for VP shunt infection - externalizes shunt until infection resolved - allows for removal of CSF to external collection - otherwise, shunt removed = increased icp
42
VP shunt complications: shunt malfunction
- typically mechanical obstruction | - most common reason for revision = displacement for growth
43
VP shunt complications: shunt malfunction - s/s
also increased icp s/s! older child: typical + personality change, neck pain, deterioration in school performance, decrease in sensory or motor functions
44
have a shunt and vomiting? then...
SHUNT SERIES! CHECK FOR MALFORMATION! always, even if suspected stomach bug etc
45
important nursing care s/p shunt malfunction
do NOT sedate child to reduce irritability - potentially masking s/s of increased icp
46
hydrocephaly: prognosis - untreated
50-60% mortality if left untreated | - survivors have high incidence of subnormal intellectual capacity
47
hydrocephaly: prognosis - surgical treatment
90% survival, 2/3 intellectually normal
48
meningitis + types (3)
inflammatory process of meninges and csf - bacterial (pyogenic) - viral (aseptic) - tuberculous
49
meningitis: bacterial - etiology > 2 mo
H flu, strep pneumo, n meningitidis
50
meningitis: bacterial - etiology neonates
gbs, e coli, listeria
51
HIB vaccine
decreased incidence of meningitis!!!!
52
bacterial meningitis*
most common route of infection*
53
bacterial meningitis: pathophysiology
- vascular dissemination from a focus of infection elsewhere* - post lp, surgical procedures - anatomic abnormalities (spina bifida!) - foreign body (VP shunt!) - infective process (exudate, wbc accumulation, tissue damage - brain: hyperemic, edematous, covered with purulent exudate, can lead to obstructive hydrocephalus
54
hyperemic
increased blood flow to certain part of body
55
bacterial meningitis: clinical manifestations in children/adolescents
abrupt or gradual onset: - fever, chills, headache, v, change in sensorium, irritable, agitated, photophobia, confusion, hallucinations, drowsiness, stupor, coma - SEIZURE can be initial manifestation! - nuchal rigidity! NOT CONSISTENTLY PRESENT IN > 18 MO - kernig sign, brudzinski sign - meningococcal purpuric/petechial rash
56
kernig sign
clinical manifestation of bacterial meningitis in children/adolescents flex thigh on body and extend leg - positive: resistance and pain in hamstring NOT CONSISTENTLY PRESENT IN > 18 MO
57
brudzinski sign
clinical manifestation of bacterial meningitis in children/adolescents pt supine: flex head - positive: pain or hips/knees flex involuntarily NOT CONSISTENTLY PRESENT IN > 18 MO
58
meningococcal-purpuric/petechial rash clinical manifestation of...
bacterial meningitis
59
bacterial meningitis: clinical manifestations in infants/young children
3 mo - 2 yo - bulging fontanel MOST SIGNIFICANT - fever, poor feeding, vomiting, irritable, restless, high pitched cry * nuchal rigidity, brudzinski, kernig NOT CONSISTENTLY PRESENT IN > 18 MO
60
bacterial meningitis: clinical manifestations in neonate
difficult to diagnose! vague like ALL neonatal sepsis | - neck is usually supple
61
bacterial meningitis: csf diagnosis
lumbar puncture (DEFINITIVE TEST) - increased spinal fluid pressure - elevated wbc, protein concentration - glucose DECREASED - cloudy - gram stain reactive
62
menectra vaccine
prevents bacterial meningitis, for ages 11-55
63
viral meningitis: csf diagnosis
lumbar puncture - wbc slightly elevated - protein WNL or slight increase - glucose WNL - clear/slightly cloudy - gram stain: not reactive
64
viral meningitis: manifestations & tx
clinical manifestations: ha, fever, malaise, gi symptoms treatment: if symptomatic, hydration, pain management, positioning