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1
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what is microcephaly
abnormally small head with resultant cognitive impairments, from mild to severe
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what is the primary cause of microcephaly
genetic, chromosomal, or hereditary problems
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what is the secondary cause of microcephaly
exposure to radiation, maternal infection (TORCH), maternal alcohol or tobacco, zika virus
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what are clinical presentations with microcephaly
global delays in cognitive and motor development
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how is microcephaly diagnosed
MRI of brain; may be detected upon fetal ultrasound
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is there a cure for microcephaly
no, nursing care is supportive

early intervention services
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what is hydrocephalus
when CSF collects in the brain causing an enlargement in the ventricles thereby causing harmful pressure on the other fragile brain tissues
8
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what can cause hydrocephalus
brain tumors, head injury, bleeding, infection, arnold chiari malformations
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symptoms of hydrocephalus
rapid increase in head circumference

dilated scalp vein and/or separation of cranial sutures

sun setting eyes

abnormal pupillary responses

difficulty holding head upright

bulging fontanel

vomiting, poor feeding

lethargy, irritability

headaches in AM
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what are some symptoms of shunt malfunction in hydrocephalus
leaking CSF at the head along the shunt area

halo sign on linens (blood tinged spot encircled by lighter ring = CSF leakage)
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diagnosis of hydrocephalus
MRI of brain

cisternogram (study of CSF flow dynamics)
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what is cisternogram
study of CSF flow dynamics
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what is the treatment for hydrocephalus
surgical ventriculostomy with placement of ventriculoperitoneal (VP) shunt
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what is preop care for hydrocephalus
frequent position changes of the head, IV antibiotics, diuretics, baseline HC, baseline abd circumference
15
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post op care of hydrocephalus
immediate - lay flat, do not elevate higher than 30 degrees to prevent rapid ventricular decompression

position on non ope side, frequent changes

close neuro assessment and incision checks

daily HC and abd circumference (girth and BS)

monitor for changes in fontanel or change in behavior

complications: paralytic ileus, peritonitis, infection, shunt malfunction
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what are some post op complications for hydrocephalus
paralytic ileus, peritonitis, infection, shunt malfunction
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why should you not elevate HOB for immediate post op for hydrocephalus
rapid ventricular decompression can occur
18
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what is meningitis
infection affecting the meninges of the brain and spinal cord

medical emergency and is life threatening
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how is meningitis classified
septic (bacterial) and aseptic (viral)
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how is meningitis spread
close contact with respiratory secretions
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what are the symptoms of meningitis
severe headache, stiff neck (nuchal), sudden high fever, photosensitivity, nausea, purpuric rash
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is septic or aseptic meningitis less severe and self limiting
aseptic (viral)
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what will the nurse see on assessment of a child greater than 2
kernig’s and brudzinski’s signs

may also see a petechiae or purpuric rach
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what will the nurse see on assessment of a child less than 2
will usually present with fever, seizures, high pitched cry, irritability, sleepiness, bulging fontanels (infants), poor feeding, and vomiting
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how do you diagnose meningitis
CBC and lumbar puncture
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what is the nursing care for meningitis
administer antibiotics (only for septic), supportive care, seizure precautions, close monitoring to recognize subtle changes or complications of increased ICP, infection control measures (droplet)
27
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what is important during lumbar puncture
positioning (lateral recumbent, prone, or sitting upright)and maintaining sterile field
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what is the post op nursing care for lumbar puncture
pressure to puncture site, place pt flat on bed for 4-6 hours, monitor VS, neuro status, I&Os, monitor puncture site for signs of CSF leakage and blood
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how is encephalitis different from meningitis
it is also characterized by an infection surrounding the meninges but with subsequent cerebral edema
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what is encephalitis associated with
mosquito born viruses or herpes virus

systemic illnesses such as polio, mono, and rabies
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what is the clinical presentation of encephalitis
lethargy, nuchal rigidity, photophobia, and behavioral changes that rapidly progress to a decreased level of consciousness, flaccid paralysis, and seizure activity
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what are the diagnostic tests for encephalitis
lab work, LP, head MRI
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how to prevent encephalitis
routine vaccinations, mosquito mitigation
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what is the nursing care for encephalitis
aimed at cause with antivirals, antipyretics, antiinflammatories, and antiepileptics, seizure precautions, close neuro monitoring, and fluid and electrolyte monitoring
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what are the long term deficits of encephalitis
cognitive, neurological, and physical
36
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is encephalitis viral or bacterial
often viral

presents as an acute febrile illness that may progress within hours
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what is reye’s syndrome
serious illness defined by rapid progression, subsequent multiorgan failure with a poor outcome
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when does reye’s syndrome occur
when aspirin is administered to a child during a viral illness which leads to cascade of events resulting in acute noninflammatory encephalitis and fatty degenerative liver failure
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how many stages of reye’s syndrome are there
0-6 with rapid progression
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what happens in stage 4 of reye’s syndrme
deep coma
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how do you diagnose reye’s syndrome
liver biopsy, LP, lab tests
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what is the treatment of reye’s syndrome
collaborative in a PICU, bleeding and seizure precautions, steroids to decrease cerebral edema, diuretics to decrease ICP, electrolytes to correct imbalances
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how to prevent reye’s syndrome
avoid aspirin in children
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why is prevention of reye’s so important
by the time the child has sx, the body has already been severely compromised
45
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what is epilepsy
condition in which seizures are recurrent and unprovoked
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what are focal seizures
abnormal electrical activity is one hemisphere (partial)
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simple focal seizures
unlikely to lose consciousness, may smell or taste something strange and may see flashes of light, may hear a buzzing sound, feel tingling or feel anxious or dizzy
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complex focal seizures
begins as a simple focal and may include lip smacking, gagging, or rhythmic movements or inappropriate mannerisms

there is impairment of consciousness
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generalized seizures
abnormal electricity in both hemispheres

may manifest as loss of consciousness and may present with motor or nonmotor activity and have a postictal state afterward
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what should parents do for seizure lasting >2 mins with loss of awareness
administer rectal diazepam or intranasal midazolam at home
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what may lengthy seizures require
resp support and IV meds
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absense seizure
(generalized)

loss of consciousness with vacant stare or unresponsiveness; may have lip mvmts, chewing, fluttering, eyelids

often misdiagnosed as ADHD or day dreamer
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myoclonic seizure
(generalized)

sudden forceful contractions of single or multiple groups of muscles

can be brief and may be mistaken for tics, tremors, or clumsiness
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tonic-clonic seizures
(generalized)

combo of body stiffening (tonic) and clonic rhythmic activity with loss of consciousness

as child begins to relax, may lose control over bowel/bladder
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atonic
(generalized)

loss of muscle tone, person suddenly drops. Known as drop attacks - complete loss of tone as muscles become limp, child falls. Brief attacks. Protective headgear recommended
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common seizure triggers
sleep deprivation, drugs and alcohol, increased stress, not eating well, flashing bright lights or patterns, illness or fever, changes in hormones (menstrual cycle)
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status epliepticus
brain in state of constant seizure

children who experience this in early life are at risk for hippocampal and temporal lobe sclerosis

lasts 5 mins or longer or is recurrent seizures without regaining consciousness between seizures for >5 minutes

medical emercency
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febrile seizures
convulsions triggered by rise in body temp >101.2

common in children 6 months to 3 years

lasts a few mins

have not been found to cause brain damage
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nursing interventions for seizure
turn on their side

protect head from injury

do not put anything in the mouth

do not restrain

loosen clothing around the neck

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what are the commonly prescribed meds for seizures
phenytoin, carbamazepine, valproic acid, fosphenytion, topiramate, gabapentin, and ethosuximide
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education for seizure meds
take at the same time every day; labs are often required at frequent intervals, do not stop abruptly
62
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what is valproic acid for
used for both focal and tonic clonic seizures

there are loading doses and maintenance doses

this requires serum drug monitoring

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what is dilantin for
focal and tonic clonic

requires serum drug monitoring
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what are the adverse effects of valproic acid
confusion, nystagmus, nausea, bleeding, gingival hyperplasia
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what are the adverse effects of dilantin (phenytoin)
dizziness, drowsiness, uncoordinated movements, gingival hyperplasia
66
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what is phenobarbital for
focal and tonic clonic seizures

loading and maintenance doses

requires serum drug monitoring
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what are adverse effects of phenobarbital
hyperactivity, inattentiveness, dizziness, fever, rash, slurred speech
68
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what are examples of primary TBI
skull fracture

bleeding

concussion

contusion
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what are examples of secondary TBI
tissue ischemia and hypoxia

cerebral edema

increased ICP
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early signs of TBI
photophobia, n/v, headaches, vertigo, shrill or high pitched cry, irritability, lethargy, difficult to wake up, poor feeding (infant), amnesia/confusion, altered LOC, leakage of CSF from ears nose, changes in behavior
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late signs of TBI
increased ICP, increased head size in kids
72
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what are symptoms of epirual hematoma
(bleeding between the dura and skull)

short period of unconsciousness followed by normal period for several hours, then lethargy or coma due to accumulation of blood
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what are symptoms of subdural hemorrhage
(bleeding between dura and arachnoid membrane)

irritability, vomiting, increased head circumference, lethargy, seizures, coma
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what can subdural hemorrhage be from
birth injuries, falls, or violent shaking
75
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what are symptoms of brain herniation
(downward shift of brain tissue)

loss of blinking, loss of gag reflex, lack of PERRLA, systemic HTN, bradycardia, coam, respiratory arrest
76
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what is decorticate positioning
upper limbs flex and lower limbs extend - less severe and indicates damage to one or both corticospinal tracts
77
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what is decerebrate positioning
upper and lower limbs extend - damage to the upper portion of the brainstem and has a poor outcome
78
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what is cerebral palsy
results from developing brain either failing to form correctly or receiving some type of injury causing permanent damage to the brain with an inability to control one’s muscles and movements
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what are risk factors for cerebral pulsy
low birth weight, premature, multiple births, use of infertility procedures, maternal infections of thyroid/seizure disorder, jaundice or kernicterus, birth complications
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causes of cerebral palsy
acquired within first 28 days of life, meningitis, head trauma, sickle cell disease, stroke, AV malformation, bleeding disorder
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what are the four type of cerebral palsy
spastic, dyskinetic, ataxic, mixed
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spastic CP
most common

stiff, rigid, muscles, difficult to move

increased muscle tone makes performing smooth, coordinated movements difficult
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presentation of spastic CP
developmental delay, gross and/or fine motor delay, increased/decreased muscle tone

feeding difficulties with potential FTT due to gagging, choking, tongue thrust, chewing, and swallowing difficulties

predisposed to seizures or cognitive delay - often phsyically but not intellectually disabled
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interventions for spastic CP
OT, PT, speech therapist
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meds for CP
baclofen, diazepam, botulinum toxin A
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baclofen
for CP, skseltal muscles relaxant to decrease muscle spasm and severe spasticity
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diazepam
for CP, skeletal muscle relaxant, often used in older children and adolescents - monitor for drowsiness and fatigue
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botulinum toxin A
for CP, reduces spasticity in specific muscle groups; lasts 3-6 months
89
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neural tube defects
two most common are spina bifida and anencephaly
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spina bifida cystica (myelomeningocele)
section of the spinal cord and nerves are exposed and visible on the outside of the body

causes partial or complete paralysis below the spinal opening
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spina bifida occulta
section of the spinal vertebrae is malformed but the cord and nerves are normal

no visible defect but usually dimpling, hair tufts, or a hemangioma in the lumbar sacral area

found incidentally and does not result in physical deficits
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spina bifida
may have paralysis of the legs or may have flaccid muscles with sensory loss. Problems with bowel and bladder control are also common
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preop nursing care for spina bifida cystica
protect sac from injury

place infant in radiant warmer without clothing

apply a sterile, non adherent dressing with NS on the cyst, rewetting as needed to prevent drying

inspect cyst closely for signs of leakage

assess for signs of infection

avoid rectal temps

avoid putting pressure on the sac
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post op nursing care for spina bifida cystica
monitor VS, weight, I&Os, head circumference

assess for s/sx of infection

pain management

assess for leakage of CSF

maintain prone position until other positions are rxd

resume oral feedings

provide ROM to extremities

assess fontanels for bulging

education on post op care
95
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what is muscular dystrophy
progressive muscle weakness due to muscle fiber degeneration and muscle wasting
96
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duchene muscular dystrophy (DMD)
x linked, affect only males

genetic defect affects the protein dystrophin - this normally provides muscles the ability to be strong and stable over time
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clinical presentation of DMD
generalized muscle weakness first noticed in the proximal muscles of the arms and legs

overly clumsy, difficulty climbing stairs

calf muscle hypertrophy, toe walk

inability to keep up with peers in age appropriate activities

gower sign

as disease progresses, loss of ambulation and impairment in respiratory and cardiac function
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what are the labs for DMD
elevated creatinine kinase
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what is gower sign
sitting on floor to standing - will use arms to help them up
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DMD interventions
early diagnosis and initiation of therapy are essential

progressive loss of function

PT help maintain muscle strength and prevent contractures

frequent resp infections common

important to monitor PFTs

usually treated with steroids but at high risk for fractures

encourage routine immunizations