peds33 Flashcards
prognosis for SMA
type 1- die by 1 yo; types 2 and 3- survival until adult years is common
infantile botulism
bulbar weakness and paralysis secondary to inget og c. botulinim spores and absorption of the toxin
food sources of botulinum toxin
honey
what does the botulinm toxin do?
prevents the presynaptic release release of Ach
when is onset of symptoms for infantile botulism
12-48 hours after ingestion of spores; constipation is classic first sx; neuro sx follow then paralysis
if you do EMG on botulism patient, what do you see?
brief, small amp muscle potentials, with an incremental response during high freq stimulation
management of botulism
treatment is supportive with NG feeding and assisted vent as needed; botulism immune globulin improves clinical course; antibiotics are CONTRAindicated
prognosis of botulism
outlook is excellent, and complete recovery is expected but may take weeks or months
myotonia
inability to relax contracted muscles
congenital myotonic dystrophy
aut dom muscle disorder that presents in the newborn period with weakness and hypotonia
genetics of myotonic dystorpy
trinucleotide repeat disorder w aut dom inheritance and variable penetration; gene on chrom 19; transmission through mother in 90% of cases
clinica lfeatures of myotonic dystrophy
polyhydram due to poor swallowing; decr fetal movements; hypotonia, feeding and respir problems; areflexia, arthrogryposis
arthrogryposis
multiple joit contractures, seen in myotonic dystrophy
when dos myotonia develop in a baby with congen myotonic dystrophy?
not at birth; develops later, by 5 yo
management of myotonic dystrophy
supprotive; infant may require assisted ventilation and G tube feedings
prognosis for myotnic dystrophy
mortality 40%; all survivors have mental retardation; feeding problems subside w time
hydrocephalus
increased CSF under pressure in the ventricle in the brain
causes of hydrocephalis
blockage of CSF flow, decr CSF absorption, or incr CSF production
non-communicating hydrocephalus
enlarged ventricles caused by obstruction of CSF flow through the ventricular system
communicating hydroceph
enlarged ventricles as a result of incr production of CSF or decr absorption
hydrocephalus ex vacuo
not true hydrocephalus; ventricular enlargement due to brain atrophy
chiari type II malformation
downward displacement of the cerebellum and medulla through the foramen magnum, blocking CSF flow
chiari type II malform is associated with what other thing?
lumbosacral myelomeningocele
dandy-walker malformation
absent or hypoplastic cerebellar vermis and cystic enlargement of the fourth ventricle, which blocks CSF flow
congenital aqueductal stenosis
X-linked trait and patient have thumb abnormalities and other CNS anomalies like spina bifida
acquired causes of hydrocephalus
intraventricular hemorrhage, bacterial meningitis, brain tumors
sunset sign
tonic downward dviation of both eyes caused by pressure from enlarged third ventricles on the upward gaze center in the midbrain
brisk DTRs but with usually downward plantar response
sign of incr ICP
evaluation of hydrocephalus
urgent head CT
management of hydrocephalu
ventriculoperitoneal shunt; complications include shunt infection and shunt obstruction
prognosis of hydroceph
patients with aqueductal stenosis have the best cognitive outcome; chiari type 2 have low-normal intelligence; patients w x-linked hydroceph may have severe mental retardation
spina bifida
any failuere of bone fusion in the posterior midline of the vertebral column
myelomeningocele
herniation of the spinal cord and meninges through a bony cleft
meningocele
herniation of the meninges only throug a bony cleft; not usually associated w any neural deficits
which is more common- myelomeningocele or meningocele?
myelomeningocele
SB occulta
hairy patch or dimple; no herniation and no neuro deficit
how does a meningocele present?
fluctuant midline mass overlying the spine; mass is filled with CSF and can be transilluminated; neuro deficits not present
melomeningocele
fluctuant midline mass anywhere along spine but most likeley in lumbosacral region; neuro deficits are varibale
spina bifida associated anomaies
hydrocephalus, cervical hydrosyringomyelia, defects in neuronal migration, orthopedic problems, GU defects,
association between hydroceph and spina bifida
90% of lumbosacral myelomeningoceles are associated with chiari type 2 malformation and hydrocephalus; cervical and thoracic myelomenin are not associated w hydroceph
cervical hydrosyringomyelia
accum of fluid within the central spinal cord and within the cord itself
management of meningocele
surgical repair
management of meningomyelocele
urgent surgical repair within 24 hrs of birth to prevent infection and further trauma to exposed tissue
most common causes of coma in kids less than 5
abuse or near-drowning
in older kids, what is the most common cause of coma?
drug overdsose and accidental head injury
CSF or blood draining from the nose indicates what?
basilar skull fracture
decerebrate posturing
extension of arms and legs; indicates subcortical injury
decorticate posturing
flexion of arms and extension of legs; suggest bilateral cortical injury
asymm motor response
suggests hemispheric brain injury
