Peds Flashcards
when does the NS first appear during gestation?
day 21
the neural tube closes at _____ days from ____ to ____
23-25
anterior to posterior
what are the 3 primary brain vesicles?
prosencephalon
mesencephalon
rhombencephalon
by week ____ of gestation there are 5 vesicles
5
which adult structures come from the telencephalon?
cerebral hemispheres
which adult structures come from the diencephalon?
thalamus
GP
hypothalamus
which adult structures come from the mesencephalon?
midbrain
which adult structures come from the metencephalon?
pons
cerebellum
which adult structures come from the myencephalon?
medulla
which supplement should be taken to prevent neural tube defects?
folate
dysraphism is disorders of the
neural tube closure
spina bifida occulta
vertebral arch defect only
10% of population
usually asymptomatic
spina bifida meningocele
dura and arachnoid herniation through vertebral defect
spina bifida myelomeningocele
herniation of spinal cord and meninges through defect
where is spina bifida occulta most common at (levels)
L5-S1
which spina bifida can present with tethered cord syndrome and persistent neuro defects?
myelomeningocele
what is the most common presentation of tethered cord?
unilateral LMN dysfunction in 1 leg
what is an Arnold-Chiari Malformation?
congenital abnormality of hindbrain downward elongation of brainstem and cerebellum in to cervical portion of SC
type 1 Arnold-Chiari Malformation
cerebellar tonsils displaced >6mm
type 2 Arnold-Chiari Malformation
associated myelomeningocele
type 3 Arnold-Chiari Malformation
associated encephalocele
how does an Arnold-Chiari HA present?
HA with increased ICP and CN Sx
what is a Dany-Walker Malformation?
cerebellar vermis developmental anomaly with large cyst in posterior fossa
sx of Dany-Walker Malformation
mental retardation
hydrocephalus
spastic diplegia
what is craniostenosis?
skull sutures fuse early
developmental delay is diagnosed when a developmental domain quotient is <___%
70%
global developmental delay is diagnosed when there is delay in ____ developmental domain(s)
2 or more
at what age should babies be walking and saying their first word?
12 months
what is a vital sign for peds?
head circumference
red flags for peds
head size crossing 2 percentiles
failure to respond to sounds
fisting beyond 3 months
rolling before 3 months
persistent MORO >6 months
persistent ATNR & not rolling >7 mo
not sitting >9 mo
delayed smiling, laughing, cooing, first words
handedness below 12 months
no words after 2 years
persistent echolalia >3 years
noticeable stutter after 4 years
decline in speech ability or vocabulary
a baby should not have handedness until
12 months
T/F: fragile X syndrome only occurs in males
T
characteristics of trisomy 21
single palmar crease
broad flat face
short nose
hypotonia
umbilical hernia
what is the most common chromosomal cause intellectual disability?
fragile X syndrome
what does TORCHS stand for when considering infectious causes of developmental delay?
Toxoplasmosis
Other (Zika)
Rubella
Cytomegalovirus
Herpes Simplex, HIV
Syphilis
what is the most important intervention for children with developmental delay?
referral to specialist
what is a key characteristic of cerebral palsy?
it’s NONPROGRESSIVE
what is the most common form of CP?
spastic
which type of CP is hypotonic? hypertonic?
hypotonic - ataxic
hypertonic - spasticity
what is the most common combination for mixed CP?
spastic with dyskinesias
what is a common cause of spastic hemiparesis?
intrauterine stroke affecting the cerebral artery
is the arm or leg more affected in spastic hemiparesis?
arm
if a child develops handedness before 18 mo, what is suspected?
spastic hemiparesis
spastic diplegia effects the ___ more
legs (weakness)
scissoring gait is seen in spastic _____
diplegia
d/t adductor spasm
what blood supply is affected in spastic diplegia?
borderzone of ACA and PCA
what is the most severe form of spastic CP?
spastic quadriplegia
dyskinetic CP occurs due to lesion at the
basal ganglia
what is the main causes of dyskinetic CP?
neonatal hyperbilirubinemia
severe anoxia
what is the rarest form of CP?
ataxic
ataxic CP occurs due to abnormal development of the
cerebellum
*trunkal and gait ataxia
meds for CP
baclofen
diazepma
tizamidine
botulinum A
neurocutaneous disease are also called
phakomatoses
why aren’t neurofibromas removed?
they are entangled within the nerve itself
sx of CNS neurofibromatosis
B acoustic neuromas
meningioma
gliomas
sx of PNS neurofibromatosis
cafe-au-lait spots
neurofibromas on skin, peripheral nerves, and/or roots
optic pathway glioma
presentation of tuberous sclerosis
white, hard nodules on surface of brain and jutting into ventricles, calcifications
where are lesions in tuberous sclerosis?
NS
skin
bones
retina
kidney
what is a common sx of tuberous sclerosis?
seizures
leukodystrophies are a _____ progressive genetic disorder affecting _____
demyelinating
myelin metabolism
adrenoleukodystrophy has issues with the ____ matter
white
when do changes appear with adrenoleukodystrophy?
around 8 years old
(normal early development)
sx of adrenoleukodystrophy
only in boys
behavioral changes
visual loss
progressive dementia
seizures
progressively spastic gait
metachromatic leukodystrophy is degeneration of myelin where?
central AND peripheral
T/F: children with tone abnormalities ALWAYS have low tone early on then develop high tone if they are going to present with high tone
T
what is the most common MD is children?
Duchenne MD
define muscular dystrophy
group of progressive hereditary disorders of muscle
define myotonias
involuntary persistent muscle activity occurring in response to percussion or activity of the muscle
2 categories of myopathies
metabolic
congenital
psudeohypertrophy of the ____ is seen in Duchenne and Becker MD
calf
hallmark of Duchenne MD
boys enter a WC by 9-12 yo
no dystrophin present
Does Becker MD have an early or later onset than Duchenne?
later
Pts with Becker MD are ambulatory until
30 yo
Becker MD has isolated ___ weakness
quad
high ____ is seen in Becker and Duchenne MD
CK
what med is used to maintain ambulation in dystrophinopathies?
corticosteroids
what can be a life changing treatment for dystrophinopathies?
gene therapy
is spinal muscular atrophy UMN, LMN, or both?
LMN only
spinal muscular atrophy results in progressive loss of
motor neurons in SC
type 1 spinal muscular atrophy never
sit
type 2 spinal muscular atrophy never
stand
type 3 spinal muscular atrophy never
run
how does type 4 spinal muscular atrophy present?
proximal leg weakness as an adult
which spinal muscular atrophy types have a normal lifespan?
3 and 4
what is a common cause of infantile botulism?
eating honey
(also corn syrup)
infantile botulism prevents the release of
ACh
sx of Infantile botulism
acute generalized weakness
loss of reflexes
hypotonia
ptosis
poorly reactive pupils**
respiratory difficulty
which part of the brachial plexus is more commonly injured?
upper
C5-C6
Erb’s palsy is due to injury of _____ (levels)
C5-C6
MOI for Erb’s palsy
head pushed down away from shoulder
how does arm rest with Erb’s palsy?
shoulder adduction
elbow extension
pronation
Klumpke Palsy is due to injury of ____ levels
C8-T1
what hand deformity does Klumpke Palsy cause?
claw hand
MOI for Klumpke Palsy
arm is pulled upwards
