Neurology, Developmental and ENT Flashcards
Three chemoreceptors important for neuronal growth, migration and differentation
NMDA, opioid, and GABA
(NMDA and GABA influenced by alcohol, GABA by benzos, NMDA/glutamate and GABA by anesthesia)
Associations in Dandy Walker
80% develop hydrocephalus
40% other CNS abnormalities: absent corpus callosum, posterior encephalocele, polymicrogyria
If Dandy Walker is syndromic, what syndrome is associated?
PHACES (posterior fossa abnormality, hemangiomas, arterial lesions [HEENT], cardiac, eye, sternal)
What is a Dandy Walker malformation?
Aplastic vermis (connection between cerebellar hemispheres)
Large posterior fossa (fourth ventricle not separated from cisterna magna)
Abnormally high tentorium
What is considered the “prolonged” anesthesia exposure that may impact neurodevelopmental outcomes in patients <3 years old?
>3 hours
Believed due to the inhibition of glutamate (specifically NMDA glutamate) and GABA receptors, leading to apoptosis
Therapeutic hypothermia decreases the incidence of death/disability in asphyxiated infants with moderate encephalopathy from ___ to ___%
50 to 30% (approximately)
Two most sensitive imaging modalities to estimate injury from hypoxic-ischemic encephalopathy (aka perinatal asphyxia) and their ideal timing
DWI MRI (diffusion-weighted imaging)
MR-spectroscopy
3-5 days from injury
Three most vulnerable/common areas of hypoxic brain injury
- Basal ganglia
- Thalamus
- Internal capsule
AKA most common type of sequelae is impaired motor function
(S. Kralik, NeoReviewsPlus 2018)
Name two longterm sequelae of cleft palate even after repair
- speech difficulties (due to velopharyngeal issues)
- hearing loss 2-2 recurrent otitis media
- severe reflux (due to velopharyngeal insufficiency)
The timing of an audiologic evaluation if an infant fails initial screening
Three months
What clinical histories warrant a brainstem auditor response test (BAER) regardless of the results of their automated auditory brainstem response (ABR)?
Culture-proven sepsis
Exchange transfusion
Extracorporeal membran oxygenation (ECMO)
Any infant with a NICU stay of greater than ______ days must have their hearing screen be a ______
Five
Automated auditory brainstem response (ABR)
This is because the OAE will only test peripheral hearing, does not catch sensorineural hearing losses
What physiologic factors make premature infants at-risk for intraventricular hemorrhage (IVH)?
- Immature and highly vascular germinal matrices
- Inability to regulate cerebral blood flow
What two neurologic issues can complicate shoulder dystocia
Erb’s palsy (C5/C6)
Diaphragm paralysis: Phrenic nerve palsy (C4/C5)
C4/5 keep the diaphragm alive, damaging C5/6 cause a waiter’s tip
Label A-C
A. Caput (most common)
B. Cephalohematoma (restricted by periosteum so can’t cross midline)
C. Subgaleal (most dangerous)
What does “selective neuronal necrosis” mean and imply
Neuronal death in areas with highest energy needs, AKA thalamus/hypothalamus/basal ganglia
Most common pattern seen with HIE
Results in feeding difficulties, seizures, ataxia, pyramidal cerebral palsy, intellectual disability
In what type of cerebral palsy do you see mixed tone within the same muscle/extremity
Athetoid AKA extrapyramidal
(Speech and hearing deficits common)
What is the most common type of stridor and its [most common] cause
Biphasic
Laryngomalacia
Remember it’s much easier to be floppy beause there’s no cartilagenous support
Both myotonic dystrophy and spinal muscular atrophy cause profound hypotonia; name two exam findings to help differentiate them
Tongue fasciculations: SMA
Ptosis/fish mouth: Myotonic dystrophy
The classic cause of a fontanelle bruit
Vein of Galen malformation
Arteriovenous malformation near posterior fossa, “typically” presents with congestive heart failure and respiratory distress
The gestational age when pupillary light reflex is present
Variably present at 30 weeks, DEFINITELY by 32
Think of how you wouldn’t want to test before then anyway because it’s the age threshold of IVH risk
The most commonly affected vessel in neonatal ischemia
LEFT middle cerebral artery
3x more likely to be affected
Gestationally, what are the first and last primitive reflexes to develop
Grasp: 28 weeks (You need to be able to hold on before anything else, think of all those preemies holding those knit octopii)
Pacing/stepping: 37 weeks (You’re not ready to STEP out of that woman until term)
Most common cause of asymmetric crying face (in absence of traumatic history)
Hypoplasia of the depressor anguli oris OR depressor labii inferiorus
(More commonly on left, associated with CHD)
If there is a left facial asymmetry with lack of movement of the eyebrow/eyelid, what is the cause
Trauma/CN VII temporal compression BEFORE branches diverge
In ex-preemies what is the “classic cause” of a weak cry, inspiratory stridor and difficulties feeding with signs of microaspiration?
Unilateral vocal cord paralysis from recurrent laryneal nerve damage from PDA ligation
50% resolve
Diagnosis: absent/dull pupillary reflex, frequent tearing, conjunctival injection and photophobia
Glaucoma
Also see corneal clouding, buphophthalmos (enlarged globe)
Two potential eye abnormalities with congenital Rubella
Cataracts
Glaucoma
CMV and Toxoplasmosis: chorioretinitis, NOT cataracts
Frequent eye finding in Sturge-Weber
Glaucoma
- 30-70% in those with SWS*
- When thinking glaucoma, also think Stickler and Neurofibromatosis*
Timing of the first ROP (retinopathy of prematurity) exam
4-6 weeks old OR 32 weeks PMA, whichever comes LATER
A normal EEG pattern’s expected to be discontinuous up until what GA/PMA
Through 30 weeks
Starting at 31-33 weeks, start to see continuous activity but still those “delta brushes” (Brodsky Review)
Two syndromes commonly associated with bitemporal wasting
Prader-Willi and myotonic dystrophy
Both with hypotonia, but in PWS also sleepy with low tone and often undescended testes (if male); in MD also “mask-like facies”
The most common sequelae of ROP (retinopathy of prematurity)
Myopia (near-sightedness) and strabismus
60% and 30% respectively with “severe” ROP (slightly different “severe” definitions ETROP vs CRYO-ROP)
Most common cause of congenital hearing loss
Genetic, specifically Connexin mutation
50% of congenital hearing loss hereditary/genetic
General sensitivity of newborn hearing screen at detecting congenital hearing loss
90%; definition of abnormal screen is 35dB or greater (normal for adults is 15 dB across frequencies)
(Brodsky Q&A Review)
A persistent _________ reflex beyond _______ weeks PMA is concerning for athetoid cerebral palsy
Palmar reflex beyond 4 months
Palmar reflex is first primitive to show (28 weeks) and first to leave
What is “threshold” ROP
Threshold ROP is when 50% change of retinal detachment if left untreated
With ETROP study, push to treat “pre-threshold” severe ROP since still confers high-risk of vision changes
