Neurology, Developmental and ENT

Question 1

Three chemoreceptors important for neuronal growth, migration and differentation

Answer 1

NMDA, opioid, and GABA

(NMDA and GABA influenced by alcohol, GABA by benzos, NMDA/glutamate and GABA by anesthesia)

Question 2

Associations in Dandy Walker

Answer 2

80% develop hydrocephalus

40% other CNS abnormalities: absent corpus callosum, posterior encephalocele, polymicrogyria

Question 3

If Dandy Walker is syndromic, what syndrome is associated?

Answer 3

PHACES (posterior fossa abnormality, hemangiomas, arterial lesions [HEENT], cardiac, eye, sternal)

Question 4

What is a Dandy Walker malformation?

Answer 4

Aplastic vermis (connection between cerebellar hemispheres)

Large posterior fossa (fourth ventricle not separated from cisterna magna)

Abnormally high tentorium

Question 5

What is considered the “prolonged” anesthesia exposure that may impact neurodevelopmental outcomes in patients <3 years old?

Answer 5

>3 hours

Believed due to the inhibition of glutamate (specifically NMDA glutamate) and GABA receptors, leading to apoptosis

Question 6

Therapeutic hypothermia decreases the incidence of death/disability in asphyxiated infants with moderate encephalopathy from ___ to ___%

Answer 6

50 to 30% (approximately)

Question 7

Two most sensitive imaging modalities to estimate injury from hypoxic-ischemic encephalopathy (aka perinatal asphyxia) and their ideal timing

Answer 7

DWI MRI (diffusion-weighted imaging)

MR-spectroscopy

3-5 days from injury

Question 8

Three most vulnerable/common areas of hypoxic brain injury

Answer 8

1. Basal ganglia
2. Thalamus
3. Internal capsule

AKA most common type of sequelae is impaired motor function

(S. Kralik, NeoReviewsPlus 2018)

Question 9

Name two longterm sequelae of cleft palate even after repair

Answer 9

1. speech difficulties (due to velopharyngeal issues)
2. hearing loss 2-2 recurrent otitis media
3. severe reflux (due to velopharyngeal insufficiency)

Question 10

The timing of an audiologic evaluation if an infant fails initial screening

Answer 10

Three months

Question 11

What clinical histories warrant a brainstem auditor response test (BAER) regardless of the results of their automated auditory brainstem response (ABR)?

Answer 11

Culture-proven sepsis

Exchange transfusion

Extracorporeal membran oxygenation (ECMO)

Question 12

Any infant with a NICU stay of greater than ______ days must have their hearing screen be a ______

Answer 12

Five

Automated auditory brainstem response (ABR)

This is because the OAE will only test peripheral hearing, does not catch sensorineural hearing losses

Question 13

What physiologic factors make premature infants at-risk for intraventricular hemorrhage (IVH)?

Answer 13

1. Immature and highly vascular germinal matrices
2. Inability to regulate cerebral blood flow

Question 14

What two neurologic issues can complicate shoulder dystocia

Answer 14

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

Question 15

Label A-C

Answer 15

A. Caput (most common)

B. Cephalohematoma (restricted by periosteum so can’t cross midline)

C. Subgaleal (most dangerous)

Question 16

What does “selective neuronal necrosis” mean and imply

Answer 16

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

Question 17

In what type of cerebral palsy do you see mixed tone within the same muscle/extremity

Answer 17

Athetoid AKA extrapyramidal

(Speech and hearing deficits common)

Question 18

What is the most common type of stridor and its [most common] cause

Answer 18

Biphasic

Laryngomalacia

Remember it’s much easier to be floppy beause there’s no cartilagenous support

Question 19

Both myotonic dystrophy and spinal muscular atrophy cause profound hypotonia; name two exam findings to help differentiate them

Answer 19

Tongue fasciculations: SMA

Ptosis/fish mouth: Myotonic dystrophy

Question 20

The classic cause of a fontanelle bruit

Answer 20

Vein of Galen malformation

Arteriovenous malformation near posterior fossa, “typically” presents with congestive heart failure and respiratory distress

Question 21

The gestational age when pupillary light reflex is present

Answer 21

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

Question 22

The most commonly affected vessel in neonatal ischemia

Answer 22

LEFT middle cerebral artery

3x more likely to be affected

Question 23

Gestationally, what are the first and last primitive reflexes to develop

Answer 23

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)

Question 24

Most common cause of asymmetric crying face (in absence of traumatic history)

Answer 24

Hypoplasia of the depressor anguli oris OR depressor labii inferiorus

(More commonly on left, associated with CHD)

Question 25

If there is a left facial asymmetry with lack of movement of the eyebrow/eyelid, what is the cause

Answer 25

Trauma/CN VII temporal compression BEFORE branches diverge

Question 26

In ex-preemies what is the “classic cause” of a weak cry, inspiratory stridor and difficulties feeding with signs of microaspiration?

Answer 26

Unilateral vocal cord paralysis from recurrent laryneal nerve damage from PDA ligation

50% resolve

Question 27

Diagnosis: absent/dull pupillary reflex, frequent tearing, conjunctival injection and photophobia

Answer 27

Glaucoma

Also see corneal clouding, buphophthalmos (enlarged globe)

Question 28

Two potential eye abnormalities with congenital Rubella

Answer 28

Cataracts

Glaucoma

CMV and Toxoplasmosis: chorioretinitis, NOT cataracts

Question 29

Frequent eye finding in Sturge-Weber

Answer 29

Glaucoma

• 30-70% in those with SWS*
• When thinking glaucoma, also think Stickler and Neurofibromatosis*

Question 30

Timing of the first ROP (retinopathy of prematurity) exam

Answer 30

4-6 weeks old OR 32 weeks PMA, whichever comes LATER

Question 31

A normal EEG pattern’s expected to be discontinuous up until what GA/PMA

Answer 31

Through 30 weeks

Starting at 31-33 weeks, start to see continuous activity but still those “delta brushes” (Brodsky Review)

Question 32

Two syndromes commonly associated with bitemporal wasting

Answer 32

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”

Question 33

The most common sequelae of ROP (retinopathy of prematurity)

Answer 33

Myopia (near-sightedness) and strabismus

60% and 30% respectively with “severe” ROP (slightly different “severe” definitions ETROP vs CRYO-ROP)

Question 34

Most common cause of congenital hearing loss

Answer 34

Genetic, specifically Connexin mutation

50% of congenital hearing loss hereditary/genetic

Question 35

General sensitivity of newborn hearing screen at detecting congenital hearing loss

Answer 35

90%; definition of abnormal screen is 35dB or greater (normal for adults is 15 dB across frequencies)

(Brodsky Q&A Review)

Question 36

A persistent _________ reflex beyond _______ weeks PMA is concerning for athetoid cerebral palsy

Answer 36

Palmar reflex beyond 4 months

Palmar reflex is first primitive to show (28 weeks) and first to leave

Question 37

What is “threshold” ROP

Answer 37

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