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NICU Board Review > Neuro > Flashcards

Neuro Flashcards

1
Q

What decreases CBF (cerebral blood flow)?

A

Decreased PCO2
Increase paO2
Increase Hb [ ]
Decreased fetal Hb

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2
Q

Muscle biopsy findings with
1. Myotubular myopathy
2. Myotonic dystrophy
3. SMA
4. Muscular dystrophy

A
  1. Large fibrils, centrally placed nuclei
  2. Small fibers, centrally placed nuclei
  3. Hypotrophy fascicles with hypertrophic fascicles
  4. Replaced by fat, connective tissue and peri placed nuclei
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3
Q

What are U/S findings with Meningomyelocele?

A

Lemon 🍋 sign - compression due to CSF leak
Banana 🍌 sign - compressed cerebellum

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4
Q

Mother with difficult labor
Polyhydramnios
Baby with respiratory failure and tent shaped mouth, and hypotonia

A

Myotonic Dystrophy

Polyhydramnios; difficulties swallowing
Dysfunctional Na /K channel
AD; chromosome 19 (DMPK gene)
CTG repeats

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5
Q

Baby with decreased fetal movements, hypotonia, bell shaped chest on CXR, frog leg position

Dx?
Test?

A

SMA

EMG - Abnormal activity with fibrillation and fasiculations

Bulbar weakness (poor suck/swallow, weak cry, tongue fasiculations)
Marked head lag
Facial sparing
Absent deep tendon réflexes (aréflexia)
Normal diaphragmatic function

AR, chromosome 5

Degeneration of anterior horn cell

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6
Q

Earliest imaging modality to detect HIE

A

MR spectroscopy
Can detect inc in brain lactate concentrations as soon as 2-8 hrs after injury

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7
Q

Neural tube defects have highest rate among which population?

A

Hispanic patients

Teenage

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8
Q

Which is most effective intervention to decrease risk of IVH?

A

Prenatal steroids

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9
Q

Neonates with transient neonatal myesthenia gravis present most commonly with?

A

3hrs-24hrs: Poor suck and swallow, weak cry, resp distress and ptosis/ophthalmoparesis

Abs against the Nicotinic Acetalcholine receptor 80% or muscle specific receptor tyrosine kinase 20%

Atypical presentation can be arthogryposis multiplex congenita

Majority need neostigmine
Duration of illness ~18 days

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10
Q

Hyperthermia following HIE leads to neuronal cell death in which region?

A

Hippocampus

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11
Q

Which type of CP do you expect in severe HIE?

A

Pyramidal CP

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12
Q

Cerebral tissue hypoxia results in reduction of?

A

ATP and Phosphocreatinine->influx of intracellular calcium

Protease activation leads to cytoskeletal disruption

Protease and phospholipase activation leads to apoptotic Pathways

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13
Q

Cellular mechanism of HIE

A

Cerebral tissue hypoxia results in decreased production of ATP and phosphocreatine
which leads to cell membrane depolarization and influx of intracellular calcium.

This influx in intercellular
Ca+ results in the activation of multiple downstream pathways including~
Endonuclease activation that results in nuclear injury
Protease activation leading to cytoskeleton disruption
Protease and phospholipase activation that results in free radical production and activation of the
apoptotic pathways
Nitric oxide synthase activation
An increase in excitatory neurotransmitter (glutamate) release

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14
Q

Most common risk factors for perinatal brachial plexus palsy?

A

Shoulder dystocia (45%)

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15
Q

Newborn with dolichostenomelia, High arched palate, mitral valve prolapse and joint contractures (fingers, Elbows and knees). Dx?

A

Congenital contractural arachnodactyly AKA Beals syndrome

Mutation in fibrillin gene-2 on chromosome 5

Think marfans with contractures

Marfans syndrome, dolichostenomelia (long extremities), aranchmodactyly, joint contractures fingers elbows knees, MVP

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16
Q

Describe DANDY walker malformation

A

Agenesis of cerebellar vermis, cystic dilation of 4th ventricle and enlargement of posterior fossa

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17
Q

Describe Arnold chiari malformation II

A

Present in childhood/adulthood
Displacement of cerebellar tonsils below foramen magna
4th ventricle and brainstem normal
Syringo or hydromelia
Ass w scoliosis

Type 2 p/w infants
Elongation and caudal displacement of cerebellar tonsils, 4th ventricle choroid plexus
Ass w/Hydrocephalus

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18
Q

What syndromes present w agenesis of corpus callosum?

A

Neuro-dandy walker, holoprosencephaly
Metabolite non ketotic hyperglycinemia, pyruvate dehydrogenase deficiency
T8, 13,18
Aicardi syndrome-infantile spasm, Coloboma hypotonia
Zellweger-peroxisomal disorder

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19
Q

A GMFCS of 5 indicates a higher or lower likelihood of severe limitations in CP?

A

Higher (5)
lower (1)

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20
Q

MC Type of CP in bilirubin encephalopathy

A

Athetoid (increased activity with reduced tone)

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21
Q

Most common CP in preterm neonates is ….

A

Spastic diplegia

Cognitive function is not usually affected (unless quadriplegia)

Fine motor usually not affected
(Is affected in dyskinetic - AKA extrapyramidal -
- dystonic: reduced activity with increased tone or -choreoathetotic : increased activity with decreased tone)

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22
Q

Prenatal repair of myelomeningocele is associated with

A

Risk of sx: maternal morbidity, preterm birth, increased Uro complications

Stopped early because of a reduced need for CSF shunt in prenatal intervention group by 12 months

Decrease in fetal/neonatal death

Improved mental/motor function at 30 months

Sx recommended in: Singleton pregnancy, Lesion at spinal level T1-S1, normal karyotype and Arnold Chiari type 2

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23
Q

Describe aEEG findings in normal vs burst suppression vS seizures.

A

Continuous amplitude 10-25mcV (appears like a fuzzy caterpillar)

Discontinuous-wide band w min amplitude below 5 and max>10

Burst suppression-discontinuous w min amplitude 0-1mcV with burst>25 (<100 bursts/hr negative burst suppression, >100 bursts/hr positive burst suppression)

Low voltage- continuous pattern around/below 5

Inactive iso electric, low background amplitude<5

Seizures- saw tooth pattern, inc min and max amplitudes

Abnormal aEEG at 48 hrs predictive of NDI and neg aEEG at 6hrs predictive of No NDI

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24
Q

What is the mechanism of action of hypothermia In decreasing hypoxic injury?

A

Decrease intracellular Ca influx and mitochondrial dysfunction
Decrease free radical production
Reduced STAT 3 expression
Enhance RBM3 protein expression
Decrease in metabolic demands

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25
Q

What are some short term Neuro outcomes from morphine use in the NICU?

A

Longer time to achieve full volume feeds

Longer # of days on ventilator

Strong predictor of severe NEC

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26
Q

Name biomarkers to predict HIE injury in MRI

A

Elevated IL 6 in neonate but not mother correlates w NDO at 2 years
Glial fibrillation acidic protein (GFAP) elevated after HIE
Brain derived neurotrophic factor (BDNF) high in cord plasma
Protein S100beta elevated in cord blood

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27
Q

What are long term outcomes for SSRI- exposed children ?

A

ADHD

28
Q

Term infant
Ashkenazi Jewish family Hypotonia
Autonomomic issues (loose, irritability, Pale blotchy skin)

(think NAS with hypotonia)

A

Riley - Day syndrome (Familial dysautonomia)

AR
Chromosome 9

Dx- pupil construction in response to metacholine eye drops

29
Q

Describe CN and function

A

I- Olfaction
II- Vision, optic fundi
III- Pupils
III, IV, VI-Extraocular movements
V-Facial sensation, masticatory power
V1: ophthalmic
V2:maxillary
V3: mandibular
VII- Facial movements
VIII-Auditory
V, VI, IX, X, XII-Sucking and swallowing
XI-Sternocleidomastoid function
XII-Tongue
VIII, IX- Taste

30
Q

Pt with expressionless face. What’s the syndrome?

A

Mobeus syndrome

Secondary to hypoplasia or absence of CN nuclei

No treatment or recovery

31
Q

Which EEG pattern is seen in PVL?

A

Central positive sharp waves

32
Q

What is the diagnostic test for neonatal myesthenia gravis?

A

Anti-acetylcholine receptor antibody assay

Anti-AChr 85% (nicotinic receptor)

Anti-Muskr 15% (muscle specific receptor tyrosine kinase)

33
Q

Which is the primary cellular target in PVL?

A

Oligodendrocyte progenitor cell

34
Q

Brachial plexus injury associated with Horners

A

Klumpke (T1)

35
Q

When do u expect full recovery of brachial plexus injuries?

A

6 months
@2 mo you see improvement

If residual impairment at 15 months, usually persists

36
Q

Brachial plexus injury with Phrenic nerve paralysis

A

Erb (C4-C5)

37
Q

Describe the presentation triad of Aicardi syndrome

A

Agenesis of corpus callosum
Infantile spasms
Chorio retinal lacunae

Autosomal dominant 46XXY

38
Q

Term female with focal clonic seizure at 5 days of life confirmed on EEG. No fhx of seizure. What type of seizure is she exhibiting?

A

Benign neonatal seizure aka “fifth day fits”

39
Q

Term newborn with hypotonia, arthrogryposis multiplex congenita, and dilated cardiomyopathy. Muscle biopsy with gomori trichrome staining with rodlike bodies. Dx?

A

Nemaline myopathy (remember Nemaline bodies)
Absent DTR and severe muscle weakness affecting face/proximal limbs

40
Q

The location of white matter injury in preterm neonates is specific to which area? And why?

A

Periventricular area
Distribution of oligodendrocyte progenitors

41
Q

Which of the following interventions is most likely to prevent IVH?

  1. Antenatal magnesium
  2. Antenatal Vit K
  3. Delayed cord clamping 60 sec
  4. Indomethacin treatment within 24 h of delivery
A

Indomethacin treatment within 24 h of delivery

** While there appears to be a benefit of delayed cord clamping for premature infants, the most recent RCT and subsequent analysis does NOT show clear benefit in reducing the risk for IVH

42
Q

What is the most common congenital posterior fossa malformation?

A

Dandy Walker Malformation

  1. Agenesis or hypoplasia of the cerebellar vermis
  2. Cystic dilation of the 4th ventricle
  3. Enlargement of the posterior fossa
  • Associated with hydrocephalus
  • Increased risk of motor and cognitive delays



43
Q

Most common hemisphere to be affected with term focal or multi focal ischemia? Which artery MC involved?

A

Left hemisphere
Left MCA

44
Q

How does Periventricular hemorrhagic infarction occur?

A

Hemorrhagic necrosis of PV white matter related to IVH. IVH obstructs blood flow in terminal vein leading to venous infarction.

Peak ~4 days of life

45
Q

Risk factors predispose premies to PVL

A
  1. Periventricular vascular anatomic factors
  2. Cerebral perfusion pressure depends on systemic BP
  3. Increased vulnerability periventricular glial cells
  4. Insult leading to oligodendroglia cell death and myelin deficiency
46
Q

Pattern of hypoglycemic injury on MRI?

A

Related to duration rather than severity of hypoglycemia

Involves bilateral posterior occipital cortex. Middle cerebral infarction and basal ganglia/thalamus abnormalities

47
Q

How does EPO play a role in HIE?

A

Prevents NMDA receptor activation 2/2 hypoxia ischemia
Decreases excitotoxicity, apoptosis, cytokines
Decreases peroxinitrite production and oxidative stress

48
Q

The effect of hypoglycemia on MRI

A

Corticospinal tract injury

  • basal ganglia and thalamus abnormalities
  • not how low - import how long (duration)?
49
Q

Neonates with congenital myotonic dystrophy at highest risk for?

A

GI dysfunction
Resp dysfunction/hypotonia
Endocrinopathies-hypothyroidism and hyperparathyroid

Adults risk for fatal arrhythmias

50
Q

Hypotonic, arreflexic neonate with resp distress, nerve biopsy shows decrease in myelin density with onion bulb formation. Dx?

A

Dejerine Sottas disease

Can also have arthrogryposis and foot deformity, decrease fetal movement IUGR

Marked decreased nerve conduction velocity <12m/sec

Nerve biopsy differentiates from congenital hypomyelinating neuropathy which has absence of myelin fibers

51
Q

MCC of CP in term neonates

A

HIE

(1-2/1000 term births)

52
Q

What is the NNT for therapeutic hypothermia with HIE?

A

NNT ~6

53
Q

Describe MRI findings of HIE

A

• Basal Ganglia and Thalami (BGT)
• Particularly susceptible to injury in acute, profound HIE
• Serve as a “relay station” for the brain
• Posterior Limb of the Interior Capsule (PLIC)
• Dense motor pathway running through the BGT
Extremely age-dependent
• Brainstem
• Involved more often in preterms or severe injury

• Cortex and White Matter
May be solely affected in milder HIE
“Watershed” pattern in partial/prolonged HIE

54
Q

Reasons to MRI with HIE

A

•Clarify timing/etiology
•Confirm diagnosis
•Aide in prognostication
•Early MRI
•Ideally day 5-21 after injury in term babies
•Diffusion changes present 7-10 days, then
“pseudonormalise”
•Day 8-30 MRI has higher sensitivity for poor outcome,
but lower specificity than day 1-7
•Lactate/NAA on MR Spectroscopy is 91-94% sensitive

55
Q

MRI Patterns in HIE

A

• Severity of BGT injury is most predictive of severity of motor impairment.
• Mild = 11% risk of CP, usually mild
• Moderate = 69% risk of CP
• Severe = 98% with CP, usually severe

• Injury to the PLIC most predictive of
walking at age 2.
• Normal = all walk by 2 years
• Equivocal = 67% walk, but after 18 mos
Abnormal = 12% walk

56
Q

Which is the most common presentation of Arterial ischemic stroke?

A

• 1:3000 term births
• Nonspecific Presentation
• Seizures most common presenting sign (>75%)
•
~20% of neonates with seizure have stroke
• Lethargy
• Vital sign fluctuation
• Poor feeding
• NOT hemiplegia
Multiple risk factors in setting of transient hypercoagulability of newborn

Thrombophilia evaluation may not be needed in AIS (doesn’t predict who will have recurrence)

MC difficulties motor deficits
1/4 have epilepsy

57
Q

What are the proposed mechanisms for IVH?

A

Fragile vessels in germinal matrix
Clinical instability
Fluctuating CBF
-Increases CBF with Impaired auto regulation (bolus, hypercarbia, anemia/hypoglycemia)
-Decrease CBF from hypotension

58
Q

How does Grade IV IVH develop?

A

GM hemorrhage leads to periventricular venous congestion.

This leads to PV ischemia which can lead to periventricular infarction

59
Q

Antenatal strategies to prevent IVH?

A

Betamethasone
Magnesium sulfate
Maternal transfer to center who routinely care for VLBW/ELBW

60
Q

EEG findings of burst suppression

A

Ohtaharra syndrome
Non ketotic hyperglycinemia

61
Q

Complication of endoscopic third ventriculostomy with choroid plexus ablation in infant with hydrocephalus?

A

Diabetes insipidus
Likely related to hypothalamus injury

Tx: water replacement, ADH

62
Q

B/l vocal cord paralysis is associated with which conditon?

A

Arnold Chiari II

63
Q

Which strategies have decreased the risk of IVH?

A

Antenatal corticosteroids
Delayed cord clamping

64
Q

Cranial nerves involved in gag reflex

A

9 and 10

65
Q

MOA of benzodiazepines

A

Enhances GABA action–benzo binds GABA-receptor

GABA is inhibitory normally, but in preemies is actually excitatory

66
Q

Describe anomalies in goldenhar syndrome

A

Oculoauriculo vertebral syndrome

Ear Tag on tragus

67
Q

Name associated findings with Meckel Gruber syndrome?

A

Occipital encephalocele
Polydactyl
Cystic dysplastic kidneys
Leads to ciliary dysfunction

AR

NICU Board Review (19 decks)

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