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My Neo Board Quickfacts > Neuro/Optho > Flashcards

Neuro/Optho Flashcards

1
Q

Recovery time for facial nerve palsy seen at birth

A

1-3 weeks for complete recovery

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2
Q

symptoms of congenital glaucoma + timing of presentation

A

usually in 1st six months of life

tearing
photophobia
enlarged globe (buphopthalmos)
corneal edema and clouding (irregular corral reflex, and dull red reflex)
visual loss
conjunctival injection

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3
Q

Syndromes a/w congenital glaucoma

A

Sturge-Weber (glaucoma in 30-70%; 45% if port wine stain includes the forehead, eye, and msxilllary area)

neurofibromatosis
retinoblastoma
homocystinuria
Tri21
congenital rubella
Stickler syndrome
long-term exposure to corticosteroids

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4
Q

Most common infectious cause of congenital cataracts

A

Rubella

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5
Q

ROP screening (timing and who)

A

infants < 30wks or BW </= 1500g (or severe instability)

examine at 4 wks post-natal age or at 31 wks, whichever is LATER

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6
Q

ROP prevalence

A

20-50% with a BW <1500g; worst for smallest and lowest gestations

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7
Q

most common outcome in cystic PVL

A

spastic diplegia

damages deeper, more medial fiber tracts that control lower extremity function

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8
Q

Language development timeline

A

1-6 months: cooing (vowel sounds)
(4-6 months: vocal play; “expansion stage”, constants+long vowels)
5-7 months: babble (p, b, m sounds)
(8-10 months more complex babble)
10 months: jargon (intimated babble)
15-18 months: echolalia

*lack of babbling by 11 months - prompt audio eval

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9
Q

cochlear implants

A

directly stimulate auditory nerve endings at basal membrane of the cochlea

For:
> 85 dB hearing impairment
have not benefited from several months of amplification and intense speech therapy
at least 12 months of age

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10
Q

normal EEG background of extremely preterm infant

A

discontinuous
(burst + low amplitude “inter burst interval”)

*vs “abnormal burst suppression” w/ periods of flat/inactive areas without activity

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11
Q

EEG changes with maturation

A

EEG background becomes more continuous with:
- shorter inter-burst intervals
- longer duration of bursts
- higher amplitude during the low-amplitude activity

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12
Q

Pupillary reflex tests what CN

A

CN III (tests both afferent and efferent)

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13
Q

GA when pupillary response observed

A

30-32 weeks

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14
Q

conjugate horizontal age (GA)

A

Term

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15
Q

visual fixation
(GA when observed and when well developed)

A

Seen at term gestation; well developed at age 2 mo

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16
Q

Conjugate vertical gaze (age)

A

2 mo

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17
Q

Visual following is well developed (age)

A

3 mo

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18
Q

visual evoked potential reaches adult level (age)

A

6 mo

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19
Q

optic nerve myelination complete (age)

A

24 mo

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20
Q

_____(ocular symptom) has been a/w:
13q deletion
Fanconi pancytopenia
FAS
Mobius sequence
Noonan
Smith-Lemli-Opitz
WAGR

A

Ptosis

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21
Q

Process resulting in cataracts

A

any process that alters the glycolytic pathway or epithelial cell mites of the avascular lens

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22
Q

Most common form of brain injury in preterm infants

(and the major cause of cognitive deficits and long-term neurodevelpmental impairment in this population)

A

DIFFUSE periventricular leukomalacia

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23
Q

Most common cause of congenital hearing loss

A

genetic (50%)

mutation in connexion gene (20-30%)

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24
Q

Threshold for abnormal hearing screening

A

35db or greater

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25
Q

% of neonates with congenital hearing loss detected in newborn period

A

90%

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26
Q

Percent breakdown of hearing loss causes

A

50% genetic (MCC connexin)
25% intrapartum/perinatal injury
25% unknown

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27
Q

Persistence of this reflex past ~4 months concerning for athetoid CP

A

palmar grasp

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28
Q

% reduction in IVH from corticosteroids

A

50%

*corticosteroids are the only antenatal strategy that have demonstrated statistically significant reduction in IVH

Note - transfer of mother prior to preterm birth has also been shown to reduce incidence of IVH vs transfer of infant after birth

Reduction in preterm birth would also reduce IVH

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29
Q

Three stages of kernicterus

A

Early
- lethargic
- hypOtonic
- poor suck
- high pitched cry

Intermediate
- irritable
- hypERtonic
- opisthotonus
- shrill cry

Advanced
- irreversible neurologic damage
- severe hypERtonia
- deep stupor/possible coma
- seizures
- possible death

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30
Q

Sequelae of kernicterus

A
  • movement issues (tone abnormalities, athetosis)
  • gaze abnormalities (esp paralysis of upward gaze)
  • auditory problems
  • dental-enamel dysplasia

*NO cognitive issues

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31
Q

Threshold ROP

A

Stage 3 w/ PLUS in zone I or II
+ 5 contiguous clock hours
or
8 total clock hours

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32
Q

When to treat ROP

A
  • Threshold (stage 3, plus in zone I or II, 5 contig or 8 total clock hours)
  • Type 1 prethreshold:
    • zone 1: any ROP + plus or stage 3 (+/- plus)
    • zone 2: stage 2 or 3 + plus
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33
Q

Congenital glaucoma genetics

A

Autosomal RECessive (usually)
males > females

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34
Q

Signs of congenital glaucoma

A

excessive tearing
corneal cloudiness (2/2 corneal edema)
dampened pupillary light reflex
photophobia
buphthalmos (enlargement of the globe)
eye rubbing

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35
Q

Risk factors of IVH

A

lower gestational age
male
surfactant deficiency
hyPERcapnia
pneumothorax
fluctuating arterial pressure
early hypotension

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36
Q

Evolution of Gr IV IVH

A

hematoma in the germinal matrix
–> occludes the venous drainage of the terminal vein
—> infarction and secondary bleeding

(veins of the periventricular white matter drain into the terminal vein, which runs through the germinal matrix)

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37
Q

pathogenesis of PVL

A

ischemia in “water shed” areas of the PVWM; hypoxia and ischemia alter the development of oligodendrocyte progenitor cells that are especially susceptible to free radical injury

*does NOT result from IVH extension/white matter compression

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38
Q

diffuse PVL characteristics on MRI

A

high-signal intensity in the white matter on T2 weighted MRI images

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39
Q

Cystic PVL evident on US at what age

A

2-4 wks

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40
Q

Diagnosis and treatment for abrupt onset of excessive purulent yellow eye discharge

A

gonorrheal infection

tx: IV/IM cephalosporin

*prompt treatment to avoid progression to corneal ulceration

*prevent with ppx

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41
Q

Onset of gonorrheal conjuctivitis

A

2-5 days

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42
Q

Onset of chlamydial conjuctivitis

A

5-14 days

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43
Q

Onset of HSV conjuctivitis

A

4d - 3 wks
(broad range)

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44
Q

Decreasing gestational age, MAP approaches _______ limit of auto regulation plateau

A

lower

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45
Q

Cerebral auto regulation in response to PaO2

A

decrease in PaO2 can blunt auto regulation response

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46
Q

Cerebral auto regulation in response to PaCO2

A

*most sensitive to changes in PaCO2

hypercarbia/acidosis –> increased blood flow to the brain

47
Q

Number of beats of clonus at the ankle that is normal in newborns and for how long

A

5-10 beats up to 3 months of age

48
Q

Cross-aductor response w/ knee jerk reflex is normal up to what age

A

8 months

49
Q

HIE EEG

A

depression or burst suppression

50
Q

HIE - areas of the brain injured

A

Cortical
Deep gray nuclei

(white > gray)

51
Q

Mobius syndrome aka__________

caused by problems in the________

A

Facial diplegia syndrome

Caused by problems in the:
cranial nerve nuclei, roots, nerves, or muscles themselves

*absence or underdevelopment of the 6th and 7th cranial nerves

52
Q

SMA type 1 = degeneration of ________
(aka _________)

A

degeneration of anterior horn cell

aka Werdnig-Hoffman

53
Q

Gestational age when hearing screen can be used (both OAE and ABR)

A

34 wks

54
Q

Definition of learning disability (2)

A
  • scoring >/= 1 SD below the mean on standardized achievement tests
  • having a 1 SD or greater discrepancy between IQ and achievement (measured by psychometric testing)
55
Q

Most common type of learning disability

A

verbal

56
Q

Low BW has disproportionate risk of what type of learning disability

A

non-verbal, math

(but overall verbal is most common type of learning disability in the US is verbal)

57
Q

Risk of CP in </= 26 weeks

A

16-28%

58
Q

EI associated with increase ______

A

a/w increase in IQs in preterm, but this does NOT correlate with improved academic achievement

59
Q

Differences in long term outcomes in extremely preterm neonates have been associated with the following characteristics

A

sex
BW
exposure to antenatal corticosteroids

60
Q

Most common nerve to be injured during birth (including side)

A

Facial nerve (CN VII) - left much more common

(incidence < 1%)
caused by nerve compression with hemorrhage and edema of the nerve sheath

61
Q

Timeline for cataracts in galactosemia

A

~ 2 weeks

(excess galactitol in lens)

62
Q

Anencephaly

Type of abnormal developmental process (generally)

A

primary neurulation (dorsal induction)

(3-4 wks gestation)

63
Q

Lissencephaly (what and when)

2 types

A

d/o of neuronal migration
during 12th-24th week
(often diagnosed after 26-28wks)
smooth cerebral cortex

a/w microcephaly, ventriculomegaly, widened Sylvian fissures
complete or partial agenesis of corpus callosum

Type 1 a/w facial dysmorphism

Type 2 a/w:
- Walker-Warburg - cerebrospinal-ocular dysplasia, hydrocephalus, cerebral malformations
- Miller-Dierker - facial dysmorphism, growth restriction,

64
Q

Agenesis of the corpus callosum

Type of abnormal developmental process (generally)

A

prosencephalic development (ventral induction)

(8-12 weeks gestation)

65
Q

Anencephaly

Type of abnormal developmental process (generally)

A

primary neurulation (dorsal induction)

(3-4 wks gestation)

66
Q

Angellman

Type of abnormal neuro developmental process (generally)

A

neuronal organization

(12 wks gestation to years)

67
Q

Arnold Chiari

Type of abnormal developmental process (generally)

A

primary neurulation

(3-4 wks gestation)

68
Q

Autism

Type of abnormal neuro developmental process (generally)

A

neuronal organization

(12wks gestation to years of age)

69
Q

Fragile X

Type of abnormal neuro neudevelopmental process (generally)

A

Neuronal organization

(beginning 12 weeks gestation through childhood)

70
Q

Holoprosencephaly

Type of abnormal developmental process (generally)

A

prosencephalic development (ventral induction)

8-12 wks gestation

71
Q

lissencephaly

Type of abnormal developmental process (generally)

A

neuronal migration

12-20 weeks gestation

72
Q

myelomeningocele

Type of abnormal developmental process (generally)

A

primary neurulation

(2-4 weeks gestation)

73
Q

pachygyria

Type of abnormal developmental process (generally)

A

neuronal migration

12-20 weeks gestation

74
Q

polymicrogyria

Type of abnormal developmental process (generally)

A

neuronal migration

12-20 weeks gestation

75
Q

schizencephaly

Type of abnormal developmental process (generally)

A

neuronal migration

12-20 weeks gestation

76
Q

Tri 21

Type of abnormal neuro developmental process (generally)

A

neuronal organization

12 weeks gestation to years

77
Q

Tethered cord
Lipoma
meningocele
teratoma

Stage of abnormal neuro development (generally)

A

Secondary neurulation

(4-7 wks gestation)

78
Q

Stages of neuronal organization

A

12 wks gestation - birth: axonal outgrowth and proliferation

6 moths gestation - age 1 year: dendritic and synapse

birth to years: synaptic rearrangements

79
Q

MicrENcepahly
MacENcephaly

stage of abnormal neuro development

A

(small or large BRAIN)

neural and glial proliferation

12-16 wks gestation

80
Q

Stages of neuronal development a/w prematurity

A

neuronal organization
myelination

81
Q

Most common side of brachial plexus birth injury

A

Right

82
Q

Erb-Duchenne palsy nerves/symptoms

A

C5-C7 (more common)

“waiter’s tip”
adduction + internal rotation of the arm
extension of elbow
pronation of the forearm
flexed wrist
flexed fingers
*biceps refills generally absent
*palmer grasp intact

83
Q

Klumpke’s palsy nerves and presentation

A

C8-T1 (rare in isolation)

  • weakness of flexors of wrist and fingers
    (inability to flex the wrist)
  • finger abduction
  • flexion at IP joins + hyperextension at MCP
    (“claw hand”)
  • BOTH biceps and palmer grasp reflexes often absent
  • reduced sensation in C8 and T1 dermatomes (pinky/ring finger)
  • may be Horner’s syndrome if sympathetic fibers of T1 involved

a/w h/o hyperABduction of the shoulder during delivery

84
Q

Recovery timeline of brachial plexus injury

A

improvement by 2 weeks
recovery by 6 months

*impairment at 15 months is often permanent

85
Q

EMG in transient myasthenia gravis and congenital myasthenia gravis

A

progressive decline in amplitude with repetitive nerve stimulation

86
Q

Muscle biopsy in myotonic dystrophy

A

abnormal small and round muscle fibers with large nuclei and sparse myofibrils

87
Q

both types of hearing screens better at detecting ____ frequecies

A

better at deteting mid to high frequency
(not as good at low)

88
Q

Total CSF volume in infants

% CSF in ventricles

A

Total 50 ml CSF

25% in ventricles

89
Q

maturation stages of oligodendrocyte lineage;

*which are most vulnerable to inflammatory and ischemic injury

which are most abundant during peak incidence of PVL

A

neural stem cell
oligodendrocyte progenitor
pre-oligodendrocyte*
immature oligodendrocyte *
mature oligodendrocyte

pre-oligodendrocytes account for 90% of total oligodendrocyte population at 28 weeks

90
Q

Oligodendrocyte population at term

A

50% immature oligodendrocytes at term
(increased from 5-10% ~28wks)

91
Q

Incidence of abnormal neurologic outcome for HIE at 18 months:

mild
moderate (not cooled/cooled)
severe (not cooled/cooled)

A

mild: normal neuro outcomes as toddlers

moderate: 53% vs 36%

severe: 86% vs 70%

*abnormal = death or major neuro dev disability
(CP, blidness, deafness, Bayley/Grifith 2 SD below the mean, IQ 2 SD below the mean)

92
Q

HIE reduces risk of death or neurodevelopment impairment (combined outcome) among infants with moderate or severe HIE by approximately ______%

A

25 (%)

93
Q

Pathognomonic feature of :

benign sleep myoclonus

A

events only occur during sleep and cease on arousal

(may be unilateral or bilateral, can be single jerk or repetitive, does not extinguish with holding)

*most common condition misdiagnosed as neonatal seizures

94
Q

shortcomings of aEEG

A
  • can miss SHORT seizures due to TIME COMPRESSION
  • can miss FOCAL seizures due to FEWER CHANNELS
95
Q

MCC of neonatal seizure (list)

A
  • global cerebral hypoxic ischemia (40%)
  • focal cerebral hypoxic ischemia (e.g. stroke) and ICH (17-18%)
  • cerebral malformations (5-9%)
  • NAS, metabolic derrangements, hypoglycemia, hypOcalcemia, hypOmagnesemia (3-5% each)
  • epileptic syndromes
96
Q

Neonatal seizure:
- mortality rate ______
- ______ % of survivors will have disability

A

10% mortality rate

54% will have disability

97
Q

Gestational range with limbs extended at rest

A

28 weeks or younger

98
Q

Flexion begins where and at what gestational age

A

flexion begins distally (knee then hips)
starting at 32 weeks

99
Q

Gestation when flexion in all four extremeties

A

by 36 weeks (but not as promnouces as at term)

100
Q

Scarf sign at 28 weeks vs term

A

28 weeks - elbow adducted to opposite shoulder

40 weeks - elbow only reaches midline

101
Q

Moro (timeline)

A

37 weeks
disappears 3-6 months

102
Q

asymmetrical tonic neck reflex (ATNR)

A

well established by 1 month
disappears by 3-4 months

103
Q

palmer grasp timeline

A

32 weeks
disappears 3 months

104
Q

stepping reflex

A

32 weeks
disappears by 1-2 months

105
Q

IUGR preterm infant has additional risk for ______ compared to appropriately grown preterm infants

(neurodevelopmental)

A

IUGR lower full scale and verbal IQ scores

(similar rates of behavioral difficulties)

106
Q

Define:
impairment
disability
handicap

A

impairment - structural/functional abnormality at the ORGAN level

disability - restriction at the PERSONAL level (that results from impairment)

handicap - occurs at the SOCIETAL level, disadvanage as a result of a disability that prevents fulfillment of that individual’s usual role

107
Q

mortality rate in smbgaleal hemorrhage

A

12-14%

due to shock and coagulopathy

108
Q

What proportion of patients respond to 1st loading dose of phenobarbital

A

only 1/3 respond to initial 15-20/kg

109
Q

phenobarbital a/w what adverse process

A

neuronal apoptosis

110
Q

____% of cerebellar development occurs in the last trimester

Brain weight at 34 weeks is _____% of term brain

Cortical surface increases by _____% in the last trimester

Most ______ neurons migrate to the cortex in third trimester

A

25 % of cerebellar development occurs in the last trimester

Brain weight at 34 weeks is 65 % of term brain

Cortical surface increases by 50 % in the last trimester

Most gaba-nergic neurons migrate to the cortex in the third trimester

111
Q

Diagnosis of at least one lateral ventricle >10mm

A

ventriculomegaly

*NOT necessarily hydrocephalus

112
Q

Describe ophtho findings of corneal clouding 2/2 trauma

when does it resolve

possible sequelae

A

Descemet membrane tears (commonly vertically or obliquely oriented)
(basement membrane b/w corneal stroma and endothelial layer of the cornea)

resolves spontaneously in several months

astigmatism; amblyopia

113
Q

GA when fetus responds to sound

A

~20th week

corresponds w/ developments in the cochlea, inner and outer ear (develop in parallel)

My Neo Board Quickfacts (11 decks)

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