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Neontal boards J > Neurology > Flashcards

Neurology Flashcards

1
Q

What is Benign familial neonatal seizures

A
  • Buzzwords:
    • seizure activity with the strong family history of similar events in the neonatal period
  • Seizure character
    • tonic motor activity and posturing associated with apnea, followed by focal or generalized clonic movements.
    • 1 to 2 minutes but may occur up to 20 to 30 times per day.
  • autosomal dominant inheritance pattern,
    • mutations in the KCNQ2
  • begin on the second or third day after birth
  • Seizure activity resolves within the first 6 weeks.
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2
Q

What is benign idiopathic neonatal seizures

A
  • fifth day fits
  • occur within the first week after birth
  • seizures are clonic and migratory, with apnea being common.
    • increasing frequency until clonic status epilepticus.
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3
Q

Prenatal ultrasound showed absent cerebral hemispheres with the basal ganglia, brainstem and meninges preserved. What is this condition?

A

Hydranencephaly

  • common cause: infarction secondary to bilateral internal carotid artery obstruction
  • It is by disruption (not considered a malformation)
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4
Q

difference between hydranencephaly and hydrocephalus

A

In hydrocephalus:

cortical mantle is preserved. There is the presence of the third ventricle, abnormal head circumference at birth, full and bulging fontanelles, and a normal vascular study.

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5
Q

Diagnosis for developmental hip dysplasia

A

Newborn:

  1. Normal exam with risk factors - US hip at six weeks (allowing time for resolution of physiologic immaturity and laxity)
  2. Inconclusive exam or hip clicks - Repeat exam in 2 to 4 weeks.
  3. Positive Ortolani or Barlow - Refer to an orthopedic specialist with experience.

Four Weeks to 4 Months

  1. Inconclusive exam - Refer to a specialist or Hip US at six weeks.
  2. Positive Barlow or Ortolani - Refer to an orthopedic specialist with experience.
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6
Q

what is sarnat score

A
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7
Q

What are the phases of global hypoxic insult

A
  1. Initial insult
  2. Secondary energy failure: mitochondrial deficiency, oxidative stress, excitotoxicity, inflammation, necrosis, apoptosis
  3. Long term cell death, inflammation, cell turnover and repair, gliosis
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8
Q

MRI findings of HIE

A
  1. symmetric bilateral parasagittal watershed area (PLIC)
  2. Involve basal ganglia, thalami, brainstem, hipocampi, rolandic cortices

Chronic changes: atrophy of cortex and deep grey nuclei, cystic encephalomalacia

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9
Q

What vessel is commonly involved in arterial ischemic stroke

It is defined as occlusive cerebral arterial event

A

Left MCA

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10
Q

What is most common sign of acute ischemic stroke

A

Acute symptomatic seizures
-focal motor seizure

absence of focal motr deficit should not be reassuring

Other findings: encephalopathy, depressed level of consciousness, abnormal tone
-42% presents older: delayed milstones, early handedness, CP
- Supportive management

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11
Q

What other differential to consider when there is an IVH in term neonate

A

Deep cerebral venous sinus thrombosis

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12
Q

What is cerebral venous sinus throbosis

A

Disruption of venous blood flow most commonly superficial venous system

Presentation: seiures, depressed LOC, diffuse jitteriness
Risk factor: GDM, gHTN, PROM, chorio, sepsis, encephalitis, dehydration, prothrombotic d/o, CHD

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13
Q

Management for cerebral venous sinus throbosis

A
  1. Hydration
  2. treat underlying cause
  3. anticoagulation

  • Dehydration thought as provoking factor
  • No treatment lead to complication: infarct, hydrocephalus, death
  • Reassure: recanalize after several months
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14
Q

What is most commonly detected compartmental hemorrhage (cranial)

A

Subdural hemorrhage

risk factors: gHTN, mat drug use, placental aburptio, assited delivery (vacuum/forceps), birth trauma, perinatal asphyxia, coagulopathy (thrombocytopenia)

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15
Q

A term baby presents with seizures and recurrent apnea
Prenatal/L&D course significant for maternal cocaine use, vacuum assisted delivery

Diagnosis?

A

Intracranial hemorrhage

DX of choice: Brain MRI include angiogram
If no clear cause think of genetics
Common complication: Obstructive hydrocephalus

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16
Q

What is affected in hypoglycemic brain injury

A

Parieto-occipital lobes

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17
Q

What is affected in kernicterus

A

symmetric injury of the globi pallidi

but can affect deep nuclie of the brainstem and cerebellum

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18
Q

A well healthy newborn who later presents with encephalopathy- think of….

Temporal latent pattern of encephalopathy

A

Inborn error of metabolism

Goal therapy: restore anabolic state
1. Hydrate with dextrose fluid- protein free!!!; no hypotnic solution: cerebral edema

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19
Q

What are the different neuroprotective measures for encephalopathy

A

a. Temperature: Therapeutic hypothermia if indicated; otherwise N
b. Ventilation Maintain normocapnia and avoid hypocapnia
c. Oxygenation Maintain normoxia
d. Glucose Maintain euglycemia
c. Blood Pressure Maintain normotension

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20
Q

True or false: neonatal seizures often do not have clinical correlate

A

True
- Importance of EEG for at least 24 hours after the last EEG seizure
- More likely to be focal
- suspect:
1. focal tonic-clonic movt
2. fixed gaze deviation
3. myoclonus
4. bicycling mov’t of legs
5. automatic paroxysms (apnea, cyanosis, cyclic tachycardia, elevated BP)

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21
Q

What to inform parents regarding antiseizure medication

A

> 50% will require 2 or more meds

  1. Phenobarb
  2. levetiracetam
  3. fosphenytoin
  4. benzo
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22
Q

True or False:
Computed tomography of the head is needed before lumbar puncture

A

False:
Consider CT if
1. focal neurologic deficit
2. abnormal level of consciousness
3. papilledema
4. seizure within one week of presentation
5. history of central nervous system disease
6. immunocompromised state.

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23
Q

clinical findings of myelomeningocele based on level of lesion

A
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24
Q

radiologic finding in sturge weber syndrome

A

xray: gyriform calcification (tram-track sign)
CT: calcification, cortical atrophy and leptomeningeal enhancement

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25
Q

when is pupillary constriction develop

A

30-32 wks

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26
Q

when does palmar and plantar grasp reflex
a. appear
b. disappear

A

a. 28-32 wks PMA
b. 3-6 months

27
Q

when is moro reflex
a. appears
b. disappears

A

a. 30-34 wks
b. 3-6 months

28
Q

folic acid prophylaxis to prevent spina bifida

A

a. 0.4mg daily prior to pregnancy
b. hx of child: 4 mg daily

29
Q

What preventive measure has the greatest impact in reducing incidence of IVH

A

Prevention of premature birth

30
Q

Obstetrical intervention that decrease incidence of IVH (2)

A
  1. Antenatal steroids (betamethasone)
  2. Maternal medications:
    * Tocolytics for PTL- nifedipine
    * Antibiotics for chorio

Possibly:
1. elective CS
2. maternal transport vs infant
3. preeclampsia
- Betamethasone vs Dexamethasone: inc risk neurodevelopmental and hearing impairment with dexa; both crosses the placenta

31
Q

Neonatal intervention that decrease incidence of IVH (4)

A
  1. DCC
  2. normothermia (97.7-99.5F)
  3. avoid fluctuation of cerebral blood flow
  4. Optimal ventilation management
32
Q

Site of germinal matrix IVH

A

subependymal germinal matrix

  • site of precursor of CNS cells
  • due to immature vascular network , most abundant 24-34 weeks
  • at term, involutes and replaced by mature capillary network
33
Q

What is Grade 4 IVH

A

Periventricular hemorrhagic infarction

34
Q

How are the following related to risk for IVH
a. Hypercapnea
b. Hypoxemia
c. Bicarbonate infusion

A

INCREASE
- Related cerebral vasodialtion and blood flow

  • Hypercapnea in dose related
  • Hypoxemia in effort to maintain oxygenation
  • HCO3 rapid rise in CO2
35
Q

Most cases of IVH are seen when

A

0-3 days (4days)

36
Q

In the aEEG, what is the preferred region for lead placement

A

Biparietal (P3-P4)

Reason:
1. Minimal impact in newborn nurisng care
2. Avoids artifacts from facial muscle activity
3. Usually hypoperfusion injury involve watershed area is here

37
Q

In a 2-channel aEEG, where is the additional electrode placed?

A

C3-C4

It increases sensitivty for detecting interhemispheric asymmetries and seizure is enchanced

38
Q

In aEEG, it is the resistance to current flow and reflect quality of electrode contact with scalp

A

Impedance

  • It should be low (<10 Ohms)
  • high impedance= artifacts, hence affects accuracy
39
Q

In aEEG, what is the background pattern in extremely preterm infants

A

Discontinuous

Discontinuous: voltage <5 uV lowermargin, >10 uV
Normal until 30 weeks PMA

40
Q

In aEEG, what is the background pattern in late term and term infants

A

Continuous

will include sleep-wake cycle

Continuous: voltages over 5 µV in the lower margins and up to 50 µV in the upper margins

41
Q

What are the classification of aEEG based on amplitude/ background pattern

A

Normal amplitude: lower limit greater than 5 µV and an upper limit greater than 10 µV

Moderately abnormal amplitude: lower limit <5 µV and an upper limit greater than 10 µV

Severely abnormal amplitude: lower limit less than 5 µV and an upper limit <10 µV

42
Q

What is the difference between burst suppression and discontinuous

A

Discontinuous tracing with periods of very low cortical activity (<5 µV) intermixed with a burst of higher amplitude (>25 µV) activity… but the suppression is prolonged

seen in term neonates with encephalopathy

43
Q

What gestational age is sleep wake cycle noted in EEG

A

as early as 30 weeks

characterized as smooth sinusoidal variations

44
Q

Reasons not all seizures are seen in aEEG

A

Seizure in aEEG:
1. measures activity in tiny area (due to limited leads)
2. seizure must be at least 30 seconds (cEEG can catch 10sec)

  • appears as abrupt rise in the lower and upper margin often followed by short period of decreased amplitude
45
Q

Which seizure medication acts on the chloride channel

A
  1. Phenobarbital- prolongs opening of the chloride channel
  2. Benzodiazepines- frequently open the chloride channel

GABA is excitatory in neonates because immature brain has higher Cl concentration (GABA opens Cl channel resulting to dec in membrane potential)
GABA function matures caudal rostral fashion hence inhibit motor at brainstem level but seizure at the cortex

46
Q

Which seizure medication acts via sodium channel blockade

A

Phenytoin/Fosphenytoin

Difficult to sustain therapeutic concentration if given PO
Highly protein bound

47
Q

Which seiure medication is not metabolized in the liver

A

levetiracetam (Keppra)

Excreted in the urine, lower dose with renal dysfunction

48
Q

What are the adverse events related to therapeutic hypothermia

A
  • thrombocytopenia
  • cardiac arrhythmia
  • subcutaneous fat necrosis
49
Q

It is the phase of neural development where the brain and spinal cord develops

A

Primary neurulation

Occurs 3-4 weeks
Associated with anecephaly, encephalocele, myelomeningocele, arnold chiari

50
Q

It is the phase of neural development that involves development of low sacral segment

A

Secondary neurulation

  • occurs 4-7 weeks
  • Abnormality: tethered cord, lipoma, teratoma, spinal cyst, myelocystocele
51
Q

It is the phase in neural development the involves the midline brain structures

A

Prosencephalic development

  • occurs 2-3 months
  • Abnormality: aprosencephaly, holoprocensephaly, agenesis of corpus callosum, agenesis of septum pellucidum, septo-optic dysplasia
52
Q

Neural development abnormality when micrenncphaly and macrencephaly occurs

A

Neural and glial proliferation

Occurs at 3-4 months

53
Q

What phase of neural development does Schizenecephaly, lissencephaly, pachygyria and polymicrogyria occurs

A

Neuronal migration

-Occurs 3-4 months
- Buzz: gyri or smoothness of brain problem think of this phase

54
Q

The phase of neural development that syndromes such as T21, BW syndrome, fragile X, autism, angleman can affect

A

Neuronal organization

Axonal growth and proliferation: 3m- birth
Dendritic and synapse: 6mos-1 yr
Synaptic rearrangement birth to years

55
Q

Disorder of myelination are affected by prematurity and malnutrition. What are the pathways that can be affected?

A

Corticospinal tract: 38 weeks- 2 yrs
Last pathway to myelninate: connections between prefrontal cortex with temporal and parietal lobes: completed at ~32 y/o

56
Q

What are the factors that increase cerebral blood flow

A
  1. Seizures
  2. Increased PCO2
  3. Decreased PaO2
  4. Increased BP in asphyxiated neonates
  5. hypoglycemia
57
Q

What is the most common craniosynostosis?
What suture is involved

A

Scaphocephaly/dolichocephaly
Sagital suture is affects

58
Q

What craniosynostosis has a high incidence of developmental abnormality and cognitive deficiency

A

Frontal plagiocephaly
- unilateral closure of the coronal suture
- Assoccited with crouzon and apert syndrome

59
Q

It is the closure of biliateral coronal sutures and associated syndrome

A

Brachycephaly
Carpenter syndrome

60
Q

What are the findings in latent phase on HIE

A

Low voltage aEEG

Cellular damage

Recovery of oxidative metabolism v. residual mitochondrial injury

Therapeutic window

61
Q

5 mechanisms of injury due to Vein of Galen Malformation

A
  • High-output cardiac failure
  • Hemorrhage from rupture
  • Thrombosis in low-flow areas
  • Vascular steal that leads to ischemic injury resulting from low diastolic flow to surrounding brain parenchyma
  • Mass effect from obstructive hydrocephalus
62
Q

Quick facts on

Myotonic dystrophy

A
  • Autosomal dominant
  • CTG repeat of the DMPK (Chro 19q13)
  • Disease of anticipation
  • Sx: hypotonia (triangular shaped mouth), weakness, feeding difficulty, resp failure, talipes
  • Mortality 17-41% from resp failure

-Cardiac: arrhythmia
-Neuro: has learning disability
-High risk surgery: erratic response to succinylcholine

63
Q

Quick facts on

Neurologic Prognosis/Outcome

A

Ischemic Infarct: spastic hemiplagia
MCA infarct: UE>LE
Global ischemia (HIE): spastic quadriplegia
IUGR: risk for decreased full scale and verbal IQ
Preterm: behavior difficulty

Neontal boards J (19 decks)

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