Neurology Flashcards
What is Benign familial neonatal seizures
- 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.
What is benign idiopathic neonatal seizures
- 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.
Prenatal ultrasound showed absent cerebral hemispheres with the basal ganglia, brainstem and meninges preserved. What is this condition?
Hydranencephaly
- common cause: infarction secondary to bilateral internal carotid artery obstruction
- It is by disruption (not considered a malformation)
difference between hydranencephaly and hydrocephalus
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.
Diagnosis for developmental hip dysplasia
Newborn:
- Normal exam with risk factors - US hip at six weeks (allowing time for resolution of physiologic immaturity and laxity)
- Inconclusive exam or hip clicks - Repeat exam in 2 to 4 weeks.
- Positive Ortolani or Barlow - Refer to an orthopedic specialist with experience.
Four Weeks to 4 Months
- Inconclusive exam - Refer to a specialist or Hip US at six weeks.
- Positive Barlow or Ortolani - Refer to an orthopedic specialist with experience.
what is sarnat score
What are the phases of global hypoxic insult
- Initial insult
- Secondary energy failure: mitochondrial deficiency, oxidative stress, excitotoxicity, inflammation, necrosis, apoptosis
- Long term cell death, inflammation, cell turnover and repair, gliosis
MRI findings of HIE
- symmetric bilateral parasagittal watershed area (PLIC)
- Involve basal ganglia, thalami, brainstem, hipocampi, rolandic cortices
Chronic changes: atrophy of cortex and deep grey nuclei, cystic encephalomalacia
What vessel is commonly involved in arterial ischemic stroke
It is defined as occlusive cerebral arterial event
Left MCA
What is most common sign of acute ischemic stroke
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
What other differential to consider when there is an IVH in term neonate
Deep cerebral venous sinus thrombosis
What is cerebral venous sinus throbosis
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
Management for cerebral venous sinus throbosis
- Hydration
- treat underlying cause
- anticoagulation
- Dehydration thought as provoking factor
- No treatment lead to complication: infarct, hydrocephalus, death
- Reassure: recanalize after several months
What is most commonly detected compartmental hemorrhage (cranial)
Subdural hemorrhage
risk factors: gHTN, mat drug use, placental aburptio, assited delivery (vacuum/forceps), birth trauma, perinatal asphyxia, coagulopathy (thrombocytopenia)
A term baby presents with seizures and recurrent apnea
Prenatal/L&D course significant for maternal cocaine use, vacuum assisted delivery
Diagnosis?
Intracranial hemorrhage
DX of choice: Brain MRI include angiogram
If no clear cause think of genetics
Common complication: Obstructive hydrocephalus
What is affected in hypoglycemic brain injury
Parieto-occipital lobes
What is affected in kernicterus
symmetric injury of the globi pallidi
but can affect deep nuclie of the brainstem and cerebellum
A well healthy newborn who later presents with encephalopathy- think of….
Temporal latent pattern of encephalopathy
Inborn error of metabolism
Goal therapy: restore anabolic state
1. Hydrate with dextrose fluid- protein free!!!; no hypotnic solution: cerebral edema
What are the different neuroprotective measures for encephalopathy
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
True or false: neonatal seizures often do not have clinical correlate
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)
What to inform parents regarding antiseizure medication
> 50% will require 2 or more meds
- Phenobarb
- levetiracetam
- fosphenytoin
- benzo
True or False:
Computed tomography of the head is needed before lumbar puncture
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.
clinical findings of myelomeningocele based on level of lesion
radiologic finding in sturge weber syndrome
xray: gyriform calcification (tram-track sign)
CT: calcification, cortical atrophy and leptomeningeal enhancement
when is pupillary constriction develop
30-32 wks
when does palmar and plantar grasp reflex
a. appear
b. disappear
a. 28-32 wks PMA
b. 3-6 months
when is moro reflex
a. appears
b. disappears
a. 30-34 wks
b. 3-6 months
folic acid prophylaxis to prevent spina bifida
a. 0.4mg daily prior to pregnancy
b. hx of child: 4 mg daily
What preventive measure has the greatest impact in reducing incidence of IVH
Prevention of premature birth
Obstetrical intervention that decrease incidence of IVH (2)
- Antenatal steroids (betamethasone)
- 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
Neonatal intervention that decrease incidence of IVH (4)
- DCC
- normothermia (97.7-99.5F)
- avoid fluctuation of cerebral blood flow
- Optimal ventilation management
Site of germinal matrix IVH
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
What is Grade 4 IVH
Periventricular hemorrhagic infarction
How are the following related to risk for IVH
a. Hypercapnea
b. Hypoxemia
c. Bicarbonate infusion
INCREASE
- Related cerebral vasodialtion and blood flow
- Hypercapnea in dose related
- Hypoxemia in effort to maintain oxygenation
- HCO3 rapid rise in CO2
Most cases of IVH are seen when
0-3 days (4days)
In the aEEG, what is the preferred region for lead placement
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
In a 2-channel aEEG, where is the additional electrode placed?
C3-C4
It increases sensitivty for detecting interhemispheric asymmetries and seizure is enchanced
In aEEG, it is the resistance to current flow and reflect quality of electrode contact with scalp
Impedance
- It should be low (<10 Ohms)
- high impedance= artifacts, hence affects accuracy
In aEEG, what is the background pattern in extremely preterm infants
Discontinuous
Discontinuous: voltage <5 uV lowermargin, >10 uV
Normal until 30 weeks PMA
In aEEG, what is the background pattern in late term and term infants
Continuous
will include sleep-wake cycle
Continuous: voltages over 5 µV in the lower margins and up to 50 µV in the upper margins
What are the classification of aEEG based on amplitude/ background pattern
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
What is the difference between burst suppression and discontinuous
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
What gestational age is sleep wake cycle noted in EEG
as early as 30 weeks
characterized as smooth sinusoidal variations
Reasons not all seizures are seen in aEEG
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
Which seizure medication acts on the chloride channel
- Phenobarbital- prolongs opening of the chloride channel
- 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
Which seizure medication acts via sodium channel blockade
Phenytoin/Fosphenytoin
Difficult to sustain therapeutic concentration if given PO
Highly protein bound
Which seiure medication is not metabolized in the liver
levetiracetam (Keppra)
Excreted in the urine, lower dose with renal dysfunction
What are the adverse events related to therapeutic hypothermia
- thrombocytopenia
- cardiac arrhythmia
- subcutaneous fat necrosis
It is the phase of neural development where the brain and spinal cord develops
Primary neurulation
Occurs 3-4 weeks
Associated with anecephaly, encephalocele, myelomeningocele, arnold chiari
It is the phase of neural development that involves development of low sacral segment
Secondary neurulation
- occurs 4-7 weeks
- Abnormality: tethered cord, lipoma, teratoma, spinal cyst, myelocystocele
It is the phase in neural development the involves the midline brain structures
Prosencephalic development
- occurs 2-3 months
- Abnormality: aprosencephaly, holoprocensephaly, agenesis of corpus callosum, agenesis of septum pellucidum, septo-optic dysplasia
Neural development abnormality when micrenncphaly and macrencephaly occurs
Neural and glial proliferation
Occurs at 3-4 months
What phase of neural development does Schizenecephaly, lissencephaly, pachygyria and polymicrogyria occurs
Neuronal migration
-Occurs 3-4 months
- Buzz: gyri or smoothness of brain problem think of this phase
The phase of neural development that syndromes such as T21, BW syndrome, fragile X, autism, angleman can affect
Neuronal organization
Axonal growth and proliferation: 3m- birth
Dendritic and synapse: 6mos-1 yr
Synaptic rearrangement birth to years
Disorder of myelination are affected by prematurity and malnutrition. What are the pathways that can be affected?
Corticospinal tract: 38 weeks- 2 yrs
Last pathway to myelninate: connections between prefrontal cortex with temporal and parietal lobes: completed at ~32 y/o
What are the factors that increase cerebral blood flow
- Seizures
- Increased PCO2
- Decreased PaO2
- Increased BP in asphyxiated neonates
- hypoglycemia
What is the most common craniosynostosis?
What suture is involved
Scaphocephaly/dolichocephaly
Sagital suture is affects
What craniosynostosis has a high incidence of developmental abnormality and cognitive deficiency
Frontal plagiocephaly
- unilateral closure of the coronal suture
- Assoccited with crouzon and apert syndrome
It is the closure of biliateral coronal sutures and associated syndrome
Brachycephaly
Carpenter syndrome
What are the findings in latent phase on HIE
Low voltage aEEG
Cellular damage
Recovery of oxidative metabolism v. residual mitochondrial injury
Therapeutic window
5 mechanisms of injury due to Vein of Galen Malformation
- 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
Quick facts on
Myotonic dystrophy
- 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
Quick facts on
Neurologic Prognosis/Outcome
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
