Neuro Flashcards
palmar reflex
one of the first, 26-28wks, strong by 32w, gone by 2-4 m - replaced by voluntary grasp
crossed extension reflex
30-34 wks, done by 2m. rub extended foot, other foot flexes, extends and toes spread, leg adducts
moro reflex
partial (spreading of arms) at 28-30w, full by 37-38w. Gone by 4-6m.
rooting reflex
starts at 28w, fully developed at 34 weeks, tests trigeminal n. Gone by 4m
tonic neck reflex/fencing
move head to one side slowly, that ipsilateral arm extends, the other flexes. latest to develop. 35w - 6m
what are common criteria accepted for TH?
hypoxia: Apgar <5 at 10 minutes, EGA >36 weeks, <6 hours since event, pH <7 or bd >16 in gas upto 1 hour of life
encephalopathy: moderate-severe based on sarnat criteria
what are three syndromes associated with craniosynostosis and how do they differ?
Crouzon: most common. +/- present with craniofacial abnormalities at birth, +/- hydrocephalus. Most commonly b/l coronal craniosynostosis.
Apert’s: less common. Do present with craniofacial abnormalities at birth. +/- hydrocephalus. MC b/l coronal synostososis. Polysyndactyly. May have devpt delay.
Pfeiffer’s: least common. Clover leaf deformity, b/l coronal suturues. Broad thumbs and great toes.
How do most encephalocele present?
In the east, most art frontobasal, in the West more are occipital. Most only have meninges and good prognosis.
How does DWS present?
Posterior fossa cyst, absent/hypoplastic cerebellar vermmis, +/- hydrocephalus.
Treated with VP shunt/cyst shunt or both.
What is a neuroenteric cyst?
a fluid filled cyst within the spinal cord, compresses cord and causes weakness in gait or pain. Tx: decompress.
What is diastometamyelia?
A septum (tissue/bone) causing two compartments of spinal cord. Can be two cords in one dura or one cord each in its own dura. Present with neurogenic bladder, leg weakness, orthopedic abnormlaities.
What is a lipomyelomeningocele?
It is a fatty connection from the spinal cord to the subQ tissue, can present with a dorsal appendage/tail. Symptoms will primarily include neurogenic bladder and bowel.
What is a dorsal dermal sinus?
sinus tract between spine and skin, can leak CSF, have recurrent meningitis. Needs to get excised. Can look like a spinal dimple far from gluteal cleft.
What did the MOMs trial show
When myelomeningoceles were repaired in utero, there was a decrease in chiari II herniation and hydrocephalus requiring shunting so the trial was ended early do to success. May have found increased incidence of tethered cord.
In myelomeningocele, what is prognosis based on?
The lower the lesion, the more the patient can do. L3 : can stand. L4-5: can ambulate. Most will be incontinent.
What is periventricular venous infarction?
Similar to peri-ventricular hemorrhagic infarction in preterm babies - an outpouching of the ventricle caused by hemmorhagic infarction after venous obstruction.
What is sinovenous thrombosis? (SVT)
This is a thrombosis in the sinus venosus. Often causes congestion –> edema –> hemorrhagic infarction. Consider heparin, consider obtain clotting factors. Most present within first 48 hours. Think about this dx in a term with IVH.
What is Neonatal Arterial Ischemic Infarction?
This is a thrombus in the intracerebral arteries causing an ischemic infarction. MCA is most common. Presents similarly to HIE/seizures but happens after a few hours of life.
If asymptomatic in neonatal period, may be symptomatic at a few months of age and present with handedness, called presumed perinatal AIS. associated with IVF.
What are the different findings of HIE on MRI?
T1, T2: T1 should should WM as white (myelinated). In neonates, internal capsule is one of the only things to be myelinated early. You may see posterior limb of internal capsule being less white (PLIC) indicating HIE. Obtain this at 7-21d of life to assess full degree, but not timing, of injury. T1-weighted images are ideal for depicting myelin, ischemia,
and subacute hemorrhage. T2-weighted images provide contrast between gray and white matter.
DWI: diffusion weighted imaging indicates restricted diffusion (dark). Best at 1-3 (5-7?)d of life, goes away by d7-10. Best for timing of injury.
MRS: best starting at 6h of life
How is an MRS helpful in HIE?
Lactate: NAA ratio can be informative. In HIE, lactate will be higher and NAA (N-acetylacetate, a marker for neuronal integrity) will be lower. So Lactat:NAA ratio will be higher in HIE.
Injury to which parts of the brain are associated with what pathology?
Globus Pallidus - kernicterus
b/l disease - metabolic
posterior parietal - hypoglcyemia
thalami/basal ganglia/WM - HIE
How does phenobarbital work?
It works on GABA mechanisms. In preterm babies, this mechanism is immature so it can actually be an excitatory response instead of inhibitory. Adding Bumetadine may help by causing Cl influx instead of efflux. With phenobarb, frequently still see subclinical seizures. Bumetadine has been associated with hearing impairment thought.
when does the blink reflex happen?
by 26 weeks, babies consistently blink in response to light. By 32 weeks, blink will remain as long as light stays present.
When is the pupillary light reflex?
CN II, CN III
Begins at 30w PMA, consistent by 32-35w
When do babies start to visually track?
by term, should be able to fixate and have limited tracking for something ~1 foot away
how much folic acid should women take?
400mcg daily pregestation, 4000mcg if hx of NTD
In what condition are babies likely to develop a latex alergy?
Myelomeningocele, 30-50% develop latex allergy
Which hydrocephalus is inherited?
X-linked hydrocephalus associated with adducted thumbs.
What is the role of glutamate?
Glutamate is an excitatory amino acid. Topiramate is a potential anti-seizure med that blocks glutamate. Decreased glutamate release by TH in HIE
What is the role of adenosine?
Adenosine suppresses fetal respiration. Theophyllines/caffeine are adenosine receptor antagonists.
What is the role of GABA?
GABA (gamma-aminobutryic acid) opens chlorie channels. It is excitatory in fetus and premature neonates due to high chloride content –> depolarization. Inhibitory in adults.
Phenobarb and benzo are gaba-ergic.
what is benign familial neonatal seizures
AKA 3rd day fits, usually resolve by one month. Potassium channel mutation.
what is benign idiopathic neonatal seizures
AKA 5th day fits, assc with clonic seizures and apnea, resolves in weeks to 1s month
where do congenital brain tumors usually exist?
supratentorium (rather than infra, which is common in childhood)
What is the most common CNS tumor, how do they typically present?
teratoma, usually from lateral ventricle > 3rd ventricle. May present with hydrocephalus, otherwise incidental. Seizures is uncommon.
teratoma > astrocytoma > craniopharyngeoma
Most supratentorial
bad prognosis
What is the classic triad of Sturge Weber
1) port wine stain over v1, v2 distribution
2) increased intraocular pressure/glaucoma
3) leptomeningeal involvement with vascular malformation/seizures
* *seizures are most likely clinical outcome** (85%, usually contralateral to port wine stain), developmental delay (75%), stroke like episodes
what is PHACES syndrome
P: posterior fossa abnormality H: hemangiomas --> if this is present, get MRI, ECHO, optho A: arterial malformation C: cardiac anomaly/coarct E: eye abnormalities S: sternal defect
congenital myopathy
no dystrophic changes on muscle biopsy
congenital muscular/myotonic dystrophy
significantly elevated CPK, may impact eye and brain so evaluate with optho and mri.
Myotonic dystrophy (DM1) may present with facial diplegia (carp appearance of mouth), bell shaped chest, talipes equinovarus, hypotonia, respiratory failure.
Dx: DMPK gene, CTG repeat (mild: 50-150, classic 100-1000, severe >1000). AD inheritance. Mother may have myopathic facies and a sustained grip during handshake.
At risk for cardiac conduction defect and erratic response to succinylcholine during surgery
Chr 19, 20-40% mortality, most die of respiratory failures
SMA
may have slightly elevated CPK, absent DTR, fasciculations. Dx: SMN1, SMN2 genes
What categories of CP does spastic CP include?
1) Spastic diplegia (35%): legs affected > arms. May present as persistent commando crawl, toe walking, MC cause is PVL and predominant CP with PT infants. 1/3 have cognitive impairment. Most will walk but may need assistance.
2) Spastic hemiplegia (25%): unilateral arm>leg impacted. May be due to stroke, unilateral IVH/PVL, vascular malformation. Typically can walk by 3y, 2/3 with cognitive impairment.
3) Spastic quadriplegia (20%): most severe form of CP, caused by PVL, ischemia, infection. Not likely to walk, high association with intellectual disability and sz.
Spastic CP is the most common type of CP 75%)
What is athetoid CP?
Athetoid CP, aka extrapyramidal CP (dyskinetic, atonic), is 15% of CP. It impacts the entire body, presents with hypotonia, poor head control and progression to hypertonicity. Arms > legs impacted. Seen in basal ganglia and thalamus injury (asphyxia, kernicterus, mitochondrial disorders)
What is the process of HIE?
Primary energy failure, which cannot be prevented by TH.
Secondary energy failure and neuronal cell death (both necrosis and apoptosis).
Necrosis is secondary to increased glutamate release, cell depolarization, swelling, ROS.
Apoptosis is secondary to caspase and TNF release.
TH most significantly decreases neuronal apoptosis.
Which vessel is involved in vein of galen malformation
What neurological problems can it cause?
How does it manifest clinically?
How is it managed?
Most commonly the medial prosencephalic vein, a vein that typically devolves after 11th week, draining choroid plexus into falcine sinus. Usually AVM with choroidal arteries. Can cause hemmorhage, ischemia, and obstructive hydrocephalus. Clinically manifests primarily as CHF –> azotemia, bounding carotid pulse. Mgmt: managing CHF and minimizing high flow through aneurysm.
what is the significance of the PLIC
An important white matter tract, one of the first areas to be myelinated in neonate, usually around 32-35 weeks. If not as white and bright on T1 imaging, indicative of poor motor outcomes after HIE.
pompes disease
aka: glycogen storage disease II, due to deficiency of the lysosomal enzyme acid alpha glucosidase
dx: GAA gene sequencing, AR, usually elevated CK
clinical presentation: central hypotonia, generalized weakness, respiratory insufficiency, hypertrophic cardiomyopathy, and feeding difficulty
what are the findings in erbs palsy
nerve roots C5-7 impacted
waiters tip
biceps reflex absent, palmar intact, absent shoulder moro, intact hand moro
c4 may be associated with diaphragm
what are the findings in klumpkes palsy
c8-t1
biceps apsent, palmar may also be absent, complete more absent, claw, t1 may be associated with horner
in nas, what neurotransmitters increase/decrease with opiate abuse
increase: acetycholine (diarrhea, vomiting, sneezing), noradrenaline (hyperthermia, tremors, tachy), corticotropin (hyperphagia)
decrease: serotonin (sleep disturbance), dopamine (irritability)
what are the CN nerves and actions
pneumonic: oh oh oh to taste a fruity vodka go visit a hotel. CN1: olfactory: smell CNII: optic: visual acuity CN III: occulomotor: eye movement CN IV: trochlear: downward eye mvmt CN V: trigeminal: facial sensation, buccal CN VI: abducens: outward eye mvmt CN VII: facial: muscle movement of face CN VIII: vestibulocochlear: hearing CN IX: glossopharyngeal: posterior 1/3 taste CN X: vagus: gag, swallowing CN XI: spinal accessory: shoulder shrug CN XII: hypoglossal: tongue movement
what are the degrees of hearing loss
mild: 20-40
moderate: 40-60
severe: 60-80db
profound: >80d
best determined by 6-10m canonical babbling
whats a normal extreme premature aeeg pattern?
discontinuous (until ~30 w), may not have sleep/wake cycles (until ~28w,, after, REM sleep predominates)
normal late pt or term aeeg should have amplitude between 5 and 10
what is the proven outcome of TH?
increase in survival without neuro disability and decrease in ‘death or moderate/severe disability’
what is the most severe form of neuronal migration disorder?
schizencephaly: complete agenesis of portion of cerebral cortex due to clefts
lissencephaly: smooth brain, microcephaly
neuronal migration disorder is an aberration of gyral development
when do you see the lemon sign
myelomeningocele, scalloping or notching of the frontal bones
when do you see the banana sign
arnold chiari malformation, herniation of the cerebellum/brain stem through the foramen magnum
The Bayley Score of Infant Development, strengths and weaknesses
- representative of US population
- III was revised to have language separate. May underestimate developmental delay
- common categories assessed: cognitive, fine motor, gross motor, language, social
- major weakness is lack of assessment of overall infant adjustment
- out of 100, 60-69 is moderate delay
- a score at 2 y may predict the score at 4y but not at 8 y
only known prevention for ROP is
caffeine
what is type 1 ROP
z1 anything with plus or s3 and z2, s2/3 with +
what is the triad of mccune albright
endo (hyperthyroidism), bone, cafe au lait spots
what is the triad in TS
benign tubers (especially in brain –> seizures), ash leaf spots, rhabodmyoma
when is appropriate follow up for high risk ROP (Z1 or S3)
one week
What other treatment in mice has been shown to be efficacious like anti-vegF
omega 3 fatty acids (LCPUFAs high in retina)
when is autism diagnosed and how
usually at 4y/o but can be as soon as 2 y/o.
MCHAT is given at 18m and 24m
signs include: no babbling/pointing by 12m, no single words by 16m, no 2-word phrases by 24m, loss of language skills at anytime
when is cystic pvl usually seen
2-4 weeks
what is the best prognosticator for HIE?
1) Sarnat staging 2) aEEG 3) presence of seizures
what is an eye sequelae of galactosemia and when does it present?
cataracts, usually around 2 months, b/l, secondary to galactose deposition
Which Sarnat staging:
- most common to have seizures
- hyperalert
- mydriasis
- profuse secretions
- decreased/absent reflexes
II I I II III
when do DTRs present by?
27 weeks
UMN –> hyper reflexia
LMN –> hypo reflexia (SMA)
what are three different kinds of intercranial cysts?
connatal cysts (frontal horn) - benign, periventricular but below ventricle
choroid plexus cyst - benign, in choroid plexus, assc w/ aneuploidy
cystic pvl - periventriculary, superior and lateral to ventricles
what is the order of neuro embyrology?
primary neuralation, secondary neuralation, ventral induction (prosencephaln dvpt), migration, organization, myelination
when do brachial plexus injuries typically resolve by?
usually some improvement by 2w, resolution by 6m. If still present at 15m, bad sign.
what are disorders of primary neurulation?
anencephaly, encephalocele, meyleomeningocelie, arnold chiari
what are disorders of secondary neurolation?
(lower sacral segment of spine): tether cord, oipoma, spinal cysts
what are disorders of prosencephalic dvpt /ventral induction?
three components: formation, cleavage, midline development:
aprosencephaly, holoprosencpahly, agensis of corpus callosum, septum pellucidum, s.o. dysplasia
what are disorders of neuronal migration?
schizencephaly, lissencephyali, pachygyria, polymicrogyria
what are disorder of neuronal organization?
found in tri 21, fragile x, autism, angelman, prematurity
what are disorders of meylination?
found in prematurity, malnutirtion and WM hypoplasia
what is the etiology of subq fat necrosis and hyper Ca?
fat necrosis –> upregulates 1, 25 vit D –> upregulates Ca absorption
management: low calcium and low vitamin D diet, adequate hydration, loop diuretics, corticosteroids, and bisphosphonates
what is a normal vs mod abnormal vs suppressed eeg?
normal: >5, >10
mod abnormal: <5, >10
suppressed: <5, <10
what is continuous vs discontinuous vs burst suppression vs low voltage vs flat?
continuous: 5-7, 10-25
discontinous: virable low border, going from <5 to >10, with greater overall band
burst suppression: discontinuous with spikes >25 with min amp and no variability, >100/hr is (+), <100/h is (-)
low voltage: cont but low amp <5
flat: extremely low amp
what type of IVH does cooling help most with
moderate encephalopathy
what is the NNT with cooling?
5-7 to have improvement in death OR NDI; mortality/ NDI
how head cooling differnt than full body?
head cooling studies cooled one degree higher to 34-35
what are the different types of drug screening for NAS?
urine: only indicates recent use
hair and mec –> both better for opioids and cocaine but hair has mroe false (+)
what is the neurologic fetal risk of ssri
possible craniosynostis, arnold chiari, anencephaly
in HIE, biomarker profile for mod-severe is:
elevated s100b, neuron specific enolase, il6, GFAP, brain derived neurotrophic factor
whats the difference between phenytoin and phenobarb?
phenytoin causes less sedation
but has increased protein binding and increased interactions, extravasation risk, bradycardia if given too fast
both equally effective and metabolized in the liver
what are associations with absence of CC
Probst bundle (the fibers that were supposed to connect the 2 hemispheres) and colpocephaly (enlargement of occipital horns of lateral ventricles)
what is aicardi syndrome
absence of CC, epilepsy, eye problems
XD (like IP, Fragile X)
what is the triad of meckel gruber
occipital encephalopathy, post axial polydactly, renal cystic dyslasia
