Neurology Flashcards

1
Q

Most common complication from perinatal asphyxia

A

Renal failure (40-70%)

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2
Q

When to initiate therapeutic hypothermia

A

Within 6 hours- reperfusion injury stage

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3
Q

NNT to prevent neuro disability for TH

A

<10

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4
Q

What stage of encephalopathy do you cool for?

A

MODERATE
SEVERE

MILD IS NOT PROVEN!!

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5
Q

Gold standard for seizure detection

A

Continuous EEG- at least 24 hours

aEEG useful adjunct or alternative
Monitoring for seizures is recommended by AAP, ACNS
25-40% of babies cooled for HIE have seizures in first 72 hrs of birth

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6
Q

HIE typical patterns of injury on MRI

A

Basal ganglia and thalami (relay station)
Posterior limb of the internal capsule (dense motor pathway through BGT)
Brain stem (preterm or severe)
Watershed- cortex and white matter

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7
Q

Most predictive of severity of motor in patient with HIE

A

Severity of basal ganglia and thalamus injury

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8
Q

Most predictive of walking at age 2 in HIE MRI

A

PLIC

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9
Q

Most common type of stroke

A

Arterial ischemic stroke (80%)

Seizures are most common presenting sign (>75%) - DO NOT PRESENT WITH HEMIPLEGIA but can have hemiplegia on contralateral side as sequelae

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10
Q

Vein of Galen

A

High output heart failure

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11
Q

Subgaleal bleed at risk for brain injury- how?

A

Size of bleed leads to ischemic brain injury

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12
Q

Major cause of neuro developmental impairment in preterm infants

A

IVH

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13
Q

Classification of IVH

A
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14
Q

Severity of outcome of IVH related to

A

Grade of hemorrhage
Unilateral/bilateral, midline shift
Extent of parenchyma involvement

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15
Q

Most common outcome from IVH/PVL

A

Spastic diplegia- hypertonia with spasticity
LE>UE

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16
Q

PVL outcomes

A

Increasing rates of disability with severity of white matter abnormalities

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17
Q

Pupillary response

A

CN 2,3
28 weeks

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18
Q

Extra-ocular movements

A

CN 2, 3, 4, 6
Fix/follow at 34 weeks
Disconjugate if not fixing
Dolls eyes

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19
Q

Facial grimace

A

CN 5 & 7
Strength, symmetry

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20
Q

Suck

A

Cranial nerves 5, 7, 12

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21
Q

Swallow

A

CN 9, 10

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22
Q

MORO

A

3 components:
1. ABDUCTION - arms/hands spread open (25-28 wks)
2. ADDUCTION- arms/shoulders “hugging/embracing” (33/34 wks)
3. Audible cry- may not always be elicited

Absent/incomplete- upper motor neuron
Asymmetric- consider brachial plexus injury

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23
Q

True or false?
Neonatal seizures are not reliably diagnosed by clinical observation alone

A

TRUE

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24
Q

True/false
Most neonatal seizures are symptomatic of acute brain illness (~80%)

A

TRUE!!

Most common:
- HIE (~50% in US)
- stroke/hemorrhage
- infection
- malformations
- electrolytes/hypoglycemia

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25
Highest yield test for neonatal seizure
BRAIN MRI- ASAP ~90% will have diagnosis apparent on MRI
26
Prognosis following neonatal seizures
Etiology is the single most important prognostic factor Higher seizure burden is associated with increased risk of poor outcome
27
Burst suppression
POOR prognosis Persistent burst suppression at 72 hours in HIE carries a very poor prognosis Neonatal onset epilepsies with burst suppression are typically ominous
28
MC localization of neonatal hypotonia
BRAIN ANTERIOR HORN CELL- SMA peripheral nerve- Riley-day Peripheral=rare
29
MC cause of hydrocephalus in neonates
IVH post hemorrhagic hydrocephalus - MC cause of obstructive and communicating hydrocephalus
30
Leads to significant increase in cerebral blood flow
Hypoglycemia Hypercapnia Anemia
31
Leads to decrease in cerebral blood flow
Polycythemia Hypotension Hypocarbia
32
When fetus becomes responsive to auditory information
26 weeks Immature auditory system functional 25-29, complete by 30 weeks
33
Compared to UA, UV blood gas
Higher pH, lower pCO2, higher pO2 V (high, low, high)
34
Therapeutic hypothermia alters first or second energy failure most?
SECOND
35
When do you initiate cooling?
Latent phase- 1-6 hrs
36
Most common cause of CP in term neonate
HIE
37
HUS findings in HIE
Serial HUS over first 4 days most helpful Edema peak=24 hrs Single HUS in first week is normal in 50% HIE Doppler US for resistive index (or pulsatility index) increases sensitivity and specificity
38
Deficits from stroke
Motor- depends on location Learning/behavior Epilepsy
39
IVH epidemiology
Highest risk <29 weeks, <1500g Grade 3/4 highest among youngest and smallest
40
Risk factors for IVH
Gestational age- younger= higher risk Lack of antenatal steroids Lack of exposure to mag sulfate Birth outside an experienced center/need for transport CV and respiratory compromise at birth-1st week Hypercapnia —> cerebral vasodilation Hypernatremia (>150)
41
CP from PVL
SPASTIC DIPLEGIA
42
Neonate with PVL
oligodendrocytes affected
43
HIE leads to decreased...
phosphocreatinine
44
HIE type of CP
spastic quadriplegia
45
Learning and behavior issues in HIE after TH are indicative of injuries in what area?
Watershed
46
Why are preemies at risk for IVH?
Germinal matrix has a rich vascular supply because it produces oligodendrocytes (glial cell) which makes myelin sheaths between 23-32 was Vascular supply of GMH does not have the same structural integrity of vessels that are built to last many years = prone to bleeding Blood flow to GMH is variable because of the limited ability of preterm infants to auto regulate cerebral blood flow
47
Why does grade IV happen?
Known as parenchymal hemorrhagic infarction Veins of the periventricular white matter drain into the terminal vein which runs through the germinal matrix Hematoma in the GM can occlude venous drainage of the terminal vein leading to infarction and secondary bleeding
48
Most common area of the brain affected by PVL
Periventricular white matter dorsolateral to the external angles of the lateral ventricles
49
How does PVL occur?
Poor vascular supply to the periventricular white matter -> Ischemia in “watershed” areas of the periventricular white matter -> alters development of vulnerable oligodendrocyte progenitor cells
50
Describe cystic PVL
Focal cystic necrotic lesions deep in the periventricular white matter Easily seen by US Usually seen around 2-4 wis and beyond Less common than diffuse PVL
51
Describe diffuse PVL
High signal intensity in the white matter on T2 weighted MRI images MORE COMMON than cystic PVL
52
Describe Arnold Chianti malformation
Structural abnormality caused by primary neurulation defect Hallmark: caudal displacement of the cerebellum before the foramen magnum Symptom severity corresponds to the extent of the lesion
53
Describe Dandy Walker
Congenital abnormality of the cerebellum Enlargement of the 4th ventricle Complete or partial absence of the cerebellar vermis Cyst formation at the skull base
54
Typical EEG pattern in HIE
Depression or burst suppression May have seizures that are refractory to medical therapy
55
Most common cranial nerve to be injured during birth
Facial nerve- VII While exiting the skull and before entering the parotid gland, this nerve becomes very superficial which makes it prone to external pressure injuries Weakness in lower and upper facial muscles Unilateral L>R Asymmetric cry Recover in 1-3 weeks Protect involved eye with drops
56
What is Lissencephaly?
Developmental disorder of neuronal migration during 12-24th week, dx by US 26-28 wks Smooth cerebral cortex without gyri or sulci Associated findings: microcephaly, ventriculomegaly, widened Sylvian fissures, complete/partial agenesis of the corpus callosum 2 types: 1. Facial dysmorphism 2. Hydrocephaly, dysgenesis of the cerebellum Associated with Walker-Warburg and Miller-Dierker syndromes
57
Highest risk for hearing loss
asphyxia craniofacial anomalies syndrome with hearing loss RDS bacterial meningitis meconium aspiration syndrome chromosomal abnormalities maternal diabetes multiple births NOT ASSOCIATED WITH INCREASED RISK OF HEARING LOSS: hydrocephalus gentamicin use birth weight < 1500g
58
Neurodevelopmental sequelae most likelly associated with IUGR and not prematurity
Decreased full scale and verbal IQ
59
Cerebral perfusion pressure
CPP = MAP - ICP MAP = (2/3)DPB + (1/3) SBP MAP = (CO x SVR) + CVP
60
Impairment
structural or functional abnormality at the organ level Can occur without a disability
61
Disability
restriction at the personal level that results from an impairment Can occur without a handicap
62
Handicap
occurs at the societal level and is a disadvantage as a result of a disability that prevents fulfillment of that individual's usual role
63
How can you clinically diagnose an encephalocele?
Transillumination demonstrates lack of bony tissue Mass will enlarge with straining or crying
64
Meckel syndrome is due to a defect in what?
Cilia! secondary to the ciliary abnormality, TRIAD: occipital encephalocele, multicystic dysplastic kidneys and polydactyly
65
Diagnosis of prenatal fetal ventriculomegaly
width of at least one ventricle > 10 mm often associated with hydrocephalus but can also be related to a decrease in amount of normal brain tissue Neurodevelopmental outcomes may correlate with severity of ventricle enlargement with wide variation based on cause and presence of other associated malformations
66
primary/secondary neurulation dorsal induction
Primary: 3-4 weeks Secondary: 4-7 weeks Issues during primary: anencephaly myeloschisis encephalocele myelomeningocele Arnold-Chiari
67
prosencephalic development ventral induction
2-3 months Issues: Formation: aprosencephaly Cleavage: holoprosencephaly Midline development: agenesis of the corpus callosum, agnesis/absence of septum pellucidum, septo-optic dysplasia
68
neural and glial proliferation
3-4 months Micrencephaly Macrencephaly
69
neuronal migration
3-5 months Schizencephaly- abnormal clefts of the brain Lissencephaly- underdevelopemnet of gyri, smooth brain Pachygyria- broad abnormally large gyri, leads to less sulci Polymicrogyria- numerous small convolutions
70
schizencephaly segmental developmental failure of cell migration to form cerebral cortex can be due to vascular ischemia of portion of germinal matrix abnormal clefting- most commonly involves posterior frontal or parietal lobes sporadic cleft filled with GREY MATTER
71
complete failure of midline division FUSED thalami, ABSENT falx cerebri
holoprosencephaly
72
Neuronal organization
axonal outgrowth and proliferation- 3 months to birth dendritic and synapse- 6 mo-1 yr synaptic rearrangements- birth to years Issues: mental deficiency Trisomy 21 Fragile Xx Autism Angelman Prematurity
73
Myelination
birth to years corticospinal tract- 38wks to 2yrs last pathway to myelinate is the association bundle- connects prefrontal cortex with the temporal and parietal lobes (complete ~ 32 yrs) Issues: Cerebral white matter hypoplasia Prematurity Malnutrition
74
Type of Arnold-Chiari that presents in childhood/adulthood caudal displacement of cerebellar tonsils below foramen magnum 4th ventricle and brainstem in NORMAL position associated with syringomyelia (cyst in the spinal cord) or hydromelia (dilation with CSF of canal)
Type I can be associated with skeletal anomalies (scoliosis)
75
Type of Arnold-Chiari that presents in infants more extensive abnormality elongation and caudal displacement into cervical spinal canal of cerebellar tonsils, 4th ventricle, choroid plexus, brainstem
Type II often associated with hydrocephalus and myelomeningocele
76
Pattern of injury in hypoglycemia
Bilateral posterior occipital cortex Can also include middle cerebral infarction and basal ganglia/thalamic abnormalities Risk of injury related to duration of hypoglycemia rather than degree
77
Most common cranial nerve to be injured during birth
Facial nerve (VII) Caused by nerve compression with hemorrhage and edema of the nerve sheath Weakness of upper and lower facial muscles, assymetric cry Unilateral L>R Usually recover in 1-3 weeks
78
upper motor neuron vs lower motor neuron
79
Disorders of anterior horn cell
Hypoxic-ischemic myelopathy SMA Type I- Werdnig-Hoffman Disease Neurogenic arthogryposis
80
Disorders of the neuromuscular junction
Acquired transient neonatal myasthenia gravis congenital myasthenia gravis Diseases involving a defect in acetylcholine release: - Clostridium tetani - Clostridium botulinum - hypermagnesemia - aminoglycoside toxicity
81
Disorders of the muscle
congenital myopathy- myotubular myopathy muscular dystrophy - congenital myotonic dystrophy - duchenne dystrophy - congenital muscular dystrophy
82
SMA Type 1 Werdnig-Hoffmann disease | AR, chromosome 5
degeneration of anterior horn cell presentation: - severe generalized hypotonia - "frog-leg" position, "jug-handle" UE abducted, rotated - marked head lag - facial sparing - AREFLEXIA - bell-shaped chest with abdominal breathing - bulbar weakness: poor suck/swallow, weak cry, tongue fasciculations Diagnosis: - CPK normal - EMG: nonspecific denervation, fasciculations, fibrillations - muscle biopsy = atrophy of motor units - nerve conduction velocity normal
83
Acquired transient neonatal myasthenia gravis
10-20% of infants born to mothers with myasthenia gravis - NO correlation to severity or duration of maternal disease IMMUNE process involving NEUROMUSCULAR JUNCTION decreased fetal movements, arthrogryposis, polyhydramnios, pulmonary hypoplasia Mean duration of illness = 18 days Require anticholinesterase therapy - neostigmine Dx: -CPK normal - EMG: progressive decline in amplitude with repetitive nerve stimulation - muscle biopsy: normal - Nerve conduction: normal
84
Congenital neonatal myasthenia gravis
Genetic defect of the NEUROMUSCULAR JUNCTION Congenital myasthenia: - AR, deficiency of endplate acetylcholine receptor - less severe - sxs in first few weeks - dx features similar to acquired transient neonatal myasthenia gravis Familial infantile myasthenia: - AR, presynaptic def in acetylcholine synthesis or packaging into vessicles - can be severe - hypotonia, respiratory failure, apnea, severe feeding difficulties, facial weakness, ptosis - occulomotor fx less affected than congenital type - typically improvement with age - Dx: EMG shows fatigue with prolonged stimuatlion of faster rates
85
Congenital myotonic dystrophy | AR, chromosome 19
- caused by **expanded CTG repeat** - inherited almost entirely from mother - caused by altered protein--> **dysfunctional Na/K channels** - polyhydramnios, prolonged labor (uterine dysfunction- may be only sign in mother) - DELAYED RELEASE OF HAND GRIP Infant symptoms: - "tent-shaped" mouth, respiratory failure, hypotonia, weakness, arthrogryposis - AREFLEXIA, HYPOREFLEXIA - muscle atrophy DX: - CPK normal - EMG: "dive-bomber" sound - Muscle biopsy: small/round muscle fibers, large nuclei, sparse myofibrils - Nerve conduction: velocities normal ## Footnote ***the more severe/earlier onset of maternal disease, > risk to baby***
86
Riley-Day syndrome familial dysautonomia
AR, Ashkenazi Jews 9p31-33 locus Disorder of peripheral nervous sytem- reduced # of small unmyelinated nerves (pain, temp, taste, autonomic functions), reduced large myelinated afferent nerve fibers symptoms evident in first year of life CONFIRM: pupil constriction in response to METACHOLINE eye drops
87
fixed joints with limitation of movement
Arthrogryposis multiplex congenita management: stretching, serial casting, tendon/ligament release
88
MC etiologies of seizures
HIE ischemic stroke intracranial hemorrhage Others: metabolic, infection, developmental cerebral anomalies, drug withdrawal (heroin, methadone, barbiturates)
89
Types of seizures most commonly found in full term infants
Multifocal clonic focal clonic
90
Most frequent neonatal seizure type
subtle
91
type of seizure more commonly found in preterm infants
tonic
92
type of seizure found in preterm and full term infants
myoclonic
93
first line choice of seizure medications
phenobarbital
94
phenobarbital pharmacokinetics
barbiturate increase time that chlorid channels are open by acting on a GABA-A receptor subunits--> depresses CNS therapeutic level: 20-40 mg/L metabolism in the cytochrome P450 system in liver decreased cerebral metabolic rate oral or IV
95
most common types of congenital intracranial tumors
Teratomas > neuroepithelial tumors SUPRATENTORIAL- cerebrum region MC presentation: increas HC with bulging fontanel ## Footnote older children = INFRATENTORIAL - cerebellum/brainstem
96
Infant with hypopigmemted macules, cardiac rhabdomyoma, CNS tumors, seizures, enamel pits in teeth
Tuberous sclerosis AD, chr 9, 16 Ash leaf spots- use Wood's lamp, trunk and buttocks
97
Infant with cafe-au-lait spots, freckling in axilla, inguinal folds, perineum, macrocephaly, seizures
Neurofibromatosis syndrome AD, chr 17 cafe-au-lait spots: - dont cross midline - sharp borders - multiple, > 1.5cm - rarely present at birth, 80% by 1yr, 100% by 4yr Associated tumors: - cutaneous neurofibroma - schwannoma - pheochromocytoma
98
Bilirubin encephalopathy
athetoid CP
99
hemiplegia
unilateral involvement of one side
100
diplegia
involvement of legs only or legs > arms
101
quadriplegia
involvement of all four limbs
102
spastic CP
MOST COMMON 85-90%, 1/3 unilateral, 2/3 bilateral Increased tone increased DTRs gross motor affected fine motor/cognitive function usually not affected
103
dyskinetic/athetoid CP | extrapyramidal
repetitive, uncontrolled, involuntary movements mixed tone in same muscle dystonic- reduced activity with increased tone manifesting as stiff movements choreoathetotic- increased activity and reduced tone manifesting as uncoordinated writing/jerky movements gross AND fine motor affected cognitive function usually not affected hearing deficits speech abnormalities
104
ataxic
least common decrease tone, poor coordination decreased reflexes severe cognitive delay
105
GMFCS gross motor functional classification system
5 levels corresponding to motor abilities in children 6-12 years
106
etiologies of mental deficiency
prenatal 60-80% perinatal ~8-12% postnatal ~10%
107
IQ for mental deficiency
108
fifth day fits issue with potassium channel AD
benign familial neonatal epilepsy
109
infant with intact skull that is macrocephalic or microcephalic, illuminates with bright light, no cortical tissue, +falx cerebri, UNFUSED thalami brain stem, cerebellum, thalamus, striatum are preserved due to early in utero vascular accident no other dysmorphic features
hydrancephaly
110
x-linked hydrocephalus
due to acqueductal stenosis- acqueduct of sylvius L1CAM gene hydrocephalus with macrocephaly thin semetric rim of cortical tissue, +falx cerebri, unfused thalami
111
porencephalic cysts
fluid filled cavities (cysts filled with CSF, lined by white matter) in the brain that typically communicate with the ventricular system due to in utero vascular injury or infection leading to encephalomalacia COLA4A1- familial form
112
most common teratogen that increases the risk for holoprosencephaly
maternal diabetes
113
presence of thalamic hemorrhage suggests..
cerebral sinovenous thrombosus] MRI immediately