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
Q

Highest yield test for neonatal seizure

A

BRAIN MRI- ASAP
~90% will have diagnosis apparent on MRI

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26
Q

Prognosis following neonatal seizures

A

Etiology is the single most important prognostic factor
Higher seizure burden is associated with increased risk of poor outcome

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27
Q

Burst suppression

A

POOR prognosis
Persistent burst suppression at 72 hours in HIE carries a very poor prognosis
Neonatal onset epilepsies with burst suppression are typically ominous

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28
Q

MC localization of neonatal hypotonia

A

BRAIN

ANTERIOR HORN CELL- SMA
peripheral nerve- Riley-day
Peripheral=rare

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29
Q

MC cause of hydrocephalus in neonates

A

IVH

post hemorrhagic hydrocephalus - MC cause of obstructive and communicating hydrocephalus

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30
Q

Leads to significant increase in cerebral blood flow

A

Hypoglycemia
Hypercapnia
Anemia

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31
Q

Leads to decrease in cerebral blood flow

A

Polycythemia
Hypotension
Hypocarbia

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32
Q

When fetus becomes responsive to auditory information

A

26 weeks
Immature auditory system functional 25-29, complete by 30 weeks

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33
Q

Compared to UA, UV blood gas

A

Higher pH, lower pCO2, higher pO2
V (high, low, high)

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34
Q

Therapeutic hypothermia alters first or second energy failure most?

A

SECOND

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35
Q

When do you initiate cooling?

A

Latent phase- 1-6 hrs

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36
Q

Most common cause of CP in term neonate

A

HIE

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37
Q

HUS findings in HIE

A

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

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38
Q

Deficits from stroke

A

Motor- depends on location
Learning/behavior
Epilepsy

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39
Q

IVH epidemiology

A

Highest risk <29 weeks, <1500g
Grade 3/4 highest among youngest and smallest

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40
Q

Risk factors for IVH

A

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)

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41
Q

CP from PVL

A

SPASTIC DIPLEGIA

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42
Q

Neonate with PVL

A

oligodendrocytes affected

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43
Q

HIE leads to decreased…

A

phosphocreatinine

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44
Q

HIE type of CP

A

spastic quadriplegia

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45
Q

Learning and behavior issues in HIE after TH are indicative of injuries in what area?

A

Watershed

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46
Q

Why are preemies at risk for IVH?

A

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
Q

Why does grade IV happen?

A

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
Q

Most common area of the brain affected by PVL

A

Periventricular white matter dorsolateral to the external angles of the lateral ventricles

49
Q

How does PVL occur?

A

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
Q

Describe cystic PVL

A

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
Q

Describe diffuse PVL

A

High signal intensity in the white matter on T2 weighted MRI images

MORE COMMON than cystic PVL

52
Q

Describe Arnold Chianti malformation

A

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
Q

Describe Dandy Walker

A

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
Q

Typical EEG pattern in HIE

A

Depression or burst suppression

May have seizures that are refractory to medical therapy

55
Q

Most common cranial nerve to be injured during birth

A

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
Q

What is Lissencephaly?

A

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
Q

Highest risk for hearing loss

A

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
Q

Neurodevelopmental sequelae most likelly associated with IUGR and not prematurity

A

Decreased full scale and verbal IQ

59
Q

Cerebral perfusion pressure

A

CPP = MAP - ICP

MAP = (2/3)DPB + (1/3) SBP
MAP = (CO x SVR) + CVP

60
Q

Impairment

A

structural or functional abnormality at the organ level

Can occur without a disability

61
Q

Disability

A

restriction at the personal level that results from an impairment

Can occur without a handicap

62
Q

Handicap

A

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
Q

How can you clinically diagnose an encephalocele?

A

Transillumination demonstrates lack of bony tissue
Mass will enlarge with straining or crying

64
Q

Meckel syndrome is due to a defect in what?

A

Cilia!

secondary to the ciliary abnormality,
TRIAD: occipital encephalocele, multicystic dysplastic kidneys and polydactyly

65
Q

Diagnosis of prenatal fetal ventriculomegaly

A

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
Q

primary/secondary neurulation
dorsal induction

A

Primary: 3-4 weeks
Secondary: 4-7 weeks

Issues during primary:
anencephaly
myeloschisis
encephalocele
myelomeningocele
Arnold-Chiari

67
Q

prosencephalic development
ventral induction

A

2-3 months

Issues:
Formation: aprosencephaly
Cleavage: holoprosencephaly
Midline development: agenesis of the corpus callosum, agnesis/absence of septum pellucidum, septo-optic dysplasia

68
Q

neural and glial proliferation

A

3-4 months

Micrencephaly
Macrencephaly

69
Q

neuronal migration

A

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
Q
A

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
Q

complete failure of midline division
FUSED thalami, ABSENT falx cerebri

A

holoprosencephaly

72
Q

Neuronal organization

A

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
Q

Myelination

A

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
Q

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)

A

Type I

can be associated with skeletal anomalies (scoliosis)

75
Q

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

A

Type II

often associated with hydrocephalus and myelomeningocele

76
Q

Pattern of injury in hypoglycemia

A

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
Q

Most common cranial nerve to be injured during birth

A

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
Q
A

upper motor neuron vs lower motor neuron

79
Q

Disorders of anterior horn cell

A

Hypoxic-ischemic myelopathy
SMA Type I- Werdnig-Hoffman Disease
Neurogenic arthogryposis

80
Q

Disorders of the neuromuscular junction

A

Acquired transient neonatal myasthenia gravis
congenital myasthenia gravis

Diseases involving a defect in acetylcholine release:
- Clostridium tetani
- Clostridium botulinum
- hypermagnesemia
- aminoglycoside toxicity

81
Q

Disorders of the muscle

A

congenital myopathy- myotubular myopathy

muscular dystrophy
- congenital myotonic dystrophy
- duchenne dystrophy
- congenital muscular dystrophy

82
Q

SMA Type 1
Werdnig-Hoffmann disease

AR, chromosome 5

A

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
Q

Acquired transient neonatal myasthenia gravis

A

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
Q

Congenital neonatal myasthenia gravis

A

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
Q

Congenital myotonic dystrophy

AR, chromosome 19

A
  • 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

the more severe/earlier onset of maternal disease, > risk to baby

86
Q

Riley-Day syndrome
familial dysautonomia

A

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
Q

fixed joints with limitation of movement

A

Arthrogryposis multiplex congenita

management: stretching, serial casting, tendon/ligament release

88
Q

MC etiologies of seizures

A

HIE
ischemic stroke
intracranial hemorrhage

Others:
metabolic, infection, developmental cerebral anomalies, drug withdrawal (heroin, methadone, barbiturates)

89
Q

Types of seizures most commonly found in full term infants

A

Multifocal clonic
focal clonic

90
Q

Most frequent neonatal seizure type

A

subtle

91
Q

type of seizure more commonly found in preterm infants

A

tonic

92
Q

type of seizure found in preterm and full term infants

A

myoclonic

93
Q

first line choice of seizure medications

A

phenobarbital

94
Q

phenobarbital pharmacokinetics

A

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
Q

most common types of congenital intracranial tumors

A

Teratomas > neuroepithelial tumors

SUPRATENTORIAL- cerebrum region

MC presentation: increas HC with bulging fontanel

older children = INFRATENTORIAL - cerebellum/brainstem

96
Q

Infant with hypopigmemted macules, cardiac rhabdomyoma, CNS tumors, seizures, enamel pits in teeth

A

Tuberous sclerosis

AD, chr 9, 16

Ash leaf spots- use Wood’s lamp, trunk and buttocks

97
Q

Infant with cafe-au-lait spots, freckling in axilla, inguinal folds, perineum, macrocephaly, seizures

A

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
Q

Bilirubin encephalopathy

A

athetoid CP

99
Q

hemiplegia

A

unilateral

involvement of one side

100
Q

diplegia

A

involvement of legs only or legs > arms

101
Q

quadriplegia

A

involvement of all four limbs

102
Q

spastic CP

A

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
Q

dyskinetic/athetoid CP

extrapyramidal

A

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
Q

ataxic

A

least common

decrease tone, poor coordination
decreased reflexes
severe cognitive delay

105
Q

GMFCS
gross motor functional classification system

A

5 levels corresponding to motor abilities in children 6-12 years

106
Q

etiologies of mental deficiency

A

prenatal 60-80%
perinatal ~8-12%
postnatal ~10%

107
Q

IQ for mental deficiency

A
108
Q

fifth day fits
issue with potassium channel
AD

A

benign familial neonatal epilepsy

109
Q

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

A

hydrancephaly

110
Q

x-linked hydrocephalus

A

due to acqueductal stenosis- acqueduct of sylvius

L1CAM gene

hydrocephalus with macrocephaly

thin semetric rim of cortical tissue, +falx cerebri, unfused thalami

111
Q

porencephalic cysts

A

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
Q

most common teratogen that increases the risk for holoprosencephaly

A

maternal diabetes

113
Q

presence of thalamic hemorrhage suggests..

A

cerebral sinovenous thrombosus]
MRI immediately