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Pediatric Imaging > Neuroradiology > Flashcards

Neuroradiology Flashcards

1
Q

Myelination begins at what month of life

A

5th

Continuous throughout life

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2
Q

In a normal infant, myelination appears complete at what MR Sequence

A

T1

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3
Q

Myelination appears complete on T2 by what month of age

A

24

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4
Q

Myelination appears complete on FLAIR at what age

A

40 months

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5
Q

In term neonate, we should see T1 hyperintense myelin in what parts of the brain

A

Deep cerebellar WM, dorsal brainstem, posterior limbs of internal capsules

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6
Q

Genu and splenium of corpus callosum are myelinated by what month of age

A

6 months

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7
Q

By what age is the appearance of white matter myelination in T1 similar to that of an adult

A

1 year

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8
Q

Interhemispheric falx become present with broad Sylvian fissure is seen at what fetal age

A

18 months

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9
Q

More operculization of the Sylvian fissures and calcarine fissures start to form by what fetal age

A

22 weeks

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10
Q

The calcarine fissure are well formed at what fetal age

A

26 weeks

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11
Q

The temporal and frontal sulci are formed or forming by what fetal age

A

30 weeks

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12
Q

What fetal age does all the primary sulci form

A

34 weeks

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13
Q

Sylvian fissure becomes visible at what GA

A

16-18

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14
Q

Parieto-occipital fissure becomes visible at what GA

A

20-22

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15
Q

Calcarine fissure and callosal sulcus becomes visible at what GA

A

22-23

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16
Q

Central sulcus and cingular sulcus become visible at what GA

A

24-25

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17
Q

Precentral sulcus become visible by what GA

A

26 weeks

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18
Q

Post central sulcus and superior temporal sulcus becomes visible at what GA

A

27 weeks

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19
Q

Superior and inferior frontal sulci are visible at what GA

A

29 weeks

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20
Q

Inferior temporal sulcus is visible at what GA

A

33 weeks

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21
Q

In complete agenesis of corpus callosum, the lateral ventricles have a parallel orientation, with posterior dilatation termed _______

A

Colpocephaly

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22
Q

What midline development abnormality demonstrates upturned anterior horns of lateral ventricle, absence of cingulate sulcus allowing the interhemispheric sulci to extend all the way to the 3rd ventricular margin with underrotation of the hippocampi—- in coronal images

A

Complete agenesis of corpus callosum

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23
Q

“Texas longhorn” appearance of lateral ventricles in coronal view can be seen in what midline abnormality

A

Complete agenesis of corpus callosum

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24
Q

Midline abnormality presenting as truncation of the anterior-posterior dimension, that can be segmental, with the callosum appearing as two separate commisures

A

Partial agenesis or hypogenesis of the corpus callosum

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25
Q

Midline abnormality that is frequently associated with interhemispheric cysts or lipomas

A

Partial agenesis or hypogenesis of the corpus callosum

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26
Q

Most common cortical development malformation associated with septo-optic dysplasia

A

Schizencephaly

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27
Q

Consist of optic nerve hypoplasia and partial or complete agenesis of septum pellucidum, dysgenesis of corpus callosum, anomalies of hypothalamic pituitary axis and malformations of cortical development, most notably schizencephaly

A

Septo-optic dysplasia

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28
Q

Encompasses a spectrum of malformations caused by abnormalities of differentiation and midline cleavage of prosencephalon during the 5th gestational week

A

Holoprosencephaly

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29
Q

Hallmarks of holoprosencephaly

A
  • Abnormal communication of gray and/or white matter across midline
  • facial dysmorphism (hypotelorism, cyclopia, ethmocephaly, cebocephaly, and midline cleft lip/palate
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30
Q

Complete absence of cleavage with anterior displacement of cerebral tissue into a “pancake” configuration and a large monoventricle that expands posteriorly into a dorsal cyst

A

Alobar holoprosencephaly

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31
Q

Presence of cleavage where some differentiation of posterior lateral ventricles is evident

A

Semilobar holoprosencephaly

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32
Q

Type or holoprosencephaly where the anterior falx cerebri can be partially present

A

Lobar holoprosencephaly

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33
Q

Interhemispheric variant of holoprosencephaly where there is lack of separation of the posterior frontal and parietal regions with cleavage of anterior frontal and occipital lobes

A

Syntelencephaly

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34
Q

Most commonly encountered chiari malformation

A

1 and 2

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35
Q

Rare chiari malformation, characterized by a cervico-occipital encephalocele containing posterior fossa contents

A

Chiari 3

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36
Q

Characterized by cerebellar tonsillar protrusion below the foramen magnum in the absence of an open spinal dysraphism or intracranial mass effect

A

Chiari 1

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37
Q

Degree of tonsillar descent necessary for the diagnosis of chiari 1 (McRae line)

A

> /= to 5mm below a line drawn from the basion to opisthion

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38
Q

Important finding in chiari malformation as this is the main cause of neurologic symptoms and deficits

A

Syringomyelia

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39
Q

Surgical treatment for chiari malformation

A

Posterior fossa decompression with suboccipital craniectomy and C1 laminectomy alone, or with the addition of duraplasty, cerebellar tonsil cautery and CSF shunting

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40
Q

Other associated symptoms in chiari malformation include

A
  • Abnormal morphollogy of cerebellar tonsils
  • Dorsal tilt of dens
  • Basilar invagination
  • Platybasia
  • Effacement of CSF spaces at the foramen magnum
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41
Q

Chiari 1 findings with open spinal dysraphism (myeloneningocele or myelocele). It can be identified on prenatal ultrasound by bifrontal concavity of the calvarium (lemon sign) and a flattened cerebellum wraping around the brainstem (banana sign)

A

Chiari 2

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42
Q

Post natal imaging findings of chiari 2 malformation

A
  • Small posterior cranial fossa with a downward sloping tentorium and herniation of the cerebellar tissue into the cervical canal
  • medullary “kink” dorsal to the upper cervical spinal cord
  • tectal beaking
  • falx insufficiency (interdigitating hemispheric sulci)
  • stenogyria
  • subependymal gray matter heterotopia
  • hypogenesis of corpus callosum
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43
Q

Chiari 2 malformation prenatal repair

A

Prenatal repair of myelomeningocele

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44
Q

Groupd of posterior fossa malformations includes a number of abnormalities characterized by varying degrees of vermian hypoplasia and malposition in association with posterior fossa cysts

A

Dandy walker continuum

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45
Q

Triad of complete or partial agenesis of the vermis, cystic dilation of fourth ventricle and an enlarged posterior fossa with upward displacement of tentorium

A

Classic dandy-walker malformation

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46
Q

Angiographic key finding to the diagnosis of dandy walker malformation

A

Torcular -lambdoid inversion

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47
Q

The hypoplastic vermis in dandy walker malformation is often rotated counterclockwise such that the tegmento-vermian angle formed by lines drawn along the brainstem and ventral vermis is ____ degrees

A

> 18 degrees

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48
Q

Structures to be checked in dandy walker continuum

A

Posterior fossa size
Vermis size
T-V angle

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49
Q

Embryonic structure that perforates in the first trimester to form the foramen of Magendie and foramina of Luschka

A

Blake pouch

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50
Q

Upward displacement of an otherwise normally formed cerebellar vermis

A

Blake pouch remnant

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51
Q

Blake pouch remnant is considered normal up to what AOG

A

20 weeks

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52
Q

Believed to be caused by delayed fenestration of blake pouch

A

Mega cisterna magna

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53
Q

Heterogeneous group of disorders mostly resulting from mutations of genes encoding ciliary proteins

A

Joubert Syndrome and related disorders

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54
Q

These patients have episodes of irregular breathing during infancy (panting or laughing respirations) with oculomotor apraxia

A

Joubert syndrome

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55
Q

Classic neuroimaging findings incluse vermian hypoplasia and “molar tooth sign”, where the thickening and elongation of the superior cerebellar peduncles resemble the root of an extracted molar tooth, hypoplasia or absence of lower brainstem nuclei, polydactyly, colobomas and polycystic kidneys

A

Joubert syndrome

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56
Q

Incomplete separation of the cerebellar hemispheres in association with partial or complete absence of the vermis

A

Rhombencephalosynapsis

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57
Q

Up to 65% of patients with rhombencephalosynapsis has coexisting _____

A

Aqueductal stenosis

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58
Q

MR imaging of this entity shows transversely oriented continuous folia and flattened fastigial recess causes by atleast partial absence of cerebellar vermis

A

Rhombencephalosynapsis

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59
Q

Three components of cortical development malformations

A

Disturbances of cellular proliferation, cellular migration and/or cortical organization

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60
Q

Most severe manifestation of decreased cellular proliferation in which there is near-complete absence of the sulci and gyri with marked microcephaly

A

Microlissencephaly

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61
Q

Milder phenotype of microlissencephaly where the brain is smaller than normal, with a reduced number of simple appearing gyri

A

Microcephaly with simplified gyral pattern

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62
Q

Hamartomatous overgrowrh of part or all of a cerebral hemisphere

A

Hemimegalencephaly

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63
Q

Appears as paradoxically enlarged lateral ventricle, thickened and ill-defined cortixal ribbon and mineralization and neuronal heterotopia causing “white matter” to appear prematurely myelinated

A

Hemimegalencephaly

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64
Q

Treatment of choice for hemimegalencephaly

A

Functional hemispherotomy (surgical disconnection of the entire affected hemisphere)

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65
Q

Leading cause of medically refractory epilepsy in children

A

Type 2 focal cortical dysplasia

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66
Q

Appears as focal cortical thickening, blurring of the gray-white matter junction, and a funnel-shaped white matter signal abnormality extending fromt he subcortical region toward the ventricle

A

Type 2 focal cortical dysplasia

67
Q

Result from the arrest of the radial migration of neurons from the germinal matrix along the walls of the ventricles to the developing cortex during migrational phase. Appears as nodules of tissue that parallel normal gray matter in signal intensity on all pulse sequences

A

Gray matter heterotopia

68
Q

A genetically driven migrational arrest that typically appears as smooth symmetric bands of gray matter found between the ventricular surface and cerebral cortex

A

Band heterotopia

69
Q

Disorder in which the overlying cortex is either normal or along the lissencephaly/pachygyria spectrum and nearly all affected patients are females

A

Band heterotopia

70
Q

Result of arrested neuronal migration resulting in an abnormally thickened cortex and absence of normal gyral-sulcal pattern

A

Lissencephaly

71
Q

Complete absence of gyri/sulci, with an “hour-glass” or “figure of 8” appearance due to broad and shallow Sylvian fissures

A

Agyria

72
Q

The gyri are present but abnormally broad, with shallow sulci. Characteristic feature is the cell-sparse zone, a very thin band of white matter signal separating a thin outer cortical layer from a thick inner cortical layer

A

Pachygyria

73
Q

Results from abnormalities of late neuronal migration and cortical organization, causing an increased number of abnormally small gyri

A

Polymicrogyria

74
Q

Most commonly, polymicrogyria is seen focally if not diffuse in the

A

Perisylvian

75
Q

Reflects prevention of neuronal migration from the germinal matrix outward, resulting in transmantle cleft lined by dysplastic gray matter extending from the ependyma to the pial surface

A

Schizencephaly

76
Q

Type of schizencephaly where the cortices of the cleft are immediately apposed

A

Closed lip schizencephaly

77
Q

Type of schizencephaly where the csf communicates between the ventricle and overlying subarachnoid space

A

Open lip

78
Q

Commonly associated with schizencephaly in unilateral cases

A

Contralateral polymicrogyria

79
Q

Schizencephaly is commonly bilateral or unilateral

A

Bilateral in 50% of cases

80
Q

Extremely preterm

A

Less than 28 weeks

81
Q

Very preterm

A

28-32 weeks

82
Q

Moderate to late preterm

A

32-37 weeks

83
Q

Majority of malformations happens at what AOG

A

12-24 weeks

84
Q

A severe in-utero brain injury generally attributes to bilateral internal carotid artery occlusion

A

Hydranencephaly

85
Q

Surviving brain in infants with hydranencephaly is supplied by the

A

Posterior circulation or meningeal branches of the external carotid artery

86
Q

Distinct pattern of white matter injury that affects the developing brain between 24-34 weeks of gestational age, caused by damage to oligodendrocyte progenitor cells. Damage results from hypoxia and ischemia superimposed upon pre-exisiting inflammatory factors

A

Periventricular leukomalacia

87
Q

PVL has strong correlation with what etiologies

A

Maternal infection and placental inflammation

88
Q

Represents as watershed zone of cerebral blood flow in the immature brain with poor autoregulation

A

Periventricular white matter

89
Q

The ability of the brain to mount a reparative response to injury is markedly limited prior to ___ weeks of gestation; as a result there is little or no gliosis

A

34 weeks

90
Q

Appearance of PVL in ultrasound

A

Abnormally hyperechoic injured white matter

91
Q

MR imaging of PVL will show

A

Abnormal hyperintensity in the periventricular white matter on T1

92
Q

Characteristic angular pattern of ventricular expansion becomes manifest in what entity, after cystic changes and break down of cystic walls

A

PVL

93
Q

Study of choice to diagnose ischemic injury in infants

A

MRI

94
Q

These receptors are overexposed in the developing brain and are concentrated in the thalamic and subthalamic nuclei.

A

Glutamate and N-methyl-d-aspartate

95
Q

Nature of Deep nuclear injury

A

Excitatory neurotoxicity:
During severe hypoxia/ischemic injury, glutamate and NMDA receptors remain open, causing influx of calcium and subsequent neuronal death

96
Q

Watershed or boarder zones

A

Between anterior and middle, middle and posterior artery territories

97
Q

When infants prior to 18 months of age show early hand preference, what brain injury is present

A

Diffuse and peripheral injury

98
Q

Hereditary syndromes that are grouped together because they primarily affect structures of ectodermal origin, that is the nervous system and skin

A

Phakomatoses

99
Q

Most common of phakomatoses

A

NF 1

100
Q

NF 1 is linked to what chromosomal abnormality which codes for neurofibromin

A

Chromosome 17

101
Q

Has numerous functions including tumor suppression, myelination, neuronal and astrocytic development. Also has expression in osteoblasts and vascular endothelium

A

Neurofibromin

102
Q

Termed as peripheral neurofibromatosis, witg multiple cutaneous lesions (cafe au lait spots, axillary freckling, cutaneous neurofibromas and Lisch nodules)

A

NF1

103
Q

Age at presentation of NF1

A

Childhood

104
Q

Age at presentation of NF2

A

Young adult

105
Q

Optic gliomas, dural ectasia, brain T2 hyperintensities and neurofibromas can be found in what phakomatosis

A

NF1

106
Q

Schwannomas and spinal glial tumors are found in what phakomatosis

A

NF2

107
Q

Malignant degenaration of nerve sheath tumor and plexiform neurofibromas are seen in what phakomatosis

A

NF1

108
Q

Skeletal findings such as scoliosis, sphenoid dysplasia, thinning bone cortex (ribbon ribs) and vascular dysplasia can be found in what phakomatosis

A

NF1

109
Q

Brain T2 hyperintensities in NF1 can be found in what areas of the brain.

A

Basal ganglia, thalami, deep cerebral white matter, cerebellum

110
Q

Spongiform changes due to abnormal myelin vacuolization in pathology are seen in what phakomatosis

A

NF1

111
Q

Distinctive bone lesions in NF1

A

Sphenoid dysplasia, long bone cortical thickening, +/- pseudoarthrosis

112
Q

Fusiform enlargement of the optic nerves with variable enhancement and extension to optic chiasm, and may also spread dorsally into the optic tracts and adjacent brain

A

Optic pathway gliomas

113
Q

Most common form of optic gliomas related to NF1

A

Pilocytic astrocytoma

114
Q

Phakomatosis that results from mutation of chromosome 22 which affects the tumor suppressor gene merlin aka schwannomin

A

NF2

115
Q

Historically names as central neurofibromatosis, which is essentially a multiple neoplasm syndrome with few cutaneous manifestations

A

NF2

116
Q

Neurofibromas of NF1 usually found in what nerve roots

A

Exiting nerve roots

117
Q

Schwannomas from NF2 are found in what nerve roots

A

Dorsal nerve roots

118
Q

Usually has a dumbbell configuration as they pass through the neural foramina, with their medial component impinging upon the spinal cord

A

Schwannomas

119
Q

Mnemonic for NF2 MISME stands for

A

Multiple, inherited, schwannomas, meningiomas, ependymomas

120
Q

An autosomal dominant disordwr caused by mutations in the TSCI and/or TSC2 genes, which encode for hamartin and tuberin, respectively

A

Tuberous sclerosis

121
Q

Syndrome characterized by dysplastic tumor-like lesions in the brain, lungs, skin, kidneys and heart. Epilepsy is present in up to 90% of cases, 50% are cognitively impaired

A

Tuberous sclerosis

122
Q

Cutaneous lesions in tuberous sclerosis include

A

Angiofibromas and depigmented nevi (aka ash-lead spots)

123
Q

Cortical-subcortical dysplastic lesion; expanded broad polygonal gyri overlying abnormal subcortical white matter can be seen in what phakomatosis

A

Tuberous sclerosis

124
Q

Most characteristic brain lesions in patients with tuberous sclerosis are

A

Subependymal nodules that protrude inward from the outer walls of the lateral ventricles

125
Q

Subependymal nodules in TS near the caudothalamic groove may develop proliferation of the balloon cells and become what entity

A

Subependymal giant cell astrocytomas (SEGA)

126
Q

Benign gliomas of the retina (giant optic drusen or retinal astrocytoma) and giant aneurysms are seen as less common CNS manifestation of what phakomatosis

A

Tuberous sclerosis

127
Q

Unlike the classic phakomatoses, this condition is not inherited, but rather a consequence of failure of development of venous structures draining the cerebral cortex, eye, skin

A

Sturge-Weber syndrome (encephalotrigeminal angiomatosis)

128
Q

Cutaneous capillary malformation seen in sturge-weber

A

Port-wine nevus in the face

129
Q

Primitive venous drainage of the cerebrum is termed

A

Pial angioma

130
Q

Eye lesion seen in sturge-weber which can result in glaucoma and exudative retinal detachment

A

Choroidal hemangioma

131
Q

An autosomal dominant disorder consisting of retinal, cerebellar and spinal hemangioblastomas. Other features include renal cell carcinoma, pheochromocytoma, cysts in the kidneys, liver and pancreas

A

Von Hippel-lindau syndrome (CNS angiomatosis)

132
Q

Develops in 50% of patient with VHL syndrome and altho considered benign neoplasm, they are common source of symptoms and have high post surgical recurrence rate. They are well-circumscribed cystic lesion with an enhancing mural nodule

A

Hemangioblastomas

133
Q

The dorsal aspect of the embryo develops a neural plate made up of neural ectoderm, which is continuous on either side with cutaneous ectoderm at what GA

A

3rd week

134
Q

In a process called ______, the neural ectoderm folds inward and forms a tube with the center of the tube becoming the ventricles and the central canal of the spinal cord

A

Neurulation

135
Q

The complete process of detachment of the neural ectoderm from the adjacent cutaneous ectoderm and then comes together to completely cover the neural tube toward the head happens between what days of gestaton

A

24-27 days of gestation

136
Q

The exposed neural tissue if the infolding neural plate fails to detach from the adjacent cutaneous ectoderm and the central canal of the spinal cord communicates with the amniotic sac is called

A

Neural placode

137
Q

If the neural placode projects beyond the plane of the back the malformation is called

A

Myelomeningocele

138
Q

If the neural placode lays flat relative to the rest of the back it is called a

A

Myelocele

139
Q

2 open spinal dysraphisms/spina bifida aperta

A

Myelomeningocele and myelocele

140
Q

If the infolding neural plate successfully detaches from the cutaneous ectoderm but mesodermal tissue becomes trapped between the 2 edges, preventing closure of neural tube. The interspersed mesodermal tissue then prevents the normal development of posterior bony elements and differentiates into fat, which is contiguous with the subcutaneous fat and tethers the cord at the site of malformation. The condition is called

A

Lipomyelomeningocele

141
Q

If the placode covered with skin and fat does not project beyond the posterior laminar line

A

Lipomyelocele

142
Q

If the mesodermal tissue completely separates from the overlying mesoderm, the posterior elements of the bony canal can close, and the result is a

A

Intradural lipoma

143
Q

Rarely, the neural tube will successfully separate from the cutaneous ectoderm and close but a defect in the skin will persist, with a tract that descends into the subcutaneous tissues or spinal canal called a

A

Dorsal dermal sinus

144
Q

Dorsal dermal sinus may have an inclusion of skin tissue at the base of the tract, consisting of squamous epithelium alone called ______ or multiple skin elements called _____

A

Epidermoid and dermoid

145
Q

Characterized by agenesis of a portion of the caudal spine usually in association with spinal cord, anorectal and /or genitourinary anomalies

A

Caudal regression syndrome or caudal agenesis

146
Q

High risk for caudal agenesis infants

A

Diabetic mothers

147
Q

What group of caudal agenesis patients have a high (L1 or higher) blunted conus with separation of the anterior and posterior nerve roots in a “double-bundle” configuration

A

Group 1

148
Q

Group of caudal agenesis patients that has a low-lying tethered cord and may have an additional closed spinal dysraphism such as intradural lipoma or lipomyelomeningocele

A

Group 2

149
Q

Its major abnormality is a focal dilatation of the central canal that causes the cord to protrude thru a defect in the dorsal elements

A

Myelocystocele

150
Q

Myelocystocele seen in the lumbar or lumbosacral region

A

Terminal myelocystocele

151
Q

Myelocystocele seen in cervical or thoracic spine is called

A

Nonterminal myelocystocele

152
Q

A streak of cells situated between the ectoderm and endoderm that causes the development of the neural plate and subsequent infolding to form the neural tube

A

Notochord

153
Q

If the notochord is divided, 2 separate neural tubes may form, resulting into to hemicords called

A

Diastematomyelia or split cord malformation

154
Q

Two hemicords that have their own dural sac, separated by a bony spur

A

Type 1 spinal cord malformation

155
Q

2 hemicords in 1 dural sac

A

Type 2 spinal cord malformation

156
Q

Clinical diagnosis based upon the constellation of pain, gait abnormalities, sensory disturbances and/or lower extremity weakness, in association with a structural abnormality of the distal cord that could be a cause of increased tension or restricted mobility of the conus medullaris

A

Tethered cord syndrome

157
Q

Term used to describe a cystic cavity within the spinal cord, usually representing the dilated central canal. It is analogous to hydrocephalus in the brain, in that there is enlargement of a space normally occupied by CSF

A

Syringe, syringomyelia or syrinx

158
Q

Most syrinxes are the result of congenital hindbrain malformations such as

A

Chiari 1 and 2

159
Q

Normal central canal has a diameter of up to

A

3mm

160
Q

Scoliosis is most frequently seen in what gender

A

Female

161
Q

Scoliosis typically presents with a primary thoracolumbar curve convex to the

A

Right

162
Q

Defined as any lateral spinal curvature of >10 degrees

A

Scoliosis

163
Q

Common causes of nonidiopathic scoliosis

A

Vertebral anomalies such as hemivertebrae or neural anomalies such as SCM or syrinx

Pediatric Imaging (5 decks)

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