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Lecom pathology year 2 > chapter 28- CNS > Flashcards

chapter 28- CNS Flashcards

1
Q

is pilocytic astrocytoma benign or malignant

A

benign

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2
Q

how does craniopharyngioma usually present

A

supratentiral mass in kid or young adult with bitemporal hemiangiopsia

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3
Q

where does pilocytic astrocytoma typically occur

A

cerebellum

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4
Q

Describe the CSF with multiple sclerosis

A

Elevated protein, 1/3 pleocyotsis, increased IgG

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5
Q

What are 80% of adult primary brain tumors

A

Infiltrating astrocytomas

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6
Q

What is elevated in the serum of adrenoleukosystrophy

A

VLCFAs

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7
Q

how does cerebral aqueduct stenosis present

A

enlarging head circumference

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8
Q

Where do choroid plexus papilloma typically occur in kids vs adults

A

Kids in lateral ventricles; adults in 4th ventricle

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9
Q

what is the term for cystic degeneration of spinal cord

A

syringomyelia

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10
Q

what should you think of if a tumor is resenthol fiber positive and eosinophilic with GFAP positive

A

pilocytic astrocytoma

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11
Q

Where are infiltrating astrocytomas usually found

A

Cerebellum

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12
Q

what is the term for absence of skull and brain that has a frog-like appearance of the fetus

A

anencephaly

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13
Q

How does freidreich ataxia general begin

A

1st decade of life with gait ataxia, followed by hand clumsiness and dysarthria

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14
Q

What grade is a diffuse astrocytoma

A

2

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15
Q

what type of people are more likely to get CJD

A

those getting human growth hormone or that have received a corneal transplant

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16
Q

what is vascular dementia due to

A

HTN, atherosclerosis, or vasculitis

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17
Q

Where is brain lesion associated with toxoplasmosis most often found

A

Cerebral cortex and deep gray nuclei

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18
Q

what artery is often ruptured with epidural hematoma

A

middle meningeal artery

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19
Q

what is deficient in metachromatic leukodystrophy

A

aryl sulfatases

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20
Q

what is the disease defined as degeneration of GABAergic neurons in caudate nucleus of basal ganglia

A

huntington disease

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21
Q

what is the term for slow, involuntary snack like movements of fingers

A

athetosis

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22
Q

What type of astrocytoma is defined as poorly defined, gray, infiltrative tumors that expand and distort the invaded brain; GFAP positive

A

Diffuse astrocytoma

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23
Q

what is the term for inherited mutations in enzymes necessary for production or maintenance of myelin

A

leukodystrophies

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24
Q

what is the presentation of Werdnig-Hoffman disease

A

floppy baby

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25
Q

what does the CT look like for epidural hematoma

A

lens shaped lesion on CT

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26
Q

What is the most important histopathologic indicators of CNS injury

A

Gliosis

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27
Q

what does anencephaly result in

A

maternal polyhydramnios

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28
Q

what is deficient in Krabbe disease

A

galactocerebroside beta galactosidase

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29
Q

why are people with down syndrome more likely to get alzheimers

A

because APP is on chromosome 21

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30
Q

where does the galactocerebroside accumulate in Krabbe disease

A

macrophages

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31
Q

what type of infarct does thrombotic infarction produce

A

PALE at peripheral of cortex

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32
Q

what is also known as “talk and die” syndrome

A

epidural hematoma

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33
Q

are schwannomas S100 positive or negative

A

positive

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34
Q

where do thrombotic infarcts typically occur

A

at branch points

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35
Q

what disease should you think of with butterfly glioma

A

glioblastoma multiforme

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36
Q

what leukodystrophy is due to impaired addition of coenzyme A to long chain fatty acids

A

adrenoleukodystrophy

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37
Q

what is the most common benign tumor of CNS

A

meningioma

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38
Q

Where are medulloblastomas in kids vs adults

A

Kids in midline of cerebellum; in adults it is more lateral locations

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39
Q

what is control pontine myelinolysis due to

A

rapid IV correction of hyponatremia

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40
Q

What is the risk factor most commonly associated with lobar hemorrhages

A

Cerebral amyloid angioplasty

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41
Q

what distinguishes Amyotrophic lateral sclerosis from syringomyelia

A

lack of sensory impairment in ALS

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42
Q

what do subfalcene herniations compress

A

anterior cerebral artery

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43
Q

Who are lafora bodies found in

A

Cytoplasm of neurons in myoclonic epilepsy

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44
Q

what is the term for damage to the anterior horn due to poliovirus

A

poliomyelitis

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45
Q

what should you think of with whorled appearance of cells and psamomma bodies in CNS

A

meningioma

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46
Q

What is the term for minute aneurysms which may be at site of rupture

A

Charcot-Bouchard microaneurysms

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47
Q

What are the most common offending organisms in brain abscess

A

Streptococci and staphylococci

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48
Q

What is a tauopathy in affected individuals that commonly develop progressive truncal rigidity, disequilibrium with frequent falls and difficulty with voluntary eye movements

A

Progressive supranuclear palsy

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49
Q

what occurs on EEG with CJD

A

spike waves

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50
Q

What is the term for incomplete separation of cerebral hemispheres across the midline

A

Holoprosencephaly

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51
Q

What is diagnostic for multiple system atrophy

A

Glial cytoplasmic inclusions

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52
Q

What neuronal inclusion is associated with herpetic infection

A

Cowdry body

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53
Q

what is the most common cause of meningitis in kids and teenagers

A

N. meningitidis

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54
Q

what is the term for congenital failure of cerebellar vermis to develop

A

dandy-walker malformation

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55
Q

When are red neurons seen

A

Acute neuronal injury

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56
Q

what cranial nerve do uncal herniations compress

A

CN III

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57
Q

what is the term for degeneration of upper and lower motor neurons

A

amyotrophic lateral sclerosis

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58
Q

what lobe do oligodendrogliomas occur

A

frontal

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59
Q

what CNS tumor has fried egg appearance

A

oligodendroglioma

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60
Q

Where do most intracerebral/ intraparenchymal hemorrhage occur

A

Putamen

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61
Q

what is an early sign of amyotrophic lateral sclerosis

A

atrophy and weakness of hands

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62
Q

What is early sign of amyotrophic lateral sclerosis

A

Asymmetric weakness of hands

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63
Q

what does the lumbar puncture for multiple sclerosis show

A

increased lymphocytes, increased Ig with oligoclonal IgG bands, and myelin basic protein

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64
Q

What is a X-linked polyglutamine repeat expansion disease characterized by distal limb amyoptrophy and bulbar signs associated with degeneration of LMNs in the spinal cord and brainstem

A

Bulbospinal atrophy (Kennedy syndrome)

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65
Q

what is the term for inherited degeneration of anterior motor horn

A

Werdnig-Hoffman disease

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66
Q

what is found within the cels with glioblastoma multiform

A

GFAP

67
Q

What syndrome has synchronous B/L optic neuritis and spinal cord demyelination

A

Neuromyelitis optics

68
Q

What is the term for depressed, retracted, yellowish-brown patches involving crests of gyri, most commonly those that are located at site of contrecoup injury

A

Plaque Jayne

69
Q

what is the most common leukodystrophy

A

metachromatic leukodystrophy

70
Q

what is the most common source of thromboembolic that causes embolic ischemic stroke

A

left side of heart

71
Q

what are early finding in liquefactive necrosis caused by ischemic stroke

A

red neurons

72
Q

What is a rare progressive clinical syndrome characterized by cognitive decline, spasticity of limbs and seizures

A

Subacute sclerosing panencephalitis

73
Q

what should you think of in a kid with a tumor that has small, round blue cells as well as homer-wright rosettes

A

medulloblastoma

74
Q

what is the presentation of dandy-walker malformation

A

massively dilated 4th ventricle with absent cerebellum; often accompanied by hydrocephalus

75
Q

What is the clinical pattern associated with Multiple system atrophy and what is the inclusion seen

A

Parkinsonism, cerebellar ataxia, autonomic failure; alpha-synuclein

76
Q

what is the most common cause of meningitis in adults and elderly

A

strep pneumonia

77
Q

What is known as the batwing deformity

A

Agenesis of corpus callosum

78
Q

what are the most common causes of meningitis in neonates

A

GBS, E. coli and L. monocytogenes

79
Q

What is shown on the external exam of a brain of someone who has rabies encephalitis

A

Intense edema and vascular congestion

80
Q

What grade is a pilocytic astrocytoma

A

1

81
Q

what is the most common CNS tumor in kids

A

pilocytic astrocytoma

82
Q

What is the hallmark finding in progressive supranuclear palsy

A

Presence of tau-containing inclusions in neurons and glia

83
Q

What grade is a anaplastic astrocytoma

A

3

84
Q

Where do ependymomas typically occur

A

Near 4th ventricle

85
Q

what is the triad that is associated with normal pressure hydrocephalus

A

urinary incontinence, gait instability, dementia

86
Q

What do mutations in DCX involved in neuronal heterotopias cause

A

Lissencephaly in males and subcritical band heterotopias in females

87
Q

what sensory loss occurs with syringomyelia

A

pain and temperature

88
Q

What should you think of with Lisch nodules and cafe au lait spots

A

Neurofibromatosis type 1

89
Q

who does hyaline arteriolosclerosis occur in

A

benign HTN and DM

90
Q

What disease should you think of with Kuru plaques

A

Variant CJD

91
Q

What is the term for tangled network of wormlike vascular channels with prominent, pulsation arteriovenous shunting with high blood flow

A

Arteriovenous malformation

92
Q

What do the inclusions associated with subacute sclerosing panencephalitis contain

A

Nucleocapsids characteristic of measles

93
Q

What is the common presentation with adrenoleukosystrophy

A

Involves ABCD1; young males presenting with behavioral changes and adrenal insufficiency

94
Q

What grade of lesion is gliomatosis cerebri

A

Grade 3/4

95
Q

what disease is athetosis associated with

A

huntington disease

96
Q

what is a benign tumor of arachnoid cells

A

meningioma

97
Q

What should you think of with candle-guttering

A

Tuberous sclerosis

98
Q

Who is korsakoff syndrome common in

A

Chronic alcoholics

99
Q

how does huntington disease present

A

chorea; progress to dementia and depression

100
Q

What disease is a leukodystrophy associated with mutations in the gene encoding GFAP, abundant rosenthal fibers found in periventricular, perivbascular and subpial locaitons

A

Alexander disease

101
Q

What grade is glioblastoma

A

Grade 4

102
Q

What is a disease of infancy characterized by lactic academia, arrest of psychomotor development, feeding problems, seizures, extraocular palsies and weakness with hypotonia

A

Leigh syndrome (subacute necrotizing encephalopathy

103
Q

what is the classic triad with meningitis

A

headache, neck stiffness, fever

104
Q

What does methanol preferentially affect

A

Retina

105
Q

what is the term for congenital extension of cerebellar tonsils through foramen magnum

A

arnold-chiari malformation

106
Q

What is mutated in fatal familial insomnia

A

Specific mutation in PRNP gene (aspartate substitute for asparagine at residue 178)

107
Q

What is the typical presentation associated with HSV-1 encephalitis

A

Alterations in mood, memory and behavior

108
Q

what is subdural hematoma due to

A

tearing of bridging veins

109
Q

what is subarachnoid hemorrhage most commonly due to

A

rupture of berry aneurysm

110
Q

What is the clinical presentation of tuberous sclerosis

A

Seizures, autism, mental retardation

111
Q

what are the 3 most common metastasis to brain

A

lung, breast, kidney

112
Q

what is the presentation of central pontine myelinolysis

A

acute paralysis; “locked in syndrome”

113
Q

what are neural tube defects associated with low levels of prior to conception

A

folate

114
Q

What are premature infants with cerebral palsy at increased risk of

A

Intraparenchymal hemorrhage

115
Q

What mutation increase the risk of kids getting HSV-1 encephalitis

A

TLR3 mutation

116
Q

what is a degenerative disease of frontal and temporal cortexes; spares parietal and occipital lobes

A

pick disease

117
Q

What are rosenthal fibers found with

A

Regions of long standing gliosis; characteristic of pilocytic astrocytoma

118
Q

What is the histology associated with cerebral amyloid angiopathy

A

No fibrosis present; instead dense and uniform deposits of amyloid are present

119
Q

where does syringomyelia typically occur

A

C8-T1

120
Q

what are the 3 ways to develop ischemic stroke

A

thrombotic, embolic, lacunar

121
Q

What is holoprosencephaly associated with

A

Trisomy 13 and SHH mutations

122
Q

what disorder has the presentation of ataxia with loss of vibratory sense and proprioception, muscle weakness in LE, and loss of DTR

A

Friedrich ataxia

123
Q

What is altered in most CJD

A

PRNP

124
Q

What is the term for compensatory increase in ventricular volume secondary to loss of brain parenchyma

A

Hydrocephalus ex vacuo

125
Q

What is the term for a gray matter cell with a large nucleus, pale staining central chromatin, an intranuclear glycogen droplet and a prominent nuclear membrane and nucleoli

A

Alzheimer’s type 2 astrocyte (not associated with Alzheimer’s disease)

126
Q

What is the diagnostic marker for Parkinson’s disease

A

Lewy body

127
Q

what virus can cause subacute sclerosing pan encephalitis

A

measles virus

128
Q

what is the most common cause of dementia

A

Alzheimers disease

129
Q

What is the clinical pattern associated with amyotrophic lateral sclerosis

A

Weakness with upper and lower motor neuron signs

130
Q

what vessels are most commonly involved in lacunar ischemic stroke

A

lenticulostriate vessels

131
Q

what artery is usually involved in embolic ischemic stroke

A

middle cerebral artery

132
Q

what is a lethal complication of epidural hematoma

A

herniation

133
Q

what is the most common malignant CNS tumor in adults

A

glioblastoma multiforme

134
Q

What is the term for perinatal ischemic lesions of cerebral cortex, resulting in depths of sucking bear the brunt of injury and result in thinned-out, gliotic gyri

A

Ulegyria

135
Q

What is the term for congregation around cell bodies of dying neurons

A

Neuronophagia

136
Q

What is the term for non-progressive neurologic motor deficit characterized by combinations of spasticity, dystopia, ataxia/athetosis and paresis

A

Cerebral palsy

137
Q

What is the term for aberrant and irregular myelinatization gives rise to marble like appearance of deep nuclei

A

Status marmoratus

138
Q

What should youthink of with bunina bodies

A

Amyotrophic lateral sclerosis

139
Q

what is the presentation of CJD

A

rapidly progressive dementia associated with ataxia and startle myoclonus

140
Q

What is the presentation of infiltrating astrocytomas

A

Seizures, headaches, focal neurologic deficits

141
Q

What is the term for infarcts in supratentorial periventricular white matter

A

Periventricular leukomalacia

142
Q

what are the lewy bodies associated with parkinson disease composed of

A

alpha- synuclein

143
Q

what is a high grade malignant tumor of astrocytes

A

glioblastoma multiforme

144
Q

What is a foci of hermitage and necrosis in mammary bodies and walls of 3rd and 4th ventricles associated with Thiamine

A

Wernicke encephalopathy

145
Q

what HLA is associated with multiple sclerosis

A

HLA-DR2

146
Q

what virus is needed for progressive multifocal leukoencephalopathy

A

JC virus infected oligodendrocytes

147
Q

what does lacunar ischemic stroke occur secondary to

A

hyaline arteriolosclerosis

148
Q

what is the most common site of intracerebral hemorrhage

A

basal ganglia

149
Q

what does imaging of meningioma reveal

A

round mass attached to dura (will press on cortex but does NOT invade)

150
Q

What is the term for thick, elongated, brightly eosinophilic, irregularly structures that occur within astrocytic process and contain alphaB-crystallin and hsp27

A

Rosenthal fibers

151
Q

What astrocytoma is a predominant neoplastic astrocyte that shows brightly eosinophilic cell body from which emanate abundant, stout processes

A

Gemistocytic astrocytoma

152
Q

what is the clinical presentation associated with parkinson disease

A

TRAPS: tremor, rigidity, Akinesia/bradykinesia, Postural instability, shuffling gait

153
Q

What antibodies are common in neuromyelitis optics

A

Antibodies against aquaporin 4 (the major water channel in astrocytes)

154
Q

What disorder is associated with molar tooth sign on imaging

A

Joubert syndrome

155
Q

What genetic thing is most associated with friedreich ataxia

A

Frataxin mutations, GAA trinucleotide repeat, cardiomyopathy

156
Q

how are neural tube defects detected during prenatal care

A

by increased AFP in amniotic fluid and maternal blood

157
Q

What is the common site for arteriovenous malformations

A

Territory of middle cerebral artery

158
Q

what is central pontine myelinolysis

A

focal demyelination of pons

159
Q

What is the disease that is venous angiomatous malformation of spinal cord and overlying meninges

A

Foix-alajouanine disease

160
Q

What does Waterhouse-Friderichsen syndrome result from

A

Meningitis–associated septicemia with hemorrhagic infarction of adrenal glands and cutaneous petechiae

161
Q

What neuronal inclusion is associated with rabies

A

Negri body

162
Q

What is the most striking alteration in fatal familial insomnia

A

Neuronal loss and reactive gliosis in anterior ventral and dorsomedial nuclei of thalamus. Neuronal loss is also prominent in inferior olivary nuclei

163
Q

What is the clinical pattern associated with spinal bulbar muscular atrophy

A

Lower motor neuron weakness, diminished androgen

Lecom pathology year 2 (18 decks)

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