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Central Nervous System Pathology > Pathology of the CNS > Flashcards

Pathology of the CNS Flashcards

1
Q

Which gyrus gets pushed through in a subfalcine herniation?

Which artery gets occluded?

A
  • cingulate

- ACA

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2
Q

Name the 3 complications of an uncal herniation.

which membrane does the uncus get pushed through?

A
  1. duret hemorrhages
  2. compression of CNIII (rostral midbrain)
  3. compression of PCA (infarcted occipital lobe)
    - tentorium cerebelli
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3
Q

name the complication of a tonsillar herniation

A

-cardiorespiratory arrest

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4
Q

name two general causes of communicating hydrocephalus

A
  1. increased CSF production

2. decreased CSF reabsorption

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5
Q

what causes noncommunicating hydrocephalus?

-does an adults head enlarge?

A
  • obstruction of CSF flow out of the ventricles

- no

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6
Q

baby presents with massive head circumference and paralysis of upward gaze.

A

perinaud syndrome

-obstructed aqueduct of sylvius

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7
Q

why do the ventricles look bigger in hydrocephalus ex vacuo?

-in which disease is this commonly seen?

A
  • the brain shrank.

- alzheimer

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8
Q

normal pressure hydrocephalus triad

-treatment

A
  • dementia, incontinence, wide based gait

- ventriculoperitoneal shunt

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9
Q

Are neutrophils usually in the CSF? what else would you expect to find with high neutrophils?

A
  • no. this implies that there is a bacterial infection

- low glucose

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10
Q

what is in the CSF due to a viral infection

A
  • normal glucose

- lymphocytes

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11
Q

what is in the CSF due to a fungal infection

A
  • low glucose

- lymphocytes

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12
Q

Maternal findings in neural tube defects

A

-increased AFP

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13
Q

Name the three types of spina bifida

A

-occulta, meningocele, myelomeningocele

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14
Q

arnold-chiari malformation

-complication

A
  • caudal extension of medulla and cerebellar vermis through foramen magnum
  • noncommunicating hydrocephalus
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15
Q

what’s missing in a dandy-walker malformation?

-name 2 complications

A

the vermis

  • fourth ventricle gets bigger
  • noncommunicating hydrocephalus
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16
Q

which neural tube defect is commonly seen with syringomyelia?

  • which two tracts get compromised?
  • what distinguishes this from ALS?
A

arnold-chiari malformation

  • lateral spinothalamic
  • anterior horn cells
  • charcot joints in the UE (progressive neuroarthropathy)
  • sensory changes
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17
Q

what are phakomatoses?

-name three examples

A
  • neurocutaneous disorders

- nuerofibromatosis, tuberous sclerosis, Sturge-Weber syndrome

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18
Q

Newborn presents with optic gliomas, lisch nodules, and axillary/inguinal freckling.

  • DX
  • what tumors are they at risk for?
  • chromosome?
A
  • NF1
  • pheochromocytoma, wilms tumor, juvenile CML
  • 17
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19
Q

child presents with bilateral sensorineural hearing loss, juvenile cataracts, and meningiomas

  • DX
  • which CN is affected?
  • chromosome and protein affected
A
  • NF2
  • CN VIII, schwannoma
  • c22, merlin protein
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20
Q

tuberous sclerosis is multiple neural tube defects separated by space. how would an infant present?
-what are they at risk for?

A
  • mental retardation and seizures

- hamartomatous lesions of the kidneys and heart

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21
Q

Child is born with a bright red vascular malformation covering a unilateral trigeminal nerve distribution. Whats the DX
Think of the mnemonic for the rest of the clinical findings

A
  • SWS

- STURGE: Sporadic, Stain, Tram track Ca, Unilateral, Retardation, Glaucoma, GNAQ gene, Epilepsy

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22
Q

name the 3 most frequent arteries involved in atherosclerotic (thrombotic) strokes

A
  • MCA by far
  • ICA near the bifurcation
  • basilar
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23
Q

what are the 4 types of strokes?

for a bonus, name the two subtypes of ischemic stroke.

A
  1. ischemic (thrombotic and embolic)
  2. intracerebral hemorrhage
  3. subarachnoid hemorrhage
  4. lacunar stroke
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24
Q

Which portion of the brain is usually affected by thrombotic strokes?

A

the periphery of the cerebral cortex

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25
Q

Pt. with history of TIAs has been placed on anti-platelets after ruling out hemorrhagic stroke. She is brought to the ER weeks later with R hemiparesis, L sensory loss in the face, and is having difficulty verbalizing. What do you suspect?
What type of visual field defect could this pt have?
Any other eye abnormalities?

A
  • thrombotic stroke of the L MCA
  • R homonymous hemianopsia
  • left eye gaze
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26
Q

-how would a pt present with a L ACA stroke?

A

-R hemiparesis and sensory loss leg>arm

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27
Q

pt. presents with R hemiparesis and sensory loss in the trunk and limbs, L sensory loss in the face, and vertigo with ataxia. where is the stroke?

A

-the L vertebral artery

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28
Q

mom and dad, where do embolic strokes come from???

A

the left side of the heart, sweetheart. that is, unless there a R to L shunt in the heart, then, quite frankly, it could come from just about anywhere in the body. now go to sleep.

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29
Q

what’s a major risk factor for embolic stroke?

A

-MI and A-fib

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30
Q

where do embolic strokes usually go?

what do they look like?

A

to the MCA

-hemorrhagic and angry AF

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31
Q

what are three most common sites of intracerebral hemorrhage?

A

-basal ganglia, thalamus, and pons/cerebellum

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32
Q

most common cause of intracerebral hemorrhage?

-what vascular deformities do they arise from?

A

HTN

-charcot-bouchard microaneurysms

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33
Q

cause of lobar hemorrhage

A

hemorrhagic diathesis

amyloid angiopathy

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34
Q

which artery is most commonly involved in AV malformations?

A

MCA

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35
Q

how are lacunar infarct related to hypertension?

A

due to arteriolosclerosis

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36
Q

What kind of nodules do you see in the CNS due to HIV encephalitis?

A

microglial nodules which fuse to attract macrophages

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37
Q

What are the three causes of global hypoxic injury?

A
  1. cardiac arrest
  2. hypovolemic shock or septic shock
  3. chronic CO poisoning
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38
Q

What are the three major categories of cerebrovascular disease?

A
  1. thrombosis
  2. infarction
  3. hemorrhage
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39
Q

what are the complications of global hypoxic injury?

A
  1. cerebral atrophy
  2. watershed infarcts
  3. stroke
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40
Q

what happens during cerebral atrophy?

which cells are most susceptible to hypoxic injury?

A

apoptosis of neurons in layers 3, 5, and 6 of the cerebral cortex.
-neurons

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41
Q

Define stroke.

A

loss of blood to a region of the brain, causing a loss of neurologic function

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42
Q

Name the four gross and microscopic findings in a thrombotic stroke.

A
  1. a pale infarct at the periphery of the cerebral cortex.
  2. swelling of the brain with lower demarcation between gray and white matter. there is a breakdown of myelin
  3. Reactive gliosis
  4. cystic formation 10 days after stroke due to liquefactive necrosis
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43
Q

how do you prevent atherosclerotic strokes?

A

aspirin, clopidogrel, or ticlopidine

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44
Q

what is a shower embolism?

A

emboli blocking numerous small vessels, typically due to fat embolus s/p femur fracture

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45
Q

what is a slit embolism?

what’s it usually caused by?

A

rupture of a small penetrating vessel that heal as a “slit” surrounded by brownish-red pigment

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46
Q

what are the two causes of subarachnoid hemorrhages?

A
  1. berry aneurysm

2. bleeding from an arteriovenous malformation

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47
Q

what are the 5 risk factors of berry aneurysm?

A
  1. hemodynamic stress
  2. HTN of any cause
  3. coarctation of the aorta
  4. atherosclerosis
  5. cigarette smoking
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48
Q

Pathogenesis of MS: name the cells that target the myelin and the protein that they degrade.
-what type of hypersensitivity reaction is this?

A
  • CD4 TH1 cells and TH17 cells
  • myelin basic protein (MBP)
  • type IV HS
49
Q

name the cytokines relevant to MS

A
  • IFN gamma (recruits macrophages)

- TNF-alpha (directly affects MBP)

50
Q

what do the antibodies attack in MS

A
  • myelin directly

- oligodendrocytes

51
Q

what are the clinical findings in meningitis?]

how are they distinguished from encephalitis?

A
  • fever, nuchal rigidity, and headaches

- mental changes and drowsiness appear in encephalitis

52
Q

list the viral infections of the CNS (ACCHHLPR)

A 
arboviruses
coxsackievirus
CMV
HSV1
HIV
lymphocytic choriomeningitis
poliovirus
rabies
53
Q

name the 2 slow viruses and 1 spongiform encephalopathy that affect the CNS

A
  • PML caused by JC virus (polyomavirus)
  • subacute sclerosing panencephalitius (rubeola paramyxovirus)
  • Creutzfield Jakob diseases (CJD) caused by infective prions
54
Q

name the causative agents of bacterial meningitis (GELNSMT)

A 
GBS
E. coli
Listeria
Neisseria meningitides
Strep pneumo
M. tuberculosis
Treponema pallidum
55
Q

names the fungal and parasitic infections of the CNS (CMNTTT)

A 
cryptococcus neoformans
nucor species
naegleria fowler
trypanosome bruce gambiense and rhodesiense
Taenia sodium
Toxoplasma gondii
56
Q

name the three genetic causes of early o set AD

A
  1. APP on c21
  2. presenillin 1 on c14
  3. presenillin 2 on c1
57
Q

what is the sporadic mutation in early onset AD

A

ApoE e4, c19

58
Q

Which parts of the brain does Abeta protein typically cause damage?

A 
medial temporal lobe (memory)
frontal cortex (personality) esp entorhinal and hippocampus
59
Q

What is the function of glycogen synthase kinase 3beta (GSK) in neurotoxicity of AD?

A

GSK-3beta phosphorylates Abeta protein, which in turn causes neuronal and synaptic dysfunction, and signaling for neuronal apoptosis.
-GSK also hyperphosphorylates tau protein, leading to NFT’s

60
Q

How is Abeta protein created?

A

beta secretases followed by gamma secretases cleave APP to produce Abeta

61
Q

What is the role of Wnt in the production of phosphorylated Abeta protein?

A

If Wnt signaling becomes dysfunctional, GSK remains in an activated state.
-this allows GSK-3beta to phosphorylate Abeta protein, progressing mental deterioration

62
Q

Where can Abeta deposits be found?

A

In cerebral vessel walls, which can lead to hypertensive cortical subarachnoid hemorrhage

63
Q

Which enzyme is decreased in type 2 diabetes and normally is involved in the clearance of Abeta protein?

A

insulin degrading enzyme

increased insulting lowers insulin degrading enzyme

64
Q

Which enzyme normally strips phosphate molecules from NF?

A

PIN1, which can be decrease in AD

65
Q

What causes senile plaques?

A

deposition of core Abeta protein surrounded by neuronal cell processes with tau protein

66
Q

treatment for AD

A
  • cholinesterase inhibitors

- memantine to block glutamate receptors

67
Q

which dopaminergic tract is altered in parkinsonism

A

nigrostriatal

68
Q

name 5 causes of parkinsonism

A
  1. CO poisoning
  2. drugs
  3. wilson disease (copper accumulation)
  4. encephalitis and ischemia
  5. MPTP
69
Q

what is the most common cause of parkinsonism?

A

idiopathic

70
Q

what are lewy bodies?

A

ubiquitinated damaged neurofilaments

71
Q

Clinical findings of parkinsonism

A

tremor
rigidity
akinesia (bradykinesia)
postural instability

72
Q

What is the trinucleotide repeat and location in huntington’s disease

A

CAG on c4

73
Q

which parts of the brain atrophy in HD? (3)

A
  1. caudate
  2. putamen
  3. globus pallidus
74
Q

what are the clinical findings of HD?

A

chorea, oculomotor abnormalities, parkinsonism in later stages

75
Q

friedrich ataxia: what is the repeat and what protein does it effect?
how does this effect homeostasis

A

GAA, frataxin

mitochondrial iron homeostasis affected, cells die more easily

76
Q

what are the degeneration sites in friedrich ataxia?

A

DRG,
posterior, spinocerebellar, corticospinal tracts
large PNS nerves

77
Q

what 2 diseases can FA patients also have?

A

type 1 diabetes

hypertrophic cardiomyopathy

78
Q

clinical findings in FA

A

progressive gait ataxia
loss of DTR’s distal before proximal
loss of vibration and proprioception
mm weakness in legs

79
Q

what type of cells die in ALS?

A

UMN and LMN

80
Q

which enzyme is defective in ALS?

A

SOD1, causes apoptosis of neurons

81
Q

where are the first signs of weakness in ALS?

A

the hand

82
Q

treatment of ALS?

A

riluzole, glutamate antagonists

83
Q

which disease is caused by excessive copper accumulation and leads to atrophy of the basal ganglia?
what are the serum findings?

A

wilson disease

  • increase free copper serum
  • decrease serum ceruloplasmin
84
Q

A pt’s urine is colorless when firs voided, but changes to a port wine color when espouser to light.

  • DX
  • what underwent the change to cause color
  • which enzyme is deficient?
A
  • acute intermittent porphyria
  • porphobilinogen
  • porphobilinogen deaminase
85
Q

clinical findings in acute intermittent porphyria

A
  • decrease uroporphyrinogen synthase synthase
  • recurrent bouts of severe abdominal pain, “bellyful of scars”
  • peripheral neuropathy
  • dementia
86
Q

what to avoid in AIP

A

carb loading, inhibits ala synthase

87
Q

which tracts are degenerated in b12 deficiency?

A

posterior column and lateral corticospinal tract

88
Q

three most common brain tumors in adults

A
  1. glioblastoma multiforme
  2. meningioma
  3. ependymoma
89
Q

three most common brain tumors in children

A
  1. cystic cerebellar astrocytoma
  2. medulloblastoma
  3. brainstem glioma
90
Q

from which layer is a meningioma derived?
which disease is it frequently associated with?
what is an environmental risk factor?
men or women? why?

A
  • arachnoid
  • NF2
  • radiation
  • women due to estrogen and progesterone receptors
91
Q

Woman presents with new-onset focal seizures, frequent headaches, and papilledema. A calcified mass is found on CT and a biopsy is performed. Name 2 characteristics that you would expect in this tumor.

A
  1. swirling masses of menningothelial cells

2. psammoma bodies (calcified bodies)

92
Q

where do ependymomas occur in adults? in children?

name a frequent complication of ependymoma

A

cauda equina in adults
fourth ventricle in children
noncommunicating hydrocephalus

93
Q

what type of cell is a medullblastoma derived from? complications?

A

granular cell of the cerebellum.

invades fourth ventricle

94
Q

40 year old man comes in to the ER due to a seizure. Complains of headaches for the past three months. CT scan shows frontal lobe tumor with calcifications. DX?

A

oligodendroglioma

95
Q

Which is more common, metastatic B cell lymphoma in the brain, of primary CNS lymphoma?

A

metastatic B cell non-hodgkin lymphomas are more common.

96
Q

which virus is associated with CNS lymphomas in AIDS patients?
which type of cell does it invade?

A

EBV

B cells

97
Q

peripheral neuropathic are typically cause by which type of pathogenesis?
which parts of the body are first to lose sensation?

A

demyelination.

hands and feet: “glove and stocking”

98
Q

What is the most common hereditary neuropathy?

What is it’s inheritance?

A

Charcot Marie Tooth disease

-AD

99
Q

Which nerve tends to atrophy first in CMT disease?

A

the peroneal nerve

-causes inverted bottle appearance of the lower legs

100
Q

What is the most common acute peripheral neuropathy?

Which type of paralysis does it lead to?

A

Guillan Barre Syndrome

-flaccid paralysis

101
Q

What are the 6 infections that typically precede Guillan Barre? (2 bacterial, 4 viral)

A
  • M. pneumoniae pneumonia
  • C. jejune enteritis
  • HIV
  • EBV
  • CMV
  • influenza
102
Q

GBS causes _____ paralysis

A

ascending

103
Q

GBS CSF labs:

A
  • increased protein, oligoclonal antibodies

- normal glucose, normal WBCs

104
Q

GBS treatment

A

-IV plasma exchange or immunoglobulin

105
Q

How does diabetes mellitus cause peripheral neuropathy?

A

Via osmotic damage to Schwann cells.

106
Q

cavernous hemangiomas in skin, mucosa, organs
bilateral renal cell aromas
hemangioblastoma
pheochromocytoma

  • DX
  • chromosome and protein defect
A
  • Von Hippel Lindau Disease
  • c3 causes mutated VHL tumor suppressor, leading to constitutive expression of HIP txt factor, causing angiogenic growth all over the place
107
Q

small round blue cell tumor
frequently in the cerebellum
sends drop metastases

A

medulloblastoma

108
Q

frequently found in posterior fossa
stains for GFAP
has rosenthal fibers

A

pilocytic astrocytoma

109
Q

typically found in the cerebral hemispheres
has regions of necrosis
shows a pseudopalisading pattern of growth
can cross the corpus callosum (butterfly)

A

glioblastoma multiforme

110
Q

benign tumor derived from the meninges

has whirling pattern of menigiocytes and psammoma bodies

A

meningioma

111
Q

closely arranged neoplasm of capillaries with minimal interleaving parenchyma
typically found in the cerebellum.
secretes EPO and causes polycythemia
caused by which disease?

A

hemangioblastoma

VHL

112
Q

typically found in the frontal lobe
has calcifications
slow growing and good prognosis
-what are the characteristic cells?

A

oligodendroglioma

-poached egg cells

113
Q

male patient presents with lactating breast and upon exam has bitemporal hemianopsia.
-Dx?

A

pituitary adenoma

114
Q

child presents with headaches and bitemporal hemianopsia.

most likely dx

A

-craniopharyngioma

115
Q

indisctint transition between normal and neoplastic cells

GFAP positive

A

diffuse astrocytoma

116
Q

GFAP positive
multiple mitotic figures
non-enhancing lesion on MRI

A

anaplastic astrocytoma

117
Q

child is known to have NF2. Presents with noncommunicating hydrocephalus and a lesion in the 4th ventricle. Name that tumor

A

ependymoma of the 4th ventricle

118
Q

adult presents with hydrocephalus
CT shows a mass in the 4th ventricle with a CT stalk
can be in the lateral ventricles in children

A

choroid plexus papilloma

119
Q

young adult complains of headaches when he leans forward

which formed is obstructed

A

colloid cyst of the third ventricle

foramina of monro

Central Nervous System Pathology (5 decks)

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