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NSMP Exam 1 > Pathology > Flashcards

Pathology Flashcards

1
Q

Intracytoplasmic inclusions of rabies

A

Negri bodies

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2
Q

Intracytoplasmic inclusions of Alzheimer’s Dz

A

Neurofibrillary Tangles of Tau protein. alpha beta plaques from APPR breakdown same amyloid as CAA

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3
Q

Intracytoplasmic inclusions of Parkinson’s Dz

A

Lewy bodies in the substantia negri and/or basal ganglia. alpha synuclein

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4
Q

Intracytoplasmic inclusions of Creutzfeldt-Jakob Dz

A

Vacuolization of perikaryon and neuronal processes

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5
Q

Tissue response in Acute neuronal injuries due to hypoxia, hypoglycemia or trauma

A

Red neurons in first 12-24 hrs.

Shrinkage of cell body, pyknosis, loss of nucleolus, loss of Nissl substance with intense eosinophilia of cytoplasm

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6
Q

Subacute or chronic degeneration/progressive dz neuronal response

A

Cell loss and reactive gliosis. Abnormal protein accumulation
(Ex. ALS or Alzheimer’s Dz)

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7
Q

Axonal reaction

A

Large increase in protein synthesis associated with axonal sprouting

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8
Q

Intranuclear inclusions of Herpes

A

Cowdry bodies

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9
Q

Neuronal inclusions in CMV

A

Both intranuclear and cytoplasmic inclusions

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10
Q

Gliosis

A

MOST IMPORTANT HISTOPATH INDICATOR OF CNS INJURY

Hypertrophy and hyperplasia of astrocytes (larger and many astrocytes)

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11
Q

Rosenthal fibers

A

Thick elongated and brightly eosinophilic ‘junk’ within astrocytes. Contain heat shock proteins alpha-beta crystallin HSP27 and ubiquitin.
Found in areas of long standing gliosis

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12
Q

Corpora Amylacea

A

PAS+, basophilic. Concentrically laminated adjacent to astrocytic end processes. Incr. # with incr. age - represents degenerative change

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13
Q

Leukodystrophy with intranuclear inclusions in immunocompromised.

A

Progressive Multifocal Leukoencephalopathy (PML) from latent JC virus

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14
Q

Demyelinating DO with glial cytoplasmic inclusions of alpha synuclein. Profound autonomic dysfunction.

A

Multiple System Atrophy (MSA)

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15
Q

CMV damages what cells?

A

Ependymal cells which line the ventricles -> form ependymal granulations

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16
Q

Vasogenic vs. Cytotoxic edema

A

Vasogenic: Incr. in EXTRAcellular fluid due to BBB disruption and incr. vascular permeability. Often follows ischemic injury

Cytotoxic: Incr. INTRAcellular fluid 2nd to neuronal glial or endothelial cell membrane injury. Due to generalized hypoxic/ischemic insult or metabolic derangement.

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17
Q

Papilledema is a sign of

A

Increased intracranial pressure

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18
Q

Communicating hydrocephalus

A

symmetrical dilatation of the ventricles due to decreased CSF absorbance at the dural sinus level.

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19
Q

Hydrocephalus ex-vacuo

A

Compensatory incr in CSF due to shrinkage of brain substance. (Aging, stroke, sports injury, degenerative dz’s). CSF PRESSURE IS NORMAL

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20
Q

Other causes of hydrocephalus

A

Choroid plexus papilloma, pyogenic meningitis, cysticercosis, aqueductal stenosis

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21
Q

Normal Pressure Hydrocephalus

A

Urinary incontinence, gait instability & dementia. Symmetric hydrocephalus in pts older than 60

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22
Q

Transtentorial/uncinated/uncal herniation

A

Medial aspect of the temporal lobe is compressed against the tentorium. CN3 compression -> decreased parasymp. -> CL dialated pupil. Posterior cerebral A and paramedian A tension.

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23
Q

Kernohan’s notch phenomenon

A

Uncal herniation where the opposite cerebellar peduncle is compressed causing ipsilateral motor impairment. False localizing sign

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24
Q

Neural tube defects causation and dz’s

A

Most common CNS malformation due to folate deficiency. Include Spina bifida, Myelomeningocele, Encephalocele, and anencephaly

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25
Q

Microcephaly causes

A

fetal alcohol syn, HIV-1 & Zika virus

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26
Q

Neuronal heterotopias associations

A

Ass. with epilepsy.

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27
Q

Holoprosencephaly

A

Incomplete separation of cerebral hemispheres. Cyclopia (eye below nose) and arrhinencoephaly (missing olfactory CN) Trisomy 13 ass.

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28
Q

Arnold-Chiari Malformation

A

Small posterior fossa
Type 1: low cerebellar tonsils, silent, may have impaired CSF flow
Type 2: More severe. Downward extension of vermis through foramen magnum. Ass. with MYELOMENINGOCELE

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29
Q

Dandy-Walker Malformation

A

Enlarged posterior fossa with expanded 4th ventricle and absent cerebellar vermis

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30
Q

Molar tooth sign

A

Joubert Syn

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31
Q

Syringomyelia (Syrinx)

A

Fluid-filled cleft-like cavity in the inner portion of the spinal cord. Isolated loss of pain and temperature sen. of upper extremities (cape-like distribution)

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32
Q

Perinatal brain injuries

A

Cerebral palsy, Intraparenchymal hemorrhage, periventricular leukomalacia, multicystic encephalopathy, ulegyria

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33
Q

Basal skull fracture signs

A

CSF drainage from ear or nose. Orbital&/or mastoid hematomas. Raccoon eyes.

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34
Q

Traumatic Brain Injury

A

Damage to the brain resulting from rapid acceleration/deceleration, impact, etc. Ass. with diminished or altered state of consciousness. Temporarily/permanent brain impairment

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35
Q

Chronic traumatic encephalopathy

A

Due to repeated head blows dx at autopsy. Abnormal buildup of tau protein and progressive loss of brain matter. Depigmentation of substantia nigra

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36
Q

Term.: Coup, Contrecoup, plaque jaune

A

Contusion at impact, diametrically opposed coup, old trauma lesions

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37
Q

Diffuse axonal injury (DAI)

A

Direct action of mech. forces causing axonal swelling. (i.e. Shaken baby syn.) Silver stain or amyloid precursor protein and alpha synuclein immunostains

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38
Q

Epidural v. Subdural bleed

A

E: Arterial, fast bleed, ass. with skull fracture. Lens sign on CT
S: Venous, slow bleed

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39
Q

Most common vessel damaged from head trauma forming an epidural hematoma

A

Middle meningeal A

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40
Q

What artery? CL leg weakness, and the person cannot recognize their hand

A

Anterior Cerebral A

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41
Q

What artery? Aphasia, hemineglect, hemianopia, upper extremity and face sensorimotor loss

A

Middle Cerebral A.

GAZE PREFERENCE TOWARDS SIDE OF THE LESION

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42
Q

What artery? Contralateral homonymous hemianopia

A

Posterior Cerebral A.

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43
Q

Watershed infarcts

A

ACA|MCA - occlusion of ICA (i.e. carotid stenosis) proximal arm and leg weakness.
MCA|PCA - Higher order visual processing

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44
Q

Primary sites of thrombosis

A

Most thrombotic occlusions are due to atherosclerosis.

Carotid bifurcation, MCA origin, either end of the basilar A.

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45
Q

Sources of Cardioembolic infarcts

A

A. Fib, MI, Valvular dz, Artery-artery emboli, dissection, PFO

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46
Q

Which A is most affected by embolic infarct?

A

MCA

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47
Q

Types of strokes

A 
Thrombotic = Ischemic
Embolic = Hemorrhagic, secondary to reperfusion causing an intracerebral or subarachnoid hemorrhage
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48
Q

Lacunar infarcts

A

Deep infarcts in lenticulostriate A’s from arteriolar sclerosis

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49
Q

Hypertensive Encephalopathy

A

Malignant HTN. Risk for deep brain parenchymal hemorrhages, Charcot-Bouchard micro aneurysms

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50
Q

Dx the pt.: 35 yo with AD NOTCH3 gene. At 47, pt has recurrent strokes and early dementia and dies. At autopsy there is thickening of the media and adventitia, loss of smooth muscle cells and basophilic PAS+ deposits

A

Cerebral Automosal Dom. Arteriopathy with Subcortical Infarcts & Leukoencephalopathy (CADASIL)

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51
Q

Cerebral amyloid angiopathy

A

alpha beta amyloid deposition in vessel walls producing microbleeds. Amyloid is congo red + (same as AD)

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52
Q

Stroke risk factors

A

HTN, DM, Hypercholesterolemia, smoking, + family hx, prior hx, Valvular dz, a. fib or PFO, Hypercoagulability.

53
Q

Most frequent cause of Subarachnoid hemorrhage

A

Saccular (berry) aneurysm in Circle of Willis at the branch pt of ACA and anterior communicating A.

54
Q

Pts most likely for SAH

A

Female, 50+ yo, who have the worst headache they have ever had from strenuous activity

55
Q

Most common VM site

A

MCA and posterior branches

56
Q

HSV-1, Herpes Zoster and Rabies

A

Are all viruses that invade the nervous system peripherally and ascend to the CNS.

HSV and Zoster replicate in Schwann cells and ascend in sensory nerves.

Rabies binds Ach R and ascends via motor nerves.

57
Q

Chronic meningitis agents and CSF interp.

A

TB, neurosyphilis, neuroborreliosis, or cryptococcus.

CSF has elevated proteins, predominant lymphocyte ct and low glucose

58
Q

Acute meningitis causation by age: Neonate

A

E. coli & group B Strep

59
Q

CSF interpretation: Turbid with neutrophils increase, large decrease in glucose and elevation in protein

A

Bacterial meningitis

60
Q

CSF interpretation: clear with lymphocyte increase, glucose is WNL and slight elevation in protein

A

Viral meningitis

61
Q

Acute meningitis causation by age: 3mo - 2yr unvaccinated

A

H. Flu type B

62
Q

Acute meningitis causation by age: Adult/young adult

A

N. meningitidis

63
Q

Acute meningitis causation by age: Elderly

A

Strep pneumo. and Listeria

64
Q

Pt presents with progressive focal neurologic deficits and Incr. IC pressure
CSF interpretation: High WBC, high protein and normal glucose

A

Abcess

65
Q

Extradural abscess association

A

osteomyelitis

66
Q

Waterhouse-Friderichsen Syn

A

Rapid septicemia, DIC, hypotension and petechial & purpuric lesions from N. medingitidis infection. Hemorrhagic infarct of adrenal glands and gangrenous skin lesions possible

67
Q

Subacute Sclerosing Panencephalitis

A

Latent condition stemming from an infantial infection with paramyxovirus (Rubeola). It arises years later with congnitive decline, limb spasticity and seizures.
Widespread gliosis and myelin degeneration

68
Q

vCJD

A

Variant form, slow progression, Kuru plaques in cerebellum. Proteins are congo red + and PAS+

69
Q

Autoimmune demylinating DO with signs & symptoms of unilateral vision impairment, ataxia, nystagmus, motor and sensory impairment, muscle fatigue. MRI shows irregular progressive plaques adjacent to ventricles

A

Multiple sclerosis
affects women more and is 15x more common in first degree relatives. HLA-DR2 IL-2 & IL-17 receptor genes. Initiated by CD4+ TH1 & TH17 cells

associated with optic neuritis development in 10-50% of cases.

70
Q

Woman with HLA-DR2 gene comes in with CSF findings of slightly elevated protein, moderate pleocytosis and IgG increase. What would you see on immunoelectrophoresis

A

Oligoclonal IgG bands. Pt has MS

71
Q

Neuromyelitis optica

A

Bilateral optic neuritis and spinal cord demyelination.
Ass. with development of MS in 10-50% of pts.

AB TO AQUAPORINS = TX W/ PLASMAPHORESIS

72
Q

Acute Disseminated Encephalomyelitis (ADEM)

A

Diffuse monophasic demyelinating dz post viral infection or rarely immunization.

73
Q

Acute Necrotizing Hemorrhagic encephalomyelitis

A

RARE - usually a distractor

Demyelination syn of CNS in young adults and children after recent URI. Fatal

74
Q

Dx pt: Pt is treated for hyponatremia and later presents with acute paralysis, dysphagia, dysarthria, diplopia & loss of consciousness

A

Central pontine myelinolysis from rapid Na+ correction.
Loss of myelin symmetrically in basis pontis and portions of the pontine tegumentum.
NO INFLAMMATION

75
Q

Inclusions found in Huntington Dz

A

Polyglutamin repeats

76
Q

Dx pt: 85 yo with no family Hx presents with dementia, global cortical atrophy seen on MRI, and epsilon 4 APOE allele, and congo red + proteins from biopsy

A

Alzheimer’s Dz

77
Q

Down Syndrome neurodegenerative association

A

Early onset of Alzheimer’s Dz

78
Q

(2) Frontotemporal Dementias (FTDS) with TAU pathology

A

Pick Dz & Progressive Supranuclear palsy

79
Q

Frontotemporal Dementias (FTDS) withOUT tau pathology

A

Vascular dementia

80
Q

Rare, distinct, progressive dementia. Early onset behavioral changes and language disturbances. Asymmetric frontal and temporal lobe atrophy with narrow knife-like gyri

A

Pick Dz

Posterior 2/3 of superior temporal gyrus is spared

81
Q

Progressive Supranuclear palsy

Symptoms? Protein?

A

Truncal rigidity with widespread neuronal loss. Loss of voluntary eye movements, especially vertical gaze. Men 2x more likely. 4R Tau straight filaments in tangles

82
Q

Vascular dementia

A

Widespread areas of infarction, 2nd most common cause of dementia. Dementia improves with tx

83
Q

Diffuse Lewy body dementia

A

Early onset dementia with diffuse Lewy bodies in the brain.

84
Q

A dz with loss of medium spiny striatal neurons and anticipation during spermatogenesis. Striking atrophy of caudate nucleus. Pt presents with chorea

A

Huntington’s Dz

85
Q

Freidreich ataxia
Inherritance? Mutation? Gene & Protein? Population distribution with symptoms?
COD?

A

AR, mitochondrial DO, GAA trinucleotide repeat on chrom. 9Q13 of FRATAXIN protein.

First seen in pts <10 yo. Gait ataxia. COD is usually intercurrent pulm. infections & cardiac dz.

86
Q

Ataxia-Telangiectasia

Inheritance? Symptoms? Gene?

A

AR, Telangiectasias, lymphoid neoplasms, gliomas and carcinomas. Immunodeficient (recurent sinopulm infections), ATM gene on chrom. 11Q22-Q23. Fails to remove cells with damaged DNA

87
Q

Amyotrophic Lateral Sclerosis

Inheritance? Gene? Histo? Symptoms?

A

aka ALS
Lower motor neuron loss and Upper motor neurons in 5th decade or later. AD with SOD1 gene superoxide dismutase on Chrom 21. PAS + bunina bodies.
ANTERIOR ROOTS OF spinal cord ARE THIN AND PRECENTRAL GYRUS IS ATROPHIC. DECREASED ANTERIOR HORN NEURONS.
Pt presents with dropping objects, cramping hands and difficulty speaking and swallowing

88
Q

Inheritance pattern of Adrenoleukodystrophy

A

X-Linked

89
Q

Tay-Sachs Dz

A

AR, HEXA gene mutation on chrom 15-> dysfunctional hexosaminidase A enzyme -> accumulation of GM2 Gangliosides. Fatal in 2-3 yr olds cherry red maculae

90
Q

Mitochondrial Encephalomyopathy Lactic Acidosis & Stroke-like episodes

A

aka MELAS,
tRNAs mut. caused by mitochondrial abnormalities. Muscle involvement with weakness & lactic acidosis. Stroke-like episodes

91
Q

Myoclonic Epilepsy & Ragged Red Fibers

A

aka MERRF,

tRNA mut. Myoclonus, seizure DO with myopathy. Ataxia and ragged red fibers present

92
Q

Kearn-Sayre Syn

A

Progressive inability to move eyes and eyebrows. Ataxia symptoms with pigmentation retinopathy and cardiac conduction defects. ragged red fibers present

93
Q

Leigh Syn

A

aka Subacute necrotizing encephalopathy.

Lactic acidemia in early childhood, seizures and hypotonia. Histo shows spongiform appearance & vascular proliferation.

94
Q

B12 Deficiency symptoms

A

Anemia, numbness, tingling and slight ataxia in lower extremities. Ascending posterior columns and descending pyramidal tracts neurons degenerate = subacute degenerating of the spinal cord

95
Q

Wernicke or Korsakoff is reversible

A 
W = Reversible
K = Irreversible -> confabulation
96
Q

B1 deficiency is seen in what populations

A

Chronic alcoholics, hemorrhage and necrosis in mammillary bodies

97
Q

What area of the brain is affected in carbon monoxide poisoning?

A

Layers 3 & 5 of cerebral cortex, Sommer’s sector, purkinje cells, and bilateral necrosis of globus pallidi. STAINS TISS RED/PINK

98
Q

Methanol poisoning symptoms

A

Selective bilateral putamenal necrosis. Degeneration of retinal ganglion cells -> blindness

99
Q

Ethanol toxicitiy symptoms

A

Truncal ataxia, unsteady gait, nystagmus. Atrophy of anterior vermis. Advanced cases demonstrate Bergmann gliosis

100
Q

Basal cell carcinoma of the eye

A

Most common malignant tumor of periocular skin. Pearly nodules, telangiectatic vessels, rodent central ulcer, rolled edges. Peripheral palisading nuclei

101
Q

Sebaceous carcinoma

Demographics? Histo?

A

Women of asian decent older than 40 yo. Masquerade syn., comedocarcinoma. Oil red O stain, +EMA, focally + NRST-1 & P-16

102
Q

Uveal Melanoma

A

MOST COMMON INTRAOCULAR TUMOR IN ADULTS.

GNAQ & GNA11 uveal melanoma oncogenes.

103
Q

3 types of cataracts

A

Nuclear sclerosis = age related
Posterior subcapsular - lens epithelium migrates posterior to the lens equator
Morgagnian (hypermature) - Lens cortex liquefies, may cause phacolytic glaucoma

104
Q

Open Angle Glaucoma

A

Complete open access to trabecular meshwork, increased resistance to aqueous outflow.
Primary - MYOC gene mut
Secondary - pseudoexfoliation, LOX1 gene

105
Q

Closed Angle Glaucoma

A

Adheres to trabecular meshwork & physically impedes outflow

106
Q

Retinoblastoma

A

MOST COMMON PRIMAY INTRAOCULAR MALIGNANCY OF CHILDHOOD
chrom 13Q14 = RB
Optic nerve infiltration can lead to extension into brain

107
Q

Pediatric tumors

A

MOST ARE BELOW THE TENTORIUM IN CEREBELLUM & BRAINSTEM
Pilocytic Astrocytoma*
Medulloblastoma
Ependymoma (4th ventricle)

108
Q

Adult tumors

A 
MOST ARE ABOVE THE TENTORIUM
Glioblastoma multiforme*
Oligodendroglioma
Myxopapilloma
Ependymoma (spinal cord)
109
Q

Infiltrating astrocytoma grading system

A

I: does not exist
II: diffuse astrocytoma, 3rd-4th decade
III: anaplastic astrocytoma, 5th decade
IV: glioblastoma (malignant), 6th+ decade

110
Q

1st two decades of life in the cerebellum. NF1 predisposition
Cystic with mural nodule
GFAP +
Hairlike, biphasic pattern, Rosenthal fibers and eosinophilic granular bodies present

A

Pilocytic astrocytoma

111
Q

Most common primary brain neoplasm in adults. EGFR oncogene & PTEN mut

Secondary: IDH1 (has better prognosis) & IDH2 mutations.

Contrast ring-enhancing lesion with central necrosis and CROSSES corpus callosum

A

Glioblastoma

112
Q

Histology of glioblastoma

A
  1. Necrosis is serpentine patterened
  2. Pseudo-palisading
  3. Vascular/endothelial proliferation (VEGF & glomeruloid body is “ball-like”)
113
Q

Primarily effects adults, CALCIFICATIONS, fried egg and chicken wire appearance on histology.
1P19Q, IDH1 & IDH2 are of favorable prognosis .
Grade III/IV is of poor prognosis

A

Oligodendroglioma

114
Q

1st two decades of life found in 4th ventricle (less common is the adult form found in the spinal cord). Rosettes on histology

A

Ependymoma

115
Q

Most common in children, lateral ventricles. Commonly causes obstructive hydrocephalus

A

Choroid plexus papilloma

116
Q

3rd ventricle cyst in young adults. Obstruction of the foramen of Monro -> noncommunicating hydrocephalus

A

Colloid Cyst of 3rd Ventricle

117
Q

Neuroectodermal tumor in kids, in the cerebellum, midline -> can occlude CSF flow, SHEETS OF ANAPLASTIC CELLS ABUNDANT MITOSES & HOMER-WRIGHT ROSETTES. Radiosensitive. “Drop Metz”

A

Medulloblastoma

118
Q

Medulloblastoma Molecular groups

A

WNT: Best, older kids, chrom 6, B-Catenin
SHH: MYCN amp. infants-young adults
MYC: WORST
I17Q: no MYC, +/- MYCN poor prognosis

119
Q

Atypical Teratoid/Rhabdoid Tumor

Histo? Gene? Prognosis?

A

+EMA & vimentin.
Resembles rhabdomyosarcoma
Chrom 22 hSNF5/INI1 mut. have good prognosis. <5yo is highly aggressive, est. 1 yr survival

120
Q

Primary CNS lymphoma

A

Immunosupressed pts - AIDS or HIV ass.
Monotonous cells, often multifocal, with periventricular spread.
B-cell origin CD20+ with latent EBV.
Accum. around vessels (“Hooping”)

121
Q

Pineal tumor and types

A

Suprasellar tumor with male & Japanese predominance in first 2 decades of life.

Germinoma is the most common form. Responds well to tx.
Pinecytoma - well differentiated, low grade in adults.
Pineblastoma is high grade and usually in kids (RB gene)

122
Q

Dx Pt.: 30+yo female presents with tumor that expresses NF2 and progesterone R’s. On MRI tumor is compressing brain and has eroded through skull. Histology reveals whirled clusters of monotonous cells

A

Meningioma, WHO I

NF2 (22Q12) - high grade. Multiple meningiomas are always NF2. Sporadic.
TRAF7 - Lower grade
Dura based is EMA+
Psamomma bodies

123
Q

Most common metastatic carcinomas mets

A

1.) Lung 2.) Breast 3.) Melanoma 4.) Kidney

Choriocarcinoma is rare but frequently metastases to brain.
Meninges is a common site of met.

124
Q

NF1, neurofibromas, Lisch nodules, Cafe-au-lait spots, optic nerve glioma

Ass. with MPNST, high grade tumors.

A

Neurofibromatosis

125
Q

What is the Dz and gene affected?

Pt has a pheochromocytoma, RCC, hemangioblastoma, pancreatic, liver and kidney cysts. Polycythemia on CBC

A

Von Hippel-Lindau (VHL)

126
Q

What is the Dz and genes affected?
AD, seizures, autism, mental retardation.
hamartomas, renal angiomyolipomas & cardiac rhabdomyomas. Shagreen patches & ash-leaf patches on skin.

A

Tuberous Sclerosis

TSC1 (Hamartin) & TSC2 (Tuberin)

127
Q

What is the tumor?

Pt presents with tinnitus & hearing loss. S100+ tumor at the cerebellar pontine angle.

A

Schwannoma. CN 8 damage -> hearing deficits.

if NF2 -> bilateral Schwannomas

128
Q

What syndrome?

AD, often found in the breast or endometrium. Dysplastic ganliocytoma with PTEN mut.

A

Cowden Syn.

NSMP Exam 1 (1 decks)

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