pathology MNI Flashcards

1
Q

what are the two types of cerebral edema

A

vasogenic bc of disruption of BBB which will make fluid go to the extracellular space. often bc of trauma or meningitis

cytotoxic bc of cell injury such as ischemia, causing inc in intracellular fluid

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2
Q

presenting sx of ex vacuo hydrocephalus vs NPH

A

ex vacuo presents like dementia or stroke causing loss of brain volume

NPH- whacky wet wobbly (reason unset but probably bc of high CSF volume at one point causing ventricles to large)

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3
Q

increased intracranial pressure characterised by headache
(positional), diplopia (CN-6 palsy), vomiting, and papilledema potentially leading to
vision loss.

epidemiology?

A

Pseudotumor Cerebri
( Idiopathic Intracranial Hypertension)

usually seen in obese women of child bearing age

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4
Q

lewy body

A

parkinsons

cytoplasmic inclusions

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5
Q

what are the presentation of the various types of herniations

A

subfalacine: crushes the anterior cerebral artery causing lock of sense or motor in the lower limbs.

transtetorial herniation: Ipsy down and out, spy blown pupil, hemiparalysis

tonsils: press of cardiac or breathing centers

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6
Q

Kernohan Phenomenon

A

continuous displacement from transtetorial herniation causes contralateral down and out blown pupil,

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7
Q

parenchymal braininjury

A

concussion
Chronic Traumatic Encephalopathy (multiple concussions)

croup and contecroup contusions

diffuse axonal injury(neurons stretch beyond elastic point separating grey and white matter)

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7
Q

protein accumulation is of hyperphosphorylated tau protein only

A

Chronic Traumatic Encephalopathy

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7
Q

linear flame shaped hemorrhages due to stretching of median and paramedian
pontine arteries. Pontine arteries branch from basilar artery running on anterior surface of pons. caused by?

A

duret hemmorage

caused by herniations/ inc in ICP

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7
Q

foamy macrophages

A

found in lipohyalinosis of the lenticulostraie arteries

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7
Q

vascular brain injury

A

epidermal hemmorage(hit the pterion, middle meningeal artery doesn’t cross culture, biconvex, LOC, then fine, then dead

subdural bc of rupture of the bridging veins. prominent in infants and elders

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7
Q
  • Swollen brain with wide
    gyri & narrowed sulci
  • Poor demarcation
    between gray & white
    matter
  • Eventually tissue
    liquefies leaving a fluid
    filled cavit
A

ischemic infracts

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7
Q

which intraparenchymal hemmorages are bc of HTN

location?

A

-charciod bouard(lenticulostriae)
-slit hemmorages
-primary intraparenchymal hemorrhage

location: basal ganglia

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7
Q

subarachnoid hemorage

A

berry aneurysm(non traumatic rupture)

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8
Q

tangled network of arteries and veins with no interposing capillaries

cuases

A

AV malformation
“tangled worms”

causes non htn hemmorage, in newborns causes high output congestive heart failure because of the shunting

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8
Q

fusiform vs saccular ansursym

A

fusiform is charcoal bouard

saccular is berry(no muscular or internal elastic leaving just hyalinized intimate and adventitia)

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8
Q

Affects larger non-penetrating vessels
supplying the cortex and is therefore typically superficial/ lobar in location, unlike small
penetrating vessels affected by hypertension which are typically subcortical/deep

A

non htn
amyloid antipathy

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8
Q

associated with
autosomal dominant polycystic kidney
disease, Ehler-Danlos syndrome and other
congenital diseases which can affect
connective tissue of blood vessels.

A

berry aneurysm

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8
Q

subarachnoid hemmorage complication and how to prevent if possible

A

-rebleed

-vasospasm give Nimodipine

-healing process causes fibrosis and can obstruct CSF resorption= hydrocephalus

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8
Q

Lumbar puncture: shows high number
of RBCs or xanthochromia (yellowish
CSF due to release of bilirubin after
RBC lysis

A

berry

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9
Q

alzheimers lobes affected and common cause of death

A

frontal, parietal, and temporal lobes affecting spacial awareness, character and memory

pneumonia or infection is common cause of death

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10
Q

alzheimer plaque mutations

A

ab extracellular plaques are APP gene

the tau just comes in after

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11
Q

tau protein cause what kind of plaques and how are they made

A

normally binds microtubules but after phosphorylation by AB plaque will be unable to bind causing *neurofibrilary tangles inside cells!! the tangles outside the cell are called neuritic plaques

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12
Q

early onset alz mutations

vs late onset

A

APP (downs 21)
the gamma secretase gene is coded on chromosome 1 and 14 in the presillin 1 and 2

late onset: Apo E4

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13
Narrowing of gyri and widening of cerebral sulci
alzheimers dizease (opposite of ischemia occlusion)
14
what are the two types of FTLD
1. Pick disease. Tau neurofibrillary tangles with pick bodies also intracellular (just tau intracellular) 2. intracellular accumulation of TDP-43 a DNA/RNA binding protein
15
Death of Striatal Cholinergic Interneurons & Death of Striatal GABAergic Neurons
huntington
16
Death of pigmented dopaminergic neurons in Substantia Nigra
parkinsons
17
Alpha Synuclein aggregates normal and abnormal
parkinsons normal:Alpha Synuclein is expressed at presynaptic neurons and binds to presynaptic secretory vesicles containing dopamine and causes exocytosis of dopamine into synaptic cleft. abnormal:aggregates are toxic to dopaminergic neurons in substantia nigra and cause neurons to undergo programmed cell death.
18
Lewy body dementia
when dementia sx arise within 1 year of motor symptoms if more than one year called dementia secondary to parkinsons disease
19
autosomal dominant movement disorder associated with degeneration of the striatum (Caudate and Putamen) causing
huntington
20
Huntington disease mutation
AD gain of function of HTT causes trinucleotide repeats of CAG (glutamine) which will impare mitochondrial and the synthesis of proteins like Ach and GABA
21
characteristic Ubiquitinated-Mutant protein intranuclear fragments made of?
huntingtons disease Ubiquitinated-Mutant protein and impaired proteolysis when staining stain for ubiquity
22
hallmark of HD
the intraNUCLEAR inclusions of ubiquinated huntingtin
23
chorea and Athetosis
huntington
24
als mutations
Chromosome 9 hexanucleotide repeat and Superoxide dismutase gene
25
what is spared with ALS
intelect, sphincter control, and eye movement, sensation
26
degeneration of corticospinal tract in the lateral portion of the spinal
ALS its not demyelination but actual nerve degeneration
27
autoimmune demyelinating disorder characterized by disease activity separated in time, that produce white matter lesions that are separated in space
MS commonly statics with optic neuritis plaques are often periventricular
28
MS genetic risk factor and non genetic
Genetic HLA DRB1 smoking and low Vitamin D
29
inc igG in CSF but not in blood
MS
30
names of post infection demyelinations
Acute Disseminated Enecephalomyelitits Acute Necrotizing Hemmoragic Encephalomyelitis
31
non-immune damage to oligodendrocytes
Central Pontine Myelinolysis/Osmotic Demyelination typically after sudden correction of hyponatremia that may result in a rapidly evolving quadriplegia and bulbar palsy bc of oligodendrocyte contraction and apoptosis
32
confusion * Ocular palsies * Ataxia if left untreated
Wernicke encephalopathy if left untreated can cause causes korsacoff syndrome where no new memories can be made and will have confabulations
33
typical brain tumors in kids vs adults
kids: medulloblastoma, pilocytic astrocytoma, ependemoma adults: glioblastoma(astrocytoma), oligocondroma, meningioma
34
genetic for the astrocytomas
pilocytic is BRAF glioblastoma is IDH 1 and 2
35
Bipolar cells with thin hair like (“pilo”) processes in a fibrillary meshwork with eosinophilic rod like structures called
Rosenthal fibers pilocytic ast rocytoma
36
Infiltrating tumor with hemorrhage and necrosis and crossing the midline producing a
producing a butterfly glioma glioblastoma
37
Microvascular proliferation with areas of “pseudo”-palisading necrosis
glioblastoma
38
IHC GFAP
for all glial cancers astrocytoma, oligodendroglioma, ependemoma
39
insidious seizure presentation
oligodendroglioma
40
Regular cells, round to oval nuclei & abundant granular chromatin Cells may form rosettes with long delicate processes extending into the lumen- “canals” * Also seen are “pseudo”-rosettes which are perivascular: tumor cells are around vessels
ependymonoa
40
association with neurofibromatosis 2
adult spinal ependymomas, meningioma, 8th nerve shwannoma
41
oligodendroglioma genetics
Codeletion of 1p and 19q: good prognosis will have IDH 1 mutation
42
Cells with round nuclei, clear cytoplasm forming halos; and thin-walled capillaries
oligodendoglioma
43
neuroectoderm cerebral tumor genetics IHC marker?
medulloblastoma Wnt-β catenin pathway(better orognosis), MYC overexpression IHC is a neuronal and glial markers
44
Sheets of small round blue anaplastic cells * Hyperchromatic nuclei, abundant mitosis, scant cytoplasm * Rosettes
medulloblastoma
45
Chromosome 22q
schwannoma
46
where do the most common brain metastases come from
Breast Lung Skin Kidneys (kids) GIT (geriatrics)
47
what kind of bone resists movement in all directions
woven bone lamellar bone is unidirectional force for weight bearing
48
which bones undergo intramembranous ossification
flat bones of face, skull, and clavicle
48
FGFR3 mutation type? what normally happens
achondroplasia \ AD gain of function normally inhibits endochondrial ossification but with GOF there's no getting it uninhibited
48
short extremeties, normal trunk, large head/buldging fontanells
achondroplasia
48
OI sx besides brittle bones
blue sclera, hearing loss, yellow/blue teeth, bruise easily
49
Defective Osteoblasts and Matrix Production
osteogenesis imperfects 2 "accordion like shortening of the limbs"
49
what is not affected in achondroplasia
intelegence, life expectancy, sex repor
49
deficient osteoclast development or function, which leads to diffuse, symmetric skeletal sclerosis
osteoporosis Mutations interfere with acidification of the osteoclast resorption pit,
49
Erlenmeyer flask deformity
long bones lack the medullary canal in osteoporosis
49
decrease in type I collagen synthesis
osteogenesis imperfecta
49
osteogenesis imprtfecta mutation
genes encoding α1 and α2 chains of type I collagen... Glycine residue replaced by another amino acid causing the helix not to form properly and be defective dominent negative effect(will effect the normal produced proteins)
50
Bone is osteopenic, kyphoscoliosis, spondylolisthesis
elder dances syndrom
50
severe AR osteopetrosis
pancytopenia, hepatospleenomegaly compressed cranial nerves renal tubular acidosis
51
marfan mutation
fibril 1 on chrom 15
52
loss of horizontal trabeculae and thickened vertical trabeculae
osteoporosis thin trabeculae and loss of apposition
52
what does RANKL do and what up regulates it where does it bind
RANKL promotes the conversion of osteoblasts to clasts and is promoted by IL-11, PTH, Vit D and malignancies RANKL bind to RANK on the osteoclasts(decoyed by osteoprogestin)
53
examples of secondary osteoporosis
vit D/C def steroids hyperthyroid/parathyroid
54
Normal calcium, phosphate and alkaline phosphatase levels
osteoporosis
55
Initiated by unregulated osteoclastic activity ➔ increased resorption ➔ stimulates increased osteoblastic activity ➔ abnormal bone deposition
pagets disease
56
phases of pagets disease
1. osteolytic, osteoclast dominent 2. mixed= woven bone 3. blastic phase= jigsaw mosaic
57
increased hat size and deafness
pagets disease
58
Increased Serum Alkaline Phosphatase * Increased ....
Urinary Hydroxyproline pagets
59
pagets disease complications
cardiac failure heart disease, osteosarcoma, giant cell tumors, extra osseous hemapoisis
60
reason for renal tubular dysfunction to cause osteopenia
renal tubular acidosis causes the hydroxyapatite to disolve
61
bone changes in end stage renal failure
1. high turnover with lots of resportiona nd formation 2. aplastic disease more like osteomalacia than remodeling 3. mixed
62
Frontal bossing and squared appearance of forehead * Persistent hyaline cartilage and overgrown costochondral junctions called
Rachitic rosary: where the palisade of cartilage is absent(stack of coins) rickets also comes with Harrison groove and pigeon chest deformity poorly calcified osteoid
63
Newly formed osteoid matrix laid down by osteoblasts is inadequately mineralized ➔producing the excess of persistent osteoid
osteomalacia
64
Subperiosteal haemorrhages Microfractures and bony deformities
scurvy poor crosslinginging
65
which bone tumors are commonly in adults
Echocondroma(within medullary cavity/ no stalk) giant cell tumors, chondrosarcoma
66
Slow growing, bone islands, in paranasal sinuses and calvaria, associated with
with FAP colonic adenomas in Gardner syndtom osteoma
67
site of: 1.osteoid osteoma 2.osteoblastoma 3. osteosarcoma 4. osteochondroma 5. enchondroma 6. chondrosarcoma 7. osteoclastoma
1. CORTEX of femur/tibia 2. vertebrae lamina and pedicle 3. MEDULLA of long bones near knee 4. near the knee 5. small bones of hands and feet 6. Axial skeleton- pelvis, shoulder, ribs 7. knee
68
which one is bigger osteoid osteoma or osteoblastoma which one has nocturnal pain which one can be relieved by NSAIDS which one has reactive scoliosis
osteoblastoma is greater than 2 cm osteoid osteoma has nocturnal pain bc of release of PGE2 , can be relieved by NSAIDs, and has sclerosis
69
bimodal age peaks
osteosarcoma 1 young bc de novo mutations 2. old bc of pagets
70
osteosarcoma mutations
Rb CDNK2 MDM2 p53 CDK4
71
genes involve in the cartilage bone tumors
osteochondroma= ext 1 and 2 enchondroma is IDH 1 and 2 chondrosarcoma: EXT 1/2, IDH 1,2 CDKN2
72
Olliers and Mafucci syndromes
enchondroma
73
chondrocytes and cartilage matrix surrounded by ring of reactive bone tissue in medullary cavity of tubular bone
enchondroma
74
where do chondrosarcoma metastitize
lungs
75
Develops in epiphysis and spreads to metaphysis
osteoclastoma/ giant cell tumor
76
Onion skin appearing because of periosteal bone reaction
ewig sarcoma
77
11:22 translocation
ewig sarcoma Primitive round cells without any obvious differentiation
78
Homer-Wright rosettes rounded cell clusters with a central fibrillary core
indicative of attempted neuroectodermal differntiation Ewing
79
bence jones proteinuria
multiple myeloma light chains K and L found in urine
80
adult and kid metastizises to bone
ADULT: breast, lung, thyroid, kidney, prostate KIDS: Osteosarcoma, wilms tumor , neuroblastoma, ewig, rhabdo
81
starts with chondrocyte injury
osteoarthritis then causes fibrillation of articular cartiledge
82
features of osteoarthritis
eburnation, subchonral reactive sclerosis, subchondral cysts, osteophytes
83
HLADR4
rheumatoid arthritis can also be caused by smoking or infection
84
CD4+T-cellmediatedproliferationofsynovialcellswith“pannus”formation leading to joint erosion
RA
85
Periarticular Osteopenia and Ankylosis
cause of morning stiffness in RA shows absent reparative activity unlike OA that has sclerosis and osteophytes
86
Ulnar aspect of forearm, elbow
RA
87
antibody found in RA
ACPA
88
HLA B27
seronegative Spondyloarthropathies: Ankylosing Spondylitis, Reactive Arthritis
89
Insidious onset back pain in the lower lumbar or buttock region i.e Lumbar Spine and Sacroiliac joints * Prolonged morning stiffness lasting >1hr -> improves with activity Pleuritic chest pain and limited chest expansion -> secondary to inflammation of costovertebral or costochondral junctions.
Ankylosing Spondylitis
90
reactive arthritis sx
conjunctavitis, uveitis, arthritis esp after salmonella, or chlamydia