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Pathology > PNS, CNS > Flashcards

PNS, CNS Flashcards

1
Q

MC inherited peripheral neuropathy

A

Charcot Marie Tooth
MC type: CMT1, 2nd decade, distal demyelinating
CMT2A: early, childhood, axonal neuropathy

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2
Q

MC chronic acquired peripheral neuropathy

A

Chronic inflammatory demyelinating poly (radiculo) neuropathy
symmetric mixed sensorimotor polyneuropathy >/2 mos
recurrent demyelination and remyelination with Schwann cell prolif

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3
Q

acute symmetric ascending demyelinating polyradiculoneuropathy
T cell mediated
ascending paralysis and areflexia

A

Guillan Barre syndrome
CSF: Albuminocytologic dissociation

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4
Q

MCC of PN

A

DM

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5
Q

Neuromuscular disease
+ weakness with exertion
EOM muscle group

A

Myasthenia gravis
HS type II
Assoc: Thymoma, thymic hyperplasia
post synaptic ACh receptor autoantibodies

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6
Q

Neuromuscular disease
- muscle weakness upon exertion
Extremities
assoc: neuroendocrine Ca of the lungs

A

Lambert eaton myasthenic syndrome (LEMS)
autoantibodies against Presynaptic Calcium channels

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7
Q

MC inflammatory myopathy

A

Juvenile - Dermatomyositis
Adult - usually paraneoplastic

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8
Q

Skin changes seen in Dermatomyositis

A

Heliotrophe rash - periorbital lilac discoloration
Gottron papules - dusky rash patches over knuckles, knees and elbows

pattern of atrophy: perifascicular

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9
Q

antibodies in Dermatomyositis for gottron and heliotrope rash

A

anti-Mi2

P155/P140 - paraneoplastic, juvenile form

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10
Q

total absence of Dystrophin X on chromosome
pelvic girdle weakness
pseudohypertrophy of lower leg muscles
Gower sign
- Dystrophin IHC

A

Duchene muscular dystrophy

Becker - milder, late onset, reduced dystrophin on IHC

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11
Q

Cell dense areas with spindle cells arranged in fascicles with nuclear palisading

A

Antoni A

Verocay bodies - Cell-free areas due to nuclear palisading

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12
Q

Cell poor areas with prominent MYXOID stroma

A

Antoni B

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13
Q

mutation: NF2 (Merlin) (Ch22) inactivation
Biphasic appearance: Antoni A and Antoni B
Verocay Bodies
+ S100

A

Schwannoma

+S100 - positive in neural crest cell derived cells (other: melanocytes)

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14
Q

mutation: NF1 (Ch17) inactivation
+ S100, spindle cells, + CD34
plexiform: Bag of worms appearance

A

Neurifibroma
“shredded carrots appearance”

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15
Q

Components of neurofibromatosis

A

Bilateral 8th nerve
Schwannoma
multiple meningioma
ependymoma

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16
Q

Most important pathologic marker of CNS injury

A

Gliosis

acute neuronal injury: Red neuron
subacute and chronic neuronal injury: Degeneration

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17
Q

Herniating part in Subfalcine

A

Cingulate
herniation thru under flax cerebri
compressed: anterior cerebral artery

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18
Q

compressed structure in Transtentorial herniation

A

CN III
Posterior Cerebral artery
CL cerebral peduncle

herniating part: medial aspect of temporal lobe
herniating thru: tentorium cerebelli

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19
Q

linear or flame-shaped lesion hemorrhagic lesion in brainstem and pons

A

Duret hemorrhages

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20
Q

tonsillar herniate through
Compressed sturcture

A

Foramen magnum
herniating part: cerebellar tonsils\
Compressed: Brainstem – cardio and respi depression

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21
Q

MC CNS malformation

A

Neural Tube defects
RF: folate deficiency

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22
Q

MC neural tube defect

A

Spinal Dysraphism/Spina bifida

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23
Q

extension of the spinal cord and meninges through the vertebral column defect

A

Myelomeningocele

meningocele: meninges only

24
Q

extrusion of malformed brain tissue thru a midline cranial defects

A

Encephalocele
Loc: Occiput

25
Q

Incomplete separation of the cerebral hemispheres across the midline

A

Holoprosencephaly
assoc. Trisomy 13
Cyclopia, Arrhinencephaly - absence of olfactory cranial nerves

26
Q

Enlarged posterior fossa (absence of hypoplasia of cerebellar vermis)
Cystic dilatation of the 4th ventricle

A

Dandy-walker malformation

27
Q

MC site of contusion

A

Frontal lobe

28
Q

Small cavitary infarcts assoc with hypertension

A

Lacunar Infarcts
Common site: Putamen

29
Q

Neurons most SENSITIVE to ischemia

A

Pyramidal layer of hippocampus
Pyramidal cells of cerebral cortex
Cerebellar Purkinje cells

30
Q

Infarcts in areas that lie at borders of arterial territories

A

Borderzone (watershed) Infarcts
MC area: ACA-MCA border zone

31
Q

MC type of intracranial aneurysm

A

Saccular aneurysm
MC location: ACA- ACoA junction

32
Q

Most clinically significant vascular malformation

A

Arteriovenous Malformation
10-30yrs, Males
MC site: Posterior branches of MCA
Large BV separated by gliotic tissue
+ shunting

33
Q

Distended loosely organized back to back vascular channels without intervening brain tissue

A

Cavernous Malformation
MC site: Cerebellum
- shunting

34
Q

MCC of Acute aseptic Meningitis

A

Enterovirus 80%
Viral: Lymphocytic
Chemical - neutrophil
N sugar, Inc Protein, - culture

35
Q

MC agents in Brain Abscess

A

Staphylococci
Strepcocci

36
Q

MC Demyelinating disorder

A

Multiple sclerosis

37
Q

AI demyelinating disorder
Cell mediated CD4+
Unilateral involvement of the optic nerve (optic neuritis)

A

Multiple Sclerosis
CSF: moderate pleocytosis, mildly elevated protein, IgG, Oligoclonal IgG bands

38
Q

MC prion disease

A

Creutzfeldt-Jakob disease
Progressive dementia, Startle myoclonus, 7th decade, Kuru plaques

Prion - rapidly progressive neurodegenerative disease

39
Q

MCC of dementia in older adults

A

Alzheimer disease
AB (forms plaques and tangles), Tau aggregates (stress response)
Chromosome 21, 19 (ApoE)
terminal event: pneumonia

40
Q

Parkinson TRIAD

A

Tremor
Rigidity
Bradykinesia

41
Q

Loss of Dopaminergic neurons in Substantia Nigra
Dementia with Lewy Bodies
Pallor of substantia nigra and locus coeruleus

A

Parkinson Disease
Dx HM: Lewy body (a-synuclein)

42
Q

Loss of striatal neurons that dampen motor output
mutation in Glucocerebrosidase
CAG trinucleotide repeat
Cortical atrophy
Huntingtin aggregates

A

Huntington Disease

43
Q

AR, progressive ataxia, cardiomyopathy
GAA trinucleotide repeat expansion (Ch9)

A

Friedreich Ataxia

44
Q

Weakness and atrophy
SOD1 (Ch21) mutation
Bunina bodies: PAS+
Corticospinal tract degeneration

A

Amytrophic lateral sclerosis
- loss of Upper motor neuron and Lower motor Neuron

45
Q

most susceptible neuron in Hypoglycemia state

A

Dorsomedial nucleus of the thalamus

46
Q

MC primary tumor

A

Gliomas: Astrocytoma, Oligodendroglioma, Ependymoma

47
Q

High grade infiltrating astrocytoma Necrosis: Palisading
Microvascular prolif: Glomeruloid

A

Glioblastoma
Necrosis: Palisading
Microvascular prolif: Glomeruloid

48
Q

WHO IV/IV

A

Glioblastoma
Necrosis: Palisading
Microvascular prolif: Glomeruloid

49
Q

WHO I/IV

A

Pilocytic astrocytoma
localized low grade glioma – found in children
mural nodule
Rosenthal fibers

50
Q

WHO II/IV
Fried egg appearance of cells

A

Oligoendroglioma
mutation in IDH1 and concurrent 1p19q codeletion
Chicken wire vascular network
Calcifications
Perineuronal Satellitosis in cortical tumors

51
Q

WHO II/IV
Perivascular rossette

A

Ependymoma
first 2 decades of life: at 4th ventricle
adult: spinal cord

52
Q

MC embryonal neoplasm

A

Medulloblastoa WHO IV/IV
afflicts children
Homer-wright rosette
small round blue cells inc mitosis

53
Q

MC CNS neoplasm in Immnocompromised individuals

A

primary CNS Lymphoma
MC subtype: DLBCL
in immunocompromised: +EBV

54
Q

NF2 deletion Ch22q12
Dural tail
I/IV: Meningothelial, Psammoma bodies

A

Menigioma

55
Q

Cytogenic Origin of Meningioma

A

Meningothelial cells of arachnoid

56
Q

AD, loss of function TSC1 (Ch9) and TSC2 (Ch16)
Hamartomatous cortical tubers
CT: Melted candled

A

Tuberous Sclerosis Complex

Pathology (18 decks)

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