Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

MS2 Unit3 Neurology > CNS pathology > Flashcards

CNS pathology Flashcards

1
Q

the wall of the neural tube forms ____
the hollow lumen forms ___
neural crests forms ____

A

CNS;
ventricles + spinal cord canal;
PNS;

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

neural tube defects can be avoided by administering ____

A

high folate BEFORE conception

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Neural tube defects are dx prenatally by

A

↑AFP in amniotic fluid + maternal blood

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Pregnant pt tests positive for maternal polyhydroaminos and ultrasound of fetus presents with frog-like appearance (prominent eyes). Dx?

A

Anencephaly

hint: polyhyrdoaminos dt ↓swallowing (absent brain fx); frog-like appearance dt øskull/brain + prominent eyes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

when the posterior vertebral arch fails to close during fetal development; this is known as

A

spina bifida (vertebral defect)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

___ presents as dimple or hair patch over vertebral defect

A

spina bifida occulta

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

____ is a protrusion of meninges only

A

spina bifida meningocele

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

_____ is a protrusion of meninges + spinal cord

A

spina bifida meningomyelocele

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

the most common cause of hydrocephalus + suture closure failure in newborns

A

cerebral aqueduct stenosis

hint: obstruction –> ↑ CSF –> ventricle swelling + ↑ head circumf

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

What produces CSF in the ventricles

A

choroid plexus (lining ventricles)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Name that channel:

  1. lateral –> ____ –> 3rd ventricle
  2. 3rd ventricle –> ____ –> 4th ventricle
  3. 4th ventricles –> ____ –> subarachnoid space
A
  1. foramen of Monro
  2. cerebral aqueduct
  3. Magendia (medial) and Luschka (lateral)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

MRI reveals massively dilated posterior fossa; newborn presents with hydrocephalus. Dx?

A

Dandy-Walker Malformation (øcerebella vermis dev + dilated 4th ventricle)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

MRI reveals cerebellar vermis + tonsil displacement thru foramen magnum.

A

Type II Arnold-Chiari Malformation

hint: Type I is asympto

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

Arnold-Chiari Malformation (II) is often associated with what 2 sx?

A

hydrocephalus (CSF obstruction)

meningomyelocele (±)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

cystic degeneration of the spinal cord is known as _____ as usually dt what 2 causes

A

Syringomyelia (C8-T1);

Trauma or Type I Arnold-Chiari Malformation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Describe the distribution of Syringomyelia

A

C8-T1: øpain/temp in upper extremity (UE)

hint: “cape-like distribution”

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

What is responsible for the capelike distribution of pain and temp sensation loss in syringomyelia?

A

ant white commissure involvement (spinothalamic tract)

hint: dorsal column is spared –> fine touch + pos’n spared

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
18
Q

Syrinx expansion in syringomyelia involves what two tracts? Explain sx of each.

A

Anterior horn (LMN) + Lateral horn (hypothalamospinal tract);

  1. Ant horn = Muscle atrophy ↓muscle tone + reflexes (∆LMN)
  2. Lateral horn = Horner’s (ptosis, miosis, anhidrosis)

hint: HTS tract supplies sympathetics to the face

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
19
Q

After a recent viral infection, pt presents with flaccid paralysis, muscle atrophy + fasciculations, weakness w/ ↓muscle tone + reflexes; but Babinski is ⊖. Dx?

A

Poliomyelitis (poliovirus inftn –> LMN sign)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
20
Q

Newborn pt presents as “floppy baby”; with no other pertinent positives (no recent honey intake, exposure)? Most likely dx?

A

Werdnig-Hoffman Dz (autosomal recessive)

hint: death a few years after birth

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
21
Q

what is the mechanism and inheritance pattern of Werdnig-Hoffman dz?

A

deg’n of ant motor horn;

autosomal rec

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
22
Q

Degeneration of upper AND lower motors neurons of the corticospinal tract is classified as

A

ALS (Amyotrophic Lateral Sclerosis)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
23
Q

45 year old pt complains of atrophy and weakness in hands. Sensation to pain, temperature, and touch are in tact. What should you check for?

A

Sporadic ALS

hint: earliest sign is atrophy + weakness in hands

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
24
Q

How can you differentiate ALS from Syringomyelia?

A
  • ALS: intact sensation

- syringo: ant horn involvement from C8-T1 (cape distr) + øP/T sensation

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
25
Q

Difference bwn UMN and LMN lesions (4 each)? What structures/tracts are involved in each?

A

UMN (lateral corticopinal tract): spastic paralysis; hyperreflexia; ↑muscle tone; ⊕Babinski

LMN (ant horn): flaccid paralysis; atrophy + fasciculations; ↓reflexes + tone; ⊖Babinski

hint: things go up for UMN lesions; things go down in LMN lesions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
26
Q

What is a common association in familial ALS

A

SOD1 mut (zinc-copper superoxide dismutase) –> free radical injury to neurons

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
27
Q

What structures are degenerated in Friedreich Ataxia? What are the subsequent sx?

A

Cerebellum + spinal cord

Cerebellum: ataxia
spinal cord (mult tracts): LE muscle weakness, ø DTRs; ø vibration + proprioception sense
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
28
Q

What is the mechanism and pattern of inheritance of Friedrich Ataxia?

A

unstable trinucleotide repeat (GAA) in frataxin gene (mt Fe reg’n);

autosomal rec

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
29
Q

What results with frataxin mutation (trinuc expansion)

A

ø mitochondrial iron regulation –> free radical dmg (via Fenton rxn)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
30
Q

4 yo pt presents with LE muscle weakness, absent DTRs, neuro exam confirms ataxia with little to no vibratory + proprioception sense; CXR reveals enlarged heart. Family is requesting wheelchair provision. Dx?

A

Friedrich Ataxia

hint: presents in early child; hypertrophic cardiomyopahty is common

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
31
Q

leptomeninges include the ___ layers (2)

A

Arachnoid and Pia menginges

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
32
Q

what 3 infectious agents commonly affect neonates

A

Group B strept (3rd trimester prophx)
E.coli
Listeria monocytogenes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
33
Q

what infectious agent commonly affect children AND teenagers

A

N. meningitidis

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
34
Q

what infectious agent commonly affect adults and elderly

A

S. pneumoniae

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
35
Q

what infectious agent commonly affect nonvax’d infants

A

H. flu

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
36
Q

what MOST COMMON infectious agent affects children

A

Coxsackievirus

hint: fecal oral tranmission

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
37
Q

what infectious agent commonly affect immunocompromised pts

A

fungi

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
38
Q

Pt presents with headache, nuchal rigidity and fever, what other symptoms might also be present? Describe how to sample CSF?

A

Meningitis (class triad: headache, nuchal rigidity and fever);

±nausea, vomiting, confusion (AMS)

lumbar puncture bwn L4 and L5 (iliact crest)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
39
Q

why is lumbar puncture done at L4 and L5?

A

spinal cord ends at L2

subarachnoid space + cauda equina end at S2

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
40
Q

describe CSF findings for Bacterial; viral; fungal meningitis

A

Bacterial: neutros + ↓CSF glucose
Viral: Lympho + normal CSF glu
Fungal: lymphos + ↓CSF

hint: viruses dont need food (øglucose consumption observed)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
41
Q

Explain the complications of bacterial meningitis (2 pathways)

A
  1. cerebral edema –> herniation –> death

2. fibrosis –> hydrocephalus (CSF obstr) + hearing loss (nerve damage) + seizures (cerebral scarring)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
42
Q

Cerebral Vasc Dz (CVD) is 85% dt ____ and 15% dt ___

A

85% ischemia (↓Q)

15% dt hemorrhage (bleed)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
43
Q

what are the 4 major etiologies of global cerebral ischemia?

A
  1. low perfusion
  2. acute ↓Q (cardiogenic shock)
  3. chronic hypoxia (anemia)
  4. ↑ hypoglycemia episodes (insulinoma)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
44
Q

Explain why glucose would result in ischemia

A

neurons need glucose for energy (necrosis within 3-5 min)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
45
Q

Transient confusion w/prompt recovery. Ischemia severity?

A

mild global ischemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
46
Q

infarcts in watershed areas and vulnerable regions. Ischemia severity?

A

moderate global ischemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
47
Q

diffuse necrosis; death or vegetative state with survival. Ischemia severity?

A

severe global ischemia

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
48
Q

Pyramidal neurons of _____ leads to laminar necrosis

A

cerebral cortex 3-5-6 (moderate global ischemia)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
49
Q

long term memory disfunction in moderate global ischemia is assc w/ dmg to what structure?

A

pyramidal neurons of hippocampus (temporal lobe)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
50
Q

Damage (dt moderate global ischemia) to what layer of the cerebellum would interfere with sensory perception + motor control

A

Purkinje Layer

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
51
Q

focal ischemia <24 hrs is considered ___

A

TIA (transient ischemic attack)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
52
Q

focal ischemia >24 hrs

A

stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
53
Q

3 subtypes of stroke?

A

thrombotic
embolic
lacunar

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
54
Q

describe etiology and sx of:

  • thrombotic stroke
  • embolic stroke
  • lacunar stroke
A

thrombotic: artherosclerotic plaque* at branch pts (IC + MCA) –> pale infarct in peripheral cortex
embolic: afib –> emboli from L heart to MCA –> hemorr* infarct in peripheral cortex
lacunar: htn –> hyaline arteriolosclerosis in LS vessels –> small cystic infarctions

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
55
Q

explain why thrombotic stroke is pale and embolic stroke is hemorrhaged?

A

thrombotic: presence of athero plaque –> thrombus reforms –> pale infarct sustained
embolic: embolism lysed –> re-perfusion –> hemorrhage

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
56
Q

Lacunar stroke of internal capsule results in

A

pure motor stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
57
Q

Lacunar stroke of thalamus results in

A

pure sensory stroke

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
58
Q

ischemic stroke results in ___

A

liquef necrosis

59
Q

List the changes (cell popn’s) assc with necrosis bwn 1 day; 1 week; 1 month of ischemic infarct

A 
red neurons (eosinophilic∆) - 12 hrs p/infarct
necrosis - 24 hrs ""
Nø - 1-3 days ""
Mø- 4-7 days ""
granulation/gliosis - 2-3wks ""
gliotic cysts - end stage
60
Q

Intracerebral hemorrhage is usually dt _____

A

htn –> rupture of Charcot-Bouchard microaneurysm of LS vessels

61
Q

most common site of intracerbreal hemorrh?

A

basal ganglia

62
Q

Severe headach, naseua, vomiting, eventual coma is characteristic of (intracerebral/subarachnoid)

A

intracerebral hemorrhage

63
Q

Pt with sudden onset of “worst headach of life” w/ nuchal rigidity most likely is suffering from?

A

subarachnoid hemorrhage

64
Q

with subarachnoid hemorrhage, blood is most likely to pool where?

A

on the bottom of the brain

65
Q

What layer is missing in berry aneurysms? What s the most frequent location for BAs?

A

ømedia layer at branch points;

Anterior Communicating Artery in (ant circle of Willis)

66
Q

what 2 dz’s is subarachnoid hemorrhage assc with?

A

Marfan Syndrome + Auto dom polycystic kidney dz

67
Q

Oligodendrocytes myelinate the______. Scwhann cells myelinate the ______

A

CNS;

PNS

68
Q

How are demyelinating disorders characterized

A

destruction of myelin or oligodendrocytes (CNS) w/ axonal sparing

hint: axons are spared, but impulse is damaged

69
Q

Luekodystrophies are characterized by what process? 3 types of leukodystrophies are?

A

enzymatic mutations lead to ∆myelin storage/synth;

  1. Metachromatic leukodystrophy
  2. Krabbe Dz
  3. Adrenoleukodystrophy
70
Q

Metachromatic Leukodystrophy is due to a deficiency of what enzyme and affects pathway?

A

arylsulfate defc; leads to sulfatide acc’m in oligodendrocytes

hint: metachromatic leukodyst is a lysosomal storage dz)

71
Q

Krabbe Dz is due to a deficiency of what enzyme and affects pathway?

A

galactocerebrosidase; leads to galactocerebroside acc’m in mø

72
Q

Adrenoleukodystrophy is due to a deficiency of what enzyme and affects pathway?

A

impaired coA addition to FAs; leads to FA acc’m –> dmg to adrenal glands and brain white matter

73
Q

The inheritance patter of Metachromatic Leukodystrophy and Krabbe Dz is _____; while Adrenoleukodystrophy is ____

A

autosomal recessive; X-linked

74
Q

Autoimmune destruction of CNS myelin + oligodendrocytes is known as ____-

A

Multiple Sclerosis (MS)

75
Q

25 yo female pt, who appears drunk, presents with blurred vision in R eye, weakness and ↓sensation in lower extremities and intermittent episodes of hemiparesis/paresthesia. MRI shows plaques in white matter. Lumbar puncture is done and shows ↑lymphocytes; high res electrophoresis is done, and shows ↑Igs, oligoclonal IgG bands, myelin basic protein.
Dx? Tx (acute and longterm)?

A

MS (autoimmune);
acute tx: high dose steroid
long term: IFN-β (↓progression)

76
Q

What 2 tools confirm MS dx?

A

MRI + lumbar puncture

77
Q

Neuro deficits of MS occur in periods of _____. Name the structure involved in each following clinical features?

  1. blurred vision in one eye
  2. Vertigo + scanning speech (~drunkenness)
  3. Internuclear opthalmoplegia
  4. Hemiparesis/unilateral sensation loss
  5. LE weakness and sensation loss
  6. Bowel, bladder, and sexual dysfx
A

remission;

  1. optic nerve
  2. brainstem
  3. medial longitudinal fasciculus (mlf)
  4. (periventricular*) cerebral white matter
  5. spinal cord
  6. autonomic nervous system
78
Q

Name the genetic predisposition and env trigger for MS

A

HLA-DR2 (gene) + regions far from equator (env)

79
Q

Common popn for chronic MS?

A

young adult (20-30); ↑in women

hint: MS is the most common chronic CNS dz of young adults

80
Q

epidural hematoma is bwn what two layers/structures

A

dura + skull

hint: temporal skill fracture + middle meningeal bleed

81
Q

A lens shaped lesion on CT and lucid interval point to what dx?

A

epidural hematoma

hint: herniation = lethal complication

82
Q

where does blood collect during a subdural hematoma

A

under dura (covers the surface of the brain)

83
Q

With trauma, tearing of what structures (bwn dura and arachnoid) leads to subdural hematoma

A

bridging veins

84
Q

A crescent shaped lesion on CT with progressive neurologic deficits points to what dx?

A

subdural hematoma

hint: herniation = lethal complication

85
Q

What ↑ occurrence subdural hematoma in elderly pts?

A

cerebral atrophy –> stretched bridging veins

86
Q

Displacement of brain tissue is also known as _____. What 2 mechanisms lead to displacement of brain tissue

A

Herniation

  1. mass effect (MVA)
  2. ↑intracranial P
87
Q

Herniation type that involves cerebellar tonsils into foramen magnum ±cardiopulmonary arrest.

A

Tonsillar herniation

hint: CP arrest dt compression of brain stem

88
Q

Herniation type that involves cingulate gyrus displacement under falx cerebri. Compression of what artery leads to infarction?

A

subfalcine herniation;

Anterior Cerebral Artery (ACA) leads to infarction

89
Q

Pt presents with the following

  1. “down and out eye” and dilated pupil (name lesion)
  2. contralateral homonymous hemianopsia (name lesion)
  3. Duret (brainstem) hemorrhage (name lesion)

Dx?

A
  1. CN III compression
  2. post cerebral artrey compression –> occipital lobe infarct
  3. paramedian artery rupture

Dx Uncal Herniation

90
Q

Uncal Herniation involves displacement of what structure?

A

temporal lobe uncus under tentorium cerebelli

91
Q

progressive/debilitating encephalitis dt to persistent measles virus inftn of the brain. Characterized by viral inclusion in gray and white matter

A

Subacute sclerosing panencephalitis

hint: infections in infancy –> neuro defects in childhood

92
Q

rapidly progressive neuro signs (øvision, weakness, dementia) dt JC virus iftn of oligoDCs (white matter)

A

Progressive Multifocal Leukoencephalopathy

hint: leads to death

93
Q

Why is progressive multifocal leukoencephalopathy typically seen in AIDS or leukemia pts?

A

immunosuppression reactivates latent JC virus

94
Q

What dz is chrz’d by pontine demyelination and presents as acute bilateral paralysis (locked in syndrome)?

A

central pontine myelinolysis

95
Q

What clinical action usually results in central pontine myelinolysis?

A

rapid IV correction of hyponatremia

hint: esp if pt is severely malnourished (alcoholic/liver dz)

96
Q

dementia is chrz’d by what process?

A

acc’m of protein –> ↓ neurons in cortical*** gray matter

97
Q

deg’n of what structured leads to movement disorders (2)

A

brainstem + basal Ganglia (BG)

98
Q

80 yo pt presents with slow-onset memory loss –> progressive disorientation; loss of motor skill and language; behavioral changes; and family members say he has recently gone mute and spends most of time in bed? Dx?

A

Alzheimer’s dz

hint:
- øfocal deficits
- presumptive dx after excluding other ddx; confirmed at autopsy (histo)

99
Q

What % of Alz is seen in the eldery and is mostly sporadic?

A

95%

hint: risk doubles every 5 yrs after 60

100
Q

which allele of APOE is assc with high risk/low risk (ℇ4/ℇ2)

A

ℇ4 - high risk;

ℇ2 - low risk

101
Q

early onset AD is seen in (2)

A
  1. familial cases (presenilin1* + 2)
  2. Down Syndrome (t21 w/ extra APP gene)

hint: onset by 40 yo in DS

102
Q

cerebral atrophy (hydrocephalus exvacuole); neuritic Aβ plaques w/entangled processes; hyperPO4’d tau + neurofibrilary tangles - are all morpho features of what dz?

A

AD

103
Q

when amyloid deposits around bvs in AD, the pt is at risk for?

A

cerebral angioid neuropathy –> hemorrhage

104
Q

how is Aβ amyloid formed?

A

APP (chrom 21) undergoes β cleavage (non degradable) –> acc’m

105
Q

What is tau?

A

microtubule assc protein

106
Q

Multifocal infarction dt htn, atherosclerosis, or vasculitis is chr-istic of what dz?

A

vascular dementia

hint: 2nd most common cause of dementia

107
Q

Degeneration of frontal and temporal cortex leading to early behavorial and language sx (resepctively) followed by dementia is what dz?

A

Pick Disease

108
Q

on histo, round aggregates of tau protein are observed in cortical neurons. What are these structures? Dx?

A

Pick bodies; Pick Dz

109
Q

damage to and loss of dopaminergic neurons in the substantia nigra of BG is chr of what dz?

A

Parkinsons’s

110
Q

nigrostriatal pathway of BG is responsible for what action?

A

movement initiation (via dopamine) - important for PD

111
Q

Name the Clinical features of PD (5)?

A 
hint: TRAPS
T remor 
R igidity (cogwheel)
A kinesia/bradykinesia (expressionless)
P ostural instability 
S huffling gait
112
Q

on histo, round eosinophilic inclusions of 𝛂-synuclein are seen. What are these structures? Dx?

A

Lewy bodies; PD

hint: øpigemented neurons in SN seen

113
Q

What is the difference bwn PD and Lewy body dementia

A
  • PD - late-onset of dementia

- LBD - early-onset dementia + cortical* Lewy bodies + hallucinations (parksonian features)

114
Q

Degeneration of GABAergic neurons in the caudate nucleus (BG) as a results of CAG trinuc expansion is what dz?

A

Huntington’s Dz

hint: autosomal dominant (chrom 4)

115
Q

in HD further expansion of repeats during _____ leads to anticipation

A

spermatogenesis

116
Q

45 yo pt presents with chorea, progressive dementia, and depression. Pt admits to suicidal ideation? dx?

A

Huntington Dz

117
Q

Describe the tremor commonly seen in PD

A

pill rolling tremor at rest***

disappears with movement

118
Q

Pt presents with urinary incontinence; gait instability, and dementia subsequent to hydrocephalus (∆reabs in vneous sinus –> inc csf –> ventricles). Dx? Tx?

A

Normal Pressure Hydrocephalus;

tx: lumbar puncture for relief; ventriculoperitoneal shunting (VP shunting)
hint: triad = wet, wobbly, wacky

119
Q

conversion and cycling of PrPc –> PrPsc (non degradable) leading to intracellular vacuoles is chr of what dz?

A

spongiform encephalopathy

120
Q

This most common* type of Spongiform encephalopathy is usually sporadic but can be transmitted via corneal/GH transplant*?

A

Creutzfeldt-Jakob dz (CJD)

121
Q

Contraction is related to bovine spongiform encephalopathy (mad cow) and is typically seen in young pt? (Variant CJD/Familial Fatal Insomnia)

A

Variant CJD

122
Q

Contraction is related to inherited prion and chrzd by exaggerated startle response and severe insomnia? (Variant CJD/Familial Fatal Insomnia)

A

Familial Fatal Insomnia

123
Q

Pt presents with rapidly progressive dementia , ataxia, and startle myoclonus. Periodic sharp waves are seen n EEG. Dx? Px?

A

CJD Spongiform Encephalopathy;
death <1 year

hint: startle myoclonus = invol movt w/min stimulus

124
Q

Forms of Spongiform Enceph conversion (3)?

A
  1. sporadic
  2. inherited
  3. transmitted
125
Q

_____ tumors are multiple, well circumscribed lesion at the gray white junction (metastatic/primary)

A

Metastatic

hint: usually arise from lung breast, kidney

126
Q

_____ tumors are classified acc to cell type of origin (metastatic/primary)

A

Primary

hint: locally destructive; rarely metastisize

127
Q

Where are primary tumors located in adults/kids?

A

adults - supratentorial

kids - infratentorial

128
Q

gliobastoma multiform, meningioma, and scwhannoma are typical found in what popn?

A

adults

129
Q

pilocytic astrocytomas, ependymoma, and medulloblastoma are most common in what popn?

A

kids

130
Q

Pseudopalisading necrosis, endothelial cell prolifn, and GFAP+ tumors are assc with what dz>

A

glioblastoma multiforme (GBM)

131
Q

40 yo pt dx’d a with malignant, astrocytic tumor; butterfly lesion is seen on autopsy. Dx?

A

GBM

hint: poor prognosis

132
Q

50 yo female pat presents with seizures. round masses attached to the dura are seen on imaging. Histo reveals whorled patterning + psammoma bodies. Dx (meningioma/oligodendroglioma)? Is this tumor benign/malignant?

A

meningioma;
benign tumor of arachnoid cells

hint: more common in women (±estrogen related); seizures dt cortical compression (øcortical invasion)

133
Q

Pt presents with hearing loss and tinnitus; on imaging, tumor involves CN VIII and CPA (cerebellopontine angle). on IHS tumor cells are s-100+ . Dx?

A

Scwannoma

134
Q

in Neurofibromatosis type 2 (Schwannoma), what is typical lesion is observed?

A

bilateral tumors

135
Q

Pt presents with seizures on biopsy cells have a ‘fried-egg’ appearance; imaging reveals calcified tumor with white matter of forntal lobe*. Dx meningioma/oligodendroglioma)? Is this tumor benign/malignant?

A

oligodendroglioma;
malignant tumor of oligodendrocytes

hint: 1p/q19 mut

136
Q

A cystic lesion with a mural nodule is detected in the cerebellum of a 6yo pt? Dx? Is this tumor benign/malignant?

A

pilocytic astrocytoma;
benign tumor of astrocytes

hint: most common CNS tumor in kids

137
Q

What diagnosis is consistent with eosinophilic Rosenthal fibers + granular bodies, and GFAP⊕ tumor cells?

A

pilocytic astrocytoma

138
Q

What diagnosis is consistent with small round blue cells and Homer-Wright rosettes? What is drop metastasis?

A

medulloblastoma;

metastasis to cauda equina

139
Q

Is medulloblastoma benign/malignant

A

malignant tumor of cerebellar granular cells (neuroectoderm)

hint: common in kids (cerebellar)

140
Q

What diagnosis is consistent with hydrocephalus and perivascular pseudorosettes (ependymoma/craniopharyngioma)? Is this tumor benign/malignant?

A

ependymoma;
malignant tumor of ependymal cells

hint: 4th ventricle involvement –> CSF obstruction –> hydroceph

141
Q

What diagnosis is consistent withbitemporal hemianopsia calcifications on imaging; and recurrence after resection (ependymoma/craniopharyngioma)? Is this tumor benign/malignant?

A

craniopharyngioma;

benign tumor of epith remnants of Rathke’s pouch

142
Q

in neurofibillary tangles, assc with AD what protein is hyperphosphorylated?

A

tau

143
Q

Loss of cholinergic neurons in AD occurs where? (basal ganglia/nucleus basalis)

A

Nucleus basalis of Meynert

MS2 Unit3 Neurology (10 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions