17. CNS Pathoma Flashcards Preview

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Flashcards in 17. CNS Pathoma Deck (51):
1

Anencephaly is associated with what involving the amniotic fluid

Polyhydramnios - too much amniotic fluid because the baby doesn't have a developed brain to swallow it

2

Whats syringomyelia? and what brain malformation is associated with it

Degeneration of the spinal cord

"CAPE/SHAWL-like" bilateral loss of pain and temp in upper extremities

usually associated with Chiari malformation

3

Whats chiari malformation

herniation of the vermis through the foramen magnum

4

What is ALS (Amyotrophic Lateral Sclerosis)

Degenerative disorder of the upper and lower motor neurons (corticospinal tract)

zinc copper superoxide dismutase mutation (SOD- removes free radicals)

5

Fredrick Ataxia

Degenerative disorder of the cerebellum

Fred the FRAT star - mess up of Frataxin (iron binding protein)

staggering and falling but has a sweet big heartdiabets and hypertrophycardiomyopathy

GAA

6

What is the classic triad of meningitis

Stiff neck, headache, fever

7

How do you differentiate the etiology of meningitis when looking at the CSF via lumbar puncture

Bacterial- neutrophils with low CSF glucose

Viral- lymphocytes with normal CSF glucose

Fungal- lymphocytes with low CSF glucose

8

What happens in an epidural hematoma and what should you see on CT

fracture of the skull that severs the middle meningeal artery

Lens/biconvex shaped lesion on Ct

9

What happens in a subdural hematoma

What should you see on CT

Rupture of the bridging veins

Blood BENEATH the dura

crescent shape

10

What is the key cell that myelinated axons in the CNS

Oligodendrocyte

11

What is the key cell that myelinated axons in the PNS

Schwann cell

12

What is multiple sclerosis

Autoimmune destruction of CNS myelin and oligodendrocytes

HLA-DR2SIII

-Sudden loss of vision (marcus gun)/bladder dysfunction

Scanning speech (sound like youre drunk), intention tremor, incontinence, INO, Nystagmus

SIIIN

13

What is a lacunar stroke and which vessels do they most commonly involve

infarcts in the deep areas of the brain.

Mostly in the lenticulostriate vessels off the MCA

14

Where does intracrebral hemorrhage mostly occur and why

in the basal ganglia due to the lenticulostriate vessels rupturing (micro aneurysms)

15

Worst headache of life

Subarachnoid hemorrhage

usually rupture of saccular (berry) aneurysm usually located in the anterior circle of willis (ACA)

Bloody or yellow CSF with tap

Only blood at the bottom of the brain

16

What is Central pontine myelinolysisand how do you get it what is the major presenting case

Focal demyelination of the pons

Caused by RAPID iv correction of Hyponatremia(from low to high the pons will die)

Presents as Locked in syndrome- acute paralysis-

17

In GREY MATTER Degenerate neurons in the cortex?

Degenerate neurons in the White matter basal ganglia (deeper structure of the brain)?

Dementia

Basal ganglia is required for movement so movement problems

18

Whats the most common disease for dementia?

What is it? and grossly brain findings

Alzheimer's dz (degeneration of the GREY matter in the cortex)

Slow onset memory loss, disorientation, loss of learned motor skills

Diffuse Brain atrophy, narrowing of Gyri/widening of sulci

Neuritic plaques (amyloid precursor protein APP with ab amyloid)

Neurofibrillary tangles (hyperphosphorylated tau protein)

19

What are risk factors for Alzheimer's dz?

Deficient Ach

ApoE2 (decreases risk)

ApoE4 (increases risk)

Presenillin -1/2 (familial)

Neuritic plaques- Amyloid precursor protein mess up that causes AB amyloid

20

What are neurofibrillary tangles, and in which dz are they seen?

Hyperphosphorylated Tau protein seen in Alzheimers Dz

21

Vascular Dementia

Dementia in elderly due to multiple infarcts of chronic ischemia

22

Pick'z DZ (Frontotemporal Dementia)

it "picks" the frontotemporal

Frontal (behavioral) and Temporal (language) dementia Pick Bodies, ROUND Hyperphosphorylated tau

23

What is parkinson

are the clinical presentations

(hint: its a a trap)

Degenerative disorder of CNS involving dopamine deficiency neurons in the Substantia Niagra

Associated with Lewy Body (alpha-synuclein)

Tremor, Rigidity (Cogwheel) Akinesia (brady) Posture (instability shuffle gait)

24

What are lewy bodies composed of?What disorder would you find these

composed of alpha-synuclein

found in Parkinson Dz

25

Difference between lewy body dementia and Parkinson Dz

Lewybody dementia has early onset (parkinson takes a while)

cortical lewy body= Ha'lew'cinations

26

What is the degeneration of gabarnergic (inhibitory) neurons in the brain? Where in the brain is the mess up?Whats the trinucleotide repeat

Huningtons Dz

Caudate nucleus

GABAnergic (inhibitory) neurons are destroyed

CAG (has anticipation so it gets worse with each generation)

CAG Caudate loses Ach and Gaba

27

What is the striatum and what composes it

Striatum is the basal ganglia which is important in movement.

The caudate and the putamen makes it

28

What is Hydrocephalus and what TRIAD does it present?

(wet whack and wobbly) and what usually improves it

Increased CSF resulting in dilated ventricles

Urinary incontinence, dementia, gat instability (magnetic feet)

Lumbar puncture (LP) relieves pressure

29

With hydrocephalus you should be thinking the stretching of the _______

corona radiata

30

Spongiform Encephalopathy

Degenerative dz of prion protein most common form is CJD

31

Creutzfield Jakob dz (CJD)

spongiform encephalopathy rapid dementia

Ataxias and startle myoclonus

32

What is the role of the astrocytes?

Forms the blood brain barrier

33

What are the tumors that arise from astrocytes?kids/adult

Kids: Pilocytic Astrocytoma

Adults: Glioblastoma Multiforme

34

What is the role of Oligodendrocytes?

Myelinate axons in the CNS

35

What are the tumors that arise from oligodendrocytes?Kids/adults

Kids: n/a

Adults: oligodendroglioma

36

What is a glioblastoma multiforme? where does it arise

Malignant tumor of the astrocytes in brain

Most common malignant CNS tumor in adults

Crosses the Corpus callosum- buffterly glioblastoma

Psuedopallisading

GFAP

37

What is a meningioma what does it look like on histo?

Benign tumor of the arachnoid cells

Most common benign tumor

Whorl cells

38

What is a Schwanomma?which cell type does it arise?What section of brain does it involve /histo

benign tumor of Schwann cells in the PNS

Usually located at the cerebellar pontine angle (CPA) and messes with the Cranial Nerve CN8

s100+

39

Oligodendrogliomas / what cell it arises from / what section of the brain does it involve / histo

Arise from the oligodendrocytes in the white matter (bc they mylelinate-white)

fucks with the frontal lobe

fried egg appearance

calcified tumor

40

What is the most common CNS Tumor in children and where does it arise

(Remember that most kid CNS tumors are below the tentorium)

Pilocytic Astrocytoma

Cerebellum

Rosenthal fibers GFAP

41

Medulloblastoma /histo

CNS tumor in kids

Neruoectodermal Tumor

Former wright rosettes

small blue cells

42

Which cells line the ventricular space in the brain

ependymal cells

43

ependymoma

Malignancy of the ependymal cells

commonly arises in 4th ventricle

hydrocephalus?

44

Craniopharygioma

tumor that arises from the remnants of the Rathe's pouch (pituitary)

compression of optic chiasm

Bitemporal hemianopsia

45

What is Bitermporal Hemianopsia and what causes it

Compression of the optic chiasm that leads to loss of lateral vision

46

Metachromatic Leukodystrophy

Demyelinating DO/lysosomal storage dz

deficiency of arylsulfatase

myelin cant get degraded so it builds up in the lysosomes

47

Krabbe Dz

Demyelinating disorder/lysosomal storage dz

deficiency of galactocerebrosidase

build up of galactocerebroside -> destroys myelin

48

Adrenoleukodystrophy

demyelinating disorder

X linked do affecting males

disrupts metabolism of very-long-chain fatty acids

leads to toxic levels in adrenal gland and white matter

 

49

whats diagnostic for Multiple sclerosis

Oligoclonal bands

50

progressive multifocal leukodystrophy

which infection is associated with it

demylinating disorder

Destruction of oligodendrocytes

JC virus infection

51

Hydrocephalus Ex Vacuo

When you have dilated ventricles but its due to Brain atrophy 

the ventricles have more space to expand. 

ICP is normal