17. CNS Pathoma Flashcards by Trevor Johnson

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

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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

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Whats chiari malformation

herniation of the vermis through the foramen magnum

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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)

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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

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What is the classic triad of meningitis

Stiff neck, headache, fever

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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

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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

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What happens in a subdural hematoma

What should you see on CT

Rupture of the bridging veins

Blood BENEATH the dura

crescent shape

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What is the key cell that myelinated axons in the CNS

Oligodendrocyte

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What is the key cell that myelinated axons in the PNS

Schwann cell

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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

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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

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Where does intracrebral hemorrhage mostly occur and why

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

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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

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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-

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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

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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)

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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

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

Hyperphosphorylated Tau protein seen in Alzheimers Dz

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Vascular Dementia

Dementia in elderly due to multiple infarcts of chronic ischemia

Pick’z DZ (Frontotemporal Dementia)

it “picks” the frontotemporal

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

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)

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

composed of alpha-synuclein

found in Parkinson Dz

Difference between lewy body dementia and Parkinson Dz

Lewybody dementia has early onset (parkinson takes a while) cortical lewy body= Ha'lew'cinations

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 **C**audate loses **A**ch and **G**aba

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

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

With hydrocephalus you should be thinking the stretching of the \_\_\_\_\_\_\_

corona radiata

Spongiform Encephalopathy

Degenerative dz of **prion protein** most common form is CJD

Creutzfield Jakob dz (CJD)

spongiform encephalopathy rapid dementia Ataxias and startle myoclonus

What is the role of the astrocytes?

Forms the blood brain barrier

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

Kids: Pilocytic Astrocytoma Adults: Glioblastoma Multiforme

What is the role of Oligodendrocytes?

Myelinate axons in the CNS

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

Kids: n/a Adults: oligodendroglioma

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

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

Benign tumor of the **arachnoid** cells Most common benign tumor **Whorl cells**

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+

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

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

Medulloblastoma /histo

CNS tumor in kids Neruoectodermal Tumor Former wright rosettes small blue cells

Which cells line the ventricular space in the brain

**ependymal** cells

ependymoma

Malignancy of the ependymal cells commonly arises in 4th ventricle hydrocephalus?

Craniopharygioma

tumor that arises from the remnants of the **Rathe's pouch** (pituitary) compression of **optic chiasm** Bitemporal hemianopsia

What is Bitermporal Hemianopsia and what causes it

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

Metachromatic Leukodystrophy

Demyelinating DO/lysosomal storage dz deficiency of **arylsulfatase** myelin _cant get degraded_ so it builds up in the lysosomes

Krabbe Dz

Demyelinating disorder/lysosomal storage dz deficiency of **galactocerebrosidase** build up of galactocerebroside -\> destroys myelin

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_

whats diagnostic for Multiple sclerosis

**Oligoclonal bands**

_progressive multifocal leukodystrophy_ which infection is associated with it

demylinating disorder Destruction of **oligodendrocytes** **JC virus infection**

Hydrocephalus Ex Vacuo

When you have dilated ventricles but its due to **Brain atrophy** the ventricles have more space to expand. **ICP** is normal