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Pathoma > Chapter 17 - CNS > Flashcards

Chapter 17 - CNS Flashcards

1
Q

What is associated with Neural Tube Defects?

A

Low folate levels PRIOR to conception.

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2
Q

Symptoms of anencephaly?

A

Polyhydramnios…

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3
Q

Cause of Dandy-Walker malformation and presentation?

A

Due to congenital failure of the cerebellar vermis. Results in massively dilated 4th ventricle, with absent cerebellum…

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4
Q

What other defects are associated with Arnold-Chiari Malformation Type II?

A

Meningomyelocele and syringomyelia

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5
Q

What is syringomelia and where does it most often occur?

A

Cystic degeneration of the spinal cord. Most often occurs at C8-T1, spares dorsal columns but compresses spinothalamic tract in the anterior white commissure.

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6
Q

“Floppy Baby” Syndrome?

A

Werdnig-Hoffman Disease (inherited degeration of the anterior motor horn.)

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7
Q

ALS involves the degeneration of what structures?

A

UMN AND LMN…

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8
Q

Cause of familial ALS?

A

Superoxide dismutase (SOD1) mutation…

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9
Q

Cause of Friedreich Ataxia?

A

Expansion of trinucleotide repeat (GAA) in frataxin gene.

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10
Q

Cardiac association with Friedreich Ataxia?

A

Hypertrophic Cardiomyopathy

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11
Q

Most common cause of bacterial meningitis in newborn?

A

Group B strep, E coli, and Listeria

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12
Q

Most common cause of bacterial meningitis in children/teenagers?

A

N meningitidis

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13
Q

Most common cause of bacterial meningitis in adults and elderly?

A

Strep pneumonia

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14
Q

Most common cause of bacterial meningitis in non vaccinated kids?

A

H influenza

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15
Q

CNS defect seen with DM, or insulinomas…?

A

Global Cerebral Ischemia, due to repeated episodes of hypoglycemia.

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16
Q

How does moderate global cerebral ischemia present hystologically?

A

Cortical Laminar Necrosis (triangular appearance) from damage to the pyramidal nuerons of layers 3, 5, and 6.

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17
Q

Red neurons are seen how long after an ischemic stroke?

A

12 hours post infarction.

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18
Q

Coagulative necrosis is seen how long after ischemic stroke? And is followed by what?

A

24 hours, followed by neutrophils (1-3 days), nicroglial cells (4-7 days), and granulation tissue (2-3 weeks)

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19
Q

What are high risk groups for berry aneurisms?

A

Marfan syndrome, ADPKD, and Ehlers-Danlos

20
Q

Classic cause and symptoms of an epidural hematoma

A

Fracture of temporal bone leading to a rupture of middle meningeal artery. Presents with Lens-shaped lesion on CT. Lucid interval may precede signs.

21
Q

Classic cause and symptoms of subdural hematoma

A

Due to tearing of bridging veins. Presents with a crescent-shaped lesion of CT and progressive neurologic signs.

22
Q

What artery is often compressed with a subfalcine herniation?

A

The ACA!

23
Q

Metachromatic Leukodystrophy

A

Due to deficiency in arylsulfatase (AR), which is necessary for the degradation of myelin. Accumulates in the lysosomes of oligodedrocytes…

24
Q

Krabbe disease

A

Due to deficiency of galactocerebroside b-galactosidase (AR). Galactocerebroside accumulates in macrophages. Also necessary for production/maintenance of myelin.

25
Q

What is lost in Multiple Sclerosis?

A

CNS myelin AND oligodendrocytes.

26
Q

What is found in the CSF of patients with Multiple Sclerosis?

A

Increased lymphocytes, increased Ig with oligoclonal IgG bands, and myelin basic protein.

27
Q

Cause of Subacute Sclerosing Panencephalitis?

A

Slowly progressing, persistent infection by measles virus.

28
Q

Cause of Progressive Multifocal Leukoencephalopathy?

A

JC virus infection of oligodendrocytes. Usually immunosuppressed.

29
Q

Cause and symptoms of central pontine myelinolysis?

A

Due to rapid correction of hyponatremia. Causes focal demyelination of the pons –> Locked in syndrome.

30
Q

Do you see focal neurological deficits early in Alzheimers?

A

NO

31
Q

What gene is protective and what is associated with risk for alzheimer’s?

A

ApoE2 protective, ApoE4 increased risk. Also mutations in presenilin1/2 linked to familial Alzheimer’s.

32
Q

What histologic changes common in Alzheimers?

A

Extracellular Ab amyloid deposition (Aa normal cleavage) and neurofibrillary tangles (hyperphosphorylated tau protein intracellular!)

33
Q

Histologic characteristics of Pick Disease?

A

Intracellular round aggregates of tau protein (Pick bodies).

34
Q

What illicit drug was early linked to Parkinson’s?

A

MPTP

35
Q

Histology of Parkinson’s?

A

Loss of pigmented neurons in the Substantia Nigra and round, eosinophilic inclusions of a-synuclein in affected neurons.

36
Q

Cause of Huntington Disease?

A

Degeneration of GABAergic neurons in the caudate nucleus due to expanded CAG repeats in the huntington gene of chrom. 4

37
Q

Common complication of Normal Pressure Hydrocephalus?

A

Stretching of the Corona Radiata! Presents as ‘wet, wacky, and wobbly’

38
Q

Common sites of primary tumor to metastasize to the brain?

A

Lung, breast, and kidney

39
Q

Histology of Glioblastoma Multiforme?

A

Pseudopalisading tumor cells surrounding areas of necrosis. Tumors are GFAP positive (tumor of Astrocytes)

40
Q

Histology of Meningioma?

A

Whorled pattern with psammoma bodies. Tumor of Arachnoid Cells

41
Q

Histology of Schwannoma?

A

S-100 positive tumors at the cerebellopontine angle. Tumor of Schwann cells…

42
Q

Histology of Oligodendroglioma?

A

Fried egg appearance. Often calcified. Malignant tumor of oligodendrocytes.

43
Q

Histology of Pilocytic Astrocytoma?

A

Rosenthal fibers (thick eosinophilic processes of astrocytes) and eosinophilic granular bodies. GFAP positive (Childhood, benign tumor of astrocytes).

See cystic lesion with a mural nodule on imaging

44
Q

Histology of medulloblastoma?

A

Homer-Wright rosettes (blue cells wrapped around pink areas), small, round blue cells. Malignant childhood tumor of the neuroectoderm…

45
Q

Histology of Ependymoma?

A

Perivascular pseudorosettes. Malignant childhood tumor of ependymal cells…

46
Q

Histology of Craniopharyngioma?

A

Cacifications derived from tooth like remnants of Rathke’s pouch. Can present as bitemporal hemianopsia due to compression of the optic chiasm…

Pathoma (15 decks)

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