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Flashcards in CNS Pathology Deck (10):
0

Types of cerebral edema.

Vasogenic: increased vascular permeability
-BBB disruption
-localized or generalized

Cytotoxic: increased intracellular fluid
-generalized insults (hypoxia)
-neuronal, glial, or endothelial cell

1

Dandy Walker malformation.

Enlarged posterior fossa
Absent or rudimentary cerebellar vermis
Greatly dilated 4th ventricle represented as a cyst-like structure
Bulging of occipital bone

2

What is Arnold Chiari type II often associated with?

Hydrocephalus (90%)
Myelomeningocele

3

What is lost and what is spared in syringomyeloma?

Lost: thermal and painful sensation
Spared: tactile, joint position, and vibration

4

Mutations of tuberous sclerosis complex.

TSC1 encodes hamartin
TSC2 encodes tuberin
Both proteins bind forming a complex that blocks the kinase mTOR leading to negative control do cell proliferation and high protein synthesis (voluminous cytoplasm)

5

Features of tuberous sclerosis complex including typical triad presentation.

Major:
-facial angiofinromas, hypomelanotic macules, shagreen patch, hamartomas, cardiac rhabdmyomas & multiple retinal nodular hamartomas

Minor: pits in enamel and hamartomatous rectal polyps

Triad: angiofibromas, epilepsy,a me developmental delay

6

Genetics and immunology of multiple sclerosis.

HLA-DR2
Single nucleotide polymorphisms in IL2 and IL7 receptors
CD4+ TH1 activate macrophages
TH17 activate leukocytes causing demyelination
-reactive against self myelin antigens

7

Morphology of MS.

Acute plaque:
-macrophages contain PAS and debris
-perivascular cuffing
-depletion of oligodentrocytes

Inactive plaque:
-little to no myelin left
-gliosis

Shadow plaque:
-border of normal and affected white matter is not sharply delineated

8

Clinical features of MS.

Bladder dysfunction
Ataxia
IgG in CSF
Weakness, spasticity
Visual disturbances
Pins and needles, numbness, pain

9

Amyotrophic lateral sclerosis (ALS).

Muscle atrophy
Mutation in SOD1 which encodes copper zinc superoxide (adverse gain of function - misfolded)
Reduced capacity to detoxify free radicals
UMN and LMN defects
No oculomotor defects
Fasiculations
Spasticity of arms and legs