Flashcard Set Preview
| Side A | Side B | ||
| 1 |
Animal prion disease
|
BSE in cows (bovine spongiform enceph)
|
|
| 2 |
Prions are...
|
transmissable damaged PrP protein coded by PRNP on chromosome 20. resistant to proteases.
|
|
| 3 |
How is PrP damaged in prions?
|
alpha-PrPc to beta-PRPsc
|
|
| 4 |
Two causes of iatrogenic (from a medical tx) infections of CJ Disease
|
corneal transplants
hGH
|
|
| 5 |
Clinical findings in CJ Disease
|
Rapidly progressing dementia
Startle myoclonus (involuntary jerking when surprised)
|
|
| 6 |
Prognosis for CJ patient
|
0--it is always fatal. Avg of 7 months
|
|
| 7 |
How does variant CJ D present differently?
|
younger people
neuro sx progress slower, but behavior issues noticeable earlier
no alterations...
|
|
| 8 |
What is vCJ D linked to?
|
BSE
|
|
| 9 |
**Microscopic findings for vCJ?
|
cortical plaques surrounded by a halo of spongiform change
|
|
| 10 |
In fatal familial insomnia, what does it have?
1. Spongiform change
2. Neuronal loss
3....
|
No
Yes
Yes
Yes
|
|
| 11 |
This inherited prion disease begins with cerebellar ataxia then progressive dementia. Death...
|
Gerstmann-Straussler-Scheinker S
|
|
| 12 |
Microscopic findings of GSSS?
|
Kuru plaques--aggregates of PrPsc
|
|
| 13 |
Most common demyelinating disorder
|
MS
|
|
| 14 |
Immune mechanism that damges myelin in MS
|
CD4+ TH1 cells vs. myelin --> ING-g --> MACs
|
|
| 15 |
Where do you see the gross glassy, gry plaques in MS?
|
White matter; around the lateral ventricles
|
|
| 16 |
From an MS pt plaque, you see fewer MACs, axons, and oligodendrocytes.
Stage of disease?
What...
|
Inactive plaque--little/no myelin anymore
Astrocytes w/ gliosis
|
|
| 17 |
From an MS pt plaque, you see active plaques.
What process is going on?
There is depletion...
|
Demyelination
Oligodendrocytes
Axons
|
|
| 18 |
What is the first common sx in MS?
|
Unilateral loss of vision (CN 2)
|
|
| 19 |
What lab test will order in MS?
Results?
|
CSF fluid
Elevated protein from B cell's oligoclonal bands
|
|
| 20 |
Two MS variants
|
1. Neuromyelitis optica / Devic
2. Marburg (acute MS)
|
|
| 21 |
How is Devic D different from MS
|
Presents as bilateral optic neuritis / spinal cord
Asians
More destructive--with more grey...
|
|
| 22 |
How is Margburg different than MS?
|
Worse--more rapidly progressive
|
|
| 23 |
A patient comes in a couple weeks after a vaccination. He has H/A, lethargy, then goes into...
|
ADEM (acute disseminated encephalomyelitis)
20% die, the rest complete recovery
viral infection...
|
|
| 24 |
Acute necrotizing hemorrhagic encephalomyelitis (ANHE)
Cause
Prognosis
Typical pt
|
URI
usually fatal
kids/young adults
|
|
| 25 |
Pt develops rapidly evolving quadriplegia and demyelination of the anterior pons.
Dx?
Causes...
|
Central pontine myelinolysis
alcoholism, liver transplant, osmolar/electrolyte imbalance
|
|
| 26 |
Degenerative diseases are cuased by a loss of neurons in the grey matter.
Cause?
|
protein aggregates that won't degrade
|
|
| 27 |
What % of Alzheimer's is familial?
|
5-10%
|
|
| 28 |
Gross findings in Alzheimer's
|
1. cortical atrophy
2. hydrocephalus ex vacuo
|
|
| 29 |
Microscopic findings in Alzheimer's (2)
|
Senile (neuritic) plaques
Neurogibrillary tangles
|
|
| 30 |
Senile plaques
1. Stain
2. What causes stain?
|
1. Congo red
2. amyloid AB
|
|
| 31 |
Neurofibrillary tangles
1. stain
2. definition
3. composed of...
|
1. silver stain
2. filaments around the neuron's nucleus
3. hyper-Ph tau protein
|
|
| 32 |
In Alzheimer's, this is made of actin and found in the pyramidal cells of the hippocampus
|
hirano bodies
|
|
| 33 |
Pt presents with alteratins in personality and language.
Dx
Cause
|
Pick D
Atrophy of frontal, temporal lobes
|
|
| 34 |
Pt has diminished facial expressions, stooped posture, dlow voluntary movements, festinating...
|
Parkinson's
Nigrostrital dopaminergic system
L-Dopa (DOES NOT REVERSE THE DISEASE)
|
|
| 35 |
What does the brain look like in PD under the microscope?
|
loss of pigmented neurons
Lewy bodies (made of alpha-synuclein)
|
|
| 36 |
Complicating disease of PD
|
dementia (10%)
|
|
| 37 |
What element of PD can cause dementia?
|
Lewy Body D
dementia is the primary complaint
|
|
| 38 |
Hungtington D
1. Inheritance
2. Age of onset
3. Damage occurs in the...
|
tri-NT with cAG repeats in HD gene
30-40s
Caudate nucleus (most sig)
|
|
| 39 |
What neurons are damaged in HD?
|
medium spiny neurons that use the neurotransmitter GABA
|
|
| 40 |
First sx of HD is usually...
Severity of sx are proportional to...
|
Choreiform (motor, involuntary jerky movements)
Degree of striatal degeneration
|
|
| 41 |
5 y/o child presents with gait ataxia, clumsiness, absent DTRs, loss of pain/temp sensation,...
|
1. Friedrich ataxia
2. low levels of frataxin
3. Posterior columns of spinal cord
4. CHF,...
|
|
| 42 |
Pt presents with red spots on the face, neck. Ataxia. Sino-pulmonary infections.
Dx?
What...
|
Ataxia-telangiectasia
Xrays (cause severe chrom damage)
Amphicytes (big nuclei in many organs)
Death...
|
|
| 43 |
Pt presents with cramping, spasticity of arms, legs. Asymmetric weakness in hands. Hyper-reflexia....
|
ALS
Pulmonary infections
|



No comments yet! Be the first to add a comment below!
Please login to post comments.
After login, we will forward you back to this flashcard.