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