Micro: Other Neuro infections Flashcards

1
Q

transmissible spongiform encephalopathies (TSE)

A

caused by prions
FATAL dementias
LONG INCUBATION but rapidly progressive
multifocal spongiform changes

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2
Q

kuru

A

to tremble with fear

transmitted by ritual cannibalism

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3
Q

Creutzfeldt-Jakob disease (CJD)

A
most common human TSE
Sx: dementia, myoclonus, ataxia
end stage: akinetic mutism
cerebellar spongiform change, lack of amyloid plaques
check CSF to rule out other disease
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4
Q

CJD

  1. sporadic
  2. iatrogenic
  3. familial
A
  1. most cases; 60 yrs; most dead in a year; PERIODIC COMPLEX on EEG
  2. dural/corneal grafts, hGH (NO blood transmission); short incubation
  3. AD inheritance; specific mutations; 45-50 yrs; 2-4 yr survival
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5
Q

Gerstmann-Straussler-Scheinker disease (GSS)

A

AD inheritance
mutation
48 y/o, 5 yr survival
Sx: ataxia, gait abnormalities (dementia less common)
atypical EEG (no characteristic findings)
amyloid plaques and spongiform changes

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6
Q

fatal familial insomnia (FFI)

A
AD inheritance
mutations
49 y/o, 13 mo survival
SLEEP DISTURBANCE, AUTONOMIC dysfunction
Neuronal loss (no plaques or spongiform change)
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7
Q

variant CJD

A

mad cow disease (cow got it from sheep)
blood transmission (France, Great Britain), CONSUMPTION of CONTAMINATED BEEF
detected in LYMPH tissue
age: 29 y/o, 14 mo survival
HOMOZYGOUS for METHIONINE at codon 129
Sx: ANXIETY, DEPRESSION, VISUAL PROBs, akinetic mutism
Dx: atypical EEG, TONSIL Bx, neuropsych probe for more than 6 months
FLORID (daisy-like) plaques, spongiform change in BASAL GANGLIA

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8
Q

scrapie

A

sheep and goats
ataxia
alimentary tract transmission

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9
Q

bovine spongiform encephalopathy

A

cow

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10
Q

chronic wasting disease

A

elk and deer

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11
Q

prion

A

cell surface glycoprotein anchored by fatty acid addition
highly conserved
exon on chromosome 20: neurons and lymphocytes

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12
Q

PrPc

A

normal
inhibits BACE1: inhibit beta amyloid
req. for PrPSc formation

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13
Q

PrPSc

A
disease causing: chain rxn causing more proteins to misfold
spontaneous or infection or ingestion
misfolded (same AA seq. as PrPc)
resistant to proteases
aggregates in cells: apoptosis
CNS disease
SPECIES BARRIER exists
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14
Q

polyoma virus

A

circular dsDNA around histone
non-enveloped icosahedral capsid
replicate in respiratory tract

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15
Q

polyoma virus

  1. permissive cells
  2. non-permissive
A
  1. replication, assembly, cell lysis

2. transformation of cell, integration into host genome

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16
Q

T antigens

  1. immortalize cells
  2. transform cells
A
early genes of polyoma
initiate DNA replication
responsible for transformation
1. small and large
2. small and middle
17
Q

polyoma late genes

A

encode structural proteins

18
Q

BK virus

A

polyoma virus
most people seropositive
isolated in urine of AIDS pts
little disease

19
Q

JC virus

A
polyoma virus
most people seropositive
only problem in immunodeficient 
PML: progressive demyelinating leukoencephalopathy 
Dx;
20
Q

PML

A

JC
lyses oligodendrocytes
Sx: clumsy, progressive weakness, speech probs
Dx: PCR amplification of virus from CSF or brain biopsy , MRI white matter lesions
high FATALITY rate
Tx: AIDS: change HAART, transplant: alter suppression chemo

21
Q

poliovirus

A
non-enveloped icosahedral capsid
ssRNA
fecal-oral
initial replication: lymph, gut
secondary viremia 
flaccid paralysis: motor neurons destroyed
22
Q
  1. abortive poliomyelitis
  2. aseptic meningitis
  3. paralytic poliomyelitis
A
  1. nonspecific febrile illness; NO CNS involvement
  2. febrile illness and stiff neck, limited CNS infection
  3. febrile illness, flaccid paralysis; gradual recovery over 6 mo: lingering paralysis is permanent; some die
23
Q

Dx of polio

A

virus isolation from stool, rectal or throat swab and CSF

24
Q

inactivated polio vaccine (IPV)

A

advantages: no reversion to virulence
disadvantage: 3 injections and booster; protects against paralysis but does NOT prevent spread of wild virus
only one recommended in US now

25
Q

live oral vaccine (OPV)

A

advantages: cheap, oral, protects against paralysis AND spread
disadvantages: can REVERT and shed in community
used for polio eradication