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Pathology finish > 68. dementia and neurodegenerative disorders, demyelinating diseases, prion disease > Flashcards

68. dementia and neurodegenerative disorders, demyelinating diseases, prion disease Flashcards

1
Q

68-1. degenerative disease and dementias

subtypes of degenerative disorder (5)

A 
alzheimer disease
pick disease
parkinson disease
huntington disease
amyotrophic lateral sclerosis
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2
Q

68-1. degenerative disease and dementias

clinical feature of alzheimer disease (5)

A 
slow onset memory loss
progressive disorientation
loss of learned motor skill and language
change in behavior and personality
patient become mute and bedridden
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3
Q

68-1. degenerative disease and dementias

pathogenesis of alzheimer disease

A

accumulation of beta amyloid

beta amyloid is produced by amyloid precursor protein (APP), undergoes beta cleavage

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4
Q

68-1. degenerative disease and dementias

related gene of alzheimer disease

A

apolipoprotein E4

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5
Q

68-1. degenerative disease and dementias

gross morphology of alzheimer disease

A

cerebral atrophy : narrow gyri, widening sulci, dilation of ventricle

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6
Q

68-1. degenerative disease and dementias

characteristics of pick disease

A

degeneration of frontal and temporal cortex

behavioral and language symptoms arise early

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7
Q

68-1. degenerative disease and dementias

microscopic morphology of pick disease

A

round aggregate of tau protein (pick body) in neuron of cortex

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8
Q

68-1. degenerative disease and dementias

affecting region of parkinson disease

A

dopaminergic neuron of substantia nigra

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9
Q

68-1. degenerative disease and dementias

clinical features of parkinson disease (4)

A

tremor
rigidity
akinesia/bradykinesia
postural instability

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10
Q

68-1. degenerative disease and dementias

microscopic morphology of parkinson disease (2)

A

loss of pigmented neuron in substantia nigra

lewy body formation on affected neuron

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11
Q

68-1. degenerative disease and dementias

affected neuron of huntington disease

A

GABAergic neuron in caudate nucleus of basal ganglia

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12
Q

68-1. degenerative disease and dementias

genetic predisposition of huntington disease

A

autosomal dominant disorder (chromosome 4), expanded trinucleotide repeats (CAG) in huntington gene

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13
Q

68-1. degenerative disease and dementias

clinical features of huntington disease (3)

A

onset on 40s
early onset with motor symptom (twitching)
progression to forgetfulness and depression

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14
Q

68-1. degenerative disease and dementias

characteristics of amyotrophic lateral sclerosis (ALS)

A

degeneration of motor neurons in the ventral horn of spinal cord

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15
Q

68-1. degenerative disease and dementias

etiology of amyotrophic lateral sclerosis

A

defect on chromosome 21, SOD1 gene

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16
Q

68-1. degenerative disease and dementias

clinical features of amyotrohpic lateral sclerosis (4)

A

onset on 50s
early symptoms include asymmetric weakness of hand
progression with muscle strength and mass diminish with involuntary contract
eventually involve respiratory muscle, cause respiratory failure and recurrent infection

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17
Q

68-2. prion disease

name of prion disease

A

spongiform encephalopathy : degenerative disease due to prion protein

18
Q

68-2. prion disease

types of spongiform encephalopathy (2)

A

Creutzfeldt Jakob disease (CJD)

variant Creutzfeldt Jakob disease (vCJD)

19
Q

68-2. prion disease

pathogenesis of spongiform encephalopathy

A

conversion of normal prion (PrPc) to beta pleated conformation (PrPsc)
- conversion can be sporadic, familial and transmitted

20
Q

68-2. prion disease

characteristics of CJD

A

rapidly progressive dementia associated with ataxia

21
Q

68-2. prion disease

characteristics of vCJD

A

slow progression of dementia, after exposure to bovine spongiform encephalopathy

22
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

subtypes of primary disease of myelin (2)

A

demyelinating disease : conditions that damage to myelin

dysmyelinating disease : malformed and defect myelin

23
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

subtypes of demyelinating disease (4)

A

multiple sclerosis
progressive multifocal leukoencephalopathy
acute disseminated encephalomyelitis
central pontine myelinolysis

24
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

etiology of multiple sclerosis

A

autoimmune destruction of myelin (HLA-DR2)

25
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

epidemiology of multiple sclerosis

A

predominant in 20-40 years old women

26
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

clinical features of multiple sclerosis

A 
relapsing, remitting symptoms of
visual impairment on one eye
ataxia
motor and sensory impairment
change in cognitive function
27
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

etiology of progressive multifocal leukoencephalopathy

A

JC virus infection

28
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

clinicals of progressive multifocal leukoencephalopathy

A

rapidly progressive visual loss, weakness, dementia

29
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

etiology of acute disseminated encephalomyelitis (3)

A

bacterial infection
viral infection
vaccine for measles, mumps, rubella

30
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

pathomechanism of acute disseminated encephalomyelitis

A

cross reaction of pathogen antigen with myelin antigen

31
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

clinicals of acute disseminated encephalomyelitis

A

develop 1-2 weeks after infection

rapidly progress symptoms (headache, lethargy, coma)

32
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

etiology central pontine myelinolysis

A

rapid correction of hyponatermia

33
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

clinical signs of central pontine myelinolysis

A

acute bilateral paralysis

34
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

etiology of leukodystrophies

A

inherited mutation of enzymes

35
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

metabolic disorders affect to brain (3)

A

hypoglycemia
hyperglycemia
hepatic encephalopathy

36
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

brain change related to hypoglycemia (2)

A

injury on hippocampus

spared purkinje cell of cerebellum

37
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

cause of hyperglycemia

A

DM

38
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

consequences of hyperglycemia (3)

A

confusion
stupor
coma

39
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

pathomechanism of hepatic encephalopathy

A

liver fails to clear ammonia, cause change in brain function

40
Q

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain

types of neurotoxic agents (7)

A 
metal (arsenic, mercury)
industrial chemicals
methanol
ethanol
environmental pollutant
chemotherapeutic agent
ionizing radiation

Pathology finish (30 decks)

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