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

Question 1

68-1. degenerative disease and dementias

subtypes of degenerative disorder (5)

Answer 1

alzheimer disease
pick disease
parkinson disease
huntington disease
amyotrophic lateral sclerosis

Question 2

68-1. degenerative disease and dementias

clinical feature of alzheimer disease (5)

Answer 2

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

Question 3

68-1. degenerative disease and dementias

pathogenesis of alzheimer disease

Answer 3

accumulation of beta amyloid

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

Question 4

68-1. degenerative disease and dementias

related gene of alzheimer disease

Answer 4

apolipoprotein E4

Question 5

68-1. degenerative disease and dementias

gross morphology of alzheimer disease

Answer 5

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

Question 6

68-1. degenerative disease and dementias

characteristics of pick disease

Answer 6

degeneration of frontal and temporal cortex

behavioral and language symptoms arise early

Question 7

68-1. degenerative disease and dementias

microscopic morphology of pick disease

Answer 7

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

Question 8

68-1. degenerative disease and dementias

affecting region of parkinson disease

Answer 8

dopaminergic neuron of substantia nigra

Question 9

68-1. degenerative disease and dementias

clinical features of parkinson disease (4)

Answer 9

tremor
rigidity
akinesia/bradykinesia
postural instability

Question 10

68-1. degenerative disease and dementias

microscopic morphology of parkinson disease (2)

Answer 10

loss of pigmented neuron in substantia nigra

lewy body formation on affected neuron

Question 11

68-1. degenerative disease and dementias

affected neuron of huntington disease

Answer 11

GABAergic neuron in caudate nucleus of basal ganglia

Question 12

68-1. degenerative disease and dementias

genetic predisposition of huntington disease

Answer 12

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

Question 13

68-1. degenerative disease and dementias

clinical features of huntington disease (3)

Answer 13

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

Question 14

68-1. degenerative disease and dementias

characteristics of amyotrophic lateral sclerosis (ALS)

Answer 14

degeneration of motor neurons in the ventral horn of spinal cord

Question 15

68-1. degenerative disease and dementias

etiology of amyotrophic lateral sclerosis

Answer 15

defect on chromosome 21, SOD1 gene

Question 16

68-1. degenerative disease and dementias

clinical features of amyotrohpic lateral sclerosis (4)

Answer 16

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

Question 17

68-2. prion disease

name of prion disease

Answer 17

spongiform encephalopathy : degenerative disease due to prion protein

Question 18

68-2. prion disease

types of spongiform encephalopathy (2)

Answer 18

Creutzfeldt Jakob disease (CJD)

variant Creutzfeldt Jakob disease (vCJD)

Question 19

68-2. prion disease

pathogenesis of spongiform encephalopathy

Answer 19

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

Question 20

68-2. prion disease

characteristics of CJD

Answer 20

rapidly progressive dementia associated with ataxia

Question 21

68-2. prion disease

characteristics of vCJD

Answer 21

slow progression of dementia, after exposure to bovine spongiform encephalopathy

Question 22

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

subtypes of primary disease of myelin (2)

Answer 22

demyelinating disease : conditions that damage to myelin

dysmyelinating disease : malformed and defect myelin

Question 23

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

subtypes of demyelinating disease (4)

Answer 23

multiple sclerosis
progressive multifocal leukoencephalopathy
acute disseminated encephalomyelitis
central pontine myelinolysis

Question 24

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

etiology of multiple sclerosis

Answer 24

autoimmune destruction of myelin (HLA-DR2)

Question 25

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

Answer 25

predominant in 20-40 years old women

Question 26

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain clinical features of multiple sclerosis

Answer 26

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

Question 27

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain etiology of progressive multifocal leukoencephalopathy

Answer 27

JC virus infection

Question 28

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain clinicals of progressive multifocal leukoencephalopathy

Answer 28

rapidly progressive visual loss, weakness, dementia

Question 29

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain etiology of acute disseminated encephalomyelitis (3)

Answer 29

bacterial infection viral infection vaccine for measles, mumps, rubella

Question 30

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain pathomechanism of acute disseminated encephalomyelitis

Answer 30

cross reaction of pathogen antigen with myelin antigen

Question 31

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain clinicals of acute disseminated encephalomyelitis

Answer 31

develop 1-2 weeks after infection | rapidly progress symptoms (headache, lethargy, coma)

Question 32

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain etiology central pontine myelinolysis

Answer 32

rapid correction of hyponatermia

Question 33

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain clinical signs of central pontine myelinolysis

Answer 33

acute bilateral paralysis

Question 34

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

Answer 34

inherited mutation of enzymes

Question 35

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain metabolic disorders affect to brain (3)

Answer 35

hypoglycemia hyperglycemia hepatic encephalopathy

Question 36

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain brain change related to hypoglycemia (2)

Answer 36

injury on hippocampus | spared purkinje cell of cerebellum

Question 37

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

Answer 37

DM

Question 38

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain consequences of hyperglycemia (3)

Answer 38

confusion stupor coma

Question 39

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

Answer 39

liver fails to clear ammonia, cause change in brain function

Question 40

68-3. primary disease of myelin. acquired metabolic and toxic disturbance of the brain types of neurotoxic agents (7)

Answer 40

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