CNS Path: Neurodegenerative

Question 1

characterized by nerve cell loss and gliosis, often in specific regions of the brain which correlate with clinical symptoms, gross atrophy may be seen

Answer 1

neurodegenerative diseases

Question 2

akinetic movement disorder of the basal ganglia

Answer 2

parkinson disease

Question 3

hyperkinetic movement disorder of the basal ganglia

Answer 3

huntington disease

Question 4

_______ symptoms occur when the degenerative process affects systmes outside of the motor tracts, where motor neurons and their processes are preserved

Answer 4

extrapyramidal

Question 5

-pill rolling tremor, stooped posture, slowness of movement, shuffling gait, diminished facial expression

Answer 5

parkinsonism

Question 6

• resting tremor
• cogwheel rigidity
• bradykinesia
• festinating gait (loss of righting reflexes)
• parkinsonian personality

Answer 6

idiopathic parkinson disease

Question 7

• loss of pigment of substantia nigra
• Lewy bodies, pigmented neuronal cell loss and gliosis
• alpha synuclein gene assoc’d with synapse

Answer 7

parkinson disease

Question 8

-dementia, Parkinsonian features, hallucinations, rapid progression

Answer 8

dementia with lewy bodies

Question 9

progressive supranuclear palsy, multiple system atrophy, punch drunk syndrome, toxic parkinsonism, post encephalitic are differentials for?

Answer 9

parkinsonian syndromes

Question 10

acute parkinsonian syndrome and destruction of neurons in the substantia nigra follows exposure to MPTP, a contaminant in the illicit synthesis of psychoactive merperidine analogs

Answer 10

toxic parkinsonism

Question 11

• parkinsonism seen in professional boxers, similar to CTE in nfl players
• related to neuronal micro trauma, due to repeated blows to the head
• gliosis and destruction of nigrostriatal neurons is the end stage, resulting in a clinical disease that is virtually identical to parkinson

Answer 11

dementia pugilistica

Question 12

• oligodendroglial alpha synuclein positive inclusions

- parkinsonism, autonomic failure (shy drager), cerebellar findings (olivopontocerebellar degeneration)

Answer 12

multiple system atrophy

Question 13

• autosomal dominant genetic disorder, characterized by choreiform movements, cognitive deterioration, emotional disturbances
• on chromosome 4, contains unstable tri nucleotide repeat, CAG that codes for glutamic acid
• complete dominance

Answer 13

Huntington disease

Question 14

• choreiform movements, neuropysch disturbances and slowly progressive dementia
• flattened, atrophic caudate
• cell loss and gliosis in caudate nucleus (dorsal putamen, globus pallidus, and nucleus accumbens affected later)

Answer 14

huntington disease

Question 15

children inheriting the gene from their father have either juvenile onset disease or onset approx 3 years early

Answer 15

anticipation

Question 16

Alzheimer’s, dementia with lewy bodies, pick’s lobar atrophy, prion disease

Answer 16

dementias

Question 17

• diffuse plaques and neuritic plaques

- neurofibrillary degeneration uncommon in neocortex, entorhinal cortex affected early

Answer 17

normal aging

Question 18

focal, spherical collections of dilated tortuous neuritic processes often around a central amyloid core, which may be surrounded by a clear halo

Answer 18

neuritic plaques

Question 19

bundles of filaments in cytoplasm of neurons that displace or encircle the nucleus, insoluble and resistance to clearance, contain tau protein

Answer 19

nuerofibrillary tangles

Question 20

pathologic changes seen in _________ disease occur in nearly all Down syndrome patients by age 45, APP coded for on chromosome 21, abnormal processing leads to deposits of insoluble Beta pleated amyloid protein

Answer 20

Alzheimer

Question 21

• aphasa, apraxia, agnosia, diminution of executive function
• progressive over time
• impaired level of function
• 50% of people >85yo are affected
• pneumonia is often final mechanism of death

Answer 21

Alzheimers, impairment of recent memory

Question 22

• onset 45-65 years, rare after 75
• 80% sporadic, 5-10 year duration
• frontal lobe symptoms: personality changes, haphazard behavior, lack of planning, antisocial, obsessive compulsive, language deficits
• anterior temporal lobe: fluent aphasia, semantic memory loss, kluver bucy syndorome
• memory relatively retained

Answer 22

pick disease

Question 23

-severe localized atrophy of frontal lobes and anterior temporal lobes
-knife edge atrophy, usually on left
-ballooned neurons, cell loss and gliosis
-

Answer 23

Pick disease

Question 24

Pick’s disease without Pick cells or pick bodies, slightly more common, characteristic laminar spongy change of cortex, subcortical gliosis

Answer 24

dementia of frontal lobe type

Question 25

• normal PrP is present in neurons, function unknown

- conformational change of PrP from alpha helix to beta pleated sheet occurs, usually after exposure to altered form

Answer 25

spongiform encephalopathies (prion diseases)

Question 26

• 50 to 70 years old, with rapidly evolving dementia, startle myoclonus, EEF repetitive sharp waves
• early: personality change, impaired judgement, gait abnormalities, vertigo
• die within 7 months of diagnosis

Answer 26

CJD

Question 27

• vacuolation of neuropil, within nerve cell bodies and neuronal processes
• PrPsc can be demonstrated in tissue

Answer 27

CJD