CNS path

Question 1

Parkinsonism

Answer 1

extrapyramidal symptoms- degeneratin outside motor tracts
motor strength is normal
pill rolling tremor resting tremor, stooped posture, slowness of movement, shuffling gait, diminished facies

Question 2

parkinsons disease morphology

Answer 2

loss of pigment in substantia nigra

lewy bodies with pigmented neuronal cell loss and gliosis

Question 3

parkinson’s path and pathogenesis

Answer 3

a-synuclein gene- found in large mts in lewy body
ubiquitin related enzymes

pigmented nerve cell loss, lewy body in brainstem and cortex

10-15% develop dementia

Question 4

dementia with lewy bodies

Answer 4

dementia, parkinsonian features, hallucinations, rapid progression

will have brainstem lewy body pathology identical to parkinson disease often with cortical lewy bodies, plus alzheimer disease pathology

Question 5

toxic parkinsonism

Answer 5

acute parkinsonian syndrome and destruction in substantia nigra follows exposure to MPTP (contaminant in illicit synthesis of psychoactive meperidine analogs)

Question 6

dementia pugilistica

Answer 6

seen in professonal boxers, retired NFL football palyers
called chronic traumatic encephalopathy
neuronal microtrauma due to repeated blows to head- gliosis and destruction of nigrostriatal neurons is end stage resulting in clinical disease identical to parkinson

Question 7

multiple system atrophy (MSA)

Answer 7

find oligodendroglial a-synuclein positive inclusions (not in neurons)
parkinsonism, autonomic failure, cerebellar findings

Question 8

Huntington disease

Answer 8

AD genetic disorder
Choreiform movements, cognitive deterioration, emotional disturbances
HD gene- chr 4, unstable trinucleotide repeat (37-86 copies CAG vs 19 in normal controls coding for glutamic acid)

complete dominance

gross- atrophic, flattened caudate
microscopic- cell loss and gliosis in caudate nucleus

Question 9

huntington’s genetics

Answer 9

children inheriting gene from father have either juvenile onset disease or onset approx 3 years early (anticipation)
onset typically in 4th-5th decade

Question 10

amyloid precursor protein coded for on

Answer 10

chromosome 21
alzheimer disease occurs in nearly all down syndrome (trisomy 21) pts by age of 45

abnormal APP processing leads to deposits of insoluble B-pleated amyloid protein

Question 11

alzheimer’s impairment of

Answer 11

recent memory
aphasia- naming
apraxia- motor
agnosia- object
dimunition of executive function- making change, keeping track of time
pneumonia is often the final mechanism death

Question 12

alzheimer’s

Answer 12

most common cause of dementia in elderly (65%)
short term memory and executive fxn most severly affected cognitive features
degenerative cell loss leads to gross atorophy of entire brain with hydrocephalus ex vacuo
neuritic plaques, neurofibrillary tangles, cell loss and gliosis, granulovacuolar degeneration, amyloid angiopathy

Question 13

Pick disease

Answer 13

pre-senil: onset 45-65 years
80% sporadic
frontal lobe dementia- personality changes, haphazard behavior, lack of planning, anti-social, ocd, language deficits
anterior temporal lobe symptoms- fluent aphasia, semantic memory loss, kluver bucy syndrome

memory is relatively retained

Question 14

gross appearance of pick disease

Answer 14

localized atrophy of frontal lobes and anterior temporal lobes, knife edge atrophy; left>right

pick cells (ballooned neurons) and pick bodies with cell loss and gliosis - anti- tau

Question 15

dementia of frontal lobe type

Answer 15

picks disease without pick cells or pick bodies

Question 16

spongiform encephalopathies

Answer 16

misfolded proteins
autocatalytic
creutzfeldt jakob, kuru
chronic wasting disease (Deer, elk)

normal PrP is present in neurons but fxn unknown- conformational change from alpha helix to beta pleated sheet

Question 17

CJD

Answer 17

patients are 50-70 years old- rapidly evolving dementia, startle myoclonus, characterstic EEG pattern of repetitive sharp waves

early symptoms- personality changes, impaired judgement, gait abnormalities, vertigo
most pts will die within 7 months, frequently within 3 months if patients are laying in bed and get pneumoniA

Question 18

don’t confuse CJD with

Answer 18

JC virus

causes progressive multifocal leukoencephalopathy

Question 19

CJD path

Answer 19

gross brain may be normal unless pt lives long enough

microscopic- vacuolation of neuropil, within nerve cell bodies and neuronal processes

status spongiosis

abnormal PrP deposits- abnormally folded, autocatalytic protein (not a virus- but features of infection)