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Degenerative Flashcards

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0
Q

What areas of the brain are affected by alZiehmers?

A

Hippocampus, partial and frontal lobe white and gray matter loss. Ventriulomegaly seen, sulcal enlargement and gyri atrophy.

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1
Q

What is the most common neurodegenerative disease of familial origin?

A

Parkinson’s

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2
Q

What is the most common cause of dementia in the elderly?

A

AlZiehmers

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3
Q

What is the 2nd most common neuro degenerative disease?

A

Parkinsons

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4
Q

What are the risk factors for parkinsons?

A 
Family history
Male
Head injury
Exposure to well water
Exposure to pesticide
Rural living
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5
Q

What are the 3 cardinal signs of Parkinson’s

A

Tremor, Rigidity(leadpipe, cogwheel), Bradykinesia

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6
Q

Which portion of the brain is affected in Parkinson’s

A

Substantia nigra and nigrostriatal fibers of basal ganglia (loss of dopaminergic neurons)

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7
Q

What is the treatment for Alziehmer’s

A

Cholinesterase inhibitors, Antioxidants, Anti-inflammatory. SSRI’s for depression and Anti-convulsants for seizures.

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8
Q

What is the pharmaceutical treatment for Parkinson’s?

A

Levodopa and dopaminergic agonists

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9
Q

What are surgical options for treating Parkinson’s?

A

Thalamotomy or pallidotomy or neurotransplantation

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10
Q

What is the pathophysiology of Parkinson’s?

A

chaperone protein alpha synuclein only in brain builds up in Lewy bodies of remaining dopaminergic neurons. Oxidative stress causes dopaminergic neruon loss.

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11
Q

What is the etiology of Alzheimers?

A

Age related and Genetic .

presilin gene on chrom 1 and 14 (early onset) and Long arm of chrom 21

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12
Q

What pathology would you see in Alzeihmer’s brain?

A

Imaging early on may show nothing.

Later on ventricularmegaly and hippocampal atrophy and sulcal enlargement.

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13
Q

What is the pathophysiology of Alzheimers?

A

Beta amyloid gene produces amyloid precursor protein
Abeta amyloid extracellular senile/neuritic plaques accumulate in arterial walls.
Neruofibrillary tangles of tau protein intracellularly.
Neuronal loss of white and gray matter in Hippocampus and basal ganglia

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14
Q

What is the age of onset for Alziehmer’s?

A

60-80 but there is an early onset form. one of leading causes of death in US

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15
Q

What is the age of onset for Parkinson’s?

A

0-45 years old

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16
Q

What is the prevalence of Alziehmer’s?

A

17/1000

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17
Q

What is the incidence of Parkinson’s in individuals over 65?

A

increase incidence with age 1/1000

prevalence is .7/1000 over 65

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18
Q

what differentiates ALS from multiple level peripheral nerve compression by spondylosis arthritis?

A

ALS has no sensory symptoms because only VH damage. ALS also involves damage to bulbar nuclei so tounge is affected.

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19
Q

What is the pathophysiology of ALS?

A

astrocytes dont remove glutamate from synaptic cleft resulting in continual MN excitation and oxidative stress. Superoxide dismutase fails and cell death of VH MN and BS bulbar nuclei and Corticospinal tract neurons. Skeletal muscle is den nervated causing weakness and atrophy.

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20
Q

What are the symptoms and causes of ALS?

A

weakness and fasciculation: loss of LMN from VH
weakness and spasticity: loss of corticospinal fibers
tounge weakness and fasiculations: loss of hypoglossal nuclei in BS

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21
Q

what is the initial presentation of ALS?

A

assymetric weakness in one extremity, muscle cramping

progress to bilateral

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22
Q

What is the most common progressive motor disease?

A

ALS

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23
Q

What is the most common cortical dementia disease?

Study These Flashcards
A

Alzhiemer’s #1
Lewy Body #2
Frontal temporal dementia #3

24
What area of the brain is affected in Frontal/Temporal Dementia semantic dementia variant?
temporal so anomia,impaired recognition, impaired word comprehension,
25
What area of the brain is affected in FTD primary progressive aphasia?
Frontal lobe and small amount parietal so can't articulate but comprehension is intact.
26
What is the pathophysiology of FTD?
Tau protein related in all variants leads neuronal cell loss and Gliosis. Cell loss leads to sulcal widening and decrease in gyral volume because of loss of white matter
27
What is the age of onset of FTD?
50-70 most commonly. Onset insidious and progressive in all variants.
28
What is the age of onset for HUntington's?
35-45 years...insidious and progressive
29
Where does atrophy take place in Huntington's?
Caudate nucleus part of basal ganglia
30
what is the etiology of Huntington's?
genetic autosomal dominant chrome 4 CAG repeats (greater than 40 pathology)
31
What is the patient presentation for Huntington's
Family history, memory loss, slurred speech, slowed motor movement Later figidity movements of hands and feet, disintrest in social activities.
32
What is the pathophysiology for Huntington's?
unstable CAG repeats (polyglutamin repeats) interfere with transcription of mitochondria and Anti-oxidation genes. Misfolded proteins and misprocessed waste Abnormal glutamine transmission causes over excitotoxicity and cell death in CC and caudate nucleus cell loss( basal ganglia).
33
What are the diagnostic test for Huntington's?
Genetic test chrom 4 40+CAG repeats | CT and MRI to rule out other diseases-see caudate nucleus atrophy
34
What is the most common of the rare forms of dementia?
Lewy body disease?
35
What 2 diseases have Lewy bodies present in the substantia nigra?
Parkinson's and Lewy Body dementia
36
What are the presenting symptoms for Lewy body dementia?
``` Parkinson sym: fenistrating gait, Bradykinesia gradually progress and accumulate sump. visual hallucinations fluctuating alertness frequent falls Adverse from anti-psychotics respond quickly to chonlinergic inhib ```
37
what is the survival time for Lewy body dementia?
5-7 years
38
how long is the progression of Huntington's?
15-20 years
39
What is the 2nd most common cause of dementia in the US and Europe?
Vascular dementia
40
what are the 3 categories of vascular dementia?
``` multi infarct (step wise increase) diffuse white matter dementia (Binswanger) cerebral autosomal dominant arteriopathy with subcortical infarcts and leaukoencephalopathy ```
41
What is the etiology of CADASIL or vascular dementia?
Notch 3 gene mutation
42
What is the pathophysiology of Vacscular dementia?
depends on the type: multiinfarct is step wise but white matter and CADASIL are slow gradual progression
43
What is the treatment for vascular dementia?
Treat the underlying cause: diabetes mellitus, HTN, Atherosclerosis
44
what is the most common hereditary ataxia?
Friedrich/Autosomal Recessive Ataxia
45
What is the etiology of Friedrich ataxia?
autosomal recessive mutation...spinocerebellar
46
What is the initial presenting symptom in Fridriech ataxia?
difficulty walking (present in legs first and lose 2 pv) then arms and lose P/T much later
47
What is the chromosome involved in Friedrich ataxia?
chrom 9q13-21 GAA
48
What is the pathophysiology of Autosomal recessive ataxia?
friedriech: GAA triplet repeats causes gliosis and loss of mylinated axons in the Corticospinal tract, spinocerebellar tract (lose neruons in dorsal nucleus of clarke and dentate nucleus and purkinje cells) and dorsal column (lose neurons in DRG of lumbar and cervical enlargements)
49
what is the presentation of Autosomal recessive ataxia?
Difficulty walking, ataxia in both legs sensory and cerebellar ataxia signs Foot slap, wide gait and Rhomberg Slow speech DTRs gone Plantar reflex extensor with flexor spasm possible optic atrophy Lose 2 PV early and P/T late arm ataxia months or years after initial leg ataxia Structural changes: hammer toes and kyphoscoliosis
50
What are accompanying conditions for Friedrich Ataxia(autosomal recessive ataxia)?
cardiomegaly in over 50% of patients | diabetes mellitus in 10%
51
What is the etiology of Multiple systems atrophy?
Alpha synuclein (same protein found in Lewy bodies) is found in oligodendrocytes
52
What is the presentation of Multiple systems atrophy?
presents like atypical Parkinson's with cerebellar ataxia and with autonomic dysfunction (various combos are possible)
53
What is the definition of MSA?
group of neurodegenerative diseases that can involve axons in the brain and autonomic nervous system
55
What do MSA-P , MSA-C and MSA-A present like?
MSA P- present like parkinson's MSA-C - present like olivoponto cerebellar atrophy MSA-A present with autonomic dysfunction (Shy-Drager syndrome)
56
What protein is found in neurofibrillary tangles intracelluarly in Alzheimers?
tau | A beta amyloid is found in senile neuritic plaques
57
Acute combined dementia presents how?
global diminishing of 2PV spastic weakness in all 4 extremities following a crash diet or unable to absorb vitB12
58
In a spinocerebellar ataxia what parts of the NS are most likely affected?
cerebellar cortex, spinal cord, peripheral nerve

Cns (12 decks)

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