Brainscape's Knowledge GenomeTM

Browse over 1 million classes created by top students, professors, publishers, and experts.


See full index

17 CNS Pathology > 17.7 Dementia and degenerative disorders > Flashcards

17.7 Dementia and degenerative disorders Flashcards

1
Q

Alzheimer’s disease

-how to dx

A
  • dx with clinical and pathological correlation
    1. clinical–diagnosis of exclusion
    2. pathology–requires autopsy
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
1
Q

Pick disease

-characterized by what, histologically

A

Pick bodies–round aggregates of tau protein in cortex neurons (as opposed to NFTs in alzheimers)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Down’s syndrome

-assoc with what CNS disorder and why

A

Alzheimer’s

-the gene for APP (amyloid precursor protein) is on c21, so 3 c21s results in increased production

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
2
Q

Lewy bodies

A

-round, eosinophilic inclusions of alpha-synuclein in affected neurons. Characteristic of parkinson disease.

Also present in Lewy body dementia. Presence of cortical Lewy bodies

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
3
Q

Alzheimer’s disease

-morphologic features include: (4)

A
  1. cerebral atrophy
  2. neuritic plaques–comprised of AB amyloide with entangled neuritic processes
  3. neurofibrillary tangles–intracellular aggregates of fibers composed of hyperphosphorylated Tau protein
  4. loss of cholinergic neurons in the nucleus basalis of Meynert
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
4
Q

Parkinson’s disease

-etiology

A

-unknown. rare cases related to MPTP exposure (comtaminant found in illicit drugs)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
5
Q

Alzheimer’s disease

-important to recognize absence of what symptom early in the disease?

A

Absence of focal neurologic defects early in disease.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
6
Q

Creutzfeldt Jakob disease (CJD)

-prognosis

A

-Results in death, usu

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Huntington’s disease

-what brain pathways affected and how?

A
  • Loss of GABAergic neurons in the caudate nucleus of basal ganglia
  • these neurons inhibit the cortex movements, so loss of inhibition means uncontrolled movements.
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
7
Q

Creutzfeldt Jakob disease (CJD)

-characteristic diagnostic test finding

A

-EEG: periodic sharp waves

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Alzheimer’s disease

  • divided into what 2 types
  • what are their risk factors (2 each)
A
  1. sporadic (95%)
    - age
    - E4 APOE allele higher risk, E2 is lower risk
  2. Early-onset
    - Presenilin gene mutations
    - Down’s syndrome (b/c gene for precursor of A beta amyloid is on c21)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

Alzheimer’s disease

-mechanism

A

APP —> A beta amyloid (cannot be degraded)

–> alpha (can be recycled)

APP (amyloid precursor protein) is a receptor on neurons. Normally alpha cleavage results in degradable material, but beta cleavage results in accumulation of A beta amyloid.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
8
Q

You see a pt with new clinical symptoms consistent with early Parkinson’s. She also has new dementia symptoms.

Think what?

A

Dementia is a late (years later) complication of Parkinsons.

early-onset dementia with parkinsonian symptoms suggests Lewy body dementia.

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
9
Q

Creutzfeldt Jakob disease (CJD)

-mech, etiology

A
  • most common spongiform encephalopathy.
  • usu sporadic, but can be transmitted through exposure to prion-infected tissue (eg human growth hormone, or corneal transplant)–infected with PrPsc (beta pleated conformation)
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Creutzfeldt Jakob disease (CJD)

-clinical symptoms (3)

A
  • rapidly progressing dementia (weeks to months), and assoc with:
  • ataxia (cerebellum)
  • startle myoclonus–involuntary muscle contractions to minimal stimuli
How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Parkinson’s disease

-clinical symptoms (4)

A

“TRAP”
Tremor–pill rolling, disappears with movement

Rigidity

Akinesia/Bradykinesia–slowing of movement

Postural instability, shuffling gait

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
12
Q

Tau protein

  • function
  • what diseases does it play a role (2)
A

microtubule associated protein important in the arrangement of cytoskeletal microtubules

  1. Alzheimer’s–neurofibrillary tangles composed of hyperphosphorylated Tau
  2. Pick disease–round aggregates of Tau protein (Pick bodies)
13
Q

Dementia

  • most common cause
  • second most common
A
  1. alzheimer’s
  2. vascular dementia (from global cerebral ischemia)
14
Q

Parkinson disease

-histology and gross appearance

A

gross: loss of pigmented neurons in substantia nigra
histology: Lewy bodies–round, eosinophilic inclusions of alpha-synuclein. In affected neurons

15
Q

Spongiform encephalopathy

-how can someone get this disease (3)

A
  1. sporadic
  2. inherited
  3. transmitted
16
Q

Normal pressure hydrocephalus

-clinical presentation (3)

A

-increased CSF leads to dilated ventricles, which stretch the corona radiata, causes symptoms.

“Wet, wobbly, and wacky”

  1. urinary incontinence
  2. gait instability
  3. dementia
18
Q

Pick disease

  • disease affects what?
  • clinical presentation
A
  • degenerative disease of:
    1. frontal cortex–behavioral symptoms
    2. temporal cortex–language symptoms

eventually progresses to dementia.

“Picks” the frontal and temporal, not parietal and occipital.

19
Q

Mad cow disease

A

“Variant Creutzfeld Jakob disease”

-related to exposure to bovine spongiform encephalopathy

21
Q

Alzheimer’s disease

-neurofibrillary tangles

A

-intracellular aggregates of fibers, composed of hyperphosphorylated Tau protein

22
Q

Creutzfeldt Jakob disease (CJD)

-gross appeaerance

A

-damage to neurons and glial cells results in intracellular vacuoles (spongy degeneration)

23
Q

Lumbar puncture improves symptoms of what?

A

Normal pressure hydrocephalus

24
Q

startle myoclonus

  • what is it
  • assoc with what disease (pathoma)
A
  • involuntary muscle contractions with minimal stimuli
  • assoc with Creutzfeld Jakob disease (along with rapidly progressing dementia and ataxia)
25
Q

Huntington’s disease

  • genetic inheritance pattern
  • what chromosome, describe genetics
A
  • auto dom
  • expanded trinucleotide CAG repeats in huntingtin gene on c4.

Anticipation–expansion of repeats occurs through spermatogenesis. Offspring experience Hungtinton’s earlier in life.

26
Q

Vascular dementia

-cause

A

result of moderate GCI–global cerebral ischemia

-moderate GCI leads to multifocal infarct/injury due to HTN, atherosclerosi, or vasculitis

27
Q

Alzheimer’s disease

  • clinical presentation over time (how do symptoms progress)
  • common cause of death?
A
  • Slow-onset memory loss, starts with short term and then long term memory loss with progressive disorientation
  • loss of motor skills, language
  • changes in bx and personality
  • mute and bedridden
  • infection is common cause of death
28
Q

Familial fatal insomnia

A
  • inherited form of prion disease
  • charaterized by severe insomnia, exaggerated startle response
30
Q

Dementia, Degenerative disorders

-general principles

A

(see picture)

Typically due to loss of gray matter neurons from accumulation of protein that damages neurons

  • degeneration of gray matter in cortex leads to dementia
  • gray matter in brainstem and basal ganglia leads to movement disorders
31
Q

Normal pressure hydrocephalus

-tx

A
  • Must reduce CSF to reduce pressure. Use ventriculoperitoneal shunting of CSF.
    (note: LP decreases symptoms )
33
Q

APOE

A

Apolipoprotein E

  • This is associated with Alzheimer’s disease, sporadic type
  • APOE E4 allele increases risk
  • APOE E2 decreases risk
34
Q

Spongiform encephalopathy

-describe mech/etiology

A
  • degnerative disease due to prion protein
  • PrP usually in alpha-helical formation (PrPc)
  • however, disease occurs with beta-pleated formation (PrPsc)

PrPsc protein is not degradable and converts normal PrP to PrPsc, causing vicious cycle.

36
Q

Presenilin

A

gene assoc with Alzheimer’s disease, early-onset type.

-mutations in presenilin increase risk

37
Q

Huntington’s disease

  • clinical presentation (early vs later symptoms)
  • average age of 1st presentation?
  • common cause of death
A
  • early: chorea and athetosis (slow, involuntary snake like movements)
  • Later: progresses to dementia and depression
  • 40 yo is average age

suicide is common cause of death

38
Q

Alzheimer’s disease

  • neuritic plaques–what are they
  • where else can amyloid deposit?
A
  • Plaques with extracellular core comprised of AB amyloid with entangled neuritic processes
  • Amyloid may deposit around vessels, increasing risk of hemorrhage (“Cerebral Amyloid Angiopathy”)
39
Q

Parkinson’s disease

-mechanism and neural pathways to know

A

(see picture)
Basal ganglia striatum modulates cortex movements through:

  1. stimulatory signals (with D1 receptors)
  2. nhibitory receptors (with D2 receptors)

Substantia nigra uses DA to initiate movement–it increases stimulation and decreases inhibition of cortex movement. (nigrostriatal pathway)

Degenerative of dopaminergic neurons of substantia nigra results in inability to initiate movements

40
Q

Normal pressure hydrocephalus

-mech, etiology

A
  • caused by a decrease in arachnoid granulations, which move CSF into venous space, causing backup of CSF into ventricles
  • expanded ventricles push on corona radiata, resulting in symptoms (wet, wobbly, wacky)
41
Q

Parkinson disease

-does it cause dementia?

A

Yes, dementia is a common feature of late disease, years later

42
Q

Lewy body dementia

  • clinical presentation
  • how does this compare with Parkinson’s?
A

-dementia, hallucinations, parkinsonian features.

Early-onset dementia is suggestive of Lewy body dementia and not Parkinson’s.

17 CNS Pathology (8 decks)

Brainscape helps you reach your goals faster, through stronger study habits.
© 2024 Bold Learning Solutions. Terms and Conditions