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House AMP > Neurodegenerative Diseases of the CNS > Flashcards

Neurodegenerative Diseases of the CNS Flashcards

1
Q

Neurodegenerative disorders are (curable or incurable?) and debilitating conditions which result in ______________ of nerve cells

A

incurable

progressive degeneration

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2
Q

Neurodegenerative disorders

Many are associated with the _____________ which serve as histologic hallmarks of certain disorders

A

accumulation of abnormal proteins

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3
Q

Neurodegenerative disorders

The clinical manifestation is usually dictated by the area of the brain affected:
○Cortical neurons - ________
○Basal ganglia - _______
○Cerebellum - ________________

A

○Cortical neurons - dementia
○Basal ganglia - movement
○Cerebellum - ataxia

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4
Q

Neurodegenerative disorders

Protein Diseases with inclusions

Aβ

TPD-43

α-synuclein

Polyglutamine aggregates

A

Alzheimer disease

Frontotemporal lobar degeneration; Amyotrophic lateral sclerosis

Parkinsons disease

Huntington disease

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5
Q

Neurodegenerative disorders

Protein Diseases with inclusions

Tau

A

Alzheimer disease

Frontotemporal lobar degeneration

Parkinsons disease (LRRK2 mutation)

Progressive supranuclear palsy

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6
Q

Neurodegenerative disorders

Protein Diseases with inclusions

______ Alzheimer disease

_____ Frontotemporal lobar degeneration

______ Amyotrophic lateral sclerosis

_______ Parkinsons disease

_______ Huntington disease

A

Aβ

TPD-43

α-synuclein

Polyglutamine aggregates

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7
Q

Neurodegenerative diseases

_________ disease
_________ disease
_____________________
___________ disease

A

Parkinson disease
Alzheimer disease
Amyotrophic lateral sclerosis
Huntington’s disease

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8
Q

Most common cause of dementia (senility) is ????

A

Alzheimer disease

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9
Q

Alzheimer disease

Dementia refers to the effect of a group of brain diseases that result in (short or long?) term and (abrupt or gradual?) decrease in the ability to ______ and _______

A

Long; gradual

think and remember

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10
Q

Alzheimer disease

Named after ________ who first described it on 1906b

A

Alois Alzheimer

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11
Q

Alzheimer disease

The incidence when considered by age group:

○___% (65 to 74 years)
○____% (75 to 84 years)
○____% (85 years and above)

A

3

19

47

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12
Q

Alzheimer disease

Most cases are _______, about 5 - 10% are ______ (may experience early onset of symptoms)

A

sporadic

familial

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13
Q

Alzheimer disease : Pathogenesis

Accumulation of a peptide ( _______ or ________) in the brain

This peptide is derived from the cleavage of _________________________

A

Aβ protein

β amyloid

Amyloid Precursor Protein(APP).

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14
Q

Alzheimer disease: Pathogenesis

The APP can be processed in two ways:
○Cleavage by ———- and _______ in a process that ________

○Cleavage by _________ cleaving enzyme and ________ to form Aβ

A

𝛼 -secretase and γ -secretase

prevents the formation of Aβ .

β site APP

β site APP

γ - secretase

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15
Q

Alzheimer disease

Sporadic: Generation and accumulation of Aβ is said to occur (slowly or rapidly?) with ___________

A

Slowly

advancing age.

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16
Q

Alzheimer disease

Risk Factors:
Increasing age

Genetic: ______ of apolipoprotein E
The e4 allele is (more or less?) effective.

A

e4 allele

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17
Q

Apolipoprotein helps breakdown Beta amyloid

T/F

A

T

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18
Q

Alzheimer’s disease

Familial

Mutations in _____,_______

A

APP

Gamma secretase

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19
Q

Alzheimer’s disease

Familial: Mutations in ____ or in components of γ - secretase ( ___________ ) is seen in early onset familial cases with increased accumulation of Aβ

A

APP

presenilin(PS) 1 or 2

20
Q

Alzheimer’s Disease

AD occurs in almost all cases of Trisomy ——- ( _______ ) who survive beyond ____ years.

This is because the gene encoding_____ is located on chromosome (gene dosage effects).

A

21

Down’s syndrome

45

APP; 21

21
Q

Accumulation of Aβ :

○Alter ________

○Toxic to ——————- endings.

○Larger deposits (______) can also lead to neuronal death, ellicit a local inflammatory response, alteration of region-region communication through their mechanical effects on axons and dendrites

A

neuronal transmission

neurons and synaptic

plaques

22
Q

The presence of Aβ also leads the neurons to hyperphosphorylate the ————— protein called ——-.

○ With hyperphosphorylation, ____ redistributes from ———- into _______ and _______ and aggregates into ______.

○ This process results in neuronal dysfunction and cell death The anatomic distribution of the _____ and ________ account for the clinical manifestations seen and occur well ahead of the onset of clinical signs and symptoms.

A

microtubule binding; tau

tau; inside the axon

dendrites and cell bodies

tangles

plaques and tangles

23
Q

Alzheimer’s disease

The presence of Aβ also leads the neurons to _________ the _______ protein called tau.

○ With ______________, tau redistributes from ___________ into _________ and aggregates into ______ .

○ This process results in neuronal dysfunction and cell death

A

hyperphosphorylate

microtubule binding; tau.

hyperphosphorylation

inside the axon into dendrites and cell bodies

tangles

24
Q

Alzheimer’s disease

The __________________________ account for the clinical manifestations seen and occur _________ of the onset of clinical signs and symptoms.

A

anatomic distribution of the plaques and tangles

well ahead

25
Q

Gross findings of Alzheimer’s disease

○Cortical ______ with (narrowing or widening?) of the sulci more pronounced in the ______________________ lobes

○Compensatory _______ and ventricular ————- (Hydrocephalus- _______)

A

atrophy; Widening

frontal, parietal and temporal

increase in CSF

enlargement; ex-vacuo

26
Q

Microscopic findings of Alzheimer’s disease

○______ (extracellular)
○_____________ (intracellular)
○Cerebral ______________

A

Plaques

Neurofibrillary tangles

amyloid angiopathy

27
Q

these changes seen in a demented patient may also be seen in the brain of elderly nondemented individuals.

T/F

A

T

Note that these changes may also be seen in the brain of elderly nondemented individuals.

Therefore clinical signs and symptoms must be part of the diagnostic work-up

28
Q

Alzheimer’s disease

Diagnosis

_____ deposition in the brain can be detected through _____ methods that rely on ____-labeled _________compounds.

Can also be used in ________ patients at ____ risk for developing AD

A

Aβ; imaging

18F; amyloid binding

asymptomatic; high

29
Q

Alzheimer’s disease

Diagnosis

CSF analysis for the presence of _________________

A

increased phosphorylated tau

30
Q

Alzheimer’s disease

Management

Current approaches focus on helping people maintain ________, manage _______ symptoms and ______ the symptoms of the disease
Assisted living facilities

A

mental function; behavioural

slow down

31
Q

Parkinson’s disease

Prevalence ranges from 42/100,000 in the ____ decade of life to >1900/100,000 among those who are ____ years and older

A

4th

80

32
Q

Parkinson’s disease

More in males or females?

A

Males

M>F

33
Q

Parkinson’s disease

Named after __________ who published the first detailed description in 1817

A

James Parkinson

34
Q

Parkinson’s disease

Mostly _______ however genetic analysis has identified certain causal mutations e.g. ________ mutations cause __________ Parkinson’s disease.

A

sporadic

α-synuclein

Autosomal dominant

35
Q

Parkinson’s disease

The diagnostic feature of the disease is an inclusion containing ________ (______ body)

A

α-synuclein

Lewy

36
Q

α-synuclein is a widely expressed _______ protein that is involved in __________ and other cellular processes

A

neuronal

synaptic transmission

37
Q

Mechanism of Parkinson’s disease is generally unknown

T/F

A

T

38
Q

Parkinson’s disease: Pathogenesis

However the presence of ______ in Lewy bodies has suggested that _________ of the protein may be culpable.

This is supported by the identification of two other genetic loci for Parkinson’s disease:
○Genes encoding ______
○ genes encoding ————

A

α-synuclein

defective degradation

parkin

UCHL-1

39
Q

Parkinson’s disease

parkin (an _______________________)

UCHL-1 (an enzyme involved in the recovery of ________ from proteins targeted to the _______________)

A

E3 ubiquitin ligase

ubiquitin

proteasome

40
Q

The α-synuclein which accumulates in the neuron is _______ resulting in the _______ of the cell.

Up to 70% of __________ secreting neurons in the ________ are affected resulting in _____ depletion

A

cytotoxic; death

dopamine

substantia nigra

dopamine

41
Q

Symptoms of Parkinsonism:

_______kinesia

________ forms.

___mimia

_____tremor

Gait _______

A

Brady

Rigidity

Hypo

Pill rolling

Shuffling

42
Q

Symptoms of Parkinsonism:

Bradykinesia ( ____ movement)

Rigidity: “________” & “__________” forms.

Hypomimia (_________ face)

Pill rolling tremor ( _____ and _______ finger)

Gait – shuffling

A

slow

Lead pipe; Cog-wheel

expressionless

thumb and index

43
Q

Parkinsonism” can be induced by:

○Drugs that affect these neurons _
particularly __________ and toxins
○________________ parkinsonism ( __________ pandemic)
○ __________ hydrocephalus
○Head trauma, stroke

A

dopamine antagonists

Post-encephalitic; post-influenza

Normal pressure

44
Q

Other symptoms seen in Parkinson disease

Autonomic disturbance e.g. ________,______

__________ impairment

A

hypersalivation, incontinence

Cognitive

45
Q

Morphology (Autopsy) of Parkinson’s disease

Gross: ______ of __________

A

Pallor of substantia nigra

46
Q

Morphology (Autopsy) of Parkinson’s disease

Microscopic
Loss of the ______ neurons in these regions

_____ bodies (intracytoplasmic, ______ inclusions). These filaments are composed of ________

A

pigmented

Lewy; eosinophilic

eosinophilic

α-synuclein

47
Q

Diagnosis of Parkinson’s disease

Clinical response to L-DOPA treatment.
○L-DOPA ________________ of the disease
○Over time, _______________

A

does not halt the progression

L-DOPA becomes less effective

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