Brainscape's Knowledge GenomeTM

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


See full index

Neuroscience 2 > Parkinson's Plus Disorders > Flashcards

Parkinson's Plus Disorders Flashcards

Define the term parkinsonism and list the CNS disorders which may present in this way Identify the key neuropathological features of progressive supranuclear palsy (PSP) Identify the key neuropathological features of multiple system atrophy (MSA) Identify the key neuropathological features of corticobasal degeneration (CBD) Research how Parkinson's plus disorders can be distinguished clinically while the patient is alive

1
Q

How many patients diagnosed with PD are found to have a different parkinsonian disorder post-mortem?

A

10-15%

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

Name the 2 PD subtypes

A

Tremor dominant and akinetic-rigid dominant

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

Which PD subtype has a better prognosis?

A

Tremor dominant

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

Give at least 5 non-PD causes of parkinsonism

A

Drug-induced (e.g. MPTP), multiple system atrophy, progressive supranuclear palsy, corticobasal degeneration, vascular pseudo-parkinsonism, frontotemporal neurodegeneration

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

Name the 3 disorders covered by the term multiple system atrophy

A

Olivopontocerebellar atrophy (OPCA), Shy-Drager syndrome, and striatonigral degeneration

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

Which disorder within multiple system atrophy most closely resembles PD?

A

Striatonigral degeneration

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

Which disorder within multiple system atrophy is largely autonomic?

A

Shy-Drager syndrome

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

Give the 2 classifications of multiple system atrophy

A

Predominant parkinsonism (MSA-P) and predominant cerebellar (MSA-C)

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

Give at least 3 major signs of multiple system atorphy

A

Tremor, squeaky hypophonia, dysrhythmia of rapid alternating movements, hypermetria

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
10
Q

Describe the macroscopic pathology caused by multiple system atrophy

A

Motor and premotor cortical atrophy, marked cerebellar atrophy, shrinkage of the middle cerebellar peduncle, pons, and inferior olivary nucleus, pallor of the substantia nigra and locus coeruleus

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
11
Q

Name the classic sign of multiple system atrophy seen on MRI

A

Hot cross bun sign

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

How is the microscopic pathology of multiple system atrophy different to that of PD?

A

It is both neuronal and glial, whereas PD pathology is just neuronal

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
13
Q

Describe the microscopic pathology seen in multiple system atrophy

A

Alpha-synuclein immunoreactive cytoplasmic and nuclear inclusions in glia and neurons, Papp-Lantos bodies, neutrophil threads

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
14
Q

In which cells are the cytoplasmic inclusions of multiple system atrophy most obvious?

A

Oligodendrocytes

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
15
Q

Define corticobasal degeneration (CBD)

A

A progressive neurodegenerative disorder causing rigidity, clumsiness, and stiffness and jerking of one limb (alien limb syndrome)

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
16
Q

Which limb is most commonly affected by alien limb syndrome in corticobasal degeneration?

A

Arm

How well did you know this?
1
Not at all
2
3
4
5
Perfectly
17
Q

Which protein is involved in CBD pathogenesis?

A

Tau

18
Q

Name the three brain areas primarily affected by corticobasal degeneration

A

Cerebral cortex (specifically frontal and parietal cortices), deep cerebellar nuclei, substantia nigra

19
Q

Describe the microscopic pathology of corticobasal degeneration

A

Glial and neuronal inclusions, prominent neutrophil threads, balloon neurons

20
Q

Which part of astrocytes is tau predominantly deposited in?

A

Proximal processes

21
Q

What is the most common form of atypical parkinsonism?

A

Progressive supranuclear palsy

22
Q

Does progressive supranuclear palsy respond to L-DOPA?

A

No

23
Q

Give at least 4 signs of progressive supranuclear palsy

A

Mild slowing of horizontal saccades, major slowing of vertical saccades, ophthalmoplegia, gradual uphaze and downgaze with no fast corrective saccades, bradykinesia worse on one side, dystonia, tremor

24
Q

Describe the macroscopic pathology of progressive supranuclear palsy

A

Atrophy of the basal ganglia, subthalamus, and brainstem

25
Q

Describe the microscopic pathology of progressive supranuclear palsy

A

Neuronal loss and gliosis, neuronal and glial tau-positive inclusions (including ‘tufted’ astrocytes and ‘coiled bodies’ in oligodendrocytes)

26
Q

Describe the difference between microscopic coiled bodies in MSA and PSP

A

In MSA they have a coiled profile, whereas in PSP they have a circular profile

27
Q

Which chromosome codes the gene for tau?

A

17q21

28
Q

How many exons code for tau?

A

16

29
Q

How many isoforms of tau can be produced?

A

6

30
Q

What makes tau 3 repeat or 4 repeat?

A

The number of microtubule binding domains

31
Q

What is the foetal form of tau?

A

3R/0N

32
Q

What is the function of tau?

A

It is a microtubule associated protein (MAP) which binds to and stabilises microtubules and promotes their polymerisation

33
Q

Which type of tau is more efficient - 3R or 4R?

A

4R

34
Q

Describe the phenotype of the tau knockout mouse

A

No overt phenotype, as other microtubule associated proteins compensate for its loss

35
Q

How many potential phosphorylation sites are there on tau, and where are they?

A

79 - with serine or threonine residues, clustered around microtubule binding sites

36
Q

How does over-phosphorylation impair the function of tau?

A

As phosphorylation sites are clustered around microtubule binding sites, it reduces microtubule binding, hence impairing function

37
Q

Name 2 kinases which phosphorylate tau

A

GSK-3beta and CDK5

38
Q

Which types of tau are involved in AD?

A

All of them

39
Q

Which types of tau are involved in PSP and CBD?

A

4R taus

40
Q

Which types of tau are involved in Pick’s disease?

A

3R taus

41
Q

Can patients have both PD and a tauopathy?

A

Yes

Neuroscience 2 (30 decks)

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