lecture 10 - - Neurodegnerative disease, Vascular Dementia, and Prion Disease Flashcards Preview

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Flashcards in lecture 10 - - Neurodegnerative disease, Vascular Dementia, and Prion Disease Deck (19):
1

Prion Disease --
how common?
Three forms?
Which is the most common form?
Biochemistry of the disease

1-2 cases per Million per years

Three forms: Familial, Sporadic, transmitted

Most common: Sporadic

Biochem: PRPC protein --> PRPSC which forms abnormal beta sheets resistant to proteases

2

Two human forms of prion disease and associated pathology?

Cruetzfeldt Jakob Disease --


Variant Form CJD

3

CJD
pathology?
Median Survival?
Manifestations?


Variant CJD -- how is it acquired ?

CJD:

Spongiform Degeneration of the gray matter with vaculoization --

Rapidly progressive (median survival 3.5 months) of dementia and startle myoclonus


Variant CJD -- thought be acquired through contaminated beef

4

What are the synucleinopathies ?

PD
LBD
Multiple System Atrophy

5

What are the Tauopathies ?

what other disease?

Fronto-Temporal Dementia (Pick's Disease, FTDP-17)

PSP -- Progressive Supranuclear Palsy

Corticobasal Degeneration


(ALZ -- tangles = tau; senile plaques = beta)

6

what is vascular dementia?

what are the three subtypes

Vascular dementia -- Brain injury from vascular insufficeincy/ischemia/HTN

Step wise progression rather than gradual decline

Subtypes:
Multiple Infarct Dementia
Biswanger's Disease
CADASIL --

7

CADASIL

stands for?
associated mutation?
Pathological changes?
how is a dx typically conducted?

Cerebral Autosomal Dominant Angiopathy w/ Subcortical Infarcts and Leukoencephalopathy

(CVD and white matter damage)

Molecular -- Notch 3 Mutation

Sclerotic Changes, which can also be present on the skin (therefore skin bx is taken for Dx)

8

Binswangers

white matter degeneration secondary to vascular disease (small artery sclerosis)

9

alz --
histopath findings
Imaging
pathogenesis
potential treatments

Tau Tangles; Beta Amyloid Senile Plaques
Imaging: Ventricular englargement with Sulcal Widening

Pathogensis: ApoE4 increased risk for development;
Mutated genes for Presnelin 1 and 2 -- involved in metabolism of Beta Amyloid
Plaques = A-Beta Fragement of the Beta Amyloid

Tx - Increased ACH
Cholinesterase Inhibitors --
NMDA Antagonist -- Memantine (neuroprotective)

10

PD --
Gross path
Micropath

What are parkinsonisms?

Possible treatment

Gross Path -- loss of pigment of the SN

Micropath -- Lewy Bodies in the SN
Loss of dopaminergic neurons

Parkinsonism -- Bradykinesia, Unilateral tremor, ridigity

Treatments: LevoDopa with dopamine metabolizing enzyme inhibitor (Carbidopa)

Other treatments -- MAOIs (block dopamine reuptake); COMT inhibitors; Dopamine Agonists

11

Lewy Body Dementia --

how does it differ from PD

Clinical onset for LBD: dementia first, then parkinsonisms

PD: Parkinsonism first

12

Multiple System Atrophy --
what 3 regions are damaged and how does the damage manifest

where does the synulcein accumulate?

SN -- dopamine loss = Parkisonisms

Olivopontocerebellar damage = ataxia

Preganglionic Neuron Autonomic Loss = Shy drager autonomic loss

Synuclein accumulates in oligodendrocytes (not neurons like PD)

13

Tauopathies:

Fronto temporal dementia (Picks and FTD-Parkinsonism Chr 17)

Progressive Supranuclear Palsy

Corticobasilar Degeneration

14

What are the Frontotemporal Dementias --

how do they manifest ?

Pick's and FTDP_Chr17

Language dysfunction, personality changes, memory loss , dementia

15

Pick's Disease --
Characterisitic pathology?

Tau positive Pick bodies; ballooned neurons

16

Progressive Supranuclear Palsy --

Characteristic symptoms?

Vertical gaze ophthalamoplegia + Parkinsonism

17

Corticobaslilar Denengeration
Characteristic Symptoms?

Alien Limb Phenomena + Parkinsonisms + Alien Limb

18

ALS --
damage to what?
Etiology?
muBUntation?
Characteristic Pathology?

UMN and LMN damage

Etiology -- idiopathic
Minority associated with mutation to SOD1

Characterisitc path -- Bunina bodies and TDP43 Inclusions

19

Is Chronic Truamatic Encephalopathy a tauopathy or synucleinopathy?

Tauopathy