Neurodegenerative Disorders Flashcards Preview

► Med - Pathology > Neurodegenerative Disorders > Flashcards

Flashcards in Neurodegenerative Disorders Deck (30):
1

Dementia

Gradual decline in cognitive function that includes memory deficits and behavioral changes

2

What are some possible causes of dementia?

- Stroke
- Infection
- Tumors
- Drugs
- Vitamin Deficiency

3

What are the diseases that primarily affect the cerebral cortex?

- Alzheimer's
- Lewy Body Disease
- Frontotemporal Dementias

4

What is the most common cause of senile dementia in people over 65?

Alzheimer's Disease

5

What genes can lead to the early onset of Alzheimer's disease?

- APP (amyloid precursor protein) on Ch21
- Presenilin 1 and 2

6

What gene is related to late onset Alzheimer's?

ApoE4 on Ch19

7

What gene is protective against Alzheimer's?

ApoE2

8

What will be seen grossly in Alzheimer's?

Cerebral atrophy

9

Hydrocephalus Ex Vacuo

Dilatation of the vacuoles in response to cerebral atrophy

10

What are the hallmarks of Alzheimer's?

- Neurofibrillary tangles of hyperphosphorylated tau protein
- Amyloid plaques

11

Amyloid Angiopathy

Amyloid deposition in the walls of arterial vessels in subarachnoid space - seen in most cases of Alzheimer's

12

What are the symptoms of Lewy Body Disease?

Initial dementia with visual hallucination with parkinsonian symptoms

13

What is the hallmark of Lewy Body disease?

Lewy Bodies of alpha-synuclein

14

What are the majority of frontotemproal lobar degenerative diseases linked to?

Mutations in tau protein on Ch17

15

Pick's Disease

A type of frontotemporal dementia that is rare

16

What is the classic presentation of Pick's Disease?

Patients present with aphasia instead of dementia, but will develop memory loss with time

17

What is generally spared in frontotemporal degeneration?

Superior Temporal Lobe

18

What are Pick bodies?

Globose neuronal inclusions of tau protein that are deposited in the hippocampus and the cortex

19

What are some diseases affecting the basal ganglia?

- Parkinson's
- Huntington's

20

Multiple System Atrophy

Symptoms consist of variable combination of parkinsonism, cerebellar ataxia, and autonomic dysfunction – depend on the neuronal system involved

21

Werdnig-Hoffmann Disease (SMA Type I)

Autosomal recessive and causes floppy baby syndrome with the degeneration of LMNs

22

Kugelberg-Walander Disease (SMA Type II)

Similar to Werdnig-Hoffmann but presents at 3 months of age or later and is more mild

23

SMA Type III

Very rare and similar to Werdnig-Hoffmann but less severe with onset in infancy to early adolescence

24

What is the cause of all 3 types of spinal muscular atrophy (SMA)?

Mutations in genes SMN1/2 on Ch5

25

Friedrich's Ataxia

• Autosomal recessive  
• Trinucleotide repeat GAA (glutamic acid) in
frataxin gene on Ch9

26

What is the most common hereditary ataxia?

Friedreich's Ataxia

27

Wernicke's Encephalopathy

• oculomotor abnormalities (ophthalmoplegia, nystagmus)
• cerebellar dysfunction
• altered mental status

28

Korsakoff Psychosis

– Loss of recent memory compensated by confabulation

29

Subacute Combined Degeneration

Myelin loss in posterior and lateral columns

30

What is the cause of central pontine myelinolysis?

‐Rapid correction of electrolyte
imbalances, especially chronic
hyponatremia

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