Neuro 22-24: Neurodegenerative Disorders, Demyelinating Disorders

Front 1

How is the diagnosis of Alzheimer's disease made when alive?

Is it always correct?

Back 1

Clinically.

No not always correct.

Front 2

What is pseudodementia?

Back 2

dementia caused by depression and hypomania.

Front 3

What is the most common primary dementia? second most common?

Back 3

Alzheimer's disease: most common

Dementia with Lewy Bodies (DLB) - second most common

Front 4

What is sundowning?

In what disorder do you observed this?

Back 4

Sundowning is episodes of delirium at night.

It is observed in Alzheimer's disease.

Front 5

List 3 most prediposing factors in development of Alzheimer's disease.

Back 5

Age, genetic influences, apolipoprotein E status.

Front 6

Is myoclonus late or early neurologic sign of Alzheimer's?

Back 6

Late

Front 7

What is snout reflex? In what dementia disorder do you observe this?

Is this an early or late sign?

Back 7

Snout reflex - frontal release sign

observed in Alzheimer's

Early neurologic sign

Front 8

What 2 regions are atrophied in Pick's disease?

Back 8

Frontal and temporal lobes

Front 9

What abnormal protein is observed in lewy bodies?

Back 9

alpha synuclein

Front 10

Name 3 associated clinical features/signs of Lewy body dementia.

Back 10

1. parkinsonism
2. fluctuating level of consciousness
3. visual hallucinations

Front 11

What 2 neurodegenerative disorders have lewy bodies?

Back 11

Dementia with lewy bodies

Parkinson's disease

Front 12

What is Percheron's artery?

What happens with occlusion of this artery?

Back 12

one artery supplying both thalami

vascular dementia

Front 13

List the features of NPH(normal pressure hydrocephalus) triad.

Back 13

gait ataxia, dementia, incontinence

Front 14

What is the cause of CJD (Creuztfeldt Jacob disease)?

List 2 features of CJD.

Back 14

Prion

spongiform encephalopathy
rapidly progressive dementia

Front 15

How do you diagnose CJD (Crueztfeldt Jacob disease)?
List 3

Back 15

a) Radiology: bright cortical signal in clinically affected areas, thalami, or basal ganglia
b) CSF 14-3-3 protein – not specific
c) EEG: periodic sharp waves

Front 16

List neurodegenerative disorders that are associated with following protein abnormalities.

1. Taupathies
2. alpha-synuclein
3. Polyglutamate (CAG repeat)

Back 16

1. Alzheimer's, Pick's diseaes (FTD), and progressive supranuclear palsy (PSP)

2. Dementia with Lewy bodies (DLB) and Parkinson's disease

3. Huntington's and spinocerebellar ataxia

Front 17

What 2 enzymes cut APP to generate Abeta-amyloid proteins?

What enzyme cut APP to make normal soluble APP?

Back 17

producing abnormal Abeta protein - beta and gamma secretase

normal - alpha secretase

Front 18

Name 4 genes involved in Alzheimer's disease and list the chromosome numbers for each gene.

Back 18

1. APP, chrom 21
2. ApoE4, chrom 19
3, Presenilin 1 (PS-1), chrom 14
4. Presenilin 2 (PS-2) , chrom 1

Front 19

What are the early clinical signs of FTD? late?

Back 19

early - personality/behavioral disturbance and language problems

Late - memory loss

Front 20

What part of the temporal lobe is saved in FTD?

Back 20

posterior 2/3 of the superior gyrus in the temporal lobe

Front 21

What 2 areas lose their pigment in Parkinson's pts?

Back 21

substantia nigra

Locus ceruleus

Front 22

Name the disease with combined symptoms of parkinsonism and vertical gaze palsy.

Back 22

progressive supranuclear palsy (PSP)

Front 23

What is the genetic problem with Huntington's disease? chrom?

What genetic quality does this mutation show down the generations?

Back 23

large CAG repeats on chrom 4.

shows anticipation

Front 24

What gene encodes normal prion protein?

What is the abnormal prion called?

Back 24

normal gene - PrPc

Abnormal - PrPsc

Front 25

this is a biopsy of Alzheimer's brain.

What protein is in the middle?

Back 25

this is a neuritic plaque.

It has Abeta amyloid protein core.

Front 26

What protein is in the above structure pointed by the arrow.

Back 26

It's a pick body with TAU protien

Front 27

This is a section of the midbrain.

What 3 diseases do you suspect?

Back 27

Parkinson's

Dementia with lewy bodies

Progressive supranuclear palsy

Front 28

What protein would be found in the structures indicated by the arrows?

Back 28

Alpha synuclein

These are lewy bodies

Front 29

This is a brain of a pt who showed symptoms of chorea and demetia.

What disease do you suspect?

What is the enlarged cavity called?

Back 29

Huntington's disease (atrophy of caudate and putamen)

Ex vacuo (enlarged ventricles due to atrophied caudate)

Front 30

How is the demyelinization process different b/t MS and leukodystrophy?

Back 30

Leukodystrophy is a dysmyelinating disease in which a metabolic inborn error causes little or no formation of meylin.

MS is a demyelinating disease in which a normally formed myelin is destroyed by a disease process.

Front 31

ID the type of leukodystrophy with the following accumulated proteins:

1. Sulphatide
2. Psychosine
3. Very long chains of fatty acid

Back 31

1. Metachromic leukodystrophy
2. Krabbe (globoid) leukodystrophy
3. adrenoleukodystrophy

Front 32

What is the most common leukodystrophy?

Back 32

metachromic leukodystrophy.

Front 33

Which leukodystorphy cause inflammation?

Back 33

Adrenoleukodystrophy

Front 34

What type of demyelinating or dysmyelinating disorder is this?

presence of rosenthal fibers and diffuse demyelinazation

Back 34

Alexander's disease

Front 35

Which functional fibers are affected in leukodystrophy? Is it bilateral or unilateral?

Back 35

both sensory and motor.

bilateral

Front 36

Describe the pathogenesis of demyelination process in MS.

Back 36

Cellular immune response activates CD4+ Th1 cells against myelin self-antigen. T cells secrete INF-gamma, which activates macrophages.

Activated macrophages and their toxic products cause demyelination.

Front 37

Where are the lesions usually found in MS brain?

Back 37

Optic nerve, paraventricular white matter and juxtacortical white matter.

Front 38

What are shadow plaques and inactive plaques in MS.

Back 38

Shadow plaques - show thin myelin sheath due to either remyelination or incomplete loss of myelin; somewhere b/t normal and inactive plaque

Inactive plaques - there is total myelin loss and loss of oligodendrocytes. Completely healed.

Front 39

Name the disorder:

Positive antibody for aquaporin-4 channels.
vision loss and paraplegia.

Back 39

Neuromyelitis optica (NMO) or Devic disease

Front 40

Describe tumefactive MS.

Back 40

Single large lesion in white matter with edema.

Biopsy reveals acute myelin loss, axonal sparing, lymphocyte, foamy macrophages, and gliosis

Front 41

What are predisposing factors for ADEM (acute disseminated encephalomyelitis)? (2)

Back 41

post viral infection or post vaccination

Front 42

Which subtype of MS is most common in early stage of MS? late?

Back 42

early - relapsing-remitting

Late - secondary progressive

Front 43

On expanded disability status scale (EDDS), what does 3.5 indicate? 7?

Back 43

3.5 - highest score at which walking assistance is not needed

7 - can't walk even with assistance.

Front 44

Waht is another name for first MS attack?

Back 44

clinically isolated syndrome.

Front 45

The arrows indicate accumulation of what protein?

What are they? What disease?

Back 45

psychosine

The arrows indicate globoid cells

Krabbe leukodystrophy

Front 46

What do the arrows indicate?

What disease do you suspect?

Back 46

inactive plaques paraventricularly located

MS

Front 47

the above picture is a cross section of pons, stained against myelin.

This was obtained from a pt with Hx of chronic alcoholism.

What disorder do you suspect?

Will the progression of disease slow or rapid?

What is the most likely and direct cause of this disorder?

Back 47

Central pontine myeliolysis

Rapid progression

due to rapid correction of hyponatremia to correct electrolyte imbalance.