Neurodegenerative disorders

Question 1

What are the key points of the Original Peterson criteria for mild cognitive impairment (MCI)?

Answer 1

Memory complaint
With preserved general cognitive function
Intact activities of daily living

New criteria allows for a NON-amnestic variant

Question 2

What is possible treatment for MCI?

Answer 2

Donepezil, an acetylcholinesterase inhibitor

Question 3

What are the key points in Alzheimer’s dz dx by DSM 4

Answer 3

1 Memory impairment with aphasia/ataxia/agnosia/disturbed executive function
2 Cognitive deficit
3 Gradual cognitive decline
4 NOT dx in the presence of delirium

Question 4

What is the first part of the cerebrum to be involved in AD? The last?

Answer 4

First: Entorhinal complex and hippocampus
Last: Occipital

Question 5

Differentiate Amyloid plaques and Neurofibrillary tangles

Answer 5

AP: Made up of beta amyloid fragments
NT: Hyperphosphorylated tau proteins; Intracellular deposits starting in neurons in the entorhinal cortex and hippocampus

Question 6

Differentiate the genetics of early onset and late onset AD

Answer 6

late onset: APOE sigma 4

early onset: Familial AD on chromosome 21 (NB: Down’s syndrome)

Question 7

What drugs may be used for AD?

Answer 7

Ach-esterase inhibitors such as donepezil, rivastigmine and galantimine
Glutamate inhibitor at the NMDA receptor ONLY AS ADD ON TREATMENT FOR MOD TO SEVERE AD

Question 8

What are the core features of Lewy body dementia?

Answer 8

Fluctuating cognition
Recurrent visual hallucination
Spontaneous parkinsonism

Two of these = probable DLB (dementia with lewy bodies)

Question 9

How to differentiate diffuse lewy body dementia and parkinson’s dementia

Answer 9

DLB dementia occurring with parkinsonism or within 1 year of onset of parkinsonism

PD dementia occurs much later in a person dx with PD

Question 10

What is the primary protein that makes up the Lewy body?

Answer 10

alpha synuclein

Question 11

Rank the variants of frontotemporal dementia according to rate of progression

Answer 11

Fast– Frontotemporal dementia > Nonfluent apahasia > Semantic– Slow

Question 12

What are microscopic findings of FTD?

Answer 12

Pick bodies: Tau protein deposits within ballooned neurons (PICK CELLS)

There is LOSS OF LARGE PYRAMIDAL cells in the cortical layer III and loss of small pyramidal and non pyramidal cells of layer 2

Question 13

What motor neuron dz is FTD associated with?

Answer 13

ALS

Question 14

What diagnosis does high levels of 1-4-3-3 protein support?

Answer 14

Creutzfeldt Jakob dz

Question 15

The Hachinski criteria is utilized to diagnose?

Answer 15

Vascular dementia

Score of more than or equal to 7 suggests multi-infarct dementia

Question 16

What is the Binswager syndrome?

Answer 16

Multiple small infarcts in the deep white matter causing dementia– chronic white matter hypoperfusion

Question 17

What is the routine lab panel to r/o reversible causes of dementia?

Answer 17

BIG THREE
1 Vit B12
2 Thyroid fxn test
3 Depression dx criteria

CT/ MRI scan
Heavy metal screen
Drug screen
RPR for syphillis

Question 18

What are the sxs of vascular dementia based on the arteries affected?
1 Middle cerebral artery
2 Anterior cerebral artery
3 Posterior cerebral artery

Answer 18

1 Middle cerebral artery: Aphasia and neglect
2 Anterior cerebral artery: Apathy, abulia, akinetic mutism (Apathy)
3 Posterior cerebral artery: Amnesia, anomia, agnosia (Porget)

Question 19

Why should acetylcholinesterase inhibitors be tried for vascular dementia?

Answer 19

Because of the high frequency of overlap with AD and possible benefit in VaD

Question 20

What is the triad of Normal Pressure Hydrocephalus?

Answer 20

Dementia
Gait apraxia NO FESTINATION UNLIKE PD!
Urinary incontinence

Question 21

What is the upper limit or normal opening pressure?

Answer 21

180mmHg

Question 22

What are the characteristic MRI findings in NPH?

Answer 22

Ventriculomegaly with an Evans ratio of >0.31

Question 23

What is the ONLY treatment for NPH?

Answer 23

VP shunt

Question 24

What are the negative and positive phenomena sxs of Parkinson’s dz?

Answer 24

Negative: Bradykinesia, postural instability and freezing
Positive: Resting tremor and rigidity

Question 25

What is the key pathologic feature in PD?

Answer 25

Lewy bodies in the substantia nigra

Question 26

Why give carbidopa with levodopa in PD?

Answer 26

Carbidopa and benserazide is a dopa decarboxylase inhibitor preventing the metabolism of L-dopa before it reaches the dopaminergic neurons

Question 27

What is an example of COMT inhibitor?

Answer 27

Tolcapone

Question 28

What PD drug increases the risk of gambling and hypersexuality?

Answer 28

Dopamine agonists: Bromocriptine, pergolide, pramipexole, cabergoline, lisuride

Question 29

Which rugs provide symptomatic relief in early PD?

Answer 29

Dopamine agonists

Question 30

What drugs inhibit MAO-B thereby inhibiting the breakdown of dopamine?

Answer 30

Selegiline and rasagiline

Question 31

Where is the primary site for the “dying back phenomenon” of Friedrich ataxia?

Answer 31

Spinal cord and roots

There is loss of neurons and secondary gliosis

Question 32

What percentage of patient’s with Friedrich ataxia will be wheel chair bound by 45?

Answer 32

95%!

Question 33

What are the cardinal features of friedrich ataxia?

Answer 33

1 limb and GAIT ATAXIA of cerebellar and sensory type BEFORE 30
2 dysarthria
3 loss of joint position and vibration sense
4 absent tendon reflexes in legs
5 extensor plantar response

Question 34

What part of the corticospinal tracts are spared in Friedrich ataxia?

Answer 34

down to the cervicomedullary junction

Question 35

What would be the findings in genetic testing of a patient with Friedrich ataxia?

Answer 35

GAA repeat expansion on chromosome 9

Question 36

What is the genetic feature of Huntington dz?

Answer 36

CAG repeats on 4p

Question 37

What would MRI show in Huntington dz?

Answer 37

Cerebral atrophy especially of the caudate and putamen

Question 38

What is the treatment for essential tremor?

Answer 38

Propranolol (beta 2 receptor antagonism), primidone and diazepam

Gets better with alcohol

Question 39

What should be avoided in essential tremor?

Answer 39

Caffeine and nervousness

Question 40

What are the four overlapping syndromes in Multiple systems atrophy?

Answer 40

1 Olivopontocerebellar atrophy
2 Striatonigral degeneration– parkinsonism and pyramidal sign secondary to degenration of neurons in caudate and putamen
3 Shy drager– parkinsonism and dysautonomia
4 Cortical basal ganglionic degeneration – fronto parietal cortical atrophy