11.13 Parkinson-Plus Syndromes

Question 1

What are some red flags that indicate a dx other than PD?

Answer 1

• Early postural instability/abn postures
• rapid progression
• respiratory dysfunction
• labile
• signs of cerebellar, corticospinal, voluntary gaze dysfunction

Question 2

What IS Parkinson-Plus?

Answer 2

Collective name for primary neurodegenerative dz that causes s/s similar to PD

Question 3

What are the Parkinson-Plus syndromes?

Answer 3

• Progressive supranuclear palsy
• Dementia with Lewy bodies
• Multiple system atrophy
• CTE

Question 4

Which of the Parkinson-plus syndromes is associated with accumulation of tau?

Answer 4

Progressive supranuclear palsy

Question 5

CTE =

Answer 5

Chronic
Traumatic
Encephalopathy

Question 6

What is CTE caused by?

Answer 6

Repetitive head injuries (concussions)

Question 7

What are the common non-motor changes seen with CTE?

Answer 7

• Mood changes
• Depression
• Cognitive issues

Question 8

What sort of physiological changes come about with CTE?

Answer 8

• atrophy of cerebral hemispheres with brain volume loss

- Eventually affects the amygdala and hippocampus

Question 9

How does a diffuse axonal injury occur and what does it affect?

Answer 9

• Shockwave from head trauma goes through the brain (connection between cell bodies and axons)
• Affects all neurons in the cortex
• Get shearing of axons away from cell bodies

Question 10

What happens when there is shearing of axons from cell bodies in the CNS? How does this kill neurons?

Answer 10

GLUTAMATE CASCADE

• kills neurons via excitotoxicity
• CNS white matter forms a scar

Question 11

Why is a scar in the CNS white matter problematic?

Answer 11

• axons can’t cross the scar

- get lots of inflammation

Question 12

What does CTE cause the accumulation of?

Answer 12

Tau (hyperphosphorylation)

Question 13

Dementia is a late presentation of this Parkinson-plus syndrome

Answer 13

Lewy body dementia

Question 14

What is accumulated in Lewy body dementia?

Answer 14

Insoluble phosphorylated alpha-synuclein proteins all over the brain

Question 15

Alpha-synuclein is (intracellular/extracellular)

Answer 15

Intracellular

Question 16

What results from accumulation of alpha-synuclein protein with Lewy body dementia?

Answer 16

Neuron death

Question 17

Symptoms of Lewy body dementia

Answer 17

First

• mood changes
• cognitive decline
• can have vivid/disturbing hallucinations

Question 18

How is Lewy body dementia similar/distinct from PD?

Answer 18

• Lewy bodies occur FIRST (they like the SN)

- Dopaminergic cell death is SECONDARY to Lewy body formation

Question 19

Why does a pt with Lewy body dementia have hallucinations?

Answer 19

It affects visual vortices

Question 20

Can Lewy bodies occur in other disorders

Answer 20

• yes

- can occur in PD secondary to neuronal death

Question 21

With Lewy body dementia, where is inflammation and neuronal death most significant?

Answer 21

• BG

- brainstem

Question 22

What is frontotemporal dementia?

Answer 22

General term used to describe progressive neurodegenerative disorders that affect

• frontal lobe
• temporal lobe

Question 23

Example of frontotemporal dementia

Answer 23

Pick’s disease

Question 24

What will an MRI show with Pick’s disease?

Answer 24

Marked loss of frontal cortex volume

Question 25

What causes Pick's disease?

Answer 25

- thought to be a problem of clearing misfolded proteins - Ubiquitin - Proteasome system

Question 26

What s/s will be seen with Pick's disease?

Answer 26

- loss of executive function (FIRST) | - neurons swell in affected tissue area

Question 27

Pick's disease: plaques/tangles

Answer 27

None seen in the disorder

Question 28

Which is the most significant symptom of Pick's disease?

Answer 28

Loss of executive function (can overshadow memory disturbance)

Question 29

Treatment for Pick's disease

Answer 29

- no tx to slow progression | - antidepressants and antipsychotics to treat six