PSP

Question 1

What are the 4 key presentations of PSP?

Answer 1

1. Parkinsonism
2. Oculomotor
3. Bulbar symptoms
4. Cognition

Question 2

What are the Parkinsonism features of PSP?

Answer 2

Symmetrical rigidity (axial > limb)
Bradykinesia
Non tremor dominant
Significant postural instability and early falls
Gunslinger gait (trunk extended) and fall backwards

Question 3

What are the oculomotor findings in PSP?

Answer 3

Vertical gaze palsy (limited downgaze more specific)
Slow vertical saccades
Square wave jerks
Apraxia of eyelid opening (staring appearance)
Decreased blink rate

Question 4

What are the bulbar symptoms of PSP?

Answer 4

Hypophonia
Hypernasal dysarthria
Dysphagia

Question 5

What are the cognitive changes in PSP?

Answer 5

Executive dysfunction
Bradyphenia (slowness of thought)
Pseudobulbar affect
Apathy/depression
Applause sign (ask to clap 3 times and they continue)

Question 6

What is Richardson syndrome?

Answer 6

Most common PSP subtype
Early falls
Axial rigidity
Postural instability
Symmetric bradykinesia
Vertical gaze abnormality
Bradyphrenia
Little to no response to levodopa

Question 7

What are the MRI findings of PSP?

Answer 7

Dorsal midbrain atrophy
- Axial view - morning glory sign, Mickey Mouse sign
- Sagittal view - hummingbird sign

Question 8

What are the mandatory inclusion criteria for PSP?

Answer 8

1. Sporadic occurrence
2. 40+ yo at onset
3. Gradually progressive neurodegenerative disease

Question 9

What are the core 4 features of diagnostic criteria for PSP?

Answer 9

1. Oculomotor dysfunction
   - Vertical SNGP
   - Slow vertical saccades
   - Square wave jerks/eyelid opening apraxia
2. Postural instability
   - Repeat unprovoked falls 3 yrs
   - Fall on pull test 3 yrs
   - >2 steps backward pull test 3 yrs
3. Akinesia
   - Prog. gait freezing 3 yrs
   - Parkinsonism, akinetic rigid, axial, levodopa resistance
   - Parkinsonism with tremor +/- asymmetric +/- LD response
4. Cognitive dysfunction
   - Speech/language disorder
   - Frontal cognitive/behavioural
   - Corticobasal syndrome

Question 10

Management of Parkinsonism in PSP

Answer 10

1. Ldopa trial - only if brady, rigid, tremor affects ADLs
2. Avoid DA, MAOB, COMT, adenosine A2A
3. Avoid non-dopaminergic and pure anticholinergics

Question 11

Pharmacologic management of PSP

Answer 11

1. Amantadine
2. Coenzyme Q10

Question 12

Non pharm management of PSP

Answer 12

1. Physical therapy - early, postural stability, gait, balance, eye movement training
2. Exercise - aerobic, recumbent bike
3. Assisted devices - weighted walker, wheelchair

Question 13

Differential diagnosis for PSP

Answer 13

PSP-RS
PSP-P
PSP-CBS