Multiple system atrophy/Progressive supranuclear Palsy/Cortical Basal Degeneration

Question 1

Multiple system atrophy (MSA), a Parkinsons plus syndrome is a fatal neurodegenerative disorder of unknown aetiology defined chiefly by autonomic dysfunction, cerebellar and parkinsonism. What is the incidence of this condition?

1 - 600 cases per 100,000
2 - 60 cases per 100,000
3 - 6 cases per 100,000
4 - 0.60 cases per 100,000

Answer 1

4 - 0.60 cases per 100,000

Question 2

Multiple system atrophy (MSA), a Parkinsons plus syndrome is a fatal neurodegenerative disorder of unknown aetiology defined chiefly by autonomic dysfunction and parkinsonism. What age does the incidence typically peak?

1 - 60-70
2 - 40-50
3 - 20-30
4 - 10-20

Answer 2

1 - 60-70

Question 3

Although the exact cause of multiple system atrophy (MSA) is unknown, which of the following has been associated with the pathophysiology of MSA?

1 - aggregates of proteins called tau protein
2 - aggregates of proteins called B amyloid
3 - aggregates of proteins called a-synuclein
4 - aggregates of proteins called BDNF

Answer 3

3 - aggregates of proteins called a-synuclein

Neuronal loss and axonal degeneration involving multiple regions of the nervous system is also observed in MSA.

MSA = occur on oligodendrocytes

Parkinsons disease = typically dopaminergic neuronal cell bodies

Question 4

Which of the following is NOT a classification of multiple system atrophy (MSA)?

1 - definitive MSA
2 - probable MSA
3 - likely MSA
4 - possible MSA

Answer 4

3 - likely MSA

Definitive MSA can only be confirmed during autopsy

Question 5

Definitive multiple system atrophy (MSA) can only be confirmed on autopsy. Which of the following features must a patient have to be classified as probably MSA?

• Autonomic failure
• Parkinsonism that is poorly responsive to levodopa
• cerebellar syndrome e.g. limb ataxia, cerebellar dysarthria

1 - all features
2 - top 2 OR bottom one
3 - >1
4 - >2

Answer 5

2 - top 2 OR bottom one

Autonomic failure presents as: urinary incontinence, erectile dysfunction in males or orthostatic hypotension

Question 6

In patients with suspected multiple system atrophy (MSA), which of the following is NOT a clinical presentations of autonomic dysfunction?

1 - genitourinary dysfunction (urinary incontinence, erectile dysfunction in males)
2 - orthostatic hypotension
3 - cardiac arrhythmias
4 - constipation

Answer 6

3 - cardiac arrhythmias

Question 7

In patients with suspected multiple system atrophy (MSA), which of the following is NOT a clinical presentations of parkinsonism?

1 - bradykinesia with rigidity
2 - hyporeflexia
3 - tremor
4 - postural instability
5 - REM sleep disorder

Answer 7

2 - hyporeflexia

Question 8

Which of the following are red flags supporting a diagnosis that is NOT multiple system atrophy (MSA):

1 - age of onset >75 years
2 - family history of Parkinson’s disease
3 - Dementia
4 - White matter lesions typical of multiple sclerosis on imaging
5 - Neuropathy
6 - all of the above

Answer 8

6 - all of the above

Dementia is not a feature of MSA and should be excluded.

Question 9

Diagnosing multiple system atrophy (MSA) is typically a clinical diagnosis. However, which of the following are typically performed top rule out other causes of the patients symptoms?

1 - FBC
2 - U&E
3 - CRP
4 - LFTs
5 - HIV, syphilis serology
6 - Copper studies
7 - all of the above

Answer 9

7 - all of the above

Screen for infection or metabolic abnormalities

Infectious causes of cerebellar and cognitive symptoms

Wilson’s disease may present with parkinsonism

Question 10

Which of the following features may be visible on brain imaging in patients with suspected multiple system atrophy (MSA)?

1 - hot cross bun sign on pons
2 - hypometabolism
3 - putaminal, pontine, and middle cerebellar peduncle atrophy
4 - all of the above

Answer 10

4 - all of the above

Question 11

Is levodopa (L-DOPA) effective in treating the Parkinsonism associated with multiple system atrophy (MSA)?

Answer 11

• no

Good way of distinguishing between MSA and Parkinsons disease

Question 12

Although multiple system atrophy (MSA) cannot be cured, which of the following can be used as treatment options?

1 - Physiotherapy and occupational therapy
2 - Orthostatic hypotension medication
3 - Midodrine can improve erect blood pressure thus help prevent falls.
4 - Anti-diuretic hormone analogues (e.g. desmopressin) can be used for incontinence.
5 - Long-term urinary catheterisation to avoid retention.
6 - all of the above

Answer 12

6 - all of the above

Essentially treating the symptoms

Question 13

Progressive supranuclear palsy (PSP) is a rare neurological condition that can cause problems with balance, movement, vision, speech and swallowing caused by neurons becoming damaged over time. Which of the following is NOT a feature of PSP?

1 - Parkinsonism
2 - Postural instability
3 - Early falls
4 - Fluctuating cognition
5 - Vertical supranuclear gaze palsy (patients can not look up or down)
6 - Pseudobulbar palsy i.e. upper motor neurone dysarthria and dysphagia
7 - Cognitive impairment

Answer 13

4 - Fluctuating cognition

There is cognitive impairment, but only fluctuates in lewy body dementia

Question 14

What is the name of the sign commonly seen on a brain MRI of a patient with Progressive supranuclear palsy (PSP)?

1 - birds peak
2 - hoovers sign
3 - hot cross bun sign
4 - humming bird sign

Answer 14

4 - humming bird sign