Multiple systems atrophy (not core condition)

Question 1

What is MSA

Answer 1

Fatal neurodegnerative disorder - autonomic dysfunction and parkinsonism

Question 2

What proteins cause MSA

Answer 2

Alpha synuclein in oligodendrocytes

Question 3

What is the classicl neuropathological features of MSA

Answer 3

Glial cytoplasmic inclusons of alpha synuclein

Question 4

Probable MSA

Answer 4

Autonomic failure involving urinary incontinence, erectile dysfunction in males or orthostatic hypotension
AND Parkinsonism that is poorly responsive to levodopa
OR a cerebellar syndrome e.g. limb ataxia, cerebellar dysarthria

Question 5

Possible MSA

Answer 5

No autonomic dysfunction:
MSA with predominant Parkinsonism (MSA-P), where the following features may be prevalent:
Babinski sign, hyperreflexia
Stridor
Rapidly progressive Parkinsonism
Poor response to levodopa
Postural instability and dysphagia within 3 years of motor onset, gait ataxia.
MSA with predominant cerebellar ataxia (MSA-C), including the following features:
Babinski sign, hyperreflexia
Stridor
Parkinsonism with suggestive imaging (hypometabolism on FDG-PET in putamen)

Question 6

Clinical features MSA

Answer 6

Autonomic failure - genitorurinary dysfucntion
Parkinsonism - bradykinesia with rigidity, tremor, postural instability
REM sleep disorder

Question 7

What does MSA show on MRI

Answer 7

w putaminal, pontine, and middle cerebellar peduncle (MCP) atrophy in MSA.

Question 8

Imaging for MSA

Answer 8

MRI
Brain stem PET scan - 18F
MR diffusion weighted imaging

Question 9

Red flags for MSA

Answer 9

Early instability, with recurrent falls within 3 years of onset
Rapid progression, wheelchair dependent within 10 years of onset
Contractures of the hands and feet
Bulbar dysfunction, including severe dysphonia, dysarthria and/or dysphagia
Emotional incontinence including inappropriate crying and/or laughing