Multiple systems atrophy

Diagnosis

MSA clinical criteria (Gilman and Wenning, 2008)

1) Probable MSA, a sporadic, progressive, adult (>30 year)-onset disease characterized by

a. Autonomic failure involving urinary incontinence (inability to control the release of urine from the bladder, with erectile dysfunction in males) or an orthostatic decrease of blood pressure within 3 minutes of standing by at least 30mm Hg systolic or 15mm Hg diastolic

b. Must have at least one of the following:

i. Poor levodopa-response Parkinsonism (bradykinesia with rigidity, tremor, or postural instability)

ii. A cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction).

2) Possible MSA, a sporadic, progressive, adult (>30 year)-onset disease characterized by

a. Parkinsonism (bradykinesia with rigidity, tremor, or postural instability)

b. A cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction)

c. At least one feature suggesting autonomic dysfunction (otherwise unexplained urinary urgency, frequency, or incomplete bladder emptying, erectile dysfunction in males, or significant orthostatic blood pressure decline that does not meet the level required in probable MSA)

d. At least one additional feature

i. Possible MSA-P or MSA-C:

1. Babinski sign with hyperreflexia

2. Stridor

ii. Possible MSA-P:

1. Rapidly progressive Parkinsonism

2. Poor response to levodopa

3. Postural instability within 3 years of motor onset

4. Gait ataxia, cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction

5. Dysphagia within 5 years of motor onset

6. Atrophy on MRI of putamen, middle cerebellar peduncle, pons, or cerebellum

iii. Possible MSA-C:

1. Parkinsonism

2. Atrophy on MRI of putamen, middle cerebellar peduncle, or pons

3. Putamen hypometabolism on FDG-PET

4. Presynaptic nigrostriatal dopaminergic denervation on SPECT or PET

References

Gilman, S. & Wenning, G. K. Second consensus statement on the diagnosis of multiple system atrophy. 7 (2008). https://pubmed.ncbi.nlm.nih.gov/18725592/