Diagnosis of Multiple System Atrophy (MSA)

About this tool

The Second Consensus Statement on the Diagnosis of Multiple System Atrophy provides an update to the previous 1988 criteria and are widely accepted. Whereas like other neurodegenerative diseases, the “definite” diagnostic category can only be used after posthumous pathological examination, the “probable” category can be readily used in clinical practice. To be diagnosed with MSA, a patient must have sporadic (i.e., not inherited), progressive disease with onset after age 30. It is well accepted that while both subtypes of MSA have prominent autonomic dysfunction, some have primarily parkinsonism (MSA-P) and others have primarily cerebellar dysfunction (MSA-C). For a diagnosis of “probable MSA” a patient must have autonomic dysfunction, including otherwise unexplained urinary urgency, frequency, or incomplete emptying, erectile dysfunction in males, or orthostatic blood pressure drop by at least 30 mmHg systolic or 15 mmHg diastolic within 3 min of standing. As well, for a diagnosis of “probable MSA-P”, the patient must have a poorly levodopa-responsive parkinsonism (bradykinesia with rigidity, tremor, or postural instability), and for a diagnosis of “probably MSA-C”, the patient must have a cerebellar syndrome (gait ataxia with cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction). In the case that a patient has orthostatic hypotension not meeting the strict numerical criteria, if one of the additional suggestive features are present (see below), a diagnosis of “possible MSA” can be made.

Additional features:

MSA-P or MSA-C
Babinski sign with hyperreflexia
Stridor

MSA-P
Rapidly progressive parkinsonism
Poor response to levodopa
Postural instability within 3 y of motor onset
Gait ataxia, cerebellar dysarthria, limb ataxia, or cerebellar oculomotor dysfunction
Dysphagia within 5 y of motor onset
Atrophy on MRI of putamen, MCP, pons, or cerebellum
Hypometabolism on FDG-PET of putamen, brainstem, or cerebellum

MSA-C
Parkinsonism (bradykinesia and rigidity)
Atrophy on MRI of putamen, MCP, or pons
Hypometabolism on FDG-PET in putamen
Presynaptic nigrostriatal dopaminergic denervation on SPECT or PET

References

S Gilman, MD, FRCP, G K. Wenning, MD, PhD, P A. Low, MD, FRACP, FRCP(Hon), D J. Brooks, MD, DSc, FRCP, FMedSci, C J. Mathias, MBBS, DPhil, DSc, FRCP, FMedSci, J Q. Trojanowski, MD, PhD, N W. Wood, MB, ChB, PhD, FRCP, C Colosimo, MD, A Dürr, MD, PhD, C J. Fowler, FRCP, H Kaufmann, MD, T Klockgether, MD, PhD, A Lees, MD, FRCP, W Poewe, MD, N Quinn, MD, FRCP, T Revesz, MD, D Robertson, MD, P Sandroni, MD, PhD, K Seppi, MD, and M Vidailhet, MD, PhD.
Second consensus statement on the diagnosis of multiple system atrophy.
Neurology. 2008 Aug 26; 71(9): 670–676.