Brain Matters

Multiple System Atrophy - Diagnostic Criteria

Adapted from Gilman S, Wenning GK, Low PA, et al.  Second Consensus statement on the diagnosis of multiple system atrophy. Neurology. 2008 Aug 26;71(9):670-6.

Table 1:  Criteria for the diagnosis of probable MSA

A sporadic, progressive, adult (>30 y) onset disease characterized by:

*  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 min of standing by at least 30 mm Hg systolic or 15 mm Hg diastolic and

* Poorly levodopa-responsive parkinsonism (bradykinesia with rigidity, tremor, or postural instability) or

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

Table 2:  Criteria for possible MSA

A sporadic, progressive, adult (>30 y).onset disease characterized by:

* Parkinsonism (bradykinesia with rigidity, tremor, or postural instability) or

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

* 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) and

* At least one of the additional features shown in table 3

Table 3:  Additional features of possible MSA

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

Possible 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, middle cerebellar peduncle, pons, or cerebellum
* Hypometabolism on FDG-PET in putamen, brainstem, or cerebellum

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

MSA = multiple system atrophy

MSA-P = MSA with predominant parkinsonism
MSA-C = MSA with predominant cerebellar ataxia
FDG = [18F] fluorodeoxyglucose

Table 4:  Features supporting (red flags) and not supporting a diagnosis of MSA

Supporting features
* Orofacial dystonia
* Disproportionate antecollis
* Camptocormia (severe anterior flexion of the spine) and/or Pisa syndrome (severe lateral flexion of the spine)
* Contractures of hands or feet
* Inspiratory sighs
* Severe dysphonia
* Severe dysarthria
* New or increased snoring
* Cold hands and feet
* Pathologic laughter or crying
* Jerky, myoclonic postural/action tremor

Nonsupporting features
* Classic pill-rolling rest tremor
* Clinically significant neuropathy
* Hallucinations not induced by drugs
* Onset after age 75 y
* Family history of ataxia or parkinsonism
* Dementia (on DSM-IV)
* White matter lesions suggesting multiple sclerosis

MSA = multiple system atrophy

DSM-IV = Diagnostic and Statistical Manual of Mental Disorders, Fourth Edition