Consensus diagnostic criteria for MSA
In 1989 Quinn first proposed diagnostic criteria for MSA, which were subsequently modified in 1994. The Quinn criteria distinguish parkinsonian and cerebellar presentations as well as three levels of diagnostic certainity (possible, probable, definite). So far, sensitivity and specificity of the Quinn criteria have never been prospectively determined. A retrospective validation based on post-mortem verified MSA cases demonstrated suboptimal diagnostic accuracy. In April 1998 an International Consensus Conference was convened to develop optimised criteria for a clinical diagnosis of MSA. The MSA conference recommended three diagnostic categories of increasing certainty: possible, probable and definite. Recently, a second consensus statement on the diagnosis of MSA has been published by Gilman and colleagues (Gilman, Wenning et al. Neurology 2008). The three diagnostic categories should be maintained according to the first consensus statement.A definite diagnosis requires the neuropathologic findings of widespread and abundant CNS-synuclein–positive glial cytoplasmic inclusions (Papp–Lantos inclusions) in association with neurodegenerative changes in striatonigral or olivopontocerebellar structures.
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) and |
● At least one feature suggesting autonomic dysfunction (otherwise unexplained urinary urgency, frequency or incomplete bladder |
● At least one of the additional features shown in table 3 |
TABLE 3 - ADDITIONAL FEATURES OF POSSIBLE MSA
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● Babinski sign with hyperreflexia |
● Stridor |
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● 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 |
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● 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 |
TABLE 4 - FEATURES SUPPORTING (RED FLAGS) AND NOT SUPPORTING A DIAGNOSIS OF MSA
Supporting features | Nonsupporting features |
● Orofacial dystonia | ● Classic pill-rolling rest tremor |
● Disproportionate antecollis | ● Clinically significant neuropathy |
● Camptocormia (severeanterior flexion of the spine) and/or Pisa syndrome (severe lateral flexion of the spine) | ● Hallucinations not induced by drugs |
● Contractures of hands or feet | ● Onset after age 75 y |
● Inspiratory sighs | ● Family history of ataxia or parkinsonism |
● Severe dysphonia | ● Dementia (on DSM-IV) |
● Severe dysarthria sclerosis | ● White matter lesions suggesting multiple |
● New or increased snoring ● Cold hands and feet |
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