Cure MSA

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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


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

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
Pathologic laughter or crying