multiple systems atrophy - endoflifewa.org · demographics • prevalence of 2-4 per 100,000...
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Multiple Systems Atrophy
John W. Roberts, MD
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Demographics
• Prevalence of 2-4 per 100,000 population (may be underestimated)
• Median age of onset is 55 years (range 33-76)
• Men : women = 1.3 : 1• Mean survival 6-9 years; half are disabled or
wheelchair bound within 5 years of onset of motor symptoms
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Former Names for MSA
• Shy-Drager syndrome (MSA-A)• Striatonigral degeneration (MSA-P)• Olivopontocerebellar atrophy “OPCA”
(MSA-C)
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MSA Subtypes
• Parkinsonian = MSA-PParkinson like features• Slowness• Muscle rigidity• Shuffling gait• But not resting tremor
Poorly or transiently responsive to levodopaand other Parkinson disease medications
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MSA Subtypes
• Autonomic = MSA-AOrthostatic hypotensionReduced ability to sweatConstipationBladder dysfunctionErectile dysfunction
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MSA-A
• Marked loss of ability to sweat over time compared to Parkinson’s disease
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MSA Subtypes
• Cerebellar = MSA-CWide based gaitIntention tremorAtaxiaNystagmus“Scanning Speech”
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MSA Subtypes
• Most patients have a mixture of MSA-P, MSA-A, or MSC-C
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Other MSA Symptoms
• Dysarthria• Laryngeal stridor• Anterocollis• Sleep apnea or RBD• Stimulus sensitive myoclonus
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Symptoms of MSA
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Not Compatible with MSA
• Asymmetric symptoms• Resting tremor• Early dementia• Prominent ophthalmoplegia
Vertical gaze palsy• Apraxia• Cortical sensory loss
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Comparisons PD MSA PSP CBDSymmetry of deficits + +++ +++ --Axial rigidity ++ ++ +++ ++Limb dystonia + + + +++Postural instability ++ ++ +++ +Vertical gaze palsy + ++ +++ ++Frontal behavior + + +++ ++Dysautonomia + ++ -- --L-dopa response early +++ + + --L-dopa response late ++ + -- --Asymmetric cortical atrophy on MRI
-- -- -- ++
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Time Course of MSA Compared to Parkinson disease and PSP
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Treatment of ParkinsonianSymptoms
• Levodopa/carbidopa (Sinemet)• Amantadine• Physical Therapy• Occupational Therapy• Speech Therapy
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Treatment of Low Blood Pressure
• Sodium• Fluid intake• Pressure stockings• Midodrine• Fludrocortisone
Matisse “Icarus”
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Treatment of Genitourinary symptoms
• Oxybutinin or tolterodine for urinary frequency or incontinence
• Sildenafil for impotence
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Treatment of Depression
• Selective serotonin reuptake inhibitors• Tricyclic antidepressants• Counseling
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Symptoms Resistant to Drug Treatment
• AtaxiaPhysical Therapy helpful though
• Dementia (seen late in disease course)Benefit of Alzheimer’s drugs uncertain
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Pathology of MSA
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Pathology of MSA
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Normal Dopamine Synapse
dopamine
dopamine receptor
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Parkinson’s disease Synapse
dopamine
dopamine receptor
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MSA Synapse
dopamine
dopamine receptor
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MRI Findings in MSA
Watanabe H et al. Brain 2002;125:1070-1083
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Hot Cross Buns
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Hot Cross Bun Sign
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Parkinson Disease PET Scan
FP-CIT dopamine SPECT IBZM dopamine receptor SPECT
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MSA PET Scan
FP-CIT dopamine SPECT IBZM dopamine receptor SPECT
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Summary
• MSA is a rare disease• There are three basic subtypes that often
overlap in one individualMSA-A, MSA-P, and MSA-C
• The diagnosis is mostly based on history and physical findings
But brain imaging may be helpful• Treatment is possible
But limited at this time