Ambulatory Report – 12.7.16 – Multiple Systems Atrophy

Thanks to Jesse Fitzpatrick for presenting a great case of a 75 yo M with h/o HTN, BPH and numerous falls at home, ultimately diagnosed with Multiple Systems Atrophy (MSA.)
  • Key Points
    • Fatal neurodegenerative dz – progressive autonomic failure, parkinsonian features, and cerebellar/pyramidal features
    • onset usually in 60s
    • mean survival is 6-10yrs
  • Natural History of MSA
    • 66f78484-1aef-4c5d-926c-a49b6ce65dd8
  • Tx
    • No effective dz-modifying or neuroprotective tx is available currently
    • For Motor Sx – PT, Botox injections (for focal dystonia), speech therapy & assessing dysphagia, CPAP/BiPAP/tracheostomy
    • Levodopa & Dopaminergic Tx – generally diagnostic, and can trial on it therapeutically (but sustained/effective response in the long term is rarely observed in patients w/ MSA)
    • Orthostatic Hypotension – 1st line fludrocortisone, 2nd line alpha-1 adrenoreceptor agonists (ephedrine, pseudo ephedrine, phenylephrine, midodrine); also behavior modifications!
    • Depression – common problem in MSA; pharmacotherapy AND support groups!
    • See UpToDate for more specific treatments for other manifestations
  • Multiple System Atrophy – NEJM 2015
  •  82aec4d4-8bdb-4e5c-b0d2-6a56ba1a9cd8

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