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Causes of death in multiple system atrophy
  1. Spiridon Papapetropoulos1,
  2. Alexander Tuchman1,
  3. Daniel Laufer1,
  4. Athanassios G Papatsoris2,
  5. Nektarios Papapetropoulos3,
  6. Deborah C Mash4
  1. 1Department of Neurology, University of Miami, Miller School of Medicine, Miami, USA
  2. 22nd Department of Urology, School of Medicine, University of Athens, Athens, Greece
  3. 3Department of ENT, Tameside General Hospital, Ashton under Lyne, Lancashire, UK
  4. 4Department of Neurology, University of Miami, Miller School of Medicine, Miami, USA
  1. Correspondence to:
 Dr Papapetropoulos Spiridon
 Department of Neurology, University of Miami, School of Medicine, Room 4004, 1501 NW 9th Avenue, Miami, FL 33136, USA;spapapetropoulos{at}med.miami.edu

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Multiple system atrophy (MSA) is a heterogeneous neurodegenerative disorder, with a clinical presentation combining extrapyramidal, cerebellar, autonomic or pyramidal symptoms. There are two major subtypes: MSA-P, with a clinical predominance of parkinsonism, and MSA-C, with a clinical predominance of cerebellar symptoms. Although various factors have been proposed to predict survival in MSA, including age at onset and several phenotypic features,1 the terminal/end of life events have never been systematically studied. We present our results from a study on the causes of death in a series of pathologically confirmed, definite MSA cases.

All patients registered with the University of Miami/NPF Brain Endowment Bank (UM/BEB) donation programme with a diagnosis of neuropathologically confirmed, definite multiple system atrophy (MSA; n = 21) were included in this study. Pertinent information was gathered by two prospectively filled questionnaires used as part of the UM/BEB’s recruitment process: (a) the UM/BEB Parkinson’s disease registry form, a 128-item, self-administered questionnaire on demographics, environmental exposures, personal and family history, comorbid conditions, activities of daily living, clinical and treatment details; and (b) the “agonal state” form, a 25-item questionnaire on events covering the 48 h before death completed by the treating doctor/nurse. For comparisons, each MSA case was closely matched for age at disease onset (±2 years) and sex with a Parkinson’s disease brain donor by an investigator blinded to the disease status and clinical information. Medical, hospital and hospice …

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