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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 …