Clinical and pathological characteristics of later onset multiple system atrophy

Hiroaki Sekiya, Shunsuke Koga, Yoshihisa Otsuka, Norio Chihara, Takehiro Ueda, Kenji Sekiguchi, Yukihiro Yoneda, Yasufumi Kageyama, Riki Matsumoto, Dennis W. Dickson

Research output: Contribution to journalArticlepeer-review


Background: In the current consensus criteria, onset after age 75 is considered as non-supporting for diagnosis of multiples system atrophy (MSA); however, some MSA patients present after age 75. Clinical and pathological characteristics of such later onset MSA (LO-MSA) compared to usual onset MSA (UO-MSA) remain poorly understood. Methods: The clinical cohort included patients from Kobe University Hospital and Amagasaki General Medical Center Hospital, while the autopsy cohort was from the brain bank at Mayo Clinic Florida. We identified 83 patients in the clinical cohort and 193 patients in the autopsy cohort. We divided MSA into two groups according to age at onset: UO-MSA (≤ 75) and LO-MSA (> 75). We compared clinical features and outcomes between the two groups in the clinical cohort and compared the findings to the autopsy cohort. Results: LO-MSA accounted for 8% in the clinical cohort and 5% in the autopsy cohort. The median time from onset to death or to life-saving tracheostomy was significantly shorter in LO-MSA than in UO-MSA in both cohorts (4.8 vs 7.9 years in the clinical cohort and 3.9 vs 7.5 years in the autopsy cohort; P = 0.043 and P < 0.0001, respectively). The median time from diagnosis to death was less than 3 years in LO-MSA in the clinical cohort. Conclusions: Some MSA patients have late age of onset and short survival, limiting time for clinical decision making. MSA should be considered in the differential diagnosis of elderly patients with autonomic symptoms and extrapyramidal and/or cerebellar syndromes.

Original languageEnglish (US)
Pages (from-to)4310-4321
Number of pages12
JournalJournal of Neurology
Issue number8
StatePublished - Aug 2022


  • Diagnostic accuracy
  • Elderly
  • Later onset
  • Multiple system atrophy
  • Neuropathology

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology


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