Abstract Details

Title Later Onset Multiple System Atrophy: Poor Prognosis and Diagnostic Challenge

Topic Movement Disorders

Presentation(s) S36 - Movement Disorders: Clinical and Pathologic Characterization of Neurodegenerative Movement Disorders (1:12 PM-1:24 PM)

Poster/Presentation
Number 002

Background

Although onset after age 75 is considered as non-supporting for MSA in the current criteria, MSA patients may present with onset after age 75. Clinical and pathological characteristics of LO-MSA remain poorly understood.

Objective To characterize clinical and pathologic characteristics of later onset multiple system atrophy (LO-MSA) compared to usual onset MSA (UO-MSA).

Design/Methods We examined the characteristics of LO-MSA in the clinical cohort and validated the result using the autopsy cohort. The clinical cohort included consecutive MSA patients admitted in Kobe University Hospital and Amagasaki General Medical Center Hospital, while the autopsy cohort included pathologically confirmed MSA patients from the brain bank at Mayo Clinic Florida. We retrospectively identified 83 patients in the clinical cohort and 193 cases 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.

Results

LO-MSA accounted for 8% in the clinical cohort and 5% in the autopsy cohort. The median time from disease 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 = 0.0434 and P < 0.0001). The median time from diagnosis to death was less than 3 years in LO-MSA in the clinical cohort. In the autopsy cohort, 83% (153/184) of UO-MSA had a confirmed diagnosis of MSA before death, while only 11% (1/9) of LO-MSA had a confirmed diagnosis of MSA before death.

Conclusions Some MSA patients have late age of onset. LO-MSA patients have short survival, which limits the 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.

Authors/Disclosures
Hiroaki Sekiya, MD, PhD (Mayo Clinic) PRESENTER	Dr. Sekiya has nothing to disclose.
Shunsuke Koga, MD, PhD (Hospital of the University of Pennsylvania)	Dr. Koga has nothing to disclose.
	No disclosure on file
	No disclosure on file
	No disclosure on file
Kenji Sekiguchi, MD, PhD (Kobe University Graduate School of Medicine)	Dr. Sekiguchi has nothing to disclose.
	No disclosure on file
	No disclosure on file
Riki Matsumoto, MD (Division of Neurology, Kobe University Grad. Sch. Med)	Dr. Matsumoto has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Eisai. Dr. Matsumoto has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for UCB Japan. Dr. Matsumoto has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Daiichi-Sankyo. Dr. Matsumoto has received personal compensation in the range of $0-$499 for serving on a Speakers Bureau for Ostuka.
Dennis W. Dickson, MD (Mayo Clinic)	Dr. Dickson has nothing to disclose.