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Clinical and pathological characteristics of later onset multiple system atrophy
Hiroaki Sekiya1,2, Shunsuke Koga3, Yoshihisa Otsuka4
1Department of Neuroscience, Mayo Clinic, 4500 San Pablo Road, Jacksonville, FL, 32224, USA. sekiya.hiroaki@mayo.edu.
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.
Insights
Later onset multiple system atrophy (MSA) affects 5-8% of patients and is associated with significantly shorter survival. Early consideration of MSA in elderly patients with neurological symptoms is crucial for timely diagnosis and management.
Area of Science:
- Neurology
- Geriatrics
- Pathology
Background:
- Current consensus criteria for multiple system atrophy (MSA) diagnosis exclude onset after age 75.
- Limited understanding exists regarding the clinical and pathological features of late-onset MSA (LO-MSA) compared to usual-onset MSA (UO-MSA).
Purpose of the Study:
- To investigate the characteristics and outcomes of LO-MSA.
- To compare LO-MSA with UO-MSA.
Main Methods:
- Retrospective analysis of clinical data from 83 patients (Kobe University Hospital, Amagasaki General Medical Center Hospital) and autopsy data from 193 patients (Mayo Clinic Florida brain bank).
- Patients were categorized into UO-MSA (onset ≤75 years) and LO-MSA (onset >75 years).
- Clinical features and outcomes were compared between the two groups.
Main Results:
- LO-MSA represented 8% of the clinical cohort and 5% of the autopsy cohort.
- LO-MSA patients exhibited significantly shorter median survival times from onset to death or tracheostomy compared to UO-MSA patients in both cohorts (4.8 vs 7.9 years, P=0.043; 3.9 vs 7.5 years, P<0.0001).
- Median survival from diagnosis to death was less than 3 years for LO-MSA in the clinical cohort.
Conclusions:
- Late-onset MSA is characterized by rapid progression and reduced survival, complicating clinical decision-making.
- MSA should be included in the differential diagnosis for elderly individuals presenting with autonomic dysfunction and extrapyramidal or cerebellar signs.
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