Objective: We report a 47-year survival study of autopsy verified Parkinson syndrome (PS) and Parkinson’s disease (PD) cases. The objectives were: 1) to identify PS and PD patients that survived >20 years; 2) to identify PS and PD cases that had similar onset age but survived <10 years; and 3) to determine their distinguishing features.

Background: PD is the most common PS. The clinical diagnosis is based on two of the three – bradykinesia, rigidity and tremor. The pathology is characterized by marked substantia nigra neuronal loss and Lewy body inclusions. Some other neurodegenerative disorders also present with similar clinical features. Pathology findings remain the gold standard for distinction between different variants of PS as the clinical diagnosis of PD is inaccurate in approximately 25% cases. Survival in PD after the motor onset is shorter than expected in the general population. It is further reduced in those with older onset age. Survival of > 20 years remains rare. Most such cases have onset <70 years. The incidence of PD onset under age 70 has remained constant for several decades. Any change in survival in these cases would therefore impact the PD prevalence rate.

Method: All PS cases autopsied at one institution during 47 years were considered. All PS cases that survived >20 years had onset before age 70. Another PS group with onset under age 70 but survival <10 years was chosen for comparison to identify features that distinguished the long from the short surviving cases.

Results: 392 PS including 314 (80%) PD cases were autopsied during 47 years. 15.6% of all PS and 16% of all PD cases with onset under age 70 survived >20 years. Survival in PD was longer than other common PS variants. However, a small tauopathy group with early onset had the longest survival (median 50 years). Older age at onset and dementia diagnosis at baseline were associated with shorter survival. Motor severity at baseline was similar between the long and short surviving groups. Prior to the widespread use of levodopa, most PD cases had onset <70 years and survived <10 years. Such cases now account for only 3.8% of PD.

Conclusion: The proportion of PD cases surviving >20 years has increased considerably in recent years and the number of <10 years survivors has decreased markedly. This has resulted in a higher prevalence rate and increased need for healthcare services.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/the-long-and-short-of-contemporary-parkinsonism-survival-a-47-year-clinicopathological-study/