The Profile of Long-term Parkinson’s Disease Survivors with 20 Years of Disease Duration and Beyond

Abstract

Abstract.Background: Parkinson's disease (PD) patients with 20 years or more survival are not well characterized. Objective: To evaluate PD-20 patient characteristics and identify areas for improvement of their health care. Methods: The international, multicenter National Parkinson's Foundation Quality Improvement Initiative (NPF-QII) study database was queried to identify PD-20 subjects. Demographic and clinical data were analyzed. Results: We identified 187 PD-20 subjects (55% men) representing 4% (187/4,619) of all NPF-QII participants. Subjects were mean age 69.5 years; mean age at PD onset was 44.0 years. The majority (75%) had 20-25 years of PD duration, the longest duration being 49 years. They were median Hoehn and Yahr stage 3, and 75% had motor fluctuations. Half (54%) reported exercising. The majority (89%) were living at home and required a caregiver (88%). They were mildly cognitively impaired for age (Montreal Cognitive Assessment estimate 22.6 ± 3.7), with most deficits in verbal fluency and delayed recall. Quality of life (Parkinson's Disease Quality of Life Questionnaire index 36 ± 15%) was mild to moderately impaired, with most impairment in mobility and activities of daily living. Caregiver strain measured by the Multidimensional Caregiver Strain Index (27 ± 16%), recorded highest subscores in social constraint. PD-20 subjects aged <70 years versus ≥70 only differed significantly by worse cognition (P < 0.0001). Conclusions: PD-20 subjects reflect an elite group of PD survivors with early-onset disease and relatively mild cognitive disability despite long disease duration. Interventions for caregivers, mobility, and activities of daily living are areas that could improve caregiver burden and patient quality of life.

Dementia in long-term Parkinson’s disease patients: a multicentre retrospective study

“…Interestingly, findings from this study revealed that only 1 out of the 36 participants exhibited a TD phenotype, which might be less than would be anticipated from previous studies suggesting that such patients are conferred with a more benign course and slower disease progression 15,17,40,41 . While the overrepresentation of patients with non-TD feature may reflect an artefact of how the motor phenotypes were classified in the present study, such overrepresentation more likely suggests the transition of predominant motor features over the long disease course.…”

“…To date, only a limited number of studies have investigated the clinical features of PD patients with disease duration of 20 years or more, as this is a small number of people with PD. 10,17 We identified 36 such patients from a cohort of 2327 patients (1.5%) and investigated the frequency of dementia within them using data from assessments ≥20 years from diagnosis. Using the MDS Level 1 PDD criteria, only 7 of the 36 (19%) participants met criteria for PDD after 20 years.…”

“…While several studies have investigated the clinical progression of cognitive decline in PD patients, there has been a paucity of data on specifically evaluating PD patients with a disease duration of over 20 years. 17 The current study retrospectively investigated the frequency of dementia in PD patients with a disease duration of over 20 years assessed in research clinics across the UK and Australia. Our aim was to determine whether these patients would demonstrate the high rates of dementia that might be anticipated from the previous longitudinal cohort studies, and if there were any phenotypic features that might ameliorate against this transition.…”

Cognitive impairment in Parkinson's disease: Association between patient-reported and clinically measured outcomes

“…As treatments for Parkinson’s disease movement symptoms advance and patients maintain an active lifestyle for longer disease duration [30], even the earliest cognitive impairments are an increasing source of disability [6]. Our findings suggest that when a PD patient reports a slight degree of cognitive impairment, declines in visuospatial function and verbal memory are most likely to be driving this complaint relative to other cognitive domains.…”

Classification of advanced stages of Parkinson’s disease: translation into stratified treatments

“…In the case of juvenile or early onset PD before the age of 35 years, a further genetic analysis, even in the absence of a positive family history, as typically related to an autosomal recessive inheritance with unaffected parents, is worthwhile (Sheerin et al 2014). Early onset patients typically present with a more benign disease course and are less frequently subject to cognitive impairments; however, motor fluctuations are typically observed and can justify interventional treatment options within the advPD concept (Hassan et al 2015) Generally, these forms of PD are typically rare and as a chronic-progressive neurodegenerative disease, most patients are diagnosed at a rather advanced age. Thus, at the age of 65, the incidence amounts to approximately 50 in 100,000, at age 75–150 in 100,000, and at age 85–400 in 100,000 (de Lau et al 2006; Pringsheim et al 2014).…”