Prognosis

At 10 years, 55% of patients with Parkinson disease died; 23% had a good outcome

Williams-Gray CH, Mason SL, Evans JR, et al. The CamPaIGN study of Parkinsons disease: 10-year outlook in an incident population-based cohort. J Neurol Neurosurg Psychiatry. 2013;84:1258-1264.

Clinical impact ratings: F ★★★★★★✩ N ★★★★★★✩ Questions

Conclusions

What are the long-term outcomes for patients with Parkinson disease (PD)? Which factors are associated with poor outcomes?

At 10 years after diagnosis of Parkinson disease, 55% of patients had died and 23% had survived without postural instability or dementia. Several factors were associated with poor outcomes.

Methods Design: Inception cohort followed for 10 years. Cambridgeshire Parkinson’s Incidence from GP to Neurologist (CamPaIGN) study. Setting: Cambridgeshire County, England, UK. Patients: 142 patients (mean age at diagnosis 70 y, 56% men, mean follow-up 7.2 y) who were newly diagnosed with parkinsonism or PD at baseline and had idiopathic PD (based on UK Parkinson’s Disease Society Brain Bank [UKPDSBB] criteria) confirmed at 3 to 4 years; and those who met UKPDSBB criteria at baseline but died or withdrew from the study before reappraisal of diagnoses at 3 to 4 years. Prognostic factors: Age, sex, history of smoking, Unified Parkinson’s Disease Rating Scale motor score, motor phenotype, depression, equivalent levodopa dose, level of comorbidity (number of other significant health conditions), verbal intelligence quotient, 6 neuropsychological variables, and 4 genotypes. Outcomes: Postural instability (Hoehn & Yahr stage 3: mildto-moderate bilateral disease, some postural instability, and physically independent), dementia (Mini-Mental State Examination score ≤ 24 out of 30 and meets Diagnostic and Statistical Manual of Mental Disorders IV criteria), mortality, and good outcome (survival without postural instability or dementia).

Main results At 10 years after diagnosis, cumulative probability was 68% for postural instability, 46% for dementia, and 55% for mortality; 23% of patients had a good outcome.* Factors associated with poor outcomes are in the Table. Factors associated with poor outcomes at 10 y in patients with Parkinson disease† Prognostic factors at baseline Increased age at diagnosis‡

Outcomes

Hazard ratio (95% CI)

Mortality

1.08 (1.05 to 1.12)

Postural instability

1.05 (1.02 to 1.08)

Dementia

1.08 (1.04 to 1.14)

Mortality

2.07 (1.12 to 3.56)

Nontremor-dominant phenotype

Postural instability

4.07 (2.28 to 7.25)

Greater comorbidity§

Postural instability

1.33 (1.11 to 1.60)

Semantic fluency||

Dementia

3.05 (1.39 to 6.67)

Impaired pentagon copying test

Dementia

2.55 (1.44 to 4.50)

UPDRS motor score ≥ 25¶

Dementia

3.18 (1.44 to 6.99)

MAPT H1/H1 genotype

Dementia

3.08 (1.40 to 6.79)

History of smoking

†MAPT H1/H1 = microtubule-associated protein tau H1/H1 genotype; UPDRS = Unified Parkinson’s Disease Rating Scale. CIs provided by author. ‡Hazard ratio per year of increased age. §Per additional comorbid condition. ||Naming < 20 animals in 90 seconds. ¶UPDRS motor subsection; possible score range 0 to 108, higher scores = more severe disease.

JC10

© 2013 American College of Physicians

*Based on Kaplan-Meier survival analysis.

Sources of funding: Medical Research Council; Parkinson’s UK; National Institutes of Health Research; Cure Parkinson’s Trust; Patrick Berthoud Trust; Van Geest Foundation; Wellcome Trust. For correspondence: Dr. C. Williams-Gray, University of Cambridge, Cambridge, England, UK. E-mail [email protected]

Commentary The 10-year study by Williams-Gray and colleagues of patients with incident PD mostly confirms other reports and general observations of a high incidence of dementia and a high risk for the postural instability gait dysfunction (PIGD) variant of the disease, but unlike other studies, found no reduction in life expectancy compared with the general population (1-3). These results are probably generalizable to the rest of the UK and the western world, but treatment paradigms and the ability to recognize PD, especially when tremor is lacking, undoubtedly vary around the world. The median age of onset of PD in the West is about 60 years; it is unclear if the community-based study population, with a mean age of 70 years at diagnosis, showed a worse prognosis than would be seen in younger populations. The study findings can be used to counsel newly diagnosed patients, informing them that 23% of patients had good outcomes at 10 years and mortality wasn’t increased compared with the general population. The study also supports the observation that the PIGD variant is less responsive to PD medication and that patients with this variant do worse in the long run (1, 3, 4). Practitioners might therefore consider using an aggressive approach to treat motor symptoms but reduce treatment when the response is not good. Most patients with PD die of the same causes as others their age, but they have an increased likelihood of dying from pneumonia (due to aspiration pneumonia and reduced ability to clear secretions due to weakened cough) and complications of falls (1-3). The study cohort is unique in that it represents all incident cases in 1 region of England, all of which were followed by neurologists. It confirms that although some patients do well over time, most develop dementia or postural instability, which seriously affects their quality of life. Joseph H. Friedman, MD Butler Hospital and Alpert Medical School of Brown University Providence, Rhode Island, USA References 1. Auyeung M, Tsoi TH, Mok V, et al. J Neurol Neurosurg Psychiatry. 2012; 83:607-11. 2. Hely MA, Morris JG, Reid WG, Trafficante R. Mov Disord. 2005;20:190-9. 3. Lethbridge L, Johnston GM, Turnbull G. Prog Palliat Care. 2013;21:140-5. 4. Jankovic J, McDermott M, Carter J, et al. Neurology. 1990;40:1529-34.

17 December 2013 | ACP Journal Club | Volume 159 • Number 12

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ACP Journal Club. At 10 years, 55% of patients with Parkinson disease died; 23% had a good outcome.

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