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July 27, 2020

Time for a New Image of Parkinson Disease

Author Affiliations
  • 1McKnight Brain Institute, Department of Neurology, University of Florida College of Medicine, Gainesville
  • 2Norman Fixel Institute for Neurologic Diseases, University of Florida, Gainesville
JAMA Neurol. Published online July 27, 2020. doi:10.1001/jamaneurol.2020.2412

Parkinson disease is now the fastest growing neurological disorder globally.1 An estimated 6.1 million individuals worldwide had a Parkinson disease diagnosis in 2016, 2.4-fold higher than in 1990.1 The most common representation of Parkinson disease continues to be the 1886 sketch by Sir William Richard Gowers, MD, published in his book A Manual of Diseases of the Nervous System (Figure, A).2 Other Parkinson disease images remain largely based on Gowers’ famous sketch: older white men who are frail, hunched forward, and shaking.

Figure.  Images of Parkinson Disease: 1886 and 2020
Images of Parkinson Disease: 1886 and 2020

A, Illustration of Parkinson disease by Sir William Richard Gowers, MD, published in his book A Manual of Diseases of the Nervous System.2 B, Individuals with mild motor-predominant Parkinson disease often present at a young age (ie, in their 50s to 60s). They have mild motor symptoms and few nonmotor symptoms, slow progression, and a good response to medications. Because of this, others may not be able to tell that the person has Parkinson disease other than the presence of tremor, mildly decreased facial expression, and sometimes foot dystonia (dotted circle). C, Individuals with the intermediate Parkinson disease phenotype have more prominent movement symptoms, including decreased facial expression, stiffness, slowness, and tremor, often with a modest response to medication. While the symptoms are more obvious than in the mild motor-predominant form, these individuals can still work and lead active lives. With disease progression, individuals may experience times when the medications successfully treat motor symptoms (on-time), but they experience levodopa-induced dyskinesias. At the end of the dose, these individuals may experience off-time where the motor symptoms return. These individuals’ disease progresses over time to more advanced disease, but they usually live many years before substantial disability. D, Individuals with the diffuse malignant Parkinson disease phenotype—and individuals with advanced Parkinson disease of any phenotype—have prominent motor and nonmotor features. They typically have marked rigidity and slowness, absent facial expression (often with lips parted most of the time), stooped posture, flexion of the arms and legs, and impaired gait with short steps and freezing. They may or may not have tremor. Many need an assistance device (eg, walker) for gait. They also have nonmotor features at onset, including rapid eye movement sleep behavior disorder, mild cognitive impairment, and orthostatic hypotension.

This commonly used image fails to accurately reflect a contemporary view of Parkinson disease and the heterogeneity in age at onset, sex, race/ethnicity, and disease experience. The incidence of Parkinson disease is highest in individuals aged 70 to 79 years,3 and the prevalence peaks between ages 85 and 89 years.1 However, these groups are considered to be late-onset Parkinson disease (70 years or older).4 In one large study,4 late-onset Parkinson disease accounted for only 39% of the people with Parkinson disease compared with 51% for middle-onset Parkinson disease (aged 50 to 69 years) and 10% for young-onset Parkinson disease (younger than 50 years).

Additionally, the male predominance of Parkinson disease, while well supported, may be overemphasized. Global analyses have shown a male to female ratio of 1.4.1 In a separate analysis, the incidence of Parkinson disease was statistically higher in men only in those aged 60 to 69 years and 70 to 79 years.3 Overall, the incidence was 37.16 per 100 000 person-years for women and 44.21 per 100 000 person-years for men,3 demonstrating that Parkinson disease is common in both sexes.

In the US, Parkinson disease is more frequently diagnosed in white non-Hispanic populations. Using 1995 and 2000 to 2005 Medicare data, the incidence ratio of Parkinson disease among black vs white individuals was 0.74 (95% CI, 0.732–0.748). The prevalence ratio was 0.58 (95% CI, 0.575–0.581).5 While Parkinson disease was less commonly diagnosed in black populations, the mean (SD) prevalence was still 1036.41 (86.01) per 100 000 Medicare recipients in this group.5 An accurate view of Parkinson disease must include individuals from different backgrounds.

The frailness and disability shown in Gowers’ 1886 picture is also not an accurate rendering of the modern experience of people with Parkinson disease. Recent subtyping identified the disabling diffuse malignant form of Parkinson disease in only 16% of cases.6 A mild motor-predominant Parkinson disease phenotype was the most common presentation (49%), followed by the intermediate form (35%).6 While all phenotypes are progressive, the mean (SD) time from diagnosis to first milestone (regular falls, wheelchair dependence, dementia, or residential/nursing home placement) was 14.3 (5.7) years for the mild motor-predominant form, 8.2 (5.3) years for the intermediate form, and 3.5 (3.2) years for the diffuse malignant form. Mean (SD) survival after diagnosis was 20.2 (7.8) years for the mild motor-predominant form, 13.2 (6.7) years for the intermediate form, and 8.1 (5.4) years for the diffuse malignant form.6 This suggests that people with Parkinson disease are living for many years without the profound disability implied by Gowers’ sketch. Current approaches to Parkinson disease subtyping have limitations, and consensus on optimal categorization is lacking. However, it is clear that the experiences of individuals with Parkinson disease are varied and include mild and slow disease progression.

Does it matter? Almost certainly. Images are an increasingly important part of medical teaching. However, medical textbooks continue to have biases relating to age, sex, and race/ethnicity. These biases result in inadequate and unrealistic information.7 For example, based on existing research, it is possible that images emphasizing the male predominance of Parkinson disease contribute to delays in women with Parkinson disease receiving specialty care. It is similarly possible that the classic picture of advanced Parkinson disease leads to lack of recognition of early Parkinson disease symptoms, especially within primary care settings.

Images such as Gowers’ figure also contribute to public assumptions that Parkinson disease is an illness of old, frail individuals. This adds to the stigma reported by people with Parkinson disease, and stigma is a significant contributor to the quality of life of people with Parkinson disease.8 Research outside Parkinson disease also suggests that expectations can be a self-fulfilling prophecy: negative perceptions of aging at baseline were associated with worse gait speed, decrements in verbal fluency, and impaired self-rated memory after 2 years of follow-up.

No single image can encapsulate the range of motor and nonmotor symptoms experienced by individuals with Parkinson disease, and there is no one common path. However, it is time that our medical images reflect modern people with Parkinson disease (Figure, B-D): young and old; male and female; active and debilitated; working, retired, or disabled; and with various symptoms and circumstances. While no single image can reflect the diversity of backgrounds, phenotypes, and experiences in Parkinson disease, it is important that our images are consistent with the advances in Parkinson disease that have occurred in the more than 130 years since Gowers’ sketch. Improving the image to include a broad diversity of people with Parkinson disease can help enhance Parkinson disease recognition and enforce the reality that modern people with Parkinson can have meaningful lives that are not universally limited by disease-related disability.

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Article Information

Corresponding Author: Melissa J. Armstrong, MD, MSc, McKnight Brain Institute, Department of Neurology, University of Florida College of Medicine, PO Box 100236, Gainesville, FL 32610 (melissa.armstrong@neurology.ufl.edu).

Published Online: July 27, 2020. doi:10.1001/jamaneurol.2020.2412

Conflict of Interest Disclosures: Dr Armstrong has received consulting fees from the American Academy of Neurology; grants from the Lewy Body Dementia Association, the Michael J. Fox Foundation, and the Agency of Healthcare Research and Quality; and royalties from Oxford University Press. Dr Okun has received grants from the National Institutes of Health, the Michael J. Fox Foundation, the Tourette Association of America, and the Parkinson's Foundation; royalties from Demos Medical Publishing, Manson Publishing, Amazon, Smashwords, Books4Patients, Perseus, Robert Rose, Oxford University Press, and Cambridge University Press for books on movement disorders; serves as the medical director for the Parkinson's Foundation; serves as Associate Editor for JAMA Neurology and Associate Editor for the New England Journal of Medicine JournalWatch Neurology; and participates in continuing medical education and educational activities on movement disorders sponsored by the Academy for Healthcare Learning, PeerView, Prime, QuantiaMD, WebMD/Medscape, Medicus, MedNet, Einstein, MedNet, Henry Stewart, American Academy of Neurology, Movement Disorders Society, and Vanderbilt University.

Disclaimer: Dr Okun is Associate Editor for JAMA Neurology, but he was not involved in any of the decisions regarding review of the manuscript or its acceptance.

Additional Contributions: We thank Erica Rodriguez, AA (freelance illustrator), for creating the illustration. She was compensated for her work.

References
1.
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Gowers  WR.  A Manual of Diseases of the Nervous System. J. & A. Churchill; 1886.
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Hirsch  L, Jette  N, Frolkis  A, Steeves  T, Pringsheim  T.  The incidence of Parkinson’s disease: a systematic review and meta-analysis.   Neuroepidemiology. 2016;46(4):292-300. doi:10.1159/000445751PubMedGoogle ScholarCrossref
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Mehanna  R, Moore  S, Hou  JG, Sarwar  AI, Lai  EC.  Comparing clinical features of young onset, middle onset and late onset Parkinson’s disease.   Parkinsonism Relat Disord. 2014;20(5):530-534. doi:10.1016/j.parkreldis.2014.02.013PubMedGoogle ScholarCrossref
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Wright Willis  A, Evanoff  BA, Lian  M, Criswell  SR, Racette  BA.  Geographic and ethnic variation in Parkinson disease: a population-based study of US Medicare beneficiaries.   Neuroepidemiology. 2010;34(3):143-151. doi:10.1159/000275491PubMedGoogle ScholarCrossref
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De Pablo-Fernández  E, Lees  AJ, Holton  JL, Warner  TT.  Prognosis and neuropathologic correlation of clinical subtypes of Parkinson disease.   JAMA Neurol. 2019;76(4):470-479. doi:10.1001/jamaneurol.2018.4377PubMedGoogle ScholarCrossref
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Parker  R, Larkin  T, Cockburn  J.  A visual analysis of gender bias in contemporary anatomy textbooks.   Soc Sci Med. 2017;180:106-113. doi:10.1016/j.socscimed.2017.03.032PubMedGoogle ScholarCrossref
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Maffoni  M, Giardini  A, Pierobon  A, Ferrazzoli  D, Frazzitta  G.  Stigma experienced by Parkinson’s disease patients: a descriptive review of qualitative studies.   Parkinsons Dis. 2017;2017:7203259. doi:10.1155/2017/7203259PubMedGoogle Scholar
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    1 Comment for this article
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    Thanks for the Image Update- I can see myself
    David Blacker, MB BS, FRACP | Perron Institute for Neurological and Translational Science
    Thanks to Armstrong and Okun for this compact, informative and timely article, co-inciding with the week of the World Federation of Neurology World Brain Day (July 22nd), which this year highlights Parkinson Disease. What we say as doctors to patients is highly impactful; words need to be chosen carefully and perceptions of illness count. I suspect that mental perception of PD may even influence progression; if a negative, nihilistic image is formed around the time of diagnosis, the self-fulfilling prophecy concept, combined with apathy, could contribute to lack of engagement in physical therapy and mobility, which I suspect accelerates progression. />
    I have recently publicised my experience as a neurologist living and working with PD (1), and I immediately related to the image of the runner with the dystonic foot; I thank the authors for giving me a more positive picture than the 1886 version of PD.

    The downside of the article is the statistics; even for my mild version, seeing the median time from diagnosis to first milestone as 14 years and death as 20 years is sobering. As a physician I know about the vagaries of clinical data and the meaning of standard deviations, but as a patient, there is a gnawing fear; does that mean I'm not going to live beyond 60? The long lead-in phase with subtle non-motor symptoms makes it difficult to know when "the clock starts".

    My message is that whilst images and words are impactful and important, maybe numbers are even more frightening,

    REFERENCE

    (1) A neurologist with Parkinson's disease. Practical Neurology 2020, Blacker D.
    CONFLICT OF INTEREST: None Reported
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