Objective: To report a case of multiple system atrophy (MSA) and progressive supranuclear palsy (PSP) in a couple.

Background: Conjugal parkinsonism denotes an entity where PD or parkinsonism^1,2,3 occurs in married couples. Some have suggested that past exposure to pesticides play a significant aetiological role⁴, while others have argued that this entity is just a chance occurrence⁵. I had the privilege to care for a couple who had conjugal parkinsonism and would like to share their stories.

Method: Husband Mr Chee had advanced MSA when I took over his care. He was afflicted by repeated aspiration pneumonitis and passed away at 81 years old. Wife Madam Chan had initially presented with recurrent falls. Within a year, she had limited vertical gaze and MRI of her brain showed midbrain atrophy consistent with PSP. By the sixth-year post diagnosis, she had become dependent on her carer. She passed away at 81 years old. The couples were from Malaysia. They were not blood related, had no family history of parkinsonism, non-smokers and teetotalers. After marriage, they moved to Bahau, Malaysia and resided in a palm oil plantation for 9 years. There, Mr Chee worked as a bookkeeper and spent significant time hunting for wild animals. The couple moved out of the plantation and to Singapore in 1969. There was no evidence of further exposure to environmental hazards thereafter.

Results: MSA and PSP have low incidence rates at 0.6 – 3⁷ and 0.16 – 2.6 per 100000 person-years⁸ respectively. The probability of these 2 syndromes occurring in a married couple by chance is thus very low. A shared external trigger as contributory factor is a possibility. The time spent in the plantation in Bahau was likely the key period. Pesticides were common in plantations in Malaysia during this period⁹. Among these were paraquat and DDT. Both are neurotoxic and have been associated with an increased risk of PD^10,11. The association with the risk of MSA and PSP is however less clear^12,13. Despite this, the possibility of pesticides as the common denominator in this couple’s case cannot be completely ruled out.

Conclusion: To my knowledge, the presence of MSA and PSP in a married couple has not been reported in the literature. Barring the lack of definitive link to exposure to past external chemicals, the history of occupational and environmental exposure in couples with parkinsonism remains important. As to whether conjugal parkinsonism is a real clinical syndrome or chance occurrence, the debate continues.

References: 1. Miwa H, Kondo T. Conjugal parkinsonism: multiple system atrophy and Parkinson’s disease. Parkinsonism Relat Disord. 2010 Mar;16(3):232.

2. Ramani M, Saur DP, Rabin M, Kurlan R. Conjugal and familial Lewy body disorders: a report of one family. Parkinsonism Relat Disord. 2013 Apr;19(4):498.

3. Coon EA, Rocca W, Melson CS, Ahlskog JE, Matsumoto JY, Low PA, Singer W. Conjugal multiple system atrophy: Chance, shared risk factors, or evidence of transmissibility? Parkinsonism Relat Disord. 2019 Oct;67:10-13.

4. Willis AW, Sterling C, Racette BA. Conjugal Parkinsonism and Parkinson disease: a case series with environmental risk factor analysis. Parkinsonism Relat Disord. 2010 Mar;16(3):163-6.

5. Rajput AH, Ferguson LW, Robinson CA, Guella I, Farrer MJ, Rajput A. Conjugal parkinsonism is coincidental. Parkinsonism Relat Disord. 2016 Dec;33:149-150.

6. Adler CH, Halverson M, Zhang N, Shill HA, Driver-Dunckley E, Mehta SH, Atri A, Caviness JN, Serrano GE, Shprecher DR, Belden CM, Sabbagh MN, Long K, Beach TG. Conjugal Synucleinopathies: A Clinicopathologic Study. Mov Disord. 2024 Jul;39(7):1212-1217.

7. Poewe W, Stankovic I, Halliday G, Meissner WG, Wenning GK, Pellecchia MT, Seppi K, Palma JA, Kaufmann H. Multiple system atrophy. Nat Rev Dis Primers. 2022 Aug 25;8(1):56.

8. Lyons S, Trépel D, Lynch T, Walsh R, O’Dowd S. The prevalence and incidence of progressive supranuclear palsy and corticobasal syndrome: a systematic review and meta-analysis. J Neurol. 2023 Sep;270(9):4451-4465.

9. Ibrahim M.S. (2007). Persistent Organic Pollutants in Malaysia. An Li, Shinsuke Tanabe, Guibin Jiang, John P. Giesy, Paul K.S. Lam (Eds.), Developments in Environmental Science (pp. 629-655). Elsevier.

10. Spivey A. Rotenone and paraquat linked to Parkinson’s disease: human exposure study supports years of animal studies. Environ Health Perspect. 2011 Jun;119(6) :A259.

11. Rossi M, Scarselli M, Fasciani I, Maggio R, Giorgi F. Dichlorodiphenyltrichloroethane (DDT) induced extracellular vesicle formation: a potential role in organochlorine increased risk of Parkinson’s disease. Acta Neurobiol Exp (Wars). 2017;77(2):113-117.

12. Nee LE, Gomez MR, Dambrosia J, Bale S, Eldridge R, Polinsky RJ. Environmental-occupational risk factors and familial associations in multiple system atrophy: a preliminary investigation. Clin Auton Res. 1991 Mar;1(1):9-13.

13. Litvan I, Lees PS, Cunningham CR, Rai SN, Cambon AC, Standaert DG, Marras C, Juncos J, Riley D, Reich S, Hall D, Kluger B, Bordelon Y, Shprecher DR; for ENGENE-PSP. Environmental and occupational risk factors for progressive supranuclear palsy: Case-control study. Mov Disord. 2016 May;31(5) :644-52.

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