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|Year : 2020 | Volume
| Issue : 2 | Page : 481-482
Parkinsonism with Newly Diagnosed Flare-up Rheumatoid Arthritis Mimicking Progressive Supranuclear Palsy
Kuo-Wei Lee1, Ching-Fang Chien1, Meng-Ni Wu2, Chiou-Lian Lai2, Li-Min Liou2
1 Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University, Kaohsiung, Taiwan
2 Department of Neurology, Kaohsiung Medical University Hospital, Kaohsiung Medical University; Department of Neurology, School of Medicine, College of Medicine, Kaohsiung Medical University, Kaohsiung, Taiwan
|Date of Web Publication||15-May-2020|
Kaohsiung Municipal Siaogang Hospital No.482, Shanming Rd., Siaogang Dist., Kaohsiung City 812
Source of Support: None, Conflict of Interest: None
In order to make a correct diagnosis of idiopathic Parkinson's disease (PD), it is essential to exclude atypical parkinsonian features, such as early dementia, fall, and autonomic dysfunction. Rheumatoid arthritis (RA), which is a systemic inflammatory disorder, although most patients present in a polyarticular manner. Still some may also present with extra-articular involvement including skin, lung, heart, and the central or peripheral nervous systems. A possible pathogenetic link between RA and PD are proposed. However, the coexistence of RA and progressive supranuclear palsy (PSP) is rarely reported. Here, we report a parkinsonian patient with a newly diagnosed flare-up RA presenting with early falls, postural instability and supra-nuclear gaze palsy, which suggestive of clinically probable PSP. Furthermore, the parkinsonian features respond to anti-rheumatic agents, but not levodopa. Finally, the patient looks like a clinical possible PD. In summary, Parkinsonian patient with newly diagnosed flare-up RA can present with clinically probable PSP. Unbearably painful limb contracture is a clue of the coexistence of RA. Both typical and atypical parkinsonian features respond dramatically to anti-rheumatic medication, but not levodopa.
Keywords: Progressive supranuclear palsy, parkinsonism, rheumatoid arthritisKey Message: Parkinsonian patient with newly diagnosed flare-up RA can present with clinically probable PSP. Unbearably painful limb contracture is a clue of the coexistence of RA.
|How to cite this article:|
Lee KW, Chien CF, Wu MN, Lai CL, Liou LM. Parkinsonism with Newly Diagnosed Flare-up Rheumatoid Arthritis Mimicking Progressive Supranuclear Palsy. Neurol India 2020;68:481-2
|How to cite this URL:|
Lee KW, Chien CF, Wu MN, Lai CL, Liou LM. Parkinsonism with Newly Diagnosed Flare-up Rheumatoid Arthritis Mimicking Progressive Supranuclear Palsy. Neurol India [serial online] 2020 [cited 2022 May 26];68:481-2. Available from: https://www.neurologyindia.com/text.asp?2020/68/2/481/284382
To accurately diagnose idiopathic Parkinson's disease (PD), atypical parkinsonian features, such as early dementia, frequent falls, autonomic dysfunction, and pattern of dysarthria, must be excluded.,, Although most patients present with rheumatoid arthritis (RA), which is a systemic inflammatory disorder, as polyarticular arthritis, some may also experience extra-articular manifestations, which involves the central nervous system. The possible pathogenetic link between RA and PD has been proposed. However, the occurrence of both concurrent RA and progressive supranuclear palsy (PSP) is rare. Here, we report a case of a 59-year-old parkinsonian patient with a newly diagnosed flare-up RA presenting with unbearably painful arthralgia, early falls, postural instability, and supranuclear gaze palsy, which mimics clinically probable PSP. Furthermore, the parkinsonian features responded to antirheumatic agents, but not to levodopa.
| » Case Report|| |
The patient developed right-sided parkinsonism 1 year ago, which progressed to bilaterally asymmetric symptoms within six months. Moreover, he experienced falling within the first six months. Neurological examinations were conducted in clinics, and the results showed predominant right-sided parkinsonism (bradykinesia, cogwheel rigidity, resting, and postural tremor), supranuclear-type upward gaze palsy with a slow velocity of vertical saccades, and postural instability. The patient's unified PD rating scale part III (UPDRS III) score was 74. Furthermore, physical examination revealed arthralgia and contractures in the bilateral finger and wrist joint with localized heat and tenderness. Brain magnetic resonance imaging result was unremarkable. Technetium-99m Labeled Tropane Derivative (Tc99 m TRODAT) brain scan showed decreased dopamine transporter radioactivity in the bilateral putamen and caudate nuclei. The ratio of C-P/O [contralateral- (putamen counts–occipital cortex counts)/occipital cortex counts)], I-P/O (ipsilateral), C-C/O [contralateral- (caudate counts–occipital cortex counts)/occipital cortex counts)], and I-C/O (ipsilateral) were 0.51, 0.53, 0.77, and 0.62, respectively. Orthostatic hypotension was not noted during the upright test. Based on these findings, clinical probable PSP was suspected. Levodopa was prescribed and titrated up to 600 mg per day within one year for parkinsonian symptoms. However, the patient's response was poor (UPDRS III score of 74). The patient was diagnosed with RA based on the following laboratory test results: Elevated erythrocyte sedimentation rate at 85 mm/h, rheumatoid factor level at 627 IU/mL, and anticyclic citrullinated peptide level >340 Eli AU/mL. Moreover, radiological examination of the hands was performed. Subsequently, a rheumatologist confirmed the diagnosis of RA and prescribed antirheumatic medications (prednisolone, sulfasalazine, and methotrexate). Not only arthralgia but also the typical and atypical parkinsonian features, such as tremor, bradykinesia, rigidity, supranuclear gaze palsy, and postural instability (a decrease in UPDRS III score from 74 to 32) improved substantially. In addition, the patient's RA titer level decreased to 107 IU/mL 2 weeks after treatment. Finally, the patient was diagnosed with PD without atypical features.
| » Discussion|| |
A pathogenetic link between PD and RA may exist. Both high concentrations of inflammatory mediators over a long time and high sensitivity of dopaminergic neurons to inflammatory mediators in the substantia nigra leading to an increased risk of PD. Although 25% of patients with RA have extrapyramidal symptoms, only 2.3% are concurrently diagnosed with PD. In fact, most extrapyramidal symptoms of systemic rheumatic disease, such as RA, are not typical features of PD. Collectively, these data explain why the symptoms of our patient appear to mimic clinical probable PSP, but not PD.
The occurrence of painful limb contractures, which are significant symptoms in this case study, is extremely rare in parkinsonian patients. Although limb contractures in levodopa-responsive parkinsonism are commonly attributed to PD, the use of ergot derivatives (such as bromocriptine), possibility of parkinsonism plus syndromes and, putaminal infarction should be considered for differential diagnosis. However, these conditions are not painful.
Although currently unknown, a co-pathogenetic link may exist between PSP and RA, such as that between PD and RA. In the previous reports, interleukin (IL)-1, IL-6, IL-1β, and tumor necrosis factor α play an important role in the pathophysiology of RA, and their expression levels are higher than normal in the subthalamus and substantia nigra of the PSP brain. Furthermore, IL-1β significantly interacts with microglia, thereby indicating its significant correlation to the tau burden in the extrapyramidal system. Thus, the neuroinflammation process in the subthalamus and substantia nigra caused the symptoms of PSP in our case study. These results may explain why the antirheumatic medication, but not levodopa, reversed the parkinsonian symptoms in our case.
| » Conclusion|| |
The parkinsonian patient presented with newly diagnosed flare-up RA that mimicked clinically probable PSP. Painful limb contractures are an indication of the coexistence of RA and PSP. Both typical and atypical parkinsonian features substantially responded to antirheumatic medication, but not to levodopa.
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Conflicts of interest
There are no conflicts of interest.
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