Objective: we describe 2 patients with paraneoplastic parkinsonism mimicking a PSP.

Background: Paraneoplastic neurological syndromes (PNS) are broad-spectrum disorders that can affect any part of the nervous system. Parkinsonism has been recently reported in association with various onconeural antibodies and tumors. However, PNS presenting with a progressive supranuclear palsy (PSP) phenotype is rare.

Method: a case series

Results: We report 2 men (P1 and P2) aged respectively of 74 and 58 years with no medical history. Both patients were admitted for one year onset of progressive gait disorder and cognitive impairment. On examination, P1 had bilateral tremo-akineto-rigid parkinsonism, gait apraxia, frontal syndrome with severe dysarthria, dysphonia and dysphagia, and peripheral neuropathy. Whereas, P2 disclosed bilateral severe parkinsonism, axial rigidity, and behavioral disorders with a sub-cortical amnestic profile. Their parkinsonism was L-Dopa resistant. Brain MRI showed a vascular leukopathy with cerebral atrophy and cerebrospinal fluid (CSF) analysis was normal. They were initially diagnosed with PSP, though PNS was still suspected. In P1, the investigation confirmed the presence of anti-CV2 antibodies (+++) with a normal Chest-Abdomen-Pelvis (CAP) CT scan, PET scan and protein electrophoresis. However, P2 had negative anti-onconeural antibodies but the CAP CT scan showed bilateral gynecomastia without a tumor. Eventually, the testicular echography revealed a right testicle tumor and the diagnosis of seminoma was confirmed through biopsy. Both patients were treated with high-dose corticosteroids and intravenous immunoglobulin with little improvement.

Conclusion: These cases illustrated that PNS should be considered when diagnosing a patient with PSP phenotype, especially patients with atypical clinical features, imaging findings and a rapid disease course.

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MDS Abstracts - https://www.mdsabstracts.org/abstract/paraneoplastic-neurological-syndrome-mimicking-a-progressive-supranuclear-palsy-a-case-series/