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Published on: September 12, 2020
Paraneoplastic movement disorders
Shyamal H Mehta1, John C Morgan, Kapil D Sethi
1Movement Disorders Program, Department of Neurology, Augusta, GA 30912, USA. shmehta@mcg.edu
Abstract:
Neurologic paraneoplastic syndromes (NPSs) result from damage to the nervous system due to the remote effects of cancer not related to metastasis, infection, or metabolic derangements. NPSs are rare, affecting 1 in 10,000 patients with cancer. Pathogenesis is likely related to the immune mechanisms: normal neural tissue is mistakenly attacked due to the similarity in the onconeural antigens expressed by the tumor cells. Among the various "classic" and other NPSs, this review focuses on paraneoplastic movement disorders, including ataxia due to cerebellar degeneration, stiff-person syndrome, opsoclonus-myoclonus syndrome, chorea, parkinsonism, and tremor. The recently described syndrome of paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis is also included, given that these patients have complex movements such as stereotypies and dyskinesias in addition to psychiatric symptoms, altered sensorium, and other neurologic signs. Although variable, treatment and prognosis of NPSs rely heavily on treatment of the underlying malignancy and immunotherapy.
Insights
Neurologic paraneoplastic syndromes (NPSs) are rare immune-mediated disorders triggered by cancer. This review focuses on paraneoplastic movement disorders and their link to underlying malignancy and immunotherapy.
Area of Science:
- Neurology
- Oncology
- Immunology
Background:
- Neurologic paraneoplastic syndromes (NPSs) arise from the nervous system's remote reaction to cancer, distinct from metastasis or infection.
- These rare conditions affect approximately 1 in 10,000 cancer patients, driven by immune responses mistaking neural tissue for tumor antigens.
Purpose of the Study:
- To review paraneoplastic movement disorders, including ataxia, stiff-person syndrome, opsoclonus-myoclonus syndrome, chorea, parkinsonism, and tremor.
- To discuss the recently identified paraneoplastic anti-N-methyl-D-aspartate receptor encephalitis, characterized by complex movements and neurological signs.
Main Methods:
- Literature review focusing on classic and emerging NPSs.
- Analysis of pathogenesis, clinical manifestations, and treatment strategies.
Main Results:
- NPSs involve immune-mediated damage to neural tissue due to shared onconeural antigens.
- Paraneoplastic movement disorders encompass a range of conditions, including cerebellar degeneration, stiff-person syndrome, and encephalitis.
Conclusions:
- Treatment and prognosis of NPSs are significantly influenced by the management of the primary cancer.
- Immunotherapy plays a crucial role in managing these complex neurological disorders.
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