Overview of treatment strategies in paraneoplastic neurological syndromes

Handb Clin Neurol. 2024:200:97-112. doi: 10.1016/B978-0-12-823912-4.00015-3.

Abstract

Treatment strategies in paraneoplastic neurological syndromes rely on the three pillars of tumor treatment, immunotherapy, and symptomatic treatment, the first one being by far the most important in the majority of patients and syndromes. Classically, antibodies against extracellular antigens are directly pathogenic, and patients with these syndromes are more responsive to immunomodulatory or immunosuppressive treatments than the ones with antibodies against intracellular targets. This chapter first discusses some general principles of tumor treatment and immunotherapy, followed by a closer look at specific treatment options for different clinical syndromes, focusing on symptomatic treatments.

Keywords: Cerebellar degeneration; Cyclophosphamide; Encephalitis; Immunotherapy; Intravenous immunoglobulins; Lambert-Eaton myasthenic syndrome; Neuronopathy; Opsoclonus-myoclonus; Paraneoplastic neurological syndrome; Plasma exchange; Rituximab; Stiff-person syndrome; Tumor treatment.

Publication types

  • Review

MeSH terms

  • Autoantibodies
  • Humans
  • Immunosuppressive Agents / therapeutic use
  • Immunotherapy
  • Lambert-Eaton Myasthenic Syndrome* / drug therapy
  • Neoplasms*
  • Paraneoplastic Syndromes* / drug therapy

Substances

  • Immunosuppressive Agents
  • Autoantibodies