Paraneoplastic syndrome in neuroophthalmology

J Neurol. 2022 Oct;269(10):5272-5282. doi: 10.1007/s00415-022-11247-z. Epub 2022 Jul 2.


Paraneoplastic syndrome is a group of clinical symptoms that occur in the state of systemic malignant tumors. Paraneoplastic syndrome of the nervous system can affect any part of the central and peripheral nervous system and may also affect the eyes. In neuroophthalmology, paraneoplastic syndrome has a variety of manifestations that can affect both the afferent and efferent visual systems. The afferent system may involve the optic nerve, retina and uvea; the efferent system may involve eye movement, neuromuscular joints or involuntary eye movements and pupil abnormalities and may also have other neurological symptoms outside the visual system. This article discusses the clinical manifestations, pathological mechanisms, detection methods and treatment methods of paraneoplastic syndrome in neuroophthalmology. The performance of paraneoplastic syndrome is diverse, the diagnosis is difficult, and the treatment should be considered systematically. Differential diagnosis, optimal evaluation and management of these manifestations is not only the key to treatment but also a challenge.

Keywords: Cancer-associated retinopathy; Lambert-Eaton myasthenic syndrome; Melanoma-associated retinopathy; Neuroophthalmology; Opsoclonus-myoclonus syndrome; Paraneoplastic syndrome.

Publication types

  • Review

MeSH terms

  • Humans
  • Immunotherapy
  • Lambert-Eaton Myasthenic Syndrome* / pathology
  • Neoplasms*
  • Paraneoplastic Syndromes* / complications
  • Paraneoplastic Syndromes* / diagnosis