Show Summary Details
Page of

Paraneoplastic neurological syndromes 

Paraneoplastic neurological syndromes

Chapter:
Paraneoplastic neurological syndromes
Author(s):

Jeremy Rees

and Angela Vincent

DOI:
10.1093/med/9780199204854.003.2421_update_001

Update:

Recent incidence data.

Onconeural antibody associated disorders and NMDAR antibody encephalitis.

Further reading updated.

Other minor changes.

A relevant case history from Neurological Case Histories: Case Histories in Acute Neurology and the Neurology of General Medicine has been added to this chapter.

Updated on 28 Nov 2012. The previous version of this content can be found here.
Page of

PRINTED FROM OXFORD MEDICINE ONLINE (www.oxfordmedicine.com). © Oxford University Press, 2015. All Rights Reserved. Under the terms of the licence agreement, an individual user may print out a PDF of a single chapter of a title in Oxford Medicine Online for personal use (for details see Privacy Policy).

date: 30 March 2017

Paraneoplastic neurological syndromes are disorders caused by the presence of an underlying tumour, but not due to either direct or metastatic invasion, or to recognized metabolic or endocrine complications. They are thought to arise from an autoimmune response to onconeural tumour antigens which are also expressed by cells of the central or peripheral nervous system.

Paraneoplastic syndromes are rare but important because (1) they often develop before the cancer has been identified, (2) serological testing for specific anti-neuronal (onconeural) antibodies may identify a neurological disorder as paraneoplastic and the results may suggest the location of the underlying tumour and/or predicts its prognosis. In some cases, the identity of the antibody predicts an immunotherapy-responsive disease.

Epidemiology—the most common tumours associated with paraneoplastic syndromes are lung (both small-cell lung cancer (SCLC) and non-SCLC), ovary, breast, thymus, lymph nodes (Hodgkin’s disease and non-Hodgkin’s lymphoma) and testis.

Treatment—a few paraneoplastic syndromes respond to immunosuppression or to treatment of the underlying cancer, particularly when they are associated with antibodies to neuronal cell-surface proteins and germ cell tumours, but treatment is unrewarding for most and the patients remain with stable but often severe neurological disability even if the cancer is cured.

Specific syndromes

A 32 yr old man presenting with drowsiness and confusion.

Brain and nerves—(1) cerebellar degeneration—most common with lung cancer (especially SCLC), breast and gynaecological cancer, and Hodgkin’s disease; (2) opsoclonus/myoclonus; (3) limbic encephalitis (see Chapter 24.22); (4) brainstem encephalitis; (5) cancer-associated retinopathy.

Spinal cord, dorsal root ganglia and peripheral nerves—(1) necrotizing myelopathy; (2) motor neurone disease (some cases); (3) myelitis; (4) sensory neuronopathy; (5) peripheral neuropathies.

Neuromuscular junction and muscle (see also Chapter 24.23)—(1) Lambert–Eaton myasthenic syndrome—typically associated with SCLC; (2) myasthenia gravis—occurs in 30% of patients with thymomas; (3) polymyositis/dermatomyositis; (4) neuromyotonia.

Access to the complete content on Oxford Medicine Online requires a subscription or purchase. Public users are able to search the site and view the abstracts for each book and chapter without a subscription.

Please subscribe or login to access full text content.

If you have purchased a print title that contains an access token, please see the token for information about how to register your code.

For questions on access or troubleshooting, please check our FAQs, and if you can''t find the answer there, please contact us.