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Oxford Textbook of Medicine (5 ed.)

Edited by David A. Warrell, Timothy M. Cox, John D. Firth

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Latest update

The November 2012 update sees updates to over 70 chapters, focusing on Neurology and Gastroenterology. This update also incorporates a selection of 29 Case Histories taken from related titles in the Oxford Case Histories series, linked to from related chapters. Each case includes several questions followed by detailed answers and discussion to enhance diagnostic and clinical understanding.

Neurology updates include substantial updates to key chapters and new material on a wide range of topics including spinal cord injury, autonomic nervous system disorders, and inherited neurodegenerative diseases. 

Gastroenterology updates include extensive revisions of key chapters on liver failure and acute pancreatitis and new material on a wide range of matters, ranging from the common to the rare: including surgical treatments for colonic diverticular disease, antibody tests for immune disorders, and a revised treatment algorithm for small bowel bacterial overgrowth.

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Publisher:
Oxford University Press
Print Publication Date:
May 2010
Print ISBN-13:
9780199204854
Published to Oxford Medicine:
May 2012
DOI:
10.1093/med/9780199204854.001.1

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Disclaimer

Oxford University Press makes no representation, express or implied, that the drug dosages in this book are correct. Readers must therefore always check the product information and clinical procedures with the most up to date published product information and data sheets provided by the manufacturers and the most recent codes of conduct and safety regulations. The authors and the publishers do not accept responsibility or legal liability for any errors in the text or for the misuse or misapplication of material in this work. Except where otherwise stated, drug dosages and recommendations are for the non-pregnant adult who is not breastfeeding.

Contents

Paraneoplastic neurological syndromes

Chapter:
Paraneoplastic neurological syndromes
Author(s):

Jeremy Rees,

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 November 2012. The previous version of this content can be found here.

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

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.

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