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Paraneoplastic neurological syndromes 

Paraneoplastic neurological syndromes

Paraneoplastic neurological syndromes

Jeremy Rees

and Angela Vincent



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.
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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.

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