Journal
CURRENT OPINION IN NEUROLOGY
Volume 25, Issue 6, Pages 795-801Publisher
LIPPINCOTT WILLIAMS & WILKINS
DOI: 10.1097/WCO.0b013e328359da15
Keywords
antibodies; cancer; cerebellar degeneration; Lambert-Eaton myasthenic syndrome; limbic encephalitis; paraneoplastic
Categories
Funding
- Fondo Investigaciones Sanitarias [FIS PS09/0193]
- National Institutes of Health [RO1NS077851]
- National Cancer Institute [RO1CA089054]
- Fundacio la Marato [TV3, FIS PI11/01780]
- Euroimmun
- ICREA Funding Source: Custom
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Purpose of review This review describes relevant advances in paraneoplastic neurological syndromes (PNS) with emphasis on particular syndromes and the impact of antibodies against surface antigens in their management. Recent findings PNS may present with symptoms that do not raise the suspicion of a paraneoplastic origin. The best example is anti-N-methyl-D-aspartate receptor encephalitis that in adult women frequently associates with ovarian teratoma. An electroencephalogram pattern described as 'extreme delta brush' was recently identified in 30% of patients with this disorder. Isolated myelopathy may have a paraneoplastic origin associated with amphiphysin or CV2 (CRMP5) antibodies. Jaw dystonia and laryngospasm can be the predominant symptom of the brainstem encephalitis associated with Ri antibodies. gamma-Aminobutyric acid (GABA)(B) receptor antibodies are the most common antibodies found in patients with limbic encephalitis and small cell lung cancer, and contactin- associated protein 2 antibodies in patients with Morvan's syndrome and thymoma. Lastly, a recent study identified delta/notch- like epidermal growth factor-related receptor (DNER) as the target antigen of Tr antibodies, a marker of cerebellar ataxia and Hodgkin's lymphoma. Summary The number of antibodies relevant to PNS is now expanded to those against surface antigens. These antibodies do not confirm the paraneoplastic origin of the syndrome but predict a better response to immunotherapy.
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