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Current Treatment Options in Neurology

, Volume 15, Issue 2, pp 201–209 | Cite as

Paraneoplastic Brain Stem Encephalitis

  • Franz BlaesEmail author
PARANEOPLASTIC SYNDROMES (F GRAUS, SECTION EDITOR)

Opinion statement

Paraneoplastic brain stem encephalitis can occur as an isolated clinical syndrome or, more often, may be part of a more widespread encephalitis. Different antineuronal autoantibodies, such as anti-Hu, anti-Ri, and anti-Ma2 can be associated with the syndrome, and the most frequent tumors are lung and testicular cancer. Anti-Hu-associated brain stem encephalitis does not normally respond to immunotherapy; the syndrome may stabilize under tumor treatment. Brain stem encephalitis with anti-Ma2 often improves after immunotherapy and/or tumor therapy, whereas only a minority of anti-Ri positive patients respond to immunosuppressants or tumor treatment. The Opsoclonus-myoclonus syndrome (OMS) in children, almost exclusively associated with neuroblastoma, shows a good response to steroids, ACTH, and rituximab, some patients do respond to intravenous immunoglobulins or cyclophosphamide. In adults, OMS is mainly associated with small cell lung cancer or gynecological tumors and only a small part of the patients show improvement after immunotherapy. Earlier diagnosis and treatment seem to be one major problem to improve the prognosis of both, paraneoplastic brain stem encephalitis, and OMS.

Keywords

Paraneoplastic neurological disease Autoantibodies Brain stem encephalitis Tumor immunology Antineuronal Treatment Differential diagnosis Immunotherapy Opsoclonus-myoclonus syndrome Immunosuppressive Corticosteroids Adrenocorticotropin Cyclophosphamide Rituximab 

Notes

Acknowledgment

Dr. Blaes is supported by the Deutsche Forschungsgemeinschaft (Bl 452/2-3) and the German OMS parents group.

Disclosure

Dr. Blaes has received honoraria for giving lectures from Grifols.

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Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of NeurologyGummersbach HospitalGummersbachGermany

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