title: Paraneoplastic brain stem encephalitis.
abstract: 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.
authors: Department of Neurology, Gummersbach Hospital, Wilhelm-Breckow-Allee 20, 51643, Gummersbach, Giessen, Germany, Franz.Blaes@kkh-gummersbach.de.
authors: Franz
authors: F
authors: Blaes
references: Lancet Neurol. 2013 Feb;12(2):157-65
references: 23290630
references: Eur J Paediatr Neurol. 2008 Jan;12(1):51-5
references: 17625938
references: Neurology. 1993 Jan;43(1):207-11
references: 8423887
references: Science. 2003 Nov 14;302(5648):1212-5
references: 14615540
references: Ann Neurol. 1991 Mar;29(3):241-51
references: 2042940
references: Acta Paediatr. 1992 Apr;81(4):371-2
references: 1606405
references: Ann Neurol. 2007 Jan;61(1):25-36
references: 17262855
references: Brain. 2001 Jun;124(Pt 6):1138-48
references: 11353730
references: Virchows Arch. 2003 Jun;442(6):555-62
references: 12709798
references: Ann Neurol. 2005 Aug;58(2):313-7
references: 15988749
references: J Neurooncol. 2010 Oct;100(1):141-3
references: 20143128
references: J Pediatr Hematol Oncol. 2006 Sep;28(9):585-93
references: 17006265
references: Med Pediatr Oncol. 1997 Apr;28(4):284-8
references: 9078325
references: J Neurol Neurosurg Psychiatry. 2004 Aug;75(8):1135-40
references: 15258215
references: Curr Opin Neurol. 2007 Feb;20(1):25-31
references: 17215685
references: Pediatr Blood Cancer. 2008 Mar;50(3):683-7
references: 17226843
references: Curr Opin Neurol. 2012 Dec;25(6):795-801
references: 23041955
references: Clin Neuropharmacol. 1996 Feb;19(1):1-47
references: 8867515
references: Neuron. 2000 Feb;25(2):359-71
references: 10719891
references: Arch Neurol. 2010 Sep;67(9):1109-15
references: 20837856
references: J Neurol. 2002 Nov;249(11):1597-8
references: 12532924
references: J Neuroimmunol. 2005 Dec 30;170(1-2):150-7
references: 16203043
references: J Neurol Neurosurg Psychiatry. 2013 May;84(5):576-83
references: 22984203
references: Med Pediatr Oncol. 2001 Jun;36(6):612-22
references: 11344492
references: J Neurol Neurosurg Psychiatry. 2009 Apr;80(4):404-7
references: 19015226
references: J R Coll Physicians Edinb. 2012;42(3):221-4
references: 22953316
references: J Neurol Neurosurg Psychiatry. 2012 Jan;83(1):15-22
references: 22056964
references: J Neuroimmunol. 2009 May 29;210(1-2):87-91
references: 19339060
references: Brain. 2001 Feb;124(Pt 2):437-43
references: 11157570
references: Curr Neurol Neurosci Rep. 2011 Dec;11(6):543-52
references: 21956758
references: Brain. 2004 Aug;127(Pt 8):1831-44
references: 15215214
references: Eur J Paediatr Neurol. 2009 May;13(3):219-23
references: 18571942