title: Improvement of paraneoplastic limbic encephalitis after systemic treatment with rituximab in a patient with B-cell chronic lymphocytic leukemia.
abstract: Limbic encephalitis is an inflammatory disease of the central nervous system characterized by diverse neurologic symptoms including mnestic disturbances, hallucinations, and seizures as well as behavioral symptoms like depression, personality changes, and acute confusional states resembling dementia. Several antibodies have been described in the pathogenesis of limbic encephalitis. It is often a paraneoplastic syndrome associated with small cell lung cancer, breast cancer, or Hodgkin's lymphoma among others. Here, we report a patient with B-cell chronic lymphocytic leukemia (B-CLL), presenting with otherwise unexplained neurologic symptoms consistent with limbic encephalitis. Despite intensive diagnostic procedures, no causing agent could be identified. Pleocytosis consisting of T cells was detected in the cerebrospinal fluid (CSF). We initiated anti-B-cell therapy with Rituximab for B-CLL with quick and durable resolution of symptoms. We speculate that disruption of interaction between autoreactive T and malignant B cells is responsible for the therapeutic effect of Rituximab.
authors: Department of Hematology, Oncology, and Tumor Immunology, Charité-Universitätsmedizin Berlin, Campus Benjamin Franklin, Hindenburgdamm 30, 12203 Berlin, Germany.
authors: Hendrik
authors: H
authors: Nogai
authors: Heike
authors: H
authors: Israel-Willner
authors: Rolf
authors: R
authors: Zschenderlein
authors: Antonio
authors: A
authors: Pezzutto
references: Brain. 2000 Jul;123 ( Pt 7):1481-94
references: 10869059
references: Blood. 2011 May 19;117(20):5463-72
references: 21385850
references: J Neurol Neurosurg Psychiatry. 2007 Apr;78(4):381-5
references: 16980333
references: Eur J Intern Med. 2003 Feb;14(1):49-52
references: 12554011
references: Ann Neurol. 2005 Nov;58(5):788-92
references: 16240354
references: J Clin Oncol. 2007 Aug 20;25(24):3783-5
references: 17704429
references: Fortschr Neurol Psychiatr. 2008 Jan;76(1):41-6
references: 18189222
references: Brain. 1958 Mar;81(1):93-111
references: 13523006
references: Neurology. 1996 Jan;46(1):19-25
references: 8559372
references: Brain. 2005 Aug;128(Pt 8):1764-77
references: 15888538
references: J Clin Oncol. 2009 Mar 1;27(7):1142-4
references: 19124810
references: J Immunol. 1979 Mar;122(3):1087-90
references: 376702
references: N Engl J Med. 2004 Jun 17;350(25):2572-81
references: 15201414