title: Paraneoplastic syndromes and autoimmune encephalitis: Five new things.
abstract: We review novel findings in paraneoplastic syndromes including the Lambert-Eaton myasthenic syndrome, and then focus on the novel disorders associated with antibodies against cell surface antigens, discussing the importance and caveats of antibody testing, and providing an algorithm for interpretation of results. In anti-NMDAR encephalitis 2 novel findings include the recognition of a characteristic EEG pattern ("extreme delta brush") in 30% of patients and the demonstration of a fronto-temporo-occipital gradient of glucose metabolism that correlates with disease activity. In limbic encephalitis, antibodies to GABA(B) receptor are the most frequently detected in patients with small-cell lung cancer who are anti-Hu negative, and antibodies to mGluR5 distinctively associate with Hodgkin lymphoma (Ophelia syndrome). We also address the syndromes associated with "VGKC-complex antibodies," a problematic term that groups well-characterized immune-mediated disorders (LGI1, Caspr2) with others that lack syndrome specificity, are less responsive to treatment, and for which the target antigens are unknown.
authors: IDIBAPS and Service of Neurology (MRR, MJT, JD), Hospital Clinic, University of Barcelona, Barcelona, Spain; Department of Neurology (MRR, MJT, JD), University of Pennsylvania, Philadelphia; and Institució Catalana de Recerca i Estudis Avançats (ICREA) (JD), Barcelona, Spain.
authors: Myrna R
authors: MR
authors: Rosenfeld
authors: Maarten J
authors: MJ
authors: Titulaer
authors: Josep
authors: J
authors: Dalmau
references: Neurology. 2011 Jun 14;76(24):2089-95
references: 21670438
references: Neuropsychopharmacology. 2012 Aug;37(9):2076-87
references: 22534626
references: Brain. 2010 Sep;133(9):2734-48
references: 20663977
references: J Neurol Neurosurg Psychiatry. 2012 Jul;83(7):681-6
references: 22566598
references: Arch Neurol. 2010 Sep;67(9):1109-15
references: 20837856
references: J Clin Oncol. 2011 Mar 1;29(7):902-8
references: 21245427
references: Lancet Neurol. 2010 Aug;9(8):776-85
references: 20580615
references: Ann Neurol. 2012 Jun;71(6):815-24
references: 22447725
references: Neurology. 2012 Sep 11;79(11):1094-100
references: 22933737
references: Nat Rev Neurol. 2012 Jun 19;8(7):380-90
references: 22710628