D De Simoni1, R Höftberger2. 1. Klinisches Institut für Neurologie, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich. desiree.desimoni@meduniwien.ac.at. 2. Klinisches Institut für Neurologie, Medizinische Universität Wien, Währinger Gürtel 18-20, 1090, Wien, Österreich. romana.hoeftberger@meduniwien.ac.at.
Abstract
BACKGROUND: Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. OBJECTIVES: This article provides an update on the most relevant PNNS, focusing on specific syndromes associated with antibodies against classical onconeuronal antigens as well as surface and synaptic proteins. RESULTS: Classical PNNS are associated with antibodies against intracellular antigens (onconeuronal antibodies). They usually precede the tumor diagnosis and lead to the detection of the neoplasm. Affected patients are often older and have an unfavorable prognosis. Patients with surface receptor autoimmunity can have a similar presentation as classical PNNS; however, the disease is not necessarily triggered by a tumor and patients usually show a good response to treatment. Some surface receptor antibodies might manifest in highly characteristic syndromes and the resulting disease is named after the antibody, such as in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Other antibodies have considerable overlap in their clinical presentation and may be difficult to distinguish, such as in limbic encephalitis associated with GABA(B)R and α‑amino-3-hydroxy-5-hydroxy-5-methyl-4-isoxazolpropionsäure receptor (AMPAR) antibodies. The diagnosis of the PNNS is important for an early recognition of a tumor and prompt initiation of treatment, which is associated with a better outcome of patients.
BACKGROUND:Paraneoplastic neurological syndromes (PNNS) are remote effects of a tumor and mediated by an altered immune reaction. In the last ten years, the spectrum of PNNS has changed profoundly with the discovery of a new category of neurological diseases that are associated with antibodies against surface or synaptic antigens. In contrast to classical PNNS, patients with surface receptor autoimmunity are often highly responsive to immunotherapy. OBJECTIVES: This article provides an update on the most relevant PNNS, focusing on specific syndromes associated with antibodies against classical onconeuronal antigens as well as surface and synaptic proteins. RESULTS: Classical PNNS are associated with antibodies against intracellular antigens (onconeuronal antibodies). They usually precede the tumor diagnosis and lead to the detection of the neoplasm. Affected patients are often older and have an unfavorable prognosis. Patients with surface receptor autoimmunity can have a similar presentation as classical PNNS; however, the disease is not necessarily triggered by a tumor and patients usually show a good response to treatment. Some surface receptor antibodies might manifest in highly characteristic syndromes and the resulting disease is named after the antibody, such as in anti-N-methyl-D-aspartate receptor (NMDAR) encephalitis. Other antibodies have considerable overlap in their clinical presentation and may be difficult to distinguish, such as in limbic encephalitis associated with GABA(B)R and α‑amino-3-hydroxy-5-hydroxy-5-methyl-4-isoxazolpropionsäure receptor (AMPAR) antibodies. The diagnosis of the PNNS is important for an early recognition of a tumor and prompt initiation of treatment, which is associated with a better outcome of patients.
Authors: M J Titulaer; R Soffietti; J Dalmau; N E Gilhus; B Giometto; F Graus; W Grisold; J Honnorat; P A E Sillevis Smitt; R Tanasescu; C A Vedeler; R Voltz; J J G M Verschuuren Journal: Eur J Neurol Date: 2010-09-29 Impact factor: 6.089
Authors: F Graus; J Y Delattre; J C Antoine; J Dalmau; B Giometto; W Grisold; J Honnorat; P Sillevis Smitt; Ch Vedeler; J J G M Verschuuren; A Vincent; R Voltz Journal: J Neurol Neurosurg Psychiatry Date: 2004-08 Impact factor: 10.154
Authors: Francesc Graus; Maarten J Titulaer; Ramani Balu; Susanne Benseler; Christian G Bien; Tania Cellucci; Irene Cortese; Russell C Dale; Jeffrey M Gelfand; Michael Geschwind; Carol A Glaser; Jerome Honnorat; Romana Höftberger; Takahiro Iizuka; Sarosh R Irani; Eric Lancaster; Frank Leypoldt; Harald Prüss; Alexander Rae-Grant; Markus Reindl; Myrna R Rosenfeld; Kevin Rostásy; Albert Saiz; Arun Venkatesan; Angela Vincent; Klaus-Peter Wandinger; Patrick Waters; Josep Dalmau Journal: Lancet Neurol Date: 2016-02-20 Impact factor: 44.182