Síndromes paraneoplásicos del sistema nervioso

  1. Pascual, F. Higes
  2. Monteiro, G. Carvalho
  3. Herán, I. Sánchez
  4. Margarit, B. Povedano
  5. Izquierdo, A. Yusta
Journal:
Medicine: Programa de Formación Médica Continuada Acreditado

ISSN: 0304-5412

Year of publication: 2019

Issue Title: Enfermedades del sistema nervioso (IX)Enfermedades desmielinizantes del sistema nervioso central

Series: 12

Issue: 78

Pages: 4616-4629

Type: Article

DOI: 10.1016/J.MED.2019.05.013 DIALNET GOOGLE SCHOLAR

More publications in: Medicine: Programa de Formación Médica Continuada Acreditado

Abstract

Resumen Los síndromes paraneoplásicos son las manifestaciones clínicas de un tumor que no están relacionadas con la invasión directa del tumor primario o de sus metástasis. Comprenden un grupo heterogéneo de síndromes que pueden afectar a cualquier parte del sistema nervioso central o periférico, unión neuromuscular y músculo y en ocasiones en el mismo paciente en múltiples áreas. Son poco frecuentes, con una incidencia general en pacientes con cáncer inferior al 1%. La presencia de anticuerpos onconeuronales apoya el diagnóstico en un síndrome clínico compatible; sin embargo, solo están presentes en aproximadamente un 50% de los casos, por lo que su ausencia no descarta el diagnóstico. Si se puede, se debe iniciar el tratamiento lo antes posible, pues los síndromes paraneoplásicos evolucionan en unas semanas, pudiendo producir un daño irreversible e incluso la muerte del paciente. Paraneoplastic syndromes are the clinical manifestations of a tumour that are not related to direct invasion of the primary tumour or of its metastases. These syndromes make up a heterogeneous group of syndromes that can affect any part of the central or peripheral nervous system, neuromuscular junction and muscle and, occasionally, in multiple areas in the same patient. The syndromes are uncommon, with a general incidence rate in patients with cancer of less than 1%. The presence of onconeural antibodies supports the diagnosis in a compatible clinical syndrome; however, these antibodies are only present in approximately 50% of cases; therefore, their absence does not rule out the diagnosis. If available, treatment should be started as soon as possible, because paraneoplastic syndromes progress in a few weeks and can cause irreversible damage and even death.

Bibliographic References

  • Bataller L, Graus F, Saiz A, Vilchez JJ. Clinical outcome in adult onset idiophatic or paraneoplastic opsoclonus-myoclonus. Brain. 2001;124:437.
  • Bataller L, Dalmau J. Paraneoplastic neurologic syndromes. Neurol Clin. 2003;21:221-47.
  • Boronat A, Gelfand JM, Gresa-Arribas N, Jeong HY, Walsh M, Roberts K. Encephalitis and antibodies to dipeptidylpeptidase like protein-6, a subunit of Kv4.2 potassium channels. Ann Neurol. 2013;73: 120.
  • Carvajal-González A, Liete MI, Walters P, Woodhall M, Coutinho E, Balint B. Glycuine receptor antibodies in PERM and related síndro-mes: characteristics, clinical features and outcomes. Brain. 2014;137:2178.
  • Dalmau J, Furneaux HM, Cordon-Cardo C, Posner JB. The expression of the Hu (paraneoplastic encephalomyelitis/sensory neuronopathy) antigen in human normal and tumor tissues. Am J Pathol. 1992;141:881.
  • Dalmau J, Geis C, Graus F. Autoantibodies to synaptic receptors and neu-ronal cells surface proteins in autoimmune diseases of the central nervous system. Physiol Rev. 2017;97:839.
  • Dalmau J, Graus F, Rosemblum MK, Posner JB. Anti-Hu associated paraneoplastic encephalomyelitis/sensory neuronopathy. A clinical study of 71 patientes. Medicine (Baltimore). 1992;71:59-72.
  • Dalmau J, Lancaster E, Martínez-Hernández E, Rosenfeld Mr, Balice-Gordon R. Clinical experience and laboratory investigations in patients vith anti-NMDAR encephalitis. Lancet Neurol. 2011; 9:776-85.
  • Dalmau J, Graus f, Villarejo A, Posner JB, Blumenthal D, Thiessen B,. Clinical analysis of anti Ma2-associated encephalitis. Brain. 2004;127:1831.
  • Ducray F, Graus F, Vigliani MC, Antoine JC, Rogemond V, Saiz A. Delayed onset of a second paraneoplastic neurological syndrome in eight patientes. J. Neurol Neurosurg Psychiatry. 2010;81:937
  • Gaig C, Graus F, Compta Y, Högl B, Bataller L, Brüggemann N. Clinical manifestations of the anti-IgLON5 disease. Neurology. 2017;88:1736
  • Graus F, Delattre JY, Antoine JC, Dalmau J, Giometto B, Grisold . Recommended diagnostic criteria for paraneoplastic neurological syndromes. J. Neurol Neurosurg psychiatry. 2004;75:1135.
  • Graus F, Deime-Guibert F, Reñe R, Benyahia B, Ribalta T, Ascaso C. Anti-Hu associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain. 2001;124:1138.
  • Gresa-Arribas N, Planaguma J, Petit-Pedrol M, Kawachi I, Katada S, Glaser CA. Human neurexin-3alfa antibodies associate with encephalitis and alter synapse development. Neurology. 2016;86:2235.
  • Hoftberger R, van Sonderen A, Leypoldt F, Houghton D, Geschwind M, Gelfand J. Encephalitis and AMPA receptor antibodies: Novel findings in a case series of 22 patients. Neurology. 2015;84:2403.
  • Hoftberger R, Titulaer MJ, Sabater L, Dome B, Rózsás A, Hegedus B. Encephalitis and GABAB receptor antibodies: novel findings in a nuew series of 20 patients. Neurology. 2013;81:1500
  • Iizuka T, Sakai F, Ide T, Monzen T, Yoshii S, Iigaya M. Anti-NMDA ceceptor encephalitis in Japan: long term outcome whitout tumor removal. Neurology. 2008;70:504.
  • Inrani SR, Alexander S, Waters P, Kleopa KA, Pettingill P, Zuliani L. Antibodies to Kv1 potassium channel complex proteins leucine rich, glioma inactivated 1 protein and contactin associated protein-2 in limbic encephalitis, Morvan’s syndrome and acquired neuromyotonia. Brain. 2010;133:2734.
  • Lai M, Huijbers MG, Lancaster E, Graus F, Bataller L, Balice-Gordon R. Investigation of LGI1 as the antigen in limbic encephalitis previously attributed to potassium channels: a case series. Lancet Neurol. 2010;9:776
  • Leypoldt F, Titulaer MJ, Aguilar E, Walther J, Bönstrup M, Havemeister S. Herpex simplex virus-1 encephalitis can trigger anti-NMDA receptor encephalitis: case report. Neurology. 2013;81:1637.
  • Patterson KT, Dalmau J, Lancaster E. Mechanisms of Caspr2 antibodies in autoimmune encephalitis an neuromyotonia. Ann Neurol. 2011;83: 40.
  • Pittock SJ, Lennon VA. Aquaporin-4 autoantibodies in a paraneoplastic context. Arch Neurol. 2008;65:629.
  • Posner JB. Neurologyc complications of cancer. Filadelfia: F.A. Davis Company; 1995.
  • Pozo Rosich P, Clover L, Saiz A, Vincent A, Graus F. Voltage gated potassium channel antiboides in limbic encephalitis. Ann Neurol. 2003;54:530-3.
  • Rosenfeld MR, Eichen JG, Wade DF, Posner JB, Dalmau J. Molecular and clinical diversity in paraneoplastic immunity to Ma proteins. Ann Neurol. 2001;50:339.
  • Rudnicki SA, Dalmau J. Paraneoplastic syndromes of the spinal cord, nerve and muscle. Muscle Nerve. 2000;23:1800-18.
  • Sabater L, Titulaer M, Saiz A, Verschuuren J, Güre AO, Graus F. SOX1 antobodies are markers of paraneoplastic Lambert-Eaton myasthenic síndrome. Neurology. 2008;70:924.
  • Saiz A, Arpa J, Sagasta A, Casamitjana R, Zarranz JJ, Tolosa E. Autoantibodies to glutamic acid decarboxylase in three patients with cerebellar ataxia, late onset insulin dependent diabetes mellitus, and polyendocrine autoimmunity. Neurology. 1997;49:1026.
  • Savage PA, Vosseller K, Kang C, Larimore K, Riedel E, Wojnoonski K. Recognition of a ubiquitous self antigen by prostate cancer, infiltrating CD8+ T lynfocytes. Science. 2008;319:215.
  • Schmitt SE, Pargeon K, Frechette ES, Hirsch LJ, Dalmau J, Friedman D. Extreme delta brush: a unique EEG pattern in adults with anti-NMDA receptor enchephalitis. Neurology. 2012;79:109430.
  • Shams ́ili S, Grefkens J, de Leeuw B, de Leeuw B, van den Bent M, Hooijkaas H, van der Holt B. Paraneoplastic cerebellar degeneration associated with antineuronal antibodies: analysis of 50 patientes. Brain. 2003;126:1409.
  • Sillevis Smitt P, Grefkens J, de Leeuw B, van den Bent M, van Putten W, Hooijkaas H. Survival and outcome in 73 anti-Hu positive pa-tients with paraneoplasltic encephalomyelitis/sensory neuronopathy. J Neurol. 2002;249:745.
  • Titulaer MJ, McCracken L, Gabilondo I, Armangué T, Glaser C, Iizuka T. Treatment and prognostic factors for long-term outcome in pa-tients with anti-NMDA receptor encephalitis: an observational cohort study. Lancet Neurol. 2013;12:157.
  • Thompson J, Bi M, Murchison AG, Makuch M, Bien CG, Chu K. The importance of early immunotherapy in patients with faciobrachial distonic seizures. Brain. 2018;141:348.
  • Tobin WO, Lennon VA, Komorowski L, Probst C, Clardy SL, Aksamit AJ. DPPX potassium channel antibody: frecuency, clinical accompaniments and outcomes in 20 patientes. Neurology. 2014;83:1797.
  • Vitaliani R, Mason W, Ances B, Zwerdling T, Jiang Z, Dalmau J. Paraneoplastic encephalitis, psychiatric symptoms and hypoventilation in ovarian teratoma. Ann Neurol. 2005;58:594.
  • Waragai M, Chiba A, Uchibori A. Anti-Ma2 associated paraneoplastic neurological syndrome presenting as encephalitis and progressive muscular atrophy. J Neurol Neurosurg Psychiatry. 2006;77:111.
  • Widdess-Walsh P, Tavee JO, Schuele S, Stevens GH. Response to intra venous immunoglobulin in anti-Yo associated paraneoplastic cerebellar degeneration case report and review of the literature. J Neurooncol. 2003;63:187.
Data source: Dialnet