nervous system paraneoplastic syndromes

Summary

Summary: Degenerative or inflammatory conditions affecting the central or peripheral nervous system that develop in association with a systemic neoplasm without direct invasion by tumor. They may be associated with circulating antibodies that react with the affected neural tissue. (Intern Med 1996 Dec;35(12):925-9)

Top Publications

  1. Ray D, Nigam A. Paraneoplastic effects on neurophthalmologic function. Otol Neurotol. 2007;28:860-2 pubmed
    ..Few studies have focused on patients presenting with paraneoplastic syndrome before a cancer is identified...
  2. Graus F, Keime Guibert F, Rene R, Benyahia B, Ribalta T, Ascaso C, et al. Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients. Brain. 2001;124:1138-48 pubmed
    ..Finally, the predictors of mortality and PEM evolution found in the study may be important in the design of future therapeutic protocols, and emphasize the importance of early diagnosis and treatment of the underlying tumour...
  3. Pittock S, Kryzer T, Lennon V. Paraneoplastic antibodies coexist and predict cancer, not neurological syndrome. Ann Neurol. 2004;56:715-9 pubmed
    ..The autoantibody profiles observed in patients with paraneoplastic disorders imply the targeting of multiple onconeural antigens and predict the patient's neoplasm, but not a specific neurological syndrome...
  4. Schimmel M, Bien C, Vincent A, Schenk W, Penzien J. Successful treatment of anti-N-methyl-D-aspartate receptor encephalitis presenting with catatonia. Arch Dis Child. 2009;94:314-6 pubmed publisher
    ..To our knowledge this is the youngest patient with anti-NMDAR encephalitis to have been described to date. We speculate that NMDAR antibodies may be directly involved in the pathogenesis of this disease...
  5. Dalmau J, Gleichman A, Hughes E, Rossi J, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7:1091-8 pubmed publisher
    ..We aimed to analyse the clinical and immunological features of patients with the disorder and examine the effects of antibodies against NMDA receptors in neuronal cultures...
  6. Ovadia H, Karussis D, Abramsky O. [Autoimmune mechanisms in paraneoplastic neurological diseases: the paradigm of opsoclonus myoclonus ataxia syndrome]. Harefuah. 2006;145:298-303, 317 pubmed
    ..The availability of onconeural antigens to the immune effectors mechanisms is probably the main reason why there are few animal models for PND...
  7. Jaster J. Antibodies to Zic4 in paraneoplastic neurologic disorders and small-cell lung cancer. Neurology. 2004;63:1339; author reply 1339 pubmed
  8. Weizman D, Leong W. Anti-Ri antibody opsoclonus-myoclonus syndrome and breast cancer: a case report and a review of the literature. J Surg Oncol. 2004;87:143-5 pubmed
    ..Based on the 15 previously reported cases in the literature, there is no uniform treatment option available, and the response to such treatments is mixed...
  9. Voltz R. [Paraneoplastic neurological autoimmune diseases]. Dtsch Med Wochenschr. 2004;129:1743-6 pubmed

More Information

Publications62

  1. Aznar A, Montero M, Rovira R, Vidal F. Intravascular large B-cell lymphoma presenting with neurological syndromes: clinicopathologic study. Clin Neuropathol. 2007;26:180-6 pubmed
    ..Immunohistochemical findings demonstrated large B-cell lymphoma. A review of the neurological features described in previously published cases of IVL is provided...
  2. Turkel S, Brumm V, Mitchell W, Tavare C. Mood and behavioral dysfunction with opsoclonus-myoclonus ataxia. J Neuropsychiatry Clin Neurosci. 2006;18:239-41 pubmed
    ..Psychiatric symptoms examined included disruptive behavior, affective dysregulation, irritability, impulsivity, cognitive impairment, and poor attention...
  3. Talbot J, Kerrou K, Gutman F, Perie S, Grahek D, Roulet E, et al. [FDG-PET in localization of cancers of unknown primary origin]. Presse Med. 2006;35:1371-6 pubmed
    ..It is precisely in cases with a kind of 'orphan' indication that FDG PET should be considered, as an effective "problem solver"...
  4. Singh M, Singh G, Singh S. Paraneoplastic neurodegeneration in a murine host following progressive growth of a spontaneous T-Cell lymphoma: role of proinflammatory internal responses. Neuroimmunomodulation. 2006;13:122-32 pubmed
    ..In the present study, the mechanism of paraneoplastic neurodegeneration associated with progressive in vivo growth of a T-cell lymphoma of spontaneous origin has been investigated...
  5. Storstein A, Vedeler C. Paraneoplastic neurological syndromes and onconeural antibodies: clinical and immunological aspects. Adv Clin Chem. 2007;44:143-85 pubmed
    ..In some of the disorders, immunosuppressive therapy is of additional benefit. The prognosis of the different PNS varies depending on the level of affection and the degree of neuronal death...
  6. Berardi R, Grilli G, Romagnoli E, Saladino T, Freddari F, Tamburrano T, et al. [Paraneoplastic syndromes: a review]. Clin Ter. 2005;156:281-8 pubmed
    ..This review discusses all the main paraneoplastic syndromes, focusing mainly on their clinical aspect and reminding the most commonly associated cancers...
  7. Graus F, Dalmau J. Paraneoplastic neurological syndromes: diagnosis and treatment. Curr Opin Neurol. 2007;20:732-7 pubmed
    ..The most relevant advances in the field of paraneoplastic neurological syndromes are described, with special emphasis on particular clinical-immunological associations and practical issues for the diagnosis and treatment of these disorders...
  8. Anderson D, Borsaru A. Case report: Lymphoma-related resolving paraneoplastic myelopathy with MRI correlation. Br J Radiol. 2008;81:e103-5 pubmed publisher
    ..This has rarely been described in the literature in relation to lymphoma or with imaging correlation...
  9. Patel R, Subramaniam R, Mandrekar J, Hammack J, Lowe V, Jett J. Occult malignancy in patients with suspected paraneoplastic neurologic syndromes: value of positron emission tomography in diagnosis. Mayo Clin Proc. 2008;83:917-22 pubmed publisher
    ..To determine the value of positron emission tomography (PET) in diagnosing occult malignancies in patients with paraneoplastic neurologic syndromes (PNSs) at Mayo Clinic's site in Rochester, MN...
  10. Grisold W, Fodinger M, Oberndorfer S. [Stroke in cancer patients. A paraneoplastic neurological syndrome?]. Nervenarzt. 2010;81:418-22 pubmed publisher
    ..However, there are several cancer-specific types and causes of stroke which need to be considered in each patient, as they can be of significance in the treatment...
  11. Suzuki S. [Lambert-Eaton myasthenic syndrome (LEMS)]. Brain Nerve. 2010;62:419-26 pubmed
    ..The efficacy of 3,4-diaminopyridine for the treatment of LEMS has also been established. Patients with LEMS require the immunotherapies such as plasma exchange and the administration of high doses of immunoglobulin and prednisolone...
  12. Tschernatsch M, Singh P, Gross O, Gerriets T, Kneifel N, Probst C, et al. Anti-SOX1 antibodies in patients with paraneoplastic and non-paraneoplastic neuropathy. J Neuroimmunol. 2010;226:177-80 pubmed publisher
    ..Anti-SOX1 antibodies are associated with paraneoplastic neuropathies and may define another group of non-paraneoplastic, immune-mediated neuropathies...
  13. Gerber R, Mazzone P, Arroliga A. Paraneoplastic syndromes associated with bronchogenic carcinoma. Clin Chest Med. 2002;23:257-64 pubmed
    ..Unraveling the mechanisms that produce these syndromes will lead to insight into tumor biology that will be translated into novel approaches for early detection and therapy...
  14. Blaes F, Klotz M, Funke D, Strittmatter M, Kraus J, Kaps M. Disturbance in the serum IgG subclass distribution in patients with anti-Hu positive paraneoplastic neurological syndromes. Eur J Neurol. 2002;9:369-72 pubmed
    ..05). Our study demonstrates a disturbance in the IgG subclass distribution in PNS patients which is partly different from SCLC patients. The isotype regulation of the anti-Hu antibody seems to be independent from this phenomenon...
  15. Tanaka Y, Nishida H, Yamada O, Takahashi Y, Moriwaki H. [Intrathecal glutamate receptor antibodies in a patient with elderly-onset refractory epilepsy]. Rinsho Shinkeigaku. 2003;43:345-9 pubmed
    ..The glutamate receptor antibodies may play a role in late-onset refractory epilepsy, and may be an autoimmune factor in paraneoplstic neurologic syndrome presenting with epilepsy...
  16. Hayward K, Jeremy R, Jenkins S, Barkovich A, Gultekin S, Kramer J, et al. Long-term neurobehavioral outcomes in children with neuroblastoma and opsoclonus-myoclonus-ataxia syndrome: relationship to MRI findings and anti-neuronal antibodies. J Pediatr. 2001;139:552-9 pubmed
  17. Rizzardi G, Campione A, Scanagatta P, Terzi A. Paraneoplastic extra limbic encephalitis associated with thymoma. Interact Cardiovasc Thorac Surg. 2009;9:755-6 pubmed publisher
    ..The patient was submitted to thymectomy through a median sternotomy and we assisted to secondary reduction of cerebral lesions and total remission of symptoms...
  18. Saito T, Kusunoko J. [Paraneoplastic neurological syndromes due to myelomas and lymphomas]. Nihon Naika Gakkai Zasshi. 2008;97:1796-804 pubmed
  19. Kylaniemi M, Koskinen M, Karhunen P, Rantala I, Peltola J, Haapasalo H. A novel frozen brain tissue array technique: immunohistochemical detection of neuronal paraneoplastic autoantibodies. Neuropathol Appl Neurobiol. 2004;30:39-45 pubmed
    ..The frozen brain array also allows the detection of RNA and DNA targets involved in neurological diseases...
  20. McCabe D, Turner N, Chao D, Leff A, Gregson N, Womersley H, et al. Paraneoplastic "stiff person syndrome" with metastatic adenocarcinoma and anti-Ri antibodies. Neurology. 2004;62:1402-4 pubmed
    ..Screening for an underlying malignancy and anti-Ri antibodies should be considered in patients with SPS when clinical presentation is atypical...
  21. Fumal A, Jobe J, Pepin J, Delvaux V, Senterre J, Bonaventure S, et al. Intravenous immunoglobulins in paraneoplastic brainstem encephalitis with anti-Ri antibodies. J Neurol. 2006;253:1360-1 pubmed
  22. Vernino S. Paraneoplastic neurologic syndromes. Curr Neurol Neurosci Rep. 2006;6:193-9 pubmed
    ..These studies not only shed light on the pathogenesis of PND and the mechanisms of effective antitumor immunity but may eventually lead to improved treatment...
  23. Corato M, Marinou Aktipi K, Nano R, Giometto B, Cereda C, Natoli G, et al. Paraneoplastic brainstem encephalitis in a patient with malignant fibrous histiocytoma and atypical anti-neuronal antibodies. J Neurol. 2004;251:1415-7 pubmed
  24. Honnorat J, Cartalat Carel S. Advances in paraneoplastic neurological syndromes. Curr Opin Oncol. 2004;16:614-20 pubmed
    ..To describe specificities and new advances in paraneoplastic neurologic syndromes (PNSs)...
  25. Bhidayasiri R, Somers J, Kim J, Ramat S, Nayak S, Bokil H, et al. Ocular oscillations induced by shifts of the direction and depth of visual fixation. Ann Neurol. 2001;49:24-8 pubmed
    ..The probable mechanism is that pontine omnipause neurons, which normally gate saccades, are inhibited during the sustained vergence movement that follows the saccadic component of the response to the Müller paradigm...
  26. Mitchell W, Davalos Gonzalez Y, Brumm V, Aller S, Burger E, Turkel S, et al. Opsoclonus-ataxia caused by childhood neuroblastoma: developmental and neurologic sequelae. Pediatrics. 2002;109:86-98 pubmed
  27. de Beukelaar J, Sillevis Smitt P, Hop W, Kraan J, Hooijkaas H, Verjans G, et al. Imbalances in circulating lymphocyte subsets in Hu antibody associated paraneoplastic neurological syndromes. Eur J Neurol. 2007;14:1383-91 pubmed
    ..Although indirect, our data provide further support for the involvement of T cells in the pathogenesis of Hu antibody associated PNS...
  28. Silveri M, Misciagna S, Laudisio A, Colosimo C. Olivopontocerebellar atrophy: paraneoplastic syndrome of brain tumour?. Eur J Neurol. 2000;7:341-5 pubmed
  29. Pranzatelli M, Hyland K, Tate E, Arnold L, Allison T, Soori G. Evidence of cellular immune activation in children with opsoclonus-myoclonus: cerebrospinal fluid neopterin. J Child Neurol. 2004;19:919-24 pubmed
  30. Waragai M, Chiba A, Uchibori A, Fukushima T, Anno M, Tanaka K. Anti-Ma2 associated paraneoplastic neurological syndrome presenting as encephalitis and progressive muscular atrophy. J Neurol Neurosurg Psychiatry. 2006;77:111-3 pubmed
    ..It is suggested that the anti-Ma2 antibody is involved not only in encephalitis, but may also play a role in the cervical spinal cord lesions resulting in a motor neurone disease-like presentation...
  31. Deepti A, Noone M, Mahadevan A, Naresh K, Yasha T, Satishchandra P, et al. Primary cardiac cytotoxic T-cell lymphoma presenting with neurological deficits: a case report. Cardiovasc Pathol. 2008;17:334-8 pubmed publisher
    ..Occasionally, lymphomas and other systemic malignancies clinically present as paraneoplastic neurological syndromes...
  32. Gambini C, Conte M, Bernini G, Angelini P, Pession A, Paolucci P, et al. Neuroblastic tumors associated with opsoclonus-myoclonus syndrome: histological, immunohistochemical and molecular features of 15 Italian cases. Virchows Arch. 2003;442:555-62 pubmed
    ..In conclusion, ganglioneuroblastoma, intermixed subtype, lack of MYCN amplification, aneuploid DNA content and presence of lymphoid infiltrates may contribute to favorable prognosis in NTs associated with OMS...
  33. Haddad L, Amsterdam A, Chi D. Celiac disease presenting as a paraneoplastic syndrome in a patient with synchronous endometrial and ovarian cancers. Gynecol Oncol. 2005;97:704-6 pubmed
    ..Infrequently, patients present with vague, non-specific symptoms which often delay diagnosis and treatment...
  34. Rajabally Y, Naz S, Farrell D, Abbott R. Paraneoplastic brainstem encephalitis with tetraparesis in a patient with anti-Ri antibodies. J Neurol. 2004;251:1528-9 pubmed
  35. Dropcho E. Immunotherapy for paraneoplastic neurological disorders. Expert Opin Biol Ther. 2005;5:1339-48 pubmed
    ..This review summarises the current understanding of the clinical immunology of paraneoplastic disorders, and outlines immunotherapy options and outcomes...
  36. Bataller L, Dalmau J. Paraneoplastic neurologic syndromes. Neurol Clin. 2003;21:221-47, ix pubmed
    ..PND often develop before the presence of a cancer is known and their recognition may lead to the tumor diagnosis...
  37. van Toorn R, Rabie H, Warwick J. Opsoclonus-myoclonus in an HIV-infected child on antiretroviral therapy--possible immune reconstitution inflammatory syndrome. Eur J Paediatr Neurol. 2005;9:423-6 pubmed
    ..Single photon emission computed tomography (SPECT) proved helpful by localizing the area of dysfunction to the cerebellar vermis...
  38. Rubio Nazabal E, Marey Lopez J, Avarez Pérez P, Lopez Facal S, Alonso Magdalena L. [Opsoclonus-myoclonus syndrome in patient with ovarian cancer]. An Med Interna. 2003;20:370-2 pubmed
    ..In our patient opsoclonus began after ovarian cancer diagnosis, after chemotherapy and radiotherapy, improving with corticoid and clonazepan therapy...
  39. Kararizou E, Markou I, Zalonis I, Gkiatas K, Triantafyllou N, Kararizos G, et al. Paraneoplastic limbic encephalitis presenting as acute viral encephalitis. J Neurooncol. 2005;75:229-32 pubmed
    ..To describe a case of limbic encephalitis which initially presented as viral limbic encephalitis and during the clinical evaluation a renal carcinoma was diagnosed...
  40. Etienne M, Weimer L. Immune-mediated autonomic neuropathies. Curr Neurol Neurosci Rep. 2006;6:57-64 pubmed
    ..Paraneoplastic autonomic disorders are clinically indistinguishable and associated with various overlapping antibody associations, including anti-Hu (ANNA-1), ganglionic acetylcholine receptors, CRMP-5, and PCA-2...
  41. Marx A, Willcox N, Leite M, Chuang W, Schalke B, Nix W, et al. Thymoma and paraneoplastic myasthenia gravis. Autoimmunity. 2010;43:413-27 pubmed publisher
    ..There must be further clues in the intriguing serological and cellular parallels in some patients with late-onset MG but without thymomas-and in others with AIRE mutations-and in the contrasts with early-onset MG, as discussed here...
  42. Fadul C, Stommel E, Dragnev K, Eskey C, Dalmau J. Focal paraneoplastic limbic encephalitis presenting as orgasmic epilepsy. J Neurooncol. 2005;72:195-8 pubmed
    ..To report orgasmic epilepsy as a manifestation of paraneoplastic limbic encephalitis in a patient with small cell lung cancer...
  43. Lim A, Scriven A, Knezevic W, Texler M. A predominantly left upper limb sensory neuronopathy as a manifestation of a metastatic neuroendocrine malignancy. J Clin Neurosci. 2009;16:838-9 pubmed publisher
    ..An 83-year-old woman presented with a predominantly left upper limb sensory ganglionopathy. She was found to have metastatic, poorly differentiated, neuroendocrine malignancy. Anti-neuronal antibodies were not detected...
  44. Greenlee J. Recommended diagnostic criteria for paraneoplastic neurological syndromes. J Neurol Neurosurg Psychiatry. 2004;75:1090 pubmed
  45. Murinson B, Guarnaccia J. Stiff-person syndrome with amphiphysin antibodies: distinctive features of a rare disease. Neurology. 2008;71:1955-8 pubmed publisher
    ..A paraneoplastic variant, less well characterized, is associated with amphiphysin antibodies (amphiphysin Ab). The objective of this study was to identify distinctive clinical features of amphiphysin Ab-associated SPS...
  46. de Bot S, Dorresteijn L, Haaxma C, Kappelle A, van de Warrenburg B. [From psychiatric symptoms to paraneoplastic syndrome]. Tijdschr Psychiatr. 2008;50:603-9 pubmed
    ..These cases illustrate that patients with paraneoplastic neurological syndromes may present with psychiatric symptoms which can hamper an early diagnosis...
  47. Kataoka H, Dalmau J, Ueno S. Paraneoplastic encephalitis associated with ovarian teratoma and N-methyl-D-aspartate receptor antibodies. Eur J Neurol. 2008;15:e5-6 pubmed
  48. Khealani B, Qureshi R, Wasay M. Motor neuronopathy associated with adenocarcinoma of esophagus. J Pak Med Assoc. 2004;54:165-6 pubmed
  49. Monstad S, Vedeler C. An immunoprecipitation assay for the detection of onconeural antibodies. Acta Neurol Scand Suppl. 2006;183:71-2 pubmed
    ..Onconeural antibodies are found in some patients with cancer, and are associated with paraneoplastic neurological syndromes...
  50. Cereda C, Lobrinus J, Maeder P, Bogousslavsky J. Memory impairment and tonic-clonic seizure in a 39-year-old woman. Lancet Neurol. 2005;4:683-8 pubmed
  51. Gesundheit B, Lerer B, Budowski E, Neuman T, Gomori J, Or R. Sudden psychotic symptoms in a 28-year-old male with thymoma. J Clin Oncol. 2008;26:4353-5 pubmed publisher
  52. Stich O, Kleer B, Rauer S. Absence of paraneoplastic antineuronal antibodies in sera of 145 patients with motor neuron disease. J Neurol Neurosurg Psychiatry. 2007;78:883-5 pubmed
    ..Previous reports indicate that pure motor neuron disease (MND) is rarely of paraneoplastic origin...
  53. Schönfeldt Lecuona C, Freudenmann R, Tumani H, Kassubek J, Connemann B. Acute psychosis with a mediastinal carcinoma metastasis. Med Sci Monit. 2005;11:CS6-8 pubmed
    ..Reversible schizophrenia-like syndromes have been reported to occur with small-cell carcinoma of the lung, thymoma, and hematological disorders...