Nervous System Paraneoplastic Syndromes

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)

Also Known As:

Networked: 61 relevant articles (0 outcomes, 1 trials/studies)

Disease Context: Research Results

Related Diseases

1.	Small Cell Lung Carcinoma (Small Cell Lung Cancer)
2.	Neoplasms (Cancer)
3.	Lung Neoplasms (Lung Cancer)
4.	Paraneoplastic Cerebellar Degeneration
5.	Opsoclonus-Myoclonus Syndrome (Kinsbourne Syndrome)

Experts

1.	Graus, F: 3 articles (02/2006 - 04/2000)
2.	D'Alessandro, Vito: 2 articles (01/2010 - 01/2008)
3.	Carella, Massimo: 2 articles (01/2010 - 01/2008)
4.	Vendemiale, Gianluigi: 2 articles (01/2010 - 01/2008)
5.	Muscarella, Lucia Anna: 2 articles (01/2010 - 01/2008)
6.	Zelante, Leopoldo: 2 articles (01/2010 - 01/2008)
7.	Vedeler, C A: 2 articles (09/2006 - 02/2004)
8.	Storstein, A: 2 articles (09/2006 - 02/2004)
9.	Storstein, Anette: 2 articles (08/2005 - 10/2004)
10.	Vedeler, Christian A: 2 articles (08/2005 - 10/2004)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Nervous System Paraneoplastic Syndromes:

1.	Messenger RNA (mRNA)IBA 08/08/1998 - "The expression of mRNA for the neuronal antigen HuD (Elavl4) associated with paraneoplastic encephalomyelitis and sensory neuronopathy was evaluated in the developing and adult rat nervous system. " 03/01/1995 - "Paraneoplastic encephalomyelitis antigens bind to the AU-rich elements of mRNA." 06/01/1993 - "In addition, Hel-N1 was shown to be reactive with autoantibodies from patients with paraneoplastic encephalomyelitis both before and after binding to c-myc mRNA." 07/01/1995 - "In the current study we examined (1) the reactivity of anti-Hu sera with HuD, HuC, and Hel-N1; (2) the expression of HuD, HuC, and Hel-N1 messenger RNA in small-cell lung cancer of patients with and those without paraneoplastic encephalomyelitis/sensory neuronopathy; (3) the correlation between anti-Hu serum reactivity with these three Hu antigens and the type of neurological symptoms; and (4) the major immunogenic sites of HuD. " 08/01/1997 - "Expression of HuD (a paraneoplastic encephalomyelitis antigen) mRNA in lung cancer."
2.	Hu Paraneoplastic Encephalomyelitis AntigensIBA 08/01/1997 - "HuD, one of the Hu antigens (HuD and HuC), was recognized in the sera of small cell lung cancer (SCLC) patients with antibody-associated paraneoplastic encephalomyelitis/peripheral sensory neuropathy (PEM/PSN). " 03/01/1997 - "Cell surface expression of paraneoplastic encephalomyelitis/sensory neuronopathy-associated Hu antigens in small-cell lung cancers and neuroblastomas." 07/01/1995 - "In the current study we examined (1) the reactivity of anti-Hu sera with HuD, HuC, and Hel-N1; (2) the expression of HuD, HuC, and Hel-N1 messenger RNA in small-cell lung cancer of patients with and those without paraneoplastic encephalomyelitis/sensory neuronopathy; (3) the correlation between anti-Hu serum reactivity with these three Hu antigens and the type of neurological symptoms; and (4) the major immunogenic sites of HuD. " 03/01/1998 - "Antibodies against the HuD antigen expressed in small-cell lung cancer (SCLC) cross-react with proteins expressed in neurons of the central and peripheral nervous system and are associated with paraneoplastic encephalomyelitis and sensory neuropathy (PEM/SN). "
3.	Immunodominant EpitopesIBA 07/01/1995 - "Study of the major immunogenic sites of HuD resulted in the identification of two major immunodominant regions with at least two distinct epitopes recognized by the serum of all patients with anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuronopathy."
4.	EpitopesIBA 07/01/1995 - "Study of the major immunogenic sites of HuD resulted in the identification of two major immunodominant regions with at least two distinct epitopes recognized by the serum of all patients with anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuronopathy."
5.	AntibodiesIBA 06/01/2015 - "One sequela of SCLC is a paraneoplastic neurological syndrome usually mediated by a high titer of anti-Hu antibodies, a disease which can present in several variations of paraneoplastic encephalomyelitis. " 06/01/2001 - "We reviewed 200 patients with paraneoplastic encephalomyelitis (PEM) and anti-Hu antibodies to show possible clinical differences with respect to previous series, and to identify patient, tumour and treatment-related characteristics associated with neurological disability and survival. " 09/15/1994 - "HuD is the target of an immune response including high titered antibodies causing paraneoplastic encephalomyelitis and sensory neuropathy. " 02/14/2006 - "Paraneoplastic encephalomyelitis associated with pancreatic tumor and anti-GAD antibodies." 02/01/2004 - "Patients with paraneoplastic encephalomyelitis(subacute sensory neuronopathy) (PEM/SSN), most commonly associated with small-cell lung cancer (SCLC), frequently harbor Hu antibodies, which are usually detected by indirect immunohistochemistry or immunoblot. "
6.	AntigensIBA 04/01/1998 - "POP66, a paraneoplastic encephalomyelitis-related antigen, is a marker of adult oligodendrocytes." 10/01/1997 - "HuD is one of a family of neural antigens recognised by the sera of patients with antibody-associated paraneoplastic encephalomyelitis. " 10/01/1995 - "Immunization with the paraneoplastic encephalomyelitis antigen HuD does not cause neurologic disease in mice." 01/01/1994 - "Sera from some patients with antibody-associated paraneoplastic encephalomyelitis/sensory neuronopathy recognize the Ro-52K antigen." 01/01/1994 - "Mapping of the gene coding for a paraneoplastic encephalomyelitis antigen (HuD) to human chromosome site 1p34."
7.	Proteins (Proteins, Gene)IBA 06/01/2012 - " Since Hu family proteins including HuC are well-known causes of paraneoplastic encephalomyelitis/sensory neuronopathy (PEM/SN), the expression of HuC as well as HuD mRNAs and their proteins was studied in 11 lung cancer cell lines. " 04/01/2001 - "The anti-Hu antibody (HuAb) is directed against RNA-associated neuronal proteins and is known to cause paraneoplastic encephalomyelitis/sensory neuronopathy syndrome mostly when associated with small cell lung cancer (SCLC). " 05/01/1995 - "Type 1 anti-neuronal nuclear antibodies (ANNA-1) associated with paraneoplastic encephalomyelitis and small-cell lung carcinoma stain the nucleus and cytoplasm of all neurons, and react with a group of 35- to 40-kd proteins in neuronal immunoblots. " 12/10/2009 - "Most patients with paraneoplastic encephalomyelitis/sensory neuronopathy PEM/SN have small-cell lung cancer (SCLC) and develop antibodies against neuronal-specific Hu proteins, which are abnormally expressed in the tumor. " 01/01/1995 - "Patients with neuroendocrine-related tumors and paraneoplastic encephalomyelitis (PEM) or paraneoplastic sensory neuronopathy (PSN) develop high titers of antibodies, called anti-Hu, against neuronal proteins expressed in their tumors, usually small cell lung cancer (SCLC). "
8.	Anti-Idiotypic AntibodiesIBA 01/01/2008 - "Anti-Hu-antibodies (anti-Hu-Ab) are typically associated with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/PSN), and low titres of anti-Hu-Ab, were found in newly diagnosed Small Cell Lung Cancer (SCLC). " 11/01/1998 - "This immune response is characterized by antibodies (anti-Hu) that at high titers are associated with a disease called paraneoplastic encephalomyelitis/sensory neuronopathy, in which infiltrates of T cells are found in the tumor and nervous system. " 01/01/2010 - "Anti-Hu-antibodies (anti-Hu-Ab) are typically associated with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/PSN), and low titres of anti-Hu-Ab were found in neural/neuroendocrine neoplasms, especially small cell lung cancer (SCLC). " 06/01/1997 - "Paraneoplastic limbic encephalitis (LE) is considered a particular manifestation of paraneoplastic encephalomyelitis (PEM), a remote effect of cancer almost always associated with anti-neuronal antibodies (anti-Hu; also called ANNA 1) and small cell lung carcinoma (SCLC). " 12/01/1996 - "Patients with small-cell carcinoma of the lung and paraneoplastic encephalomyelitis develop antibodies (anti-Hu) against HuD, a member of a family of neuronal specific RNA-binding proteins, which are also expressed by the tumor. "
9.	Complementary DNA (cDNA)IBA 09/01/2006 - "By serological screening of a rat cerebellum cDNA expression library using anti-Hu-positive sera from three patients with paraneoplastic encephalomyelitis (PEM), we identified an open reading frame encoding an isoform of the BTB-kelch protein KLHL7. " 01/01/1994 - "A cDNA clone coding for a human brain protein (HuD), which is highly homologous to the Drosophila neuronal determination protein Elav and elicits antibody formation in a high proportion of patients with paraneoplastic encephalomyelitis, was used to isolate a lambda phage recombinant clone, including a large fragment of the relevant human genomic region. " 08/01/1994 - "Hel-N1 is a novel human complementary DNA encoding a neuronal RNA-binding protein which shares considerable sequence homology with the HuD protein, a target of type I anti-neuronal nuclear antibodies in patients with paraneoplastic encephalomyelitis. "
10.	Immunoglobulin G (IgG)IBA 01/01/1994 - "Analysis of the IgG subclass distribution and inflammatory infiltrates in patients with anti-Hu-associated paraneoplastic encephalomyelitis." 01/01/1994 - "Using immunohistochemistry, we studied the IgG subclass distribution of the anti-Hu antibody in serum, nervous system, and tumor of patients with anti-Hu-associated paraneoplastic encephalomyelitis/sensory neuropathy (PEM/PSN). " 10/01/1995 - "Paraneoplastic encephalomyelitis/paraneoplastic sensory neuronopathy (PEM/PSN) associated with small cell lung cancer is characterized by high serum and CSF titers of anti-neuronal (anti-Hu) antibodies and by intrathecal synthesis of anti-Hu IgG. A pathologic role for the anti-Hu antibodies in PEM/PSN is further suggested by reported intraneuronal accumulation of the antibodies in the nervous system of PEM/PSN patients at autopsy. " 10/01/2004 - "We have employed isoelectric focusing and peroxidase-labeled anti-IgG or 35S-labeled Hu or Yo antigens to identify oligoclonal bands (OCB) representing either total IgG or Hu or Yo antibodies in serum and CSF of patients with paraneoplastic encephalomyelitis (PEM) or paraneoplastic cerebellar degeneration (PCD). "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 03/01/1992 - "The case is presented of a woman with severe paraneoplastic encephalomyelitis that was characterized predominantly by cerebellar degeneration associated with small cell lung cancer, both of which responded rapidly to cytotoxic chemotherapy alone. " 05/01/1996 - "Two patients with paraneoplastic encephalomyelitis manifested predominantly as subacute sensory neuronopathy did not improve after prednisone treatment and chemotherapy. "
2.	Plasmapheresis 03/01/1992 - "We retrospectively evaluated the effect of plasmapheresis (PE) in seven patients with paraneoplastic encephalomyelitis (PEM), small-cell lung carcinoma, and anti-Hu antibodies, and four patients with paraneoplastic cerebellar degeneration (PCD), ovarian or breast cancer, and anti-Yo antibodies. "
3.	Immunotherapy 11/10/1999 - "To evaluate 1) the effect of the tumor treatment on the clinical course of paraneoplastic encephalomyelitis (PEM) with anti-Hu antibodies, 2) the impact of immunotherapy on the tumor evolution, and 3) the outcome of the small cell lung cancer (SCLC) of PEM patients compared with that of patients without PEM. "