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At least 19 recordsLinked to original sources
PubMed1992

Pathogenesis of central nervous system paraneoplastic syndromes.

The pathogenesis of central nervous system paraneoplastic syndromes has not been established. Increasing evidence supports the hypothesis that CNS paraneoplastic syndromes are immune disorders arising from an immune response to the underlying neoplasm. The hypothesis is that antigens or epitopes shared between the tumor and the nervous system are recognized as foreign by the host and elicit an immune response. The immune response is directed appropriately against the tumor leading to a more indolent course in those patients with paraneoplastic syndromes but is misdirected against the nervous system often leading to severe nervous system destruction. The evidence for this hypothesis consists of: 1) The finding of high titer autoantibodies that react with antigens in both tumor and central nervous system. 2) Intrathecal synthesis of the antibody. 3) The presence of the antibody in the central nervous system at autopsy. The development of animal models will be required to determine the exact nature of the pathogenesis of these disorders.

Autoantibodies↗
PubMed2000

Paraneoplastic syndromes of the nervous system.

Paraneoplastic syndromes affecting the nervous system are unique among immune-mediated disorders in that the trigger of the immune response is known: tumor expression of proteins normally restricted to neurons (or other immunoprivileged sites, such as testis) but ectopically expressed in some cancers results in an immunological response characterized by high titers of antibodies targeting the "onconeuronal" antigen. A T-cell response is also elicited in some paraneoplastic syndromes and may be the cause of neuronal destruction. In some instances genes that code for the antigens recognized by the autoantibodies have been identified, cloned and sequenced. Some of the proteins so identified are RNA binding proteins but their specific function has not been identified. In some individuals with cancer but no paraneoplastic syndrome, low titers of antibody can be identified in the serum. Low titers of antibody are associated with a better prognosis of the cancer. Experimental animals immunized against a paraneoplastic antigen are partially protected against tumors that express that antigen.

Animals↗
PubMed1997

Paraneoplastic syndromes affecting the nervous system.

Paraneoplastic syndromes can affect virtually any portion of the nervous system. Most paraneoplastic syndromes are believed to be caused by an autoimmune reaction to an "onconeural" antigen shared by the cancer and the nervous system. The immune reaction may retard growth of the cancer, but it also damages the nervous system. Specific autoantibodies found in some individual paraneoplastic syndromes are usually associated with specific tumors. Neurological disorders, clinically and pathologically identical to paraneoplastic syndromes, may occur in some patients without cancer, but paraneoplastic antibodies are not found in these patients. The diagnosis of a paraneoplastic syndrome is based on its increased incidence in patients with cancer, the occasional response of the neurological syndrome to treatment of the underlying cancer, or the presence of specific autoantibodies. Some paraneoplastic syndromes respond to treatment of the underlying cancer or to immunosuppression but, for most syndromes, no effective treatment exists.

Autoantibodies↗
PubMed1997

Paraneoplastic syndromes affecting the central nervous system.

Paraneoplastic syndromes affecting the nervous system are rare neurologic syndromes caused by cancer but not ascribable to metastases. Any portion of the nervous system may be involved in a paraneoplastic syndrome. The pathogenesis of these disorders appears to be an immune reaction against antigens shared by the cancer and the nervous system. Some disorders, such as the Lambert-Eaton myasthenic syndrome, are effectively treated by removal of autoantibodies directed against the presynaptic cholinergic synapse. In other disorders, such as paraneoplastic cerebellar degeneration or paraneoplastic sensory neuronopathy, neither removal of the autoantibody nor treatment of the cancer is effective.

Autoantibodies↗
PubMedDate not supplied

[Paraneoplastic syndromes of the central nervous system].

Paraneoplastic syndromes of central nervous system are rare neurologic syndromes caused by cancer but not secondary to metastases. The physiopathologic mechanisms underlying these syndromes are still under debate. We report the biological and clinical features of the most frequent paraneoplastic syndromes involving the central nervous system. Their early clinical identification might be an useful marker of an otherwise unknown visceral malignancy. Furthermore, they might also be suggestive for the particular type of cancer present. Once, therefore, the diagnosis of these paraneoplastic syndromes has been established, an appropriate evaluation for the asymptomatic neoplasm in cancer-free individuals or investigation for the malignancy recurrences in oncologic patients might be performed.

Diagnosis, Differential↗
PubMed1996

Paraneoplastic nervous system syndromes.

Recent progress in the understanding of the paraneoplastic neurologic syndromes has included further deliniation of the clinical syndromes and their treatment, attempts at standardization of the diagnostic nomenclature, investigations of pathogenetic mechanisms, and molecular characterization of the paraneoplastic antigens with implications as to their biologic relevance. Despite more than 30 years of investigation, the pathogenesis of these presumably autoimmune conditions remains unclear. Furthermore, no effective therapy has been identified for these conditions, with the possible exception of the opsoclonus-myoclonus ataxia and Lambert-Eaton myasthenic syndromes. Future investigations must focus on the disrupted genetic regulatory events involved in generation and propagation of the autoimmune response, antigen presentation and processing; target cell destruction, and consideration of alternative pathologic causes. Effective management strategies are most likely to develop from a better understanding of the disease pathogenesis, the development of animal model systems, and a creative look beyond the usual therapies employed in autoimmune conditions.

Antibody Specificity↗
PubMed1998

Stiff-man syndrome in a woman with breast cancer: an uncommon central nervous system paraneoplastic syndrome.

We report a patient who developed stiff-man syndrome, including disabling shoulder subluxation and wrist ankylosis, in association with breast cancer. Immunologic investigations disclosed autoimmunity directed against not only glutamic acid decarboxylase but also amphiphysin, a 128-kd protein located in the presynaptic compartment of neurons. The patient improved after surgery and corticosteroid treatment and has been stable for nearly 4 years on only anti-estrogenics. The triad of stiff-man syndrome, breast cancer, and autoantibodies against amphiphysin identifies a new autoimmune paraneoplastic syndrome of the CNS.

Animals↗
PubMed1992

Clinical and pathological advances on central nervous system paraneoplastic syndromes.

In the last decade, several features have improved our knowledge of CNS paraneoplastic syndromes. Patients with paraneoplastic cerebellar degeneration (PCD) and breast or ovarian cancer, but not with other tumors, harbor an antibody against Purkinje cells (called anti-Yo). Clinical features of anti-Yo positive and negative PCD are similar but the latter may have a less progressive clinical course with occasional remissions. In addition to the association of opsoclonus with neuroblastoma, this syndrome has been identified in patients with breast or small-cell lung cancer (SCLC). Patients with opsoclonus and breast cancer have an antineuronal antibody (called anti-Ri) not present if opsoclonus is associated with SCLC or neuroblastoma. Paraneoplastic encephalomyelitis (PEM) is almost always associated with SCLC. Most patients present with sensory neuronopathy, limbic or brainstem encephalitis but involvement of multiple levels is usual. An antibody (called anti-Hu) against neuronal nuclear antigens is present in patients with PEM and SCLC. Autopsy studies demonstrate deposits of anti-Hu specific IgG in the neurons and a predominance of T cells in the inflammatory infiltrates. Treatment of the tumor and immunosuppressors are effective in opsoclonus whereas patients with PCD or PEM with circulating antibodies do not improve.

Brain Diseases↗
PubMed1997

Paraneoplastic syndromes.

Most nervous system paraneoplastic syndromes probably result from an immune attack against antigens normally expressed only in the nervous system but aberrantly expressed in a cancer. Specific antibodies in serum and cerebrospinal fluid that react with the nervous system and the cancer can be used to characterize proteins usually restricted to the nervous system and to identify neurologic disorders as paraneoplastic caused by a specific tumor type.

Animals↗
PubMed1985

Paraneoplastic syndromes of the nervous system.

The paraneoplastic disorders of the nervous system are clinicopathologic syndromes that occur in association with systemic cancer. Dysfunction of any part of the nervous system or its effector organs may be produced by a paraneoplastic process. The temporal relationship between the onset of a paraneoplastic syndrome and the diagnosis of the associate cancer varies. Most of the paraneoplastic syndromes of the nervous system may appear months to years before the cancer is discovered. The pathogenesis of most of the paraneoplastic syndromes of the nervous system is unknown and specific, effective therapy is lacking. With rare exceptions, treatment of the associated cancer seldom alters the course of patients with these syndromes. However, in other paraneoplastic syndromes such as myasthenia gravis, myasthenic syndrome of Eaton-Lambert and polymyositis-dermatomyositis an autoimmune pathogenesis is established or strongly suspected.

Cerebellar Diseases↗
PubMed1987

Autoimmune pathogenesis of paraneoplastic neurological syndromes.

"Remote effects" of cancer on the nervous system (paraneoplastic syndromes) are disorders of the nervous system of unknown cause that occur almost exclusively, or with greatly increased frequency, in patients with identifiable or occult cancer. There are several hypotheses concerning the pathogenesis of these rare disorders. One hypothesis is that the underlying tumor and portions of the nervous system share antigens and that an autoimmune response generated against the tumor causes the nervous system disorder. Evidence supporting this hypothesis includes the ability to transmit the Lambert-Eaton Syndrome (a paraneoplastic syndrome involving the neuromuscular junction) to experimental animals by infusing IgG from patients with the disorder, the presence of autoantibodies against Purkinje cell neurons in some patients with paraneoplastic cerebellar degeneration, and the presence of autoantibodies against many neurons in patients with sensory neuronopathy and encephalomyelitis. Other evidence supporting the hypothesis is presented in this review.

Antibodies, Monoclonal↗
PubMed1989

The remote effects of cancer on the nervous system.

Paraneoplastic syndromes are heterogeneous in their clinical presentations and their associations with particular tumor types and are an important part of the differential diagnosis of neurologic dysfunction in patients with or without a known neoplasm. Patients presenting with one of the more distinctive syndromes, such as subacute cerebellar degeneration, opsoclonus-myoclonus, and the Lambert-Eaton syndrome, should undergo a careful evaluation for the presence of an occult malignancy. The importance of looking for a monoclonal gammopathy in patients with certain polyneuropathies and motor neuron syndromes is also becoming clear. At this time, an autoimmune pathogenesis has been clearly demonstrated only for the Lambert-Eaton syndrome. Specific autoantibodies in other syndromes appear to be valuable diagnostic markers for the presence of an underlying malignancy, but the actual role of these antibodies in producing tissue damage and clinical disease is still unknown.

Brain Diseases↗
PubMed1998

[Immunology in the clinical practice. XVI. Paraneoplastic syndromes of the nervous system: pathogenesis and diagnosis].

Paraneoplastic neurological syndromes are believed to result from ectopic expression of onconeural antigens by tumours. The resulting immune response is not only directed against the tumour but also cross-reacts with the same or similar antigens in the nervous system. The immune response generates high titred autoantibodies that are associated with specific tumours and neurological syndromes. Paraneoplastic autoantibodies help diagnose neurological syndromes and help direct the search for an underlying tumour. In paraneoplastic syndromes, the course of the tumour is relatively mild. Detection of the autoantibodies might lead to early diagnosis and immunomodulation and anti-tumour treatment before irreversible neuronal cell loss and deficits set in.

Antibodies, Neoplasm↗
PubMed2006

Paraneoplastic syndromes affecting the nervous system.

The paraneoplastic neurologic disorders (PND) are a diverse group of diseases characterized by the presence of neurologic dysfunction in the setting of a remote cancer. PND can affect almost any part of the nervous system, and are most commonly associated with lung cancer (small cell) and gynecologic tumors. Laboratory studies have demonstrated that an autoimmune response links the neurologic disorder and the cancer, and established a model whereby the cancer is believed to initiate the syndrome by expressing a protein antigen normally expressed in the nervous system, leading to anti-tumor immune response followed by autoimmune neurologic symptoms. We review the currently known PND and their pathogenesis.

Autoantibodies↗
PubMed2004

Paraneoplastic opsoclonus-myoclonus syndrome associated with malignant fibrous histiocytoma: neuropathological findings.

Paraneoplastic syndromes related to soft tissue tumors are very uncommon and an association of paraneoplastic diseases with a malignant fibrous histiocytoma (MFH) has not been reported so far. Opsoclonus-myoclonus is a rare paraneoplastic nervous system syndrome, that was well documented in adult patients with neoplasms particularly of the lung and breast. A 77-year-old woman developed typical opsoclonus-myoclonus syndrome and rapidly progressed to coma. The opsoclonus and generalized myoclonus continued until the patient's death seven months later. An autopsy examination identified a pleomorphic MFH in the retroperitoneum without a metastatic spread. Microscopic examination of the cerebellum revealed an atrophy of the granular layer along with a marked patchy loss of Purkinje cells, and a loss of neurons in the cerebellar nuclei accompanied by distinct peridental demyelination and astrogliosis. A moderate loss of neurons and neuronal chromatolysis were observed also in the inferior olivary nuclei. However, the omnipause neurons of the nucleus raphe interpositus, that are supposed to be responsible for opsoclonus generation under pathological conditions, as well as ocular brain stem nuclei were all intact. We failed to prove the presence of known antineuronal anti-Yo, anti-Hu or anti-Ri autoantibodies in both serum and cerebrospinal fluid (CSF) of the patient. However, the confirmation of the intrathecal IgG synthesis by oligoclonal bands in CSF and the immunohistochemical detection of IgG deposits on membranes of Purkinje cells, neurons of cerebellar granular layer and in cerebellar nuclei in our case support the presumed autoimmune nature of the disease.

Aged↗
PubMed2002

[Paraneoplastic neurologic autoimmune diseases].

All complications of tumor disease that do not result from the tumor itself, its metastases, or vascular, infectious, metabolic, or therapy-related causes are referred to as paraneoplastic neurologic syndromes. This earlier represented an excluding diagnosis and the association was established only statistically, but since the first description of the anti-Hu antibody in 1985, we know that around 2/3 of patients with paraneoplastic disorders display highly specific antibody reactions in the serum. These reactions not only prove a paraneoplastic etiology in almost 100% of cases, but, due to their highly specific associations with certain tumor types, also aid tumor detection in patients whose tumor types are not known.

Animals↗
PubMed1997

Neurological paraneoplastic syndromes.

Paraneoplastic neurological syndromes are uncommon, however, their diagnosis is of major practical importance. The identification of antibodies in the serum or cerebrospinal fluid in central nervous system paraneoplastic syndromes confirms the clinical diagnosis of a paraneoplastic syndrome and allows early identification of an underlying tumour at a stage when it is localised and more amenable to treatment. The failure to identify antibodies in patients with characteristic presentations of underlying neurological paraneoplastic syndromes does not exclude an underlying cancer. Necrotising myelopathy, dermatomyositis, and chronic inflammatory demyelinating polyneuropathy all occur more frequently than expected in patients with cancer but autoantibodies have not yet been identified. Although significant advances have been made in diagnosis, further research is needed in the detection of autoantibodies and the elucidation of their role in the aetiology of neurological disease.

Autoantibodies↗