RESUMO
Prostate cancer is the most common cancer among American and European men. Nervous system affection caused by local tumor growth or osseous metastases are the main causes of neurological symptoms in prostate cancer patients. Prostate cancer is rarely reported in association with paraneoplastic neurological syndromes (PNS). We have, therefore, studied clinical and paraclinical findings of a series of patients with prostate cancer and PNS, and reviewed cases reported in the literature. Case histories of 14 patients with definite PNS from the PNS Euronetwork database and from the authors' databases were reviewed. A PubMed literature search identified 23 patients with prostate cancer and PNS. Thus, a total of 37 case histories were reviewed with respect to syndrome type, cancer evolution, paraclinical investigations, antibody status, treatment and outcome. The three most frequent isolated PNS were paraneoplastic cerebellar degeneration, paraneoplastic encephalomyelitis (PEM)/limbic encephalitis and subacute sensory neuronopathy (SSN). Onconeural antibodies were detected in 23 patients, in most cases the Hu antibody (17 patients, 74 % of all antibody-positive cases). Other well-characterized onconeural antibodies (Yo, CV2/CRMP5, amphiphysin, VGCC antibodies) were found in a minority. PNS was diagnosed prior to prostate cancer diagnosis in 50 % of the cases. The association of PNS with prostate cancer is quite infrequent, but clinically important. PNS often heralds prostate cancer diagnosis. Syndromes associated with Hu antibodies predominate. Another tumor more prone to associate with PNS should always be excluded.
Assuntos
Autoanticorpos/sangue , Proteínas ELAV/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/complicações , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Neoplasias da Próstata/complicações , Neoplasias da Próstata/imunologia , Idoso , Idoso de 80 Anos ou mais , Bases de Dados Factuais , Europa (Continente) , Humanos , Masculino , Pessoa de Meia-Idade , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico por imagem , Síndromes Paraneoplásicas do Sistema Nervoso/terapia , Estudos Prospectivos , Neoplasias da Próstata/diagnóstico por imagem , Neoplasias da Próstata/terapia , Resultado do TratamentoRESUMO
Pseudoprogression is a treatment-related effect seen on imaging in high-grade glioma. Enhancement of gadolinium contrast on control MRI can be misinterpreted as tumor recurrence and is also difficult to distinguish from radiation necrosis. Pseudoprogression is seen in up to 30% after standard treatment for glioblastoma multiforme (GBM), which is radiotherapy concurrent with chemotherapy with temozolomide (TMZ) and adjuvant cycles of TMZ. In this article, the current literature on pseudoprogression in high-grade glioma is reviewed by searches in PubMed. We also present two clinical cases, one of which had medullary pseudoprogression. No articles on this subentity of pseudoprogression were found in PubMed. Standard MRI with gadolinium contrast cannot differentiate between pseudoprogression, tumor recurrence and radiation necrosis. More advanced imaging techniques are often not available. Pseudoprogression seems to be related to methylated promoter of the O(6)--methyl-guanine methyl transferase (MGMT) gene, which is associated with improved treatment effect. Discontinuation or change of therapy on the basis of misinterpretation of MRI as disease progression is thus unfortunate. MRI should be interpreted with caution the first 6 months after standard treatment of high-grade glioma. In a GBM patient with contrast enhancement on MRI but few or no new symptoms and/or stable steroid doses, treatment should be continued and control imaging performed after 2-3 months.
Assuntos
Neoplasias Encefálicas/fisiopatologia , Glioma/fisiopatologia , Adulto , Neoplasias Encefálicas/diagnóstico , Neoplasias Encefálicas/genética , Metilases de Modificação do DNA/genética , Enzimas Reparadoras do DNA/genética , Progressão da Doença , Feminino , Predisposição Genética para Doença , Glioma/diagnóstico , Glioma/genética , Humanos , Imageamento por Ressonância Magnética , Lesões por Radiação/diagnóstico , Estudos Retrospectivos , Proteínas Supressoras de Tumor/genéticaRESUMO
BACKGROUND: Onconeural antibodies are strongly associated with cancer and paraneoplastic neurological syndromes (PNS). Most of these antibodies are well-characterized (antibodies against Hu, Yo, Ri, CRMP5, amphiphysin, Ma2 and Tr) and are in common use for the diagnosis of definite PNS. MATERIALS AND METHODS: Literature on detection and clinical significance of onconeural antibodies were identified by using relevant search terms in PubMed and reviewed. CONCLUSIONS: The onconeural antibodies are directed against intracellular antigens and their pathogenic role is still largely unknown. They are highly specific markers of paraneoplastic aetiology in patients with neurological symptoms. Detection of an onconeural antibody in a patient with neurological symptoms should lead to prompt investigation for cancer. However, absence of detectable onconeural antibodies does not exclude the PNS diagnosis. In particular, failure to detect antibodies in patients without classical PNS symptoms may result in less vigorous cancer screening and diagnostic delay. Neuronal antibodies that are directed to synaptic proteins or proteins of the cell membrane are also associated with neurological symptoms, and probably have pathogenic effects. The association between these antibodies and cancer is less robust, and they are usually not included among the onconeural antibodies.
Assuntos
Anticorpos Antineoplásicos/isolamento & purificação , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico , Anticorpos Antineoplásicos/imunologia , Humanos , Síndromes Paraneoplásicas do Sistema Nervoso/imunologiaRESUMO
Autoimmune limbic encephalitis (LE) can arise both by paraneoplastic and non-paraneoplastic mechanisms. Patients with LE usually have a subacute onset of memory impairment, disorientation and agitation, but can also develop seizures, hallucinations and sleep disturbance. The following investigations may aid the diagnosis: analysis of cerebrospinal fluid (CSF), electroencephalography, magnetic resonance imaging, fluorodeoxyglucose positron emission tomography and neuronal antibodies in the serum and CSF. Neuronal antibodies are sometimes, but not always, pathogenic. Autoimmune LE may respond to corticosteroids, intravenous IgG (IVIG) or plasma exchange. The cornerstone of paraneoplastic LE therapy is resection of the tumour and/or oncological treatment. Several differential diagnoses must be excluded, among them herpes simplex encephalitis.
Assuntos
Doenças Autoimunes do Sistema Nervoso/patologia , Doenças Autoimunes do Sistema Nervoso/fisiopatologia , Encefalite Límbica/patologia , Encefalite Límbica/fisiopatologia , Autoanticorpos , Doenças Autoimunes do Sistema Nervoso/diagnóstico , Doenças Autoimunes do Sistema Nervoso/terapia , Diagnóstico Diferencial , Eletroencefalografia , Humanos , Imunoterapia , Encefalite Límbica/diagnóstico , Encefalite Límbica/terapia , Imageamento por Ressonância Magnética , Tomografia por Emissão de PósitronsRESUMO
INTRODUCTION: Immunohistochemical studies of paraneoplastic cerebellar degeneration (PCD) are rare, and the findings vary. MATERIALS AND METHODS: We performed morphological and immunohistochemical characterization of the brain, medulla and tumour of two patients with PCD, Yo antibodies and ovarian adenocarcinoma. RESULTS: The cerebellum of both patients had extensive loss of Purkinje cells. Microglia activation and T cells were found in the cerebellum, but B cells or deposits of IgG or complement were not detected. Microglia activation was also present in the brain stem and medulla. T cells were found in the ovarian adenocarcinoma. CONCLUSION: PCD is characterized by loss of Purkinje cells and microglia activation, and the presence of T cells indicates cellular immune reactions in PCD and in ovarian cancer.
Assuntos
Adenocarcinoma/patologia , Autoanticorpos/imunologia , Proteínas do Tecido Nervoso/imunologia , Neoplasias Ovarianas/patologia , Degeneração Paraneoplásica Cerebelar/patologia , Células de Purkinje/patologia , Adenocarcinoma/imunologia , Idoso de 80 Anos ou mais , Feminino , Gliose/imunologia , Gliose/patologia , Humanos , Imuno-Histoquímica , Microglia/imunologia , Microglia/patologia , Pessoa de Meia-Idade , Neoplasias Ovarianas/imunologia , Degeneração Paraneoplásica Cerebelar/imunologia , Células de Purkinje/imunologiaRESUMO
Proteasome antibodies were detected by enzyme-linked immunosorbent assay in two of the 45 (4.4%) patients with lung cancer, 0 of the 39 patients with breast cancer and six of the 51 (11.8%) patients with ovarian cancer. Six of the 47 (12.8%) patients with relapsing remitting multiple sclerosis had proteasome antibodies, as well as two of the 100 (2%) blood donors. Significant higher odds ratios compared to the blood donors were found for the patients with ovarian cancer (OR: 6.4; 95% CI: 1.1-68) and multiple sclerosis (OR: 7.1; 95% CI: 1.2-74). There was no association between proteasome antibodies and metastases or onconeural antibodies. The antibodies showed reactivity to 23, 25 and 27 kD proteins of the 20S proteasome using Western blot. The increased prevalence of proteasome antibodies in patients with ovarian cancer or multiple sclerosis may reflect cellular damage and release of intracellular antigens. Whether the antibodies take part in the clearance of released proteasomes and thus participate in the pathogenesis of cancer or autoimmune disease is not known.
Assuntos
Autoanticorpos/sangue , Esclerose Múltipla/sangue , Neoplasias/sangue , Complexo de Endopeptidases do Proteassoma , Ensaio de Imunoadsorção Enzimática/métodos , Humanos , Peso Molecular , Complexo de Endopeptidases do Proteassoma/química , Complexo de Endopeptidases do Proteassoma/imunologiaRESUMO
We present a case with subacute limbic encephalitis (LE) and thymoma. Neither classical onconeural antibodies nor antibodies to voltage gated potassium channels (VGKC) were detected, but the serum was positive for anti-glutamic acid decarboxylase (GAD). The patient serum also stained synaptic boutons of pyramidal cells and nuclei of granule cells of rat hippocampus. The objective of the study was to identify new antibodies associated with LE. Screening a cDNA expression library identified collapsin response mediator protein 3 (CRMP3), a protein involved in neurite outgrowth. The serum also reacted with both CRMP3 and CRMP4 by Western blot. Similar binding pattern of hippocampal granule cells was obtained with the patient serum and rabbit anti-serum against CRMP1-4. The CRMP1-4 antibodies stained neuronal nuclei of a biopsy from the patient's temporal lobe, but CRMP1-4 expression in thymoma could only be detected by immunoblotting. Absorption studies with recombinant GAD failed to abolish the staining of the hippocampal granule cells. Our findings illustrate that CRMP3-4 antibodies can be associated with LE and thymoma. This has previously been associated with CRMP5.
Assuntos
Autoanticorpos/análise , Encefalite Límbica/etiologia , Proteínas Musculares/imunologia , Timoma/complicações , Neoplasias do Timo/complicações , Animais , Western Blotting , Células Cultivadas , DNA Complementar/genética , Biblioteca Gênica , Hipocampo/imunologia , Humanos , Encefalite Límbica/imunologia , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Neurônios/imunologia , Síndromes Paraneoplásicas/etiologia , Síndromes Paraneoplásicas/imunologia , Ratos , Lobo Temporal/imunologiaRESUMO
The aim of the study was to search for novel targets of autoantibodies in patients with paraneoplastic neurological syndromes (PNS). PNS are mediated by immune reactions against autoantigen(s) shared by the cancer cells and the nervous system. 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. Immunohistochemical studies demonstrated that the KLHL7 protein is expressed in the nuclei of neurones, but not in other tissues including various cancers. However, the KLHL7 protein was detected in the nuclei of cancer cell lines. Antibodies to KLHL7 were detected by an immunoprecipitation assay in sera from 12 of 254 (4.7%) patients with various cancers and 2 of 170 blood donors (1.2%). None of 50 sera from patients with multiple sclerosis were positive for KLHL7 antibodies. Sixteen patients with classical PNS and anti-Hu or anti-Yo antibodies were also negative for KLHL7 antibodies. Seven cancer patients with KLHL7 antibodies had various signs of neurological disease that could be related to cancer, whereas the remaining five seropositive cancer patients had no clinical signs of possible PNS. The present results indicate that KLHL7 antibodies are associated with various cancers, and in some patients also with neurological disease. Whether KLHL7 antibodies can be used as paraneoplastic markers for PNS remains to be determined.
Assuntos
Autoanticorpos/sangue , Autoantígenos/sangue , Neoplasias/sangue , Polineuropatia Paraneoplásica/sangue , Sequência de Aminoácidos , Animais , Autoanticorpos/biossíntese , Autoanticorpos/metabolismo , Autoantígenos/genética , DNA Recombinante , Biblioteca Gênica , Humanos , Dados de Sequência Molecular , Polineuropatia Paraneoplásica/imunologia , RNA Mensageiro/química , Ratos , Distribuição TecidualRESUMO
OBJECTIVES: To review autopsy findings in paraneoplastic cerebellar degeneration. MATERIALS AND METHODS: We report the autopsy results of two individuals with paraneoplastic cerebellar degeneration and ovarian cancer. RESULTS: Both patients had extensive loss of cerebellar Purkinje cells and general activation of microglia in the central nervous system, as well as signs of immunactivation in the medulla. CONCLUSION: The immunoactivation in PCD is widespread and involving more than the cerebellum.
Assuntos
Carcinoma/patologia , Neoplasias Ovarianas/patologia , Degeneração Paraneoplásica Cerebelar/patologia , Idoso de 80 Anos ou mais , Autopsia , Feminino , Humanos , Pessoa de Meia-Idade , Células de Purkinje/fisiologiaRESUMO
Onconeural antibodies are found in patients with cancer and are associated with paraneoplastic neurological syndromes (PNS). The objective of the present study was to assess the frequency of Yo antibodies in ovarian and breast cancer using a sensitive immunoprecipitation technique, and to look for any association of Yo antibodies with neurological symptoms and prognostic factors. A multiwell adapted fluid-phase immunoassay using radiolabelled recombinant cerebellar degeneration related protein (cdr2), produced by coupled in vitro transcription/translation was used for the detection of Yo antibodies. This technique combines high specificity and sensitivity with high sample analysing capacity for the antibody in question. Sera or EDTA-blood from 810 ovarian (n = 557) and breast cancer (n = 253) patients were analysed for Yo antibodies by immunoprecipitation, as well as immunofluorescence and immune blots. Two hundred healthy blood donors and sera from 17 patients with paraneoplastic cerebellar degeneration and Yo antibodies served as controls. Immunoprecipitation was more sensitive in detecting Yo antibodies than immunofluorescence and immune blots. The prevalence of Yo antibodies was 13/557 (2.3%) in ovarian cancer and 4/253 (1.6%) in breast cancer using immunoprecipitation. Yo antibodies were not correlated with specific histological subgroups. The Yo index of ovarian cancer patients in FIGO stage IV was higher compared to FIGO stage I-III. The prevalence of Yo antibodies was 3 times higher in patients with stage III breast cancer than in stage I and II. Only 2/17 (11.8%) patients with Yo antibodies detected during the screen of 810 cancer patients had PNS. The results show that the prevalence of Yo antibodies is low in ovarian and breast cancer. Yo antibodies may be associated with advanced cancer, but less often with PNS.
Assuntos
Anticorpos Antineoplásicos/análise , Neoplasias da Mama/imunologia , Imunoprecipitação/métodos , Proteínas do Tecido Nervoso/imunologia , Neoplasias Ovarianas/imunologia , Adenocarcinoma/imunologia , Idoso , Idoso de 80 Anos ou mais , Neoplasias da Mama/patologia , Feminino , Humanos , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Neoplasias Ovarianas/patologia , Degeneração Paraneoplásica Cerebelar/imunologiaRESUMO
Autoantibodies against many proteins are common in sera from patients with various types of cancer. These antibodies are sometimes involved in the development of conditions associated with cancer, such as paraneoplastic neurologic disorders. We used a human brain cDNA expression library and serum from a paraneoplastic neurologic disorder patient to search for new autoantigens in the nervous system. Pyridoxal phosphatase was identified as a novel autoantigen. Expression studies showed that pyridoxal phosphatase was strongly expressed in various parts of the central nervous system. Sera contained antibodies against pyridoxal phosphatase in 22 of 243 (9.1%) patients with lung cancer and eight of 113 (7.1%) with other forms of cancer vs two of 88 (2.3%) healthy control subjects. In addition, 2-4% of patients with different autoimmune diseases had autoantibodies against pyridoxal phosphatase. None of the antipyridoxal phosphatase-positive patients were known to have a paraneoplastic neurologic disorder. Hence, autoantibodies against pyridoxal phosphatase correlate with cancer but not necessarily with the subset of patients with paraneoplastic neurological disorders although serum from such a patient was used to screen the cDNA library. This study showed that yet another enzyme involved in pyridoxal 5'-phosphate metabolism is an autoantigen. Thus, pyridoxal 5'-phosphate seems to be a common denominator for autoantigens involved in autoimmune diseases.
Assuntos
Anticorpos Antineoplásicos/análise , Autoantígenos/imunologia , Doenças do Sistema Nervoso Central/enzimologia , Neoplasias/enzimologia , Monoéster Fosfórico Hidrolases/imunologia , Animais , Doenças Autoimunes/enzimologia , Doenças Autoimunes/imunologia , Encéfalo , Estudos de Casos e Controles , Diabetes Mellitus Tipo 1/enzimologia , Diabetes Mellitus Tipo 1/imunologia , Biblioteca Gênica , Humanos , Esclerose Múltipla/enzimologia , Esclerose Múltipla/imunologia , Coelhos , RadioimunoensaioRESUMO
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. We developed a new radioimmunobinding assay to detect Hu antibodies based on in vitro transcribed and translated (ITT) HuD. High levels of Hu antibodies were detected in all seven PEM/SSN patients tested, but not in any of 15 patients with other paraneoplastic syndromes, 20 patients with Sjögren's syndrome, 20 patients with miscellaneous tumors and 99 of 100 blood donors. One of the blood donors had low levels of Hu antibodies. The radiobinding assay detected Hu antibodies in 45 of 191 (24%) patients with SCLC, in comparison with immunofluorescence and immuno dot blot, where only 34 of 191 (18%) were detected. All patients with Hu antibodies detected by immunofluorescence and immuno dot-blot were also positive by the radioimmunobinding assay. The results demonstrate that this assay is very specific and sensitive for the detection of Hu antibodies.
Assuntos
Anticorpos/sangue , Anticorpos/imunologia , Proteínas do Tecido Nervoso/imunologia , Síndromes Paraneoplásicas do Sistema Nervoso/sangue , Síndromes Paraneoplásicas do Sistema Nervoso/imunologia , Proteínas de Ligação a RNA/imunologia , Radioimunoensaio/métodos , Proteínas ELAV , Proteína Semelhante a ELAV 4 , Feminino , Imunofluorescência/normas , Humanos , Immunoblotting/normas , Masculino , Neurônios Aferentes/imunologia , Neurônios Aferentes/patologia , Síndromes Paraneoplásicas do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso Periférico/sangue , Doenças do Sistema Nervoso Periférico/diagnóstico , Doenças do Sistema Nervoso Periférico/imunologia , Valor Preditivo dos Testes , Valores de Referência , Reprodutibilidade dos Testes , Síndrome de Sjogren/sangue , Síndrome de Sjogren/diagnóstico , Síndrome de Sjogren/imunologiaRESUMO
PURPOSE: Hu antibodies previously have been associated with longer survival of patients with small-cell lung cancer (SCLC). Voltage-gated calcium channel (VGCC) antibodies play a pathogenic role in Lambert Eaton myasthenic syndrome, which is also associated with SCLC. These antibodies may reduce tumor growth in patients with the neurologic disease, but it is not clear whether they provide prognostic information in those without neurologic symptoms. PATIENTS AND METHODS: Two hundred patients with SCLC (age 39 to 79 years; mean, 62.3 years; 129 males and 71 females) receiving chemotherapy were studied for the presence of Hu and VGCC antibodies. Sera were examined for Hu antibodies by an in vitro transcription-translation-based immunoprecipitation technique and by immunohistochemistry/dot blot. VGCC (P/Q subtype) antibodies were detected by radioimmunoassay. Survival analysis was used to analyze the data. Results Hu antibodies were detected in 51 of 200 patients (25.5%) by in vitro transcription-translation-based immunoprecipitation and in 37 of 200 patients (18.5%) by immunohistochemistry or dot blot, whereas VGCC antibodies were detected in only 10 of 200 patients (5%). The presence of Hu antibodies did not correlate with VGCC antibodies, and there was no association between Hu or VGCC antibodies and the extent of disease or survival. CONCLUSION: Hu and VGCC antibodies are found in a proportion of SCLC patients, irrespective of neurologic symptoms, but their presence does not correlate with the prognosis of the SCLC.
Assuntos
Anticorpos Antineoplásicos/análise , Canais de Cálcio Tipo N/imunologia , Carcinoma Pulmonar de Células não Pequenas/imunologia , Carcinoma Pulmonar de Células não Pequenas/mortalidade , Neoplasias Pulmonares/imunologia , Neoplasias Pulmonares/mortalidade , Adulto , Idoso , Análise de Variância , Anticorpos Antineoplásicos/imunologia , Biomarcadores/análise , Canais de Cálcio Tipo N/análise , Carcinoma Pulmonar de Células não Pequenas/patologia , Estudos de Coortes , Feminino , Técnica Indireta de Fluorescência para Anticorpo , Humanos , Ativação do Canal Iônico , Neoplasias Pulmonares/patologia , Masculino , Pessoa de Meia-Idade , Estadiamento de Neoplasias , Prognóstico , Estudos Prospectivos , Radioimunoensaio , Sensibilidade e Especificidade , Estatísticas não Paramétricas , Taxa de SobrevidaRESUMO
Neuropathy is a quite common finding in cancer patients. If the cancer is known, the main clinical problem is often whether the symptoms and signs are caused by metastasis or recurrence, or if the neuropathy can be related to effects of the oncological treatment. In addition to the clinical findings, radiological and neurophysiological examination can often be of considerable diagnostic help. Peripheral neuropathy may also be the first manifestation of the cancer, as in paraneoplastic neuropathies. Detecting the tumor in these cases may improve the oncological prognosis, as the neuropathy often precedes tumor symptoms by several years. A determined approach in searching for an occult cancer is necessary. The neuropathic symptoms are seldom life-threatening. However, the neuropathy may cause distressing symptoms, and symptomatic treatment and rehabilitation is often required.
Assuntos
Neoplasias/complicações , Doenças do Sistema Nervoso Periférico/etiologia , Antineoplásicos/efeitos adversos , Humanos , Plexo Lombossacral/patologia , Plexo Lombossacral/efeitos da radiação , Imageamento por Ressonância Magnética , Metástase Neoplásica/patologia , Neoplasias/metabolismo , Neoplasias/patologia , Doenças do Sistema Nervoso Periférico/induzido quimicamente , Doenças do Sistema Nervoso Periférico/patologia , Lesões por Radiação/patologiaRESUMO
Peripheral neuropathy is a common condition with a variety of causes. In some cases the neuropathy is the first symptom of an underlying malignancy. The frequency of occult cancer in patients with neuropathy is of importance in planning the investigations and follow-up of these patients. This retrospective study examined the incidence of cancer-related neuropathy among patients originally diagnosed with idiopathic peripheral neuropathy. Between 1981-1995 our department discharged 187 patients with the diagnosis of idiopathic peripheral neuropathy. Their names were cross-matched with the Norwegian Cancer Registry, and the medical records of the patients with known cancer were reviewed. In 14 patients (7.5%) we found cancer as the probable cause of the peripheral neuropathy originally classified as idiopathic. In eight of the patients the neuropathic symptoms preceded the tumour. In six cancer was already diagnosed when the neuropathic symptoms developed, but in three of these the neuropathy heralded a tumour recurrence. Idiopathic progressive neuropathy should always raise the suspicion of an underlying malignancy, especially if there are atypical accompanying symptoms and signs.
Assuntos
Neoplasias Primárias Desconhecidas/complicações , Doenças do Sistema Nervoso Periférico/etiologia , Adulto , Idoso , Diagnóstico Diferencial , Feminino , Humanos , Incidência , Masculino , Pessoa de Meia-Idade , Recidiva Local de Neoplasia , Neoplasias Primárias Desconhecidas/diagnóstico , Doenças do Sistema Nervoso Periférico/diagnóstico , Sistema de Registros/estatística & dados numéricos , Estudos RetrospectivosRESUMO
POEMS syndrome is a rare multisystemic disorder characterized by a variable constellation of polyneuropathy, organomegaly, endocrinopathy, monoclonal gammopathy and skin changes. The syndrome is believed to be immune-mediated and is probably related to the pathological monoclonal component caused by a myeloma or a plasmocytoma. The treatment is aimed at the underlying plasma cell dyscrasia. We have studied three patients with this rare syndrome. This article presents the clinical picture of each one and a survey of the literature.
Assuntos
Síndrome POEMS , Adulto , Diagnóstico Diferencial , Evolução Fatal , Humanos , Masculino , Pessoa de Meia-Idade , Síndrome POEMS/diagnóstico , Síndrome POEMS/tratamento farmacológico , Síndrome POEMS/imunologiaRESUMO
Paraneoplastic cerebellar degeneration is a rare remote effect of ovarian and breast carcinoma especially, and is characterised clinically by rapidly evolving pancerebellar symptoms. A woman aged 83 developed progressive vertigo, cerebellar ataxia, nystagmus and dysarthria. The cerebrospinal fluid showed slight mononuclear pleocytosis, elevated total protein and IgG concentrations, and oligoclonal bands. A magnetic resonance investigation performed within the first month of symptoms was normal. A left pelvic mass was found, possibly a carcinoma of the colon or the left ovary. Cancer antigen 125 was elevated in the serum and antibodies against Purkinje cells (anti-Yo antibodies) were demonstrated in the serum and cerebrospinal fluid. These results suggested a carcinoma of the ovary as primary site of cancer. Autopsy revealed a left ovarian adenocarcinoma and marked loss of Purkinje cells in the cerebellum. The case illustrates that anti-Yo antibodies may serve as a marker not only for paraneoplastic cerebellar degeneration, but also for the nature of the neoplasm that caused it.