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Francesc Graus - One of the best experts on this subject based on the ideXlab platform.

  • Protein kinase Cγ antibodies and Paraneoplastic Cerebellar Degeneration.
    Journal of neuroimmunology, 2012
    Co-Authors: Romana Höftberger, Josep Dalmau, Gabor G Kovacs, Lidia Sabater, Peter Nagy, Gergely Racz, Rosa Miquel, Francesc Graus
    Abstract:

    Onconeural antibodies are diagnostic markers for Paraneoplastic neurological syndromes and indicate the underlying tumor type. Recently, a new antibody against protein-kinase Cγ (PKCγ) was detected in a patient with Paraneoplastic Cerebellar Degeneration (PCD). We report here a second patient. A 70-year-old woman presented with Cerebellar ataxia, dysdiadochokinesia, and dysarthria. Her serum showed immunoreactivity against the cytoplasm, dendrites and axons of Purkinje cells in tissue-based screening, later confirmed by immunoblot and phage plaques as anti-PKCγ. Tumor search revealed an adenocarcinoma of hepatobiliary origin. Detection of PKCγ antibodies should be considered in PCD and adenocarcinoma without typical onconeural antibodies.

  • Anti-Ri-associated Paraneoplastic Cerebellar Degeneration and breast cancer: an autopsy case study.
    Clinical neurology and neurosurgery, 2008
    Co-Authors: L. Brieva-ruíz, M. Diaz-hurtado, X. Matias-guiu, D. Márquez-medina, J. Tarragona, Francesc Graus
    Abstract:

    Several antineuronal antibodies are associated with Paraneoplastic Cerebellar Degeneration. Anti-Ri is one of these antibodies in some cases but it is more commonly associated with Paraneoplastic opsoclonus myoclonus in the context of gynecological neoplasia. Anti-Ri autoantibodies are thought to be directed against onconeural antigens, NOVA-1 and NOVA-2, that are expressed by the tumor as well as by neurons. The results of the treatment of both syndromes have been disappointing, although aggressive multimodality immunosuppressive treatments have been used. There are few cases of anti-Ri Paraneoplastic Cerebellar Degeneration and none has been pathologically studied. We report the pathological study of a patient who died from anti-Ri-positive Paraneoplastic Cerebellar Degeneration associated with breast cancer only confirmed at autopsy.

  • ZIC antibodies in Paraneoplastic Cerebellar Degeneration and small cell lung cancer.
    Journal of neuroimmunology, 2008
    Co-Authors: Lidia Sabater, Josep Dalmau, Luis Bataller, Albert Saiz, Marc Suárez-calvet, Francesc Graus
    Abstract:

    Patients with isolated ZIC4 antibodies usually have Paraneoplastic Cerebellar Degeneration (PCD) and small cell lung cancer (SCLC) but the frequency is unknown. We analyzed the presence of ZIC1, ZIC2 and ZIC4 antibodies in 27 patients with PCD and SCLC negative for other onconeural antibodies. ZIC antibodies were detected in nitrocellulose filters with phage plaques. Four (15%) PCD sera recognized ZIC2. Three of these positive sera also reacted with ZIC1 and two with ZIC4. Our study suggests that 1) the incidence of isolated ZIC antibodies is low in PCD patients and SCLC and 2) ZIC antibodies are probably directed to epitopes shared by the three ZIC proteins.

  • carbonic anhydrase related protein viii autoantigen in Paraneoplastic Cerebellar Degeneration
    Annals of Neurology, 2004
    Co-Authors: Luis Bataller, Lidia Sabater, Albert Saiz, Carles Serra, Berta Claramonte, Francesc Graus
    Abstract:

    Serum of a patient with Paraneoplastic Cerebellar Degeneration (PCD) and malignant melanoma had a unique reactivity with Purkinje cells that was not observed in serum of patients with PCD and other tumors, or with malignant melanoma without Paraneoplastic syndromes. The screening of a human Cerebellar complementary DNA expression library with the patient's serum resulted in the isolation of the CA8 gene. CA8 encodes carbonic anhydrase–related protein (CARP) VIII, preferentially expressed in Purkinje cells. The patient had intrathecal synthesis of CARP VIII antibodies. One of seven melanomas tested expressed CARP VIII. These data suggest CARP VIII may be an autoantigen involved in the pathogenesis of melanoma-associated PCD. Ann Neurol 2004

  • Carbonic anhydrase–related protein VIII: Autoantigen in Paraneoplastic Cerebellar Degeneration
    Annals of neurology, 2004
    Co-Authors: Luis Bataller, Lidia Sabater, Albert Saiz, Carles Serra, Berta Claramonte, Francesc Graus
    Abstract:

    Serum of a patient with Paraneoplastic Cerebellar Degeneration (PCD) and malignant melanoma had a unique reactivity with Purkinje cells that was not observed in serum of patients with PCD and other tumors, or with malignant melanoma without Paraneoplastic syndromes. The screening of a human Cerebellar complementary DNA expression library with the patient's serum resulted in the isolation of the CA8 gene. CA8 encodes carbonic anhydrase–related protein (CARP) VIII, preferentially expressed in Purkinje cells. The patient had intrathecal synthesis of CARP VIII antibodies. One of seven melanomas tested expressed CARP VIII. These data suggest CARP VIII may be an autoantigen involved in the pathogenesis of melanoma-associated PCD. Ann Neurol 2004

Mariana Spitz - One of the best experts on this subject based on the ideXlab platform.

  • Paraneoplastic Cerebellar Degeneration as initial presentation of renal cell carcinoma
    Cerebellum & Ataxias, 2019
    Co-Authors: Sara M. Souza, Barbara O. Santos, Isadora C.a. Sodré, Ana Luiza P. Oliveira, Diogo Terrana, Mariana Spitz
    Abstract:

    Background Paraneoplastic Cerebellar Degeneration is usually associated with gynecological and breast cancer, lung cancer, and Hodgkin’s lymphoma. Renal cell carcinoma has rarely been described as an underlying malignancy in these cases. Case presentation We report the case of a 75 year-old woman who develop Cerebellar ataxia following a respiratory viral infection. During investigation, around 1 year afterward, she noticed constitutional symptoms suggestive of malignancy. Renal carcinoma was found and the hypothesis of Paraneoplastic Cerebellar Degeneration was considered. Conclusions As no specific antineuronal antibodies have been described in the setting of renal cell carcinoma, Paraneoplastic Cerebellar Degeneration should be considered when the tumor is detected and other causes are excluded. Immunotherapy should be prescribed as soon as possible.

  • Paraneoplastic Cerebellar Degeneration as initial presentation of renal cell carcinoma
    Cerebellum & ataxias, 2019
    Co-Authors: Sara M. Souza, Barbara O. Santos, Isadora C.a. Sodré, Ana Luiza P. Oliveira, Diogo Terrana, Mariana Spitz
    Abstract:

    Paraneoplastic Cerebellar Degeneration is usually associated with gynecological and breast cancer, lung cancer, and Hodgkin’s lymphoma. Renal cell carcinoma has rarely been described as an underlying malignancy in these cases. We report the case of a 75 year-old woman who develop Cerebellar ataxia following a respiratory viral infection. During investigation, around 1 year afterward, she noticed constitutional symptoms suggestive of malignancy. Renal carcinoma was found and the hypothesis of Paraneoplastic Cerebellar Degeneration was considered. As no specific antineuronal antibodies have been described in the setting of renal cell carcinoma, Paraneoplastic Cerebellar Degeneration should be considered when the tumor is detected and other causes are excluded. Immunotherapy should be prescribed as soon as possible.

Lidia Sabater - One of the best experts on this subject based on the ideXlab platform.

  • Plasticity-related gene 5: A novel surface autoantigen in Paraneoplastic Cerebellar Degeneration
    Neurology(R) neuroimmunology & neuroinflammation, 2015
    Co-Authors: Marleen H. Van Coevorden-hameete, Esther Hulsenboom, Lidia Sabater, Esther De Graaff, Maarten J. Titulaer, Casper C. Hoogenraad, P. S. Smitt
    Abstract:

    Paraneoplastic Cerebellar Degeneration (PCD) is one of the most frequent Paraneoplastic syndromes affecting the CNS. It is associated with antibodies targeting intracellular neuronal antigens (Hu, Yo, Ri, CV2/CRMP5), which are not thought to be directly pathogenic, and surface antigens (DNER, mGluR1, VGCC), which are potentially pathogenic.1,2 However, in many patients the immunologic target remains unidentified, resulting in diagnostic and therapeutic challenges. We report a patient with PCD and a squamous cell lung carcinoma with antibodies to a novel neuronal surface antigen, plasticity-related gene 5 (PRG5).

  • Protein kinase Cγ antibodies and Paraneoplastic Cerebellar Degeneration.
    Journal of neuroimmunology, 2012
    Co-Authors: Romana Höftberger, Josep Dalmau, Gabor G Kovacs, Lidia Sabater, Peter Nagy, Gergely Racz, Rosa Miquel, Francesc Graus
    Abstract:

    Onconeural antibodies are diagnostic markers for Paraneoplastic neurological syndromes and indicate the underlying tumor type. Recently, a new antibody against protein-kinase Cγ (PKCγ) was detected in a patient with Paraneoplastic Cerebellar Degeneration (PCD). We report here a second patient. A 70-year-old woman presented with Cerebellar ataxia, dysdiadochokinesia, and dysarthria. Her serum showed immunoreactivity against the cytoplasm, dendrites and axons of Purkinje cells in tissue-based screening, later confirmed by immunoblot and phage plaques as anti-PKCγ. Tumor search revealed an adenocarcinoma of hepatobiliary origin. Detection of PKCγ antibodies should be considered in PCD and adenocarcinoma without typical onconeural antibodies.

  • ZIC antibodies in Paraneoplastic Cerebellar Degeneration and small cell lung cancer.
    Journal of neuroimmunology, 2008
    Co-Authors: Lidia Sabater, Josep Dalmau, Luis Bataller, Albert Saiz, Marc Suárez-calvet, Francesc Graus
    Abstract:

    Patients with isolated ZIC4 antibodies usually have Paraneoplastic Cerebellar Degeneration (PCD) and small cell lung cancer (SCLC) but the frequency is unknown. We analyzed the presence of ZIC1, ZIC2 and ZIC4 antibodies in 27 patients with PCD and SCLC negative for other onconeural antibodies. ZIC antibodies were detected in nitrocellulose filters with phage plaques. Four (15%) PCD sera recognized ZIC2. Three of these positive sera also reacted with ZIC1 and two with ZIC4. Our study suggests that 1) the incidence of isolated ZIC antibodies is low in PCD patients and SCLC and 2) ZIC antibodies are probably directed to epitopes shared by the three ZIC proteins.

  • carbonic anhydrase related protein viii autoantigen in Paraneoplastic Cerebellar Degeneration
    Annals of Neurology, 2004
    Co-Authors: Luis Bataller, Lidia Sabater, Albert Saiz, Carles Serra, Berta Claramonte, Francesc Graus
    Abstract:

    Serum of a patient with Paraneoplastic Cerebellar Degeneration (PCD) and malignant melanoma had a unique reactivity with Purkinje cells that was not observed in serum of patients with PCD and other tumors, or with malignant melanoma without Paraneoplastic syndromes. The screening of a human Cerebellar complementary DNA expression library with the patient's serum resulted in the isolation of the CA8 gene. CA8 encodes carbonic anhydrase–related protein (CARP) VIII, preferentially expressed in Purkinje cells. The patient had intrathecal synthesis of CARP VIII antibodies. One of seven melanomas tested expressed CARP VIII. These data suggest CARP VIII may be an autoantigen involved in the pathogenesis of melanoma-associated PCD. Ann Neurol 2004

  • Carbonic anhydrase–related protein VIII: Autoantigen in Paraneoplastic Cerebellar Degeneration
    Annals of neurology, 2004
    Co-Authors: Luis Bataller, Lidia Sabater, Albert Saiz, Carles Serra, Berta Claramonte, Francesc Graus
    Abstract:

    Serum of a patient with Paraneoplastic Cerebellar Degeneration (PCD) and malignant melanoma had a unique reactivity with Purkinje cells that was not observed in serum of patients with PCD and other tumors, or with malignant melanoma without Paraneoplastic syndromes. The screening of a human Cerebellar complementary DNA expression library with the patient's serum resulted in the isolation of the CA8 gene. CA8 encodes carbonic anhydrase–related protein (CARP) VIII, preferentially expressed in Purkinje cells. The patient had intrathecal synthesis of CARP VIII antibodies. One of seven melanomas tested expressed CARP VIII. These data suggest CARP VIII may be an autoantigen involved in the pathogenesis of melanoma-associated PCD. Ann Neurol 2004

Christian A. Vedeler - One of the best experts on this subject based on the ideXlab platform.

  • Localization of CDR2L and CDR2 in Paraneoplastic Cerebellar Degeneration
    Annals of clinical and translational neurology, 2020
    Co-Authors: Ida Herdlevaer, Torbjørn Kråkenes, Manja Schubert, Christian A. Vedeler
    Abstract:

    Objective Identify the subcellular location and potential binding partners of two Cerebellar Degeneration-related proteins, CDR2L and CDR2, associated with anti-Yo-mediated Paraneoplastic Cerebellar Degeneration. Methods Cancer cells, rat Purkinje neuron cultures, and human Cerebellar sections were exposed to cerebrospinal fluid and serum from patients with Paraneoplastic Cerebellar Degeneration with Yo antibodies and with several antibodies against CDR2L and CDR2. We used mass spectrometry-based proteomics, super-resolution microscopy, proximity ligation assay, and co-immunoprecipitation to verify the antibodies and to identify potential binding partners. Results We confirmed the CDR2L specificity of Yo antibodies by mass spectrometry-based proteomics and found that CDR2L localized to the cytoplasm and CDR2 to the nucleus. CDR2L co-localized with the 40S ribosomal protein S6, while CDR2 co-localized with the nuclear speckle proteins SON, eukaryotic initiation factor 4A-III, and serine/arginine-rich splicing factor 2. Interpretation We showed that Yo antibodies specifically bind to CDR2L in Purkinje neurons of PCD patients where they potentially interfere with the function of the ribosomal machinery resulting in disrupted mRNA translation and/or protein synthesis. Our findings demonstrating that CDR2L interacts with ribosomal proteins and CDR2 with nuclear speckle proteins is an important step toward understanding PCD pathogenesis.

  • CDR2L Is the Major Yo Antibody Target in Paraneoplastic Cerebellar Degeneration.
    Annals of neurology, 2019
    Co-Authors: Torbjørn Kråkenes, Ida Herdlevaer, Manja Schubert, Margrethe Raspotnig, Mette Haugen, Christian A. Vedeler
    Abstract:

    The pathogenesis of Yo-mediated Paraneoplastic Cerebellar Degeneration (PCD) is unclear. We applied cerebrospinal fluid and serum from PCD patients as well as CDR2 and CDR2L antibodies to neuronal tissue, cancer cell lines, and cells transfected with recombinant CDR2 and CDR2L to elucidate which is the major antigen of Yo antibodies. We found that Yo antibodies bound endogenous CDR2L, but not endogenous CDR2. However, Yo antibodies can bind the recombinant CDR2 protein used in routine clinical testing for these antibodies. Because Yo antibodies only bind endogenous CDR2L, we conclude that CDR2L is the major antigen of Yo antibodies in PCD. ANN NEUROL 2019;86:316-321.

  • Autopsy findings in the nervous system and ovarian tumour of two patients with Paraneoplastic Cerebellar Degeneration.
    Acta Neurologica Scandinavica, 2006
    Co-Authors: Anette Storstein, Bård Kronen Krossnes, Christian A. Vedeler
    Abstract:

    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.

  • Autopsy findings in the nervous system and ovarian tumour of two patients with Paraneoplastic Cerebellar Degeneration.
    Acta neurologica Scandinavica. Supplementum, 2006
    Co-Authors: Anette Storstein, Bård Kronen Krossnes, Christian A. Vedeler
    Abstract:

    To review autopsy findings in Paraneoplastic Cerebellar Degeneration. We report the autopsy results of two individuals with Paraneoplastic Cerebellar Degeneration and ovarian cancer. 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. The immunoactivation in PCD is widespread and involving more than the cerebellum.

  • Proteasome antibodies in Paraneoplastic Cerebellar Degeneration.
    Journal of neuroimmunology, 2005
    Co-Authors: Anette Storstein, Anette Knudsen, Christian A. Vedeler
    Abstract:

    Antibodies to proteasome have been detected in several autoimmune diseases, including multiple sclerosis. We have investigated the presence of such antibodies in patients with Paraneoplastic neurological syndromes, by Western blotting and immunohistochemistry. Antibodies to 20S proteasome were detected in the majority of patients with Paraneoplastic Cerebellar Degeneration (PCD), but in only one of nine sera from patients with Paraneoplastic encephalomyelitis/sensory neuronopathy (PEM/SN), and were not found in cancer patients in general. The results suggest that the immune responses in PCD differ from those of PEM/SN, whereas the functional significance of proteasome antibodies in PCD is yet to be determined.

Sara M. Souza - One of the best experts on this subject based on the ideXlab platform.

  • Paraneoplastic Cerebellar Degeneration as initial presentation of renal cell carcinoma
    Cerebellum & Ataxias, 2019
    Co-Authors: Sara M. Souza, Barbara O. Santos, Isadora C.a. Sodré, Ana Luiza P. Oliveira, Diogo Terrana, Mariana Spitz
    Abstract:

    Background Paraneoplastic Cerebellar Degeneration is usually associated with gynecological and breast cancer, lung cancer, and Hodgkin’s lymphoma. Renal cell carcinoma has rarely been described as an underlying malignancy in these cases. Case presentation We report the case of a 75 year-old woman who develop Cerebellar ataxia following a respiratory viral infection. During investigation, around 1 year afterward, she noticed constitutional symptoms suggestive of malignancy. Renal carcinoma was found and the hypothesis of Paraneoplastic Cerebellar Degeneration was considered. Conclusions As no specific antineuronal antibodies have been described in the setting of renal cell carcinoma, Paraneoplastic Cerebellar Degeneration should be considered when the tumor is detected and other causes are excluded. Immunotherapy should be prescribed as soon as possible.

  • Paraneoplastic Cerebellar Degeneration as initial presentation of renal cell carcinoma
    Cerebellum & ataxias, 2019
    Co-Authors: Sara M. Souza, Barbara O. Santos, Isadora C.a. Sodré, Ana Luiza P. Oliveira, Diogo Terrana, Mariana Spitz
    Abstract:

    Paraneoplastic Cerebellar Degeneration is usually associated with gynecological and breast cancer, lung cancer, and Hodgkin’s lymphoma. Renal cell carcinoma has rarely been described as an underlying malignancy in these cases. We report the case of a 75 year-old woman who develop Cerebellar ataxia following a respiratory viral infection. During investigation, around 1 year afterward, she noticed constitutional symptoms suggestive of malignancy. Renal carcinoma was found and the hypothesis of Paraneoplastic Cerebellar Degeneration was considered. As no specific antineuronal antibodies have been described in the setting of renal cell carcinoma, Paraneoplastic Cerebellar Degeneration should be considered when the tumor is detected and other causes are excluded. Immunotherapy should be prescribed as soon as possible.