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      Degeneración cerebelosa paraneoplásica como presentación de una recidiva de carcinoma de mama Translated title: Praneoplastic cerebellar degeneration as a presentation of breast cancer recurrence

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          Abstract

          La degeneración cerebelosa paraneoplasica (DCP) es un síndrome paraneoplásico poco frecuente que se caracteriza por disfunción cerebelosa global. La DCP a menudo precede meses o años a una neoplasia potencialmente curable, con menos frecuencia ocurre en pacientes con neoplasia conocida o indica recurrencia. La presencia de anticuerpos específicos en suero ayuda a guiar la identificación de una neoplasia oculta. Comunicamos el caso de una paciente de 74 años que presentó disfunción cerebelosa. Había sido diagnosticada de carcinoma ductal de mama hacia 5 años, permaneciendo asintomática hasta entonces. Con la pruebas de imagen convencionales no fue posible detectar recidiva tumoral, el diagnóstico se realizó con la ayuda de la tomografía con emisión de positrones que detectó adenopatías abdominales. Revisamos brevemente las características clínicas de este síndrome, remarcando la importancia de un rápido reconocimiento del síndrome, que en muchas ocasiones permite una rápida detección y tratamiento de la enfermedad primaria.

          Translated abstract

          Paraneoplastic cerebellar degeneration (PCD) is a rare condition which is characterised by global cerebellar dysfunction. Patients may present with these syndromes months or years before the diagnosis of underlying malignancy is established. Less often, PCD occurs in patients with a known malignancy or heralds the onset of a recurrence. The presence of specific antibodies in serum simples helps to guide identification the occult malignancy. We report here the case of a PCD in 74-year-old lady underwent a left mastectomy for breast cancer 5 years ago. She remained well until now. The diagnosis of the primary tumor, that is clinically undetectable with conventional imaging processes, is preformed with the aid of positron mission tomography (PET) to detect the presence of abdominal lymph node metastases. We briefly review the clinical and laboratory features of this syndrome, and emphasize the importance of its prompt recognition, which many times makes possible the early detection and treatment of the primary disorder.

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          Most cited references39

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          Anti-Hu-associated paraneoplastic encephalomyelitis: analysis of 200 patients.

          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. The median age of the 200 patients was 63 years (range 28-82 years) and 75% were men. The predominant neurological syndromes were sensory neuropathy (54%), cerebellar ataxia (10%), limbic encephalitis (9%) and multifocal involvement (11%). Sensorimotor neuropathies with predominant motor involvement were observed in only 4% of the patients. Pathological or X-ray evidence of a tumour was obtained in 167 patients (83%) and was a small-cell lung cancer (SCLC) in 74% of those with histological diagnosis. Coexistence of extrathoracic tumours with SCLC was rare (0.5%). Positive Hu immunoreactivity was observed in the extrathoracic tumours of six out of seven patients in whom autopsy or long-term follow-up ruled out a coexisting SCLC. PEM preceded the diagnosis of the tumour in 71% of patients (mean delay +/- SD 6.5 +/- 7.0 months; range 0.1-47 months). In the 24 patients in whom the tumour diagnosis was the initial event, PEM predicted the progression or relapse of the tumour in 87% of them. No tumour was found in 33 patients, including four who had a post-mortem study and four with >5 years of follow-up. In a logistic regression analysis, treatment of the tumour, associated or not with immunotherapy, was an independent predictor of improvement/stabilization of PEM [odds ratio 4.56; 95% confidence interval (CI) 1.62-12.86]. Cox multivariate analysis indicated that the variables independently associated with mortality were: age >60 years [relative risk (RR) 1.49; 95% CI 1.05-2.12], Rankin score at diagnosis >3 (RR 1.60; 95% CI 1.12-2.28), more than one area of the nervous system affected (RR 1.61; 95% CI 1.08-2.40), and absence of treatment (RR 2.56; 95% CI 1.76-3.71). We conclude that, unlike previous series, the majority of our patients were male, and there was a low occurrence of predominantly motor neuropathies and extrathoracic tumours coexisting with SCLC. When the diagnosed extrathoracic tumour expresses Hu antigens, further tests to rule out a coexisting SCLC are probably unnecessary. 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.
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            Paraneoplastic cerebellar degeneration. I. A clinical analysis of 55 anti-Yo antibody-positive patients.

            We reviewed the clinical findings in 55 patients with cerebellar degeneration associated with the anti-Yo antibody (an anti-Purkinje cell antibody identified in this study by histochemistry and Western blot). The patients were all women, 26 to 85 years old. Fifty-two of them proved to have malignancies, almost exclusively breast or gynecologic cancers and usually confined to the involved organs and local lymph nodes. One woman had adenocarcinoma of the lung, and in three no malignancy has yet been identified. In 34 of 52 patients with cancer, the neurologic syndrome preceded the diagnosis of cancer and in many led to that diagnosis. Patients subacutely developed a pancerebellar disorder that was substantially disabling in most, with 37 of 48 assessable patients being unable to walk or sit unassisted. Laboratory evaluation revealed lymphocytic pleocytosis in 35 patients, with eventual cerebellar atrophy on imaging studies in seventeen. The disabling neurologic syndrome generally did not respond to treatment, but the cancer was often successfully treated. The presence of the anti-Yo antibody in patients with cerebellar symptoms warrants an aggressive approach to diagnosis and treatment of the underlying cancer, as many are curable at the time neurologic symptoms develop.
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              Treatment of paraneoplastic neurological syndromes with antineuronal antibodies (Anti-Hu, anti-Yo) with a combination of immunoglobulins, cyclophosphamide, and methylprednisolone.

              To evaluate the effect of a combination of immunoglobulins (IVIg), cyclophosphamide (CTX), and methylprednisolone (MP) on the clinical course of patients with paraneoplastic neurological syndrome (PNS) and antineuronal antibodies (Abs). Seventeen patients with paraneoplastic encephalomyelitis/sensory neuropathy (PEM/SN) with anti-Hu Abs (n = 10) or cerebellar degeneration (PCD) with anti-Yo Abs (n = 7) received one to nine cycles (mean 3.5) of a combination of IVIg (0.5 g/kg/day from days 1 to 5), CTX (600 mg/m2 at day 1) and MP (1g/day from day 1 to 3). The Rankin scale (RS) was used to evaluate the response. A positive response was considered as either improvement or stabilisation in patients who were still ambulatory (RS or =4). Tolerance was good and no patient experienced grade 3/4 toxicity (World Health Organisation). Sixteen patients were evaluable for response. Of the seven patients with RS> or =4, none improved. Of the nine patients with RS< or =3, none improved but three (two SN and one PCD) stabilised for 4, 35, and 16 months. This study suggests that vigorous immunosuppressive treatment is not useful in severely disabled PNS patients with antineuronal Abs. In a minority of patients (mainly with SN) who are not severely disabled at the onset of treatment, a transient stabilisation is possible and deserves further evaluation.
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                Author and article information

                Contributors
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Role: ND
                Journal
                ami
                Anales de Medicina Interna
                An. Med. Interna (Madrid)
                Arán Ediciones, S. L. (, , Spain )
                0212-7199
                March 2007
                : 24
                : 3
                : 132-134
                Affiliations
                [03] Lleida orgnameHospital Universitario Arnau de Vilanova orgdiv1Servicio de Neurología
                [01] orgnameHospital Universitario Arnau de Vilanova orgdiv1Servicio de Medicina Interna
                [02] orgnameHospital Universitario Arnau de Vilanova orgdiv1Servicio de Oncología Médica
                Article
                S0212-71992007000300007
                10.4321/s0212-71992007000300007
                74aa299d-d3b0-4c2c-9b0a-1410d6d8f7ff

                This work is licensed under a Creative Commons Attribution-NonCommercial 3.0 International License.

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                Degeneración cerebelosa paraneoplásica,Cáncer de mama,Anticuerpos antineuronales,Paraneoplastic cerebellar degeneration,Breast cancer,Antineuronal antibodies

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