Anti-Ri positive opsoclonus-myoclonus-ataxia in ovarian duct cancer

Jongen, Joost L.M.; Moll, Wibe; Vecht, Charles J.; Tijssen, Cees

doi:10.1007/s004150050270

Cited by 33 publications

(17 citation statements)

References 9 publications

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“…Sensorimotor neuropathy and dysesthesias have been reported in other anti-Ri patients, and severe gastro-intestinal dysmotility in one patient with opsoclonus and breast carcinoma [3,8,12]. Our patient did not respond to plasma exchanges, whereas immunosuppressive drugs and thiamine led to improvement of neurological deficit over a period of months, as in other reports [5,10,12]. The relapse after lowering the prednisone and remission after increasing the dose, suggests that the immunosuppressive treatment did contribute to recovery of the patient.…”

Section: Discussionsupporting

confidence: 58%

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Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma

Wirtz¹,

Hoff²,

Leeuw

et al. 2002

Journal of Neurology

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A 65-year-old male patient developed truncal ataxia, opsoclonus and myoclonus. In the serum anti-Ri antibodies were found, which led to the detection of a small adenocarcinoma of the breast. Other prominent clinical features were an excessive startle response and behavioral disorders, such as anxiety and impatience. These features suggest an immune response against both Nova-1 and Nova-2 antigens throughout the central nervous system.

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Section: Discussionsupporting

confidence: 58%

“…In female patients, the syndrome was most often associated with breast carcinoma [2,7,12]. Gynecological or small cell lung cancer have also been reported in anti-Ri positive patients [4,10,12]. Two male patients with anti-Ri antibodies have been described [11,14].…”

Section: Discussionmentioning

confidence: 97%

Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma

Wirtz¹,

Hoff²,

Leeuw

et al. 2002

Journal of Neurology

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“…However, improvement following the administration of steroids, cyclophosphamide, azathioprine, i.v. Ig, plasma exchange, or plasma filtration with a protein A column has been described in single cases [55,[70][71][72].…”

Section: Treatment and Prognosismentioning

confidence: 99%

Managing Paraneoplastic Neurological Disorders

2006

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“…Der Anti-Yo-Antikörper ist der typische serologische Marker bei diesen Karzinomen [9,12]. Beim seltenen Opsoklonus-Myoklonus-Syndrom ist der Anti-Ri-Antikörper typisch [15,16]. Wenn das Krankheitsbild mit einem Anti-Hu-Antikörper auftritt, so liegt ihm immer ein Bronchialkarzinom zugrunde [10].…”

Section: Tumormanifestationunclassified

Neurologische paraneoplastische Syndrome bei gynäkologischen Malignomen

Besser¹

2005

Geburtsh Frauenheilk

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Neurologische paraneoplastische Syndrome sind seltene, meist immunvermittelte Begleiterscheinungen von bösartigen Tumoren. Unter den zahlreichen Tumoren sind das Ovarial-und das Mammakarzinom eine häufige Ursache und damit eine Herausforderung an den Gynäkologen. Das typische Erscheinungsbild besteht in der raschen Entwicklung einer schweren neurologischen Behinderung bei einem bisher nicht bekannten und lokal begrenzten Tumor. Neben der paraneoplastischen zerebellären Degeneration mit einer schweren Ataxie als häufigstem Erscheinungsbild sind die limbische Enzephalitis, die Hirnstammenzephalitis, die sensorische Neuronopathie, das Stiff-Person-Syndrom, die Dermatomyositis und die paraneoplastische Retinopathie beschrieben. Der Meilenstein für die Diagnose sind onkoneurale Antikörper im Serum und Liquor, die sowohl an Tumorzellen als auch an Nervenzellen binden. Neurologische paraneoplastische Syndrome bei Frauen mit Ovarial-und Mammakarzinomen haben immer eine ungünstige Prognose. Die meisten Patienten bleiben trotz einer effektiven Antitumortherapie, die Voraussetzung für eine wirksame Immuntherapie ist, schwer behindert und die Mortalität ist hoch. Schlüsselwörter AbstractParaneoplastic neurologic disorders are rare manifestations of a malignant neoplasm and mostly immune-mediated. Out of a broad spectrum of tumors ovarian and breast carcinomas are frequently the underlying cause presenting a challenge for the gynecologist. The combination of a rapidly progressive severe neurologic disability and a small, as yet unknown tumor is the typical clinical presentation. In addition to the most frequent paraneoplastic cerebellar degeneration resulting in severe ataxia, limbic encephalitis, brain stem encephalitis, sensory neuropathy, stiff-person syndrome, dermatomyositis and paraneoplastic retinopathy have been described. The cornerstone for the diagnosis are onconeural antibodies in serum and cerebrospinal fluid reacting with both the nervous system and the underlying cancer. Paraneoplastic neurologic syndromes in women with ovarian or breast cancer imply a grave prognosis. Although antitumor therapy may be effective in many cases and are essential for the success of additional immunotherapy, most patients remain severely disabled and mortality is remarkably high.

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Anti-Ri positive opsoclonus-myoclonus-ataxia in ovarian duct cancer

Cited by 33 publications

References 9 publications

Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma

Anti-Ri antibody positive opsoclonus-myoclonus in a male patient with breast carcinoma

Managing Paraneoplastic Neurological Disorders

Neurologische paraneoplastische Syndrome bei gynäkologischen Malignomen

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