Abstract

IntroductionParaneoplastic cerebellar degeneration is a rare non-metastatic manifestation of malignancy. In this report, to the best of our knowledge we describe for the first time a diagnosis of paraneoplastic cerebellar degeneration several months prior to the diagnosis of clear carcinoma of the uterus.Case presentationA 75-year-old Caucasian woman manifested a rapidly progressive cerebellar syndrome with nystagmus, past-pointing, dysdiadochokinesis, dysarthria, truncal ataxia and titubation. The paraneoplastic cerebellar degeneration was associated with anti-Yo and anti-glutamic acid decarboxylase antibodies. 14-3-3 protein was detected in the cerebrospinal fluid. She was treated with intravenous immunoglobulin prior to laparotomy, hysterectomy and bilateral salpingoophorectomy. Our patient has survived for three years following diagnosis and treatment.ConclusionsTo the best of our knowledge this is the first report of an association of clear cell carcinoma of the uterus and paraneoplastic cerebellar degeneration with both anti-Yo and anti-glutamic acid decarboxylase antibodies. The findings imply that both antibodies contributed to the fulminating paraneoplastic cerebellar degeneration observed in our patient, and this was of such severity it resulted in the release of 14-3-3 protein in the cerebrospinal fluid, a marker of neuronal death.

Highlights

  • Paraneoplastic cerebellar degeneration is a rare non-metastatic manifestation of malignancy

  • To the best of our knowledge this is the first report of an association of clear cell carcinoma of the uterus and paraneoplastic cerebellar degeneration with both anti-Yo and anti-glutamic acid decarboxylase antibodies

  • The findings imply that both antibodies contributed to the fulminating paraneoplastic cerebellar degeneration observed in our patient, and this was of such severity it resulted in the release of 14-3-3 protein in the cerebrospinal fluid, a marker of neuronal death

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Summary

Conclusions

To the best of our knowledge this is the first report of a PCD caused by clear cell carcinoma of the uterus with both anti-Yo and anti-GAD antibodies, in association with 14-3-3 proteins in CSF. These observations increase our understanding of the antibodies related to PCD in gynecological malignancies and is of relevance to neurologists, gynecologists, general internists, oncologists and clinical immunologists. Consent Written informed consent was obtained from the patient for publication of this case report. Authors’ contributions PKP and AG were both responsible for diagnosis and patient management, and both wrote and approved the final manuscript

Introduction
November 2011
Discussion
Klockgether T
Full Text
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