Paraneoplastic cerebellar ataxia associated with anti-hu antibodies and benign ganglioneuroma

Roberto Fancellu, Elena Corsini, Gaetano Bernardi, Paolo Buzzo, Maria Luisa Ferrari, Eleonora Lamantea, Alberto Garaventa, Mauro Truini, Sandro Salvarani

Research output: Contribution to journalArticle

Abstract

We describe a case of cerebellar ataxia associated with anti-Hu antibodies and benign ganglioneuroma. A 28-year-old woman developed progressive ataxia with hyporeflexia at the age of 19. Brain MRI showed progressive cerebellar atrophy. Neurophysiological studies, screening of immune-mediated ataxias, oncological markers, vitamin E and genetic tests for spinocerebellar ataxia types 1,2,3, Friedreich ataxia and POLG1 were negative. Anti-Hu antibodies were positive in Western blot and indirect immunofluores- cence (1:640). Total-body computed tomography revealed a mediastinum mass; the histological diag- nosis was maturing ganglioneuroma. Immunohisto- chemistry showed a mild reaction between the tumor and the patient’s serum, and no reaction between the tumor and control serum. After surgery, serum anti-Hu titer decreased, while ataxic symptoms initial- ly worsened and then stabilized. Ganglioneuroma is a benign tumor, usually derived from the maturation of a neuroblastoma. The benign histology and the pres- ence of anti-Hu antibodies could be related to the positive oncological prognosis and to the slow clini- cal course mimicking a degenerative ataxia.

Original languageEnglish
Pages (from-to)277-280
Number of pages4
JournalFunctional Neurology
Volume29
Issue number4
Publication statusPublished - Oct 1 2014

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Keywords

  • Anti-Hu antibodies
  • Ganglioneuroma
  • Immune-medi- ated ataxia
  • Neuroblastoma
  • Paraneoplastic syndrome

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Fancellu, R., Corsini, E., Bernardi, G., Buzzo, P., Ferrari, M. L., Lamantea, E., Garaventa, A., Truini, M., & Salvarani, S. (2014). Paraneoplastic cerebellar ataxia associated with anti-hu antibodies and benign ganglioneuroma. Functional Neurology, 29(4), 277-280.