Clinico-pathological findings in a patient with progressive cerebellar ataxia, autoimmune polyendocrine syndrome, hepatocellular carcinoma and anti-GAD autoantibodies

Giovanni Piccolo, Eleonora Tavazzi, Tiziana Cavallaro, Alfredo Romani, Roberto Scelsi, Gianvito Martino

Research output: Contribution to journalArticlepeer-review

Abstract

Objective: To report clinical and pathological findings of a patient with late onset insulin-dependent diabetes mellitus (IDDM), progressive cerebellar ataxia (PCA) and hepatocellular carcinoma (HCC). Patient: A 64-year-old woman, with a long lasting IDDM, progressively developed a severe cerebellar syndrome and died 2 years after the onset of the symptoms for a systemic infection. Autoantibodies to antigastric parietal cell and anti-pancreatic islet cell resulted positive. Autopsy showed a selective loss of Purkinje cells in the cerebellum, with an increase of Bergmann glia and variable microglial proliferation; furthermore, it disclosed an HCC. GAD-Abs were detected both in serum and CSF. Conclusions: Clinical and experimental reports suggest a possible role of neoplastic cells in producing GAD-Abs. We postulate, in our case, that HCC could have been responsible for an overproduction of GAD-Abs, leading to the onset of PCA. Thus, GAD-Abs could be considered as a paraneoplastic marker in a subgroup of patients with PCA.

Original languageEnglish
Pages (from-to)148-149
Number of pages2
JournalJournal of the Neurological Sciences
Volume290
Issue number1-2
DOIs
Publication statusPublished - Mar 15 2010

Keywords

  • Anti-GAD antibodies
  • Cerebellar syndrome
  • Neuropathology
  • Paraneoplastic
  • Progressive ataxia

ASJC Scopus subject areas

  • Clinical Neurology
  • Neurology

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