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Ataxia in the setting of complicated enteropathy: double jeopardy

Abstract

The differential diagnosis of subacute onset ataxia in the setting of enteropathy is wide. A 54 year old patient with a pancerebellar syndrome and known ulcerative jejunoileitis is described. Small bowel biopsy showed evidence of enteropathy associated T cell lymphoma and subsequent neuropathological analysis and immunophenotyping confirmed metastasis of this tumour to the cerebellum. The presence of anti-gliadin antibodies and MRI evidence of a more longstanding process suggested additional immunologically mediated cerebellar dysfunction. Lymphomatous involvement of the CNS is rare in patients with complicated enteropathies, and has not been previously reported to involve the cerebellar parenchyma. This diagnostic possibility should be borne in mind before attributing cerebellar dysfunction in patients with the coeliac related enteropathies to nutritional compromise or immunological dysfunction (gluten ataxia) alone.

  • ataxia
  • enteropathy associated T cell lymphoma
  • coeliac disease
  • anti-gliadin antibodies
  • SCA, spinocerebellar atrophy
  • CD45, leucocyte common antigen
  • TIA-1, cytotoxic granule marker
  • IEL, intraepithelial lymphocytes
  • EATL, enteropathy associated T cell lymphoma

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