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Fatal CNS vasculopathy in a patient with refractory celiac disease and lymph node cavitation

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Abstract

Celiac disease is an enteropathy occurring in genetically predisposed individuals due to a dietary intolerance to gluten. Patients with celiac disease may develop a neurological disorder of unknown cause, although autoimmune mechanisms are suspected. We report on a 56-year-old man with celiac disease, who became refractory to a gluten-free diet and died of a rapidly progressive encephalopathy. Magnetic resonance imaging indicated focal lesions of the cerebellum and brainstem, and electrodiagnostic studies suggested an axonal neuropathy. Autopsy revealed a flattened small-bowel mucosa with intraepithelial lymphocytosis, a spectrum of degenerative changes of the intra-abdominal and mediastinal lymph nodes, including cavitary degeneration, and splenomegaly. Histologically, the lymph nodes showed pseudocyst formation and lymphocytic vasculitis with fibrinoid necrosis, and sections of the brain exhibited fibrinoid degeneration of small blood vessels, sparse perivascular lymphocytic infiltrates, and perivascular ischemic lesions. Identical T-cell clones were identified in the duodenum, stomach, lymph nodes, and spleen. This patient had an unusual neurological disorder related to a vasculopathy, probably mediated by a circulating neoplastic clone of activated T cells.

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Authors and Affiliations

  1. Department of Pathology, College of Physicians and Surgeons, Columbia University, New York, NY, USA

    Christian E. Keller, Arthur P. Hays & Govind Bhagat

  2. Department of Neurology, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, New York, NY, USA

    Eugenia T. Gamboa

  3. Department of Radiology, College of Physicians and Surgeons, Columbia University, New York, NY, USA

    Gina Lowe

  4. Department of Medicine, College of Physicians and Surgeons, Columbia University, New York, NY, USA

    Jordan Karlitz & Peter H. R. Green

  5. College of Physicians and Surgeons, Columbia University, VC 14th, Room 236A 630 West 168th Street, New York, NY, 10032, USA

    Govind Bhagat

Authors
  1. Christian E. Keller
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  2. Eugenia T. Gamboa
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  3. Arthur P. Hays
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  4. Jordan Karlitz
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  5. Gina Lowe
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  6. Peter H. R. Green
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  7. Govind Bhagat
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Correspondence to Govind Bhagat.

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Keller, C.E., Gamboa, E.T., Hays, A.P. et al. Fatal CNS vasculopathy in a patient with refractory celiac disease and lymph node cavitation. Virchows Arch 448, 209–213 (2006). https://doi.org/10.1007/s00428-005-0060-x

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  • DOI: https://doi.org/10.1007/s00428-005-0060-x

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