Fatal CNS vasculopathy in a patient with refractory celiac disease and lymph node cavitation.
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.
Keller
Christian E
CE
Department of Pathology, College of Physicians and Surgeons, Columbia University, 630 West 168th Street, New York, NY 10032, USA.
Gamboa
Eugenia T
ET
Hays
Arthur P
AP
Karlitz
Jordan
J
Lowe
Gina
G
Green
Peter H R
PH
Bhagat
Govind
G
D001927:Brain Diseases / Q000209:etiology / Q000473:pathology*; D018801:CD2 Antigens / Q000032:analysis; D017252:CD3 Complex / Q000032:analysis; D002446:Celiac Disease / Q000150:complications / Q000473:pathology*; D015922:Complement C1q / Q000032:analysis; D003176:Complement C3 / Q000032:analysis; D015935:Complement C4b / Q000032:analysis; D017809:Fatal Outcome; D015334:Gene Rearrangement, gamma-Chain T-Cell Antigen Receptor / Q000235:genetics; D006801:Humans; D007150:Immunohistochemistry; D008198:Lymph Nodes / Q000098:blood supply / Q000378:metabolism / Q000473:pathology*; D008297:Male; D008875:Middle Aged; D010446:Peptide Fragments / Q000032:analysis; D016133:Polymerase Chain Reaction; D014657:Vasculitis / Q000209:etiology / Q000473:pathology*
D018801:CD2 Antigens; D017252:CD3 Complex; D003176:Complement C3; D010446:Peptide Fragments; D015922:Complement C1q; D015935:Complement C4b; C032261:complement C4d