Primary angiitis of the central nervous system as a first presentation in Hodgkin's disease: a case report and review of the literature

Neurosurgery. 2000 Jun;46(6):1504-8; discussion 1508-10. doi: 10.1097/00006123-200006000-00037.

Abstract

Objective and importance: Granulomatous angiitis of the central nervous system is a rare cause of neurological deterioration. It is often diagnosed posthumously, and a high index of suspicion is necessary to make the correct diagnosis on a timely basis.

Clinical presentation: A 27-year-old woman presented to the emergency room with complaints of worsening headache, nausea, and vomiting for 10 days, which were preceded by swelling of her tongue. At the examination, she had mild ocular tenderness, but no cranial nerve abnormalities. Radiographic examination revealed a right temporal lobe area with edema, and mild contrast enhancement was noted on computed tomography and magnetic resonance imaging. A similar but smaller region was present in the left frontal lobe.

Intervention: Stereotactic biopsy of the left temporal lobe revealed granulomatous angiitis. Further workup revealed Hodgkin's disease in the mediastinum. Dexamethasone as well as chemotherapy for Hodgkin's disease was initiated. The patient's symptoms resolved, and she returned to work with her disease in remission.

Conclusion: Previous reports of central nervous system angiitis have shown an association with Sjogren's syndrome, herpes zoster infection, human immunodeficiency virus, and Hodgkin's disease. A review of the literature revealed a total of 12 patients with central nervous system angiitis and Hodgkin's disease. As a group, these patients had very poor outcomes. However, of six patients who presented with central nervous system angiitis and concurrent Hodgkin's disease and who underwent aggressive treatment for both conditions, three had a full recovery, two had a partial recovery, and one died.

Publication types

  • Case Reports

MeSH terms

  • Adult
  • Biopsy
  • Cerebral Arteries / pathology
  • Diagnosis, Differential
  • Female
  • Frontal Lobe / blood supply*
  • Hodgkin Disease / diagnosis*
  • Hodgkin Disease / pathology
  • Humans
  • Paraneoplastic Syndromes / diagnosis*
  • Paraneoplastic Syndromes / pathology
  • Temporal Lobe / blood supply*
  • Vasculitis, Central Nervous System / diagnosis*
  • Vasculitis, Central Nervous System / pathology