Central nervous system sarcoidosis--diagnosis and management

J P Zajicek, N J Scolding, O Foster, M Rovaris, J Evanson, I F Moseley, J W Scadding, E J Thompson, V Chamoun, D H Miller, W I McDonald, D Mitchell

Research output: Contribution to journalArticlepeer-review

552 Citations (Scopus)

Abstract

A series of 68 patients with neurosarcoidosis is reported, with particular emphasis on clinical aspects, diagnosis and treatment. A classification system based on clinical diagnostic probability is proposed, consisting of probable and definite disease, the latter being dependent on finding sarcoid granulomas on nervous system histology, which was obtained in 12 patients (18%). The role of investigations, including magnetic resonance imaging (MRI), chest radiography, Kveim skin test, Gallium 67 isotope scanning and cerebrospinal fluid (CSF) studies, is considered. Sixty-two percent of patients presented with nervous system disease, most commonly affecting the optic nerve and chiasm. Other common presentations included cranial nerve palsies, spinal cord and brainstem manifestations. Investigations yielding most diagnostic information included the Kveim test (41/48, 85% positive), raised CSF protein and/or cells (50/62, 81%) and gallium 67 scan (14/31, 45%). Eleven out of 29 patients (38%) patients showed meningeal enhancement on MRI scanning and 43% of scans demonstrated multiple white-matter lesions. Mean follow-up for the group was 4.6 years. Forty-seven patients were seen for > 18 months, and over half of these patients progressed despite corticosteroid and other immunosuppressive therapies. The benefit of a large patient database prospectively studied, with extended follow-up is discussed in order to learn more about prognosis and advance therapy in neurosarcoidosis.

Original languageEnglish
Pages (from-to)103-17
Number of pages15
JournalQJM: An International Journal of Medicine
Volume92
Issue number2
Publication statusPublished - Feb 1999

Keywords

  • Adrenal Cortex Hormones
  • Adult
  • Central Nervous System Diseases
  • Cyclosporine
  • Drug Combinations
  • Female
  • Humans
  • Immunosuppressive Agents
  • Magnetic Resonance Imaging
  • Male
  • Middle Aged
  • Prognosis
  • Prospective Studies
  • Sarcoidosis
  • Tomography, X-Ray Computed

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