Central nervous system sarcoidosisdiagnosis and management

TLDR

The study proposes a clinical diagnostic probability classification for neurosarcoidosis and highlights the value of a large prospective database to improve prognosis understanding and therapy development. The authors report a 68‑patient neurosarcoidosis series, detailing clinical presentation, diagnostic work‑up—including MRI, chest radiography, Kveim skin test, Gallium‑67 scan, and CSF studies—and a mean 4.6‑year follow‑up. Most patients (62%) had neurologic disease, mainly involving the optic nerve and chiasm, with cranial nerve palsies, spinal cord, and brainstem manifestations also common; diagnostic yield was highest for the Kveim test (85%), CSF abnormalities (81%), and Gallium‑67 scans (45%); MRI showed meningeal enhancement in 38% and white‑matter lesions in 43%; and more than half of patients followed >18 months progressed despite corticosteroid and immunosuppressive therapy.

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.