Central nervous system sarcoidosisdiagnosis and management

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.

Clinical features of spinal cord sarcoidosis: analysis of 17 neurosarcoidosis patients

“…The diagnosis of neurosarcoidosis was based on the diagnostic criteria proposed by Zajicek et al [2] and the response to steroid treatment. We retrospectively reviewed medical records, and collected data on the course of neurosarcoidosis, diagnostic imaging, laboratory findings, and treatment regimens as well as their results.…”

“…Neurosarcoidosis lesions mainly involve the optic and cranial nerves and the spinal cord [2]. The diagnosis of neurosarcoidosis is often difficult in clinical practice and requires magnetic resonance imaging (MRI) to detect the abnormalities and the evidence of systemic sarcoidosis [2,3].…”

“…The diagnosis of neurosarcoidosis is often difficult in clinical practice and requires magnetic resonance imaging (MRI) to detect the abnormalities and the evidence of systemic sarcoidosis [2,3]. Spinal cord sarcoidosis exhibits intramedullary high-intensity signals on T2-weighted images (T2WI) and enhancements on gadolinium (Gd) DTPA-enhanced T1 weighted images (Gd-T1WI) [4,5]; however, these signs also resemble cervical myelopathy with spondylosis [6].…”

A case of neurosarcoidosis with necrotizing granuloma expressing angiotensin-converting enzyme

“…Some cases improve rapidly while others do not respond at all. Failure of response to steroid and clinical deterioration during late reduction after long term administration of oral corticoids warrant additional immunosuppressive agent, such as azathioprine, methotrexate, cyclophosphamide, cyclosporine and mycophenolate mofetil [5,19,20]. In cases resistant to immunosuppressive treatment, the TNF-α antagonists, pentoxifylline and thalidomide are reported to be effective and safe treatment with good steroid sparing effects [21,22,23].…”

“…[3,5,6]. At 6 months after the initiation of glucocorticoid therapy, his symptom disappeared and serum ACE level returned to normal (11.5 IU/l).…”