Two cases of sarcoidosis presenting as longitudinally extensive transverse myelitis
Neurosarcoidosis is uncommon with an incidence of approximately 5 to 15%. Central nervous system involvement can be divided into brain and spinal cord neurosarcoidosis. Spinal cord sarcoidosis is extremely rare, occurring in less than 1% of all sarcoidosis cases. Its manifestations may include cauda equina syndrome, radiculopathy, syringomyelia, cord atrophy, arachnoiditis, and myelopathy or transverse myelitis. We highlight two cases of spinal cord sarcoidosis, each presenting with longitudinally extensive transverse myelitis, that demonstrate the dilemmas that physicians face with regard to diagnosis and treatment. Given its rarity and the diversity of possible manifestations, establishing the diagnosis of spinal cord sarcoidosis is often very difficult. Extensive evaluation must be conducted to rule out primary neurologic, primary rheumatologic, infectious, and neoplastic diseases. MRI often demonstrates hyperintensity on T2-weighted images and enhancement following gadolinium administration. CSF analysis most consistently shows a lymphocytic pleocytosis and elevated proteins. While these less invasive investigations may be helpful, the gold standard for diagnosis is biopsy of neurologic or non-neurologic tissue confirming the presence of non-caseating granulomas. Evidence-based guidelines for the treatment of transverse myelitis secondary to sarcoidosis are lacking due to its rarity; therefore, therapy is based on expert and anecdotal experience and usually consists of high doses of steroids in combination with various immunosuppressive agents. The use of infliximab in particular appears promising, but there is a need for further investigation into the ideal treatment regimen.
KeywordsImmunosuppressive therapy Neurosarcoidosis Sarcoidosis Spinal cord sarcoidosis Transverse myelitis
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- 4.Durel CA, Marignier R, Maucort-Boulch D, Iwaz J, Berthoux E, Ruivard M, André M, le Guenno G, Pérard L, Dufour JF, Turcu A, Antoine JC, Camdessanche JP, Delboy T, Sève P (2016) Clinical features and prognostic factors of spinal cord sarcoidosis: a multicenter observational study of 20 biopsy-proven patients. J Neurol 263(5):981–990CrossRefPubMedGoogle Scholar
- 9.Cerar T, Strle F, Stupica D, Ruzic-Sabljic E, McHugh G, Steere AC, Strle K (2016) Differences in genotype, clinical features, and inflammatory potential of Borrelia burgdorferi sensu stricto strains from Europe and the United States. Emerg Infect Dis 22(5):818–827CrossRefPubMedPubMedCentralGoogle Scholar