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Erschienen in: Clinical Rheumatology 10/2018

17.05.2018 | Case Based Review

Two cases of sarcoidosis presenting as longitudinally extensive transverse myelitis

verfasst von: Amanda Mary Scott, Janeth Yinh, Timothy McAlindon, Robert Kalish

Erschienen in: Clinical Rheumatology | Ausgabe 10/2018

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Abstract

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.
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Metadaten
Titel
Two cases of sarcoidosis presenting as longitudinally extensive transverse myelitis
verfasst von
Amanda Mary Scott
Janeth Yinh
Timothy McAlindon
Robert Kalish
Publikationsdatum
17.05.2018
Verlag
Springer London
Erschienen in
Clinical Rheumatology / Ausgabe 10/2018
Print ISSN: 0770-3198
Elektronische ISSN: 1434-9949
DOI
https://doi.org/10.1007/s10067-018-4144-9

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