Objectives Neurosarcoidosis may present with longitudinally extensive transverse myelitis (LETM) posing a diagnostic challenge. We describe the clinical features, radiology and management of five patients with spinal neurosarcoidosis (SNS).
Methods We retrospectively identified five patients with a diagnosis of SNS and the clinical, radiological and pathological data were reviewed.
Results There were three females and two males who were, on average, 49 years at onset. A histopathological diagnosis of sarcoid was confirmed in two cases (lymph node and cerebral lesion) and the diagnosis was radiological in the others (LETM with persistent enhancement in 3 patients and the trident sign in 2 patients). The average span of the spinal cord lesions was 4 vertebral bodies. Three patients had FDG-avid mediastinal lymph nodes. Cerebral disease was identified in two cases, and cardiac involvement in one. Two patients required spinal decompression surgery. All patients received intravenous and oral steroids and some had rituximab (n=2), tocilizumab (n=1), cyclophosphamide(n=1), adalimumab (n=2), and infliximab (n=4). Disease control was achieved with TNF-alpha (tumour necrosis factor-alpha) blocking in 4 cases and another responded to cyclophosphamide. The mean follow-up was 55 months.
Conclusions SNS is a cause of LETM and can be suspected by the trident sign on MRI. Persistent enhancement may be another differentiating feature. Spinal cord oedema requiring surgery may occur and patients typically respond to treatment with TNF-alpha inhibitors.
Flanagan EP, Kaufmann TJ, Krecke KN, Aksamit AJ, Pittock SJ, Keegan BM, Giannini C, Weinshenker BM. Discriminating long myelitis of neuromyelitis optica from sarcoidosis. Ann Neurol 2016 Mar;79(3):437–47.
Zalewski NL, MD, Krecke KN, Weinshenker BG, MD, Aksamit AJ, Conway BL, McKeon A, Flanagan EP. Central canal enhancement and the trident sign in spinal cord sarcoidosis. Neurology 2016 Aug 16;87(7):743–4.
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