Longitudinally extensive transverse myelitis as neurosarcoidosis accompanying duropathy

Abstract

A 61year old male, who had had a motorbike accident 30 years ago and slowly progressed paralysis of bilateral upper extremities, subacutely developed spastic paraplegia, numbness under inferior thoracic level, and bladderintestinal dysfunction. Spinal magnetic resonance imaging (MRI) revealed intramedullary lesions from the C2 to the L2 vertebral level with leptomeningeal gadorium (Gd)enhancement, and ventral intraspinal fluid collection with hemosiderin deposition (Figure 1A– H). Brain MRI revealed Gdenhancement and hemosiderin deposition around the cerebellum and brainstem (Figure 1K– N). Laboratory tests showed no evidence of collagen disease, infection, and malignant tumor, and negative results for antiaquapolin4 antibody and antimyelin oligodendrocyte glycoprotein antibody. Although the pathological findings were not confirmed, neurosarcoidosis accompanying duropathy was suspected on the basis of elevated soluble interleukin2 receptor and lysozyme (normal angiotensinconverting enzyme level), lymphocytes with xanthochromia in CSF, enlarged hilar lymph nodes with positive gallium scintigraphy (Figure 1I, J), and elevated CD4/CD8 ratio in bronchoalveolar lavage.1 Corticosteroid treatment was effective, and remission was maintained with methotrexate, but the upper extremity paralysis did not improve. This case presented longitudinally extensive transverse myelitis. We have reported that neurosarcoidosis had asymptomatic cerebral microbleeds,2 but conversely, stimulation of hemosiderin by exogenous hemorrhages, such as superficial siderosis,3 may lead to more severe neurosarcoidosis.