Fulminant Myelopathy following Neurogenic Proximal Weakness Associated with Human T-Cell Lymphotropic Virus Type I Infection

We report a patient with human T-cell lymphotropic virus type I (HTLV-I) infection, who presented with proximal extremity neurogenic muscular weakness followed by fulminant myelopathy, but with no upper motor symptoms. The symptoms were inconsistent with the World Health Organization or El Escorial criteria for HTLV-I-associated myelopathy/tropical spastic paraparesis (HAM/TSP) or amyotrophic lateral sclerosis (ALS). This case indicates that fulminant myelopathy without upper motor neuronal symptoms may occur long after the onset of HTLV-I-associated neurogenic proximal muscular weakness. Additionally, we report that treatment with high-dose steroid pulse therapy partially improves symptoms of lightning pain and sensory disturbance.