We describe six men with a slowly progressive myelopathy characterized by asymmetric, incomplete spinal cord syndrome manifested with a thoracic sensory level, mild spastic paraparesis, and urinary incontinence. The spinal cord lesions were evident by MRI in four of them. Coxiella burnetii infection was confirmed in the blood of all patients by immunofluorescence microscopic assay (IFA) and transmission electron microscopy (TEM). In two patients, we detected C burnetii by TEM and IFA using CSF from the patients inoculated onto fresh peripheral blood lymphocyte. Four patients, treated with appropriate antibiotics, responded either with partial resolution of symptoms or arrest of further neurologic progression. In three, the MRI lesions decreased in size.