A 4-year-old female Maltese suffering from rheumatoid arthritis was presented to our clinic because of acute cervical pain. Neurologic examination was normal. Myelogram disclosed marked swelling of the cervical spinal cord from the 2nd to 5th cervical vertebrae, and cerebrospinal fluid showed elevated protein content (194 mg/dl) and pleocytosis (800/μl, lymphocytes: 90%). Serum antibody to canine distemper virus (CDV) was 1: 1, 024. Corticosteroid treatment did not improve the clinical signs, and the dog was euthanatized on the 42 nd day of illness. Autopsy revealed severe granulomatous inflammation mainly localized in the cervical spinal cord, while CDV antigen or inclusions were not detectable.