ABSTRACT. A 4-year-old intact female Pekingese dog was presented with ataxia and seizure episodes. Based on magnetic resonance imaging and cerebrospinal fluid analysis results, meningoencephalitis of unknown etiology was suspected. The present case survived for 1,096 days under cyclosporine plus prednisolone therapy and was definitively diagnosed with necrotizing meningoencephalitis. This report describes the clinical findings, serial magnetic resonance imaging characteristics and pathologic features of a necrotizing meningoencephalitis and long-term survival after cyclosporine with prednisolone therapy. KEY WORDS: canine, cyclosporine, necrotizing meningoencephalitis (NME).doi: 10.1292/jvms.11-0468; J. Vet. Med. Sci. 74(6): 765-769, 2012 Necrotizing meningoencephalitis (NME) is an idiopathic noninfectious inflammatory disorder of the brain in dogs and has been reported in various breeds, including the Pekingese, Yorkshire terrier, Maltese, Chihuahua, Pug and Shih tzu [2,5,7,8,[12][13][14]. While there is little information about the pathogenesis of NME, several previous reports [9,10,12] have described the presence of autoantibodies against glial fibrillary acidic protein in the cerebrospinal fluid (CSF) of affected dogs, indicating an autoimmune pathophysiology of NME. Thus, NME is suggested to be an idiopathic autoimmune CNS disease like granulomatous meningoencephalitis (GME). Beneficial but limited effects of various immunosuppressive drugs in autoimmune CNS inflammatory diseases have been reported previously [1,2,4,6,7,16].This report describes the clinical findings, serial magnetic resonance imaging (MRI) characteristics, histopathologic changes, and long-term management with cyclosporine in an NME case.A 4-year-old intact female Pekingese dog with a body weight of 3.9 kg was presented with a 1-week history of ataxia and seizure episodes. Neurological signs were observed suddenly and worsened gradually. On neurological examination, postural reactions of 4 limbs were reduced, and other reflexes, including cranial nerve and spinal reflexes, revealed no remarkable findings. The results of a fundus examination and other ophthalmologic examinations were normal. The results of complete blood count, serum chemistry profiling, and radiography were not remarkable. On the basis of a neurological examination, the clinical signs were deemed to be likely due to an intracranial lesion.We then performed a brain MRI scan using a 0.2-T scanner (E-scan ® , ESAOTE, Genova, Italy). T1-and T2-weighted images, and postcontrast T1-weighted images were obtained. Analysis of the images revealed multifocal lesions in the cerebrum (Fig. 1). Multiple, ill-defined hyperintense lesions were noted in the white matter area on both sides (left and right) of the frontal, parietal and temporal lobes including the cortical portion on T2-weighted images (Fig. 1B, 1D and 1F). These lesions appeared isointense or hypointense on T1-weighted images (Fig. 1A, 1C and 1E) and were not enhanced after a contrast study. Results of CSF analysis indi...