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Anti-aquaporin 4 antibody in selected Japanese multiple sclerosis patients with long spinal cord lesions

Department of Neurology, Brain Research Institute, Niigata University, Niigata Japan, keiko{at}bri.niigata-u.ac.jp

T. Tani

Department of Neurology, Brain Research Institute, Niigata University, Niigata Japan

M. Tanaka

Department of Neurology, Utano National Hospital, Kyoto, Japan

T. Saida

Department of Neurology, Utano National Hospital, Kyoto, Japan

J. Idezuka

Department of Neurology, Ojiya Sakura Hospital, Ojiya, Japan

M. Yamazaki

Department of Cellular and Neurobiology, Brain Research Institute, Niigata University, Niigata, Japan

M. Tsujita

Department of Cellular and Neurobiology, Brain Research Institute, Niigata University, Niigata, Japan

T. Nakada

Center for Integrated Human Brain Science, Brain Research Institute, Niigata University, Niigata, Japan

K. Sakimura

Department of Cellular and Neurobiology, Brain Research Institute, Niigata University, Niigata, Japan

M. Nishizawa

Department of Neurology, Brain Research Institute, Niigata University, Niigata Japan

Multiple sclerosis (MS) in Asian populations is often characterized by the selective involvement of the optic nerve (ON) and spinal cord (SP) (OSMS) in contrast to classic MS (CMS), where frequent lesions are observed in the cerebrum, cerebellum or brainstem. In Western countries, inflammatory demyelinating disease preferentially involving the ON and SP is called neuromyelitis optica (NMO). Recently, Lennon et al. discovered that NMO-IgG, shown to bind to aquaporin 4 (AQP4), could be a specific marker of NMO and also of Japanese OSMS whose clinical features were identical to NMO having long spinal cord lesions extending over three vertebral segments (LCL). To examine this antibody in larger populations of Japanese OSMS patients in order to know its epidemiological and clinical spectra, we established an immunohistochemical detection system for the anti-AQP4 antibody (AQP4-Ab) using the AQP4-transfected human embryonic kidney cell line (HEK-293) and confirmed AQP4-Ab positivity together with the immunohistochemical staining pattern of NMO-IgG in approximately 60% of Japanese OSMS patients with LCL. Patients with OSMS without LCL and those with CMS were negative for this antibody. Our results accorded with those of Lennon et al. suggest that Japanese OSMS with LCL may have an underlying pathogenesis in common with NMO. Multiple Sclerosis 2007; 13: 850—855. http://msj.sagepub.com

Key Words: aquaporin 4 water channel • long spinal cord lesion • neuromyelitis optica • NMO-IgG • opticospinal multiple sclerosis

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