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Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia
  1. Kazuyuki Ishida (k-ishida{at}iom.twmu.ac.jp)
  1. Institute of Oriental Medicine, Tokyo Women?fs Medical University, School of Medicine, Japan
    1. Hiroshi Mitoma
    1. Mitoma Neurological Clinic, Japan
      1. Yoshiaki Wada
      1. Department of Rehabilitation, Tamagawa Hospital, Japan
        1. Teruaki Oka
        1. Department of Pathology, Kanto Central Hospital, Japan
          1. Junji Shibahara
          1. Department of Pathology, University of Tokyo, Japan
            1. Yuko Saito
            1. Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Japan
              1. Shigeo Murayama
              1. Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Japan
                1. Hidehiro Mizusawa
                1. Department of Neurology, Tokyo Medical and Dental University, Japan

                  Abstract

                  Anti-glutamic acid decarboxylase (GAD) antibody is associated with the development of progressive cerebellar ataxia (PCA) and slowly progressive insulin-dependent diabetes mellitus (SPIDDM). Previously, we reported the neurophysiological characteristics of IgG in the cerebrospinal fluid (CSF) in a patient with anti-GAD antibody-associated PCA and SPIDDM. Using a voltage-gated whole-cell recording technique, we observed that the IgG in the CSF of the patient, selectively suppressed the inhibitory postsynaptic currents in the Purkinje cells. The patient died due to aspiration pneumonia. Post-mortem examination revealed almost complete depletion of the Purkinje cells with Bergmann gliosis. Therefore, the main cause of cerebellar ataxia that was observed in this case may be attributed to the near-complete depletion of the Purkinje cells. In this study, we have discussed the pathomechanisms underlying the Purkinje cell damage.

                  • Purkinje cell
                  • anti-GAD antibody
                  • cerebellar ataxia
                  • islets of Langerhans
                  • slowly progressive insulin dependent diabetes mellitus

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