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Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia
  1. Kazuyuki Ishida1,
  2. Hiroshi Mitoma2,
  3. Yoshiaki Wada3,
  4. Teruaki Oka4,
  5. Junji Shibahara5,
  6. Yuko Saito6,
  7. Shigeo Murayama6,
  8. Hidehiro Mizusawa7
  1. 1Department of Neurology, Tamagawa Hospital, Setagaya-ku, Tokyo, Japan
  2. 2Mitoma Neurological Clinic, Shinjuku-ku, Tokyo, Japan
  3. 3Department of Rehabilitation, Tamagawa Hospital, Setagaya-ku, Tokyo, Japan
  4. 4Department of Pathology, Kanto Central Hospital, Setagaya-ku, Tokyo, Japan
  5. 5Department of Pathology, University of Tokyo, Bunkyo-ku, Tokyo, Japan
  6. 6Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Itabashi-ku, Tokyo, Japan
  7. 7Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School, Bunkyo-ku, Tokyo, Japan
  1. Correspondence to:
 Dr Kazuyuki Ishida
 Institute of Oriental Medicine, Tokyo Women’s Medical University, School of Medicine, 4th floor, Shinjuku NS Building, 2-4-1 Nishi-Shinjuku, Shinjuku-ku, Tokyo 163-0804, Japan; k-ishida{at}iom.twmu.ac.jp

Abstract

Anti-glutamic acid decarboxylase antibody is associated with the development of progressive cerebellar ataxia and slowly progressive insulin-dependent diabetes mellitus. Previously, the neurophysiological characteristics of IgG in the cerebrospinal fluid of a patient with anti-glutamic acid decarboxylase antibody-associated progressive cerebellar ataxia and slowly progressive insulin-dependent diabetes mellitus were reported. Using a voltage-gated whole-cell recording technique, it was observed that the IgG in the cerebrospinal fluid of the patient selectively suppressed the inhibitory postsynaptic currents in the Purkinje cells. The patient died from aspiration pneumonia. Postmortem examination showed almost complete depletion of the Purkinje cells with Bergmann gliosis. Therefore, the main cause of cerebellar ataxia observed in this case may be attributed to the near-complete depletion of the Purkinje cells. In this paper, the pathomechanisms underlying Purkinje cell damage are discussed.

  • GAD, glutamic acid decarboxylase
  • PCA, progressive cerebellar ataxia
  • SPIDDM, slowly progressive insulin-dependent diabetes mellitus
  • SPS, stiff-person syndrome

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Footnotes

  • Competing interests: None declared.

  • Informed consent was obtained from the family of the patient for the publication of her details in this paper.

  • Published Online First 17 November 2006

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