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Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia

¹ Department of Neurology, Tamagawa Hospital, Setagaya-ku, Tokyo, Japan
² Mitoma Neurological Clinic, Shinjuku-ku, Tokyo, Japan
³ Department of Rehabilitation, Tamagawa Hospital, Setagaya-ku, Tokyo, Japan
⁴ Department of Pathology, Kanto Central Hospital, Setagaya-ku, Tokyo, Japan
⁵ Department of Pathology, University of Tokyo, Bunkyo-ku, Tokyo, Japan
⁶ Department of Neuropathology, Tokyo Metropolitan Institute of Gerontology, Itabashi-ku, Tokyo, Japan
⁷ Department of Neurology and Neurological Science, Tokyo Medical and Dental University Graduate School, Bunkyo-ku, Tokyo, Japan

Correspondence to:
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.

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

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