Selective loss of Purkinje cells in a patient with anti-glutamic acid decarboxylase antibody-associated cerebellar ataxia.
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.
Ishida
Kazuyuki
K
Department of Neurology, Tamagawa Hospital, Setagaya-ku, Tokyo, Japan. k-ishida@iom.twmu.ac.j.
Mitoma
Hiroshi
H
Wada
Yoshiaki
Y
Oka
Teruaki
T
Shibahara
Junji
J
Saito
Yuko
Y
Murayama
Shigeo
S
Mizusawa
Hidehiro
H
D000368:Aged; D001323:Autoantibodies; D001344:Autopsy; D002524:Cerebellar Ataxia / Q000276:immunology* / Q000473:pathology*; D005260:Female; D005968:Glutamate Decarboxylase / Q000276:immunology*; D006801:Humans; D007074:Immunoglobulin G / Q000134:cerebrospinal fluid; D011689:Purkinje Cells / Q000473:pathology* / Q000502:physiology
D001323:Autoantibodies; D007074:Immunoglobulin G; D005968:Glutamate Decarboxylase