Ishida Kazuyuki, Mitoma Hiroshi, Wada Yoshiaki, Oka Teruaki, Shibahara Junji, Saito Yuko, Murayama Shigeo, Mizusawa Hidehiro
Department of Neurology, Tamagawa Hospital, Setagaya-ku, Tokyo, Japan.
J Neurol Neurosurg Psychiatry. 2007 Feb;78(2):190-2. doi: 10.1136/jnnp.2006.091116. Epub 2006 Nov 21.
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.
抗谷氨酸脱羧酶抗体与进行性小脑共济失调和缓慢进展的胰岛素依赖型糖尿病的发生有关。此前，已有报道抗谷氨酸脱羧酶抗体相关的进行性小脑共济失调和缓慢进展的胰岛素依赖型糖尿病患者脑脊液中IgG的神经生理特征。采用电压门控全细胞记录技术观察到，该患者脑脊液中的IgG选择性抑制浦肯野细胞的抑制性突触后电流。患者死于吸入性肺炎。尸检显示浦肯野细胞几乎完全缺失，并伴有伯格曼胶质细胞增生。因此，该病例中观察到的小脑共济失调的主要原因可能归因于浦肯野细胞几乎完全缺失。本文讨论了浦肯野细胞损伤的发病机制。
