Yokota Osamu, Tsuchiya Kuniaki, Terada Seishi, Oshima Kenichi, Ishizu Hideki, Matsushita Masaaki, Kuroda Shigetoshi, Akiyama Haruhiko
Department of Neuropathology, Tokyo Institute of Psychiatry, Tokyo, Japan.
Acta Neuropathol. 2006 Jul;112(1):43-51. doi: 10.1007/s00401-006-0059-7. Epub 2006 Apr 19.
Alcoholic cerebellar degeneration (ACD) is a pivotal neurological complication in alcoholics. However, although there are a few autopsy reports and some data on its frequency, it is considered very rare in Japan. The aims of this study were (1) to estimate the frequency of the disease in Japanese autopsy cases, and (2) to examine the clinicopathological features of symptomatic and asymptomatic cases of ACD. We reviewed the records of 1,509 Japanese autopsies obtained from three autopsy series in Japan, and selected all 55 cases (3.6%) with alcoholism. On neuropathological reexamination, ACD was confirmed in six male alcoholics [0.4% of all subjects; 10.9% of all alcoholics; mean age at death 59.3+/-13.4 years (+/- SD)], including three asymptomatic cases. These frequencies were much lower than some previous Western findings, but more common than that has been expected in Japan. The frequencies of memory impairment and ataxia in ACD cases were significantly higher than those in alcoholics without any alcohol-related pathologies. In ACD cases, loss of Purkinje cells, narrowing of the width of the molecular layer, and tissue rarefaction in the granular layer were observed in the anterior and superior portions of the vermis of the cerebellum. In adjacent regions, the Purkinje cell and molecular layers were more mildly affected. The distribution of severely affected regions was more restricted in the asymptomatic cases than in the symptomatic cases. This study confirmed the frequency of asymptomatic cerebellar degeneration in alcoholics, suggesting that early intervention in alcoholism in the subclinical phase is important to prevent the development of cerebellar symptoms.
酒精性小脑变性（ACD）是酗酒者的一种关键神经并发症。然而，尽管有一些尸检报告以及关于其发病率的一些数据，但在日本它被认为非常罕见。本研究的目的是：（1）估计日本尸检病例中该疾病的发病率；（2）研究有症状和无症状的ACD病例的临床病理特征。我们回顾了从日本三个尸检系列中获取的1509例日本尸检记录，并挑选出所有55例（3.6%）酗酒者。经神经病理学复查，在6名男性酗酒者中确诊为ACD[占所有受试者的0.4%；占所有酗酒者的10.9%；死亡时平均年龄59.3±13.4岁（±标准差）]，其中包括3例无症状病例。这些发病率远低于一些先前西方的研究结果，但比日本此前预期的更为常见。ACD病例中记忆障碍和共济失调的发病率显著高于无任何酒精相关病变的酗酒者。在ACD病例中，在小脑蚓部的前部和上部观察到浦肯野细胞丢失、分子层宽度变窄以及颗粒层组织稀疏。在相邻区域，浦肯野细胞层和分子层受影响较轻。无症状病例中严重受累区域的分布比有症状病例更局限。本研究证实了酗酒者中无症状小脑变性的发病率，表明在亚临床阶段对酗酒进行早期干预对于预防小脑症状的发展很重要。
