Louis Elan D, Vonsattel Jean Paul G, Honig Lawrence S, Lawton Arlene, Moskowitz Carol, Ford Blair, Frucht Steven
Department of Neurology, Taub Institute for Research on Alzheimer's Disease and the Aging Brain, College of Physicians and Surgeons, Columbia University, 710 W. 168th Street, New York, NY 10032, USA.
Arch Neurol. 2006 Aug;63(8):1189-93. doi: 10.1001/archneur.63.8.1189.
Although essential tremor (ET) is one of the most common neurologic disorders, there have been few postmortem studies. We recently reported postmortem changes (torpedoes and Bergmann gliosis) in the cerebellar cortex in a few ET cases.
To describe more extensive postmortem changes in the cerebellum in another ET case.
Case report.
A 90-year-old woman had a 30-year history of ET. At postmortem examination, there was segmental loss of Purkinje cells, presence of torpedoes, and Bergmann gliosis in the cerebellar cortex. Moreover, there were extensive changes in the dentate nucleus, in the form of neuronal loss, neuronal atrophy, microglial clusters, and reduction in the number of efferent fibers (ie, pallor of the hilum).
The brain in the current case exhibited more marked cerebellar pathologic features than noted in previously reported ET cases and thereby extends the described cerebellar findings in this common, yet pathologically poorly characterized, neurologic disorder.
尽管特发性震颤（ET）是最常见的神经系统疾病之一，但尸检研究却很少。我们最近报告了几例ET病例小脑皮质的尸检变化（鱼雷样小体和伯格曼胶质细胞增生）。
描述另一例ET病例小脑更广泛的尸检变化。
病例报告。
一名90岁女性有30年ET病史。尸检时，小脑皮质出现浦肯野细胞节段性缺失、鱼雷样小体和伯格曼胶质细胞增生。此外，齿状核有广泛变化，表现为神经元丢失、神经元萎缩、小胶质细胞簇以及传出纤维数量减少（即门部苍白）。
与先前报道的ET病例相比，本例患者大脑的小脑病理特征更为明显，从而扩展了这种常见但病理特征尚不明确的神经系统疾病的小脑研究结果。
