Abstract
In 1912, Ferdinand Lewy described the presence of hyaline intracellular inclusions within neurons from the substantia nigra of brains with Parkinson’s disease (PD). These Lewy bodies have become the pathognomonic hallmark of PD, although they are not exclusive to PD and are also seen in Diffuse Lewy Body disease as well as Alzheimer’s disease. 5 Unlike PD, where the pathology of nigral cell loss and Lewy body formation has been well studied, information on the pathologic processes underlying ET has been fairly limited. The Essential Tremor Centralized Brain Repository was established in 2003 at Columbia University (New York, NY) and has helped improve the understanding of the pathology of ET. A recent report 6 examined 33 brains of patients with ET and 21 controls. Two broad categories of pathology were seen. The first was the presence of prominent cerebellar changes seen in about 75% of the ET brains. These changes included a significant decline in the number of Purkinje cells and a marked increase in the number of swollen Purkinje cell axons called torpedoes. The remaining 25% of brains showed Lewy bodies confined, for the most part, to the locus ceruleus. These 2 pathologic subtypes appear to be mutually exclusive with the former type, called cerebellar ET, and the latter, the Lewy body variant of ET. The presence of 2 distinct pathologic subtypes further casts doubt on the concept of ET as a homogenous clinicopathologic entity. 7 It remains to be
Published Version
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