Reduced Purkinje cell number in essential tremor: a postmortem study

Archives of Neurology
Jordan E AxelradJean Paul G Vonsattel

Abstract

Clinical and functional imaging evidence suggests that cerebellar dysfunction occurs in essential tremor (ET). In recent postmortem studies, we documented increased numbers of torpedoes (Purkinje cell axonal swellings) in ET patients without Lewy bodies. Purkinje cell loss, however, has never been rigorously assessed. To quantitatively assess the number of Purkinje cells in brains of ET patients and similarly aged controls. Postmortem cerebellar tissue was available in 14 ET cases (6 with Lewy bodies and 8 without Lewy bodies) and 11 controls. Calbindin immunohistochemistry was performed on paraffin sections of the cerebellum. Images were digitally recorded and blinded measurements of the number of Purkinje cells per millimeter of cell layer (linear density) were made. Purkinje cell linear density was inversely correlated with age (r= - 0.53, P= .006) and number of torpedoes (r= - 0.42, P= .04). Purkinje cell linear density differed by diagnosis (mean [SD], controls, 3.46 [1.27] cells/mm; ET cases with Lewy bodies, 3.33 [1.06] cells/mm; and ET cases without Lewy bodies, 2.14 [0.82] cells/mm; P= .04), with the most significant difference between ET cases without Lewy bodies and controls, where the reduction was 38.2% (P= .04). I...Continue Reading

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