Title | Quantification of cerebellar hemispheric purkinje cell linear density: 32 ET cases versus 16 controls. |
Publication Type | Journal Article |
Year of Publication | 2013 |
Authors | Louis ED, Babij R, Lee M, Cortés E, Vonsattel J-PG |
Journal | Mov Disord |
Volume | 28 |
Issue | 13 |
Pagination | 1854-9 |
Date Published | 2013 Nov |
ISSN | 1531-8257 |
Keywords | Aged, Aged, 80 and over, Calbindin 1, Cell Count, Cerebellum, Essential Tremor, Female, Functional Laterality, Humans, Male, Purkinje Cells, Statistics, Nonparametric |
Abstract | Although essential tremor (ET) is among the most prevalent neurological diseases, its precise pathogenesis is not understood. Purkinje cell loss has been observed in some studies and is the focus of interest and debate. Expressing these data as Purkinje cells/layer length allows one to adjust for the inherent curved nature of the cerebellar folia. Capitalizing on the Essential Tremor Centralized Brain Repository, we quantified Purkinje cell linear density in cases versus controls. Free-floating 100-μm parasagittal cerebellar hemispheric sections were subjected to rabbit polyclonal anti-Calbindin D28k antibody, and 10 random fields/brain were selected for quantification of Purkinje cells/mm(-1) Purkinje cell layer. Purkinje cell linear density was lower in 32 ET cases than in16 controls (1.14 ± 0.32 vs. 1.35 ± 0.31/mm(-1) , P = 0.03). Purkinje cell linear density was inversely associated with torpedo count (r = -0.38, P = 0.028). The current sample of ET cases demonstrates a reduction in Purkinje cell number relative to that of controls. Greater Purkinje cell axonal remodeling (torpedoes) was found in individuals who had the most Purkinje cell drop out. The role of Purkinje cell loss in the pathogenesis of this disorder merits additional study. |
DOI | 10.1002/mds.25629 |
Alternate Journal | Mov. Disord. |
PubMed ID | 23925732 |
PubMed Central ID | PMC3830681 |
Grant List | T32 NS007153 / NS / NINDS NIH HHS / United States R01 NS039422 / NS / NINDS NIH HHS / United States R21 NS077094 / NS / NINDS NIH HHS / United States R01 NS073872 / NS / NINDS NIH HHS / United States R01 NS042859 / NS / NINDS NIH HHS / United States R01 NS036630 / NS / NINDS NIH HHS / United States |
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