Movement Disorders (revue)

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Quantification of Cerebellar Hemispheric Purkinje Cell Linear Density: 32 ET Cases vs. 16 Controls

Identifieur interne : 000818 ( Main/Exploration ); précédent : 000817; suivant : 000819

Quantification of Cerebellar Hemispheric Purkinje Cell Linear Density: 32 ET Cases vs. 16 Controls

Auteurs : Elan D. Louis [États-Unis] ; Rachel Babij [États-Unis] ; Michelle Lee [États-Unis] ; Etty Cortés [États-Unis] ; Jean-Paul G. Vonsattel [États-Unis]

Source :

RBID : PMC:3830681

English descriptors

Abstract

Background

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 vs. controls.

Methods

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.

Results

Purkinje cell linear density was lower in 32 ET cases than 16 controls (1.14 ± 0.32 vs. 1.35 ± 0.31 per mm−1, p = 0.03). Purkinje cell linear density was inversely associated with torpedo count (r = −0.38, p = 0.028).

Discussion

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.


Url:
DOI: 10.1002/mds.25629
PubMed: 23925732
PubMed Central: 3830681


Affiliations:


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<title level="j">Movement disorders : official journal of the Movement Disorder Society</title>
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<term>Aged</term>
<term>Aged, 80 and over</term>
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<term>Cell Count</term>
<term>Cerebellum (pathology)</term>
<term>Essential Tremor (pathology)</term>
<term>Female</term>
<term>Functional Laterality</term>
<term>Humans</term>
<term>Male</term>
<term>Purkinje Cells (metabolism)</term>
<term>Purkinje Cells (pathology)</term>
<term>Statistics, Nonparametric</term>
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<term>Essential Tremor</term>
<term>Purkinje Cells</term>
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<term>Aged</term>
<term>Aged, 80 and over</term>
<term>Cell Count</term>
<term>Female</term>
<term>Functional Laterality</term>
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<div type="abstract" xml:lang="en">
<sec id="S1">
<title>Background</title>
<p id="P1">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 vs. controls.</p>
</sec>
<sec id="S2">
<title>Methods</title>
<p id="P2">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
<sup>−1</sup>
Purkinje cell layer.</p>
</sec>
<sec id="S3">
<title>Results</title>
<p id="P3">Purkinje cell linear density was lower in 32 ET cases than 16 controls (1.14 ± 0.32 vs. 1.35 ± 0.31 per mm
<sup>−1</sup>
, p = 0.03). Purkinje cell linear density was inversely associated with torpedo count (r = −0.38, p = 0.028).</p>
</sec>
<sec id="S4">
<title>Discussion</title>
<p id="P4">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.</p>
</sec>
</div>
</front>
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