Purkinje cell loss in essential tremor

Louis, Elan D.; Faust, Phyllis L.

doi:10.1002/mds.25965

Cited by 17 publications

(10 citation statements)

References 5 publications

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“…Two studies have not detected Purkinje cell loss in patients with ET, although a number of valid methodological concerns have been raised about both studies . Several plausible explanations could account for the differences across studies, and more specifically, the null and/or inconclusive findings reported by the Arizona and Canada studies .…”

Section: Discussionmentioning

confidence: 99%

Purkinje cell loss in essential tremor: Random sampling quantification and nearest neighbor analysis

et al. 2016

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Objective Purkinje cell loss has been documented in some although not all postmortem studies of essential tremor. Hence, there is considerable controversy concerning the presence of Purkinje cell loss in this disease. To date, few studies have been performed. Methods Over the past eight years, we have assembled 50 prospectively-studied cases and 25 age-matched controls; none were reported in our prior large series of 33 essential tremor and 21 controls. In addition to methods used in prior studies, the current study used a random sampling approach to quantify Purkinje cells along the Purkinje cell layer with a mean of 217 sites examined in each specimen, allowing for extensive sampling of the Purkinje cell layer within the section. For the first time, we also quantified the distance between Purkinje cell bodies - a nearest neighbor analysis. Results In the Purkinje cell count data collected from fifteen 100x-fields, cases had lower counts than controls in all three counting criteria (cell bodies, nuclei, nucleoli, all p<0.001). Purkinje cell linear density was also lower in cases than controls (all p<0.001). Purkinje cell linear density obtained by random sampling was similarly lower in cases than controls in all three counting criteria (cell bodies, nuclei, nucleoli, all p≤0.005). In agreement with the quantitative Purkinje cell counts, the mean distance from one Purkinje cell body to another Purkinje cell body along the Purkinje cell layer was greater in cases than controls (p=0.002). Conclusions These data provide support for the neurodegeneration of cerebellar Purkinje cells in essential tremor.

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Section: Discussionmentioning

confidence: 99%

Purkinje cell loss in essential tremor: Random sampling quantification and nearest neighbor analysis

et al. 2016

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“…In relation to pathology, several studies have demonstrated degenerative changes in the cerebellum with an increase in torpedoes [4] and other axonal changes [5], as well as a reduction in the number of Purkinje cells [6]. Other studies have not detected Purkinje cell loss in ET, although a number of methodological limitations have been raised about these studies [7,8].…”

Section: Introductionmentioning

confidence: 93%

Atrophy of the Cerebellar Vermis in Essential Tremor: Segmental Volumetric MRI Analysis

HyeEun

Lee

et al. 2015

Cerebellum

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Postmortem studies of essential tremor (ET) have demonstrated the presence of degenerative changes in the cerebellum, and imaging studies have examined related structural changes in the brain. However, their results have not been completely consistent and the number of imaging studies has been limited. We aimed to study cerebellar involvement in ET using MRI segmental volumetric analysis. In addition, a unique feature of this study was that we stratified ET patients into subtypes based on the clinical presence of cerebellar signs and compared their MRI findings. Thirty-nine ET patients and 36 normal healthy controls, matched for age and sex, were enrolled. Cerebellar signs in ET patients were assessed using the clinical tremor rating scale and International Cooperative Ataxia Rating Scale. ET patients were divided into two groups: patients with cerebellar signs (cerebellar-ET) and those without (classic-ET). MRI volumetry was performed using CIVET pipeline software. Data on whole and segmented cerebellar volumes were analyzed using SPSS. While there was a trend for whole cerebellar volume to decrease from controls to classic-ET to cerebellar-ET, this trend was not significant. The volume of several contiguous segments of the cerebellar vermis was reduced in ET patients versus controls. Furthermore, these vermis volumes were reduced in the cerebellar-ET group versus the classic-ET group. The volume of several adjacent segments of the cerebellar vermis was reduced in ET. This effect was more evident in ET patients with clinical signs of cerebellar dysfunction. The presence of tissue atrophy suggests that ET might be a neurodegenerative disease.

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“…Clinicopathological and neuroimaging investigations in ET patients have pointed out different types of anomalies in the cerebellum that could be involved in tremor generation (Zeuner and Deuschl 2012;Louis 2014). For example, a reduction in the number of Purkinje cells (PC) together with axonal morphometric changes have been described in the cerebellum of a significant subgroup of ET patients Louis et al 2013), but PC loss was not detected in all ET studies Symanski et al 2014), possibly due to methodological differences (Louis and Faust 2014). Still in the cerebellum of ET patients, other studies have reported higher levels of Lingo-1 and neurofilaments compared to controls (Louis et al 2012;Kuo et al 2013;Delay et al 2014).…”

Section: Introductionmentioning

confidence: 96%