Cerebellar Pathology in Early Onset and Late Onset Essential Tremor

Sheng Han Kuo, Jie Wang, William J. Tate, Ming Kai Pan, Geoffrey C. Kelly, Jesus Gutierrez, Etty P. Cortes, Jean Paul G. Vonsattel, Elan D. Louis, Phyllis L. Faust

Research output: Contribution to journalArticle

17 Scopus citations

Abstract

Early onset and late onset essential tremor (ET) cases differ in several respects. Whether they differ with respect to cerebellar pathologic changes remains to be determined. We quantified a broad range of postmortem features (Purkinje cell (PC) counts, PC axonal torpedoes and associated axonal changes, heterotopic PCs, and hairy basket ratings) in 30 ET cases with age of tremor onset <50 years, 30 ET cases with age of tremor onset ≥50 years, and 30 controls (total n = 90). We also used two alternative age of onset cut-points (<40 vs. ≥40 years, and <60 vs. ≥60 years) to define early onset vs. late onset ET. We found that ET cases with tremor onset <50 years and tremor onset ≥50 years had similar PC counts (8.78 ± 1.70 vs. 8.86 ± 1.24, p = 0.839), PC axonal torpedo counts (17.87 ± 18.27 [median =13.00] vs. 12.90 ± 10.60 [median =9.0], p = 0.486) and associated axonal pathology (all p values >0.05), heterotopic PC counts (9.90 ± 11.55 [median =6.00] vs. 5.40 ± 5.10 [median =3.50], p = 0.092), and hairy basket ratings (1.95 ± 0.62 [median =2.00] vs. 2.05 ± 0.92 [median =2.00], p = 0.314). When using the age of onset cut-points of 40 or 60 years, results were similar. Early onset and late onset ET cases share similar cerebellar postmortem features. These data do not support the notion that these age-of-onset related forms of ET represent distinct clinical-pathological entities.

Original languageEnglish (US)
Pages (from-to)473-482
Number of pages10
JournalCerebellum
Volume16
Issue number2
DOIs
StatePublished - Apr 1 2017
Externally publishedYes

Keywords

  • Age of onset
  • Cerebellum
  • Essential tremor
  • Neurodegenerative
  • Pathology
  • Purkinje cell

ASJC Scopus subject areas

  • Neurology
  • Clinical Neurology

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  • Cite this

    Kuo, S. H., Wang, J., Tate, W. J., Pan, M. K., Kelly, G. C., Gutierrez, J., Cortes, E. P., Vonsattel, J. P. G., Louis, E. D., & Faust, P. L. (2017). Cerebellar Pathology in Early Onset and Late Onset Essential Tremor. Cerebellum, 16(2), 473-482. https://doi.org/10.1007/s12311-016-0826-5