Characterizing Lewy Pathology in 231 Essential Tremor Brains From the Essential Tremor Centralized Brain Repository

Elan D. Louis, Daniella Iglesias-Hernandez, Nora C. Hernandez, Xena Flowers, Sheng Han Kuo, Jean Paul G. Vonsattel, Phyllis L. Faust

Research output: Contribution to journalArticlepeer-review


The Essential Tremor Centralized Brain Repository is the largest repository of prospectively collected essential tremor (ET) brains (n = 231). Hence, we are uniquely poised to address several questions: What proportion of ET cases has Lewy pathology (LP)? What is the nature of that pathology and how does it relate to other comorbidities? Each brain had a complete neuropathological assessment, including α-synuclein immunostaining. We created a 10-category classification scheme to fully encapsulate the patterns of LP observed. Four metrics of cerebellar pathology were also quantified. Mean age at death = 89.0 ± 6.4 years. Fifty-eight (25.1%) had LP and 46 (19.9%) had early to late stages of Parkinson disease (PD). LP was very heterogeneous. Of 58 cases with LP, 14 (24.1%) clinically developed possible PD or PD after a latency of 5 or more years. There was a similar degree of cerebellar pathology in ET cases both with and without LP. In summary, 1 in 4 ET cases had LP-a proportion that seems higher than expected based on studies among control populations. Heterogeneous LP likely reflects clinical associations between ET and PD, and ET with Alzheimer disease-type neuropathology. These data further our understanding of ET and its relatedness to other degenerative diseases.

Original languageEnglish (US)
Pages (from-to)796-806
Number of pages11
JournalJournal of neuropathology and experimental neurology
Issue number10
StatePublished - Sep 19 2022
Externally publishedYes


  • Alzheimer disease
  • Brain repository
  • Cerebellar degeneration
  • Essential tremor
  • Lewy pathology
  • Neurodegeneration
  • Parkinson disease

ASJC Scopus subject areas

  • Medicine(all)


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