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Cerebellum. 2017 Apr;16(2):473-482. doi: 10.1007/s12311-016-0826-5.

Cerebellar Pathology in Early Onset and Late Onset Essential Tremor.

Author information

1
Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA. sk3295@columbia.edu.
2
Neurological Institute, Columbia University, New York, NY, 10032, USA. sk3295@columbia.edu.
3
Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY, USA.
4
Department of Basic and Community Nursing, School of Nursing, Nanjing Medical University, Nanjing, Jiangsu Province, China.
5
Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York, NY, USA.
6
Department of Medical Research, National Taiwan University, Taipei, Taiwan.
7
Department of Neurology, Yale School of Medicine, Yale University, New Haven, CT, USA.
8
Taub Institute for Research on Alzheimer's disease and the Aging Brain, Columbia University, New York, NY, USA.
9
Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, CT, USA.
10
Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, CT, USA.

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.

KEYWORDS:

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

PMID:
27726094
PMCID:
PMC5336493
DOI:
10.1007/s12311-016-0826-5
[Indexed for MEDLINE]
Free PMC Article

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