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Cerebellum Ataxias. 2018 Jan 15;5:1. doi: 10.1186/s40673-018-0080-3. eCollection 2018.

Inferior Olivary nucleus degeneration does not lessen tremor in essential tremor.

Author information

1Department of Neurology, Yale School of Medicine, Yale University, 15 York Street, PO Box 208018, New Haven, CT 06520-8018 USA.
2Department of Chronic Disease Epidemiology, Yale School of Public Health, Yale University, New Haven, CT USA.
3Center for Neuroepidemiology and Clinical Neurological Research, Yale School of Medicine, Yale University, New Haven, CT USA.
4Department of Neurology, College of Physicians and Surgeons, Columbia University, New York, NY USA.
5Department of Neurology and Institute of Neurology, First Affiliated Hospital, Fujian Medical University, Fuzhou, China.
6Department of Pathology and Cell Biology, Columbia University Medical Center and the New York Presbyterian Hospital, New York, NY USA.
7Taub Institute for Research on Alzheimer's Disease and the Aging Brain, Columbia University, New York, NY USA.



In traditional models of essential tremor, the inferior olivary nucleus was posited to play a central role as the pacemaker for the tremor. However, recent data call this disease model into question.

Case presentation:

Our patient had progressive, long-standing, familial essential tremor. Upper limb tremor began at age 10 and worsened over time. It continued to worsen during the nine-year period he was enrolled in our brain donation program (age 85 - 94 years), during which time the tremor moved from the moderate to severe range on examination. On postmortem examination at age 94, there were degenerative changes in the cerebellar cortex, as have been described in the essential tremor literature. Additionally, there was marked degeneration of the inferior olivary nucleus, which was presumed to be of more recent onset. Such degeneration has not been previously described in essential tremor postmortems. Despite the presence of this degeneration, the patient's tremor not only persisted but it continued to worsen during the final decade of his life.


Although the pathophysiology of essential tremor is not completely understood, evidence such as this suggests that the inferior olivary nucleus does not play a critical role in the generation of tremor in these patients.


Cerebellum; Essential tremor; Inferior olivary nucleus; Neurodegenerative; Pathology; Purkinje cell

Conflict of interest statement

The following text appears in the Methods section of our manuscript: “…enrolled in the Essential Tremor Centralized Brain Repository (ETCBR), a joint effort between investigators at Yale and Columbia Universities. Upon enrollment, the patient signed a written informed consent form. This ethical form was approved by both the Yale and Columbia University ethics committees.”Not applicable.The authors declare that they have no competing interests.Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

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