Â鶹´«Ã½Ó³»­

Â鶹´«Ã½Ó³»­

Explore the latest content from across our publications
Join The Â鶹´«Ã½Ó³»­

Log In

Forgot Password?
Create New Account

Loading... please wait

Abstract Details

Are cerebellar signs seen in pre-manifest essential tremor cases? A study of unaffected first-degree relatives of cases
Movement Disorders
P14 - Poster Session 14 (8:00 AM-9:00 AM)
3-012

To quantify the presence of cerebellar signs and/or cerebellar dysfunction in two groups of first-degree relatives at risk for essential tremor (ET).
ET cases can exhibit a range of subtle cerebellar signs. The time course of these signs relative to tremor onset is not known. Whether they are seen in some form in pre-manifest cases is not known either. Here, we stratified unaffected first-degree relatives of ET cases into two groups, hypothesizing that those with more tremor (“pre-ET”) would also exhibit more signs of cerebellar dysfunction than those with less tremor. 
273 first-degree relatives of ET cases, none of whom reported tremor or an ET diagnosis, and none who were diagnosed with ET based on our detailed neurological examination, completed tasks to screen for cerebellar abnormalities. Tremor was quantified using a total tremor score (TTS, 0 – 30). TTS ranged from 0 – 14 (median = 6.0). The cohort was divided into two groups based on the median TTS: higher TTS (HTTS, “pre-ET”) vs. lower TTS (LTTS).

The two groups were equal in size (137 HTTS vs. 136 LTTS) and had similar mean ages (55.5 HTTS vs. 57.7 LTTS). Pre-ET had more missteps while performing tandem gait (p = 0.033), more intention tremor in the arms (p < 0.001), more intention tremor in the legs (p = 0.045) and marginally diminished ability to stand with both feet touching side-by-side (p = 0.069) than did those with LTTS. In regression models, these differences persisted after adjusting for potential confounding factors.

The links between ET and the cerebellum are multiple. These data provide intriguing evidence that cerebellar signs are more prevalent even among individuals who are likely in the process of converting to ET. The data suggest that the signs are an early disease feature that can occur even in pre-manifest cases.
Authors/Disclosures
Elan D. Louis, MD, MS, FÂ鶹´«Ã½Ó³»­ (University of Texas Southwestern Medical Center)
PRESENTER 		Dr. Louis has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Wolters Kluwer - Merritt's Textbook of Neurology. Dr. Louis has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for Legal Firm. The institution of Dr. Louis has received research support from National Institutes of Health. Dr. Louis has received publishing royalties from a publication relating to health care. Dr. Louis has a non-compensated relationship as a Board of Directors with International Essential Tremor Foundation that is relevant to Â鶹´«Ã½Ó³»­ interests or activities. Dr. Louis has a non-compensated relationship as a Medical Advisory Board with HopeNET that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.
No disclosure on file
No disclosure on file
No disclosure on file
No disclosure on file