Abstract Details

Title Prevalence estimates and clinical characterization for tremor and dystonia in Progressive Supranuclear Palsy

Topic Movement Disorders

Presentation(s) P3 - Poster Session 3 (5:30 PM-6:30 PM)

Poster/Presentation
Number 10-005

Objective

Determine the prevalence rate and clinical characteristics of tremor and dystonia in patients with progressive supranuclear palsy (PSP).

Background Tremor and dystonia are known to occur in PSP and can sometimes present together. However, there is scant literature on the prevalence and the clinical characteristics of these features, which may help with diagnosis of PSP and development of better-targeted therapies.

Design/Methods We conducted a retrospective analysis of 217 PSP patients. Diagnosis of PSP was established using recent criteria recommended by the Movement Disorders Society. Data for patients exhibiting tremor, dystonia or both were extracted. Demographics and disease-related characteristics were noted. Tremor and dystonia were both classified based on body distribution. Arm tremor was further classified based on severity and activation condition (rest/posture/action).

Results 71 patients (33% of 217) with tremor were identified (35 males, 36 females; mean age 72.8 ± 8.2 years; mean disease duration 5.4 ± 4.2 years). Tremor distribution included 68 arm (58 mild, 10 moderate, none severe); 3 head; 6 leg; and 5 with tremor affecting multiple body parts. Arm tremor was noted as rest in 34; postural in 35 and action in 61 patients. 66 patients (30%) with dystonia were identified (28 males, 38 females, mean age 73.0 ± 7.6 years, mean disease duration 5.3 ± 4.3 years). Distribution of dystonia included 33 cervical, 17 blepharospasm, 43 limb, 4 cranial (face, jaw, tongue) and 5 generalized. Only 27 patients (12%) presented with combined tremor + dystonia, of which 16 had tremor and dystonia affecting the same body part (mainly limbs).

Conclusions In a large PSP cohort, nearly a third of patients had tremor or dystonia, but these were infrequently co-occurring. Tremor was characteristically a mild, action tremor involving the arms. Dystonia was more prevalent in females and mainly focal, involving the limbs more than the axial or cervical region.

Authors/Disclosures
Aparna Wagle-Shukla, MD, FAAN (UF Fixel) PRESENTER	Dr. Wagle-Shukla has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Encora. Dr. Wagle-Shukla has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Supernus. Dr. Wagle-Shukla has received personal compensation in the range of $500-$4,999 for serving as an officer or member of the Board of Directors for Tremor research group. The institution of Dr. Wagle-Shukla has received research support from NIH. Dr. Wagle-Shukla has received personal compensation in the range of $500-$4,999 for serving as a reviewer with NIH.
	No disclosure on file
Zakia Jabarkheel (University of Florida Department of Neurology)	No disclosure on file
Nikolaus McFarland, MD, PhD, FAAN (University of Florida)	Dr. McFarland has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sutter Health. Dr. McFarland has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ONO Pharmaceuticals. Dr. McFarland has received personal compensation in the range of $0-$499 for serving on a Scientific Advisory or Data Safety Monitoring board for Ferrer. The institution of Dr. McFarland has received research support from NIH, Michael J. Fox Foundation, Huntington Disease Society for America, CurePSP, and Mission MSA.. Dr. McFarland has received publishing royalties from a publication relating to health care.

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