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

Prevalence of Surgical Procedures in Patients with Prion Disease
Aging, Dementia, and Behavioral Neurology
Aging and Dementia Posters (7:00 AM-5:00 PM)
091
To determine the prevalence of surgeries within one year of onset of symptoms attributed to definite or probable prion disease.
Patients with prion disease commonly present with visual, sensorimotor or gait complaints that may prompt surgical interventions for related disorders (e.g., cataracts, carpal tunnel syndrome and joint disease). To prevent iatrogenic transmission of prion disease, surgical instruments used on a patient with possible prion disease must be decontaminated or decommissioned. This is only done when the diagnosis of possible prion disease is recognized by the healthcare team.
The Mayo Clinic’s Advanced Cohort Explorer was used to identify patients assessed since 1/1/2014 with prion disease diagnostic codes (n=296). Patients with prion disease enrolled in prospective studies of rapidly progressive dementia beginning February 2015 at Washington University School of Medicine (St. Louis, MO) were also included (n=14). Charts were independently reviewed by two team members, and details extracted concerning demographics, clinical course, results of investigations, surgical interventions and outcomes. 

108 patients with probable or definite prion disease were identified, including 62 (57%) with autopsy- or genetically-proven prion disease (57=sporadic, 4 familial, 1 fatal familial insomnia). The majority of patients experienced a rapidly progressive course (mean symptom duration 10.0±9.7 months). Twenty-nine patients (26.9%) underwent 36 surgeries or invasive procedures using durable instruments, including 3 high-risk procedures directly involving the central nervous system, and 13 intermediate- (e.g., joint replacement) and 20 low-risk procedures (e.g., endoscopy). Surgeries were performed a median of 10.2 months before death (range: 0.8-62.3), with 20 (55.6%) performed before symptom onset. Surgical operators were unaware of the diagnosis of possible prion disease in all but one case (diagnostic brain biopsy).
Surgeries were prevalent in patients with new diagnoses of prion disease in this series. Coordinated approaches to screening and reporting may be needed to prevent iatrogenic transmission of prion disease.
Authors/Disclosures
Christian C. Prusinski, DO (Christopher J. Prusinski, DO, PA)
PRESENTER 		Dr. Prusinski has nothing to disclose.
Amanda L. Porter, MD (Mayo Clinic) Dr. Porter has nothing to disclose.
Evelyn Lazar, MD (JFK Medical Center) Dr. Lazar has nothing to disclose.
No disclosure on file
Bob Bucelli, MD, PhD (Washington University) Dr. Bucelli has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen . Dr. Bucelli has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Biogen. Dr. Bucelli has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Regeneron. Dr. Bucelli has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Hamilton Weber. Dr. Bucelli has received personal compensation in the range of $5,000-$9,999 for serving as an Expert Witness for O'Bryan Brown and Toner. Dr. Bucelli has stock in Neuroquestions.com. An immediate family member of Dr. Bucelli has stock in Neuroquestions.com. The institution of Dr. Bucelli has received research support from Biogen. The institution of Dr. Bucelli has received research support from Ionis.
Gregory S. Day, MD, MSc, FÂ鶹´«Ã½Ó³»­ (Mayo Clinic) Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Arialys Therapeutics. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for DynaMed (EBSCO Health). Dr. Day has or had stock in ANI Pharmaceuticals. The institution of Dr. Day has received research support from National Institutes of Health / NIA. The institution of Dr. Day has received research support from National Institutes of Health / NINDS. The institution of Dr. Day has received research support from Amgen Pharmaceuticals. The institution of Dr. Day has received research support from AVID Radiopharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Presenter at Annual Meeting (CME) with Â鶹´«Ã½Ó³»­. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development (CME) with PeerView, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Continuing Â鶹´«Ã½Ó³»­, Inc. Dr. Day has received personal compensation in the range of $5,000-$9,999 for serving as a Content Development (CME) with Ionis Pharmaceuticals. Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Â鶹´«Ã½Ó³»­al Case Development + Presentation (video) with PeerDirect (P\S\L Group). Dr. Day has received personal compensation in the range of $500-$4,999 for serving as a Content Development / Presentation (non-CME) with MJH Life Sciences (NeurologyLive). Dr. Day has a non-compensated relationship as a Clinical Director with Anti-NMDA Receptor Encephalitis Foundation that is relevant to Â鶹´«Ã½Ó³»­ interests or activities.