Medicine and Health Sciences Commons^™

Progression Of Friedreich Ataxia: Quantitative Characterization Over 5 Years, Maya Patel, Charles J. Isaacs, Lauren Seyer, Karlla Brigatti, Sarah Gelbard, Cassandra Strawser, Debbie Foerster, Julianna Shinnick, Kimberly Schadt, Eppie M. Yiu, Martin B. Delatycki, Susan Perlman, George R. Wilmot, Theresa Zesiewicz, Katherine Mathews, Christopher M. Gomez, Grace Yoon, Sub H. Subramony, Alicia Brocht, Jennifer Farmer, David R. Lynch

Neurology Faculty Publications

Objective Friedreich ataxia (FRDA) is a progressive neurodegenerative disorder of adults and children. This study analyzed neurological outcomes and changes to identify predictors of progression and generate power calculations for clinical trials.

Methods Eight hundred and twelve subjects in a natural history study were evaluated annually across 12 sites using the Friedreich Ataxia Rating Scale (FARS), 9-Hole Peg Test, Timed 25-Foot Walk, visual acuity tests, self-reported surveys and disability scales. Cross-sectional outcomes were assessed from recent visits, and longitudinal changes were gaged over 5 years from baseline.

Results Cross-sectional outcomes correlated with measures of disease severity. Age, genetic severity (guanine-adenine-adenine …

Application Of Pre-Participation Cardiovascular Screening Guidelines To Novice Older Runners And Endurance Athletes, Justin R. Abbatemarco, Courtney Bennett, Adrian J. Bell, Laura Dunne, Martin E. Matsumura

Neurology Faculty Publications

Objectives: Despite robust growth in participation in marathons and endurance sports among older individuals, guidance regarding pre-participation cardiovascular evaluation of these athletes is lacking. The objective of this study was to assess the utility of currently available pre-participation cardiovascular evaluation guidelines as applied to a cohort of older novice endurance athletes.

Methods: We applied data from 1457 novice runners and endurance athletes aged 35 years and older to two pre-participation screening tools, the American Heart Association/American College of Sports Medicine Pre-Participation Questionnaire and the 2001 Working Group recommendations for pre-participation screening of masters athletes (2001 Masters).

Results: Application of the …

Unusual Late Onset Of Parenchymal Neuro-Behçet Disease, Wai Wai Miller, Demetrios Konstas, Chetan Gandhy, Derrick Robertson

Neurology Faculty Publications

Neuro-Behçet disease (NBD) is a multisystem inflammatory disorder characterized by oral lesions, genital lesions, uveitis, and neurological deficits. If left untreated, it may lead to worsening neurological function and can be fatal. Here we present a case of a 52-year-old woman who was diagnosed with Behçet disease (BD) as a teenager and had a relatively mild disease course. Decades later after her initial DB diagnosis, she presented to our hospital with a chief complaint of headache. She did not have focal neurological deficits or any active mucosal lesions. Upon further investigation, the patient was found to have multiple inflammatory changes …