Michael Benatar, USA

“Charcot and Pick walk into a bar …”
Competing and Complementary Ideas in ALS and FTD

Presentation:

Historically, ALS and FTD have been considered distinct disorders, but we now recognize their phenotypic and biological overlap. The phenotypic spectrum of ALS-FTD, however, should (minimally) be expanded to include a variety of extrapyramidal disorders. Traditional notions of these disorders as solely clinical syndromes are increasingly being abandoned as evidence emerges for prodromal states – mild motor impairment, mild cognitive impairment, mild behavioral impairment, and mild extrapyramidal impairment – that precede phenoconversion to fully fledged clinically manifest syndromes. Moreover, biomarker evidence of underlying biology during the pre-symptomatic stage of disease has prompted a shift towards considering ALS and FTD as biological entities (rather than clinical syndromes), even though the temporal course of identified and emerging biomarkers is yet to be uncovered. These distinct, but related concepts, are embodied in the Miami Framework for ALS and Related Neurodegenerative Disorders. This presentation will explore the overlap between ALS, FTD and related disorders; describe what is known about prodromal stages of these diseases; discuss if and how prodromal “diagnoses” should be communicated to those at risk for ALS/FTD; evaluate extant biomarker data relevant to both ALS and FTD; address competing and complementary ideas; and identify challenges and opportunities for the ALS and FTD communities to partner and to learn from each other. Despite their differences, ALS and FTD are interconnected, and progress in one field is very likely to impact the other. Disease prevention efforts will require close collaboration across these often-siloed communities.

Bio:

Michael Benatar, MBChB, MS, DPhil, is a professor of neurology and the Walter Bradley Chair in ALS Research at the University of Miami. He obtained his medical degree at the University of Cape Town (South Africa), and his doctorate in neuroscience while a Rhodes Scholar (University of Oxford).  He runs an active clinical/translational research program focused on ALS biomarker and therapy development, with a particular focus on pre-symptomatic disease and the intersection between ALS, FTD and related neurodegenerative disorders.