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Biomarkers to predict symptom onset in Huntington disease (HD) could provide an important tool for testing new therapies to forestall progression. Such a biomarker must fulfill 2 key characteristics, among others: it must predict timing of symptom onset better than extant means and changes in the biomarker must reflect pathophysiologic changes. Ferraro et al.1 used functional MRI to quantify blood oxygen level–dependent (BOLD) responses during an inhibition phase of an eye movement task in presymptomatic HD. The response magnitude in right frontal oculomotor cortex correlated (r2 = 0.52) with the probability of developing symptoms in the next 5 years as determined by the Langbehn formula, based upon age and CAG triplet repeat length,2 and did so better than volumetric measures of striatal atrophy (e.g., right putamen, r2 = 0.29).
Footnotes
Study funding: NIH (TR000448, NS41509, NS057105, and NS075321), the American Academy of Neurology, American Parkinson Disease Association (APDA) Center for Advanced PD Research at Washington University, Greater St. Louis Chapter of the APDA, McDonnell Center for Higher Brain Function, Barnes-Jewish Hospital Foundation (Elliot Stein Family Fund and Parkinson Disease Research Fund).
Disclosure: The author reports no disclosures relevant to the manuscript. Go to Neurology.org for full disclosures.
- © 2014 American Academy of Neurology
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