Cluster of an Unusual Amnestic Syndrome - Massachusetts, 2012-2016

Abstract

In November 2015, a neurologist in the Boston, Massachusetts, area reported four cases of an uncommon amnestic syndrome involving acute and complete ischemia of both hippocampi, as identified by magnetic resonance imaging (MRI), to the Massachusetts Department of Public Health (MDPH) (1). A subsequent e-mail alert, generated by the Massachusetts Board of Registration in Medicine and sent to relevant medical specialists (including neurologists, neuroradiologists, and emergency physicians), resulted in the identification of 10 additional cases that had occurred during 2012-2016. All 14 patients (mean and median age = 35 years) had been evaluated at hospitals in eastern Massachusetts. Thirteen of the 14 patients underwent routine clinical toxicology screening at the time of initial evaluation; eight tested positive for opioids, two for cocaine, and two for benzodiazepines. Apart from sporadic cases (2-6), this combination of clinical and imaging findings has been reported rarely. The apparent temporospatial clustering, relatively young age at onset (19-52 years), and associated substance use among these patients should stimulate further case identification to determine whether these observations represent an emerging syndrome related to substance use or other causes (e.g., a toxic exposure).

FIGURE

Diffusion-weighted imaging (DWI)* findings at initial brain MRI in a patient with unusual amnestic syndrome — Massachusetts, 2012 Abbreviations: MIP = maximum intensity projection; mm = millimeter; MRI = magnetic resonance imaging. * Axial DWI demonstrates bright signal consistent with complete bilateral hippocampal ischemia. The complete extent of hippocampal ischemia is best evident on thick 20 mm MIP images constructed from the axial DWI data.