352. Towards Earlier Diagnosis of Transmissible Spongiform Encephalopathies (TSEs): A Case Series, Including One Associated With Squirrel Brain Consumption

Abstract

BackgroundTSEs present diagnostic and infection control (IC) challenges. Creutzfeldt-Jakob Disease (CJD) is the most common human TSE, occurring in 1–2/million/year in the United States, but other zoonotic factors or transmissions remain incompletely understood. Prompted by the occurrence of four suspected cases from November 2017 to April 2018, we present a case series of suspected CJD to illustrate its variable presentation and the need for more rapid identification for implementation of disease-specific disinfection, sterilization, and quarantine measures.MethodsWe defined a case as any patient with a rapidly progressive dementing or neurologic illness and laboratory tests for CJD. IC and laboratory databases, and electronic medical records were reviewed to identify possible cases from 2013 to 2018.ResultsFive patients met case definition. The average time to suspecting and confirming a diagnosis was 5.2 and 14.2 days, respectively.Case12345Age/sex61 M65 F51 F61 F80 MCognitive symptomsPsychosis, schizophrenia, cognitive declineDysphasia, depression, psychosisVertigo, progressive encephalopathyMemory loss, aphasiaAphasia, dysarthria, dysphagiaMotor symptomsImpaired gaitImpaired gaitBilateral ataxiaImpaired gait incontinence, abnormal muscle tone with paratoniaUnilateral weakness, jerking movementsEEGTriphasic patternAbundant generalized dischargesOccasional bi-frontal sharp wave dischargesGeneralized encephalopathyNSCMRIIncreased T2 signal in the pulvinar of the thalamus and cortex (especially frontal lobes)NSCNSCNSC/small vessel infarctsNSC/small vessel infarctsRT-QuIC+++–P14-3-3+++–PT-tau8,750>4,000>4,000390PEpidemiologyIntake of squirrel brainsConcurrent apheresis and GYN surgeryHotel HousekeepingIndustrial ChemistResidence in UK, intake of dog foodJanitorCJDVSSNoPDays to suspecting diagnosis113246Days to confirmation16121812>11Months of Illness53>2PPOutcomeDeadDeadAliveAliveAliveNSC, nonspecific changes; P, pending; S, poradic; V, variant; RT-QulC, Realtime Quaking Induced Conversion.ConclusionProtean in presentation, the diagnosis of CJD can be delayed. Variant CJD and emerging zoonotic TSEs should be considered in differential diagnoses and IC measures. Improved empiric classification algorithms and tests with faster turnaround times are needed.Disclosures All authors: No reported disclosures.