352. Towards Earlier Diagnosis of Transmissible Spongiform Encephalopathies (TSEs): A Case Series, Including One Associated with Squirrel Brain Consumption
Session: Poster Abstract Session: CNS Infections
Thursday, October 4, 2018
Room: S Poster Hall
Posters
  • CJD Poster 9-24.pdf (130.7 kB)
  • Background: TSEs 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 US, but other zoonotic factors or transmissions remain incompletely understood. Prompted by the occurrence of 4 suspected cases from 11/2017-04/2018, 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.

    Methods: We defined a case as any patient with a rapidly progressive dementing or neurologic illness and lab tests for CJD. IC and lab databases, and electronic medical records were reviewed to identify possible cases from 2013 to 2018.

    Results: 5 patients met case definition. The average time to suspecting and confirming a diagnosis was 5.2 and 14.2 days respectively.

    Case

    1

    2

    3

    4

    5

    Age/Sex

    61 M

    65 F

    51 F

    61 F

    80 M

    Cognitive symptoms

    Psychosis, schizophrenia, cognitive decline

    Dysphasia, depression, psychosis

    Vertigo, progressive encephalopathy

    Memory loss, aphasia

    Aphasia, dysarthria, dysphagia

    Motor symptoms

    Impaired gait

    Impaired gait

    Bilateral ataxia

    Impaired gait incontinence, abnormal muscle tone with paratonia

    Unilateral weakness, jerking movements

    EEG

    Triphasic pattern

    Abundant generalized discharges

    Occasional bi-frontal sharp wave discharges

    Generalized encephalopathy

    NSC

    MRI

    Increased T2 signal in the pulvinar of the thalamus and cortex (especially frontal lobes)

    NSC

    NSC

    NSC / small vessel infarcts

    NSC / small vessel infarcts

    RT-QuIC

    +

    +

    +

    -

    P

    14-3-3

    +

    +

    +

    -

    P

    T-tau

    8750

    >4000

    >4000

    390

    P

    Epidemiology

    Intake of squirrel brains

    Concurrent apheresis and GYN surgery

    Hotel Housekeeping

    Industrial Chemist

    Residence in UK, intake of dog food

    Janitor

    CJD

    V

    S

    S

    No

    P

    Days to suspecting diagnosis

    1

    13

    2

    4

    6

    Days to confirmation

    16

    12

    18

    12

    >11

    Months of Illness

    5

    3

    >2

    P

    P

    Outcome

    Dead

    Dead

    Alive

    Alive

    Alive

    NSC=Nonspecific changes; P=pending; S=Sporadic; V=Variant; RT-QulC=Realtime Quaking Induced Conversion

    Conclusion: Protean 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.

    Tara Chen, MD, John Hanna, MD, Laura Eckert-Davis, BSN, Julie Giles, BSN, Kelly Vore, PhD, Maryrose Laguio-Vila, MD and Emil Lesho, DO, FACP, FIDSA, FSHEA, Rochester Regional Health, Rochester, NY

    Disclosures:

    T. Chen, None

    J. Hanna, None

    L. Eckert-Davis, None

    J. Giles, None

    K. Vore, None

    M. Laguio-Vila, None

    E. Lesho, None

    See more of: CNS Infections
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