Towards Earlier Diagnosis of Transmissible Spongiform Encephalopathies (TSEs): A Case Series, Including One Associated with Squirrel Brain Consumption
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
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.
Disclosures:
K. Vore, None
M. Laguio-Vila, None
E. Lesho, None
T. Chen, None
J. Hanna, None
L. Eckert-Davis, None
J. Giles, None
so, media picks this up and runs with it all the while ignoring the other likely routes and sources of tse prion infection staring them right in the face.
Hunter died of mad cow-like disease after eating squirrel's brain!
NO, this has not been confirmed, nor has any other suspect squirrel brain eaters
that died from cjd.
cdc has never confirmed this.
there are so many other routes and sources staring us in the face, and unless
these squirrels were eating ruminant mammalian feed, then i don't believe this
story.
no transmission studies were done, this is just a case of circumstance, imo..
IF this is all that they can come up with in a CJD Questionnaire, God help us...
UPDATE;
Rochester Regional researchers: no clear link between squirrel brain consumption and death
By BRETT DAHLBERG • OCT 19, 2018
Rochester Regional Health cannot directly connect the reported death of a man with Creutzfeldt-Jakob disease to his consumption of squirrel brain, researchers said Friday.
National and international media reports had indicated that the man’s squirrel consumption was linked to his development of the illness, which is similar in symptoms to mad cow disease.
But Emil Lesho, a hospital epidemiologist at Rochester Regional Health, said that’s not the conclusion he and a team of researchers had drawn.
The team presented research at a conference in San Francisco, after which LiveScience ran the initial story. That research did not show causation between squirrel brain consumption and the man’s disease, said Lesho. Instead, it showed an association.
“This is just typical epidemiology,” Lesho said. “You look for anything that could indicate where a disease came from.”
The research was not meant for public consumption, Lesho said. “Our original intent with this report was to present it in front of a jury of our peers at a scientific meeting, to become vetted.”
It started, Lesho said, when he and other researchers noticed what looked like a series of cases where patients had symptoms resembling mad cow disease. Statistically, Lesho said, they should see that disease about once a year. But now they were seeing “what looked like two or three patients over a four-month period.”
That would be concerning, because that family of diseases, caused by malformed proteins called prions, is very difficult to disinfect. Surgical equipment that comes in contact with patients who have prion diseases sometimes can’t be cleaned and need to be disposed of entirely.
“We want to be very careful with this at our hospital,” Lesho said.
In the end, further research showed that some suspected cases were not confirmed, and the frequency of the disease was actually in line with statistical expectations.
Still, though, because prion diseases are transmissible through the consumption of an infected animal’s nerve cells, Lesho says the bottom line is, “be careful if you eat brain tissue.”
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Terry S. Singeltary Sr.
posted by Terry S. Singeltary Sr. at
4:24 PM
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