Abstract Details

Title Pathological Findings in Autoimmune Encephalitis Autopsy Specimens from Cases of Suspected Prion Disease

Topic Autoimmune Neurology

Presentation(s) P5 - Poster Session 5 (5:30 PM-6:30 PM)

Poster/Presentation
Number 14-015

Objective Our objective was to describe pathological findings of autoimmune encephalitis (AE) specimens submitted to the U.S. National Prion Disease Pathology Surveillance Center.

Background

The underlying pathology of AE is not well characterized due to the limited opportunities to study tissue specimens. Autopsy specimens available at prion surveillance centers from patients with suspected Creutzfeldt-Jakob disease offer a unique opportunity to study the pathology of AE.

Design/Methods Pathology reports were obtained from the National Prion Center. Specimens negative for prion disease were screened for inflammatory pathology and those suggestive of AE were analyzed. Cases identified on autopsy were compared to institutional cases with fatal seronegative AE and available brain biopsy.

Results Between 1998 and 2022, 7934 specimens were evaluated of which 2998 (38%) were negative for prion protein. Querying the database for alternative diagnoses of encephalitis/encephalopathy yielded 43 cases that were screened by an experienced neuropathologist yielding 14 (0.5%) cases consistent with AE. Most specimens showed diffuse inflammation involving the limbic system (86%), basal ganglia (86%), cortex (71%), diencephalon (71%), and in some cases the brainstem (43%) and cerebellum (43%). Lymphocytic inflammatory infiltrate was predominantly perivascular with parenchymal extension in 64%. Microglial activation/nodules were seen in 64% of cases. Neuronal loss was present only in 50%. Pathological findings were identical to biopsy specimens from our institutional cohort.

Conclusions Seronegative AE may have consistent pathology with diffuse or multifocal perivascular inflammation and microglial activation. Half the patients do not have neuronal loss suggesting a potential for neurological recovery. The pathological distribution of inflammation seemed to exceed the typical radiological patterns of AE. These findings are preliminary and require further confirmation.

Authors/Disclosures
Hesham A. Abboud, MD (University Hospitals Cleveland Medical Center) PRESENTER	Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Biogen. The institution of Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech . Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alexion. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Alpine Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Cycle Pharma. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Axonics. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for TG Therapeutics. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Biogen. Dr. Abboud has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for Genentech. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for BMS. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Horizon. Dr. Abboud has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TG Therapeutics. The institution of Dr. Abboud has received research support from Genentech . The institution of Dr. Abboud has received research support from Novartis. The institution of Dr. Abboud has received research support from BMS. The institution of Dr. Abboud has received research support from Sanofi-Genzyme. The institution of Dr. Abboud has received research support from The Guthy-Jackson Charitable Foundation. The institution of Dr. Abboud has received research support from UCB. Dr. Abboud has received publishing royalties from a publication relating to health care.
Christina Kerner	No disclosure on file
Keisi Kotobelli (Case Western Reserve University)	No disclosure on file
Brian Appleby, MD (University Hospitals Case Medical Center)	Dr. Appleby has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Acadia. Dr. Appleby has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Ionis. Dr. Appleby has received personal compensation in the range of $0-$499 for serving as a Consultant for Sangamo. The institution of Dr. Appleby has received research support from CJD Foundation. The institution of Dr. Appleby has received research support from Ionis. The institution of Dr. Appleby has received research support from Alector. The institution of Dr. Appleby has received research support from CDC. The institution of Dr. Appleby has received research support from NIH. Dr. Appleby has received publishing royalties from a publication relating to health care. Dr. Appleby has received publishing royalties from a publication relating to health care.
Mark Cohen	Mark Cohen has nothing to disclose.

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