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Abstract Details

Cortical Hypometabolism Associated with Neurocognitive disorder and Thyroid Antibodies (CHANT Syndrome)
Autoimmune Neurology
P3 - Poster Session 3 (11:30 AM-12:30 PM)
1-090

To characterize clinical and imaging findings using 18F-FDG PET scans in patients with subacute neurocognitive decline, which demonstrate similar patterns of multifocal cortical hypometabolism.

Reversible neurocognitive disorders remain poorly understood, underdiagnosed, and undertreated. These disorders are often characterized by variable neuropsychiatric presentations, grossly normal magnetic resonance imaging, nonspecific findings on cerebrospinal fluid analysis, and electroencephalography that may reveal nonspecific generalized slowing. As a result, they remain controversial with unclear pathogenesis and limited objective biomarkers. Herein, we report on patients with subacute neurocognitive decline and a novel constellation of findings characterized by a regional pattern of multifocal cortical hypometabolism on brain FDG-PET in addition to consistent blood biomarkers.

This retrospective case series includes seven patients presenting with subacute neurocognitive decline and elevated thyroid antibodies. Results of brain 18F-FDG PET were compared to identify shared features of metabolic patterns.

Mean age 47 ±6.52, 6 female, 1 male. All presented with neurocognitive syndrome with elevation of thyroglobulin or thyroid peroxidase antibodies or both. Three were initially diagnosed with functional neurological disorders. All showed relative hypometabolism on FDG-PET: temporal (100%), parietal (57%), prefrontal (43%), cingulate (14%), precuneus (14%), and occipital (14%) cortices. Often, radiology interpreted the findings as consistent with Alzheimer’s disease pattern. Following immunomodulation (steroids, intravenous immunoglobulins, or rituximab), all patients clinically improved, and 2/2 patients who had repeated FDG-PET showed improved metabolism.

We describe a novel subset of patients with consistent clinical and radiological patterns with response to immunomodulation. We propose calling this subset CHANT Syndrome: Cortical Hypometabolism Associated with Neurocognitive disorder and Thyroid antibodies. Our findings highlight the importance of utilizing brain FDG-PET as a biomarker to detect multifocal cortical hypometabolism in patients presenting with subacute neurocognitive syndrome. Recognition of this pattern may facilitate earlier diagnosis and treatment. Future studies are needed to understand the pathophysiology of this neuroinflammatory disorder.

Authors/Disclosures
Athena Dao, MD
PRESENTER
Dr. Dao has nothing to disclose.
Sam Hooshmand, DO (Medical College of Wisconsin) Dr. Hooshmand has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genetech USA. Dr. Hooshmand has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG Therapeutics . Dr. Hooshmand has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen. Dr. Hooshmand has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Genetech USA. Dr. Hooshmand has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for EMD Serono. Dr. Hooshmand has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for TG Therapeutics . Dr. Hooshmand has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Amgen . The institution of Dr. Hooshmand has received research support from Novartis .
Elise Johnson, MD Dr. Johnson 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. Johnson has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Sanofi.
Kaylan Fenton Ms. Fenton has nothing to disclose.
Kayla Grundman, CRC Miss Grundman has nothing to disclose.
Samantha O'Dell, BA, CRC Mrs. O'Dell has nothing to disclose.
Saad Ali, MD Dr. Ali has nothing to disclose.
Christopher Kleefisch, MD Dr. Kleefisch has nothing to disclose.
Ahmed Z. Obeidat, MD, PhD (Medical College of Wisconsin) Dr. Obeidat has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Alexion. Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Biogen . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Bristol Myers Squibb. Dr. Obeidat has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for Genentech. Dr. Obeidat has received personal compensation in the range of $50,000-$99,999 for serving as a Consultant for EMD Serono. Dr. Obeidat has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Sanofi/Genzyme . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Horizon pharmaceuticals . Dr. Obeidat has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Sandoz. Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for TG Therapeutics. Dr. Obeidat has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cycle pharama . Dr. Obeidat has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for Alexion. Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Biogen. Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Bristol Myers Squibb. Dr. Obeidat has received personal compensation in the range of $100,000-$499,999 for serving on a Speakers Bureau for EMD Serono. Dr. Obeidat has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Banner life sciences . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Sanofi/genzyme. Dr. Obeidat has received personal compensation in the range of $50,000-$99,999 for serving on a Speakers Bureau for Horizon therapeutics . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for Amgen. Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for TG Therapeutics . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving on a Speakers Bureau for AstraZeneca. The institution of Dr. Obeidat has received research support from NIH. The institution of Dr. Obeidat has received research support from NMSS and PCORI. Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Expert opinion and key opinion leader with MJH life sciences . Dr. Obeidat has received personal compensation in the range of $10,000-$49,999 for serving as a Faculty Speaker with CMSC. Dr. Obeidat has a non-compensated relationship as a Board member with CMSC that is relevant to AAN interests or activities. Dr. Obeidat has a non-compensated relationship as a Editorial Board Member with IJMSC that is relevant to AAN interests or activities. Dr. Obeidat has a non-compensated relationship as a Reviewer Editor with Frontiers Neurology that is relevant to AAN interests or activities. Dr. Obeidat has a non-compensated relationship as a Board of Trustee Member with National MS Society that is relevant to AAN interests or activities.