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Abstract Details

Multi-institutional Retrospective Analysis of Hypertrophic Pachymeningitis
Autoimmune Neurology
P8 - Poster Session 8 (11:45 AM-12:45 PM)
2-003
To characterize clinical, paraclinical, pathological features of hypertrophic pachymeningitis (HP).    
HP is a rare clinico-radiologic inflammatory syndrome associated with thickening/enhancement of dura mater. HP can be due to various inflammatory, infectious, and neoplastic processes. Dural biopsy remains the gold standard for diagnosis but is frequently non-diagnostic.

Patients with HP were identified through comprehensive electrical medical records query. We required that all patients have dural thickening and/or enhancement on MRI. Investigators--neurologists at 7 academic medical centers – abstracted demographic, clinical, radiographic, and histopathologic data. Descriptive statistics are presented.

 
Of the 113 patients with HP, 55% were female, most were White (n=78, 69.0%), followed by Black/African American (n=14, 12.4%), and Others (n=8, 7.1%). The most common presenting symptoms were headaches (n=69, 59.3%), visual loss (n=20, 17.7%), other (n=21, 18.6%), and cognitive impairment (n=18, 15.9%). MRI pattern showed diffuse dural enhancement in 69 (61.1%) and focal in 41 (36.3%); smooth enhancement was seen in 82 (72.6%) and nodular in 27 (23.9%); 28 (24.8%) demonstrated leptomeningeal enhancement, and 23 (20.4%) were markedly thickened (>5mm). The most frequent additional sites of involvement were the cranial nerves (n=13, 11.5%), spinal cord (n=13, 11.5%), and orbits (12, 10.6%). 60 (53.1%) underwent dural biopsy. Idiopathic pachymeningitis was the most common working diagnosis (n=68, 60.2%), followed by sarcoidosis (n=16,14.2%), ANCA-associated vasculitis (n=8, 7.1%), rheumatoid arthritis (n=4, 3.5%), other (n=4, 3.5%), IgG4-related disease (n=3, 2.6%), infection (n=3, 2.6%), malignancy (n=3, 2.6%), Sjogren’s syndrome (n=2, 1.7%) and histiocytosis (n=2, 1.7%). 88 patients (80%) received any treatment, with 77 (87.5%) demonstrating clinical and/or radiographic improvement. The most common treatments were IVMP (n=70, 69.3%), IVMP with prednisone taper (n=42, 41.2%), and rituximab (n=26, 25.5%).  
We present a large, multi-institutional dataset on HP with diverse etiologies. Further analysis of etiology-specific characteristics and outcomes will inform future diagnostic and treatment strategies.  
Authors/Disclosures
Jiyeon Son, MD
PRESENTER 		Ms. Son has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen .
Rohini D. Samudralwar, MD (The University of Pennsylvania) Dr. Samudralwar has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for EMD Serono. Dr. Samudralwar 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. Samudralwar 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. Samudralwar has received personal compensation in the range of $500-$4,999 for serving as a Speaker with Multiple Sclerosis Association of America.
Joseph R. Berger, MD, FAAN Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Bristol Myers Squibb/Celgene. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Cycle Pharma. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Dice Therapeutics. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Genentech/Roche. Dr. Berger has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Gilead. Dr. Berger has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Merck/. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Morphic. Dr. Berger has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Novartis. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sandoz. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Takeda. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving as a Consultant for TG Therapeutics. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for MAPI. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for ExcisionBio. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Population Bio. Dr. Berger has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for TG Therapeutics. Dr. Berger has received personal compensation in the range of $10,000-$49,999 for serving as an Expert Witness for Assorted .
Giovanna Manzano, MD Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Gilead Sciences. Dr. Manzano has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for InfuCare Rx.
Stacey Clardy, MD, PhD, FAAN (University of Utah) Dr. Clardy has received personal compensation for serving as an employee of Veterans Health Administration (VHA). Dr. Clardy has received personal compensation for serving as an employee of University of Utah Health. Dr. Clardy has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for AstraZeneca/Alexion. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Amgen/Horizon. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Arialys. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Kyverna. Dr. Clardy has received personal compensation in the range of $10,000-$49,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Neurology/AAN Publications. The institution of Dr. Clardy has received research support from NIH/NINDS. The institution of Dr. Clardy has received research support from SRNA. The institution of Dr. Clardy has received research support from Alexion/AstraZeneca. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a AAN Summer Meeting CoDirector Travel and Lodging with AAN. Dr. Clardy has received personal compensation in the range of $500-$4,999 for serving as a Grand Rounds Travel/Lodging/Honoraria with U of Iowa, Miami, Stanford, Barrow, Beaumont Health, CCF, Emory, Penn State, Mayo Clinic, Walter Reed.
Ilya Kister, MD, FAAN (NYU School of Medicine) Dr. Kister has received personal compensation in the range of $5,000-$9,999 for serving as a Consultant for Genentech-Roche. Dr. Kister has received personal compensation in the range of $500-$4,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Horizon. The institution of Dr. Kister has received research support from Genentech. The institution of Dr. Kister has received research support from Novartis. Dr. Kister has received publishing royalties from a publication relating to health care.
Spencer Hutto, MD (Emory University: Neurology Residency Program) Dr. Hutto has nothing to disclose.
Kiran Thakur, MD, FAAN (Columbia University College of Physicians and Surgeons) Dr. Thakur has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Delve Bio.
Sarah F. Wesley, MD, MPH (Columbia University College of Physicians and Surgeons) Dr. Wesley 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. Wesley has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Novartis. Dr. Wesley 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.
Andrew Morrison, MD (Hospital of the University of Pennsylvania) Dr. Morrison has nothing to disclose.
Sage T. Hewitt, MD (VUMC) Dr. Hewitt has nothing to disclose.
Wesley Peng, MD, PhD (Massachusetts General Hospital) Dr. Peng has nothing to disclose.
Hannah M. Kelly, MD Ms. Kelly has nothing to disclose.
Trevor Glenn, MD (Mass General Brigham) Dr. Glenn has nothing to disclose.
Alexandra R. Balshi Ms. Balshi has nothing to disclose.
Yoji Hoshina, MD (University of Utah Health) Dr. Hoshina has nothing to disclose.
Amanda Zhao, MD Dr. Zhao has nothing to disclose.
Gerome B. Vallejos, MD Mr. Vallejos has nothing to disclose.
Wen-Yu Lee, MS Mr. Lee has nothing to disclose.
Ruyu Shi, PhD Ms. Shi has nothing to disclose.
Siddharama Pawate, MD (Vanderbilt University Medical Center) The institution of Dr. Pawate has received research support from Genentech. The institution of Dr. Pawate has received research support from Biogen.