Abstract Details

Title Diagnosis and Management of Pachymeningitis

Topic Multiple Sclerosis

Presentation(s) P1 - Poster Session 1 (5:30 PM-6:30 PM)

Poster/Presentation
Number 15-056

Objective

To report the observations on a multi-center cohort of patients with dural inflammation (pachymeningitis), focusing on differential diagnosis and suggesting a plan of workup.

Background

Literature on pachymeningitis is limited and exists mostly in the form of single case reports and small case series. Pachymeningitis has been associated with a variety of causes including infectious, neoplastic and autoimmune. Magnetic resonance imaging (MRI) of the brain identifies the thickening and contrast enhancement of pachymeningitis but is not specific in identifying a cause. The diagnosis of pachymeningitis involves an extensive workup that typically includes a dural biopsy.

Design/Methods

Electronic medical records at academic medical centers were queried using the keyword ‘pachymeningitis’. Cases thought to be reflective of dural inflammation were included in the study. The authors personally reviewed the MRI images. Laboratory features including serum and CSF serologies, investigations for likely causes of pachymeningitis (such as chest imaging, PET scan) were documented in detail.

Results

We identified 17 cases of pachymeningitis (7 male, 10 female). Age at presentation was 48+/-1.8 years. The most common clinical presentation was headache (10 patients), followed by unilateral visual loss (5 patients) and diplopia (5 patients). Brain MRI in all cases showed focal or diffuse dural thickening and contrast enhancement. Biopsy was performed in 13 cases. Granulomatous inflammation (sarcoidosis) was the commonest finding, seen in 5 cases. IgG4-related disease (2), B-cell lymphoma (1), inflammatory myofibroblastic tumor (1) were other findings. The remaining 4 cases showed nonspecific inflammation. Treatment and outcomes varied according to the cause.

Conclusions

Pachymeningitis can be due to varied causes. Occasionally, as in patients with established systemic sarcoidosis, the diagnosis may be apparent, but in the majority of patients, a biopsy is required to establish the diagnosis, and is important as the treatment depends on the specific etiology of pachymeningitis.

Authors/Disclosures
Derek Wood, MD (UW Medicine Valley Medical Center) PRESENTER	No disclosure on file
Rae E. Bacharach, DO (Penn State University, Milton S Hershey Medical Center)	Dr. Bacharach has received personal compensation in the range of $500-$4,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for ��ɫ��.
Chu-Yueh Guo, MD (UCSF Medical Center)	Dr. Guo 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.
Lana Zhovtis Ryerson, MD, FAAN (Jersey Shore University Medical Center)	Dr. Zhovtis Ryerson has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Sanofi. Dr. Zhovtis Ryerson has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Sanofi.
Michael J. Bradshaw, MD, FAAN	Dr. Bradshaw has nothing to disclose.
	No disclosure on file
Yunxia Wang, MD, FAAN (KUMC)	Dr. Wang has nothing to disclose.
Jeffrey M. Gelfand, MD, MS, FAAN (University of California, San Francisco)	Dr. Gelfand has received personal compensation in the range of $10,000-$49,999 for serving as a Consultant for Arialys. Dr. Gelfand has received personal compensation in the range of $500-$4,999 for serving as a Consultant for Ventyx Bio. An immediate family member of Dr. Gelfand has received personal compensation in the range of $50,000-$99,999 for serving as an Editor, Associate Editor, or Editorial Advisory Board Member for Headache: The Journal of Head and Face Pain. The institution of Dr. Gelfand has received research support from Genentech/Roche. The institution of Dr. Gelfand has received research support from Vigil Neurosciences. An immediate family member of Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has received publishing royalties from a publication relating to health care. Dr. Gelfand has a non-compensated relationship as a Trial Steering Committee Chairperson and member with Roche / Genentech that is relevant to AAN interests or activities.
Eoin P. Flanagan, MBBCh, FAAN (Mayo Clinic)	The institution of Dr. Flanagan has received personal compensation in the range of $10,000-$49,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Roche. Dr. Flanagan has received personal compensation in the range of $500-$4,999 for serving on a Speakers Bureau for Pharmacy times. The institution of Dr. Flanagan has received personal compensation in the range of $5,000-$9,999 for serving on a Speakers Bureau for UCB. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Roche. The institution of Dr. Flanagan has received research support from UCB. The institution of Dr. Flanagan has received research support from Merck. The institution of Dr. Flanagan has received research support from Roche. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has received publishing royalties from a publication relating to health care. Dr. Flanagan has a non-compensated relationship as a Member of medical Advisory Board with The MOG Project that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Journal of The Neurologic Sciences that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial board member with Neuroimmunology Reports that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology, Neuroimmunology Neuroinflammation (N2) Journal that is relevant to AAN interests or activities. Dr. Flanagan has a non-compensated relationship as a Editorial Board Member with Neurology that is relevant to AAN interests or activities.
Allen J. Aksamit, Jr., MD, FAAN (Mayo Clinic)	Dr. Aksamit has nothing to disclose.
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.
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.
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.

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