Abstract Details

Title Association Between Radiographic Features of Hypertrophic Pachymeningitis and its Underlying Diagnoses

Topic Autoimmune Neurology

Presentation(s) C11 - Central Nervous System Space-occupying Lesions (11:11 AM-11:18 AM)
P1 - Poster Session 1 (12:00 PM-1:00 PM)

Poster/Presentation
Number 016

Background

HP refers to thickening of the dura mater of the brain and or spinal cord that can be idiopathic or secondary to various conditions, including sarcoidosis, vasculitis, rheumatoid arthritis (RA), Behcet’s disease, and IgG4-related disease (IgG4-RD).

Objective

To investigate the clinical, laboratory, radiographic, and pathological characteristics of patients with hypertrophic pachymeningitis (HP) and identify predictors for underlying diagnoses.

Design/Methods

At a single institution we used the electronic medical record to identify patients with a diagnosis of HP in the past 10 years along with available dural biopsies from the same time. 35 cases met inclusion criteria, having both imaging with HP and available dural tissue for analysis. Group comparisons of numerical variables were performed using Student’s t-test for 2 groups and ANOVA for more than 2 groups. Independence between categorical variables was performed using Chi-squared test. A p-value of <0.05 was considered significant.

Results

Median age was 53.71 (SD 19.14); 54.3% female. 37.1% were “other race,” followed by white 31.4%, black or African-American 28.6%, and Asian 2.9%. 45.7% were of Hispanic or Latino ethnicity. Confirmed etiologies of HP included IgG4-RD 25.7% (n=9), idiopathic 22.9% (n=8), sarcoidosis 20% (n=7), infection 11.4% (n=4), RA 8.6% (n=3), vasculitis 5.7% (n=2), Behcet’s disease 2.9% (n=1), and intracranial hypotension 2.9% (n=1). Spinal dural involvement was mostly seen in IgG4-RD, sarcoidosis, and idiopathic (p=0.88). Nodular pattern of dural involvement was significantly associated with idiopathic, RA, and sarcoidosis, whereas smooth pattern was associated with Behcet’s disease, infection, intracranial hypotension, and vasculitis (p=0.035). Diffuse involvement was more likely to be smooth, and focal (less than 50% of the entire brain circumference) more likely to be nodular (p=0.35).

Conclusions

To our knowledge, this is the largest single-center study of HP with biopsy results. Imaging features can be suggestive of underlying diagnoses, many of which are inflammatory.

Authors/Disclosures
Jiyeon Son, MD PRESENTER	Ms. Son has nothing to disclose.
Chloe Y. Li, MD	Dr. Li has nothing to disclose.
Ceren Tozlu	No disclosure on file
Sugeidy Ferreira Brito (Columbia University Medical Center)	No disclosure on file
Michael T. Miller, MD	No disclosure on file
Sarah F. Wesley, MD (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.

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