Abstract Details

Title MRI Analysis in 54 Cases of Sarcoidosis-associated Myelitis Identifies Characteristic Imaging Features and Clues to Pathogenesis

Topic Multiple Sclerosis

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

Poster/Presentation
Number 15-055

Objective To describe the imaging patterns seen in sarcoidosis-associated myelitis (SAM).

Background SAM is a rare, disabling and diagnostically challenging manifestation of sarcoidosis, an inflammatory multisystem disorder of unknown cause. Imaging features of SAM have not been well-described.

Design/Methods We performed detailed MRI analysis of 66 cases of sarcoidosis-associated myelitis (SAM) diagnosed at the Johns Hopkins Transverse Myelitis Center between 2000 and 2018. Diagnoses were classified according to the 2018 Consensus Diagnostic Criteria for Neurosarcoidosis. We included 7 patients with definite neurosarcoidosis (with pathological confirmation of granulomatous disease in the nervous system) and 47 patients with probable neurosarcoidosis (with pathological confirmation of systemic granulomatous disease). We excluded 12 patients with possible neurosarcoidosis (with no pathological confirmation of granulomatous disease).

Results Of the 54 patients included, 53% were male, 51% were African-American and 26% had a known diagnosis of sarcoidosis prior to onset of SAM. The temporal profile of neurological symptom-onset was chronic (>21 days from onset to neurological nadir) in 80% of patients. Five distinct MRI patterns were identified; 1) longitudinally extensive cord lesions with posterior-predominant subpial enhancement and/or nodular leptomeningeal enhancement [23 patients, 43%], 2) focal tumefactive cord lesions [15 patients, 28%], 3) meningeal/nerve root based disease [10 patients, 19%], 4) anterior cord lesions with anterior enhancement [5 patients, 9%] and 5) isolated small monofocal cord lesions [4 patients, 7%]. The trident enhancement sign was present in 5 patients (9%). Overall, in 45% of cases lesion enhancement was prominent in areas where there were co-existing spinal structural changes.

Conclusions Distinct imaging patterns occur in SAM and recognition of these features may aid in differentiating this myelopathy from other subacute or chronic evolving myelopathies. Furthermore, our findings suggest that blood-spinal-cord barrier permeability may play an important role in the development of SAM, as avid enhancement was frequently located at areas of mechanical stress.

Authors/Disclosures
Olwen Murphy, MD (Johns Hopkins Hospital) PRESENTER	Dr. Murphy has nothing to disclose.
	No disclosure on file
Paula Barreras, MD (Cedars-Sinai Medical Center)	Dr. Barreras has received personal compensation in the range of $5,000-$9,999 for serving on a Scientific Advisory or Data Safety Monitoring board for Alexion. The institution of Dr. Barreras has received research support from Foundation for Sarcoidosis Research. The institution of Dr. Barreras has received research support from �鶹��ýӳ��.
Luisa A. Diaz-Arias, MD (Luisa Diaz Arias)	Dr. Diaz-Arias has nothing to disclose.
Maria A. Garcia-Dominguez, MD (UMass Memorial Medical Center)	Dr. Garcia-Dominguez has received personal compensation for serving as an employee of Genentech.
Ruth Andrea Salazar Camelo, MD (Johns Hopkins School of Medicine)	Dr. Salazar Camelo has nothing to disclose.
Maria I. Reyes-Mantilla, MD (Johns Hopkins University, Neurology)	Dr. Reyes-Mantilla has nothing to disclose.
Carlos A. Pardo-Villamizar, MD (Johns Hopkins U, Med Dept of Neurology)	The institution of Dr. Pardo-Villamizar has received research support from National Institutes of Health. The institution of Dr. Pardo-Villamizar has received research support from Bart McLean Fund for Neuroimmunology Research .

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