Abstract Details

Title Tumefactive Neurosarcoidosis

Topic Autoimmune Neurology

Presentation(s) P9 - Poster Session 9 (5:30 PM-6:30 PM)

Poster/Presentation
Number 6-007

Objective

To define and characterize tumefactive neurosarcoidosis, a unique phenotype of neurosarcoidosis distinct from the more commonly seen smaller, perivascular, subpial parenchymal lesions.

Background Enhancing brain parenchymal disease is an uncommon manifestation of neurosarcoidosis (10-27%). Intraparenchymal tumefactive lesions are even rarer (6-8%). As no studies dedicated to tumefactive lesions have been published, their clinical characteristics and impact on management and outcomes are unknown.

Design/Methods Patients were included if: 1) lesions were within brain parenchyma, 2) the lesion’s largest dimension was greater than 1 cm, 3) perilesional edema and/or mass effect were present, and 4) sarcoidosis was confirmed by neural or extraneural biopsies.

Results Nine patients were included with a median age of 37 years. Pathological confirmation came from brain parenchymal biopsies in 5 (55.6%) and extraneural tissues in the remainder. Isolated neurosarcoidosis was seen in 1 patient (11.1%). Only 1 patient (11.1%) was known to have systemic sarcoidosis previously at the time the parenchymal mass was evaluated. Median modified Rankin scale (mRS) score at nadir was 2 (range 1-4). Common manifestations included headache (77.8%), cognitive dysfunction (55.6%), and seizures (44.4%). MRI characteristics included spherical morphology (77.8%), perilesional edema (100.0%), mass effect (55.6%), well-demarcated lesions (66.7%), and contrast enhancement (100.0%; 55.6% inhomogeneous). The frontal lobe (44.4%) was most commonly affected, followed by the subinsular region (22.2%), putamen (22.2%), and internal capsule (22.2%). Leptomeningitis was a frequent neuroinflammatory accompaniment (77.8%). All patients received steroids and required steroid-sparing treatments. 55.6% of patients required second- and third-line treatments. Infliximab was needed in 44.4%. After a median follow-up period of 86 months, the median last mRS was 1.0, with significant residual deficits in 44.4% of patients.

Conclusions Tumefactive neurosarcoidosis most commonly affects the supratentorial brain in patients without a known history of sarcoidosis, is typically associated with leptomeningitis, and often is refractory to initial treatments.

Authors/Disclosures
Gabriela A. Bou, MD (Emory University School of Medicine) PRESENTER	Dr. Bou has nothing to disclose.
Sally El Sammak, MD (Emory University)	Dr. El Sammak has nothing to disclose.
	No disclosure on file
Julien Cavanagh, MD (Emory University)	Dr. Cavanagh has nothing to disclose.
Spencer Hutto, MD (Emory University: Neurology Residency Program)	Dr. Hutto has nothing to disclose.

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