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Tumefactive brain parenchymal neurosarcoidosis
Gabriela A Bou1, Sally El Sammak1, Ling-Chen Chien2
1Department of Neurology, Emory University School of Medicine, 12 Executive Park Drive NE, Atlanta, GA, 30329, USA.
Journal of Neurology
|May 23, 2023
Summary
Tumefactive neurosarcoidosis brain lesions are rare and often require advanced treatments. Despite initial therapy, these lesions frequently relapse, leading to significant long-term neurological deficits.
Area of Science:
- Neurology
- Neuroimmunology
- Radiology
Background:
- Neurosarcoidosis can present as tumefactive brain parenchymal lesions, which are uncommon.
- Limited data exists on the clinical characteristics, management, and outcomes of these specific lesions.
Purpose of the Study:
- To characterize the clinical features of tumefactive brain lesions in neurosarcoidosis.
- To investigate the management strategies and patient outcomes associated with these lesions.
Main Methods:
- Retrospective review of patients with pathologically confirmed sarcoidosis and specific brain lesion criteria.
- Inclusion criteria: intraparenchymal lesions >1 cm with edema and/or mass effect.
Main Results:
- Nine patients (4.2%) met criteria; median age 37. Frontal lobe most affected (31.3%). Lesions showed edema (100%), contrast enhancement (100%), and often leptomeningitis (77.8%).
- Most patients required corticosteroid-sparing agents, with 55.6% needing third-line treatment (e.g., infliximab in 44.4%). All patients experienced relapses (median 3).
- Median modified Rankin Scale (mRS) was 1.0 at follow-up (86 months), but 55.6% had significant residual deficits.
Conclusions:
- Tumefactive brain lesions in neurosarcoidosis are rare, typically supratentorial, and frequently involve leptomeningitis.
- These lesions are refractory to initial treatments and carry a high risk of relapse.
- Despite a favorable median mRS, significant long-term sequelae are common.
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