American Journal of Medicine, internal medicine, medicine, health, healthy lifestyles, cancer, heart disease, drugs

Leptomeningeal Infiltration, the Hallmark of Neurosarcoidosis

Brain computed tomography showing bifrontal hypodensity (A; white arrows, precontrast) with focal meningeal enhancement (B; white arrows, postcontrast). Brain magnetic resonance imaging confirmed marked bifrontal edema (C; white arrows, hyperintense on axial T2-weighted image) and leptomeningeal thickening (D; white arrows, axial T1-weighted image with contrast). Salivary gland histopathology showing typical noncaseating granuloma with giant cells (E; black arrows, hematoxylin staining, ×10 magnification).

Brain computed tomography showing bifrontal hypodensity (A; white arrows, precontrast) with focal meningeal enhancement (B; white arrows, postcontrast). Brain magnetic resonance imaging confirmed marked bifrontal edema (C; white arrows, hyperintense on axial T2-weighted image) and leptomeningeal thickening (D; white arrows, axial T1-weighted image with contrast). Salivary gland histopathology showing typical noncaseating granuloma with giant cells (E; black arrows, hematoxylin staining, ×10 magnification).

A 22-year-old human immunodefiency virus–negative man was admitted to the intensive care unit for generalized seizures. The patient’s family reported a 1-month history of asthenia and intermittent confusion. Clinical examination revealed isolated inappropriateness of behavior related to a frontal lobe syndrome, without any neck stiffness or neurologic deficit. The patient’s temperature was 37°C. Results from routine biological tests and toxicologic analysis were negative. Cerebrospinal fluid analysis was normal except for a slight increase of protein (0.8 g/L). Ziehl and Gram staining were negative. Precontrast brain computed tomography showed bifrontal hypodensity (Figure A) with contrast leptomeningeal enhancement (Figure B). Brain magnetic resonance imaging confirmed marked bifrontal edema (Figure C) associated with leptomeningeal involvement around the frontal falx (Figure D). Because of this specific localization we suspected a neurosarcoidosis,12 which was confirmed by a high plasma level of angiotensin-converting enzyme activity (395 U/L, Normal range <70) and the presence of noncaseating granulomas on minor salivary gland biopsy (Figure E). A combined treatment of steroids and methotrexate induced improvement of neurologic disorders and regression of brain lesions within 6 months.

To read this article in its entirety please visit our website.

-Hafid Ait-Oufella, MD, PhD, Caroline Gabignon, MD, Jean Capron, MD, Arsene Mekinian, MD, PhD

This article originally appeared in the August 2017 issue of The American Journal of Medicine.

Comments are closed.

UA-42320404-1