Fig. 2
From: Cerebellar ataxia and exercise intolerance in Erdheim-Chester disease
Magnetic resonance imaging findings suggestive of ECD. Before starting Vemurafenib, MRI of the brain (a, b; fluid attenuated inversion recovery: FLAIR sequences) showed cerebellar atrophy and hyperintensity of the dentate nuclei (DN). T2 hyperintense inflammatory lesions (c, d; arrows) of the DN seen at treatment initiation (c) and after 6 months of BRAF inhibition (d). Susceptibility weighted imaging (e, f) demonstrated paramagnetic compound accumulation in the DN (e, arrows), caudate nuclei, and globi pallidi (f, arrow). Spectroscopy MRI (g, h) showed a reduction of N-acetyl aspartate (NAA) in the DN (g: red box in axial FLAIR MRI was the region of measurements reported in (h))