Figure 1.

Brain MRI of a patient with adult-onset Alexander disease. a-f: T2-weighted axial images showing marked atrophy of the medulla oblongata (a) with slight cerebellar atrophy (a, b) but little atrophy of the pontine base (b), enlargement of the fourth ventricle (b), atrophy of the midbrain, especially the dorsal part (c), bilateral changes in the posterior part of globus pallidus (d), bilateral lesions of the fronto-orbital areas, predominating on the left, caused by brain contusion (d, e, g, h), moderate cortical atrophy with ventricular enlargement (e, f), and bilateral lacunae in the deep white matter, but no leukoencephalopathy (f). g and k: T1-weighted images of the lesions on axial (g) and sagittal (k) sections. h, i and j: FLAIR images of the lesions on axial (h, i) and coronal (j) sections. The lesions on coronal (j) and sagittal (k) sections are indicated by arrows. Note the absence of ventricular garlands [15]. l: T1-weighted sagittal section showing typical tadpole-like brainstem atrophy, consisting of marked cervico-medullary atrophy with an intact pontine base; note that atrophy of the midbrain tegmentum also contributes to the formation of the tadpole.