Table 2:

MR imaging findings in 11 patients with AOAD

Patient/ Age/Sex	Supratentorial Periventricular White Matter Abnormalities	Brain stem: M.O. Atrophy/Signal Changes	Cerebellum: Dentate Hilum Signal Changes	Spinal Cord: C1-C2 Atrophy and Signal Changes	Postcontrast Enhancement
1/26/M	Thin band, also visible on T2-weighted images (garl)	Severe atrophy/severe abnormalities	Absent	Present	Present, mult
2/36/F	Thin band, also visible on T2-weighted images	Severe atrophy/severe abnormalities	Present	Present	Not performed
3/26/M	Large band of signal changes (garl)	Severe atrophy/mild to moderate	Present	Present	Present, mult
4/39/F	Thin band, also visible on T2-weighted images	Mild to moderate atrophy/severe abnormalities	Present	Present	Present
5/30/M	Very thin band recognizable on FLAIR, not on T2-weighted images	Severe atrophy/mild to moderate	Present	Present	Present, mult
6/43/F	Large band of signal changes	Mild to moderate atrophy/severe abnormalities	Present	Present	Absent
7/61/M	Absent	Severe atrophy/severe abnormalities	Present	Present	Absent
8/58/M	Absent	Mild to moderate atrophy/severe abnormalities	Absent	Present	Absent
9/52/F	Thin band, also visible on T2-weighted images	Severe atrophy/severe abnormalities	Present	Present	Present
10/64/M	Absent	Mild to moderate atrophy/severe abnormalities	Absent	Present	Absent
11/45/M	Very thin band recognizable on FLAIR, not on T2-weighted images	Severe atrophy/severe abnormalities	Present	Present	Absent

Note:—AOAD indicates adult-onset Alexander disease; garl, ventricular garlands; M.O., medulla oblongata; mult, multiple areas of enhancement; FLAIR, fluid-attenuated inversion recovery.