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.