Clinical features in 11 patients with AOAD
| Pt/Age/Sex | Disease Duration (y) | Spastic Paraparesis | Ataxia | Dysphagia | Dysarthria Dysphonia | Palatal Myoclonus | Others |
|---|---|---|---|---|---|---|---|
| 1/26/M | 7 | + | ± | + | + | − | Ny, diplopia, sphincter d. |
| 2/36/F | 2 | + | − | + | + | − | sphincter d. (f) |
| 3/26/M | 13 | − | + | + | + | + | Ny, ptosis, sphincter d., dysautonomia, scoliosis |
| 4/39/F | 5 | − | ± | + | + | + | Sphincter d., diplopia |
| 5/30/M | 0 | − | − | − | − | − | |
| 6/43/F | 3 months | − | + | − | + | + | Ny, ptosis |
| 7/61/M | 4 | ± | ± | + | + | − | Ny, shoulder girdle wasting & weakness (f) |
| 8/58/M | 4 | + | + | − | − | − | Ny, sphincter d. |
| 9/52/F | 0 | − | − | − | − | − | (f) |
| 10/64/M | 2 | + | − | − | − | − | |
| 11/45/M | 2 | ± | ± | + | + | + | Ny, ptosis, sphincter d. (f) |
Note:—AOAD indicates adult-onset Alexander disease; Ny, nystagmus; sphincter d., sphincter disturbances; +, moderate to severe; ±, mild; (f), familial case based on clinical, MR imaging, or genetic findings in relatives.