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Table 1: 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.