Table 1 Summary of the clinical features and MRI features of adult-onset Alexander disease reported in the literature [10, 11, 15–36].

Average age at onset

37.0 ± 17.9 (n = 36),

Range: 12.5-62

Clinical features

   Bulbar symptom

35/45 (78%)

   Pyramidal tract signs

33/45 (73%)

   Ataxia

31/44 (71%)

   Palatal myoclonus

15/38 (39%)

MRI findings

   Marked medullary atrophy

37/42 (88%)

   Deep white matter abnormalities

21/43 (49%)

Brainstem signal change

(including nodular lesions)

16/36 (44%)