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. 2018 Jan 22;75(4):495–502. doi: 10.1001/jamaneurol.2017.4373

Table 2. Main Quantitative Variables of Patients With AOA1.

Quantitative Variables
(n = 80)
Median (Range) Mean (SD)
Age at onset, y 6 (2-40) 7.7 (7.4)
Age at examination, y 26 (9-59) 28.5 (13.7)
Duration of disease, y 17 (4-52) 20.9 (12.6)
Age at wheelchair use (n = 40), y 18 (4-57) 20 (11.4)
Duration of disease until wheelchair use (n = 40), y 11.8 (2-38) 13.6 (8.6)
SARA score at last examinationa 28 (6-37) 26.4 (7.6)
SDFS score at last examinationb 6 (3-7) 5.5 (0.9)
Progression rate, SDFS:DD ratio 0.3 (0.1-1.2) 0.3 (0.2)
AFP level, ng/mL 6.0 (1.1-17.0) 6.6 (4)
Albumin level, g/dL 3.25 (1.78-4.59) 3.32 (0.65)
Total cholesterol level, g/L 2.1 (1.0-3.8) 2.2 (0.7)
LDL cholesterol level, g/L 1.7 (0.8-3.2) 1.7 (0.6)
CK level, U/L 185 (32-360) 168.4 (101.5)

Abbreviations: AFP, α-fetoprotein; AOA1, ataxia with oculomotor apraxia type 1; CK, creatine kinase; DD, disease duration; LDL, low-density lipoprotein; SARA, scale for the assessment and rating of ataxia; SDFS, spinocerebellar degeneration functional score.

SI conversion factors: To convert AFP to micrograms per liter, multiply by 1.0; albumin to grams per liter, multiply by 10.0; total and LDL cholesterol to millimoles per liter, multiply by 0.259; and CK to microkatals per liter, multiply by 0.0167.

a

Score range from 0 to 40, with higher scores indicating more more severe ataxia.

b

Scores range from 1 to 7, with higher scores indicating severe impairment.