Table 1.

Comparison of clinical features of AOA2 and AT patients with prolonged survival.

	I (AT)	II-1 (AOA2)	II-2 (AOA2)
Ataxia onset	Noted by age 2	Noted by age 5	Noted by age 14
Wheelchair bound	Age 11	Age 48	Age 57
Sensorimotor neuropathy	Yes	Yes	Yes
Oculomotor apraxia	No	No	Yes
Nystagmus	Yes, lateral	Yes, horizontal and lateral	Yes, horizontal and lateral
Dysarthria	Yes	Yes	Yes
Cerebellar atrophy	Age 38, MRI brain with midline cerebellar atrophy	Not obtained	Age 59, MRI brain with marked cerebellar atrophy
Elevated AFP	509.9 ng/mL at age 45, repeat 434.6 ng/mL	Not obtained	70.5 ng/mL at age 70
Cognition	Low normal as a child, not demented in adulthood	Normal intellect	Normal intellect
Lifespan	Diagnosed with pancreatic cancer, died 5 months after diagnosis at age 48	Currently alive at age 76, with well- controlled heart failure	Currently alive and well at age 73