Objective: To identify POLG mutations in patients with sensory ataxia and CNS features.

As it evolves, some patients develop a mild sensory ataxia.

He made a full recovery but 8 years later developed progressive and disabling sensory ataxia.

The clinical picture comprised a chronic neuropathy with marked sensory ataxia and areflexia, and with relatively preserved motor function in the limbs.

All tests were performed by 115 volunteers, 13 patients with cerebellar ataxia and 25 patients with sensory ataxia due to neuropathy.