Distal symmetric polyneuropathy phenotype in patients with sensory neuronopathy at the time of electrodiagnosis.

Patients with SNN rarely present with a DSP phenotype at the time of electrodiagnosis. The finding that one third of cases resemble DSP at onset highlights the importance of clinical monitoring. In patients with a DSP phenotype, the presence of ataxia at onset or significant progression within 6 months may suggest the possibility of SNN and should prompt additional investigations, such as electrodiagnosis.