SCA3 Presenting as an Isolated Axonal Polyneuropathy

To highlight an unexpected clinical presentation and to review the associated polyneuropathy phenotypes of SCA3.Clinical follow-up.Neurological referral center.Middle-aged man with no family history for SCA3.Presentation with an isolated axonal, distal, symmetric, sensorimotor polyneuropathy for 6 years before developing a cerebellar syndrome prompting genetic testing for SCA3.SCA3 can present with an isolated axonal, distal, symmetric, sensorimotor polyneuropathy.