Sacsin‐related autosomal recessive ataxia without prominent retinal myelinated fibers in Japan

Abstract
Autosomal recessive spastic ataxia of Charlevoix–Saguenay (ARSACS) has been described in the Quebec region and in Tunisia. We report on two Japanese siblings with a new homozygous mutation (6543 del A) of the SACS gene. Compared with previously reported ARSACS patients, both of these patients had a unique phenotype characterized by dementia, ophthalmoplegia, and the absence of prominent retinal myelinated fibers. © 2004 Movement Disorder Society