A 58-year-old man presented with optic disc edema as a rare associatio
n with spinocerebellar degeneration (SCD). The patient also had chroni
c idiopathic intestinal pseudo-obstruction with hypoalbuminemia. No el
evation of intraspinal pressure and no intracranial lesion was observe
d. The hypoalbuminemia reacted promptly to treatment, whereas the opti
c disc edema regressed gradually. An association between SCD and optic
atrophy has often been described, but to our knowledge this is the fi
rst report of SCD in association with optic disc edema.