Marines co-Sjogren syndrome is an inherited neuro-ocular disease with
spinocerebellar symptoms and cataracts. The authors report three cases
, two of them belonging to the same family, with unusual magnetic reso
nance imaging findings, consisting of atrophy of optic nerve, optic ch
iasm and occipital cortex. Like in other progressive ataxias, the auth
ors are probably dealing with different neuropathological and clinical
forms, some of which combining atrophy of the optic nerve and optic p
athway with the basic features of cerebellar and brain stem atrophy.