Measurements of the intraorbital optic nerve were made using high-resolutio
n coronal MRI in 10 adults with autosomal dominant optic atrophy. Compariso
ns were made with previous studies of 10 normal adult subjects. The cross-s
ectional diameters of the optic nerve and the perineural subarachnoid space
were measured and a ratio of there diameters at anterior, mid and posterio
r positions along the optic nerve was determined. We found a statistically
significant difference in the mean optic nerve: sheath ratio between the co
ntrol group and patients with autosomal dominant optic atrophy. At anterior
, mid and posterior locations along the optic nerve it is significantly sma
ller in patients with optic atrophy. We have demonstrated that the loss of
ganglion cells, previously documented in dominant optic atrophy, is associa
ted with a significant loss of optic nerve tissue and thinning of the nerve
along its length.