In this brief communication we wish to report the finding of a metachromatic substance in retinal ganglion cells of two patients with metachromatic leukoencephalopathy. This disease has pathologic features which, as its name implies, have directed attention primarily to the white substance of the brain.1-3 The finding of metachromatic substances in certain cells of the retina, which has no myelin, affords an opportunity to study changes in portions of the nervous system which are unassociated with white matter. So far as we are aware, the retina has not previously been studied in this disease.
The two cases which we had an opportunity to study were typical instances of metachromatic leukoencephalopathy. Since the clinical and neuropathologic findings are to be reported elsewhere in detail,* only a brief resume of these cases will be presented here.
Case 1.
—At the time of death the patient was 13 years old. We had