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Fifty-one patients with congenital optic nerve hypoplasia (CONH) were reviewed. It was found that the risk of having an affected child is higher in an adolescent mother, and that maternal alcohol or drug abuse may be important factors. Frequently the disorder is associated with other neuropsychiatric handicaps, and with neuro-endocrine abnormalities. The findings suggest that CONH probably is not a homogeneous group of disorders; some may be caused by primary failure of differentiation of the retinal ganglion cells, while others may be the product of an acquired transsynaptic degeneration of optic-nerve fibres.

Pupillary dilatation was observed in a young boy who had a sudden onset of right focal seizures. Clinical and laboratory investigation revealed the presence of a benign left frontal epileptic focus. Lacking signs of third cranial nerve compression, this transient pupillary abnormality could be caused by the contralateral frontal epileptic focus. Such a focus might inhibit dilatation of the homolateral pupil, thus permitting dilatation of the contralateral pupil only. Unilateral mydriasis as described hereby could also represent a pupillary "Todd's Paralysis" caused by the contralateral frontal epileptic focus.

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