Infantile uniocular blindness with bilateral nystagmus. A syndrome
B. J. Kushner
Department of Ophthalmology and Visual Sciences, University of Wisconsin, Madison, USA.
BACKGROUND: A syndrome has been described in which some patients with
uniocular infantile blindness have a horizontal nystagmus in their
contralateral, structurally sound eye. This nystagmus has the
characteristics of latent nystagmus. This study investigated the hypothesis
that this syndrome is present when infantile monocular blindness occurs in
patients who are genetically predisposed to congenital strabismus. In these
patients, nystagmus, which would be latent, is made manifest by media
opacity or suppression acting as an occluder. PATIENTS: 1 prospectively
studied all patients with this syndrome whom I examined between 1982 and
1994. Evaluation included a careful investigation of whether there was a
family history of congenital strabismus. Three patients underwent
electro-oculography. RESULTS: The series consisted of 24 patients with the
syndrome of infantile uniocular blindness with bilateral nystagmus, of whom
14 (58%) had a family history of congenital strabismus. In a consecutive
series of 50 patients with monocular congenital blindness caused by opacity
of the ocular media but not manifesting nystagmus of the contralateral eye,
only three patients (6%) had a family history of congenital strabismus.
This difference was statistically significant (P < .01). CONCLUSIONS: It
appears likely that the syndrome of monocular infantile blindness with
bilateral nystagmus represents a manifest nystagmus of the latent type in
patients who have inherited a genetic predisposition for congenital
strabismus. In these patients, the monocular blindness (opacity of the
media or suppression) acts as an occluder, making manifest what would have
been latent nystagmus.
