THE NEURO-OPHTHALMOLOGIC manifestations ascribed to lesions in the mesencephalon involving the periaqueductal gray matter include impairment of vertical gaze (Parinaud's syndrome), retraction nystagmus, convergence nystagmus, convergence spasm, vertical nystagmus, and extraocular palsies.1 Lesions in this area also have been associated with pathologic lid retraction2 and "lightning eye movements."3 Combinations of these eye signs are referred to as the Koeber-Salus-Elschnig sylvian aqueduct syndrome. There have been relatively few clinicopathologic studies on patients with this constellation of neuro-ophthalmologic signs. Most of these studies have revealed a neoplasm in the region of the sylvian aqueduct and the adjacent structures in the midbrain and diencephalon.4-10 Although the syndrome commonly is associated clinically with cerebrovascular disease, discrete infarcts in the periaqueductal gray matter rarely have been demonstrated at autopsy.11-12
The present paper describes the clinical and neuropathologic findings in a patient with the characteristic neuro-ophthalmologic manifestations of the
HATCHER MA, KLINTWORTH GK. The Sylvian Aqueduct Syndrome: A Clinicopathologic Study. Arch Neurol. 1966;15(2):215–222. doi:10.1001/archneur.1966.00470140105015
Coronavirus Resource Center
Customize your JAMA Network experience by selecting one or more topics from the list below.