Ræder's Syndrome

Abstract

Raeder's syndrome was first described by the Norwegian ophthalmologist J. G. Raeder in 1918, and the description extended in 1924 by the same author. The seminal report was a description of a young, male patient with unilateral periocular pain combined with ipsilateral miosis and ptosis, and with slight objective signs of trigeminal nerve involvement. Autopsy demonstrated a tumor at the base of the skull in the middle cranial fossa. The term “paratrigeminal” was coined for the picture reported. Later case reports by the same and other authors have included patients with a more benign clinical course, including spontaneous remissions, with unilateral periocular pain and ipsilateral signs of oculosympathetic paresis as the common denominator. This review is a chronological survey of the main contributions that have appeared in the literature and an outline of the various definitions of the syndrome, including a recent classification as well as some pathophysiological and prognostic considerations.