Note on a reflex mechanism in the fixation of the eyeballs. By WR Gowers, MD, Assistant-Professor of Clinical Medicine in University College (London). Brain 1879: 2; 39-41 and Supranuclear palsy of eyelid closure. By RW Ross Russell. The National Hospital for Nervous Diseases, Queen Square, London WC1; St Thomas's Hospital, London SE1; Moorfield's Eye Hospital, City Road, London EC1. Brain

Abstract

Closure of the eyelids—voluntarily or as part of the reflex response to corneal stimulation, noise or visual threat—involves coordinated contraction of the orbital and preseptal portions of the orbicularis oculi muscles with relaxation of the elevator of the upper lid. Perhaps the pretarsal portion of the muscle is preferentially activated in the reflex movements and the more peripheral parts in voluntary eye closure. Ralph Ross Russell describes three patients, each with Creutzfeldt–Jakob disease, having a variant of supranuclear palsy in which voluntary and reflex eyelid closure are dissociated. A professional musician, aged 52 years, complains of difficulty with rapid movements of the left hand and he cannot close his eyes unless by gently pressing downwards on the upper lids with his fingertips whereupon they remain shut but can voluntarily be opened. His eyes close spontaneously during sleep. Neurological examination confirms the dissociation between impaired voluntary eye closure and intact reflex blinking, and (initially) in the absence of other signs apart from a brisk jaw jerk. Over the next 6 months, he progresses through stages of mild upper motor involvement of the bulbar and limb musculature to impairment of voluntary and pursuit eye movements and a complete inability to close the eyes, although the lids continue to move during downgaze. The disorders of bulbar function, eye movement and limb spasticity increase and are eventually associated with focal muscle wasting and fasciculation, and dementia leading to death at 2 years after presentation. A motor mechanic, aged 32 years, complains of difficulty using the right hand and slurred speech. He has a pseudobulbar palsy and upper motor neuron signs in the limbs. After 12 months slow progression of these complaints, and the emergence of dementia, he develops difficulty with ocular refixation such …