The Holmes-Adie plus syndrome

Abstract

A clinical syndrome of tonic pupil associated with tendon areflexia was first described by Holmes and Adie; autonomic neuropathy and peripheral neuropathy can be associated. The postulated mechanism of areflexia in Holmes-Adie syndrome is a synaptic disorder of the spinal reflex pathways. We report a case of a Holmes-Adie syndrome variant with hitherto unreported cranial neuropathy. A 41 year old woman developed insidious onset of sensory symptoms related to her left trigeminal and chorda tympani nerves over a few months. Physical examination showed generalised tendon areflexia and a left sided Adie's pupil. Imaging did not reveal any structural abnormality. Electrophysiological studies demonstrated an absent blink reflex on stimulating the left supraorbital and infraorbital nerves. These findings were suggestive of a dysfunction affecting the brain stem reflex arc. The pathophysiological process of Holmes-Adie syndrome may be more widespread than previously thought. (C) 2000 Harcourt Publishers Ltd.