The Holmes-Adie plus syndrome

A clinical syndrome of tonic pupil associated with tendon areflexia was fir st described by Holmes and Adie; autonomic neuropathy and peripheral neurop athy can be associated. The postulated mechanism of areflexia in Holmes-Adi e syndrome is a synaptic disorder of the spinal reflex pathways. We report a case of a Holmes-Adie syndrome variant with hitherto unreported cranial n europathy 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. Electroph ysiological studies demonstrated an absent blink reflex on stimulating the left supraorbital and infraorbital nerves. These findings were suggestive o f a dysfunction affecting the brain stem reflex are. The pathophysiological process of Holmes-Adie syndrome may be more widespread than previously tho ught. (C) 2000 Harcourt Publishers Ltd.