[Tonic pupil and tendon areflexia: the Holmes-Adie's syndrome]

Abstract

The Holmes-Adie syndrome (HAS) is characterised by initially monolateral, then bilateral enlargement of the pupil with delayed responses to near vision and subsequent redilation, and absent tendon reflexes. The diameter of the affected pupil is oval or irregular, sometimes with spontaneous movements of the pupillary sphincter muscle. Reaction to light may require several minutes' exposure to bright light, as it does redilation after a prolonged stay in a dark room. The reaction to near vision is tonic and delayed, as the subsequent redilation. In 10% of cases there is a permanent failure of the pupil to react either to light or to near vision. Absence of deep tendon reflexes is acquired and permanent, most frequently involving the Achilles' tendon reflex. Loss of different degrees of ciliary and spinal ganglia neurons, without evidence of inflammatory findings, are the neuropathological data accounting for the clinical signs of HAS. Polyneuropathies of different origin can be complicated by a symptomatic form of HAS.