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A Journal on Ophthalmology
Minerva Oftalmologica 2007 September;49(3):127-30
Minniti D., Palamara F.
Dipartimento delle Specialità Chirurgiche Sezione di Oftalmologia Policlinico “G. Martino” Università degli Studi di Messina, Messina
Adie syndrome is a rare neurological disorder caused by damage to the parasympathetically innervated iris sphincter and ciliary muscles, leading to impaired pupil reflexes and accommodation, as well as the absence of direct and consensual photomotor reflex. During accommodation, the pupil constricts, becoming smaller than normal and dilates slowly. The histological picture resembles that of the new variant Creutzfeldt-Jakob disease (nv-CJD), which causes a degeneration of the central nervous system, without stimulating an immune or inflammatory response, and is associated with the accumulation of an abnormal isoform of prion protein; the anatomic features of the lesions are very similar in the two diseases. There is a constellation of syndromes: CJD, Kuru, fatal insomnia, Gerstmann-Straussler-Scheinker syndrome—all of which are correlated by a morphologic picture characterized by vacuolization of the encephalic nuclei and cortex, with abundant intra- and extracellular deposition of prion protein. The size of pupil opening is controlled by two antagonist smooth muscles, the dilator and the sphincter. The dilator muscle is located in the anterior layer of the epithelial pigment of the iris, and its tone changes in response to two types of stimuli: the brightness of light surrounding the retina and the visual distance of objects, which are part of the triade of accommodation, convergence and miosis. Testing with eye drops and pupillometry are useful for measuring pupil response. Numerous published reports associate Adie syndrome with emerging diseases such as CJD spongiform encephalopathy.