- Andrew G Lee, MD
Andrew G Lee, MD
- Professor of Ophthalmology, Neurology, and Neurological Surgery
- Weill Cornell College of Medicine
INTRODUCTION AND DEFINITION
The tonic pupil, sometimes called Adie's tonic pupil or simply the Adie pupil, is the term used to denote a pupil with parasympathetic denervation that constricts poorly to light but reacts better to accommodation (near response), such that the initially larger Adie pupil becomes smaller than its normal fellow and remains tonically constricted, redilating very slowly when exposed to dark.
The tonic pupil is a common cause of anisocoria, or unequal pupils. The prevalence of the Adie's pupil is about two cases per 1000 population. Although patients of all ages are affected, the mean age is 32 years, and there is a female predominance (2.6:1) for the idiopathic variety (Adie's tonic pupil).
The tonic pupil is the result of damage to the parasympathetic ciliary ganglion. The parasympathetic innervation to the iris and ciliary body travels with the third cranial nerve and synapses in the ciliary ganglion. After damage to the ciliary ganglion, reinnervation and up-regulation of the post-synaptic receptors occurs, a process known as denervation supersensitivity. The number of axons destined for the ciliary body, however, is about 30 times the number that supply the pupil. After denervation occurs, the reinnervation may be aberrant, and fibers formerly destined for the ciliary body now travel to the pupil (aberrant regeneration).
Activation of the near response in this situation produces a better near reaction than light reaction (light-near dissociation of the pupils), and the reaction is tonic.
Subscribers log in hereLiterature review current through: Nov 2017. | This topic last updated: Oct 19, 2015.References
- Bruyn GW, Gooddy W. Adie's syndrome. In: Neurological Eponyms, Koehler PJ, Bruyn GW, Pearce JMS (Eds), Oxford University Press, New York 2000. p.181.
- Adie WJ. PSEUDO-ARGYLL ROBERTSON PUPILS WITH ABSENT TENDON REFLEXES: A BENIGN DISORDER SIMULATING TABES DORSALIS. Br Med J 1931; 1:928.
- Morgan OG, Symonds CP. Internal Ophthalmoplegia with Absent Tendon-jerks. Proc R Soc Med 1931; 24:867.
- Symonds CP. Internal ophthalmoplegia with absent tendon jerks. Proc R Soc Med 1931; 24:868.
- Holmes G. Partial iridoplegia with symptoms of other diseases of the nervous system. Trans Ophthalmol Soc UK 1931; 51:209.
- Thompson HS. Adie's syndrome: some new observations. Trans Am Ophthalmol Soc 1977; 75:587.
- Leavitt JA, Wayman LL, Hodge DO, Brubaker RF. Pupillary response to four concentrations of pilocarpine in normal subjects: application to testing for Adie tonic pupil. Am J Ophthalmol 2002; 133:333.
- Sakai T, Shikishima K, Mizobuchi T, et al. Bilateral tonic pupils associated with neurosyphilis. Jpn J Ophthalmol 2003; 47:368.
- Fletcher WA, Sharpe JA. Tonic pupils in neurosyphilis. Neurology 1986; 36:188.
- Kawana K, Okamoto F, Nose H, Oshika T. A case of angle closure glaucoma caused by plateau iris and adie's pupil. Am J Ophthalmol 2003; 135:717.
- Leibovitch I, Kurtz S, Almog Y. Adie's tonic pupil-induced angle-closure glaucoma. Ophthalmologica 2002; 216:71.
- Bachmeyer C, Zuber M, Dupont S, et al. Adie syndrome as the initial sign of primary Sjögren syndrome. Am J Ophthalmol 1997; 123:691.
- Bennett JL, Pelak VA, Mourelatos Z, et al. Acute sensorimotor polyneuropathy with tonic pupils and an abduction deficit: an unusual presentation of polyarteritis nodosa. Surv Ophthalmol 1999; 43:341.
- Bowie EM, Givre SJ. Tonic pupil and sarcoidosis. Am J Ophthalmol 2003; 135:417.
- Caputo AR, Mickey KJ, Guo S. A varicella-induced pupil abnormality. Pediatrics 1992; 89:685.
- Chakravarty A, Mukherjee A, Roy D. Ross syndrome--a case documentation. Acta Neurol Scand 2003; 107:72.
- Corridan PG, Laws DE, Morrell AJ, Murray PI. Tonic pupils and human parvovirus (B19) infection. J Clin Neuroophthalmol 1991; 11:109.
- Cox TA, Goldberg RA, Rootman J. Tonic pupil and Czarnecki's sign following third nerve palsy. J Clin Neuroophthalmol 1991; 11:55.
- Davies DR, Smith SE. Pupil abnormality in amyloidosis with autonomic neuropathy. J Neurol Neurosurg Psychiatry 1999; 67:819.
- Foroozan R, Buono LM, Savino PJ, Sergott RC. Tonic pupils from giant cell arteritis. Br J Ophthalmol 2003; 87:510.
- Foster RE, Kosmorsky GS, Sweeney PJ, Masaryk TJ. Horner's syndrome secondary to spontaneous carotid dissection with normal angiographic findings. Arch Ophthalmol 1991; 109:1499.
- Goldstein SM, Liu GT, Edmond JC, et al. Orbital neural-glial hamartoma associated with a congenital tonic pupil. J AAPOS 2002; 6:54.
- Haider S. Tonic pupil in lymphomatoid granulomatosis. J Clin Neuroophthalmol 1993; 13:38.
- Heger T, Kolling GH, Dithmar S. [Atypical tonic pupil as a complication of chickenpox infection]. Ophthalmologe 2003; 100:330.
- Hodgkins PR, Luff AJ, Absolon MJ. Internal ophthalmoplegia--a complication of ocular varicella. Aust N Z J Ophthalmol 1993; 21:53.
- Jacobson DM. A prospective evaluation of cholinergic supersensitivity of the iris sphincter in patients with oculomotor nerve palsies. Am J Ophthalmol 1994; 118:377.
- Jacobson DM. Pupillary responses to dilute pilocarpine in preganglionic 3rd nerve disorders. Neurology 1990; 40:804.
- Kardon RH, Corbett JJ, Thompson HS. Segmental denervation and reinnervation of the iris sphincter as shown by infrared videographic transillumination. Ophthalmology 1998; 105:313.
- Kimber J, Mitchell D, Mathias CJ. Chronic cough in the Holmes-Adie syndrome: association in five cases with autonomic dysfunction. J Neurol Neurosurg Psychiatry 1998; 65:583.
- Bruno MK, Winterkorn JM, Edgar MA, et al. Unilateral Adie pupil as sole ophthalmic sign of anti-Hu paraneoplastic syndrome. J Neuroophthalmol 2000; 20:248.
- Lambert SR, Yang LL, Stone C. Tonic pupil associated with congenital neuroblastoma, Hirschsprung disease, and central hypoventilation syndrome. Am J Ophthalmol 2000; 130:238.
- Mabuchi K, Yoshikawa H, Takamori M, et al. Pseudo-Argyll Robertson pupil of patients with spinocerebellar ataxia type 1 (SCA1). J Neurol Neurosurg Psychiatry 1998; 65:612.
- Patel JI, Jenkins L, Benjamin L, Webber S. Dilated pupils and loss of accommodation following diode panretinal photocoagulation with sub-tenon local anaesthetic in four cases. Eye (Lond) 2002; 16:628.
- Phillips PH, Newman NJ. Tonic pupil in a child. J Pediatr Ophthalmol Strabismus 1996; 33:331.
- Purvin VA. Adie's tonic pupil secondary to migraine. J Neuroophthalmol 1995; 15:43.
- Radziwill AJ, Steck AJ, Borruat FX, Bogousslavsky J. Isolated internal ophthalmoplegia associated with IgG anti-GQ1b antibody. Neurology 1998; 50:307.
- Serra Mitjans M, Callejas Pérez MA, Valls Solé J, et al. [Surgical treatment for compensatory hyperhidrosis in Adie syndrome]. Arch Bronconeumol 2004; 40:97.
- Shin RK, Galetta SL, Ting TY, et al. Ross syndrome plus: beyond horner, Holmes-Adie, and harlequin. Neurology 2000; 55:1841.
- Söylev MF, Saatci O, Kavukcu S, Ergin M. Adie's syndrome in childhood. Acta Paediatr Jpn 1997; 39:395.
- Stromberg BV, Knibbe M. Anisocoria following reduction of bilateral orbital floor fractures. Ann Plast Surg 1988; 21:486.
- Vetrugno R, Liguori R, Cevoli S, et al. Adie's tonic pupil as a manifestation of Sjögren's syndrome. Ital J Neurol Sci 1997; 18:293.
- Wabbels BK, Elflein H, Lorenz B, Kolling G. Bilateral tonic pupils with evidence of anti-hu antibodies as a paraneoplastic manifestation of small cell lung cancer. Ophthalmologica 2004; 218:141.
- Weller M, Wilhelm H, Sommer N, et al. Tonic pupil, areflexia, and segmental anhidrosis: two additional cases of Ross syndrome and review of the literature. J Neurol 1992; 239:231.
- Wirtz PW, de Keizer RJ, de Visser M, et al. Tonic pupils in Lambert-Eaton myasthenic syndrome. Muscle Nerve 2001; 24:444.
- Wolfe GI, Galetta SL, Teener JW, et al. Site of autonomic dysfunction in a patient with Ross' syndrome and postganglionic Horner's syndrome. Neurology 1995; 45:2094.