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Show Journal of Neuro- Ophthalmology 21( 3): 205- 206, 2001. • 2001 Lippincott Williams & Wilkins, Inc., Philadelphia Original Contribution Bilateral Tonic ( Adie's) Pupils in Vogt- Koyanagi- Harada Syndrome Ji Soo Kim, MD, Chang Ho Yun, MD, and Chul Shin Moon, MD We report a 38- year- old woman with bilateral tonic ( Adie's) pupils who was diagnosed as having Vogt- Koyanagi- Harada syndrome. The tonic pupils persisted after other clinical features of this syndrome had disappeared. Key Words: Tonic pupils- Harada syndrome. Adie's pupils- Vogt- Koyanagi- Vogt- Koyanagi- Harada ( VKH) syndrome is an autoimmune disorder characterized by depigmentary inflammation of melanocyte- containing tissues. Findings include bilateral panuveitis with exudative retinal detachment, vitiligo, alopecia, poliosis ( patchy whitening of the scalp hair, eyelashes, and eye brows), tinnitus, hearing impairments, and meningismus ( 1). Tonic ( Adie's) pupil is characterized by a poor pupillary light reflex, accommodation paresis, strong pupillary response to near stimuli, and slow redilation of the pupil after constriction to near stimuli. Damage to the postganglionic parasympathetic innervation of the intraocular muscles produces this syndrome. Tonic pupils can develop in several conditions such as ocular inflammation, peripheral or autonomic neuropathy, and Adie syndrome ( 2). Rarely has tonic pupil been described in VKH syndrome ( 3,4). We report a young woman who developed bilateral tonic pupils associated with VKH syndrome. Manuscripts received May 15, 2001; accepted June 21, 2001. Supported by an unlimited fund for clinical research from Cheju Medical Center. From the Department of Neurology, College of Medicine, Cheju National University ( JSK); and the Departments of Neurology ( CHY) and Ophthalmology ( CSM), Cheju Medical Center, Cheju, Korea. Address correspondence and reprint requests to Ji Soo Kim, MD, Department of Neurology, College of Medicine, Cheju National University, Ara 1- dong, Cheju 690- 756, Korea. E- mail: jisookim@ cheju. cheju. ac. kr CASE REPORT A 38- year- old woman noticed bilateral visual loss on awakening. She denied any pain on eye movements. She had been well without significant medical history. Initial ophthalmologic evaluation revealed visual acuity of count fingers at 2 m in the right eye ( OD) and 20/ 200 in the left eye ( OS). Both pupils measured 7 mm, and there was no light reaction. Slit- lamp examination showed inflammatory cells in the anterior chamber and vitreous body. Posterior synechia was not present. Both optic disks were hyperemic and swollen. The retinal vessels were tortuous. Macular edema was noted in both eyes, but exudative retinal detachments were not observed. Visual evoked potentials ( VEP) revealed delayed response in both eyes. She was diagnosed as having bilateral optic neuritis and uveitis and treated with a topical steroid and oral prednisolone for 1 month. Approximately 1 week after symptom onset, she developed severe headache with nausea, vomiting, and myalgia. Over the next 2 months, her visual acuity improved, and examination showed no inflammatory cells in the anterior chamber and vitreous body. However, her unreactive and dilated pupils remained unchanged. At this stage, she was referred to a neuro- ophfhal-mology clinic for evaluation of persistent bilateral pupillary mydriasis of unknown cause. She complained of persistent blurred vision and photophobia while reading. She reported severe hair loss during the acute phase of her illness. She denied tinnitus or hearing loss. Neuro- ophthalmologic examination revealed vitiligo and poliosis in the hair and upper eyelashes that had evidently developed well after the acute phase of her illness ( Fig. 1A). She had visual acuity of 20/ 20 in each eye ( OU) with normal visual fields and full ocular motility. Near visual acuity was 20/ 25 OU. Both pupils were 7 mm and unreactive to light and near targets. Fundus examination showed normal discs and maculae. The general neurologic examination was normal including deep tendon reflexes. Thirty minutes after instillation of one 205 206 J. S. KIMETAL. FIG. 1. A: Patchy whitening ( poliosis) of upper eyelashes ( arrow). B: Thirty minutes after instillation of one drop of 0.125% pilocarpine, the left pupil demonstrates denervation supersensitivity by constricting from 7 to 3 mm drop of 0.125% pilocarpine OS, the pupil constricted to 3 mm ( Fig. IB). Follow- up VEP was normal. She also had normal brain magnetic resonance imaging, C-reactive protein, and antinuclear antibody. Based on the combination of tonic pupils, vitiligo, poliosis, and the history of uveitis, retinal edema, and optic neuritis, VKH syndrome was diagnosed. Three months later, or 6 months after symptom onset, both pupils measured 6 mm and began to react to light and near targets, although she still experienced blurred vision in the daylight. The pupils constricted more strongly with near stimuli than with light and showed slow redilation after viewing near targets. The poliosis had almost resolved, leaving the tonic pupils as the only residual sign of VKH syndrome. DISCUSSION Tonic pupils can be associated with a variety of inflammatory and infectious disorders that damage the ciliary ganglion alone or as part of a systemic process. VKH syndrome is a systemic disease affecting the uvea, retina, meninges, and skin. Associated neurologic findings are cranial nerve palsies, horizontal nystagmus, diminished labyrinthine function, sensorineural hearing loss, tinnitus, and increased vestibulo- ocular reflex ( 5). Tonic pupils have rarely been reported in this syndrome ( 3,4). The pupillary tonicity in VKH syndrome may be owing to degeneration at the ciliary ganglion or involvement of short ciliary nerves owing to the diffuse ocular inflammation. This is presumably followed by aberrant reinnervation. VKH syndrome takes a course that can be divided into three stages: the meningeal ( prodromal), the ophthalmic, and the convalescent stage ( 6). The characteristic skin and hair manifestations such as vitiligo, poliosis, and alopecia usually develop in the convalescent stage, approximately 2 to several months after symptom onset ( 1). In our patient, the only residual ophthalmologic finding was bilateral tonic pupils when the diagnosis of VKH syndrome was made. Tonic pupils in VKH syndrome may be transient, lasting several months, or can persist more than a year ( 3,4). Our patient had visual blurring and photophobia from tonic pupils even after resolution of the poliosis. In patients with tonic pupils, preceding or accompanying symptoms and signs of VKH syndrome should be sought. REFERENCES 1. Moorthy RS, Inomata H, Rao NA. Vogt- Koyanagi- Harada syndrome. Surv Ophthalmol 1995; 39: 265- 92. 2. Thompson HS. Adie's syndrome: Some new observations. Trans Am Ophthalmol Soc 1977; 75: 587- 626. 3. Levy NS, Kramer SG, Barros TD. Pupillary and accommodative abnormalities in the Vogt- Koyanagi- Harada syndrome. Am J Ophthalmol 1970; 69: 582- 8. 4. Brouzas D, Chatzoulis D, Galina E, et al. Corneal anesthesia in a case with Vogt- Koyanagi- Harada syndrome. Acta Ophthalmol Scand 1997; 75: 464- 5. 5. Yoshimoto Y. Otoneurological observation and classification of Harada's disease presenting with aural symptoms, especially vertigo. Acta Otolaryngol Suppl 1995; 519: 114- 7. 6. Cowper AR. Harada's disease and Vogt- Koyanagi syndrome: uveoencephalitis. Arch Ophthalmol 1951; 45: 367- 76. / Neuro- Ophthalmol, Vol. 21, No. 3, 2001 |
