Tonic Pupils in a Young Female with Vogt Koyanagi Harada Disease

Introduction

Vogt-Koyanagi-Harada (VKH) disease is an inflammatory disorder attributed to an autoimmune reaction against tyrosinase-like proteins in melanocytes in the uvea, the inner ear, the meninges, and the integumentary system [1]. VKH mainly manifests in people with higher melanin levels (e.g., Asians, Asian Indians, Hispanics, and American Indians) [2], [3], and less often in Caucasians [4]. At the onset of the disease, the first anatomical structure to be affected is the melanocyte islets in the choroidal stroma [5]. The inflammatory process starts as choroiditis [6], which evolves to granulomatous panuveitis with exudative retinal detachments. Sometimes, auditory and meningeal symptoms may also develop [2], with mononuclear pleocytosis in the cerebrospinal fluid [7], [8]. Commencing patients on prompt and vigorous immunosuppressive treatment is crucial to prevent uveitis from becoming chronic and protect the integumentary and auditory systems [8]. Interestingly, in some rare cases, the tonic pupil has been described in patients with VKH syndrome [9], [10], [11], [12], [13]. Apart from ocular inflammation, tonic pupil can develop in several conditions including peripheral or autonomic neuropathy. Adieʼs tonic pupil, also known as Adie Syndrome or Holmes-Adie Syndrome, is defined by a poor pupillary light reflex, strong pupillary response to near stimuli, slow redilation of the pupil after constriction to near stimuli, and accommodation paresis. This disorder is caused by the damage postganglionic parasympathetic innervation of the intraocular muscles [14]. Herein, with this report, we present a case of a young female patient who presented with bilateral tonic pupils associated with VKH syndrome.

Publikationsverlauf

Eingereicht: 04. Februar 2025

Angenommen: 23. April 2025

Artikel online veröffentlicht:
28. Mai 2025

• References

• 1 Herbort Jr CP, Tugal-Tutkun I, Abu-El-Asrar A. et al. Precise, simplified diagnostic criteria and optimised management of initial-onset Vogt-Koyanagi-Harada disease: an updated review. Eye (Lond) 2022; 36: 29-43
• 2 Lavezzo MM, Sakata VM, Morita C. et al. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet J Rare Dis 2016; 11: 29
• 3 Yokoyama MM, Matsui Y, Yamashiroya HM. et al. Humoral and cellular immunity studies in patients with Vogt-Koyanagi-Harada syndrome and pars planitis. Invest Ophthalmol Vis Sci 1981; 20: 364-370
• 4 Tran VT, Auer C, Guex-Crosier Y. et al. Epidemiological characteristics of uveitis in Switzerland. Int Ophthalmol 1994; 18: 293-298
• 5 Elahi S, Herbort jr. CP. Vogt-Koyanagi-Harada Disease and Birdshot Retinochoroidopathy, Similarities and Differences: A Glimpse into the Clinicopathology of Stromal Choroiditis, a Perspective and a Review. Klin Monbl Augenheilkd 2019; 236: 492-510
• 6 Hirooka K, Saito W, Namba K. et al. Significant role of the choroidal outer layer during recovery from choroidal thickening in Vogt-Koyanagi-Harada disease patients treated with systemic corticosteroids. BMC Ophthalmol 2015; 15: 181
• 7 Fang W, Yang P. Vogt-Koyanagi-Harada syndrome. Curr Eye Res 2008; 33: 517-523
• 8 Herbort jr. CP, Abu El Asrar AM, Yamamoto JH. et al. Reappraisal of the management of Vogt-Koyanagi-Harada disease: sunset glow fundus is no more a fatality. Int Ophthalmol 2017; 37: 1383-1395
• 9 Levy NS, Kramer SG, de Barros T. Pupillary and accommodative abnormalities in the Vogt-Koyanagi-Harada syndrome. Am J Ophthalmol 1970; 69: 582-588
• 10 Brouzas D, Chatzoulis D, Galina E. et al. Corneal anesthesia in a case with Vogt-Koyanagi-Harada syndrome. Acta Ophthalmol Scand 1997; 75: 464-465
• 11 Kim JS, Yun CH, Moon CS. Bilateral tonic (Adieʼs) pupils in Vogt-Koyanagi-Harada syndrome. J Neuroophthalmol 2001; 21: 205-206
• 12 Narang S, Sood S, Malik A. Probable Vogt-Koyanagi-Haradaʼs syndrome associated with tonic pupils. Nepal J Ophthalmol 2010; 2: 154-156
• 13 Garza Leon M, Herrera-Jimenez IP, González-Madrigal PM. Complete Vogt-Koyanagi-Harada disease and Holmes-Adie syndrome: case report. Ocul Immunol Inflamm 2014; 22: 336-340
• 14 Thompson HS. Adieʼs syndrome: Some new observations. Trans Am Ophthalmol Soc 1977; 75: 587-626
• 15 Nene AS, Pirdankar OH, Shah S. et al. Adieʼs Tonic Pupil: An Uncommon Ophthalmological Emergency. Neurol India 2021; 69: 1487-1488
• 16 Macé T, Haution D, Vautier A. et al. Vogt-Koyanagi-Harada syndrome and Adieʼs tonic pupil: Case report. J Fr Ophtalmol 2022; 45: e355-e356
• 17 Robles-Cedeño R, Fures JF, Molins A. et al. A Moroccan patient with Vogt-Koyanagi-Harada syndrome and bilateral Adieʼs pupils. Neurol Sci 2014; 35: 483-485