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Year : 1966  |  Volume : 14  |  Issue : 3  |  Page : 128-130

Vogt - Koyanagi syndrome with optic neuritis

Gandhi Medical College, Bhopal (M.P.), India

Date of Web Publication16-Jan-2008

Correspondence Address:
B S Bhatnagar
Gandhi Medical College, Bhopal (M.P.)
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How to cite this article:
Bhatnagar B S, Nahar S. Vogt - Koyanagi syndrome with optic neuritis. Indian J Ophthalmol 1966;14:128-30

How to cite this URL:
Bhatnagar B S, Nahar S. Vogt - Koyanagi syndrome with optic neuritis. Indian J Ophthalmol [serial online] 1966 [cited 2024 Mar 5];14:128-30. Available from: https://journals.lww.com/ijo/pages/default.aspx/text.asp?1966/14/3/128/38641

Vogt-Koyanagi syndrome is a rare condition of uncertain etiology and poor prognosis as regards vision. Bi­lateral anterior and/or posterior uveitis is a prominent feature in almost all cases associated with poliosis, vitiligo and alopecia in various proportions. Disacousia may be present in majority of cases. All the manifestations of this disease may not be present and differ­ent authors have commented on indivi­dual features of the disease.

The exact etiology of the disease is still unknown. A virus infection involv­ing the uveal tract and brain particu­larly the hypothalamic area has been considered to be the probable cause­-Hague (1944), but so far no virus has been isolated. It. has been suggested that clinically and pathologically. Vogt-Koyanagi, Harada's disease, and sympathetic ophthalmitis are classified under granulomatous uveitis, present­ing different grades of severity. Ana­phylactic effect of the uveal pigment on skin and hair has been attributed as an alternative theory by Gregory (1959). The widespread nature of the lesion may be suggestive of some toxic factor responsible for the condition.

Cowper (1951) classified the disease in three phases.

(a) Meningeal of abrupt onset-This is of 2-4 weeks duration with low fever and headache.

(b) Ophthalmic-This may accom­pany or follow anterior and/or posterior uveitis which may last for 3-4 months.

(c) Convalescent-Alopecia, poliosis may develop and this phase lasts for 6-12 months.

The response to corticosteroids in Vogt-Koyanagi syndrome is quite satis­factory. Cordes (1955) and Lambert (1955) obtained good results with the topical and general administration of cortisone acetate.

The prolonged use of steroids in their maximum therapeutic dosage is suggested for beneficial effects (Bron­stein 1957, Crawford 1953).

  Case Report Top

Patient M.S., 20 years male, farmer by occupation was admitted to the Eye ward of the Hamidia Hospital, Bhopal, on 30-4-64. About two months back one day he suddenly observed dimness of vision in the right eve, followed 24 hours later by dimness of vision of the other eye. At that time he could only see the red glow of the lamp illuminat­ion the room. He was treated by some indigenous drugs for about a month and ~a half without any relief. Family history and history of past illness were noncontributory.

He was a young man of average build and was fairly nourished. There was alopecia-peripheralis, axillary hair were very few in number, patches of alopecia were also present in the temporal regions. White depigmented patches (vitiligo) were present on the skin of both upper lids and the eye lashes were grey (poliosis) in both the lids in both eyes.

Vision on admission was R.E. finger counting at I m. and L.E. at 1½ m.

External examination of the eye balls revealed slight loss of the pattern of iris and deposits of uveal pigment on the anterior capsule of the lens in both eyes. Pupillary margin was slightly irregular on the right side.

Fundus examination revealed bilate­ral blurring of disc margins with about I D swelling and slight hyperemia. Irregular pigment deposits were seen all over the fundii, foveal reflexes were dull and macular areas showed irregu­lar mottling. Arteries were slightly narrowed. The fundus picture typically resembeled optic neuritis and poste­rior uveitis.

Visual fields could not be charted due to poor visual acuity. Systemic ex­amination did not reveal any abnormal findings in the body. Blood pressure was within normal limits. Hearing was found to be normal.

  Laboratory Investigations Top


Total R.B.C. - 4.62 Mill/C.mm.

Total W.B.C.-8900/C.mm.

Differential count­

Polymorphs-55 %

Lymphocytes- 40%

Eosinophils-3 %


E.S.R.-40 mm. (Wintrobe) 1st hour.


Urine examination, stool examina­tion and skiagrams of skull and chest showed no abnormality.

  Treatment Top

On admission, the patient was start­ed on a regime of Corlin (cortisone acetate. 5 mgm.), one tablet 6 hourly, injection of Vitamin B 1 -100 mgm. and Vitamin B 12 -500 microgram intramus­cular daily. Strepto-Penicillin (1 Gm) injections were also given for 7 days.

Retrobulbar injections of 0.25 cc. of Decadron (Dexamethasone 21-Phos­phate, 4 mgm. per ml.) were given daily for 4 days and subsequently on alternate days for six turns and finally biweekly. On 31-5-1964 retrobulbar injections were stopped. Corlin tab­lets were stopped on 24-6-1964. Intra­muscular injections of Vitamin B,-100 mgm. and Vitamin B 12 -500 micrograms were continued on every 4th day along with salicylates during the subsequent period of hospitalisation.

  Progress Top

By 25-5-1964 vision had improved to 6/60 B.E. and on 31-5-64 the vision was 6/24 each eye. Retrobulbar injec­tions were stopped now, but the rest of the treatment was continued. By 15-6-64 the vision had improved to 6/9 each eye. Fundus examination now revealed slight blurring of the disc margins, mottling and hyperoemia were less. Vision on 24-6-64 was 6/6 B.E. Fundii showed almost nor­mal discs with irregular choroidal pig­mentation and slight attenuation of the arteries.

No changes were observed in alo­pecia, vitiligo and pigment deposits on the anterior capsule of the lens re­mained unchanged. During the sub­sequent period of observation in the hospital upto 8-9-1964 there was no recurrence of dimness of vision or any other change in the fundus picture.

  Discussion Top

The case reported here presented with a picture of typical papillitis, posterior uveitis and anterior uveitis along with alopecia and pigmentary changes in the skin and cilia of the lids without involvement of hearing. Parker, reported impairment of hearing in 69 per cent of cases. It has been reported that in some cases low grade optic neuritis, posterior uveitis and involvement of retina may be visible at the onset of the disease but in the later stages, details of the fundi become invisible due to a progressive anterior uveitis.

In our case the vision returned to normal in both eyes after prolonged therapy with corticoids given orally and by retrobulbar route and there were no relapses after stopping the cortisone therapy. Boke (1959) found that when the disease involved both the anterior and posterior segments, the eye would eventually become totally blind. In cases where the dis­ease was limited to the posterior seg­ment oral corticosteroid therapy pro­duced a good response with return of full function of the eyes. In this parti­cular case the response to cortisone therapy was dramatic as the patient mainly started with a picture of optic neuritis and posterior uveitis.

  Summary Top

A case of Vogt-Koyanagi syndrome with optic neuritis, pigmentation of choroid, alopecia, poliosis and vitiligo has been reported. There was no in­volvement of hearing in this case. There was dramatic improvement in the vision with corticosteroids (orally and by retrobulbar injections) and ad­juvant therapy with Vitamin B 1 and B 12 . There were no relapses on stop­page of corticosteroids during the fol­low up period.

We thank Dr. Santokh Singh, Professor and Head of the Department of Ophthalmology for his valuable suggestions and guidance.[15]

  References Top

Bennet, F. W. (1962), Brit. J. Ophthal. Vol. No. 46: 563.  Back to cited text no. 1
Boke, W. (1959). Klin. Mbl. Augenh Vol. No. 134: 407 cited by Bennet in 1  Back to cited text no. 2
Bronstein M. (1957). Am. J. Ophthal. Vol. 57. 503-507.  Back to cited text no. 3
Cordes F. C. (1955). Am. J. Ophthal. Vol. 39, 499.  Back to cited text no. 4
Crawford H. E. (1953), Hawaii M. J. 13: 26.  Back to cited text no. 5
Carrasquillo, H. F. (1942): Arch. Oph­thal. 31: 520.  Back to cited text no. 6
Cowper A. R. (1951). Arch. Ophthal. Vol. 45: 367.  Back to cited text no. 7
Duke-Elder. S. (1940), Text Book of Ophthal. Vol. 3 p. 2367. Kimpton. London.  Back to cited text no. 8
Gregory. D. E. (1959). Brit. J. Ophthal. Vol. 43: 113.  Back to cited text no. 9
Hague, E. B. (1944), Arch. Ophthal. Vol. 31: 520.  Back to cited text no. 10
Lambert (1955). Am. J. Ophthal. Vol. 40: 148  Back to cited text no. 11
Parker. W. R. (1940), Arch. Ophthal. Vol. 437.  Back to cited text no. 12
Swartz. E. A. (1955), Amer. J. Ophthal. Vol. 39: 498.  Back to cited text no. 13
Walsh F. B. (1957). "Clinical Neuro Ophthalmology." page 462-465. The Williams and Wilkins Co., Baltimore. II Edition.  Back to cited text no. 14
Wilson P. (1962). Brit. J. Ophthal. Vol. 46: 626.  Back to cited text no. 15


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