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| ARTICLE |
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| Year : 1966 | Volume
: 14
| Issue : 3 | Page : 128-130 |
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Vogt - Koyanagi syndrome with optic neuritis
BS Bhatnagar, S Nahar
Gandhi Medical College, Bhopal (M.P.), India
| Date of Web Publication | 16-Jan-2008 |
Correspondence Address:
B S Bhatnagar
Gandhi Medical College, Bhopal (M.P.)
India
 Source of Support: None, Conflict of Interest: None  | Check |
How to cite this article:
Bhatnagar B S, Nahar S. Vogt - Koyanagi syndrome with optic neuritis. Indian J Ophthalmol 1966;14:128-30 |
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 | |  |
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 | | |
Blood:-
Total R.B.C. - 4.62 Mill/C.mm.
Total W.B.C.-8900/C.mm.
Differential count
Polymorphs-55 %
Lymphocytes- 40%
Eosinophils-3 %
Monocytes-2%
E.S.R.-40 mm. (Wintrobe) 1st hour.
V.D.R.L.-Negative.
Urine examination, stool examination and skiagrams of skull and chest showed no abnormality.
| Treatment | | |
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 | | |
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 | | |
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 | | |
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 | | |
| 1. | Bennet, F. W. (1962), Brit. J. Ophthal. Vol. No. 46: 563.  |
| 2. | Boke, W. (1959). Klin. Mbl. Augenh Vol. No. 134: 407 cited by Bennet in 1 |
| 3. | Bronstein M. (1957). Am. J. Ophthal. Vol. 57. 503-507. |
| 4. | Cordes F. C. (1955). Am. J. Ophthal. Vol. 39, 499. |
| 5. | Crawford H. E. (1953), Hawaii M. J. 13: 26. |
| 6. | Carrasquillo, H. F. (1942): Arch. Ophthal. 31: 520. |
| 7. | Cowper A. R. (1951). Arch. Ophthal. Vol. 45: 367. |
| 8. | Duke-Elder. S. (1940), Text Book of Ophthal. Vol. 3 p. 2367. Kimpton. London. |
| 9. | Gregory. D. E. (1959). Brit. J. Ophthal. Vol. 43: 113. |
| 10. | Hague, E. B. (1944), Arch. Ophthal. Vol. 31: 520. |
| 11. | Lambert (1955). Am. J. Ophthal. Vol. 40: 148 |
| 12. | Parker. W. R. (1940), Arch. Ophthal. Vol. 437. |
| 13. | Swartz. E. A. (1955), Amer. J. Ophthal. Vol. 39: 498. |
| 14. | Walsh F. B. (1957). "Clinical Neuro Ophthalmology." page 462-465. The Williams and Wilkins Co., Baltimore. II Edition. |
| 15. | Wilson P. (1962). Brit. J. Ophthal. Vol. 46: 626. |
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