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Table of Contents
Year : 2013  |  Volume : 16  |  Issue : 2  |  Page : 264-265

Vogt-Koyanagi-Harada syndrome presenting with encephalopathy

1 Department of Infectious and Tropical Diseases, Isfahan University of Medical Sciences, Isfahan, Iran
2 Department of Neurology, Isfahan University of Medical Sciences, Isfahan, Iran

Date of Submission24-May-2012
Date of Decision24-May-2012
Date of Acceptance23-Jun-2012
Date of Web Publication24-May-2013

Correspondence Address:
Alireza E Naeini
Shams Abadi St. No.106, Razi Medical building, 8134776514, Isfahan
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Source of Support: None, Conflict of Interest: None

DOI: 10.4103/0972-2327.112490

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VogtKoyanagi-Harada (VKH) is a rare syndrome affecting tissues with melanocytes. The possibility that VKH syndrome has an autoimmune pathogenesis is supported by the high frequency of human leukocyte antigen-DR4 commonly associated with other autoimmune diseases. Eyes are the main affected organ, resulting in blindness. Brain disease as a late onset event is extremely rare. Here, we are reporting a 57-year-old woman with previously diagnosed VKH syndrome, presenting with a late-onset brain encephalopathy. She was treated with corticosteroids and discharged from hospital with good general condition.

Keywords: Autoimmunity, encephalopathy, human leukocyte antigen (HLA), Vogt-Koyanagi-Harada syndrome

How to cite this article:
Naeini AE, Daneshmand D, Khorvash F, Chitsaz A. Vogt-Koyanagi-Harada syndrome presenting with encephalopathy. Ann Indian Acad Neurol 2013;16:264-5

How to cite this URL:
Naeini AE, Daneshmand D, Khorvash F, Chitsaz A. Vogt-Koyanagi-Harada syndrome presenting with encephalopathy. Ann Indian Acad Neurol [serial online] 2013 [cited 2021 Dec 7];16:264-5. Available from:

   Introduction Top

Vogt--Koyanagi--Harada syndrome is a rare multisystemic disease of presumed autoimmune etiology, affecting various melanocyte-containing organs. [1] The classic course of VKH syndrome has three phases. The first phase is the meningoencephalitis phase, with various neurological symptoms. The second phase is the ophthalmic auditory phase, presenting with bilateral, chronic, diffuse granulomatous panuveitis that is frequently associated with auditory and integumentary manifestations with the onset of ocular disease. The third phase is convalescent, characterized by cutaneous signs. [2],[3],[4] The clinical features of VKH syndrome vary depending on the phase of the disease. [5] The consequence of eye disease are granulomatous anterior uveitis, vitritis, thickening of the posterior choroid with elevation of the peripapillary retinal choroidal layer, heperemia and edema of the optic nerve and multiple serous retinal detachments. [6] This eye disease will progress to blindness. Neurological findings that commonly appear at the acute stage of this syndrome are meningismus, tinitus and cerebrospinal fluid pleocytosis. [7] Systemic corticosteroids and immunomodulatory agents have been used for therapy of this syndrome. [8] Diffuse brain disease has not been reported in the late course of this syndrome. Here, we are reporting a 57year-old woman with VKH syndrome presenting with a diffuse brain involvement.

   Case Report Top

An Isfahanian, 57-year-old blind woman presented to the Al-Zahra Hospital Emergency Room, suffering with fever and loss of consciousness. She had become blind 48 years ago due to the VKH syndrome. Her underlying syndrome was diagnosed in an ophthalmology center in London, and she had been taking prednisolone for a period of time. She had no other symptoms during these years. Five days before referring to the hospital, she had been experiencing fever, headache, vomiting and intermittent disorientation to time and place. At the day of admission, she gradually lost her consciousness. On examination the axillary temperature was 39°C and Glasgow Consciousness Score was 3/15. No signs of meningeal irritation were detected. Cerebrospinal fluid (CSF) analysis showed a mild increase of protein (60 mg/dL), and other parameters such as herpes simplex virus - polymeaerase chain reaction (HSV-PCR) were normal. Brain magnetic resonance imaging (MRI) showed diffuse T2 and FLAIR hyperintensity in the right temporal, both frontal and right parietal lobes that were hyposignal in T1 [Figure 1]. Other laboratory data were normal. The patient dramatically responded to systemic corticosteroid therapy and was discharged from the hospital with good general condition.
Figure 1: MRI showing diffuse T2 and FLAIR hyperintensity in the right temporal, both frontal and right parietal lobes that were hypointense in T1 weighted images

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   Discussion Top

Our patient had loss of consciousness without meningeal irritation signs and nearly normal CSF analysis. The brain MRI showed involvement of the right temporal, both frontal and right parietal lobes. The neurological manifestations of VKH syndrome usually occurs in the initial phase of the disorder, and consists of headache, meningismus and, occasionally, focal neurological signs, including cranial neuropathies, hemiparesis, aphasia, transverse myelitis and ganglionitis. [6] Rarely peripheral neuropathy occurs among a constellation of other neurologic problems in patients with VKH syndrome. Neurological findings in VKH essentially have been attributed to the aseptic meningitis-like syndrome. [7] Wang has reported VKH in a 16year-old girl with weakness of lower extremities. Her spinal MRI revealed longtudinal spinal cord lesions. [8] Lohman has described a 28-year-old man whose bran MRI finding included white matter abnormalities on FLAIR and leptomeningeal enhancement. [9] Late-onset paranchymal diffuse brain disease due to underlying VKH syndrome is extremely rare.

   References Top

1.Andreoli CM, Foster CS. Vogt- Koyanagi- Harada disease. Int Ophthalmol Clin2006;46:111-2.  Back to cited text no. 1
2.Fang W, Yang P. Vogt-koyanagi-harada syndrome. Curr Eye Res 2008;33:517-23.  Back to cited text no. 2
3.Chee SP, Jap A,- Bascal K. Spectrum of Vogt- Koyanagi- Harada disease in Singapore. Int Ophthalmol 2007;27:137-42.  Back to cited text no. 3
4.Rao NA, Sukavatcharin S, Tsai JH. Vogt-Koyanagi-Harada disease diagnostic criteria. Int Ophthalmol 2007;27:1905-9.  Back to cited text no. 4
5.Read RW, Rechodouni A, Butani N, Johnston R, LaBree LD, Smith RE, et al. Complications and prognostic factors in Vogt-Koyanagi-Harada Syndrome. Am J Ophthalmol 2001;131:599- 606.  Back to cited text no. 5
6.Sheu SJ. Update on uveomeningoencephalitides. Curr Opin Neurol 2005;18;323-9.  Back to cited text no. 6
7.Tang Y, Wang X, Ding Y, Li C, JiaJ. Longitudinal lesion of the spinal cord in a patient with Vogt - Koyanagi- Harada disease. J Neurol Neurosurg Psychiatry 2010;81:941-2.  Back to cited text no. 7
8.B.D. Lohman, C.A. Gustafson, A.M. McKinney, B. Sarikaya, S.C. Silbert. MR Imaging of Vogt - Koyanagi- Harada Syndrome with Leptomeningeal Enhancement. AJNR 2011;32: 169-71.  Back to cited text no. 8
9.Read RW, Rao NA, Cunningham ET. Vogt- Koyanagi- Harada disease. Curr Opin Ophthalmol 2000;11:37-42.  Back to cited text no. 9


  [Figure 1]

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