Vogt-Koyanagi-Harada syndrome presenting with encephalopathy.

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.

Introduction

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.[2-4] 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

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

Discussion

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.