Vogt-Koyanagi-Harada syndrome presenting with encephalopathy.

Vogt-Koyanagi-Harada (VKH) is a rare syndrome affecting tissues containing melanocytes. The possibility of its autoimmune pathogenesis is supported by high frequent HLA-DR4 presentation, commonly associated with other autoimmune diseases. Eyes are the main affected organs, resulting in blindness. Brain disease is a late-onset event, and is extremely rare. Here, we are reporting a 57-year-old woman, a known case of VKH syndrome, presenting with brain encephalopathy several decades after the initial presentation. We think this long period between initial presentation and presentation of encephalopathy due to VKH syndrome has not been described before. She was treated with corticosteroids and discharged home with a good general condition.

Introduction

Vogt-Koyanagi-Harada (VKH) 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 one is the meningoencephalitis phase, with various neurological symptoms. The second is the ophthalmic auditory phase, which presents with bilateral, chronic and diffuse granulomatous panuveitis, which is frequently associated with auditory and integumentary manifestations with the onset of ocular disease. The third phase is the convalescent phase, presenting with cutaneous signs.[234] The clinical features of VKH syndrome depend on the phase of disease. The consequences 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.[56] The eye involvement 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 the therapy of this syndrome.[8] Diffuse brain disease has not been reported in the late course of this syndrome. Here, we are reporting a 57-year-old woman with VKH syndrome presenting with diffuse brain involvement.

Case Presentation

A 57-year-old Iranian blind woman was admitted to the Al- Zahra Hospital Emergency Room in Isfahan, with complaints of fever and loss of consciousness. She was blind since 38 years ago because of the VKH syndrome. Her underlying syndrome was diagnosed in an ophthalmology center in London, UK, and she was on prednisolone for a while. Since then, she had no other symptoms. She also had no co-morbidities and had no head trauma during her life. Five days before referring to the hospital, she developed fever, headache, vomiting and intermittent disorientation to the time and place. At the day of admission, she gradually lost her consciousness. On arrival, her exam was: axillary temperature 39°Cand Glasgow Coma Scale 3/15. No sign of meningeal irritation was detected. Cerebrospinal fluid (CSF) analysis showed a mild increase in the protein level (60 mg/dL), while other parameters such as herpes simplex virus- polymerase chain reaction (HSV-PCR) were in normal limits. Brain magnetic resonance imaging (MRI) showed diffuse hypointensities in T1, hyperintensity in T2 and FLAIR techniques in the right temporal, both frontal and right parietal areas [Figure 1]. Other laboratory data were normal. The patient dramatically responded to systemic corticosteroid therapy and was discharged from the hospital with a good general condition.

Figure 1

Brain magnetic resonance imaging

Discussion

Our patient had loss of consciousness without signs of meningeal irritation and nearly normal CSF analysis. The brain MRI showed involvement of right temporal, both frontal and right parietal regions. The neurological manifestation of VKH syndrome usually presents in the initial phase of the disorder; and includes headache, meningimus and, occasionally, focal neurologic signs including cranial neuropathies, hemiparesis, aphasia, transverse myelitis and ganglionitis.[6] Rarely, peripheral neuropathy occurs among a constellation of other neurologic problems. Neurological findings in VKHS essentially have been attributed to the aseptic meningitis-like syndrome.[7] Yi, Xiangbo Wang had reported a case of a 16-year- old girl case of VKH syndrome who presented with weakness of the lower extremities. Her spinal MRI revealed longitudinal spinal cord lesions.[6] Lohman described a 28-year-old man whose brain MRI findings were white matter abnormalities on FLAIR and leptomeningeal enhancement.[8] Hashimoto reported a 28-year-old man with a 2-year history of VKH syndrome who presented with facial hypoesthesia, facial nerve palsy, hearing loss and limb ataxia on the right side. An MRI showed inflammatory lesions in the Pons extending into the left middle cerebellar peduncle.[9] However, the temporal intervals between the initial presentation of VKH syndrome and encephalitis in this case was significantly shorter. Our case presented with encephalopathy several decades after the initial presentation of VKH syndrome.