How high is high in steroid treatment of Vogt-Koyanagi-Harada syndrome

Abstract

Vogt-Koyanagi-Harada syndrome (VKH) is a disease with a bilateral uveitis, dermatologic findings, and neurologic deficits. Highdose corticosteroid therapy frequently improves visual involvement in VKH.' Recently, Helveston and G:ilmore2 reported treatment of VKH with intravenous immunoglobulin, although prednisone (80 mg/d) treatment had not ameliorated neurologic signs in their patient. We report a patient who had successful treatment with high-dose oral betamethasone after having failed with standard oral prednisolone. We discuss initial treatment of neurologic signs in VKH with high-dose corticosteroids.Case report. A 37-year-old Japanese man noticed dysesthesia of the feet late December 1995. Gait disturbance, dysuria, and impotence developed early January 1996. He was not able to walk and developed bilateral blurred vision and ocular pain mid-February 1996. Ophthalmologic examination showed anterior uveitis and retinal detachments. Physical examination was normal. Neurologic examination indicated spastic paraplegia and loss of all sensation to the level of T9. He did not have deafness, meningeal signs, or cerebellar ataxia. Brain and spinal cord MRI were normal. CSF study revealed only mild lymphocytosis (16/mm3). We diagnosed uveitis and transverse myelitis associated with VKH. Oral prednisolone therapy (100 mg/d) for 1 week did not improve his neurologic signs and caused liver dysfunction. Lymphocytestimulating test using prednisolone was abnormal, suggesting prednisolone-induced liver dysfunction. We changed to oral betamethasone treatment (20 mg/d, corresponding to 133.3 mg of prednisone). From 1 week after betamethasone administration, many of the ocular signs, the paraplegia, and dysesthesia in the legs had markedly improved. Betamethasone was slowly tapered.

Síndrome de Vogt-Koyanagi-Harada e o seu acometimento multissistêmico

“…Patients may exhibit resistance to corticosteroid therapy with prednisone and/or prednisolone and may suffer liver dysfunction induced by the corticosteroids. In these cases there is the option of using 20mg/day of betamethasone or 30mg/day of dexamethasone 56 . The efficacy of different routes of corticosteroid administration has been studied, but there was no evidence in the prognostic results comparing oral and intravenous routes 54 .…”

Short Report

“…Neuropsychological deficits were always of short duration and stopped either spontaneously or due to corticosteroids. Brain CTs are usually normal in VKHS, but brain MRIs may show widespread lesions extending from the cortex and the white matter to the brainstem [4,8,10]. Contrary to CT findings, MRI lesions did not show any dynamics.…”

“…Ophthalmologic investigation showed irregular pupils, normal fundi and normal fluorescein angiography. After a second lumbar puncture on the 7th hospital day (table 1), uveomeningoencephalitis was suspected and prednisolone was begun [4]. The confusional state improved to normal within a few days.…”

Chronic and recurrent meningitis

“…There is evidence that the dose of steroids needs to be very high28 in order to be effective for neurological complications.…”