Hiraki Y, Kuwasaki N, Shoji H, Kaji M, Kuboshiro T
Rinsho Shinkeigaku. 1989 Jan;29(1):54-8.
Two cases of Vogt-Koyanagi-Harada disease with rare neurological complications were reported, which are one case with vestibular and cerebellar ataxia, and one with multiple cranial nerve palsies. The first case, a 32-year-old man, was admitted to our ophthalmologic department because of visual impairment. Two weeks later, he complained of gait disturbance. Limb and truncal ataxia, vestibular dysfunction and perceptive deafness were observed. Ophthalmologic examination revealed typical findings of Vogt-Koyanagi-Harada disease. CSF showed elevated protein and mononuclear pleocytosis. After treatment with oral steroid therapy, vestibular and cerebellar ataxia, and visual impairment improved. The second case, a 22-years-old man, showed the VIIth, VIIIth, IXth, and Xth cranial nerve palsies with visual impairment, and he was diagnosed to have cranial nerve palsies associated with Vogt-Koyanagi-Harada disease. After steroid therapy, there was a marked clinical improvement. Vogt-Koyanagi-Harada disease is an autoimmune disease against the melanocytes and involving tissues containing melanocytes such as uvea, meningea, skin and inner ear, etc.. Neurological complications except for meningitis and perceptive deafness have been rarely reported in Japan. Related to the present cases, possible pathogenesis was discussed.
报告了2例伴有罕见神经并发症的Vogt-小柳-原田病，其中1例伴有前庭和小脑共济失调，另1例伴有多发性颅神经麻痹。第一例为一名32岁男性，因视力障碍入住我院眼科。两周后，他主诉步态不稳。观察到肢体和躯干共济失调、前庭功能障碍和感音神经性耳聋。眼科检查发现了Vogt-小柳-原田病的典型表现。脑脊液显示蛋白升高和单核细胞增多。经口服类固醇治疗后，前庭和小脑共济失调以及视力障碍有所改善。第二例为一名22岁男性，表现为第VII、VIII、IX和X颅神经麻痹并伴有视力障碍，他被诊断为与Vogt-小柳-原田病相关的颅神经麻痹。类固醇治疗后，临床症状有明显改善。Vogt-小柳-原田病是一种针对黑素细胞的自身免疫性疾病，累及含有黑素细胞的组织，如葡萄膜、脑膜、皮肤和内耳等。在日本，除脑膜炎和感音神经性耳聋外，神经并发症鲜有报道。结合本病例，对可能的发病机制进行了讨论。
