付格特-小柳-原田综合征及其中枢神经系统的表现

付格特-小柳-原田综合征(VKHS)是一种主要累及视觉、听觉、脑膜和皮肤的自身免疫性炎性疾病。其靶目标是选择性侵犯抗原相关黑色素细胞。一般而言,VKHS的症状以听觉和神经系统的症状为多见,但多继发于视乳头水肿和视网膜剥离等所致的视力下降之后。VKHS患者神经系统的表现可与急性脑膜脑炎相似,脑脊液亦可表现为多形核白细胞的增多,故需与感染性脑膜脑炎相鉴别。总之,VKHS是一个累及多个系统的自身免疫疾病。发病机制未明,临床表现多样,漏诊、误诊率高。其中枢神经系统表现可与脑膜炎相似。临床上,当患者出现急性视物不清、耳鸣、感觉神经性耳聋、白癫风、秃发和脑膜炎等症状和(或)体征时,应考虑到VKHS的可能。并尽早行大剂量激素冲击治疗以缩短病程、改善预后和减少并发症的发生。

Vogt-Koyanagi-Harada''''s Syndrome and Its Manifestation in Central Nervous System

Vogt-Koyanagi-Harada's Syndrome (VKHS) is an autoimmune inflammatory disease mainly affecting the visual and auditory system, meninges, and skin. The target of the attack seems to be antigens associated with melanocytes. However, the mechanism that triggers this attack is unknown. In general, the initial symptoms of VKHS are auditory and neurological symptoms, such as meningitis, followed by the onset of decreased vision along with the finding of papilledema and retinal detachment.VKHS with a neurological manifestation is similar to acute meningoencephalitis with polymorphonuclear pleocytosis in the cerebrospinal fluid (CSF). It should be considered as a differential diagnosis from infectious meningoencephalitis. In summary, VKHS is a systemic autoimmune disease involving various systems. The etiologic and pathogenic factors remain unclear. The clinical manifestations are variable and the misdiagnosis rate is high. Clinically, when the patient complains of acute blurring of vision, tinnitus,sensorineural hearing loss, vertigo, alopecia and meningitis etc, neurologist should be aware of the possible VKHS. Prompt initiation of high dose systemic corticosteroids pulse therapy is effectively to shorten the course, to improve prognosis and diminish the complications.