Infantile Opsoclonus-Polymyoclonus Syndrome Associated with Presacral Ganglioneuroma: Report of a Case

神經性腫瘤(neurogenic tumors)併肌－眼障癴(opsomyoclonus)乃是一罕見卻著名的疾病。其中常被報告的神經性腫瘤爲神經母細胞瘤(neuro blasloma)。本文報告一例極爲罕見的神經節細胞瘤(ganglioneusoma)併肌－眼陣癴。患者爲兩歲男孩，主訴自十三個月大時開始出現軀幹運動失調，眼球及手足末端陣癴等症狀。經全身電腦斷層掃瞄發現骶骨前腫瘤後開刀取出，病理檢查証實爲一神經節細胞瘤。術後投以ACTH治療，可控制其神經學症狀，然減藥後症狀仍會出現，惟腫瘤迄今未再發。此症致病機轉至今未明，文獻提及之假說包括病毒，腫瘤分泌物及自體免疫機轉等。此症之診斷並不困難，端視懷疑與否。特提出此一病例，希望引起大家對此病的重視。

關鍵字

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並列摘要

Neurogenic tumor associated with infantile opsoclonus-polymyoclonus syndrome (IOPS) (synonyms: Kinsbourne encephalopathy, opsomyoclonus, infantile myoclonic encehalopathy) is quite a well-known disease. However, it has been infrequently seen in this country. The most common associated neurogenic tumor reported in the literature was neuroblastoma. The authors described a 2 year-old boy presented with truncal ataxia, myoclonic jerks and opsoclonus since 13 month old who was demonstrated to have a presacral tumor by whole body CT scan. The pathological examination proved it to be a ganglioneuroma. Though the exact pathogenesis of this diseease has been controversial, adrenal corticosteroid and ACTH have been shown to be useful to control the neurological symptoms.

並列關鍵字

infantile opsoclonus-polymyoclonus syndrome ； opsomyoclonus ； IOPS ； ganglioneuroma

國際替代計量

Infantile Opsoclonus-Polymyoclonus Syndrome Associated with Presacral Ganglioneuroma: Report of a Case

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