先天性胼胝體缺損：病例報告及文獻回顧

胼胝體的主要功能為聯繫兩側大腦半球。胼胝體的後天缺損常造成患者左側失用症（left apraxia）及左側失讀症（left alexia）等右大腦無法取得左大腦資訊的症狀；然而先天性胼胝體完全或部分缺損（complete or incomplete congenital corpus callosum agenesis）卻不見得會有這些症狀，相對的，它伴隨其他症候群的機會則很大。曾被提及伴隨先天性胼胝體缺損的肢體殘障包括高張型或低張型、對稱性四肢或兩下肢麻痺；智障及癲癇亦常見。本文報告一先天性胼胝體缺損併右側肢體攣縮型麻痺的病例，並回顧先天性胼胝體缺損的發生原因。文獻指出，胚胎早期神經管前端的發育異常使胼肢體發育缺損，可以造成多樣的症候群，但神經成熟皆段的功能可塑性則可能代償可能發生的各種功能缺損，包括左側失用症、左側失讀症及各種發展遲緩。相較於曾被報導的對稱型肢體麻痺，本例只有右側肢體有攣縮型麻痺；患者可以在復健治療、特殊教育下，於學齡回歸普通班，也證明了早期療育在這類兒童的重要性。

關鍵字

先天性胼胝體缺損；左側失用症；左側失讀症

並列摘要

The main function of corpus callosum is to connect bilateral hemispheres. Patients with mech-anical injuries to corpus callosum, e,g., tumor compression, anterior or posterior cerebral artery infarction may have in neurological defects including left apraxia, left alexia or anomia to colors. Nevertheless, these symptoms may not occur in congenital corpus callosum agenesis, complete or incomplete. Congenital corpus callosum agenesis frequently coexists with other deficits, such as mental retardation, spastic quadriplegia or spastic diplegia, and seizure. We report a case with corpus callosum agenesis, spastic right hemiplegia, mild bilateral eyelid ptosis and hyperoptic astigmatism. Literatures were reviewed. In early development of embryo, deficit of neural tube often result in various types of congenital syndromes. Since there is considerable functional plasticity of brain during neural maturation, early detection, rehabilitation and special education programs are very important for the functional outcome in patients with congenital corpus callosum agenesis. Our case represented a good example.

並列關鍵字

congenital corpus callosum agenesis ； left apraxia ； left alexia

國際替代計量

先天性胼胝體缺損：病例報告及文獻回顧

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