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伴钙化与囊变的脑白质病(LCC)是一种少见的综合征,于1996年由Labrune等人首次报道。确诊LCC需要进行病理学检查,多数学者认为微血管病变是LCC的原发性病理改变,但也有人认为LCC可能是几种不同病理机制共同作用的结果。其典型的影像学特征包括大脑深部核团和白质的钙化、广泛的脑白质变性和多发脑内囊肿。但是相对于影像学的广泛病变而言,LCC患者的临床症状比较隐匿,且表现多样,主要与病变血管和囊肿累及的部位有关,治疗上主要是通过手术切除、分流等方法解除颅内囊肿对脑组织的压迫。由于LCC的临床表现及影像学特征与Coats plus综合征相似,因此Linnankivi等认为两者属于同一种疾病,将之命名为伴钙化和囊变的脑视网膜微血管病(CRMCC),但是,由于此类疾病的发病机制尚未完全阐明,因此在诊断及分型上仍存在争议。
Leukemia associated with calcification and cystic degeneration (LCC) is a rare syndrome that was first reported by Labrune et al in 1996. Confirmed LCC requires pathological examination, most scholars believe that microvascular disease is a primary pathological changes of LCC, but some people think that LCC may be the result of several different pathological mechanisms together. Typical imaging features include calcification of deep nuclei and white matter in the brain, extensive white matter degeneration and multiple intracranial cysts. However, relative to the extensive lesions of the imaging, the clinical symptoms of LCC patients are relatively obscure and have various manifestations, which are mainly related to the sites involved in the diseased vessels and cysts. The treatment is mainly to remove the intracranial cyst by means of surgical resection and shunt The oppression of brain tissue. Since the clinical and imaging features of LCC are similar to those of Coats plus syndrome, Linnankivi et al. (1999) consider both of them to belong to the same disease and name them retinocytic microangiopathy with calcification and cystic degeneration (CRMCC). However, The pathogenesis of the disease has not been fully elucidated, so there is still controversy in the diagnosis and typing.