Abstract：
BACKGROUND: Labrune syndrome is a rare white matter disease characterized by angiomatous leukoencephalopathy, diffuse intracranial calcifications and supratentorial and infratentorial parenchymal cysts. The clinical worsening is often related to cyst expansion, and surgery may be advocated for symptomatic management in about one third of cases. However, no consensus exists on the surgical timing, the most effective procedure, and the long-term results.
METHODS: Electronic databases PubMed/MEDLINE and Google Scholar were searched for studies published up to April 2022 using the search string (Labrune syndrome OR leukoencephalopathy with calcifications and cysts OR brain calcifications OR brain cysts) AND (therapy OR surgery).
RESULTS: We found 28 studies in the literature, and we added a new case from our institution, comprising 37 patients. All the patients in this series underwent surgical intervention. We reviewed all the pertinent literature to discuss clinical-radiological features and etiopathogenesis, specifically addressing the surgical options, clinical results, and prognosis.
CONCLUSIONS: Leukoencephalopathy with cerebral calcifications and cysts is a rare neurodegenerative disorder for which effective medical treatment is lacking. Surgery remains the only therapeutic option to control the disease to reduce the mass effect of growing cystic lesions. Almost half of the patients who underwent surgery required further approaches, with great concern for the associated disabilities. Several procedures have been described, with no evidence regarding which procedure is the most effective. Individual-based surgical planning must be advocated, tailoring the approach to limit side effects. Mini-invasive neuroendoscopic approaches may be considered to achieve satisfactory results.
METHODS: Electronic databases PubMed/MEDLINE and Google Scholar were searched for studies published up to April 2022 using the search string (Labrune syndrome OR leukoencephalopathy with calcifications and cysts OR brain calcifications OR brain cysts) AND (therapy OR surgery).
RESULTS: We found 28 studies in the literature, and we added a new case from our institution, comprising 37 patients. All the patients in this series underwent surgical intervention. We reviewed all the pertinent literature to discuss clinical-radiological features and etiopathogenesis, specifically addressing the surgical options, clinical results, and prognosis.
CONCLUSIONS: Leukoencephalopathy with cerebral calcifications and cysts is a rare neurodegenerative disorder for which effective medical treatment is lacking. Surgery remains the only therapeutic option to control the disease to reduce the mass effect of growing cystic lesions. Almost half of the patients who underwent surgery required further approaches, with great concern for the associated disabilities. Several procedures have been described, with no evidence regarding which procedure is the most effective. Individual-based surgical planning must be advocated, tailoring the approach to limit side effects. Mini-invasive neuroendoscopic approaches may be considered to achieve satisfactory results.
摘要：
目的：Labrune综合征是一种罕见的以血管瘤性白质脑病为特征的白质疾病，弥漫性颅内钙化和幕上和幕下实质囊肿。临床恶化通常与囊肿扩张有关，在约三分之一的病例中，可能主张手术进行对症处理。然而,在手术时机上没有共识，最有效的程序,和长期结果。
方法：电子数据库，如PubMed/MEDLINE和谷歌学者，对2022年4月发表的研究进行了分析，使用搜索词“Labrune综合征或白质脑病伴钙化和囊肿或脑钙化或脑囊肿和治疗或手术结果：我们发现文献中报道了28项研究，我们增加了一个我们在研究所治疗的新病例，一系列37名患者。本系列所有患者均接受了手术干预。我们旨在回顾所有相关文献，以讨论临床放射学特征和病因病因学见解，解决手术选择的具体问题，临床结果和预后。
结论：LCC是一种罕见的神经退行性疾病，没有有效的药物治疗。手术仍然是控制疾病以减少不断增长的囊性病变的质量影响的唯一治疗选择。几乎一半接受手术的患者需要进一步的方法，非常关注相关的残疾。已经描述了几个程序，没有最有效的证据。必须提倡以个人为基础的手术计划，调整限制副作用的方法。可以考虑采用微创神经内镜方法来获得令人满意的结果。
方法：电子数据库，如PubMed/MEDLINE和谷歌学者，对2022年4月发表的研究进行了分析，使用搜索词“Labrune综合征或白质脑病伴钙化和囊肿或脑钙化或脑囊肿和治疗或手术结果：我们发现文献中报道了28项研究，我们增加了一个我们在研究所治疗的新病例，一系列37名患者。本系列所有患者均接受了手术干预。我们旨在回顾所有相关文献，以讨论临床放射学特征和病因病因学见解，解决手术选择的具体问题，临床结果和预后。
结论：LCC是一种罕见的神经退行性疾病，没有有效的药物治疗。手术仍然是控制疾病以减少不断增长的囊性病变的质量影响的唯一治疗选择。几乎一半接受手术的患者需要进一步的方法，非常关注相关的残疾。已经描述了几个程序，没有最有效的证据。必须提倡以个人为基础的手术计划，调整限制副作用的方法。可以考虑采用微创神经内镜方法来获得令人满意的结果。
