Leukoencephalopathy with Cerebral Calcifications and Cysts: The Neurosurgical Perspective. Literature Review

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.

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).

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.

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.