Case report: Clinical and neuroradiological longitudinal follow-up in Leukoencephalopathy with Calcifications and Cysts during treatment with bevacizumab

Leukoencephalopathy with Calcifications and Cysts (LCC) is a rare genetic microangiopathy exclusively affecting the central nervous system caused by biallelic mutations in SNORD118 . Brain magnetic resonance imaging (MRI) often diagnostic due to highly characteristic triad of leukoencephalopathy, intracranial calcifications, brain cysts. Age at onset, presentation disease evolution can all vary, ranging from pauci-symptomatic rapid signs loss motor cognitive abilities. No specific therapies for LCC are currently licensed. According literature, bevacizumab might represent an effective modality improve clinical MRI features disease. However, uncertainty remains as true efficacy this approach, when begin therapy, appropriate dosing, consequences drug withdrawal. CARE guidelines, we describe long-term neuro-radiological follow-up 10-year-old child LCC. We report following repeated cycles treatment bevacizumab. Our case suggests that effectively modify progression, possibly indicating time-dependent effect.