Teaching NeuroImages: Adult-onset leukoencephalopathy with intracranial calcifications and cysts (Labrune syndrome).

A 42-year-old woman was admitted for a complex partial seizure. At age 18 years, she had transient diplopia, which was considered a simple strabismus. Current examination revealed a left VI cranial nerve palsy and right arm hyperreflexia. Neuroimaging showed multiple intraparenchymal cystic lesions in white matter (figure), multiple calcifications in basal ganglia, and extensive bilateral leukoencephalopathy. Lack of extraneurologic involvement and genetic testing (CTC1 gene) ruled out other disorders (mainly Coats plus syndrome). Leukoencephalopathy with intracranial calcifications and cysts is considered a diffuse microangiopathy affecting children. Adult-onset patients may present a heterogeneous and milder clinical picture, with slow progression.