Article
The Effect of Interleukin-1 Antagonists on Brain Volume and Cognitive Function in Two Patients With Megalencephalic Leukoencephalopathy With Subcortical Cysts.
Pediatric neurology - 1 Jul 2023
Sönmez Hafize Emine, Savaş Merve, Aliyeva Bülbül, Deniz Adnan, Güngör Mesut, Anık Yonca, Kara Bülent
Abstract excerpt
BACKGROUND: Megalencephalic leukoencephalopathy with subcortical cysts (MLC) is a rare leukodystrophy characterized by early-onset macrocephaly and progressive white matter vacuolation. The MLC1 protein plays a role in astrocyte activation during neuroinflammation and regulates volume decrease following astrocyte osmotic swelling. Loss of MLC1 function activates interleukin (IL)-1β-induced inflammatory signals....
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