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Adult-Onset Leukoencephalopathy with Calcifications and Cysts: Focusing on Hemorrhagic Propensity and Cysts
Zhaojian Li1, Kun Han1, Weicheng Yao1
1Department of Neurosurgery, Affiliated Hospital of Qingdao University, Qingdao City, Shandong Province, China.
Background:
An uncommon disorder, adult-onset leukoencephalopathy with calcifications and cysts (ALCC) has been recognized clinically for approximately a decade. Its typical radiologic signs and pathologic characteristics have been investigated thoroughly and described fully in a series of cases. However, little attention has focused on the propensity of hemorrhage in this entity, and the etiology of cyst occurrence in ALLC remains uncertain. To the best of our knowledge, there is a lack of relevant articles addressing the relationship between hemorrhage and cyst development in ALCC.
Case Description:
A 30-year-old woman presented with headache, diminishing eyesight, and face numbness over the course of 16 months. Repeat radiologic examination showed the formation of a new cyst and the enlargement of former cyst after hemorrhage. She was diagnosed formerly with ALCC with the triad of leukoencephalopathy, calcifications, and cyst in imaging. Staging gross total resections of cyst were achieved with neurologic improvement postoperatively. Histologic examination revealed angiomatous vessels, Rosenthal fiber formation, microcalcification, and deposits of hemosiderin, and ALCC was confirmed pathologically.
Conclusions:
After analyzing the clinical data about the hemorrhage and cysts in our case and all 15 reported ALCC cases in the literature, we conclude that intermittent hemorrhage and cysts development are 2 outstanding features for ALCC and that hemorrhage is a probable mechanism for the formation and expansion of cyst.
