Leukoencephalopathy with cerebral calcifications and cysts: clinical and pathological features in two adults

Two adult patients diagnosed with Leukoencephalopathy with cerebral calcifications and cysts (LCC) were presented. Both patients had a long-term (8-10 years) following-up. Radiological findings of both patients revealed the characteristic signs of LCC: cerebral white matter abnormalities, calcifications, and cysts. In case 1, the initial CT scan showed a low-density area in the right frontal lobe and it had developed into a large cystic lesion after 8 years. Histopathological determination revealed that the cyst wall was associated with hemorrhage, angiomatous formation, and some Rosenthal fibers. In case 2, a major cystic lesion was located at the left parietal lobe which was resected and an old hematoma was found inside the cyst. Nine years later, the follow-up neuroimaging of case 2 showed a remarkable improvement of white matter abnormalities and cystic lesions. Hemorrhagic fluid was observed inside the cysts. Additionally, follow-up CT and MR scans showed a rapid enlargement of cystic lesions accompanied with hemorrhagic fluid levels after a year. Then, a major cyst was surgically removed to relieve pressure symptoms. Pathology of the resected cyst exhibited an organized hemorrhage inside the cyst and a large amount of hemosiderin surrounding the cyst wall. In conclusion, our two cases demonstrated that angiomatous changes subsequent with hemorrhage may be the major mechanism of cyst formation and development.