Leukoencephalopathy with cerebral calcifications and cysts: 2 cases report / 中华神经科杂志

ABSTRACT

Objective To explore the features of leukoencephalopathy with cerebral calcifications and cysts (LCC) in clinic, radiology and pathology in order to improve skills in diagnosis. Methods Two female patients had CT and/or MRI scan, and case 2 had contrast enhancement MRI scan additionally. Both cases had blood biochemistry examinations including calcium, phosphorus and alkaline phosphatase et al. Case 2 had lumbar puncture and cerebrospinal fluid test. The major cystic lesion was surgically removed in both patients and offered a histopathological examination. Results CT scan reveaed diffuse calcifications in the bilateral basal ganglia, white matter of frontal lobe and/or dentate nuclei of cerebellum in both cases, and major cystic lesion in right frontal lobe (case 1) and the left parietal lobe (case 2). The rim of enhancement was observed in cystic lesion on MRI. Histopatbological examination revealed angiomatous rearrangements of the microvessels with fibroid, hyaline degeneration and haemosiderin deposits, brain tissue associated with areas of demyelinization, some Rosenthal fibers, gliosis, calcium deposits and hemorrhage, fibrinoid necrosis occurs in partial vessels associated with thrombogenesis and stenosis as changes in arteriolitis. Blood biochemistry examination showed normal. Cerebrospinal fluid test in case 2 showed increased intracranial pressure(350 mm H_2O,I mm H_2O =0. 0098 kPa). Conclusions The onset of LCC varies and occurs from early infancy to adult. The asymmetrical calcification is characteristic in LCC. Hemorrhage could be involved in the pathogenesis of cystic formation. LCC is characterized by a cerebral obliterative microangiopathy, both demyelinization and the edematous changes could probably result in white matter abnormalities on neuroimaging.