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A 55-year-old female with leukoencephalopathy with cerebral calcifications and cysts: Case report and radiopathologic
Jorge Novo1, Diana Lin1, Megan Shanks2
1Rush University Medical Center, Department of Pathology, 1653 West Congress Parkway, 570 Jelke, Chicago, IL 60612, USA.
Insights
This case study details Leukoencephalopathy with cerebral Calcifications and Cysts (LCC), a rare adult-onset neurological disorder. Histological findings reveal unique mineralization patterns, aiding in diagnosis.
Area of Science:
- Neurology
- Pathology
- Radiology
Background:
- Adult-onset leukoencephalopathies pose diagnostic challenges.
- Leukoencephalopathy with cerebral Calcifications and Cysts (LCC) is a rare condition.
Purpose of the Study:
- To present a case of adult-onset LCC.
- To discuss the diagnostic features of LCC.
Main Methods:
- Case presentation of a 55-year-old woman.
- Radiological imaging (CT and MRI).
- Histopathological analysis of brain biopsy.
Main Results:
- CT and MRI revealed white matter lesions, calcifications, and cystic changes.
- Biopsy showed geode-like mineralization, calcospherites, sclerosis, Rosenthal fibers, and vascular abnormalities.
- Differential diagnoses included infection, thromboembolic disease, and neoplasm.
Conclusions:
- LCC presents with characteristic radiologic and histologic findings.
- Accurate diagnosis requires integration of clinical, imaging, and pathological data.
- Understanding LCC's features aids in differentiating it from other leukoencephalopathies.
Abstract:
Adult-onset leukoencephalopathies with increased cerebral volume can present a potentially challenging diagnosis for the pathologist. We present the case of a patient with a rare adult-onset disease called Leukoencephalopathy with cerebral Calcifications and Cysts (LCC). A 55-year-old woman with a history of morning headaches, mild memory loss, diabetes, and hypertension presented to the emergency department with acute onset altered mental status. CT scan revealed multiple small hypodense lesions in the white matter with calcifications in the bilateral cerebral hemispheres, basal ganglia, pons, and cerebellar hemispheres. MRI showed multiple complex/hemorrhagic cystic lesions with partial enhancement in addition to calcifications bilaterally in the frontotemporal white matter, pons, and cerebellar hemispheres, and diffuse white matter signal abnormality. The differential diagnosis included chronic infection, chronic thromboembolic disease, and neoplasm. The biopsy revealed extensive geode-like mineralization as well as smaller calcifications (calcospherites) with associated sclerosis, Rosenthal fibers, angiomatous proliferation of blood vessels with thrombosis and microbleeds. We discuss the differential diagnosis, radiologic and detailed histologic features of LCC.
