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Computed tomography findings of severe mineralizing microangiopathy in the brain
Abstract:
The intracranial CT findings of mineralizing microangiopathy, a rare condition, are described in seven patients. The underlying pathology was posterior fossa medulloblastoma (four patients), cerebelloastrocytoma (two patients), and acute lymphatic leukemia (one patient). Each of the seven patients was less than 10 years old. Dystrophic calcification was present in the corticomedullary junction, lentiform nucleus of the basal ganglia, corticomedullary junction, and dentate nucleus of the cerebellum. Three of the patients had subtle neurologic signs related to the mineralizing microangiopathy, but none died of central nervous system disease. Although three factors--radiation therapy, chemotherapy, and increased intracranial pressure--probably have a synergistic role in the pathogenesis, radiation is believed the dominant factor. The minimum dose required to induce this damage is 2000 rad.
Insights
Mineralizing microangiopathy, a rare condition, presents with intracranial calcifications in children with brain tumors or leukemia. Radiation therapy is the likely dominant cause of this CT finding.
Area of Science:
- Neuroradiology
- Pediatric Oncology
- Neuropathology
Background:
- Mineralizing microangiopathy is a rare condition affecting the brain's vasculature.
- It is often associated with specific pediatric cancers and their treatments.
Purpose of the Study:
- To describe the intracranial CT findings in pediatric patients with mineralizing microangiopathy.
- To identify associated pathologies and potential causative factors.
Main Methods:
- Retrospective review of intracranial CT scans from seven pediatric patients.
- Correlation of imaging findings with underlying diagnoses and treatment history.
Main Results:
- CT revealed dystrophic calcifications in specific brain regions (corticomedullary junction, basal ganglia, cerebellum).
- Underlying pathologies included medulloblastoma, cerebelloastrocytoma, and acute lymphatic leukemia.
- Three patients exhibited subtle neurological signs; none died from CNS disease.
Conclusions:
- Mineralizing microangiopathy in children is linked to posterior fossa tumors and leukemia.
- Radiation therapy, chemotherapy, and increased intracranial pressure likely play a synergistic role, with radiation being dominant.
- A minimum radiation dose of 2000 rad may induce this condition.