L-2-hydroxyglutaric aciduria and brain malignant tumors: a predisposing condition?
case_series · Level IV
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Abstract
L-2-hydroxyglutaric aciduria is a rare metabolic encephalopathy displaying a subcortical leukoencephalopathy on MRI. Diagnosis rests on detection of an abnormal accumulation of L-2-hydroxyglutaric acid in body fluids. The authors report on four patients who developed a malignant brain tumor during the course of the disease. This association points to a possible role of L-2-hydroxyglutaric aciduria in predisposing to brain tumorigenesis.
Medical subject headings
- Astrocytoma
- Brain Diseases, Metabolic
- Brain Neoplasms
- Frontal Lobe
- Glioblastoma
- Glutarates
- Neuroectodermal Tumors, Primitive