Idiopathic Hypertrophic Pachymeningitis Manifesting with Frontal Brain Edema
DOI:
https://doi.org/10.34024/rnc.2011.v19.8359Keywords:
Brain Edema, Hypertrophic Pachymeningits, Idiopathic, TreatmentAbstract
Introduction. Hypertrophic pachymeningitis (HP) is a rare inflammatory disease that results in thickening of the dura mater. Atypically, it can progress to include the involvement of the cerebral parenchyma. Method. In this paper, we report the rare case of a 31-year-old man with a three-week history of headaches, seizures, impairments of cognitive function, and changes in behavior and mood. Magnetic resonance imaging (MRI) of the brain showed a thickening and an abnormal enhancement of the dura mater over the falx cerebri with extensions into the adjacent cranial base and with brain edemas in the frontal lobes. Histopathological study of meningeal and brain biopsies showed an inflammatory process that was compatible with HP. The results of an extensive laboratory investigation were unremarkable and did not provide additional information on the cause of the meningeal disease. The patient exhibited relapses despite immunosuppressive therapy. Conclusion. This case shows the challenges associated with the management of the disease and the importance of early diagnosis to avoid worsening of the condition and cerebral damage.
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