Hypertrophic cranial pachymeningitis in a patient with aplastic anemia

We report on a 13-year old girl with severe aplastic anemia and hypertrophic cranial pachymeningitis. She was admitted to our hospital with severe headache and vomiting. A computerized tomographic (CT) scan of the brain on the third day of symptoms showed a hyperdense area in the tentorial region. Magnetic resonance imaging (MRI) showed iso-intensity in the same tentorial region in T-1- and T-2-weighted images, and gadolinium enhancement of this region suggested a thickened dura mater. Initially, a diagnosis of subdural or subarachnoid hemorrhage was made. Since her platelet count was low (3000/mu l) making the patient a poor-risk candidate for surgery, and the area was limited to the dura mater, conservative therapy, including glycerol administration and platelet transfusion, was carried out, Despite clinical improvement 10 days after admission without specific therapy, the iso-intense region on the left side of the tentorial region remained unchanged on MRI, On the other hand, the iso-intense area on the right side of the tentorial region became hyperdense on T-1-weighted MRI images and was also enhanced by gadolinium. Cerebrospinal fluid findings were normal except for slightly elevated protein at 62 mg/dl. A diagnosis of hypertrophic cranial pachymeningitis of the tentorial dura mater with hemorrhage on the right side was made. Although hypertrophic cranial pachymeningitis is a rare disease, it must be considered in the differential diagnosis of severe headache in a case of aplastic anemia.