Meningioangiomatosisの1乳児例

An Infant of Meningioangiomatosis

I．はじめに

　Meningioangiomatosisは，Worster-Droughtら^15）に提唱されたhamartomaの性格の強い病態であり，その大部分がvon Reckhnghausen病に合併する疾患である．von Recklinghausen病に合併しないmeningioangioma—tosisの症f列はKasantikulら²¹⁾の報告にはじまるが，現在まで11例が報告されている．われわれは，von Reck—linghausen病に合併していないと考えられるMeningio—angiomatosisの乳児例を経験したので神経放射線学的特徴及び病態に関して，若干の文献的考察を加えて報告する．

Abstract

We report here a rare case of meningioangiomatosis in an infant, not associated with von Recklinghausen's disease.

A 14-month-old female was admitted because of sei-zures. Neurological findings on admission were normal. Computed tomography showed a slightly high densitymass with marked contrast enhancement in the left temporoparietal lobe. Magnetic resonance image (MRI) revealed a sightly hypointensive lesion surrounded by an isointensive band on T1-weighted image, and a hy-perintensive lesion surrounded by a slightly hypointen-sive band on T2-weighted image. Brain edema was shown to a certain extent around the lesion on MRI. Left carotid angiography demonstrated a slightly up-ward shift of the left middle cerebral artery, but no abnormal vascularity was shown.

A temporoparietal craniotomy was performed. A yel-lowish red, elastic soft tumor was observed in the left temporal lobe. The tumor resembled hyperemic hyper-trophic gyri and was well demarcated. Total removal of the tumor was performed.

Pathological diagnosis was meningioangiomatosis. The patient is still doing well 3 years and 5 months after the operation. There was no evidence of recur-rence on computed tomography at the 3-year follow up. She didn't have any stigmata, such as cafe au lait spots or neurofibromas suggesting von Recklinghausen's dis-ease.