大脳両半球に広汎な萎縮, 硬化をきたしたウイリス動脈管のIntimal Fibromuscular Dysplasiaの幼児剖検例 : 特発性ウイリス動脈輪閉塞症の成因をめぐって

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  • An autopsy case of an infant with stenosis of the arteries in the circle of Willis due to intimal fibromuscular dysplasia : In relation to the etiology of spontaneous occlusion of Willis circle in "moyamoya" disease
  • ダイノウ リョウ ハンキュウ ニ コウハン ナ イシュク コウカ オ キタシタ

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An autopsy case of a 17 month-old male infant with stenosis of the arteries of the circle of Willis, due to intimal fibromuscular dysplasia, was presented. The child's suffering began with a sudden onset of clonic spasm of the face, fingers and limbs subsequently followed by hemiparesis at the left side 7 months after birth. Carotid angiograms disclosed stenosis of bilateral internal carotid arteries at the siphon portion and the cervical internal carotid artery to the left. No suggestion of the existence of cerebral arterial rete was seen. Symmetrical enlargement of the lateral ventricles was noted on PEG. Cerebrospinal fluid gave no abnormal values in cytological and biochemical examinations. Spastic episodes disappeared largely with antispastic drugs, but left hemiparesis persisted. One day prior to death, the patient's temperature rose remarkably and following general convulsion the patient died. The direct cause of death was acute purulent meningitis. Clinical examination detected coli bacilli septicemia. Autopsy revealed that besides meningitis purulenta, sclerosis of the brain was found in the parietotemporal region of both hemispheres which extended into the precentral and frontal gyri, but was found to be more diffuse and intense in distribution and degree at the right side, including entire involvement of the occipital lobe. Meningeal blood vessels were anemic in the sclerosing area, but otherwise intensively hyperemic, which was suggestive of constriction of blood vessels entering into the sclerosed brain. Microscopically laminar or pseudolaminar necrosis of the cortex and demyelination and gliosis of the white matter were observed, whereas the basal ganglions were spared somewhat, only showing a slight degeneration. The internal capsule, crus cerebri and the corticopontine and corticospinal tracts were blanched. The bilateral distal portions of the internal carotid arteries, the bilateral middle and posterior cerebral arteries at the circle, and the hasilar artery were narrowed in decreasing order of intensity of intimal cushions or pads which consisted of proliferation of smooth muscle cells each encircled with collageneous and reticular fibers. Duplication and fragmentation of elastic fibers and the acidmucopolysaccharide metamorphosis of subendothelial ground substances were also apparent. A few fat granules and no disruption of internal elastic lamina were observed. The intimal thickening was considered to be caused by abnormal growth of cushions themseves which are found even in normal fetuses, infants and juveniles at the branching sites of the middlesized arteries such as the distal portion of the internal carotid artery, the middle cerebral artery and so on. This histological consideration might give a key to clarify the morphogenesis of spontaneous stenosis or occlusion of the arteries in the circle of Willis in “moyamoya” disease. The cause of the sclerotic lesion of the brain was attributable to the stenosis of the arteries in the distal portion of the internal carotid ateries and in the circle of Willis mentioned above, but other factors, for example, spastic constriction of blood vessels may also contribute to the induction of the widespread lesion of the right hemisphere. The brain sclerosis might be related partly to spasm of blood vessels.

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