The origin of intracranial fibrosarcoma.
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Biomedical subjects
Publications and source records attributed to R Okeda.
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Twins with high-risk globoid cell leukodystrophy (GLD) at the 6th gestational month were studied morphologically and enzymatically. One of them was affected and the other was probably the carrier of this disease. Central nervous system and other viscera developed normally for the gestational age in both fetuses. The lesions of GLD were found in the fetus with marked deficiency of galactocerebrosidase, but not in the probable carrier. The lesions were located only in the considerably myelinated areas viz., the brain stem and the spinal cord. Ultrastructurally, the globoid cells in the fetal GLD seemed to originate from glial cells, probably oligodendroglia.
Two cases of cryptic vascular malformation that were not demonstrated by cerebral angiography were detected by computerized tomography. One of these patients had a cavernous angioma in the fourth ventricle with recurrent subarachnoid hemorrhages, and the other harbored a small arteriovenous malformation and intracerebral hematoma. The usefulness and limitations of computerized tomography in the identification of cryptic vascular malformations are discussed.
Two cases of extracranial heterotopia of the brain are reported. Case 1, in an otherwise normal female baby, had a brain heterotopia in the submandibular region. Case 2, in a still-born female baby with anencephaly, had the heterotopia in the lung. The lesion in case 1 is believed to be an encephalocele that has been pinched off through a cleft in the bone or through a foramen of the skull; the pathogenesis is similar to that of the so-called "nasal glioma". Concerning the pathogenesis of case 2, contrary to the generally supported embolism theory, observation of serial sections of the tumor in the lung favors the aspiration theory according to which amniotic brain tissue fragments, which are dislocated into the amnion because of nonclosure of the ventricle or following destruction of the fetal brain, are aspirated by fetal respiratory movements in the 4th to 6th month of intra-uterine life.
An autopsy cases of Menkes kinky hair disease in a 1 year and 8 months old male infant is presented and compared with the morphological findings of the previous literatures. The main pathological changes are atrophy of the whole cerebellar cortex and bilateral temporal lobe, atrophy with demyelination of the white matter, tortuous running of the cerebral arteries, multiple diverticulosis of the urinary bladder and hyaline-like deposition in the gastric submucosa. Microscopically, the peculiar degenerative change of Purkinje cell (somal sprout) is the only characteristic lesion in our case and the others. It is suggested that Menkes kinky hair disease may be a syndrome due to metabolic disturbance appearing not only in ectoderm such as the central nervous system, but also in mesoderm such as connective tissue and bone.
Bilateral xanthogranulomas arising from both choroid plexues were found in a 6-year-old child with epileptic seizures and psychomotor retardation. Both masses were surgically removed without adding any neurological deficits. The xanthogranulomas were fairly large, and the posterior part of both lateral ventricles were slightly dilated, but clinical symptoms and the location of the xanthogranulomas could not be clearly correlated in this case.
An autopsy case of mesenchymal hamartoma of the liver was reported and from the macroscopical observation and serial sections of the tumor the following findings were found: (1) macroscopically the tumor is supplied by only one large triad, (2) in the tumor hepatic lobular structure is fundamentally preserved and prolifereated bile ducts with surrounding fibrosis and many bile thrombi are mainly elongated and dilated Hering's canals, (3) the cysts connect with dilated interlobular bile ducts, (4) there are two portions, where connection of bile ducts is incomplete, that is, from Hering's canals to interlobular bile ducts and from interlobular ducts to large ducts in the large triad of (1), (5) almost all portal veins in the large triad of (2) are stenosed or obliterated by loose intimal fibrosis. From these findings it is considered that intrahepatic bile duct obstruction resulting in regional biliary cirrhosis is the fundamental process of this disease, and in addition the role of hemodynamic disturbance was discussed.
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PURPOSE: To investigate the expression of nitric oxide synthase (NOS) in the ischemic retina. METHODS: Retinal ischemia was induced in rats by bilateral common carotid artery occlusion (BCCAO) for various lengths of time. Using the retina after BCCAO, expression of neuronal NOS (nNOS) and inducible NOS (iNOS) and identification of their positive cells were studied by histological and immunohistochemical examinations. RESULTS: Histological examinations revealed significant reduction in the thickness of the inner plexiform layer and the outer plexiform layer of the retina. Expression of nNOS was detected in retinal ganglion cells, amacrine cells, and Müller cells after BCCAO. The expression of nNOS and iNOS detected in Müller cells became stronger and persisted long after BCCAO. CONCLUSIONS: In the ischemic retina, Müller cells and retinal ganglion cells expressed nNOS and iNOS. These phenomena may be involved in the ischemic damage to the retina.
We re-examined the measurement of the mass flow ratio at model bifurcations as a function of the inlet Reynolds number, which has been used to study the role of arterial bifurcation in distal blood supply. However, this relationship was found not to be applicable to investigations of blood circulation, because the measured mass flow ratio is strongly dependent on the resistance downstream of the bifurcation, and therefore is specific to the experimental set-up. An alternative approach for studying the role of arterial bifurcation is presented, in which the flow division ratio is used as a parameter.
A case of neuronal intranuclear hyaline inclusion disease (NIHID) is described. The patient was a 26-year-old man who died of a progressive neurologic disorder, the onset of which occurred at the age of 11 years. Clinically, the disease presented as juvenile parkinsonism, and pathologically it was characterized by multiple-system degeneration in conjunction with the ubiquitous presence of intranuclear hyaline inclusions in neurons of the central and peripheral nervous system including the autonomic ganglia. Smaller and less eosinophilic intranuclear inclusions were also present in a small number of glial cells. The neuronal inclusions emitted a strong yellow-green autofluorescence under ultraviolet light and were composed of filaments 10-15 nm in diameter. The glial inclusions also consisted of similar filaments but their autofluorescence could not be determined with certainty because of their small size and background autofluorescence. A review of the literature revealed 19 similar autopsy cases up to 1987. Since the clinical presentation and distribution of neuronal loss as well as the characteristics of the inclusions showed some differences among the cases, some authors speculated that NIHID represented more than one variant of a multiple-system degenerative disease. However, about half of the reported cases had favorable sites of neurodegeneration, such as the pallidum, substantia nigra, motor nuclei of the brain stem, anterior horn cells, Clarke's column and spinal ganglion as well as similarities among the inclusions. Thus, there seems to be a discrete group among cases of NIHID.
A case of a 2-year-old girl with tuber cinereum hamartoma is evaluated by computed tomography (CT). Special reference is made to the application of CT image reconstruction, which has not yet been reported in the literature. The location, extent, shape and size of the mass lesion and also the anatomical relationship to adjacent structures are clearly demonstrated in sagittal and coronal CT images reconstructed from serial thin-section CT images in conjunction with target imaging and a review program.