摘要:

In order to study the relationship between changes in total cytoplasmic RNA and the incidence of neuritic plaques (NP) and neurofibrillary tangles (NFT) in the aging human brain, a series of parahippocampal gyri (PHG) was obtained, post mortem, from 50 aging, mostly non‐Alzheimer disease human brains, grouped in five decades of life. Absolute and relative NP and NFT counts in silver stained preparations were performed with the aid of a semi‐automated image analysis system, and total cytoplasmic RNA was estimated in azure b (ab) preparations. Progressive ab‐RNA depletion corresponded to the decades of life in which the incidences of NP and NFT increased significantly. Multivariate analysis of variance (MANOVA) indicated not only isolated effects of increasing age and decreasing mean ab‐RNA, upon the incidence of NP, but also interactive effects of the two former parameters. In addition, highly significant isolated and interactive effects of age and mean ab‐RNA decrease were also observed upon the incidence of NFT. It is put forward that the rate at which total neuronal cytoplasmic RNA (mainly rRNA species) is reduced may play an important part in the pathophysiology of the neuronal degeneration as marked histologically by the onset of N

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00203.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00204.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

The pathological findings of an adult woman and clinical features of her brothers identified a new type of congenital muscular dystrophy with brain malformation. The 40‐year‐old woman, born to parents who were first cousins, was retarded in both mental and physical development from early infancy. She had a monkey‐like face with macroglossia, dwarfism, decreased subcutaneous fat with hard skin, joint contracture, and muscular atrophy and weakness with myopathic changes, but she was able to walk until the age of 31 years. Two brothers showed clinical features more or less similar to hers, but their mental and physical development was not severely retarded. Autopsy of the patient revealed that the brain malformation consisted of micropolygyria and pachygyria, and the muscular atrophy was of the normal dystrophin type. In addition, presenile occurrence of Alzheimer's neurofibrillary tangles was prominent in the malformed brain. The overall picture was that of congenital muscular dystrophy of the Fukuyama type, demonstrating an adult variant of the di

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00205.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00206.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Three cases with cavitary lesions on the cerebral convexities in twin gestation complicated by a single intrauterine fetal demise were investigated clinicopathologically. They all exhibited profound mental retardation and severe motor disturbance due to rigidospastic tetraplegia. The co‐twins were macerated.Neuropathologically, malformed lesions and destructive lesions coexisted in varying degrees according to the time in fetal life, at which insults probably occurred. The cavitary lesions were located bilaterally and symmetrically on the central, parietal and occipital region of the cerebral hemispheres, in some cases, including the frontal region. Case 1 showed an incomplete gyral pattern and porencephaly with polymicrogyria bordering the defect, thus allowing us to date the insult before the 5th fetal month. In case 2, multicystic encephalomalacia was disclosed with cortical dysgenesis exhibiting status marmoratus of the cortex. Case 3 displayed sclerotic cavitary lesions with sclerosis of the insula and a few instances of cortical dysgenesis. Based on the clinical data and pathological findings, the cavitary lesions of case3 may be dated in the third trimester of gestation. Case 2 can be considered to have sustained injuries between the times estimated for cases 1 and 3.As the pathogenesis of the congenital cavitary lesions, several concepts have been proposed: intrauterine DIC (Mooreet al. 1969;Romeroet al. 1984), vascular disruption (Hoymeet al. 1981;Junget al. 1984), intrauterine infection such as cytomegalic inclusion disease (Friedeet al. 1976), and hypoxia due to CO intoxication of the mother (Bank1et al. 1967) or other cause. Though the pathological observation of our cases revealed no evidence of intrauterine DIC, renal cortic necrosis, intrauterine infection or occlusion of the intracranialvessels, long‐term exposure to the dead twin might have contributed to the lesions of the liveborn co

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00207.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Twenty‐two cases of delivery of twins following the antepartum death of one twin are presented. Serious complications were noted in 12 of the live‐born twins. Nine live‐born twins were delivered more than 1 week after the antepartum death of their counterpart. Two who were delivered 2 days and 5 days, respectively, thereafter showed fatal cerebral and/or renal necrosis caused by multiple infarction. In one case the course from the death of the counterpart to delivery was unclear. In all of these cases monochorionic placenta with superficial vascular anastomosis was noted. The remaining 10 twins had good outcomes. Monochronic placenta was noted in 7 cases and placental superficial vascular anastomosis in 4. In these cases, the surviving twin was delivered within 4 days of the antepartum death of the counterpart. In 3 cases in which the dichorionic placenta lacked vascular anastomoses, the surviving twin also showed excellent prognosis even though the duration after demise of the counterpart was prolonged. The incidence of venous anastomosis of the placental vessels and the type of anastomosis apparently is very important in determining the live‐born twin survives. The autopsies revealed no definite evidence of intrauterine disseminated intravascular coagulation but intrauterine disseminated intravascular coagulation but intrauterine fetal embolism was considered to be the pathogenetic factor in multi‐organ i

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00208.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

The lesions in periventricular leukomalacia (PVL) comprise necrosis and a glial reaction in the deep cerebral white matter of fetuses and neonates. The purpose of this study was to elucidate the role of glial cells in the formation of the lesions in PVL. Ten PVL brains and 22 control brains were immunohistochemically compared using anti‐glial fibrillary acidic protein (GFAP) and anti‐ferritin antibodies, and lectin‐Ricinus communis agglutinin (RCA‐1).The numbers of GFAP‐positive glia and RCA‐1 positive glia increased in the whole white matter and the periventricular white matter, respectively. Ferritin was predominantly stained in the cytoplasm of oligodendrocytes, and the number of ferritin‐positive oligodendrocytes gradually increased with age in normal brains. However, ferritin was stained in microglia and partially reactive astrocytes, instead of ferritin‐positive oligodendrocytes, in PVL brains.A relationship of the glial cellular reaction with the time scale of histologic change in PVL was shown by the appearance of RCA‐1‐positive microglia around fresh necrotic regions, accumulation of RCA‐1‐positive macrophages in necrotic regions, and then proliferation of GFAP‐positive reactive astrocytes with long processes outside the microglial reaction sites. On cavity formation, the end‐stage of PVL, rough walls of moderately dense gliosis consisted of slightly GFAP‐positive fibrillary astrocytes.PVL involves glial cellular reactions not only in regional necrotic lesions, but also in the whole cerebral white matter. The decrease in ferritin‐positive oligodendrocytes in PVL brains may be related to the delayed myelination in the brains of

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00209.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

One‐day‐old mice were subjected to hypoxia by 8‐hour exposure to a 5% oxygen, 95% nitrogen mixture. The brains from ten surviving mice with moderate brain hemorrhage were microscopically examined at 36 and 60 hours after the hypoxic event. These brains showed the coexistence of parasagittal cerebral injury, periventricular leukomalacia, multicystic encephalomalacia and subependymal germinolysis. Parasagittal cerebral injury is regarded as the principal ischemic lesion of the full‐term human infant, and periventricular leukomalacia as that of the premature infant. These results suggest the important role of hypoxia/ischemia in the pathogenesis of subependymal germinolysis and regional differences in maturation between mouse and huma

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00210.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Neuropathological abnormalities in Down syndrome are reviewed and reported. Among these are subtle but characteristic alterations in brain growth and development most apparent after 1 year of age. Neurons are also altered in Down syndrome. There are reductions in total dendritic length and number of spines begining at around 4 months of age. In addition, the number of the S‐100 protein positive cells is increased in the perinatal period correlating perhaps with increased expression in Down syndrome because of the presence of three chromosome 21s. Down syndrome is a complete disorder which can only be analyzed by utilizing a wide variety of technique

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00211.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY

摘要:

Down syndrome (DS) is a representative disorder of mental retardation caused by chromosomal aberration. However, its pathophysiologies have remained unclarified, though its neuropathological similarity to Alzheimer disease is attended. There have been few reports of precise neurological examination of this syndrome for detecting the loci of the lesion. In this report, we present the results of neurlogical and clinical neurophysiological studies on patients with DS and suggest the involvement of serotonergic (5HTergic) neurons as the main pathophysiology of this syndrome.

ISSN:0919-6544    

DOI:10.1111/j.1440-1789.1993.tb00212.x

出版商:Blackwell Publishing Ltd    

年代:1993

数据来源: WILEY