bluuebunny/crossref_metadata_2025 huggingface dataset:
Intrauterine Embolism Syndrome: Multiple Infarction of Co‐Twin of Dead Counterpart in Utero
Authors: Nakayama Masahiro
Published: October 2007

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 infarction.
Neuropathological Findings of Neonatal Mice Following Hypoxic Encephalopathy
Authors: Yoshioka Hiroshi , Sato Noriko , Okano Sozo , Yamazoe Ichiro , Nishiki Tetsuo , Kotani Hiromi , Sawada Tadashi
Published: October 2007

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 human brain.
Acute Stage Neuropathology of a Case of Infantile Acute Encephalopathy with Thalamic Involvement: Widespread Symmetrical Fresh Necrosis of the Brain
Authors: Nakano Imaharu , Otsuki Noriyuki , Hasegawa Akio
Published: October 2007

The neuropathology of the acute stage of infantile acute encephalopathy characterized by symmetrical thalamic involvement is not yet known. We describe this disorder in detail for the case of a one‐year‐old Japanese boy who presented with high fever associated with convulsions and vomiting two days after common cold‐like symptoms, rapidly fell into coma and died 29 hours after the first occurrence of convulsions. Brain CT one day before death demonstrated widespread and symmetrical low density lesions in the thalami, the deep portion of the cerebral and cerebellar hemispheres, and the tegmentum of the brain stem. The unfixed brain was swollen but without uncal or tonsillar herniation. Sections of the formalin‐fixed brain showed symmetrical softening of the same regions as shown by brain CT as low density lesions. The essential histopathology of these areas was fresh necrosis with neither astrocytic proliferation nor microglial or inflammatory cell infiltration. This histopathological finding, combined with the peculiar distribution of the lesions which is irrelevant to the irrigation field of any major cerebral artery, raises the possibility of some metabolic disturbance as the cause of this condition.
Human Prion Diseases: Creutzfeldt‐Jakob Disease, Gerstmann‐Sträussler Syndrome, and Unknown Dementia
Authors: Kitamoto Tetsuyuki
Published: October 2007

Recent molecular genetic and immunohistochemical studies have revealed that various dementias belong to the group of prion disease. The clinicopathological variabilities of these disorders can be explained by prion protein (PrP) gene polymorphisms. Here we report on the usefulness of classifying prion diseases into plaque types and non‐plaque types, based on the distribution of PrP in the central nervous system. The non‐plaque type prion diseases are characterized by rapidly progressing dementia, myoclonus, and periodic synchronous discharges in the electroencephalogram, and by the presence of diffuse gray matter accumulations of PrP, including the synaptic strutures. The plaque type prion diseases have a long clinical course without myoclonus and periodic synchronous discharges, and the major PrP accumulation sites are extracellular PrP plaques. The distribution of abnormal PrP accumulations, either plaque type or non‐plaque type is influenced by the polymorphism of the PrP gene and in turn the distribution of PrP deposits within the central nervous system influences the clinical and pathological features of prion diseases.
A Morphological Study on the Microcirculation of the Central Nervous System. Selective Vulnerability to Hypoxia
Authors: Akima Michio
Published: October 2007

We have examined the microvasculature of the human cerebral cortex, basal ganglia, hippocampus, cerebellar cortex and pons. In this presentation we summarize the results andy on the discuss the relationship between vasculature and the selectivere and its senile vulnerability of the brain to hypoxia.

One of most characteristic features of the cerebral cortex is the presence of fountain‐like ramifications in the middle andn the deep cortical branches and the intracortical branches of theecure and its senile subcortical artery. The fountain‐like ramifications appeari as branches that originate from small arteries by repeated bifurcations occurring within short segments of the arteries. This particular structural blood vessel pattern of the corticalanese). gray matter is probably responsible for the ischemia that readily develops in generalized hypotension and may contributend cerbral to the development of pseudolaminar necrosis.

Numerous reverse ramifications are present in the basal ganglia. It is thought that they play a significant role in bloodams JH, pressure regulation and occurrence of circulatory disturbances London, Mebone which are prone to involve these structures.

Within the hippocampus, h1 is supplied only by the sectora pathogenetic artery, whereas h2 is nourished by the SA, DA, and DA2 but h2 is located at the most peripheral end of these branches. In addressing the question of the vulnerability of the hippocampalypoxamia, end plate it is evident that this cannot be accounted for by they WH, notion proposed by Lindenberg. Thus, the problem of vulnerability of h1 and the end plate still remains to be explained by the vascular theory.

The distribution of arteries in the cerebellar cortex closely resembles that of the cerebral cortex. There is no particular vascular distribution to the Purkinje cell layer, but this layerarchiture of has numerous arterial branches that are derived from the middle and deep cortical branches. The capillary networklar architure ofthe density is roughly equivalent to that of the molecular and granular layers. Hence it is not possible to readily account for the selective vulnerability of the Purkinje cells to hypoxia by the vascular distribution alone.

Intertwinings of arterial branches particularly with formation of rope‐like structures are seen in fountain‐like ramifications. This type of configuration is observed in allshornerkrankung. regions examined and appear more frequently with advancing age, thus suggesting a certain correlation with the concomitant brain atrophy.
Joseph Disease with Severe Atrophy of the Brain Stem Tegmentum: Report of an Autopsied Case
Authors: Fukutani Yuken , Katsukawa Kazuhiko , Kobayashi Katsuji , Miyazu Kenji , Hayashi Masahiro , Yamaguchi Nariyoshi , Nakamura Ichiro
Published: October 2007

An autopsied case of Joseph disease with severe atrophy of the brain stem tegmentum is reported. A Japanese male noticed unsteady gait at age 30 and showed cerebellar ataxia, pyramidal signs, progressive external ophthalmoplegia, peripheral amyotrophy, sensory disturbance, and bradykinesia. He also developed mild dementia and nocturnal delirium at the terminal stage of his illness. His mother, older and younger brothers and daughter also presented progressive ataxia. The mode of inheritance is considered to be autosomal deminant. He died of cardiac failure at age 57. The autopsy revealed significant atrophy of the brain stem and spinal cord. Microscopic examination showed severe degeneration of the dentato‐rubral and subthalamo‐pallidal systems, pontine nuclei, spinal anterior horn, Clarke's column, accessory cuneate nucleus and spinocerebellar tract, moderate degeneration of the brain stem motor nuclei including the oculomotor nuclei and substantia nigra, and mild degeneration of the spinal posterior and intermediolateral column, and cerebellar cortex. An additional finding, which has received little attention in this disease, was severe atrophy of the brain stem tegmentum associated with degeneration of the reticular formation, raphe nuclei and locus ceruleus. The significance of brain stem tegmental atrophy in Joseph disease is discussed.
Detection of Neurofibrillary Tangles by the Methenamine‐Silver Method: Comparison with Other Silver Stains and with Immunostaining Methods
Authors: Haga Chie , Ikeda Kenji , Iwabuchi Kiyoshi , Akiyama Haruhiko , Kondoh Hiromi , Kosaka Kenji
Published: October 2007

Recently we devised the methenamine‐silver (M‐S) staining method for detecting amyloid of senile plaques. Subsequently it was noticed that this procedure also readily stains neurofibrillary tangles (NFT) and neuropil threads. However, the sensitivity of the M‐S method as an NFT stain has not been determined. In order to clarify this point, we performed a quantitative study on NFT, comparing the M‐S method with conventional Bodian method, Gallyas‐Braak method, and Bielschowsky‐Hirano method on brain samples from three Alzheimer‐type dementia patients and two non demented subjects. In addition, NFT staining by the M‐S method was also compared with staining by anti‐tau and anti‐ubiquitin antibodies. Our results indicate that the M‐S method was as sensitive for NFT as the Gallyas‐Braak procedure. Further‐more, the M‐S method stained not only intraneuronal NFT, but also extracellular NFT, some of which could not be identified by conventional silver staining techniques and by immunostaining for tau and ubiquitin. We conclude that the M‐S method is useful for the routine examination of senile plaques and NFT.
Histopathological Changes in Two Chinese Cases of Senile Dementia of Alzheimer Type
Authors: Wang Lu Ning , Huang Ke‐Wei
Published: October 2007

This report concerns two cases of senile dementia of Alzheimer type (SDAT) selected from among 150 consecutive autopsies. Clinically, both patients had progressive dementia after the age of 76 and typical Alzheimer's changes, including neurofibrillary tangles, senile plaques and granulovacuolar degeneration were found at autopsy. One case was complicated by congophilic angiopathy. The histological description presented makes emphasis on the presence of neurofibrillary tangles throughout the amygdala and of senile plaques that involved primarily its cortical nuclei and the ventral parts of basal and accessory basal nuclei.
HTLV‐1 Infection of a Human Monocytic Cell Line and of Primary Cultures of Human Monocyte‐Derived Macrophages
Authors: Kato Kyozo , Douglas Steven D. , Koprowski Hilary , Wroblewska Zofia
Published: October 2007

This report concerns the pathogenesis of the myelopathy associated with HTLV‐1 infection and here we present the results of investigating the effect of HTLV‐1 on cultures of human monocytes and of monocyte‐derived macrophages. The monocytic cell line U937 was successfully infected by cocultivation with lethally irradiated cells carrying HTLV‐1, as well as by inoculation of concentrated cell‐free virus preparations. On the basis of immunoperoxidase staining, we determined that 0.3 to 0.5% of the cell population infected by cocultivation, and 0.5 to 0.7% of that treated with the cell‐free virus expressed HTLV‐1 antigen. U937 cells were also transfected with HTLV‐1 proviral DNA. Two days after transfection, specific staining for HTLV‐1 p24 antigen was evident in 4 to 5% of the cell population. Primary cultures of monocyte‐derived human mocrophages could also be infected with the concentrated cell‐free virus preparations and 0.8 to 1.0% of the cells expressed the viral antigen as determined by the alkaline phosphatase‐anti‐alkaline phosphatase immunocytochemical proceduce. The presence of TNF ß in the medium supernatant of HTLV‐1‐carrying cells was documented by a bioassay in which L929 cells were used. A cytotoxic effect of cell‐free supernatants from virus‐carrying cells could be demonstrated in the case of one CNS‐derived continuous cell line. No TNF activity was detected in supernatants of U937 cells or of macrophages experimentally infected with HTLV‐1.
Two Cases of Juvenile Type Generalized Ceroid‐Lipofuscinosis: An Ultrastructural Study of Storage Material
Authors: Kurihara Mana , Kumagai Komei , Goto Kazutoshi , Yagishita Saburo , Itoh Yoji
Published: October 2007

The clinical course, distribution and morphology of the characteristic inclusions in two cases of generalized ceroid‐lipofuscinosis of juvenile type were investigated. The first case was a Japanese man who died aged 21. He developed visual disturbances at the age of 6 years, and became blind within a year. Epileptic seizures and dementia progressed from the age of 12. The second case was a Japanese woman who also died aged 21 years. She developed dementia, convulsions and visual disturbances at the age of 6 years and progressed gradually. She became almost completely blind at the age of 14 years. In both of the cases, the autopsy revealed diffuse atrophy of the brain. The histology showed numerous lipofuscin‐like granules in the swollen cytoplasm of nerve cells and astrocytes. The storage materials revealed four distinct ultrastructural profiles as follows; 1) multilamellar body (MLB), 2) curvilinear body (CB), 3) crystalloid profile (CP), 4) finger print profile (FPP). The distribution of these storage materials in both central and peripheral nervous system and in various tissue of the body was studied systematically and shown in a table.
Sarcolemmal Coexpression of Intercellular Adhesion Molecule‐1 (ICAM‐1) and HLA‐DR in Inflammatory Myopathy
Authors: Shimizu Jun , Kawai Mitsuru , Kanazawa Ichiro
Published: October 2007

This report concerns the immunohistochemical detection of the intercellular adhesion molecule‐1 (ICAM‐1), HLA‐ABC and HLA‐DR in consecutive cryostat sections of skeletal muscle biopsies from 38 patients with inflammatory myopathy. Among these were 12 cases of dermatomyositis, 22 cases of polymyositis, two cases each of inclusion body myositis and sarcoid myopathy. Specimens from eight cases of Duchenne muscular dystrophy, three cases each of alcoholic polyneuropathy and myotonic dystrophy, two cases of motor neuron disease, and three normal individuals were included for comparison. Sarcolemmal ICAM‐1 expression by undamaged muscle fibers was detected in 18/38 cases of inflammatory myopathy who had large numbers of endomysial inflammatory cells, but not in normals or patients with non‐inflammatory myopathy. Sarcolemmal immunostaining was more intense in the vicinity of the inflammatory cells. Some regenerating muscle fibers, irrespective of disease, expressed sarcoplasmic ICAM‐1. Sarcolemmal HLA‐ABC and HLA‐DR immunoreactivity was detected in undamaged muscle fibers of 37/38 and 18/38 cases of inflammatory myopathy, respectively. Sarcolemmal immunostaining for HLA‐DR was observed in cases with large amounts of endomysial inflammatory cells, and was stronger in muscle fibers that were in their vicinity. HLA‐DR and ICAM‐1 immunoreaction product deposits were generally colocalized in the sarcolemma of the same fibers. The specific sarcolemmal expression of ICAM‐1, and its colocalization with HLA‐DR may be important for the recognition of muscle fibers by invading inflammatory cells in inflammatory myopathy.
Extremely Low Magnification Electron Microscopy for Observation of Degenerative Changes of the Central Nervous System
Authors: Nakamura Minako , Izumiyama Yoko , Hayashida Kentaro , Arima Kunimasa , Mizutani Yoshihiko , Oyanagi Shinsaku
Published: October 2007

With the purpose of achieving a better correlation between the neuropathologic features observed by light microscopy (LM) and those seen by electron microscopy (EM), the authors attempted to use a conventional electron microscope (EMe) to obtain images at the extremely low magnification (mag) of 500x. This goal was successfully accomplished by using the methodology devised for this investigation, for which 100 nm thick, 2×2 mm sections were prepared. These were mounted on 75 hexagonal grids (VECO), double‐stained for a slightly longer period of time than usual, and then examined with a conventional EMe using the low mag mode lens system and a 100 KV accelerating voltage. The technique was used for the examination of three degenerative structures, to wit: grumose degeneration, stellate body and senile plaques. The present method allowed satisfactory viewing at an extremely low mag, as a given degenerative structure readily fit in its entirety on one sheet of film. The structural features were strikingly more distinct when viewed at low mag EM than at a similar LM mag. For instance, by using the former, the central cores of classical senile plaques had the appearance of chestnut burrs or of a herring‐bone pattern with numerous amyloid bundles. Based on their results, the authors believe that the low mag EM technique provides an essential step toward the correlation of LM and high mag EM findings.
Parkinson's Disease: Distribution of Lewy Bodies in the Peripheral Autonomic Nervous System
Authors: Takeda Shigeki , Yamazaki Kazunori , Miyakawa Teruo , Arai Hiroyuki
Published: October 2007

This report concerns the presence and distribution of Lewy bodies in the peripheral nervous system of three patients with Parkinson's disease and ten age‐matched nonparkinsonian individuals. We examined the paravertebral and celiac ganglia as representatives of the sympathetic system; the submandibular ganglion as representative of the parasympathetic system; the esophagus, stomach, duodenum, jejunum, ileum and colon as representatives of the enteric system, and the trigeminal and dorsal spinal ganglia as representatives of the somatic sensory system. Lewy bodies were observed in the peripheral sympathetic and enteric systems of two of the three patients with Parkinson's disease. These two patients had numerous Lewy bodies in the submandibular ganglia. The third patient did not have Lewy bodies in the peripheral autonomic nervous system, nor did the ten control individuals studied. Lewy bodies were not detected in the somatic sensory system of any of the three parkinsonian patients. Our findings indicate that the peripheral autonomic system, including the parasympathetic system is affected in Parkinson's disease.
Machado‐Joseph Disease in Japan: Clinicopathological Study of 6 Autopsy Cases with Special Reference to the Clinicopathological Correlation to Cerebellar Ataxia and Lower Motor Neuron Signs
Authors: Tsuchiya Kuniaki , Wakabayashi Masatoshi , Oyanagi Shinsaku , Ikeda Kenji , Mizutani Toshio , Morirnatsu Yoshio , Ohbu Sadayoshi , Saiki Shigeki , Makifuchi Takao
Published: October 2007

This report concerns a clinicopathological study of six Japanese patients with Machado‐Joseph disease. All were unrelated females; their ages at disease onset ranged from 17 to 36, and the length of the clinical course from 12 to 50 years. The results of neurological examinations indicated that lower motor neuron signs such as ophthalmoparesis, dysphagia, atrophy of the tongue, and muscular weakness of the extremities appeared in the intermediate stages of the disease, and that from these to the end stages, involvement of the lower motor neurons became cardinal signs of the disease. Urinary and/or fecal incontinence was evident in four patients.

Post‐mortem investigations revealed that, in comparison with European and American cases, the involvement of the globus pallidus (particularly the medial segment) and grumose degeneration of the dentate nucleus were salient pathological features of Japanese Machado‐Joseph disease patients. We also noticed involvement of the Onufs nucleus of the two cases in which this structure was examined.

The application of a semiquantitative method revealed that neuronal loss in the pontine nucleus, hypoglossal nucleus, and anterior horn of the cervical cord increased in relation to the duration of the disease. By contrast, the severe loss of neurons in the dentate nucleus and Clarke's colum was not related to the length of the clinical course.

We suggest that in the initial stages of Machado‐ Joseph disease the cerebellar ataxia is probably due to the involvement of the dentate nucleus and Clarke's column, and that in the advanced stages it is caused by alterations of the pontine nucleus as well. We believe that bulbar signs and muscular weakness of the extremities that appear in the middle stages of the disease are due to progressive neuronal loss in the hypoglossal nucleus and anterior horn. The issue regarding the neuropathological similarities of Machado‐Joseph disease in Japanese patients, “Marie's ataxia” and spinopontine degeneration is addressed in the discussion.
A Case of Presenile Dementia with Neurofibrillary Tangles but without Senile Plaques in Small Neurons of the External Granular Layer of the Cerebral Cortex
Authors: Iijirna Masaaki , Ishino Hiroshi , Seno Haruo , Inagaki Takuji , Yamashita Yozo , Yoshioka Shinichi
Published: October 2007

This report describes a case of unclassifiable presenile dementia. A 38‐year‐old man, blind since shortly after birth, started to exhibit personality changes, such as irritability and withdrawal. The following year he began to experience disorientation, auditory hallucinations, delusions of persecution, memory disturbances, and generalized convulsions. Two years later, the patient exhibited snout reflex, grasp reflex, and small steppage gait. He became apallic and died of acute heart failure at the age of 41, three years after the onset of neurologic symptoms. The neuropathological examination revealed numerous ring‐, crescent‐, or horseshoe‐shaped neurofibrillary tangles around the nuclei of neurons, particularly those in the external granular layer of the cerebral cortex and the dentate gyrus of the hippocampus. However, no senile plaques were found. Neuropil threads and glial cytoplasmic inclusions of the oligodendroglia were detected in the cerebral white matter, hippocampus, basal ganglia, diencephalon, and brainstem. To our knowledge, no similar case of presenile dementia has been described heretofore.
Striatal, Accumbent, and Nigral Degeneration with Rosenthal Fibers, Presenting as Shy‐Drager Syndrome
Authors: Ozawa Eisuke , Tsukagoshi Hiroshi , Morimatsu Yoshio , Nagashima Kazuo
Published: October 2007

Shy‐Drager syndrome (SDS) is a clinical disease entity that, because of its pathological features, is included in the category of multiple‐system atrophy (MSA). However, we describe in this report a case of SDS that differed clinicopathologically from MSA. A 65‐year‐old man gradually developed impotence, orthostatic hypotension, parkinsonism, dysarthria, and bulbar signs in the terminal stage, all compatible with SDS. In addition to these signs, involuntary movements of the mouth and toes were noted in the course of the disease, and severe myoclonus‐like movements were observed during the terminal stage. The postmortem examination revealed degenerative neuron changes with astrocytosis in the substantia nigra, putamen, caudate nucleus and nucleus accumbens that were accompanied by the presence of Rosenthal fibers. Loss of myelin sheaths which were replaced by fiber gliosis with Rosenthal fibers, was evident in the hilus of the dentate nucleus. By comparison, the neurons of the autonomic spinal centers were intact. The overall features of the present case were different from those hitherto described for SDS, suggesting that they may reflect presentation of a new disease category for this syndrome.
Neuropathological Diagnosis of Sen1ile Dementia of the Alzheimer Type (SDAT): Proposal of Diagnostic Criteria and Report of the Japanese Research Meeting on Neuropathological Diagnosis of SDAT
Authors: Mizutani Toshio
Published: October 2007

Tentative neuropathological diagnostic criteria of senile dementia of the Alzheimer type (SDAT) are proposed in this report of the activities of the Japanese Research Meeting on Neuropathological Diagnosis of SDAT. The criteria include: 1) The exclusion of other diseases that cause dementia; 2) Senile plaques (SPs) and Alzheimer's neurofibrillary tangles (NFTs) above the upper normal limits in more than one cerebral gyrus, and 3) Laminar degeneration in the entorhinal cortex. Our criteria are considered to be better suited for confirmation of clinical SDAT than the previous ones, since more than 90% of the cases that satisfied the proposed criteria were clinically diagnosed as having SDAT. On the other hand, the application of our criteria to 1,000 consequent autopsies showed that not all cases with clinically diagnosed SDAT had pathologically stereotypical features of this disease. Such cases include: 1) those that only had SPs in the cerebral cortex, but no significant numbers of NFTs and no apparent cerebral atrophy; 2) those in which numerous NFTs were concentrated in the hippocampus, and 3) those with marked fibrillary gliosis in the temporal white matter and unusual clinical symptoms. These results could reflect the pathological heterogeneity of SDAT and the difference between Alzheimer's disease and SDAT.
Molecular Biology of 'Szheimer's Disease and Neuron's Death
Authors: Kuroda Yoichiro , Kawahara Masahiro
Published: October 2007

'Szheimer's disease has aroused much interest among neuroscientists within the recent decades. The deposition of amyloid β‐protein in senile plaques is one of the major h'slmarks of the disease, and is thought to be a primary factor for its occurrence. Recent studies have shown that amyloid β‐protein causes degeneration of cultured hippocamp's neurons, and that its neurotoxicity is enhanced by its aggregation and by conformation's changes. Therefore, factors that stimulate or inhibit amyloid β‐protein aggregation may play key roles in the etiology of 'Szheimer's disease. Moreover, environment's factors, such as 'suminum and zinc, as well as genetic factors may 'sso be involved in the pathogenesis of the disease.
Borderline State between Senile Dementia of 'Szheimer Type and Norm's Aging: A Clinico‐pathologic's An'sysis
Authors: Ikeda Kenji
Published: October 2007

This report concerns the retrospective clinic's ev'suation of non‐demented individu'ss who had amyloid β‐protein deposits (AmD) at a level corresponding to that of the mild stage of senile dementia of 'Szheimer type (SDAT). Our investigation disclosed a considerable incidence of psychologic's symptoms and slight intellectu's impairment in the group of subjects studied. The same relationship was found by the neuropathologic's re‐examination of patients with a clinic's diagnosis of senile psychosis. These findings indicate that the pre‐clinic's state of SDAT must be taken into account, and provide evidence for the concept of a borderline state between norm's aging and SDAT. The neuropathologic's comparison of borderline cases and patients with mild, but definite SDAT showed parti's overlap of the two conditions, which is due to the different compensative abilities of each individu's. Therefore, it is proposed here that in addition to the definite criteria for SDAT, there is need for a neuropathologic's criterion of probable SDAT that would cover demented and borderline subjects with slight intellectu's impairments.
Clinicopathologic's Investigations of Atypic's Senile Dementia of 'Szheimer Type
Authors: Amano Naoji , Mizutani Toshio , Otani Takeshi , Kase Akihiko , Saitoh Atsushi , Matsushita Masaaki , Yagishita Saburo
Published: October 2007

This report concerns clinicopathologic's investigations of atypic's cases of senile dementia of 'Szheimer type (SDAT). Most cases presented are patients with severe neuron's loss in the medi's and lower portions of the tempor's lobe. In addition, we 'sso describe one case who had significant loss of neurons and numerous neurofibrillary tangles, but no senile plaques, and another patient with a peculiar plaque‐like angiopathy. The ev'suation of these cases reve'sed that these unusu's histopathologic's features may underlie their clinic's manifestations. Speci's emphasis is given to the notion that SDAT is a heterogeneous condition that may include various types of senile dementia.
Granulomatous angiitis of the central nervous system
Authors: Hamaya Kazuo , Nose Soichiro , Takeshima Masayuki , Shioji Yasuyuki
Published: October 2007

An 88 year old female had an epileptic attack, followed by fever, vomiting and stupor. A computerized tomogram disclosed bilateral periventricular lucency and a low density area in the right occipital lobe. Antibiotics, hypertonic sodium chloride, steroids, and restricted water intake were ineffective. She died 6 weeks after admission. Autopsy showed that there were extensive irregular necroses in both cerebral hemispheres. Microscopic study disclosed diffuse meningitis and non‐caseating granulomatous inflammation with macrophages and giant cells of Langhans' type in the small‐ and medium‐sized meningeal vessel walls. Some giant cells engulfed elastic membranes in their cytoplasms. The lumina were often thrombosed and her neurological progression was due to diffuse cerebral infarction.
Coexistence of Creutzfeldt‐Jakob disease and senile dementia of the Alzheimer type
Authors: Wakabayashi Koichi , Hinokuma Kaoru , Takahashi Hitoshi , Seki Koji , Tanaka Masaharu , Ikuta Fusahiro
Published: October 2007

An 88 year old woman with slowly progressive dementia of 5 years' duration experienced rapidly progressive consciousness disturbance, myoclonus and periodic synchronous discharge on electro‐encephalography from 3 months before her death. Post‐mortem examination revealed numerous senile plaques reactive with antibody against p‐peptide and widespread occurrence of spongiform change, astrocytosis and marked neuronal loss in the entire cerebral cortex and amygdaloid nucleus. These findings indicate that Creutzfeldt‐Jakob disease (CJD) and senile dementia of the Alzheimer type (SDAT) occurred in the same patient. As the number of aged patients with dementia in care homes and hospitals for the elderly is increasing, it is important to know that CJD can occur late in life during the course of SDAT, as shown in the present case.
Dentatorubropallidoluysian atrophy: Clinicopathological study of eight autopsy cases with special reference to the clinicopathological correlation between pyramidal sign and involvement of the pyramidal tract
Authors: Tsuchiya Kuniaki , Oyanagi Shinsaku , Ikeda Kenji , Arima Kunimasa , Ando Susumu , Sunohara Nobuhiko , Anami Kimitaka , Akashi Toshio , Kurosawa Takashi , Shimada Hiroyuki
Published: October 2007

This paper reports a clinicopathological study of eight Japanese patients with dentatorubropallidoluysian atrophy (DRPLA) with special reference to the clinicopathological correlation between the pyramidal sign and involvement of the pyramidal tract. None of the patients were related. There were five males and three females with age at disease onset ranging from 8 to 43 years, and the length of clinical course from 12 to 28 years. In each case the pattern of inheritance of the disease was consistent with that of an autosomal dominant trait. The neurological examination revealed that all eight individuals had dementia and showed cerebellar signs. Seven patients had epilepsy and choreoathetoid involuntary movement; myoclonus was evident in six cases. Degeneration of the globus pallidus (the lateral segment in particular) and of the dentate nucleus were the principal pathological features of the DRPLA patients. Pyramidal signs were noted in four patients. Evidence of pyramidal tract involvement was found in three cases. However, the clinicopathological correlation between pyramidal sign and pyramidal tract involvement was weak as there was concurrence of both in only one case. We suggest that pyramidal signs are being overlooked in DRPLA.
Glial tangles in Pick's disease
Authors: Ishizu Hideki , Kuroda Shigetoshi , Nishinaka Tetsuya , Yoshiki Satoh , Namba Masuyuki
Published: October 2007

In seven of nine patients with Pick's disease (PD), tau‐positive glial inclusions were observed. Coiled, wavy or globular shaped oligodendroglial fibrillary tangles (OFT) were found mainly in the cerebral white matter. However, thorn or corolla shaped astrocytic fibrillary tangles (AFT) were observed in the cerebral cortex. Three types of fibers were observed ultrastructurally in the OFT in the white matter. The first type was loose, fascicular and straight microfibrils measuring about 15 nm in diameter with a center core, which was observed in the coiled type OFT. The second type, observed in the wavy shaped OFT, was fibrils with about 200 nm periodic constrictions, the width was approximately 22 nm at the widest point and 11 nm at the constrictions. The third, observed in globular type OFT, was microfibrils measuring about 15 to 25 nm in diameter crossing at random. Astrocytic fibrillary tangles in the cortex were composed of 15 nm straight tubules. Our findings indicate that OFT and AFT are often present in classical PD and that the argyrophilic Pick bodies, OFT and AFT are distinct pathological structures occurring in different types of cells.
Immunohistochemical and ultrastructural study of the cerebellar medulloblastoma‐neuroblastoma group and cerebral primitive neuroectodermal tumors
Authors: Yagishita Saburo , Kawano Nobuyuki , Kameya Toru
Published: October 2007

Cerebellar medulloblastoma has been recognized as a distinct clinicopathological entity; however, its origin and its differentiating capacity are still disputed because of the primitive nature of the tumor cells. To assess the nature of so‐called medulloblastoma, 12 cases of medulloblastoma‐neuroblastomas, two primitive neuroectodermal tumors (Rorke's NOS category) and two cerebral neuroblastomas were studied. Moreover, some mature neuronal tumors, and some glial and mesenchymal tumors as controls, were examined by electron microscopy and immunohistochem‐istry using a panel of the antibodies. The relatively high frequencies of neuronal differentiation in the so‐called medulloblastoma and the paucity of glial differentiation, albeit with the absence of similar neurofilament immuno‐reactivity in the neoplastic cells, suggest the histogenesis of these neoplasms is more closely aligned with the histogenesis of neuronal elements in the central nervous system.

Human β4 tubulin isotype (TuJl) first becomes positive when primitive neuronal neoplastic cells start to form neuritic processes from the cytoplasm. A maturation‐associated switch from vimentin filament synthesis to (34 tubulin may occur in the primitive neuronal neoplastic cells. TuJl is a reliable marker of neuronal tumors.
Morphometrical comparison of neurofibrillary tangles and senile plaques in Alzheimer's disease and the senile dementia of Alzheimer type
Authors: Arai Tetsuaki , Ikeda Kenji
Published: October 2007

The densities of senile plaques (SP) and neurofibrillary tangles (NFT) were studied in the cerebral cortex in nine autopsy cases of Alzheimer's disease (AD) and seven cases of senile dementia of Alzheimer type (SDAT). The number of SP and NFT were counted in 14 selected areas of the cerebral cortex using methenamine‐silver and Gallyas‐Braak stains, respectively. The ratio of cortical atrophy in each AD or SDAT case to the mean value in normal age‐matched cases was estimated, and the results were corrected for cortical atrophy using this ratio. The SP density in AD after correction was significantly higher than that in SDAT in the superior and inferior frontal, rectal, anterior cingulate, superior temporal and suprapar‐ietal cortices. The NFT density in AD was also significantly higher in all of these areas and also in the medial temporo‐occipital cortex. The significant difference between AD and SDAT for NFT density after correction was greater than that for SP density in the superior and inferior frontal, anterior cingulate and superior temporal cortices. These results suggest that the higher densities in AD of SP and NFT in these areas are useful in distinguishing AD from SDAT. The NFT density was a more useful indicator than the SP density in differentiating between the two conditions. The greater density in AD of SP and NFT in the cerebral neocortex may reflect the focal clinical symptoms, frequently observed in AD. The quantitative difference between AD and SDAT in the two changes observed in this study was not due only to the difference in the duration of illness, because the SDAT cases with long survival showed significantly lower NFT density than AD cases with similar disease duration and brain weight. It is suggested that degeneration process in AD progresses more rapidly than that in SDAT.
Axonal dystrophy in monkey spinal ganglia: Involvement of the initial segment of axons
Authors: Fujisawa Kohshiro
Published: October 2007

Examination of spinal ganglia of ageing and old Japanese monkeys, Macaca fuscata, by light microscopy and electron microscopy revealed unidentified pathology of the axon initial segment. Although the incidence of this change is infrequent in any one histological section of ganglion, its morphology is quite remarkable. The change is characterized by a progressive swelling of the unmyelinated portion of axon followed by continuous involvement of the first myelin segment. Neurofilaments proliferate in rather randomly oriented bundles within the affected axons accompanied with a considerable amount of mitochondria. The axons, originally 3–5 um in diameter, thus become nodularly varicose and become as thick as 20 um or more in diameter. The second stage of the axonal pathology is characterized by massive dense accumulation of tiny vesicular granules which infiltrates and/or replaces the neurofilament mass, almost fills the whole axon and the affected axons could result in a thickness of 70 um or more in diameter (i.e. larger than the mother perikaryon itself). The third stage of the axonal pathology is characterized by a catabolic transformation of those dense massive aggregates of the vesicular granules into irregular patches of amorphous electron dense substance. Smooth endoplasmic reticulum may make a focus of proliferation within the affected axon, but its development is relatively poor and restricted. It is surprising that the mother perikaryon of the affected axon initial segment remains as a rule quite normal in appearance. It is also remarkable that the part of the axon which is distal to the swelling remains viable, apparently for a long time. The significance of this axonal pathology is discussed in relation to ageing and diabetic axonal dystrophy in the sympathetic ganglia, and to ageing axonal dystrophy in the gracile nucleus.
Argyrophilic meshwork structures in the cerebral cortex of patients with progressive supranuclear palsy
Authors: Komori Takashi , Shibata Noriyuki , Kobayashi Makio , Sasaki Shoichi
Published: October 2007

We describe the presence of tau protein‐positive argyrophilic meshwork structures in the cerebral corticomedullary junctions of three patients with progressive supranuclear palsy (PSP). Meshwork structures are composed of fine, winding fibrils, partly connected to coiled inclusions in oligodendrocytes, and are present on neurofilament protein‐positive axons. The novel abnormal structures are numerous in the middle frontal and precentral gyri, and at the ultrastructural level they are present mainly in the outer myelin sheath. Our results suggest that the unusual meshwork structures are formed in processes of oligodendroglia in the cerebral cortex. Further study of well characterized cases is required in order to determine the significance of these structures in cerebral cortical functions in patients with PSP.
Membranous cytoplasmic bodies in the anterior horn neurons in two patients with amyotrophic lateral sclerosis
Authors: Okamoto Koichi , Watanabe Mitsunori , Amari Masakuni , Hirai Shunsaku
Published: October 2007

We observed abundant membranous cytoplasmic bodies in several remaining anterior horn neurons in two patients with sporadic amyotrophic lateral sclerosis (ALS). These bodies were rare in the neurons of the frontal cortex and of the rectum examined in one case. The link between membranous cytoplasmic bodies and ALS is vague; however, pathogenesis of ALS is unknown and there are few reports on the lysosomal system in ALS. Therefore, further study is needed in order to evaluate abnormalities in the lysosomal system to clarify the degenerative processes in ALS.
Demonstration of neurofibrillary tangles in the indusium griseum and of axonal disturbances in sagittal sulcal lesions of the corpus callosum: An immunohistochemical investigation
Authors: Umahara Takahiko , Hirano Asao , Shibata Noriyuki , Kato Shinsuke , Kawanami Toru
Published: October 2007

This is an immunohistochemical investigation of the indusium griseum (supracommissural hippocampus) of the brains of control subjects and patients with Alzheimer's disease (AD). Our assays revealed the presence of fine deposits of synaptophysin‐positive dots in the neuropil and around neurons in the indusium griseum of control subjects. By using an antibody to Alzheimer neurofibrillary tangles (NFT) we identified NFT and neuropil thread‐like structures in the indusium griseum of AD patients; no β‐amyloid protein‐containing senile plaques were seen. To our knowledge, this is the first documentation of the occurrence of NFT in the indusium griseum. We also performed immunohistochemical assays on anterior sagittal sulcal lesions of the corpus callosum (ASSLCC), also known as surface lesions of the corpus callosum. These studies revealed a uniformly increased expression of the 200 kDa phosphorylated neurofilament protein by the axons in the pale myelin areas around the zonal depressions caused by the mechanical pressure of the anterior pericallosal artery. By contrast, immunoreactivity with an antibody to glial fibrillary acidic protein was either unchanged or reduced in the ASSLCC.
Suppression of relapsing experimental allergic encephalomyelitis by mizoribine: Clinical, histological and immunohistochemical studies
Authors: Hosoda Yoshito , Kato Shinsuke , Ohama Eisaku
Published: October 2007

Experimental allergic encephalomyelitis (EAE) is an organspecific, cell‐mediated inflammatory autoimmune disease and is regarded as a model of multiple sclerosis (MS). Relapsing EAE was induced in Lewis rats and the effect of mizoribine on the relapsing EAE was examined clinically, histologically and immunohistochemically. Mizoribine (4‐carbamoyl‐1‐β‐D‐ribofuranosyl‐imidazolium‐5‐olate), an immunosuppresive agent, is an imidazole nucleotide isolated from a culture infiltrate of Eupenicillium brefeldianum M‐2166. Most control and treated rats suffered two attacks. Treated rats at a dose of 10mg/kg per day showed clinically significant delay of the disease attacks and histologically reduction of infiltrating cell number. Treated rats at a dose of 20 mg/kg per day showed a further delay in the attacks and attenuation of clinical signs, and a smaller number of inflammatory cells and Ia positive cells were revealed at the first attack. This study suggests that mizoribine suppresses the clinical severity and inflammation in relapsing EAE.
Aneurysmal bone cyst of the skull
Authors: Nakajima Hiroshi , Kubo Osami , Himuro Hiroshi , Takakura Kintomo
Published: October 2007

The case of a 6 year old girl with aneurysmal bone cyst (ABC) presenting as a subperiosteal hematoma is reported. Postoperative computerized tomography revealed a new multicystic lesion suggestive of ABC which disappeared spontaneously about 1 month after the operation. The slowly progressing process and spontaneous disappearance of ABC in this case may be noteworthy when considering the surgical indication of this pathology.
A unique type of multiple system degeneration involving the subthalamic nucleus, substantia nigra, dentate nucleus, superior olivary nucleus, and posterior column: A clinicopathological study
Authors: Tsuchiya Kuniaki , Ozawa Eisuke , Irie Hiroshi , Mizutani Toshio , Morimatsu Yoshio , Shimada Hiroyuki
Published: October 2007

This report concerns an autopsy case of a unique type of multiple system degeneration of the central nervous system. The patient was a Japanese man who was 57 years old at the time of death. The family history did not reveal hereditary burden. His clinical manifestations included deafness in the latter half of the second decade of life, followed by cerebellar ataxia with disturbance of deep sensation. Slight dementia, including psychiatric signs, appeared at the terminal stage of the illness. The neuropathological examination revealed severe degenerative changes in the subthalamic nucleus, substantia nigra, dentate nucleus, superior olivary nucleus, inferior olive, and posterior column. As far as we are aware there is no report of a patient with multiple system degeneration, whose lesions involved such a wide range of anatomical sites as the present case. Because some of his alterations are seen in other disorders, we will address differences between our case and those of other disorders. It is concluded that our patient did not have optico‐cochleo‐dentate atrophy, Friedreich's ataxia, or Machado‐Joseph disease.
A clinico‐pathologico‐biochemical study of four autopsy cases of dentatorubropallidoluysian atrophy: With special reference to choreic movement
Authors: Mizutani Yoshihiko , Kondo Tomoyoshi
Published: October 2007

We evaluated four autopsy cases of dentatorubropallidoluysian atrophy (DRPLA) which had been diagnosed clinically as Huntington's chorea based on choreic movements, mental symptoms, and family history. These cases showed cerebellar ataxia at an early stage and developed choreic movements including a ballistic element. Pathological examination after autopsy revealed findings of DRPLA in all cases, although each case appeared to be in a different stage. Comparison of the clinical signs with the pathological findings suggests that the cerebellar ataxia observed clinically may have been related to pathological changes in the dentate nucleus of the cerebellum, and choreic movements may have been related to the pallidoluysian system, in particular, the nucleus of Luys. Biochemical analysis using frozen brain tissue revealed a decrease in γ‐aminobutyric acid (GABA) in the substantia nigra similar to that seen in Huntington's chorea in three cases. The findings of this study help to clarify the pathological processes that are responsible for the motor disturbances seen in patients with DRPLA.