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  • 1
    ISSN: 1432-0533
    Keywords: Marchiafava-Bignami disease ; Corpus callosum ; Striatal degeneration ; Wernicke's encephalopathy ; Alcoholism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A Japanese man with a variety of neurological complications, had drunk Japanese rice wine (sake) daily for about 25 years. There was a progressive development of parkinsonism, cerebellar ataxia, and mental deterioration by the time he was 32. He died of pneumonia at age 50 and the autopsy revealed Marchiafava-Bignami disease (MBD), striatal degeneration, pseudolaminar sclerosis of Morel, atrophy of the corpus mamillare and pons, cortical cerebellar atrophy, pseudopellagra, and polyneuropathy. This is the first case of MBD in a Japanese related to the ingestion of Japanese “sake”, and it is also a rare case in that almost all of the neurological complications seen with chronic alcoholism were apparent. Striatal degeneration seems to be a rare complication of chronic alcoholism.
    Type of Medium: Electronic Resource
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  • 2
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 51 (1980), S. 127-134 
    ISSN: 1432-0533
    Keywords: Experimental spongiform encephalopathy ; Creutzfeldt-Jakob disease ; Kuru ; Transmission to small rodents
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Experimental transmission of subacute spongiform encephalopathy from three human cases to small rodents is reported. The first case with atypical CJD with spongiform change, kuru plaques, and leukomalacia was transmitted directly to mice, rats, and guinea pigs and indirectly to hamsters and Mongolian gerbils through rats. From two other typical SSE cases the disease was also successfully transmitted; from the second case to mice and rats, and from the third case to guinea pigs. Brain showed the highest infectivity; the spleen, liver, blood, and cerebrospinal fluid of diseased animals were also infective. Intracerebral inoculation was the route for the fastest transmission, followed by intrathecal, intraperitoneal, submucosal, and subcutaneous routes. The incubation periods and clinical features were characteristic in each inoculated species and did not vary within several passages, except for the shortening of incubation period from the first to the second passage. Histologically, a marked spongy state and proliferation of astrocytes were observed in all diseased animals, though the distribution of the lesion was peculiar to each species. The severe lesion in the white matter in mice was similar to that seen in mice inoculated with scrapie and also to that seen in the first case.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 67 (1985), S. 272-278 
    ISSN: 1432-0533
    Keywords: Amyloidosis ; Amyloid fibril protein ; Creutzfeldt-Jakob disease ; Senile dementia of Alzheimer type ; Autoclaving
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The permanganate method, the immunoperoxidase method, and a newly developed autoclave method were used to distinguish different types of amyloid fibril proteins in formalin-fixed, paraffinembedded tissue sections. All tissues from permanganate-sensitive cases (AA type) lost the affinity of Congo red and green birefringence under polarized light after incubation with special autoclave treatment. AL type systemic amyloidosis and amyloid plaques of CJD and GSS were permanganate-resistant, but decreased markedly the affinity of Congo red after prolonged autoclaving. On the other hand, prealbumin type systemic amyloidosis and senile plaques of SDAT were resistant to both permanganate oxidation and prolonged autochlaving. Thus, amyloid plaques of CJD and GSS are identical to AL type in systemic amyloidosis, and senile plaques are similar to the prealbumin type. However, anti-prealbumin antiserum did not stain senile plaque amyloid. The anti-human P component stained positively systemic amuyloids and cerebral amyloid plaques of SSE, but failed to stain senile plaques of SDAT. Therefore, the amyloid fibril protein of senile plaues is apparently different from other types of amyloid depositions. Amyloid plaques of SSE are different from senile plaques not only with regard to fibril proteins, but also to globular protein in the amyloid.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 63 (1984), S. 24-32 
    ISSN: 1432-0533
    Keywords: Inclusion bodies ; Myositis ; Neuron ; Oligodendroglia ; Adrenal medulla
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 30-year-old man had had chronic progressive wasting and weakness of muscles for 17 years. A muscle biopsy 5 years prior to death revealed myopathic changes with rimmed vacuoles and intranuclear inclusions which corresponded to “inclusion body myositis”. At autopsy, intranuclear inclusions were observed in neurons, oligodendroglia, and inparenchymal cells of the adrenal medulla. Ultrastructurally, the inclusions in muscles, nervous tissue, and adrenal medulla were identical and consisted of abnormal tubulolinear structures measuring 10–20 nm in diameter. Similar inclusions have been reported in muscles with “inclusion body myositis” and in the nervous system with “neuronal intranuclear hyaline inclusion disease”, respectively. Absence of clinical symptoms related to the CNS and adrenal gland, and well-preserved parenchymal cells in these organs of our patient suggest a benign nature of the disease except in the muscular system. Attempts to isolate a virus from the brain were fruitless. This patient may serve to connect both diseases in muscles and the nervous system, and to disclose the etilogy of these inclusions.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 64 (1984), S. 85-88 
    ISSN: 1432-0533
    Keywords: Gerstmann-Sträussler-Scheinker's disease ; Spongiform encephalopathy ; Slow virus ; Experimental transmission ; Small rodents
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Spongiform encephalopathy was transmitted to mice from a patient belonging to the “Sch” family with Gerstmann-Sträussler-Scheinker's disease (GSS). Incubation periods in the first passage were much shorter than those in mice infected with Creutzfeldt-Jakob disease. Clinical and pathologic findings of mice infected with both disease were almost identical. This is the first successful transmission from a typical GSS case without severe spongiform change which suggests the possible transmissible nature of this disorder.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 7 (1967), S. 349-356 
    ISSN: 1432-0533
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Description / Table of Contents: Summary A case of spring-summer-encephalomyelitis (“tick-borne-encephalitis”) occuring in South Germany in 1940, is reported: A 32 year old male patient, who acquired the infection most likely in the southeastern part Bavaria, near the Austrian border, became suddenly sick with severe symptoms of meningitis, rapidly followed by increasing cloudiness of consciousness and coma. The patient died 5 days after onset of symptoms. When reviewing the history and the clinical data, there is a suggestion of tick-borne-encephalitis, which we assume with reasonable certainty on the basis of the histological findings. It is a “polioencephalomyelitis” of patchy distribution with meningitis affecting mainly the gray matter of the brain and spinal cord. The findings of this polioencephalomyelitis are consistent with those described as typical for this virus disease of the CNS.
    Notes: Zusammenfassung Bericht über einen Fall von Frühjahr-Sommer-Encephalomyelitis (“Zeckenencephalitis”) in Süddeutschland aus dem Jahre 1940. Der 32 jährige Patient, der die Infektion mit ziemlicher Wahrscheinlichkeit in Südost-Bayern erworben hatte, erkrankte plötzlich mit schweren meningitischen Symptomen, die von rasch zunehmender Bewußtseinstrübung und Coma gefolgt waren. Die Erkrankung führte bereits nach 5 Tagen zum Tode. Retrograd läßt sich aus den anamnestischen und klinischen Daten die Diagnose einer Zeckenencephalitis vermuten, die wir auf Grund des pathologischen Befundes als hinreichend gesichert annehmen. Es handelt sich um eine “Polioencephalomyelitis” in fleckförmiger Verteilung mit Meningitis, die in den wesentlichen Punkten mit den für diese Viruserkrankung des ZNS beschriebenen feingeweblichen Merkmalen übereinstimmt.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 79 (1989), S. 129-136 
    ISSN: 1432-0533
    Keywords: Ferritin ; Microglia ; Immunohistochemistry ; Scrapie-associated fibrils
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An immunohistochemical analysis of formalin-fixed, paraffin-embedded brain sections was performed with antisera against holoferritin and the light(L)-subunit of ferritin. Sections immunostained using anti-glial fibrillary acidic protein (GFAP), Ricinus communis agglutinin-1 (RCA-1) stain for microglia and iron stain (Berlin blue stain) were compared. The L-subunit of ferritin was purified from normal human spleen according to the modified scrapie-associated fibrils purification, and the antiserum was raised in a rabbit. Both ferritin antisera positively stained resting and, more markedly, reactive microglia, both of which were also stained with RCA-1 but not with GFAP. Ferritin-positive resting microglia were seen more abundantly in cerebral and cerebellar cortices than in white matter. The advantages of ferritin antisera over RCA-1 are as follows. (1) RCA-1 heavily stains blood vessels, while anti-ferritin does not, hence the microglial cells are more readily visualized with ferritin immunohistochemistry. (2) Reactive microglia and macrophages are more strongly stained with anti-ferritin. (3) The staining intensity of ferritin is independent of the length of tissue fixation in formalin. However, anti-ferritin is inferior to RCA-1 in staining resting microglia with a scanty cytoplasm, especially in the white matter, probably because the former recognizes cytoplasmic components, while the latter recognizes cell membrane. Iron stain only gave a reaction to microglial cells in brains with neurosyphilis and to hemosiderin-laden macrophages. Thus, in addition to RCA-1, ferritin antisera are useful as a microglia marker in formalin-fixed, paraffin-embedded sections.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-0533
    Keywords: Parvalbumin ; Ca2+-binding protein ; Interneuron ; Alzheimer's disease
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Immunohistochemical localization of the Ca2+-binding protein parvalbumin (PV) was investigated in the adult human central nervous system (CNS). The antiserum against purified rat skeletal muscle PV specifically recognized certain neuronal populations and their processes. Strongly positive were Purkinje, basket and stellate cells of the cerebellum, cerebral cortical nonpyramidal cells, and neurons in the thalamic reticular and ventrolateral nuclei, subthalamic nucleus, lateral and medial geniculate bodies, vestibular and cochlear nuclei, spinal trigeminal nucleus, cuneate and gracile nuclei, and dorsal nucleus of Clarke. Negative were cortical pyramidal neurons, neurons of the autonomic nerves, and neurons in the caudate nucleus, putamen, dentate nucleus, inferior olive, and substantia gelatinosa. The number and size of PV-immunoreactive neurons were significantly decreased in Alzheimer's disease. However, the decrease was not disease specific.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 82 (1991), S. 260-265 
    ISSN: 1432-0533
    Keywords: Immunohistochemistry ; Gerstmann-Sträussler syndrome ; Alzheimer's disease ; Kuru plaque ; Senile plaque
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We investigated paraffin-embedded brain sections from three patients with Gerstmann-Sträussler syndrome and three patients with Alzheimer's disease or senile dementia of Alzheimer type using anti-human prion protein antisera and anti-β/A4 protein antisera after protein denaturation treatments. After incubation with guanidine-thiocyanate, trichloroacetate, and phenol, the immunoreactivity of kuru plaques and senile plaques was enhanced to the same level as the formic acid treatment. These treatments revealed small compact amyloid deposits, amyloid deposits surrounding the plaque cores, and diffuse plaques. Most of these chemicals changed the congophilia of both amyloids. It is possible that these treatments denature amyloid fibril proteins and break down the structure of amyloid fibrils, thus revealing buried epitopes.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-0533
    Keywords: Modified tau ; Subcortical nuclei ; Alzheimer's disease ; Progressive supranuclear palsy ; Normal brain
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Tau-positive neurons relating to neurofibrillary tangles and diffuse cytoplasmic stainings were quantitatively examined in the brains of 61 nondemented persons including 24 age-matched controls, 10 patients with Alzheimer's disease (AD) and 5 with progressive supranuclear palsy (PSP). In nondemented persons, the locus ceruleus (LC) was found to contain tau-positive neurons initially in persons in their 30s, whereas the hippocampus contained such neurons initially in persons in their 40s. The LC had a higher incidence and density than the hippocampus in almost all age classes. As neuronal tau accumulation is considered a histological change occurring with normal aging, the LC might be involved in the earliest aging in the normal brain. In AD there was conspicuous tau accumulation in the same sites which were vulnerable to tau accumulation in the age-matched controls. In PSP tau accumulated heavily in a set of sites different from the age-matched controls and AD. Thus, subcortical tau accumulation in AD is increased far more than that under normal aging process, while that in PSP is not simply in an increased state of the normal aging process.
    Type of Medium: Electronic Resource
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