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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 75 (1988), S. 345-353 
    ISSN: 1432-0533
    Keywords: Lewy body ; Immunocytochemistry ; Ubiquitin ; Paired helical filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The nature of Lewy bodies (LBs) in the brain stem and cerebral cortex in five cases of diffuse Lewy body disease and one case of Parkinson's disease with dementia were investigated immunocytochemically with various antibodies to cytoskeletal proteins, paired helical filaments (PHF) and ubiquitin. Antibodies to 200-kDa component of neurofilament, tau and PHF showed no significant reactions with most of LBs. Antibodies to high-molecular weight microtubule-associated proteins (HMWMAPs) moderately stained the periphery of a few of LBs. A monoclonal antibody to PHF (DF2) which recognizes ubiquitin, and polyclonal antibodies to ubiquitin immunostained virtually all of the typical and cortical LBs as intensely as Alzheimer's neurofibrillary tangles and senile plaque neurites: the periphery of LBs was darkly stained, whereas the central core of typical LBs and central zone of cortical LBs were less intensely stained or remained unstained. Immunoelectron microscopy of the LBs with DF2 revealed that immune reaction products were located on the filaments exclusively in the periphery of LBs, but not on those in the center. These findings suggest that both types of LBs are immunocytochemically indistinguishable despite some structural differences, and that peripherally located filaments in LBs are tagged with ubiquitin, an element required for the ATP-dependent proteolysis system in the cell. Antibodies to ubiquitin are the most useful marker of LBs ever known.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Familial amyotrophic lateral sclerosis ; Lewy body-like hyaline inclusion ; Ubiquitin ; Neurofilament
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Lewy body-like hyaline inclusion (LI) in the neuronal soma and swollen cord-like processes is a characteristic feature in the anterior horn cells and neurons in thoracic nucleus (Clarke) of familial amyotrophic lateral sclerosis (ALS) with posterior column involvement. We have studied the LI in the case of two sisters with this disorders. Microscopically the LI consists of an eosinophilic “core” surrounded by a basophilic “halo”. Ultrastructurally the core consists of granule-associated filaments, while the halo consists of normal-looking neurofilament. Immunocytochemistry with anti-ubiquitin antibody shows that these granule-associated filaments in the core are highly ubiquitinated, while the normal-looking neurofilaments in the halo are not recognized by antiubiquitin antibody. Our study proves that LI consists of an aggregation of ubiquitinated filaments among a neurofilamentous accumulation, possibly representing a form of neuronal cytoskeletal disorganization in familial ALS.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 62 (1984), S. 194-200 
    ISSN: 1432-0533
    Keywords: Tubulomembranous structure ; Fingerprint-like structure ; Distal myopathy ; Rimmed vacuole ; Autophagic vacuole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Muscle biopsies from four patients were studied histochemically and electron-microscopically: they had myopathy of juvenile or early-adult onset, in which distal limb muscles were most severely affected but muscles supplied by cranial nerves were spared. Common histochemical findings included variation in fiber size, necrosis, phagocytosis, fiber splitting, central nuclei, endomysial fibrosis, and particularly rimmed vacuoles. Electron-microscopic examination revealed frequent autophagic vacuoles with numerous myeloid bodies. In addition, sarcoplasmic inclusion bodies with periodically laminated structures similar to the tubulomembranous structures (TMSs) first described by Fukuhara et al. (1981) in an atypical myopathy were found in all four cases, and in one, there were fingerprint-like structures resembling those described in neuronal ceroid-lipofuscinoses. These inclusions occasionally contained areas resembling lipofuscin pigment. They are certainly residual bodies of lysosomal origin, which might be related to the rimmed-vacuolar degeneration of the muscle, but whether or not they represent some specific metabolic abnormalities seems to remain an open question since the present cases differed clinically from either of the atypical myopathies with TMSs (Fukuhara et al. 1981) or any type of neuronal ceroid-lipofuscinosis.
    Type of Medium: Electronic Resource
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  • 4
    ISSN: 1432-0533
    Keywords: Curly fibers ; Anti-tau antibody ; Immunoelectron microscopy ; Rapid-freeze and deep-etch method ; Neuritic paired helical filaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The ultrastructure of the curly fibers was examined by the transmission and immunoelectron microscopy as well as by the rapid-freeze, deep-etch and replica method. The curly fibers consisted mainly of paired helical filaments (PHF) in the neuropils, both pre- and post-synaptic. On the deep-etch replicas, PHF in the neurites had similar dimensions to those of Alzheimer's neurofibrillary tangles in the nerve cell somata, having the width of 28 to 36 nm. The two component filaments, 14 to 18 nm in width, were twisted left-handedly with about 70- to 90-nm intervals. There were also cross-bridging fibrils of about 6 nm in diameter connecting the neighboring PHF.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    Amsterdam : Elsevier
    Journal of Molecular Structure: THEOCHEM 280 (1993), S. 261-272 
    ISSN: 0166-1280
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Chemistry and Pharmacology , Physics
    Type of Medium: Electronic Resource
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  • 6
    ISSN: 1364-6753
    Keywords: Keywords: amyotrophic lateral sclerosis, high incidence area, Cn/Zn superoxide dismutase, mutation, low penetrance, neurofilament
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: ABSTRACT Unusually high incidences of amyotrophic lateral sclerosis (ALS) have been observed in the natives of the Kii Peninsula of Japan as well as the indigenous Chamorro people of Guam. Given the relatively high incidence of familial onset of the disease in the Kii Peninsula, we performed mutational analyses of the SOD1 gene of 23 patients (three familial cases and 20 sporadic cases) with ALS from the Kii Peninsula and its vicinity. In two of the 23 patients, we identified the same missense mutation (substitution of Thr for Ile 113) in exon 4 as a heterozygous state. The Ile113Thr mutation in the SOD1 gene has been identified in some familial as well as sporadic cases with ALS, as a mutation with a low penetrance. This mutation has been reported to be associated with the formation of neurofibrillary tangles in an English family, which is a characteristic feature of ALS in the Kii Peninsula. These results suggest that the Ile113Thr mutation is a characteristic and relatively prevalent mutation in this area.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1432-1459
    Keywords: Toxic leucoencephalopathy ; Carmofur ; 5-FU derivative ; Cerebral CT
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Three cases of leucoencephalopathy induced by carmofur (1-hexylcarbamoyl-5-fluorouracil), an antineoplastic derivative of 5-fluorouracil are reported and the literature is reviewed. Initial symptoms were unsteady gait and dementia developing several weeks or months after carmofur had been started. Symptoms increased gradually even after stopping the drug. Severe encephalopathy with confusion, delirium or coma appeared frequently. Symptoms were usually reversible but death occasionally occurred. The EEG showed marked slowing. Computed tomography of the brains of severely intoxicated patients showed marked hypodensity of the entire cerebral white matter. Carmofur must be discontinued immediately if any psychomotor symptoms develop.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 235 (1987), S. 90-94 
    ISSN: 1432-1459
    Keywords: Alzheimer's disease ; Myoclonus ; Cortical myoclonus ; Subcortical myoclonus
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Myoclonus was studied electrophysiologically in seven patients with clinically diagnosed Alzheimer's disease. There seem to be at least two physiological types of myoclonus in Alzheimer's disease. Cerebral cortical structures might participate in the generation of myoclonus in one type, while the other type is probably generated by subcortical structures.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-1459
    Keywords: Anticoagulant therapy ; Non-valvular atrial fibrillation ; Cerebral embolism ; Recurrent stroke
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary For the prevention of recurrent embolic stroke, 23 elderly patients with non-valvular atrial fibrillation (NVAF) were treated with oral anticoagulants (warfarin) during a mean period of 3.8 years. Only one patient suffered recurrent embolism, and another had acute myocardial infarction. There was no cerebral haemorrhage during the treatment. In an untreated control group (from an autopsy series), recurrent embolic strokes occurred in 18 of 70 NVAF patients (26%) during a mean period of 1.3 years. Long-term anticoagulant therapy appears to be effective in the prevention of recurrent embolic stroke in elderly patients with NVAF.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 1432-1459
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Type of Medium: Electronic Resource
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