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  • 1
    ISSN: 1432-0533
    Keywords: Key words Betz cells ; Multiple system atrophy ; Neuropathology ; Pyramidal signs ; Pyramidal tract ¶degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated clinicopathologically the pyramidal signs, including spasticity, hyperreflexia, and Babinski’s sign, and the involvement of the pyramidal tract and primary motor cortex, in seven Japanese autopsy cases of multiple system atrophy (MSA). Pyramidal signs were observed in six (86%) of the seven autopsy cases. Hyperreflexia and Babinski’s sign were each evident in five patients, but spasticity was observed in only one patient. Loss of Betz cells and presence of glial cytoplasmic inclusions in the primary motor cortex were noticed in all seven cases. Astrocytosis in the fifth layer of the primary motor cortex was noticed in five cases, but its presence was not related to the duration of the disease. Involvement of the pyramidal tract in the spinal cord, particularly of the small myelinated fibers, was observed in all seven cases, but no involvement of the pyramidal tract in the midbrain was evident in any of the six cases in which this structure was examined. In MSA, pyramidal signs were shown to be present more frequently than believed before, and the clinicopathological correlation between pyramidal signs and involvement of the pyramidal tract was obvious. Constant involvement of Betz cells in MSA has not been reported. Our clinicopathological findings may also make a contribution to the understanding of the clinicopathological hallmarks of MSA.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0509
    Keywords: Pancreas, neoplasms ; Islet cell tumors, diagnosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report three cases of cystic islet cell tumors, two caused by gastrinomas and the other by an islet cell carcinoma. All three patients underwent computed tomography (CT) and angiography and two also had magnetic resonance (MR) imaging. Several common radiographic findings were present as follows: thickening of the cyst wall and irregularity of the inner surface on postcontrast CT and MR images, neovascularity and a densely staining hypervascular rim on angiography, and moderately increased signal intensity of the cyst content on T1-weighted MR images, which indicated fluid containing blood and/or necrotic tissue. These findings are helpful in defining the nature of these lesions and strongly suggest that cystic islet cell tumor should be included in the differential diagnosis.
    Type of Medium: Electronic Resource
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  • 3
    ISSN: 1432-0533
    Keywords: Key words Bunina bodies ; Cu/Zn superoxide ; dismutase ; Familial amyotrophic lateral sclerosis ; Neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report a Japanese family with autosomal dominant adult-onset amyotrophic lateral sclerosis (FALS) with onset in the bulbar musculature, clinically benign course, absence of the Cu/Zn superoxide dismutase-1 (SOD 1) gene mutation, and many Bunina bodies, in addition to involvement of the upper and lower motor neurons. The proband was a Japanese woman who was 66 years old at the time of death. Family history disclosed five patients with FALS over three generations. She developed dysarthria at age 57, followed by dysphagia, muscle weakness of the upper extremities, and difficulty in respiration. She could walk without support until her death. The elder sister of the proband developed dysarthria at age 48 and died at age 58. A genetic study of the nephew of the proband showed the absence of a mutation in the SOD 1 gene. Neuropathological examination of the proband disclosed neuronal loss in the upper and lower motor neurons, and numerous Bunina bodies in the lower motor neurons without Lewy body-like inclusions or ubiquitin-immunoreactive neuronal inclusions. No degeneration of the Clarke’s column, middle root zone of the posterior column, or posterior spinocerebellar tract was present. Review of the literature revealed that only patients with FALS with a long survival period of over 5 years had pathological findings consistent with FALS with posterior column involvement. This study contributes to the elucidation of the clinicopathological heterogeneity of FALS.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 81 (1990), S. 155-161 
    ISSN: 1432-0533
    Keywords: Pick's disease ; Substantia nigra ; Pigmented neurons ; Morphometry ; Parkinsonism
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Loss of neurons from the substantia nigra (SN), which is often encountered in Pick's disease, was quantitatively analyzed in 13 cases of Pick's disease and 19 age-matched controls. On sections from the upper and lower portions of the SN, the pigmeted zone (zona compacta) and the non-pigmented zone (zona reticulata) were delineated, and these zones were partitioned into quarters: medial, mid-medial, mid-lateral and lateral. Neuronal loss was fairly severe and more evident in the upper section of the SN (−40%), especially in the midmedial and lateral quarters. In the lower section (neuronal loss:−28%), the medial quarter was most severely affected. Non-pigmented neurons were preserved. Fibrillary gliosis was denser in the zona reticulata, where neuronal loss was minimal. These findings revealed a selective vulnerability of nigral neurons according to their topography and pigmentation and suggests the primary involvement of some neuronal groups (especially the pigmented neurons) of the SN in Pick's disease.
    Type of Medium: Electronic Resource
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  • 5
    Electronic Resource
    Electronic Resource
    [S.l.] : International Union of Crystallography (IUCr)
    Acta crystallographica 46 (1990), S. 514-517 
    ISSN: 1600-5724
    Source: Crystallography Journals Online : IUCR Backfile Archive 1948-2001
    Topics: Chemistry and Pharmacology , Geosciences , Physics
    Notes: The absolute values of the structure factors of diamond are determined for nine low-order reflections by measuring the X-ray Pendellösung beats on the wavelength scale. Parallel-sided wafers of synthetic diamond single crystals are used for specimens. The deformation charge density and the Debye-Waller B factor are evaluated from the structure factors. The charge density of pile-up electrons is estimated to be 0.44(17) eÅ-3 at the midpoint between the nearest-neighbour atoms. The density is slightly smaller than that determined by the powder diffraction method. The obtained B factor, 0.142 (9) Å2, is in good agreement with that evaluated to date from neutron diffraction measurements.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Oxford, UK : Blackwell Science Ltd
    Plant pathology 52 (2003), S. 0 
    ISSN: 1365-3059
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Agriculture, Forestry, Horticulture, Fishery, Domestic Science, Nutrition
    Notes: Phenotypic and genetic characteristics of nine bacterial strains isolated from mulberry (Morus spp.), which were originally described as Erwinia carotovora ssp. carotovora (Ecc), were investigated. Based on the results of biochemical tests, these bacterial strains were divided into two different types, type 1 and type 2. Two strains of type 1 were similar to Ecc, whereas seven strains of type 2 were distinct from Ecc. A polyphasic study that included serological assay, specific PCR assay for E. carotovora ssp. atroseptica (Eca), PCR-RFLP of a pectate lyase (pel) gene and RAPD-PCR was performed on the type 2 strains, and the data were compared with those of related E. carotovora subspecies. The results of serological and specific PCR assays for Eca showed that the type 2 strains were distinct from Eca. In RFLP analysis of the pel gene using Sau3AI, the type 2 strains showed a unique RFLP pattern. On the basis of RAPD analysis, similarity of RAPD patterns within the type 2 strains was very high. A unique RAPD fragment was isolated from the type 2 strains and used as a probe for Southern hybridization. This probe hybridized only with PCR products from the type 2 strains. Based on phenotypic, serological and genetic characteristics, the type 2 strains isolated from mulberry may belong to a distinct E. carotovora subspecies other than Eca or Ecc.
    Type of Medium: Electronic Resource
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  • 7
    ISSN: 1365-2133
    Source: Blackwell Publishing Journal Backfiles 1879-2005
    Topics: Medicine
    Notes: Summary  While β2-microglobulin amyloidosis occurring in patients undergoing long-term dialysis is frequently associated with joint involvement, skin lesions have rarely been encountered. We report a 57-year-old man with extensive subcutaneous amyloid deposition forming large nodules on the buttocks; the patient had been on maintenance dialysis for 28 years. Although this condition is rare, a review of the literature indicates that the majority of such lesions occur around the buttock region.
    Type of Medium: Electronic Resource
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  • 8
    ISSN: 1432-1254
    Keywords: Key words Brown adipose tissue ; Fatty acid profile ; Docosahexaenoic acid (DHA) ; Heat acclimation ; In vitro oxygen consumption
    Source: Springer Online Journal Archives 1860-2000
    Topics: Geography , Physics
    Notes: Abstract  Thermogenesis, especially facultative thermogenesis by brown adipose tissue (BAT), is less important in high ambient temperature and the heat-acclimated animals show a lower metabolic rate. Adaptive changes in the metabolic activity of BAT are generally found to be associated with a modification of membrane phospholipid fatty acid composition. However, the effect of heat acclimation on membrane phospholipid fatty acid composition is as yet unknown. In this study, we examined the thermogenic activity and phospholipid fatty acid composition of interscapular BAT from heat-acclimated rats (control: 25±1°C, 50% relative humidity and heat acclimation: 32±0.5°C, 50% relative humidity). Basal thermogenesis and the total thermogenic capacity after noradrenaline stimulation, as estimated by in vitro oxygen consumption of BAT (measured polarographically using about 1-mm3 tissue blocks), were smaller in the heat-acclimated group than in the control group. There was no difference in the tissue content of phospholipids between the groups when expressed per microgram of DNA. The phospholipid fatty acid composition was analyzed by a capillary gas chromatograph. The state of phospholipid unsaturation, as estimated by the number of double bonds per fatty acid molecule, was similar between the groups. The saturated fatty acid level was higher in the heat-acclimated group. Among the unsaturated fatty acids, heat acclimation decreased docosahexaenoic acid and oleic acid levels, and increased the arachidonic acid level. The tissue level of docosahexaenoic acid correlated with the basal oxygen consumption of BAT (r=0.6, P〈0.01) and noradrenaline-stimulated maximum values of oxygen consumption (r=0.5, P〈0.05). Our results show that heat acclimation modifies the BAT phospholipid fatty acids, especially the n-3 polyunsaturated fatty acid docosahexaenoic acid, which is possibly involved in the metabolic thermosuppression.
    Type of Medium: Electronic Resource
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  • 9
    Electronic Resource
    Electronic Resource
    Springer
    Journal of neurology 237 (1990), S. 103-106 
    ISSN: 1432-1459
    Keywords: Vitamin E deficiency ; Fat malabsorption ; Diabetes mellitus ; Chronic pancreatitis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Thirteen patients with adult-onset vitamin E deficiency due to fat malabsorption were investigated clinically and electrophysiologically. These patients had slightly or moderately decreased serum vitamin E (1.7–4.8 μg/ml, normal 〈6.0) or vitamin E/cholesterol ratio (0.21–0.31 mg/g, normal 〈0.35). Only one patient had typical neurological manifestations of vitamin E deficiency, which improved with supplementary vitamin E. The pathological findings in this patient were also compatible with vitamin E deficiency. This patient had poorly controlled diabetes mellitus due to advanced chronic pancreatitis. Reviewing previously reported cases of vitamin E deficiency with diabetes mellitus in chronic pancreatitis, the duration of deficiency until the onset of symptoms was shorter than in those cases without complications. Although adult patients with early, slight deficiency of vitamin E are generally asymptomatic, patients with diabetes mellitus tend to have early neurological symptoms. The vitamin E tolerance test should be used, because even in some patients with vitamin E deficiency due to malabsorption, the deficiency can be overcome by large oral doses of vitamin E.
    Type of Medium: Electronic Resource
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  • 10
    Electronic Resource
    Electronic Resource
    Springer
    Neuroradiology 34 (1992), S. 494-496 
    ISSN: 1432-1920
    Keywords: Subdural empyema-Epidural empyema ; Gadopentetate dimeglumine ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Contrast-enhanced magnetic resonance images (MRI) of three patients with subdural (SDE) and two with epidural empyemas (EDE) were reviewed. In each case, the capsule of the lesion demonstrated enhancement, and distinction between capsule and contents was obvious on contrast-enhanced images. In SDE, contrast-enhanced images clearly depicted thickening of the neighbouring dura mater and a co-existent brain abscess. In EDE, part of the displaced dura mater did not enhance, which facilitated differentiation from SDE. Contrast-enhanced MRI was thus of value in diagnosis.
    Type of Medium: Electronic Resource
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