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  • 11
    Electronic Resource
    Electronic Resource
    Rapidly progressive aphasia and motor neuron disease: a clinical, radiological, and pathological study of an autopsy case with circumscribed lobar atrophy (2000)
    Springer
    Acta neuropathologica 99 (2000), S. 81-87 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Circumscribed lobar atrophy ; Dementia ; Neuropathology ; Progressive aphasia
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns an autopsy case of rapidly progressive aphasia and motor neuron disease. The patient was a Japanese woman who was 75 years old at the time of death. The family history did not reveal hereditary burden. She developed language disturbances and difficulty in swallowing at age 74. Neurological examination 1 month after the disease onset revealed motor aphasia without dementia and bulbar sign, followed by muscle weakness of the four extremities. Neuroradiological examination revealed progressive atrophy of the anterior part of the left temporal lobe. She died of respiratory difficulty 10 months after the disease onset. Macroscopically, neuropathological examination showed circumscribed atrophy of the left perisylvian region and, histologically, neuronal loss in the cerebral cortex, including the primary motor area, substantia nigra, brain stem motor nuclei, and anterior horns of the spinal cord, in addition to obvious degeneration of the pyramidal tracts and presence of Bunina bodies. Ubiquitin-immunoreactive neuronal inclusions were present in the hippocampal dentate granular cells and frontotemporal cortical layer II neurons. Based on these clinicopathological findings and a review of the literature, we concluded that our case is the first reported case of amyotrophic lateral sclerosis with dementia that clinically showed rapidly progressive aphasia.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/PL00007411
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  • 12
    Electronic Resource
    Electronic Resource
    Dentatorubropallidoluysian atrophy: clinicopathological study of dementia and involvement of the nucleus basalis of Meynert in seven autopsy cases (1998)
    Springer
    Acta neuropathologica 96 (1998), S. 502-508 
    Details
    ISSN: 1432-0533
    Keywords: Key words Dentatorubropallidoluysian atrophy ; Dementia ; Nucleus basalis of Meynert ; Neuropathology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns a clinicopathological study including a quantitative pathological study on the nucleus basalis of Meynert (nbM) of seven Japanese autopsy cases (four male, three female) of dentatorubropallidoluysian atrophy (DRPLA) with special reference to the clinicopathological correlation of dementia in DRPLA. In each case the pattern of the inheritance was consistent with that of an autosomal dominant trait. The neurological examination revealed that all seven individuals had cerebellar signs. Six patients had epilepsy and choreoathetoid involuntary movement; myoclonus was evident in five patients. Dementia was noted in all seven patients. Degeneration of the globus pallidus (particularly the lateral segment) and of the dentate nucleus was the principal pathological feature. Brain weights at autopsy ranged from 1020 to 1400 g (average 1241 g: male 1320 g, female 1135 g). The quantitative evaluation revealed no significant loss of neurons in the nbM as compared with a control group. There was no clinicopathological correlation between dementia and involvement of the nbM. We suggest that the dementia of DRPLA is due not to the involvement of the nbM, but to – as yet – unidentified pathology elsewhere.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050925
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  • 13
    Electronic Resource
    Electronic Resource
    Sporadic amyotrophic lateral sclerosis with multiple system degeneration: a report of an autopsy case without respirator administration (1999)
    Springer
    Acta neuropathologica 98 (1999), S. 512-515 
    Details
    ISSN: 1432-0533
    Keywords: Key words Amyotrophic lateral sclerosis ; Dentate ; nucleus ; Multiple system degeneration ; Respirator ; Substantia nigra
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract This report concerns an autopsy case of amyotrophic lateral sclerosis (ALS) with unusual clinical and neuropathological findings. The patient was a Japanese man without hereditary burden who was 49 years old at the time of death. His clinical manifestation included dysarthria at age 48, followed by dysphagia, atrophy and fasciculation of the tongue, muscle weakness in the four extremities, tremor, rigidity, increased deep tendon reflexes in the upper and lower extremities, and incoordination of the four extremities. He died of respiratory failure 12 months after the disease onset. No respirator administration was performed throughout the clinical course. The neuropathological examination revealed not only degeneration of upper and lower motor neuron systems, including the presence of Bunina bodies and ubiquitin-immunoreactive neuronal inclusions in the lower motor neurons, but also prominent degeneration of the substantia nigra and dentate nucleus with slight neuronal loss in the locus ceruleus and pontine nucleus. To our knowledge, this is the first reported case of sporadic ALS without dementia and respirator support, showing degeneration of the substantia nigra and dentate nucleus. This report may contribute to the resolution of the question concerning the neuropathological heterogeneity of sporadic ALS with respiratory support.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010051117
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  • 14
    Electronic Resource
    Electronic Resource
    Distribution of cerebral cortical lesions in corticobasal degeneration: a clinicopathological study of five autopsy cases in Japan (1997)
    Springer
    Acta neuropathologica 94 (1997), S. 416-424 
    Details
    ISSN: 1432-0533
    Keywords: Key words Cerebral cortical lesion ; Clinicopathological correlation ; Corticobasal degeneration ; Pick’s disease ; Progressive supranuclear palsy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We investigated five Japanese patients with autopsy-proven corticobasal degeneration (CBD) both clinically and pathologically, and examined the distribution of their cerebral cortical lesions in hemisphere specimens. The lesions were classified into three categories (slight, moderate and severe). Only two of our patients had clinical features considered to be typical of CBD. Severe lesions were present in the posterior portions of the frontal lobe, anterior to the precentral gyrus in two patients with the clinical diagnosis of CBD. By comparison, in two patients with clinically diagnosed frontal Pick’s disease, and one with the clinical diagnosis of progressive supranuclear palsy (PSP), severe lesions were seen in the anterior portions of the frontal lobe. The primary motor area of all five had mostly slight to moderate lesions. We postulate that the clinical features of CBD have a much wider spectrum than previously believed. Our data also indicate that the lesion responsible for limb-kinetic apraxia in CBD is in the premotor cortex. We suggest that when the anterior portions of the frontal lobe are damaged, the clinical picture mimics those of Pick’s disease and PSP. In addition, we consider that focal cerebral atrophy of CBD is multicentric.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/s004010050728
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  • 15
    Electronic Resource
    Electronic Resource
    Familial amyotrophic lateral sclerosis with onset in bulbar sign, benign clinical course, and Bunina bodies: a clinical, genetic, and pathological study of a Japanese family (2000)
    Springer
    Acta neuropathologica 100 (2000), S. 603-607 
    Details
    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
    URL: http://dx.doi.org/10.1007/s004010000237
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  • 16
    Electronic Resource
    Electronic Resource
    Cystic islet cell tumors: Radiologic findings in three cases (1994)
    Springer
    Abdominal imaging 19 (1994), S. 225-228 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00203512
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  • 17
    Electronic Resource
    Electronic Resource
    Adaptive modification of membrane phospholipid fatty acid composition and metabolic thermosuppression of brown adipose tissue in heat-acclimated rats (2000)
    Springer
    International journal of biometeorology 43 (2000), S. 163-168 
    Details
    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
    URL: http://dx.doi.org/10.1007/s004840050003
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  • 18
    Electronic Resource
    Electronic Resource
    Moyamoya disease: diagnosis with three-dimensional CT angiography (1994)
    Springer
    Neuroradiology 36 (1994), S. 432-434 
    Details
    ISSN: 1432-1920
    Keywords: Moyamoya disease ; Computed tomography ; Three-dimensional imaging ; Angiography
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Our purpose was to assess the value of threedimensional (3D) CT angiography in the diagnosis of moyamoya disease. We studied seven patients with moyamoya disease proved by conventional angiography. Three-dimensional (3D) CT angiography was performed using rapid sequence or helical (spiral) scanning in conjunction with a bolus injection of intravenous contrast medium. All seven patients could be diagnosed as having moyamoya disease on the basis of the following 3D CT angiographic findings: poor visualisation of the main trunks and/or major branches of anterior and middle cerebral arteries (7 patients); dilated leptomeningeal anastomotic channels from the posterior cerebral arteries (4); and demonstration of “moyamoya vessels” in the basal ganglia (2). Although conventional angiography remains the principal imaging technique for demonstrating anatomical changes in detail, less invasive 3D CT angiography provides a solid means of diagnosing moyamoya disease when it is suspected on CT, MRI, or clinical grounds.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00593677
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  • 19
    Electronic Resource
    Electronic Resource
    Contrast-enhanced magnetic resonance imaging of sub- and epidural empyemas (1992)
    Springer
    Neuroradiology 34 (1992), S. 494-496 
    Details
    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
    URL: http://dx.doi.org/10.1007/BF00598958
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  • 20
    Electronic Resource
    Electronic Resource
    MRI appearances of calcified regions within intracranial tumours (1993)
    Springer
    Neuroradiology 35 (1993), S. 341-344 
    Details
    ISSN: 1432-1920
    Keywords: Brain tumour ; Calcification ; Magnetic resonance imaging ; Gadopentetate dimeglumine
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The MRI appearances of calcification within intracranial tumours was assessed by reviewing MR images of 11 calcified tumours documented by CT. The signal intensity of the calcified regions was varied and nonspecific on both T1-and T2-weighted images. They were seen as signal void exclusively on T2-weighted images in only 2 patients. Gadolinium enhancement of the calcified portion occurred in 7 of 10 patients. These findings reflect the presence of tumour parenchyma within the calcified region, as proved in 5 lesions examined histologically.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00588364
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