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  • 1
    Electronic Resource
    Electronic Resource
    An autopsied case of the Crow-Fukase syndrome: a neuropathological study with emphasis on spinal roots (1990)
    Springer
    Acta neuropathologica 80 (1990), S. 563-567 
    Details
    ISSN: 1432-0533
    Keywords: Crow-Fukase syndrome ; Demyelination and remyelination ; Spinal root ; Dorsal tract degeneration ; Satellitosis
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary An autopsied case of the Crow-Fukase syndrome is reported. Neuropathological findings were as follows: (1) in the sural nerve, there was marked decrease of large and small myelinated fibers. Myelinated fibers showing axonal degeneration and segmental demyelination and remyelination were moderately increased. (2) In the lumbar spinal roots, myelinated fibers showing segmental demyelination and remyelination were frequently observed. The density of myelinated fibers of the ventral root was less at the dural site than the spinal site, while that of the dorsal roots was less at the spinal site than the dural site. (3) In the dorsal root ganglion, there were Nageotte's residual nodules and satellitosis; (4) in the lumbar and thoracic spinal cord, there was pallor of the dorsal column; and (5) nerve cells showing central chromatolysis were frequently observed in the spinal anterior horn cells. Segmental demyelination and remyelination in the spinal roots and loss of myelinated fibers with axonal degeneration in the sural nerve are fibers with axonal degeneration in the sural nerve are main neuropathological features of this syndrome.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294621
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  • 2
    Electronic Resource
    Electronic Resource
    Chronic demyelinating neuropathy and intra-axonal polyglucosan bodies (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 95-99 
    Details
    ISSN: 1432-0533
    Keywords: Polyglucosan body ; Neuropathy ; Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary In this study we evaluated the relationship between polyglucosan bodies and peripheral nerve lesions. The biopsied sural nerve from a patient with late-onset chronic sensori-motor neuropathy showed many intra-axonal polyglucosan bodies and segmental demyelination/remyelination. The formation of Schwann cell hyperplasia around the demyelinated axons was found at the sites of polyglucosan bodies. These findings suggest that demyelinating neuropathy is a part of the spectrum of the diseases characterized by the accumulation of polyglucosan bodies within cellular compartments.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00454906
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  • 3
    Electronic Resource
    Electronic Resource
    Experimental germanium myopathy (1991)
    Springer
    Acta neuropathologica 82 (1991), S. 55-59 
    Details
    ISSN: 1432-0533
    Keywords: Germanium intoxication ; Mitochondrial myopathy ; Ragged-red fiber ; Autophagic vacuole
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The long-term administration of germanium dioxide (GeO2) to rats produced Ge myopathy characterized by the formation of ragged-red fibers. The earliest pathological changes in experimental Ge myopathy were a decrease in cytochrome c oxidase activity and accumulation of high electron-dense materials in mitochondria. These findings suggest that a mitochondrial dysfunction may be most important in the genesis of experimental Ge myopathy, which could be a useful animal model for the investigation of and therapeutic trials for human mitochondrial myopathies.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00310923
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  • 4
    Electronic Resource
    Electronic Resource
    Histochemical and ultrastructural pathology of skeletal muscle in a patient with abetalipoproteinemia (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 529-531 
    Details
    ISSN: 1432-0533
    Keywords: Abetalipoproteinemia ; Muscle pathology ; Giant lysosome ; Concentric laminated body
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Pathological examination was carried out of the skeletal muscle of an 8-year-old boy with abetalipoproteinemia. The patient complained of diarrhea, and showed a deficiency of betalipoprotein, decreased fatsoluble vitamins, acanthocytosis and a mild incrase in serum creatine kinase. The prominent histochemical finding was punctate deposits of acid phosphatase activity in most fibers. Ultrastructural lesions revealed a number of giant lysosomes. Although these pathological findings seemed to be related to vitamin E deficiency, other pathological findings such as concentric laminated bodies of filamentous bodies were also observed. The clinical course and the changes in the pathological findings in our patient after long-term vitamin E therapy need to be observed.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00228592
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  • 5
    Electronic Resource
    Electronic Resource
    Dominantly inherited motor and sensory neuropathy with excessive myelin folding complex (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 602-608 
    Details
    ISSN: 1432-0533
    Keywords: Excessive myelin folding ; Segmental demyelination ; Dominant inheritance ; Globule ; Hereditary motor and sensory neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract The two patients in a family having the clinical and electrodiagnostic features of hereditary motor and sensory neuropathy (HMSN) are described. The main histological features of sural nerve were segmental demyelination and remyelination with moderate to marked loss of myelinated fibers, and myelin folding complex along all of the large and small myelinated fibers. These features appeared morphologically similar to those observed in HMSN with excessive myelin outfolding, or globular neuropathy. Southern blot analysis suggests that there were neither duplication nor deletion of the peripheral myelin protein-22 gene in the patients. The presented two patients may be a rare form of dominantly inherited HMSN with myelin folding complex.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294299
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  • 6
    Electronic Resource
    Electronic Resource
    Experimental germinaium dioxide-induced neuropathy in rats (1993)
    Springer
    Acta neuropathologica 86 (1993), S. 547-553 
    Details
    ISSN: 1432-0533
    Keywords: Germanium dioxide ; Neurotoxicity ; Demyelinating neuropathy ; Nerve edema
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract We report and experimental model of germanium dioxide (GeO2)-induced neuropathy in rats. More than 6 months administration of GeO2 to young rats produced neuropathy characterized by segmental demyelination/remyelination and nerve edema. Electron microscopic studies demonstrated that changes in Schwann cells, such as an increased cytoplasmic volume or disintegration of the cytoplasm, were the earliest pathological findings. Schwann cell mitochondria contained high electron-dense materials. Subsequent removal of necrotic Schwann cell debris and myelin by invading macrophages was evident. These findings suggested that the Schwann cells themselves are the primary target of the toxin. The deposition of electron-dense granules in the intra-axonal vesicles, which was suggestive of glycogen granules in mitochondria, was observed in the advanced stage of the neuropathy. The findings of endoneurial edema with splitting of myelin lamellae were noted at the early stage of demyelination. Nerve edema may be the result of GeO2-induced endothelial cell injury.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00294291
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  • 7
    Electronic Resource
    Electronic Resource
    Polyneuropathy induced by m-tolyl methyl carbamate intoxication (1991)
    Springer
    Journal of neurology 238 (1991), S. 47-48 
    Details
    ISSN: 1432-1459
    Keywords: Carbamate ; Polyneuropathy ; Axonal degeneration
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 55-year-old woman who attempted suicide by ingesting 200 ml of m-tolyl methyl carbamate (MTMC) is reported. She was comatose for 3 days and, upon recovery, had notable paraesthesia in her lower limbs and difficulty in walking. Neurological examination revealed sensorimotor polyneuropathy. Sural nerve biopsy revealed marked axonal degeneration with a moderate decrease of myelinated fibres.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00319711
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  • 8
    Electronic Resource
    Electronic Resource
    Cerebrotendinous xanthomatosis: cranial CT and MRI studies in eight patients (1992)
    Springer
    Neuroradiology 34 (1992), S. 308-312 
    Details
    ISSN: 1432-1920
    Keywords: Cerebrotendinous xanthomatosis ; Computed tomography ; Magnetic resonance imaging
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary We report the findings on cranial computed tomography (CT) and magnetic resonance imaging (MRI) and their correlation with the clinical manifestations, disease severity and biochemical abnormalities in eight patients with cerebrotendinous xanthomatosis. CT revealed cerebral atrophy in seven cases, cerebellar atrophy in four and focal low density lesions in the cerebral white matter in two. T2-weighted MRI showed high signal lesions in the cerebral white matter, focal in four cases and diffuse in one, and in the globus pallidus in three patients, two of whom also had lesions in the cerebellar white matter. While severely affected patients showed variable CT and MRI abnormalities, our cases did not show the dramatic findings expected from the neurological manifestations. Diffuse lesions in the cerebral and cerebellar white matter have been emphasized in previous reports, but in our study the focal lesions in the cerebral white matter were also present; the globus pallidus was frequently involved.
    Type of Medium: Electronic Resource
    URL: http://dx.doi.org/10.1007/BF00588188
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