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  • 1
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 36 (1976), S. 21-30 
    ISSN: 1432-0533
    Keywords: Status spongiosus ; Intramyelinic oedema ; Actinomycin D
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The effect on central myelin of Actinomycin D, an RNA — and, secondarily, a protein-synthesis inhibitor, has been studied by light and electron microscopy. The intracranial injection of this drug produced an extensivestatus spongiosus of the white matter in the cerebrum, cerebellum, brain stem and optic nerve within 48 h. The status spongiosus was due to vacuole formation within the myelin sheath and to enlargement of the extracellular space. Three types of vacuoles were observed: (a) the most common varieties formed between the inner tongue and the remainder of the myelin sheath; (b) a second variety formed by enlargement of the periaxonal space with separation of the axon from its myelin sheath, and (c) a less common type of vacuolization was due to splitting of the myelin lamellae at the interperiod line to form large intramyelinic vacuoles. Myelinic vacuoles were preceded by nuclear and cytoplasmic changes in oligodendrocytes, which included nucleolar segregation, disaggregation, and diminution in number of ribosomes. These changes were similar to those previously reported in a variety of cells exposed to Actinomycin D. It is suggested that myelin vacuoles result secondarily from the Actinomycin D inhibitory effect on oligodendroglial RNA — and protein-synthesis, rather than from a direct effect of this drug on the myelin sheath.
    Type of Medium: Electronic Resource
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  • 2
    ISSN: 1432-0533
    Keywords: Key words Charcot-Marie-Tooth neuropathy type 1B ; Myelin protein zero (P0) ; Tomacula ; Outfolding ; Calf hypertrophy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Charcot-Marie-Tooth disease type 1B (CMT1B) is a demyelinating neuropathy caused by mutations in the myelin protein zero (P0) gene (MPZ). A few cases of CMT1B were recently found to be characterized by focally folded myelin sheaths in nerve biopsy specimens; the significance of this association is unknown. Here, we describe two unrelated pedigrees harboring a heterozygous Ser49Leu substitution in P0ex. In both pedigrees, the mutation caused a late-onset, relatively mild CMT1B; in one pedigree, two patients had atrophy of peroneal muscles but hypertrophy of the gastrocnemius muscles. The sural nerve biopsy performed in the two index cases revealed an identical chronic demyelinating and remyelinating neuropathy dominated by focal foldings of the myelin sheath shaped either as tomacula or as out/infoldings. The report adds Ser49Leu to the mutations of P0ex associated with focally folded myelin and provides strong evidence that such a structural alteration of the myelin sheath reflects a distinct pathogenetic mechanism in a subgroup of CMT1B.
    Type of Medium: Electronic Resource
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  • 3
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 50 (1980), S. 237-240 
    ISSN: 1432-0533
    Keywords: Rosenthal fiber ; Alexander's disease ; Astrocyte
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 20-year-old woman was admitted to the Hospital because of tetraparesis and respiratory failure, requiring mechanical ventilation. No disturbances of consciousness were associated. She had a residual left lower limb paresis from the age of 14, lasting unchanged until this final episode of bulbar paralysis. She did not recover: death occurred 3 months later. Neuropathological examination revealed Rosenthal fibers in the gray and white matter with typical perivascular and subpial distribution. Areas of mild demyelination were found in the periventricular white matter of the centrum ovale and in the hilum of the nucleus dentatus. In the brain stem the highest density of Rosenthal fibers was found in the medulla where the deposits extended deeply into parenchyma. The relationship of this case with Alexander's disease is discussed.
    Type of Medium: Electronic Resource
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  • 4
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 59 (1983), S. 216-224 
    ISSN: 1432-0533
    Keywords: Nerve crush ; PNS regeneration ; 2,5HD ; neurotilaments
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The pattern of recovery of myelinated axons in the posterior tibial nerve after crushing was studied in rats chronically intoxicated with 2,5-hexanedione. It was given for 2 weeks before curshing (200 mg/kg i.p. 5 times a week) or additionally for two further weeks after the nerve crush. Two animals were examined from each group at approximately, 1, 2, 3, 4, and 8 weeks later. Return of function in poisoned animals was slower than in the controls. The numbers of regenerating myelinated fibres was severely reduced in poisoned animals, up to 4 weeks later, but by 8 weeks the numbers equalled those in the control nerves. Marked impairment of initiation of neurite outgrowth was found, but once begun, axonal growth was comparable to controls and myelination occurred normally. Above the crush for 10 mm, filament-filled axonal swellings were found in poisoned animals accompanied by varying amounts of retrograde axonal degeneration. These findings are discussed in relation to the role of normal neurofilaments in axonal growth and the effects of probably cross-linking of these by 2,5-hexanedione on regnerating neurites.
    Type of Medium: Electronic Resource
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  • 5
    ISSN: 1432-0533
    Keywords: Tissue culture ; Human peripheral neurons ; Myelin-associated glycoprotein ; Immunocytochemistry ; Morphology
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary Dorsal root ganglion cells, obtained from 8–10-week human foetuses, were isolated by enzymatic procedure and grown on poly-l-lysine-coated coverslips. Most of the cultured cells showed the ultrastructural and immunological features of normal peripheral neurons. By immunocytochemistry neurons reacted with IgM antibodies with specificity for myelin-associated glycoprotein (MAG) from patients affected with IgM k gammopathy and peripheral neuropathy. The antigen was located on the plasmalemma of both perikarion and axon. We suggest that anti-MAG antibodies do not recognize neuronal MAG, but rather an epitope shared with different glycoproteins.
    Type of Medium: Electronic Resource
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  • 6
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 34 (1976), S. 267-271 
    ISSN: 1432-0533
    Keywords: Sudanophilic leucodystrophy ; Pelizaeus-Merzbacher disease ; Löwenberg-Hill type ; Diffuse sclerosis ; Demyelination
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary A 17-year-old girl with negative familial and personal history developed a progressive neurologic disorder characterized by cortical amaurosis, mental deterioration and spastic tetraparesis. Death occurred one year after the onset of symptoms. Histopathologic examination of the C.N.S. showed the presence of extensive, discontinuous demyelination of the white matter of the centrum ovale, with preservation of perivascular myelin islets, associated with massive fat degradation, loss of axons and severe astroglial proliferation. This case belongs to the group of sudanophilic leucodystrophies, being an atypical case, a transition between pure sudanophilic leucodystrophy and the group of Pelizaeus-Merzbacher's disease.
    Type of Medium: Electronic Resource
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  • 7
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 54 (1981), S. 311-314 
    ISSN: 1432-0533
    Keywords: Gliomatosis cerebri ; Diffuse gliomas ; Malignant gliomas
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinico-pathologic findings in an additional case of gliomatosis cerebri are reported: a 60-year-old woman died 8 months after the onset of a progressive deterioration of both the neurologic and mental conditions. Neuropathologic examination disclosed wide demyelination of both hemispheres, communicating through the corpus callosum, extending downward along the internal capsule to the brainstem structures. Cellular stains showed the presence of elongated astrocytes, multinucleated cells, mitotic and anaplastic figures, involving the demyelinated areas and the neighboring regions, and allowed the diagnosis of gliomatosis cerebri diffusa. The nosologic and pathogenetic aspects of this rare entity are discussed.
    Type of Medium: Electronic Resource
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  • 8
    Electronic Resource
    Electronic Resource
    Springer
    Acta neuropathologica 56 (1982), S. 179-186 
    ISSN: 1432-0533
    Keywords: Chronic relapsing polyneuritis ; Guillain-Barré-Strohl syndrome ; Demyelinating neuropathy
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Summary The clinico-pathologic findings in two patients with chronic relapsing polyneuritis are reported. The patients had several attacks of diffuse polyneuritis followed by partial recovery. The tendency to persisting disability increased following relapses and in the late stages there was a progressive motor deterioration suggesting a continuing activity of nerve damage. The biopsies of peripheral nerves, obtained in the advanced stages of the disease, showed severe loss of myelinated fibers and aspectes of repeated demyelination and remyelination, with formation of onion bulb complexes. Completely demyelinated axons together with remyelinating fibers as well as remyelinated fibers invested by macrophages were seen at the same time. The clinical and pathological evidences suggest that in chronic relapsing polyneuritis, at least in the late stages of the disease, besides clinical relapses, there is a continuing activity of nerve damage. Thus, due to this ongoing activity of demyelination and remyelination, the elimination of the supernumerary Schwann cells does not take place satisfactorily and hypertrophic changes of the nerve fascicles are produced.
    Type of Medium: Electronic Resource
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  • 9
    ISSN: 1432-0738
    Keywords: Organophosphates ; Chlorpyrifos ; Polyneuropathy ; Lymphocytic NTE
    Source: Springer Online Journal Archives 1860-2000
    Topics: Medicine
    Notes: Abstract Neuropathy Target Esterase (NTE) is the molecular target in the nervous system for organophosphorus esters (OP) when they cause delayed polyneuropathy. Some NTE activity was recently found also in blood lymphocytes. An unsuccessful suicide attempt with the widely used pesticide chlorpyrifos (0,0-diethyl-0-3,5,6,-trichloro-2-pyridyl phosphorothioate) is reported, where prior inhibition of lymphocytic NTE correlates with the delayed development of polyneuropathy. A 42-year-old man drank approximately 300 mg/kg chlorpyrifos. The subsequent severe cholinergic syndrome lasted for 17 days with varying degrees of severity. Thirty days after intoxication the clinical and electrophysiological examination of the peripheral nervous system was normal but lymphocytic NTE was about 60% inhibited. On day 43 the patient began to complain of paresthesia and leg weakness. Clinical examination, electrophysiology and a nerve biopsy revealed signs of a peripheral polyneuropathy, axonal in type. This case report indicates that measurement of lymphocytic NTE might be used as a clinical test to predict the development of OP-induced delayed polyneuropathy.
    Type of Medium: Electronic Resource
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  • 10
    ISSN: 0885-4505
    Source: Elsevier Journal Backfiles on ScienceDirect 1907 - 2002
    Topics: Medicine
    Type of Medium: Electronic Resource
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