Electron Microscopical Study of Neuroaxonal Dystrophy

Coster, W. De; Roels, H; Eecken, H. vander

doi:10.1159/000114061

Cited by 12 publications

(1 citation statement)

References 16 publications

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“…We have had already a substantial number of studies on the ultrastructural morphology of axonal dystrophy in various morbid conditions of both human (Hedley-Whyte, Gilles & Uzman, 1968;Herman, Huttenlocher & Bensch, 1969;Toga, Bbrard-Badier & Gambarelli-Dubois, 1970;de Coster, Roels & Van der Eecken, 1971;Martin & Martin, 1972;Martin, Trelles & Martin, 1972;Sengel & Stoebner, 1972;Kimura et al, 1974;Liu, Larson & Mizuno, 1974;Vakili, et al, 1977;Scheithauer, et al, 1978;Yagishita, 1979) and animals (Lampert & Pentschew, 1964;Lampert, Blumberg & Penschew, 1964;Blakemore & Cavanagh, 1969;Nakamura & Okamoto, 1973;Johnson, Mehler & Miquel, 1975;Suzuki & Suu, 1978). Its pathogenesis and sequential development as a morbid disturbance of terminal axons, as well as its neurocytological background, however, still remain to be clarified.…”

Section: Introductionmentioning

confidence: 99%

Study of Axonal Dystrophy. Ii. Dystrophy and Atrophy of the Presynaptic Boutons: A Dual Pathology

Fujisawa¹,

Shiraki²

1980

Neuropathology Appl Neurobio

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In succession to the previous quantitative work, a qualitative study has been carried out on the nature of a dual pathology affecting presynaptic boutons in the posterior tract nuclei of ageing rats. Based on the morphology of dystrophic boutons in early stage, it is concluded that the initial and therefore essential characteristic of dystrophic process is an abnormal increase of normal axonal components within the presynaptic boutons, and that various abnormal substructures of spheroids hitherto reported in the literature are probably the results of their secondary metamorphosis. The dystrophic process within the posterior tract nuclei is a selective one, involving presynaptic boutons and preterminal axons only of the posterior tract fibres. Comparison of the frequency of early dystrophic boutons and of fully grown-up spheroids indicates that a small percentage of boutons deriving from posterior tract fibres become dystrophic and of these dystrophic boutons only a small percentage again continue to develop unto large spheroids, throughout lifespan of the animals. On the other hand, in search of a morphological counterpart for the age-related decrease of volume ratio of presynaptic boutons to the neuropil, some dubious atrophic changes were also found in presynaptic boutons, which could have been easily missed from observation if studied qualitatively alone. Accordingly, no less numerous boutons other than dystrophic ones are supposed to atrophy 'independently' and to disappear 'silently' during the same period. The dystrophic and the atrophic changes involve different boutons (of different or the same terminal axons) within the same gray matter. This dual pathology of boutons needs further elucidation of its neurocytopathological as well as neurobiological background in the future.

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Section: Introductionmentioning

confidence: 99%

Study of Axonal Dystrophy. Ii. Dystrophy and Atrophy of the Presynaptic Boutons: A Dual Pathology

Fujisawa¹,

Shiraki²

1980

Neuropathology Appl Neurobio

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Morphological investigations on axonal swellings and spheroids in various human diseases

Yagishita

1978

Virchows Arch. A Path. Anat. and Histol.

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Axonal swellings and spheroids in various human diseases were studied by light and electron microscopy. 4 cases of infantile neuroaxonal dystrophy, 2 of degenerative diseases, 2 brain tumors and 3 of cerebrovascular disease were examined. Ultrastructurally most spheroids in infantile neuroaxonal dystrophy consisted of interconnected tubules, stacked membranotubular profiles, alternating layered membranes and accumulations of neurofilaments. Combinations of these four constituents were seen only in infantile neuroaxonal dystrophy. "Torpedos" (fusiform swelling of the axon of a Purkinje cell) consisted exclusively of neurofilaments. Spheroids in case 6 (mental retardation) and 7 (atypical teratoma) consisted of interwoven skeins of neurofilaments and grouped mitochondria. Spheroids in case 8 (demyelination) and 9 (cerebrovascular disease) consisted of packed complex bodies and mitochondria. Spheroids in cases 10 and 11 (cerebrovascular disease) consisted of degenerating organelles only. The morphological differences between cases 9, 10 and 11 probably depends on the severity and timing of the cerebral injury. Most spheroids show similar histological and histochemical properties, but ultrastructural study may give some clue to the origin of the bodies.

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Infantile neuroaxonal dystrophy (Seitelberger's disease)

Yagishita

Kimura

1975

Acta Neuropathol

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The findings in a case of infantile neuroaxonal dystrophy are presented. Light microscopy shows wide distribution of spheroids, cerebullar atrophy and striatal degeneration. Electron microscopy shows numerous spheroids consisting of tubulo-vesiculo-membranous profiles. The dystrophic changes appear first in the axon seem to be modified by axostasis and/or transneuronal degeneration in addition to the dystrophic changes. The participation of the endoplasmic reticulum and microtubules in spheroid formation and their relation to the malformation of the synaptic vesicles have been discussed. Diverse mitochondrial abnormalities and Hiranolike body are also described.

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Electron Microscopical Study of Neuroaxonal Dystrophy

Cited by 12 publications

References 16 publications

Study of Axonal Dystrophy. Ii. Dystrophy and Atrophy of the Presynaptic Boutons: A Dual Pathology

Study of Axonal Dystrophy. Ii. Dystrophy and Atrophy of the Presynaptic Boutons: A Dual Pathology

Morphological investigations on axonal swellings and spheroids in various human diseases

Infantile neuroaxonal dystrophy (Seitelberger's disease)

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