Summary
Electronmicroscopic examination of brain tissue from patients with juvenile amaurotic idiocy (Batten-Spielmeyer-Vogt disease) revealed a highly characteristic fine architecture of the lipid bodies accumulated in the perikarya. These pleomorphic bodies present two different types of internal structure, either a densely granular stroma, or round, oval and tubular membranes giving the cross section a multilocular appearance. There is some suggestion that the bodies might derive from degenerating mitochondria. In all four patients, regardless of age, the lipid bodies were similar in structure but they were distinctly different from the membranous cytoplasmic bodies of infantile amaurotic idiocy (Tay-Sachs disease) as described byTerry andKorey.
Zusammenfassung
Elektronenmikroskopische Studien am Gehirngewebe von vier Patienten mit juveniler amaurotischer Idiotie (Batten-Spielmeyer-Vogtsche Krankheit) ergaben, daß die akkumulierten Lipoide in der Form von cytoplasmischen Organellen vorhanden sind. Diese Lipoidkörper sind pleomorph, von überaus wechselnder Größe, und zeigen zwei Typen von verschiedener interner Strukturierung. Einmal handelt es sich um ein dichtes, granuläres Stroma; in anderen Lipoidkörpern finden sich runde, ovale und tubuläre Membranen, die dem Ganzen ein multilokuläres Aussehen verleihen. Auf Grund gewisser morphologischer Ähnlichkeiten wird die Entstehung der Lipoidkörperchen aus degenerierten Mitochondrien erwogen. Daß ihre Feinstruktur nicht altersbedingt ist, geht daraus hervor, daß die Lipoidkörperchen von einer 9 Jahre alten Patientin mit denen von einer 23 Monate alten Patientin identisch waren. Die Lipoidkörper der juvenilen amaurotischen Idiotie sind grundverschieden von denen der infantilen amaurotischen Idiotie, den sogenannten „membranous cytoplasmic bodies” (Terry andKorey).
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Part of this study was supported by research grants B1620 C4 and the Neuropathology Training Grant 2 B5062 C7 from the NINDB of the USPHS and the Association for the Aid of Crippled Children Grant R-15262 from the United Cerebral Palsy Research and Education Foundation, Inc.
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Zeman, W., Donahue, S. Fine structure of the lipid bodies in juvenile amaurotic idiocy. Acta Neuropathol 3, 144–149 (1963). https://doi.org/10.1007/BF00687063
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DOI: https://doi.org/10.1007/BF00687063