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Pathologie pp 169-173 | Cite as

Neuroaxonale Dystrophien

  • W. Paulus

Zusammenfassung

Sphäroidesind gut abgrenzbare, run de oder ovale, leicht eosinophile, homo gene oder auch zentral unterschiedlich grob granulierte Strukturen von 20–60 μm(10–120 μm) (Abb.1.69a—c). In den Silberimprägnationennach Bodian oder Bielschowsky sind sie in der Regel argyrophil (Abb.1.69d), in der Berliner-BlauReaktion wegen ihres Eisengehaltes häufig positiv. Immunhistologischenthalten sie Ubiquitin, phosphorylierte und nichtphosphorylierte Neurofilamente. Degenerierte Sphäroidekönnen basophil, vakuolig aufgelockert, unregelmäβig geformt. fragmentiert oder verkalkt sein. Wenn sie sich auflbsen oder phagozytiert werden, bleiben spongiösc Dcfcktc zurück. In ausgeprägten Fällen kommt es zu einer umgebenden Gliose.

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Literatur

  1. 1.
    Seitelberger F, (1986) Neuroaxonal dystrophy: its relation to aging and neurological diseases. In: Vinken PL, Bruyn OW, Klawans HL, (eds) Handbook of clinical neurology. Vol 5. Elsevier. Amsterdam. pp391–415Google Scholar
  2. 2.
    Angelini L, Nardocci N., Rumi V, et al. (1992) Hallervorden-Spatz disease: clinical and MRI study of 11 cases diagnosed in life. J Ncuro 1239: 417–425Google Scholar
  3. 3.
    Barlow JK., Sims KB., Kolodny EH, (1989) Early cerebellar degeneration in twins with infantile neuroaxonal dystrophy. Ann Neuroi 25: 413–415CrossRefGoogle Scholar
  4. 4.
    Clark AW., Parhad IM., Griffin IW., Price DL, (1984) Neurofilamentous axonal swellings as a normal finding in the spinal anterior horn of man and other primates. J Neuropathol Exp Neurol 43:253–262PubMedCrossRefGoogle Scholar
  5. 5.
    Donaghy M., King RHM., Thomas PK., Workman JM, (1988) Abnormalities of the axonal cytoskeleton in giant axonal neuropathy. J Neurocytol 17: 197–208PubMedCrossRefGoogle Scholar
  6. 6.
    Gaytan-Garcia S., Kaufmann JCE., Young GB, (1990) Adult onset Hallervorden-Spatz syndrom or Seitelberger‘s disease with late onset: variants of the same entity? Clin Neuropathol 9: 136–142PubMedGoogle Scholar
  7. 7.
    Gilman S., Barrett RE, (1973) Hallervorden-Spatz disease and infantile neuroaxonal dystrophy. Clinical characteristics and nosological considerations. J Neurol Sci 19: 189–205PubMedCrossRefGoogle Scholar
  8. 8.
    Hayashi S., Akasaki Y., Morimura Y, et al. (1992) An autopsy case of late infantile and juvenile neuroaxonal dystrophy with diffuse Lewy bodies and neurofibrillary tangles. Clin Neuropathol 11: 1–5PubMedGoogle Scholar
  9. 9.
    Jellinger K., Jinásck A, (1971) Neuroaxonal dystrophy in man: character and natural history. Acta Neuropathol [Suppl 5]: 3–16Google Scholar
  10. 10.
    Kimura S., Sasaki Y., Warlo I., Goebel HH, (1987) Axonal pathology of the skin in infantile neuroaxonal dvstrophy. Acta Neuropathol 75: 212–215PubMedCrossRefGoogle Scholar
  11. 11.
    Kretzschmar HA., Berg BO., Davis RL, (1987) Giant axonal neuropathy. A neuropathological study. Acta Neuropathol 73: 138–144PubMedCrossRefGoogle Scholar
  12. 12.
    Lampert PW, (1967) A comparative electron microscopic study of reactive. degenerating. regenerating. and dystrophic axons. J Neuropathol Exp Neurol 26: 345–368PubMedCrossRefGoogle Scholar
  13. 13.
    Ouvrier RA (1989) Giant axonal neuropathy. A review. Brain Dev 11: 207–214Google Scholar
  14. 14.
    Peiffer J., Brunner N., Landolt RF., Müller G., Schlote W, (1976) Generalisierte infantile neuroaxonale Dystrophie mit Pallidum-Literatur 173 pigmentation und -lipophanerose bei einem eineiigen Zwillingspaar. Neuropadiatrie 7: 327–350PubMedCrossRefGoogle Scholar
  15. 15.
    Peiffer J., Schlote W., Bischoff A., Boltshauser E, Müller G, (1977) Generalized giant axonal neuropathy. Acta Neuropathol 40: 213–218PubMedCrossRefGoogle Scholar
  16. 16.
    Perry TL, Norman MG, Yong VW, et al. (1985) HallervordenSpatz disease: cysteine accumulation and cysteine dioxygenase deficiency in the globus pallidus. Ann Neurol 18: 482–489PubMedCrossRefGoogle Scholar
  17. 17.
    Pettigrew AL., Jackson LG., Ledbetter DH, (1991) New X-linked mental retardation disorder with Dandy-Walker malformation, basal ganglia disease, and seizures. Am J Med Genet 38: 200–207PubMedCrossRefGoogle Scholar
  18. 18.
    Schmidt RE, Chae HY, Parvin CA, Roth KA, (1990) Neuroaxonal dystrophy in aging human sympathetic ganglia. Am J Pathol 130: 1327–1338Google Scholar
  19. 19.
    Southam E., Thomas PK., King RHM, Goss-Sampson MA, Muller DPR, (1991) Experimental vitamin E deficiency in rats. Morphological and functional evidence of abnormal axonal transport secondary to free radical damage. Brain 114: 915–936PubMedCrossRefGoogle Scholar
  20. 20.
    Sung JH, Park SH, Mastri AR., Warwick WJ, (1980) Axonal dystrophy in the gracile nucleus in congenital biliary atresia and cystic fibrosis (mucoviscidosis): beneficial effect of vitamin E therapy. J Neuropathol Exp Neurol 39: 584–597PubMedCrossRefGoogle Scholar
  21. 21.
    Tandan R., Bradley WG., Fillyaw MJ, (1990) Giant axonal neuropathy: studies with sulfhydryl donor compounds. J Neurol Sci 95: 153–162PubMedCrossRefGoogle Scholar
  22. 22.
    Thomas C., Love S, Powell HC, Schultz P, Lampert PW, (1987) Giant axonal neuropathy: correlation of clinical findings with postmortem neuropathology. Ann Neurol 22: 79–84PubMedCrossRefGoogle Scholar
  23. 23.
    Wang AM., Schindler O, Desnick RJ, (1990) Schindler disease: the molecular lesion in the α-N-acetylgalactosaminidase gene that causes an infantile neuroaxonal dystrophy. J Clin Invest 86: 1752–1756PubMedCrossRefGoogle Scholar
  24. 24.
    Williams OJ., Ironside JW, (1989) Liver and pituitary abnormalities in Hallervorden-Spatz disease. J Neurol Neurosurg Psychiatry 52: 1410–1414PubMedCrossRefGoogle Scholar
  25. 25.
    Wongmongkolrit T, Wyszynski R., Hershey CO, Varnes AW, (1985) Evidence of subclinical extrapyramidal hemosiderosis in cystic fibrosis. Acta Neuropathol 65: 265–269PubMedCrossRefGoogle Scholar
  26. 26.
    Yagishita S., Itho Y, Nakano T, et al. (1978) Infantile neuroaxonal dystrophy. Acta Neuropathol 41: 257–259PubMedCrossRefGoogle Scholar

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© Springer-Verlag Berlin Heidelberg 1995

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  • W. Paulus

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