Alexander’s disease (AD) is a rare, nonfamilial disorder of the nervous system which is also called dysmyelinogenetic leukodystrophy. The disease occurs sporadically without clear familial incidence. Three clinical subgroups of AD can be distinguished: infantile, juvenile and adult.


Multiple Sclerosis Metachromatic Leukodystrophy Rosenthal Fiber Optic Nerve Glioma Hyaline Droplet 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.


Unable to display preview. Download preview PDF.

Unable to display preview. Download preview PDF.


  1. Allen IV (1984) Demyelinating diseases. In: Hume Adams J, Corsellis JAN, Duchen LW, eds. Greenfield’s Neuropathology, 4th ed. London: Edward Arnold: 338–384Google Scholar
  2. Borrett D, Becker LE (1985) Alexander’s disease. Brain 108: 367–385PubMedCrossRefGoogle Scholar
  3. Cox NR, Kwapien RP, Sorjonen DC, Braund KG (1986) Myeloencephalopathy resembling Alexander’s disease in a Scottish terrier dog. Acta Neuropathol 71: 163–166PubMedCrossRefGoogle Scholar
  4. Farrell K, Chuang S, Becker LE (1984) Computed tomography in Alexander’s disease. Ann Neurol 15: 605–607PubMedCrossRefGoogle Scholar
  5. Habib M, Hassoun J, Ali-Cherif A, Alonzo B, et al. (1984) Maladie d’Alexander de l’adulte. Rev Neurol 140: 174–189Google Scholar
  6. Pfeiffer J (1968) Alexander’s disease–really a leucodystrophy? Path Europ 3: 305–312Google Scholar
  7. Pietrini V, Tagliavini F, Tedeschi F, Lechi A (1983) Megalencephaly with formation of Rosenthal fibers in symmetric subependymal gliomatous proliferations: clinicopathologic report. Clin Neuropathol 2: 16–22PubMedGoogle Scholar
  8. Sherwin RM, Berthrong M (1970) Alexander’s disease with sudanophilic leukodystrophy. Arch Path 89: 321–328PubMedGoogle Scholar
  9. Sorjonen DC, Cox NR, Kwapien RP (1987) Myeloencephalopathy with eosinophilic refractile bodies ( Rosenthal fibers) in a Scottish terrier. J Am Vet Med Assoc 190: 1004–1006Google Scholar
  10. Stam FC (1970) Megalencephalic type of congenital leucodystrophy. In: Vinken PJ, Bruyn GW, eds. Handbook of clinical neurology, vol 10. Amsterdam: North Holland Publishing Company: 94–102Google Scholar
  11. Towfighi J, Young R, Sassani J, Ramer J, et al. (1983) Alexander’s disease: further light-, and electron-microscopic observations. Acta Neuropathol 61: 36–42PubMedCrossRefGoogle Scholar
  12. Townsend JJ, Wilson JF, Harris T, Coulter D, et al. (1985) Alexander’s disease. Acta Neuropathol 67: 163–166PubMedCrossRefGoogle Scholar
  13. Trommer BL, Naidich TP, Dal Canto MC, McLone DG, et al. (1983) Noninvasive CT diagnosis of infantile Alexander disease: pathologic correlation. J Comput Assist Tomogr 7: 509–516PubMedCrossRefGoogle Scholar
  14. Ule G, Jacob H (1983) Die astrogliale Dystrophie mit Rosenthalischen Fasern. Nervenarzt 54: 69–73PubMedGoogle Scholar
  15. Vogel FS, Hallervorden J (1962) Leukodystrophy with diffuse Rosenthal fiber formation. Acta Neuropathol 2: 126–143CrossRefGoogle Scholar
  16. Walls TJ, Jones JRA, Cartlidge N, Saunders M (1984) Alexander’s disease with Rosenthal fibre formation in an adult. J Neurol Neurosurg Psych 47: 399–403CrossRefGoogle Scholar
  17. Wohlwill FJ, Bernstein J, Yakovlev PI (1959) Dysmyelinogenic leukodystrophy. J Neuropathol Exp Neurol 18: 359–383PubMedCrossRefGoogle Scholar

Copyright information

© Springer-Verlag Berlin Heidelberg 1989

Authors and Affiliations

  • Jacob Valk
    • 1
  • Marjo S. van der Knaap
    • 2
  1. 1.Department of Diagnostic Radiology and NeuroradiologyFree University HospitalAmsterdamThe Netherlands
  2. 2.Department of Child NeurologyAcademic HospitalUtrechtThe Netherlands

Personalised recommendations