Alexander’s Disease

  • Jacob Valk
  • Marjo S. van der Knaap


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.


Dementia Cavitation Hydrocephalus Astrocytoma Rosen 


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