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Acta Neuropathologica

, Volume 33, Issue 2, pp 165–171 | Cite as

Electron microscopic investigation of inclusion material in a case of adult metachromatic leukodystrophy; Observations on kidney biopsy, peripheral nerve and cerebral white matter

  • E. Joosten
  • M. Hoes
  • A. Gabreëls-Festen
  • O. Hommes
  • H. Schuurmans Stekhoven
  • J. L. Slooff
Short Original Communications

Summary

The fine structural characteristics of storage products in peripheral nerve, kidney and cerebral white matter, from a case of adult metachromatic leukodystrophy are described. There were pronounced differences from the fine structural aspects in late infantile cases. A large proportion of the inclusions did not exhibit a unit membrane. An hypothesis is proposed to clarify the delayed manifestation of this type of metachromatic leukodystrophy until adulthood.

Key words

Adult metachromatic leukodystrophy Brain Peripheral nerve Kidney Electron microscopy 

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Copyright information

© Springer-Verlag 1975

Authors and Affiliations

  • E. Joosten
    • 1
    • 4
  • M. Hoes
    • 2
  • A. Gabreëls-Festen
    • 2
  • O. Hommes
    • 2
  • H. Schuurmans Stekhoven
    • 3
  • J. L. Slooff
    • 3
  1. 1.Department of Neurology and Submicroscopic MorphologySt. Radboud Hospital, Catholic University NijmegenThe Netherlands
  2. 2.Department of NeurologySt. Radbout Hospital, Catholic University NijmegenThe Netherland
  3. 3.Department of Pathological AnatomySt. Radboud Hospital, Catholic University NijmegenThe Netherland
  4. 4.Hospital “De Goddelijke Voorzienigheid”SittardThe Netherlands

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