Electron microscopic investigation of inclusion material in a case of adult metachromatic leukodystrophy; Observations on kidney biopsy,peripheral nerve and cerebral white matter |
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| Authors: | E Joosten M Hoes A Gabreëls-Festen O Hommes H Schuurmans Stekhoven J L Slooff |
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| Institution: | (1) Department of Neurology and Submicroscopic Morphology, St. Radboud Hospital, Catholic University Nijmegen, The Netherlands;(2) Department of Neurology, St. Radbout Hospital, Catholic University Nijmegen, The Netherland;(3) Department of Pathological Anatomy, St. Radboud Hospital, Catholic University Nijmegen, The Netherland;(4) Hospital  De Goddelijke Voorzienigheid , Sittard, The Netherlands |
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| Abstract: | 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. |
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| Keywords: | Adult metachromatic leukodystrophy Brain Peripheral nerve Kidney Electron microscopy |
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