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  • Title: Ultrastructural findings of peripheral nerve in a preclinical case of adult metachromatic leukodystrophy.
    Author: Argyrakis A, Pilz H, Goebel HH, Müller D.
    Journal: J Neuropathol Exp Neurol; 1977 Jul; 36(4):693-711. PubMed ID: 196053.
    Abstract:
    In a 13-year-old neurologically healthy boy from a family with adult-onset of metachromatic leukodystrophy (MLD) showing arylsulfatase A-deficiency in the adult, sural nerve biopsy probably was performed 2-3 decades before clinical manifestation of the disease could be expected. Ultrastructurally 4 basic types of inclusion bodies in Schwann cells could be demonstrated (pleo-morphic "zebra body"-like inclusions, double-lamellated inclusions, "tuff-stone"-like inclusions, granular osmiophilic inclusions). Additionally, endoplasmatic reticulum, mitochondria and lysosomes showed marked alterations. Advanced damage of myelin was only rarely seen, but initial segmental demyelination was a common finding. These early pathological changes in chronic MLD are thought to represent a subcellular metabolic insufficiency of Schwann cells in this disease.
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