Ultrastructural study of a nerve biopsy from a case of early infantile chronic neuropathy

Abstract

The principal lesion in this case of chronic early infantile neuropathy is a modification of the Schwann cell resulting in the formation of a concentric network of lamellae formed by a double-layered sheet of basement membrane around numerous, large myelinated and unmyelinated fibers. The abnormalities in the myelin sheaths and the frequency of desmosomes are noteworthy. These peculiarities distinguish this case from other chronic neuropathies described up to now. Nevertheless, it is impossible to say at present whether the ultrastructural aspect of the nerve studied here is in any way specific.