Uncompacted myelin lamellae in dysglobulinemic neuropathy

Uncompacted lamellae, located preferentially in inner layers of myelin sheath, were observed in biopsied sural nerves of 3 cases of dysglobulinemic neuropathy, in which the main pathological findings of myelinated fibers were those of segmental demyelination and remyelination, and axonal degeneration with concurrent marked decrease of myelinated fiber density. The presence of uncompacted myelin lamellae is well explained by the irregular distribution of adaxonal, incisural and paranodal cytoplasm of the Schwann cell.