ACUTE PRESENTATION OF TANGIER POLYNEUROPATHY - A CLINICAL AND MORPHOLOGICAL-STUDY

We describe a patient with Tangier disease and a peripheral neuropathy with an unusual acute onset. The morphological studies of sural nerve biopsy revealed both axonal degeneration and demyelination, and the f iber loss was preferentially restricted to two of ten nerve fascicles. The cytoplasm of Schwann cells, fibroblasts, macrophages and pericyte s were vacuolated because of the presence of numerous lipid droplets. The clinical and morphological findings are consistent with the possib ility that ischemia plays a major role in causing this neuropathy.