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.