Acute presentation of Tangier polyneuropathy: a clinical and morphological study

Raffaella Fazio, Raffaello Nemni, Angelo Quattrini, Giacomo Ruotolo, Sandro Iannaccone, Daniela Mamoli, Monica Lodi, Nicola Canal

Research output: Contribution to journalArticlepeer-review


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 fiber loss was preferentially restricted to two of ten nerve fascicles. The cytoplasm of Schwann cells, fibroblasts, macrophages and pericytes were vacuolated because of the presence of numerous lipid droplets. The clinical and morphological findings are consistent with the possibility that ischemia plays a major role in causing this neuropathy.

Original languageEnglish
Pages (from-to)90-94
Number of pages5
JournalActa Neuropathologica
Issue number1
Publication statusPublished - Jun 1993


  • Pericyte
  • Polyneuropathy
  • Storage disease
  • Tangier disease

ASJC Scopus subject areas

  • Neuroscience(all)
  • Pathology and Forensic Medicine
  • Clinical Neurology


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