Acute intermittent porphyria with peripheral neuropathy complicated by small-fiber neuropathy

Ching Hua Hsieh, Hui Hong Tsai, Tsu Hsiang Lu, Yi Chun Chen, Min Wei Hsieh, Yao Chung Chuang*

*Corresponding author for this work

Research output: Contribution to journalJournal Article peer-review

10 Scopus citations

Abstract

We describe a 60-year-old male patient with acute intermittent porphyria (AIP) who presented with initial abdominal pain and subsequent quadriplegia and respiratory failure. Small fiber neuropathy was demonstrated by measuring intra-epidermal nerve fiber density (IENFD) using protein gene product 9.5 (PGP 9.5) immunostaining on three consecutive skin punch biopsies of the distal lower limb. The biopsy findings demonstrated some correlation with progression of the patient's clinical condition. Neuropathy in AIP can have a small-fiber component rather than being solely a large-fiber neuropathy.

Original languageEnglish
Pages (from-to)133-138
Number of pages6
JournalNeuropathology
Volume27
Issue number2
DOIs
StatePublished - 04 2007

Keywords

  • Acute intermittent porphyria
  • Intra-epidermal nerve fiber density
  • Peripheral neuropathy
  • Protein gene product 9.5
  • Small-fiber neuropathy

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