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Establishing a Mouse Model of a Pure Small Fiber Neuropathy with the Ultrapotent Agonist of Transient Receptor Potential Vanilloid Type 1
Published on: February 13, 2018
Acute intermittent porphyria with peripheral neuropathy complicated by small-fiber neuropathy
Ching-Hua Hsieh1, Hui-Hong Tsai, Tsu-Hsiang Lu
1Department of Neurology, Chang Gung Memorial Hospital, Kaohsiung Medical Center, Chang Gung University College of Medicine, Taiwan.
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.
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