Porphyric neuropathy: an ultrastructural and quantitative case study.
We report a case of acute neuropathy in a 46 year old female with porphyria variegata. Histologic, electron microscopic, and quantitative examinations of peripheral nerves were performed at onset of the neuropathy and at autopsy. The results revealed severe qualitative and quantitative changes in myelinated and unmyelinated fibers showing features indicative of an axonopathy with a distribution in keeping with a dying-back phenomenon.
D001706:Biopsy; D005260:Female; D006801:Humans; D008107:Liver Diseases / Q000150:complications*; D008875:Middle Aged; D009420:Nervous System / Q000648:ultrastructure*; D009422:Nervous System Diseases / Q000209:etiology / Q000473:pathology; D011164:Porphyrias / Q000150:complications*; D013126:Spinal Nerve Roots / Q000648:ultrastructure; D013497:Sural Nerve / Q000473:pathology / Q000648:ultrastructure; D013979:Tibial Nerve / Q000648:ultrastructure