Acute peripheral neuropathy due to hereditary coproporphyria

Muscle Nerve. 1994 Jul;17(7):793-9. doi: 10.1002/mus.880170715.


A 23-year-old man with epilepsy and a past history of abdominal pain and ileus, developed hypertension and arm and bulbar weakness when valproic acid and carbamazepine were reinitiated. Electrophysiologic studies demonstrated a peripheral neuropathy with features of axonal degeneration and demyelination. Axonal degeneration was documented by sural nerve biopsy. Markedly elevated urinary delta-aminolevulinic acid and porphobilinogen indicated a diagnosis of acute porphyria. Other laboratory studies were most consistent with hereditary coproporphyria. Motor function improved considerably but incompletely over 1 year. An acute, primarily motor neuropathy can occur in several forms of porphyria, including acute intermittent porphyria, variegate porphyria, and hereditary coproporphyria, sometimes even in the absence of concomitant gastrointestinal symptoms.

Publication types

  • Case Reports

MeSH terms

  • Acute Disease
  • Adult
  • Electrodiagnosis
  • Humans
  • Male
  • Peripheral Nervous System Diseases / diagnosis
  • Peripheral Nervous System Diseases / etiology*
  • Peripheral Nervous System Diseases / physiopathology
  • Porphyrias, Hepatic / complications*
  • Sural Nerve / pathology