Acute peripheral neuropathy due to hereditary coproporphyria

R. J. Barohn, J. A. Sanchez, Karl Anderson

Research output: Contribution to journalArticle

21 Citations (Scopus)

Abstract

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 δ-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.

Original languageEnglish (US)
Pages (from-to)793-799
Number of pages7
JournalMuscle and Nerve
Volume17
Issue number7
DOIs
StatePublished - 1994

Fingerprint

Hereditary Coproporphyria
Acute Intermittent Porphyria
Peripheral Nervous System Diseases
Variegate Porphyria
Porphobilinogen
Porphyrias
Aminolevulinic Acid
Sural Nerve
Ileus
Carbamazepine
Valproic Acid
Demyelinating Diseases
Abdominal Pain
Epilepsy
Hypertension
Biopsy

Keywords

  • hereditary coproporphyria
  • peripheral neuropathy
  • porphyria

ASJC Scopus subject areas

  • Clinical Neurology
  • Neuroscience(all)

Cite this

Acute peripheral neuropathy due to hereditary coproporphyria. / Barohn, R. J.; Sanchez, J. A.; Anderson, Karl.

In: Muscle and Nerve, Vol. 17, No. 7, 1994, p. 793-799.

Research output: Contribution to journalArticle

Barohn, R. J. ; Sanchez, J. A. ; Anderson, Karl. / Acute peripheral neuropathy due to hereditary coproporphyria. In: Muscle and Nerve. 1994 ; Vol. 17, No. 7. pp. 793-799.
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