Journal Review: Porphyric neuropathy-Article

Article

Pdf Summary

Porphyric neuropathy is a neurological manifestation of acute hepatic porphyrias, characterized by a motor predominant axonal neuropathy that primarily affects the upper extremities. Diagnosis involves testing for porphyrin precursors in a urine sample. Treatment includes intravenous heme and avoidance of trigger medications. The exact mechanisms behind the neuropathy are not fully understood. Clinical manifestations include autonomic dysfunction, peripheral neuropathy, and central nervous system symptoms. Electrodiagnostic studies aid in diagnosis. Early diagnosis and treatment are important to prevent complications.

Keywords

Porphyric neuropathy

neurological manifestation

acute hepatic porphyrias

axonal neuropathy

diagnosis

urine sample

treatment

intravenous heme

trigger medications

complications