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Journal Review: Porphyric neuropathy
Journal Review: Porphyric neuropathy

Acute hepatic porphyrias are inherited metabolic disorders that may present with polyneuropathy, which if not diagnosed early can lead to quadriparesis, respiratory weakness, and death. Porphyric neuropathy is an acute to subacute motor predomi-nant axonal neuropathy with a predilection for the upper extremities and usually preceded by a predominantly parasympathetic autonomic neuropathy. The rapid pro-gression and associated dysautonomia mimic Guillain-Barré syndrome but are distin-guished by the absence of cerebrospinal fluid albuminocytologic dissociation, progression beyond 4 wk, and associated abdominal pain. Spot urine test to assess the porphyrin precursors delta-aminolevulinic acid and porphobilinogen can provide a timely diagnosis during an acute attack. Timely treatment with intravenous heme, carbohydrate loading, and avoidance of porphyrinogenic medications can prevent further neurological morbidity and mortality.

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The AANEM designates this enduring material for a maximum of 1 AMA PRA Category 1 Credits TM.  Physicians should claim only the credit commensurate with the extent of their participation in the activity. Credit expires 8/6/2024.

Rachana K. Gandhi Mehta, MD: Dr. Gandhi has served on advisory board for Alnylam Pharma, Alexion, Akcea and Argenx, and has received research support from Akcea Therapeutics and UCB. James B. Caress, MD: Dr. Caress has no disclosure. Sean R. Rudnick, MD: Dr. Rudnick consults for and is on the speaker Bureau for Alnylam pharmaceuticals. Herbert L Bonkovsky, MD: Dr. Bonkovsky is supported in part by cooperative agreements between NIDDK and Wake Forest University School of Medicine U01 DK 065201, by way of UNC School of Medicine, Chapel Hill, NC, and U54 DK 083909, by way of Icahn School of Medicine at Mount Sinai, New York, NY. During the past 3 years, Dr. Bonkovsky has served as a consultant to Alnylam Pharma and Recordati Rare Chemicals; he has received funding awarded to Wake Forest, for clinical studies sponsored by Alnylam, Gilead Sciences, and Mitsubishi-Tanabe, North America. Any conflicts have been resolved by the Journal in accordance with the Muscle & Nerve editorial process.



Rachana K. Gandhi Mehta MD; Herbert L. Bonkovsky MD; James B. Caress, MD; Sean R. Rudnick, MD.
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