Highlights
- •Acute intermittent porphyria is a disorder of heme metabolism caused by genetic mutation in porphobilinogen deaminase.
- •Accumulation of toxic heme precursors leads to a variety of systemic and neurological symptoms.
- •Peripheral nervous system manifestations include recurrent axonal polyradiculoneuropathy.
- •Triggers include smoking, alcohol, anti epileptics, hormone supplements and barbiturates.
- •Prompt treatment of acute attacks by glucose and hematin infusions.
Keywords
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References
- Porphyrias.Lancet. 2010; vol. 375 (Elsevier Ltd): 924-937
- Pathogenesis and treatment of immune-mediated neuropathies.Ther. Adv. Neurol. Disord. 2009; 2: 261-281
- Paraproteinemic neuropathies.Continuum (Minneap Minn). 2014; 20: 1307-1322
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Article info
Publication history
Published online: May 28, 2019
Accepted:
May 20,
2019
Received in revised form:
May 18,
2019
Received:
June 18,
2018
Identification
Copyright
© 2019 Elsevier B.V. All rights reserved.
ScienceDirect
Access this article on ScienceDirectLinked Article
- Corrigendum to “Porphyria: A rare differential diagnosis of polyradiculoneuropathy”. Journal of the Neurological Sciences. Volume 402, 15 July 2019, Pages 153–155Journal of the Neurological SciencesVol. 403