Porphyric neuropathy This article is a US Government work and, as such, is in the public domain in the United States of America.

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dc.contributor.author Albers, James W. en_US
dc.contributor.author Fink, John K. en_US
dc.date.accessioned 2006-04-19T13:43:36Z
dc.date.available 2006-04-19T13:43:36Z
dc.date.issued 2004-10 en_US
dc.identifier.citation Albers, James W.; Fink, John K. (2004)."Porphyric neuropathy This article is a US Government work and, as such, is in the public domain in the United States of America. ." Muscle & Nerve 30(4): 410-422. <http://hdl.handle.net/2027.42/34640> en_US
dc.identifier.issn 0148-639X en_US
dc.identifier.issn 1097-4598 en_US
dc.identifier.uri http://hdl.handle.net/2027.42/34640
dc.identifier.uri http://www.ncbi.nlm.nih.gov/sites/entrez?cmd=retrieve&db=pubmed&list_uids=15372536&dopt=citation en_US
dc.description.abstract The hepatic porphyrias are a group of rare metabolic disorders characterized by enzymatic defects in the biosynthesis of heme, a metalloporphyrin that is the principal product of porphyrin metabolism. The hepatic porphyrias are genetically transmitted as autosomal-dominant disorders with variable expression that produce a particularly severe form of neuropathy. Most medical students readily recognize acute attacks of porphyria when the classic triad of abdominal pain, psychosis, and neuropathy is present. Yet, porphyric neuropathy is a source of confusion in practice, and patients with porphyria rarely receive the correct diagnosis early in the course of the illness. Porphyric neuropathy is manifest by symptoms, signs, and cerebrospinal fluid abnormalities resembling acute Guillain–BarrÉ syndrome. However, accompanying psychological features, a proximal predilection of asymmetric weakness, and electrodiagnostic findings indicative of an axonal polyradiculopathy or neuronopathy all suggest the diagnosis of porphyria. Confirmation of the diagnosis depends on use of appropriate laboratory studies. The underlying pathophysiology of porphyric neuropathy has not been established, but it may be related to direct neurotoxicity of elevated levels of Δ-aminolevulinic acid. The severity of the neuropathy and the availability of potential treatments, including avoidance of provocative factors, make identification important. Muscle Nerve 30: 410–422, 2004 en_US
dc.format.extent 216838 bytes
dc.format.extent 3118 bytes
dc.format.mimetype application/pdf
dc.format.mimetype text/plain
dc.language.iso en_US
dc.publisher Wiley Subscription Services, Inc., A Wiley Company en_US
dc.subject.other Life and Medical Sciences en_US
dc.subject.other Neuroscience, Neurology and Psychiatry en_US
dc.title Porphyric neuropathy This article is a US Government work and, as such, is in the public domain in the United States of America. en_US
dc.type Article en_US
dc.rights.robots IndexNoFollow en_US
dc.subject.hlbsecondlevel Neurosciences en_US
dc.subject.hlbtoplevel Health Sciences en_US
dc.description.peerreviewed Peer Reviewed en_US
dc.contributor.affiliationum Department of Neurology, 1C325/0032 University Hospital, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109-0032, USA ; Neurobehavioral Toxicology Program, University of Michigan, Ann Arbor, Michigan, USA ; Department of Neurology, 1C325/0032 University Hospital, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109-0032, USA en_US
dc.contributor.affiliationum Department of Neurology, 1C325/0032 University Hospital, University of Michigan Health System, 1500 East Medical Center Drive, Ann Arbor, Michigan 48109-0032, USA ; Geriatric Research Education and Clinical Center, Ann Arbor Veterans Affairs Medical Center, Ann Arbor, Michigan, USA en_US
dc.identifier.pmid 15372536 en_US
dc.description.bitstreamurl http://deepblue.lib.umich.edu/bitstream/2027.42/34640/1/20137_ftp.pdf en_US
dc.identifier.doi http://dx.doi.org/10.1002/mus.20137 en_US
dc.identifier.source Muscle & Nerve en_US
dc.owningcollname Interdisciplinary and Peer-Reviewed
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