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