Document Detail


Acute intermittent porphyria presenting as a diffuse encephalopathy.
MedLine Citation:
PMID:  15786449     Owner:  NLM     Status:  MEDLINE    
Abstract/OtherAbstract:
Although acute intermittent porphyria presents with dramatic neurological findings, the diagnosis is difficult. An 18-year-old woman had a clinical picture of porphyric encephalopathy. Magnetic resonance (MR) imaging demonstrated multiple large contrast-enhancing subcortical white matter lesions, which regressed with glucose and hematin infusions. Diffusion-weighted MR imaging was normal, and MR spectroscopy excluded acute demyelination or tissue necrosis. MR findings of acute intermittent porphyria can differ from those in posterior reversible encephalopathy syndrome by virtue of intense contrast enhancement. Because diffusion-weighted MR imaging and spectroscopy were normal, the lesions are likely caused by reversible vasogenic edema and transient breakdown of the blood-brain barrier.
Authors:
Boby V Maramattom; Renzo A Zaldivar; Simon M Glynn; Scott D Eggers; Eelco F M Wijdicks
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Annals of neurology     Volume:  57     ISSN:  0364-5134     ISO Abbreviation:  Ann. Neurol.     Publication Date:  2005 Apr 
Date Detail:
Created Date:  2005-03-29     Completed Date:  2005-05-20     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  7707449     Medline TA:  Ann Neurol     Country:  United States    
Other Details:
Languages:  eng     Pagination:  581-4     Citation Subset:  IM    
Affiliation:
Division of Critical Care Neurology, Mayo Clinic College of Medicine, Rochester, MN, USA.
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MeSH Terms
Descriptor/Qualifier:
Adolescent
Brain Diseases / diagnosis*,  physiopathology
Carbohydrates / therapeutic use
Diagnosis, Differential
Female
Hemin / therapeutic use
Humans
Magnetic Resonance Imaging
Porphyria, Acute Intermittent / diagnosis*,  drug therapy,  physiopathology*
Chemical
Reg. No./Substance:
0/Carbohydrates; 16009-13-5/Hemin

From MEDLINE®/PubMed®, a database of the U.S. National Library of Medicine


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