Document Detail

Successful management of refractory intracranial hypertension from acute hyperammonemic encephalopathy in a woman with ornithine transcarbamylase deficiency.
MedLine Citation:
PMID:  20422467     Owner:  NLM     Status:  MEDLINE    
BACKGROUND: Ornithine transcarbamylase deficiency (OTCD) is the most common of the urea cycle disorders and results in an accumulation of ammonia and its metabolites. Excess ammonia in the brain is metabolized to glutamine, which increases intracellular osmolarity and contributes to cytotoxic edema. METHODS: We report a case of a woman heterozygous for OTCD who developed acute hyperammonemic encephalopathy and increased intracranial pressure (ICP). RESULTS: Despite hemodialysis, protein restriction, and administration of pharmacologic nitrogen scavengers, she developed progressive cerebral edema and increased ICP that was refractory to maximal medical management. She underwent a bifrontal decompressive craniectomy resulting in resolution of her intracranial hypertension. CONCLUSION: Aggressive multimodality management of the patient coupled with bifrontal decompressive hemicraniectomy was a life-saving measure, offering the patient a reasonable outcome. At 6 month follow-up she had moderate disability on the Glasgow Outcome Score associated with cognitive difficulties.
Linda C Wendell; Amir Khan; Jonathan Raser; Shih-Shan Lang; Neil Malhotra; W Andrew Kofke; Peter LeRoux; Soojin Park; Joshua M Levine
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Publication Detail:
Type:  Case Reports; Journal Article    
Journal Detail:
Title:  Neurocritical care     Volume:  13     ISSN:  1556-0961     ISO Abbreviation:  Neurocrit Care     Publication Date:  2010 Aug 
Date Detail:
Created Date:  2010-07-05     Completed Date:  2010-10-14     Revised Date:  -    
Medline Journal Info:
Nlm Unique ID:  101156086     Medline TA:  Neurocrit Care     Country:  United States    
Other Details:
Languages:  eng     Pagination:  113-7     Citation Subset:  IM    
Department of Neurology, Hospital of the University of Pennsylvania, Philadelphia, PA 19104, USA.
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MeSH Terms
Acute Disease
Brain Diseases / etiology*,  radiography
Brain Edema / etiology
Decompressive Craniectomy / methods*
Diet, Protein-Restricted
Follow-Up Studies
Gas Scavengers
Hyperammonemia / complications*,  etiology*
Intracranial Hypertension / etiology*,  physiopathology,  radiography,  therapy
Nitrogen / antagonists & inhibitors
Ornithine Carbamoyltransferase Deficiency Disease / complications*,  genetics
Renal Dialysis
Tomography, X-Ray Computed
Reg. No./Substance:

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