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Cerebral edema and intracranial hypertension in an adult with maple syrup urine disease

Sutter, R. and Killer, H. E. and Bilz, S. and Baumgartner, M. and Ott, H. W. and Steck, A. J. and Renaud, S.. (2009) Cerebral edema and intracranial hypertension in an adult with maple syrup urine disease. European journal of neurology : the official journal of the European Federation of Neurological Societies, Vol. 16, H. 3 , e45-e46.

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Official URL: http://edoc.unibas.ch/dok/A6165109

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Abstract

Maple syrup urine disease (MSUD) is an autosomal recessive disorder with dys- functional branched-chain a-keto acid dehydrogenase complex, leading to accu- mulation of branched-chain amino acids in all organs. Clinical symptoms were first described 1954. Several variants, have been identified. Decreased cognitive ability, hyperactivity, hallucinations, dys- tonia, ataxia, and altered consciousness have been described during acute meta- bolic intoxication. Cerebral edema and intracranial hypertension (IH) are fre- quent complications. We present an adult MSUD-patient with cerebral edema and IH with a favorable course after metabolic control and optic nerve sheath fenestration.
Faculties and Departments:03 Faculty of Medicine > Bereich Medizinische Fächer (Klinik) > Neurologie
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Medizinische Fächer (Klinik) > Neurologie
03 Faculty of Medicine > Bereich Medizinische Fächer (Klinik) > Neurologie > Neuroimmunologie (Kappos)
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Medizinische Fächer (Klinik) > Neurologie > Neuroimmunologie (Kappos)
03 Faculty of Medicine > Bereich Spezialfächer (Klinik) > Ophthalmologie USB
03 Faculty of Medicine > Departement Klinische Forschung > Bereich Spezialfächer (Klinik) > Ophthalmologie USB
03 Faculty of Medicine
UniBasel Contributors:Killer, Hanspeter E. and Renaud, Susanne and Sutter, Raoul Christian
Item Type:Article, refereed
Article Subtype:Book Review
Bibsysno:Link to catalogue
Publisher:Blackwell
Note:Publication type according to Uni Basel Research Database: Journal item
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Last Modified:13 Sep 2013 08:00
Deposited On:13 Sep 2013 07:58

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