The Electroencephalogram in Neonatal Maple Syrup Urine Disease-Reference-Cited by-同舟云学术

The Electroencephalogram in Neonatal Maple Syrup Urine Disease

Published:2012-01 Issue:1 Volume:43 Page:64-67
ISSN:1550-0594
Container-title:Clinical EEG and Neuroscience
language:en
Short-container-title:Clin EEG Neurosci

Author:

Agadi Satish¹,Sutton Vernon R.²,Quach Michael M.¹,Riviello James J.¹

Affiliation:

1. Section of Neurology and Developmental Neuroscience, Department of Pediatrics, and Section of Neurophysiology, Department of Neurology, Baylor College of Medicine, Houston, TX, USA

2. Department of Molecular and Human Genetics, Baylor College of Medicine.

Abstract

Untreated maple syrup urine disease (MSUD) leads to encephalopathy in neonates and causes abnormalities on the electroencephalogram (EEG). A case is presented of MSUD with unique features consisting of a comb-like rhythm before the therapy and its disappearance with therapy is presented. This case illustrates the potential use of the EEG in the identification of this specific cause of a neonatal encephalopathy.

Publisher

SAGE Publications

Subject

Neurology (clinical),Neurology,General Medicine

Link

http://journals.sagepub.com/doi/pdf/10.1177/1550059411429521

Reference9 articles.

1. A NEW SYNDROME: PROGRESSIVE FAMILIAL INFANTILE CEREBRAL DYSFUNCTION ASSOCIATED WITH AN UNUSUAL URINARY SUBSTANCE

2. Maple syrup urine disease: Clinical, eeg, and plasma amino acid correlations with a theoretical mechanism of acute neurotoxicity

3. Les rythmes rapides dans les maladies metaboliques chez l'enfant

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