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Brain 2005 128(4):697-699; doi:10.1093/brain/awh482
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© The Author (2005). Published by Oxford University Press on behalf of the Guarantors of Brain. All rights reserved. For Permissions, please email: journals.permissions@oupjournals.org

Scientific Commentary

Glutaric aciduria type 1: a clinician's view of progress

The first 150 words of the full text of this article appear below.

Glutaric aciduria type 1 (GA1) arises from an enzymatic block in the common degradation pathway for lysine and tryptophan. It is a cause of crippling striatal necrosis during infancy (Strauss et al., 2003Go). Clinical experience teaches us two things about GA1. First, predicting precisely when and if basal ganglia injury will occur in an individual is presently difficult, if not impossible. Second, when such injuries ensue, we have no therapeutic instruments to stop them. Thus, to prevent injuries we need prediction, and there is ample clinical evidence that plasma and urine organic acid measurements are inadequate for this purpose (Strauss et al., 2003Go).

Real progress in the treatment of GA1 requires a deeper understanding of the premorbid state—the set of physiological adaptations entrained by abnormal organic acid metabolism in the brain. For this knowledge to be applied physiological changes that precede the catastrophic event must be . . . [Full Text of this Article]

Kevin A. Strauss

Clinic for Special Children, Strasburg, PA, USA

Correspondence to: Kevin A. Strauss, MD, Clinic for Special Children, 535 Bunker Hill Road, Strasburg, PA 17579, USA E-mail: kstrauss@clinicforspecialchildren.org


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K. A. Strauss, J. Lazovic, M. Wintermark, and D. H. Morton
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W. J. Zinnanti, J. Lazovic, E. B. Wolpert, D. A. Antonetti, M. B. Smith, J. R. Connor, M. Woontner, S. I. Goodman, and K. C. Cheng
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