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Rett Syndrome: 1H Spectroscopic Imaging at 4.1 Tesla

Center for Nuclear Imaging Research, University of Alabama at Birmingham, AL

Jane B. Lane, BSN

Department of Pediatrics, University of Alabama at Birmingham, AL, Department of Neurology, University of Alabama at Birmingham, AL

Hoby Hetherington, PhD

Center for Nuclear Imaging Research, University of Alabama at Birmingham, AL, Department of Medicine University of Alabama at Birmingham, AL

Alan K. Percy, MD

Department of Pediatrics, University of Alabama at Birmingham, AL, apercy{at}peds.uab.edu, Department of Neurology, University of Alabama at Birmingham, AL

Rett syndrome, a neurodevelopmental disorder predominantly affecting girls, is characterized by regression of psychomotor development, communication dysfunction, and hand stereotypies. Brain morphologic studies demonstrate increased neuronal packing density and reduced dendritic arborizations, suggesting an arrest or interruption of normal maturation. Numerous neurotransmitter systems have been implicated. Among these, cerebrospinal fluid glutamate levels are elevated and glutamate receptors, particularly in putamen, are reduced. Therefore, ¹H spectroscopy at 4.1 Tesla was used to evaluate glutamate, creatine, and N-acetylaspartate in six girls with Rett syndrome and four normal sibling controls. The ratio of creatine to N-acetylaspartate was significantly elevated in white matter, primarily reflecting reduced N-acetylaspartate levels, and normal in gray matter. The glutamate to N-acetylaspartate ratio was elevated in gray matter and normal in white matter. These findings are consistent with previous neuropathologic and neurochemical findings and indicate the feasibility of imaging these metabolites in vivo. (J Child Neurol 1999;14:524-528).

Journal of Child Neurology, Vol. 14, No. 8, 524-528 (1999)
DOI: 10.1177/088307389901400808


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