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Altered neurochemical markers in Rett's syndrome

Neuromnemonics Laboratory (Dr. Wenk), Department of Psychology, The Johns Hopkins University; Kennedy Institute for Handicapped Children (S. Naidu and Dr. Moser), Department of Neurology and Pediatrics, The Johns Hopkins Hospital, Baltimore, MD; Clinical Brain Disorders Branch (Dr. Casanova), Intramural Research Program, National Institute of Mental Health, Washington, DC; and the Neuropathology Laboratory (Dr. Kitt), Department of Pathology, The Johns Hopkins University School of Medicine, Baltimore, MD.

Rett's syndrome (RS) is a neurologic disorder associated with severe mental deficiency and neurologic manifestations of cortical and extrapyramidal origin. The present report is a preliminary postmortem brain study that compares the levels of endogenous biogenic amines and selected neurotransmitter receptors in five cases with RS and six normal controls of similar age. The level of choline acetyltransferase activity was reduced in several cortical and subcortical regions. Endogenous levels of dopamine in the superior frontal and superior temporal gyri, occipital cortex, and putamen were reduced. The changes in specific neurotransmitter markers, particularly those associated with the basal ganglia and cortex, may underlie the progressive deterioration in motor and cognitive function characteristic of this progressive disorder.

Address correspondence and reprint requests to Dr. G.L. Wenk, Division of Neural Systems, Memory and Aging, 384 Life Sciences North Building, University of Arizona Health Sciences Center, Tucson, AZ 85724.

Supported by PHS grant NIH PO1-HD-23540.

Received January 28, 1991. Accepted for publication in final form April 26, 1991.

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