## Abstract ## Objective Brain metabolism, as studied by magnetic resonance spectroscopy (MRS), has been previously shown to be abnormal in Rett syndrome (RTT). This study reports the relation of MRS findings to age, disease severity, and genotype. ## Methods Forty RTT girls (1–14 years old) and
Abnormal brain tryptophan metabolism and clinical correlates in Tourette syndrome
✍ Scribed by Michael Behen; Harry T. Chugani; Csaba Juhász; Emily Helder; Albert Ho; Mohsin Maqbool; Robert D. Rothermel; Jacquie Perry; Otto Muzik
- Publisher
- John Wiley and Sons
- Year
- 2007
- Tongue
- English
- Weight
- 101 KB
- Volume
- 22
- Category
- Article
- ISSN
- 0885-3185
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✦ Synopsis
Abstract
Symptoms in Tourette syndrome (TS) are likely related to abnormalities involving multiple neurotransmitter systems in striatal‐thalamo‐cortical circuitry. Although prior studies have found abnormal levels of tryptophan, serotonin, and their metabolites in blood, cerebrospinal fluid and brain tissue of TS patients, understanding of focal brain disturbances and their relationship to clinical phenotype remains poor. We used α‐[^11^C]methyl‐L‐tryptophan (AMT) positron emission tomography (PET) to assess global and focal brain abnormalities of tryptophan metabolism and their relationship to behavioral phenotype in 26 children with TS and nine controls. Group comparisons on regional cortical and subcortical AMT uptake revealed decreased AMT uptake in bilateral dorsolateral prefrontal cortical and bilaterally increased uptake in the thalamus (P = 0.001) in TS children. The ratio of AMT uptake in subcortical structures to dorsolateral prefrontal cortex was significantly increased bilaterally (P < 0.01) in TS patients also. Behaviorally defined subgroups within the TS sample revealed differences in the pattern of AMT uptake in the fronto‐striatal‐thalamic circuit. This study demonstrates cortical and subcortical abnormalities of tryptophan metabolism in TS and provides neuroimaging evidence for a role of serotonergic mechanisms in the pathophysiology of TS. © 2007 Movement Disorder Society
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