Case ReportsTourette syndrome associated with unilateral cystic changes in the gyrus rectus
Introduction
Gilles de la Tourette syndrome (TS) is a familial disorder manifested by chronic multifocal involuntary motor and vocal tics [1]. Most research has focused on the basal ganglia and its dopaminergic pathways as the involved neurologic substrate, yet isolated abnormalities in other brain regions, often with connections to the basal ganglia, have been demonstrated in patients with TS. Surgical ablative and stimulation studies, as well as neuroimaging with positron emission tomography, single-photon emission computed tomography, magnetic resonance imaging (MRI), and computed tomography, have demonstrated that prefrontal and cingulate gyrus, midbrain, limbic regions, and thalamus are among the brain regions that may be involved in the pathophysiologic features of TS [2], [3], [4].
The exact relationship between TS and dysfunction remote from the basal ganglia is complex. For instance, comorbid conditions of TS, such as obsessive-compulsive disorder and attention-deficit disorder, have also been associated with abnormalities in nonbasal ganglia regions, such as the cingulate gyrus and prefrontal cortex [5], [6]. Whether a similar pathway is dysfunctional in TS is unknown. Nevertheless, most evidence supports the belief that abnormal modulation of circuits involving the basal ganglia and prefrontal, limbic, subcortical, and thalamic regions is the involved substrate.
This is the first report of TS associated with an anatomic abnormality of the gyrus rectus in the orbitofrontal region of the prefrontal cortex. Unilateral cystic changes of the gyrus rectus were demonstrated on MRI in an 11-year-old male with TS. A possible neurobiologic connection between TS and the gyrus rectus is discussed.
Section snippets
Case report
This 11-year-old male presented at 9 years old with a 2-history of frequent motor tics involving his eyes and face, as well as involuntary vocalizations. The tics became exacerbated when he was stressed or excited. Birth history was normal. Although early developmental milestones were mildly delayed, he was an honor roll student in the sixth grade, and his neurologic examination was normal. Mirror movements were absent. Connor’s score for attention-deficit disorder was low. No signs
Discussion
The orbitofrontal region of the prefrontal cortex primarily includes the gyrus rectus and consists mainly of Brodmann’s areas 11 and 13 [7]. The importance of the prefrontal cortex to motor function and activity is demonstrated by its extensive and reciprocating corticocortical circuits (e.g., involving the cingulate gyrus and supplementary motor areas) and corticosubcortical circuits (e.g., involving the basal ganglia and thalamus).
Connections from the prefrontal cortex, including the
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