Neurophysiology of Tourette's syndrome: pathophysiological considerations.
At present the neurophysiology of Tourette's syndrome (TS) has been investigated largely from two perspectives; one for evaluation of the dysfunction of the cerebral cortex and the other for clarification of the neuronal mechanisms that underlie tics and related symptoms. For the former the following examinations have been conducted: quantitative analyses of scalp electroencephalography (EEG), premovement EEG potentials, contingent negative variation, transcranial magnetic stimulation, and neuroimaging studies, including echo-planar images and positron emission tomography scans. These explorations have revealed the likely involvement of the subcortical and the cortical structures, particularly of the basal ganglia, in the pathophysiology of TS. For the latter, surface electromyography, evoked potentials, saccadic eye movements, and polysomnographies have been performed, and again have suggested a dysfunction of the basal ganglia and the brainstem neurons in TS patients. These neurophysiological studies suggest dysfunction of both motor and non-motor basal ganglia-thalamocortical circuitries in TS patients, which is hypothesized to be caused by hypofunction of the dopamine (DA) neurons associated with DA receptor supersensitivity, a well as hypofunction of the serotonergic neurons of the brainstem. Polysomnographical examination suggests that the dysfunction of the nigrostriatal (NS)-DA neurons is not a progressive process, but that the dysfunction is closely associated with an early occurrence of the developmental decrement of the activities of the NS-DA system to mature in a normal fashion. The associated DA receptor supersensitivity is assumed to be a consequence of this developmental abnormality and not due to denervation supersensitivity.
Segawa Neurological Clinic for Children, Tokyo, Japan. segawa@t3.rim.or.jp
Masaya
M
Segawa
D001479:Basal Ganglia / Q000254:growth & development / Q000378:metabolism / Q000503:physiopathology; D001921:Brain / Q000254:growth & development / Q000378:metabolism / Q000503:physiopathology*; D004298:Dopamine / Q000378:metabolism; D004568:Electrodiagnosis; D006801:Humans; D009434:Neural Pathways / Q000254:growth & development / Q000378:metabolism / Q000503:physiopathology*; D017286:Polysomnography; D011954:Receptors, Dopamine / Q000378:metabolism; D012701:Serotonin / Q000378:metabolism; D013378:Substantia Nigra / Q000254:growth & development / Q000378:metabolism / Q000503:physiopathology; D005879:Tourette Syndrome / Q000175:diagnosis / Q000503:physiopathology*
D011954:Receptors, Dopamine; D012701:Serotonin; D004298:Dopamine