A personal 35 year perspective on Gilles de la Tourette syndrome: assessment, investigations, and management.

After having examined the definition, clinical phenomenology, comorbidity, psychopathology, and phenotypes in the first paper of this Series, here I discuss the assessment, including neuropsychology, and the effects of Gilles de la Tourette syndrome with studies showing that the quality of life of patients with Tourette's syndrome is reduced and that there is a substantial burden on the family. In this paper, I review my local and collaborative studies investigating causal factors (including genetic vulnerability, prenatal and perinatal difficulties, and neuro-immunological factors). I also present my studies on neuro-imaging, electro-encephalograms, and other special investigations, which are helpful in their own right or to exclude other conditions. Finally, I also review our studies on treatment including medications, transcranial magnetic stimulation, biofeedback, target-specific botulinum toxin injections, biofeedback and, in severe refractory adults, psychosurgery and deep brain stimulation. This Review summarises and highlights selected main findings from my clinic (initially The National Hospital for Neurology and Neurosurgery Queen Square and University College London, UK, and, subsequently, at St George's Hospital, London, UK), and several collaborations since 1980. As in Part 1 of this Series, I address the main controversies in the fields and the research of other groups, and I make suggestions for future research.

Post-traumatic tics and tetrabenazine treatment: a blinded video assessment.

Traumatic head injury is a very rare cause of secondary tic disorders. We add another case by describing, for the first time, the response to tetrabenazine in a blinded video assessment. Our patient had a severe traumatic head injury and subsequently developed tics refractory to various agents including neuroleptics. We assessed tetrabenazine treatment by virtue of patient's impression, the treating neurologist's non-blinded (Yale Global Tic Severity Scale) and a second neurologist's blinded assessment (modified Rush Video Scale). The Yale Global Tic Severity Score improved by 24% on 12.5 mg twice daily and 45% on 12.5 mg thrice daily. Subjective improvement was 50% and 70%, respectively. The modified Rush Video scores improved by 21% and 28.5%, respectively. Post-traumatic tourettism can respond to tetrabenazine. The magnitude of benefit though, may be overestimated with open-label observations, thus there is a need for studies examining objectively the effect of tetrabenazine in tic disorders.