Definition
- Onset of motor and vocal tics before 18 years of age that cannot be ascribed to another medical condition.
Clinical features
- Tics must occur multiple times over at least 1 year and must evolve over time.
- The first tics are usually observed around the age of 5 or 6
- Tic severity
- Peaks 4-5 years later,
- Lowest early 20s
- Coincident with frontal lobe maturation.
- Tics are worsened by
- Heightened emotional states,
- Stress
- Fatigue.
- The tics of Tourette’s syndrome are commonly accompanied by
- Attention-deficit/hyperactivity disorder (ADHD)
- Obsessive-compulsive disorder (OCD).
- In Tourette’s syndrome
- Obsessions center on concerns with
- Symmetry
- Fear of violent thoughts
- A need to perform activities in a particular manner
- (Rather than fears of contamination and checking seen in primary OCD).
- Obsessions may lead to self-injurious behavior.
Genetic
- Sex-linked autosomal dominant mode of inheritance
Pathophysiology
- Dysfunction in the corticostriatal-thalamocortical loop.
Treatment
- DBS targets
- Thalamic nuclei (centromedian nucleus-parafascicular complex and ventral oral nuclei)
- The limbic (anteromedial) or motor (posteroventral) regions of GPi.
- GPi:
- A double-blind, randomized crossover trial on GPi DBS has reported a significant improvement in tic severity, with an overall acceptable safety profile.