Tourette’s syndrome

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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

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.