Malignant Tourette syndrome

  • Rare, potentially life-threatening complications secondary to severe tics
  • Presentation may include:
    • Self-injurious tics (eg tics of the neck producing “whiplash” injury and compressive myelopathy)
    • Severe self-injurious behavior in response to inner obsessions or the need to satisfy a sexual urge
    • Violent, uncontrollable shouting, coprolalia or copropraxia
    • Extreme rage attacks, depression and suicidal ideation
  • Symptoms are difficult to treat with typical medications for tic suppression such as monoamine-depleters, neuroleptics or topiramate
  • Patients with malignant Tourette syndrome are more likely to have a history of obsessive-compulsive disorder, complex phonic tics, corpolalia, copropraxia, mood disorder, suicidal ideation and poor response to medications than patients with non-malignant Tourette syndrome

Diagnosis

Definition of malignant Tourette syndrome:

≥2 emergency room visits or ≥1 hospitalizations for Tourette syndrome symptoms or associated behavioral comorbidities

Management

  • Neuroleptics are usually the first line of treatment. Haloperidol, pimozide, fluphenazine, risperidone as well as atypical neuroleptics such as aripiprazole, olanzapine, quetiapine, and ziprasidone may be effective
  • The monoamine-depleting agent tetrabenazine may be effective in reducing tic severity
  • Antiadrenergic drugs (eg clonidine) have shown moderate benefit for tics
  • Behavioral techniques such as habit-reversal training and distraction tasks may provide some benefit
  • Botulinum toxin injections are useful in treating focal motor and phonic tics
  • Deep brain stimulation may be considered in selected medication-refractory patients



References

  • Diagnostic and statistical manual for mental disorders. 4th ed. Washington, DC: American Psychiatric Association, 1994:739-42.
  • Malignant Tourette syndrome. Mov Disord. 2007;22: 1743–50 doi: 10.1002/mds.21599
  • Movement Disorder Emergencies Diagnosis and Treatment, Second edition Humana Press (2013) ISBN 978-1-60761-834-8
  • Movement disorder emergencies. J Neurol 2008;255 Suppl4:2-13.doi: 10.1007/s00415-008-4002-9
  • Botulinum toxin into vocal cord in the treatment of malignant coprolalia associated with Tourette’s syndrome. Mov Disord. 1996;11:431–3 doi: 10.1002/mds.870110413