Dystonic Storm

  • Melissa J. Nirenberg
  • Blair Ford
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Patient 1: A 16-year-old non-Jewish boy had an 8-year history of idiopathic torsion dystonia resulting from the DYT1 mutation. At baseline, he was able to sit upright in a chair, but required help with activities of daily living, including feeding, dressing, and bathing. Treatment with a combination of baclofen, trihexyphenidyl, and clonazepam alleviated some of his symptoms. Trials of levodopa, diazepam, carbamazepine, and phenytoin provided no additional benefit.

Keywords

Fatigue Lithium Dopamine Morphine Meningitis 

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References

  1. 1.
    Fahn S. Concept and classification of dystonia. Adv Neurol 1988; 501–508.Google Scholar
  2. 2.
    Friedman J, Standaert DG. Dystonia and its disorders. Neurol Clin 2001;19:681–705.PubMedCrossRefGoogle Scholar
  3. 3.
    Jankovic J, Penn AS. Severe dystonia and myoglobinuria. Neurology 1982;2:1195–1197.Google Scholar
  4. 4.
    Marsden CD, Marion MH, Quinn N. The treatment of severe dystonia in children and adults. J Neurol Neurosurg Psychiatry 1984;47:1166–1173.PubMedGoogle Scholar
  5. 5.
    Manji H, Howard RS, Miller DH, et al. Status dystonicus: the syndrome and its management. Brain 1998;121:243–252.PubMedCrossRefGoogle Scholar
  6. 6.
    Svetel M, Sternic N, Pejovic S, et al. Penicillamine-induced lethal status dystonicus in a patient with Wilson’s disease. Mov Disord 2001;16:568–569.PubMedCrossRefGoogle Scholar
  7. 7.
    Opal P, Tintner R, Jankovic J, et al. Intrafamilial phenotypic variability of the DYT1 dystonia: from asymptomatic TOR1A gene carrier status to dystonic storm. Mov Disord 2002;17:339–345.PubMedCrossRefGoogle Scholar
  8. 8.
    Dalvi A, Fahn S, Ford B. Intrathecal baclofen in the treatment of dystonic storm. Mov Disord 1998;13:611–612.PubMedCrossRefGoogle Scholar
  9. 9.
    Vaamonde J, Narbona J, Weiser R, et al. Dystonic storms: a practical management problem. Clin Neuropharmacol 1994;17:344–347.PubMedCrossRefGoogle Scholar
  10. 10.
    Paret G, Tirosh R, Ben-Zeev B, et al. Rhabdomyolysis due to hereditary torsion dystonia. Pediatr Neurol 1995;13:83–84.PubMedCrossRefGoogle Scholar
  11. 11.
    Narayan RK, Loubser PG, Jankovic J, et al. Intrathecal baclofen for intractable axial dystonia. Neurology 1991;41:1141–1142.PubMedGoogle Scholar
  12. 12.
    Samson-Fang L, Gooch J, Norlin C. Intrathecal baclofen withdrawal simulating neuroepileptic malignant syndrome in a child with cerebral palsy. Dev Med Child Neurol 2000;42:561–565.PubMedCrossRefGoogle Scholar
  13. 13.
    Watemberg N, Leshner RL, Armstrong BA, et al. Acute pediatric rhabdomyolysis. J Child Neurol 2000;15:222–227.PubMedCrossRefGoogle Scholar
  14. 14.
    Ravi SD, Borge GF, Roach FL. Neuroleptics, laryngeal-pharyngeal dystonia and acute renal failure. J Clin Psychiatry 1982;43:300.PubMedGoogle Scholar
  15. 15.
    Cavanaugh JJ, Finlayson RE. Rhabdomyolysis due to acute dystonic reaction to antipsychotic drugs. J Clin Psychiatry 1984;45:356–357.PubMedGoogle Scholar
  16. 16.
    Delhaas EM, Brouwers JR. Intrathecal baclofen overdose: report of 7 events in 5 patients and review of the literature. Int J Clin Pharmacol Ther Toxicol 1991;29:274–280.PubMedGoogle Scholar
  17. 17.
    Ford B, Greene P, Louis ED, et al. Use of intrathecal baclofen in the treatment of patients with dystonia. Arch Neurol 1996;53:1241–1246.PubMedGoogle Scholar
  18. 18.
    Teddy P, Jamous A, Gardner B, et al. Complications of intrathecal baclofen delivery. Br J Neurosurg 1992;6:115–118.PubMedCrossRefGoogle Scholar
  19. 19.
    Fahn S. High dosage anticholinergic therapy in dystonia. Neurology 1983;33:1255–1261.PubMedGoogle Scholar
  20. 20.
    Penn RD. Intrathecal baclofen for spasticity of spinal origin: seven years of experience. J Neurosurg 1992;77:236–240.PubMedCrossRefGoogle Scholar
  21. 21.
    Kravitz HM, Corcos DM, Hansen G, et al. Intrathecal baclofen. Effects on nocturnal leg muscle spasticity. Am J Phys Med Rehabil 1992;71:48–52.PubMedCrossRefGoogle Scholar
  22. 22.
    Ford B, Greene PE, Louis ED, et al. Intrathecal baclofen in the treatment of dystonia. Adv Neurol 1998;78:199–210.PubMedGoogle Scholar
  23. 23.
    Hou JG, Ondo W, Jankovic J. Intrathecal baclofen for dystonia. Mov Disord 2001;16:1201–1202.PubMedCrossRefGoogle Scholar
  24. 24.
    Albright AL, Barry MJ, Shafton DH, et al. Intrathecal baclofen for generalized dystonia. Dev Med Child Neurol 2001;43:652–657.PubMedCrossRefGoogle Scholar
  25. 25.
    Walker RH, Danisi FO, Swope DM, et al. Intrathecal baclofen for dystonia: benefits and complications during six years of experience. Mov Disord 2000;15:1242–1247.PubMedCrossRefGoogle Scholar
  26. 26.
    Silbert PL, Stewart-Wynne EG. Increased dystonia after intrathecal baclofen. Neurology 1992;42:1639–1640.PubMedGoogle Scholar
  27. 27.
    Jaffe MS, Nienstedt LJ. Intrathecal baclofen for generalized dystonia: a case report. Arch Phys Med Rehabil 2001;82:853–855.PubMedCrossRefGoogle Scholar
  28. 28.
    Penn RD, Gianino JM, York MM. Intrathecal baclofen for motor disorders. Mov Disord 1995;10:675–677.PubMedCrossRefGoogle Scholar
  29. 29.
    Siegfried RN, Jacobson L, Chabal C. Development of an acute withdrawal syndrome following the cessation of intrathecal baclofen in a patient with spasticity. Anesthesiology 1992;77:1048–1050.PubMedCrossRefGoogle Scholar
  30. 30.
    Kupsch A, Kuehn A, Klaffke S, et al. Deep brain stimulation in dystonia. J Neurol 2003;250:I47–I52.PubMedGoogle Scholar
  31. 31.
    Yianni J, Bain PG, Gregory RP, et al. Post-operative progress of dystonia patients following globus pallidus internus deep brain stimulation. Eur J Neurol 2003;10:239–247.PubMedCrossRefGoogle Scholar
  32. 32.
    Krauss JK, Loher TJ, Weigel R, et al. Chronic stimulation of the globus pallidus internus for treatment of non-dYT1 generalized dystonia and choreoathetosis: 2-year follow up. J Neurosurg 2003;9:785–792.Google Scholar
  33. 33.
    Yianni J, Bain P, Giladi N, et al. Globus pallidus internus deep brain stimulation for dystonic conditions: a prospective audit. Mov Disord 2003;18:436–442.PubMedCrossRefGoogle Scholar
  34. 34.
    Krauss JK. Deep brain stimulation for dystonia in adults. Overview and developments. Stereotact Funct Neurosurg 2002;78:168–182.PubMedCrossRefGoogle Scholar
  35. 35.
    Vercueil L, Pollak P, Fraix V, et al. Deep brain stimulation in the treatment of severe dystonia. J Neurol 2001;248:695–700.PubMedCrossRefGoogle Scholar
  36. 36.
    Krack P, Vercueil L. Review of the functional surgical treatment of dystonia. Eur J Neurol 2001;8:389–399.PubMedCrossRefGoogle Scholar
  37. 37.
    Ondo WG, Desaloms JM, Jankovic J, et al. Pallidotomy for generalized dystonia. Mov Disord 1998;13:693–698.PubMedCrossRefGoogle Scholar
  38. 38.
    Beric A, Kelly PJ, Rezai A, et al. Complications of deep brain stimulation surgery. Stereotact Funct Neurosurg 2001;77:73–78.PubMedCrossRefGoogle Scholar
  39. 39.
    Lozano AM, Kumar R, Gross RE, et al. Globus pallidus internus pallidotomy for generalized dystonia. Mov Disord 1997;12:865–870.PubMedCrossRefGoogle Scholar
  40. 40.
    Abosch A, Lozano A. Stereotactic neurosurgery for movement disorders. Can J Neurol Sci 2003;30:S72–S82.PubMedGoogle Scholar
  41. 41.
    Hariz MI, De Salles AA. The side effects and complications of posteroventral pallidotomy. Acta Neurochir Suppl (Wien) 1997;68:42–48.Google Scholar
  42. 42.
    Hariz MI. Complications of deep brain stimulation surgery. Mov Disord 2002;S3:S162–S166.CrossRefGoogle Scholar
  43. 43.
    Angelini L, Nardocci N, Estienne M, et al. Life-threatening dystonia-dyskinesias in a child: successful treatment with bilateral pallidal stimulation. Mov Disord 2000;15:1010–1012.PubMedCrossRefGoogle Scholar

Copyright information

© Humana Press Inc. 2005

Authors and Affiliations

  • Melissa J. Nirenberg
    • 1
  • Blair Ford
    • 1
  1. 1.Department of NeurologyColumbia University Medical CenterNew York

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