Advertisement

Hemiballism–Hemichorea

  • Ronald B. PostumaEmail author
  • Anthony E. LangEmail author
Chapter
Part of the Current Clinical Neurology book series (CCNEU)

Abstract

Hemiballism is an uncommon movement disorder that presents with unilateral flinging movements of the limbs. It varies considerably in intensity and severity—often in its acute phase it can be of sufficient severity to present as a true emergency. It is most classically associated with ischemic lesions to the subthalamic nucleus (STN), although the majority of cases involve basal ganglia structures outside the STN. An important recently described association is with diabetes, during crises of severe non-ketotic hyperosmolar hyperglycemia. Pathophysiology is related to abnormal firing patterns in the globus pallidus interna, with intermittent firing bursts followed by pauses during which movements occur. The key treatment is anti-dopaminergic therapy, either with dopamine blockers such as neuroleptics or with dopamine depleters such as tetrabenazine. In extreme cases, functional neurosurgery can be performed; either lesioning or deep brain stimulation of the globus pallidus can be considered the treatments of choice. Most patients respond well to medical treatment and spontaneous resolution is common.

Keywords

Basal Ganglion Deep Brain Stimulation Globus Pallidus Subthalamic Nucleus Sodium Valproate 
These keywords were added by machine and not by the authors. This process is experimental and the keywords may be updated as the learning algorithm improves.

Supplementary material

Movement_Disorders_Emergencies_C12.mov (77,617 KB)

References

  1. 1.
    Dewey Jr RB, Jankovic J. Hemiballism-hemichorea. Clinical and pharmacologic findings in 21 patients. Arch Neurol. 1989;46:862–7.PubMedCrossRefGoogle Scholar
  2. 2.
    Ghika-Schmid F, Ghika J, Regli F, Bogousslavsky J. Hyperkinetic movement disorders during and after acute stroke: the Lausanne Stroke Registry. J Neurol Sci. 1997;146:109–16.PubMedCrossRefGoogle Scholar
  3. 3.
    Pekmezovic T, Svetel M, Ristic A, et al. Incidence of vascular hemiballism in the population of Belgrade. Mov Disord. 2004;19:1469–72.PubMedCrossRefGoogle Scholar
  4. 4.
    Ristic A, Marinkovic J, Dragasevic N, Stanisavljevic D, Kostic V. Long-term prognosis of vascular hemiballismus. Stroke. 2002;33:2109–11.PubMedCrossRefGoogle Scholar
  5. 5.
    Chung SJ, Im JH, Lee MC, Kim JS. Hemichorea after stroke: clinical-radiological correlation. J Neurol. 2004;251:725–9.PubMedCrossRefGoogle Scholar
  6. 6.
    Postuma RB, Lang AE. Hemiballism: revisiting a classic disorder. Lancet Neurol. 2003;2:661–8.PubMedCrossRefGoogle Scholar
  7. 7.
    Vidakovic A, Dragasevic N, Kostic VS. Hemiballism: report of 25 cases. J Neurol Neurosurg Psychiatry. 1994;57:945–9.PubMedCrossRefGoogle Scholar
  8. 8.
    Oh SH, Lee KY, Im JH, Lee MS. Chorea associated with non-ketotic hyperglycemia and hyperintensity basal ganglia lesion on T1-weighted brain MRI study: a meta-analysis of 53 cases including four present cases. J Neurol Sci. 2002;200:57–62.PubMedCrossRefGoogle Scholar
  9. 9.
    Cherian A, Thomas B, Baheti NN, Chemmanam T, Kesavadas C. Concepts and controversies in nonketotic hyperglycemia-induced hemichorea: further evidence from susceptibility-weighted MR imaging. J Magn Reson Imaging. 2009;29:699–703.PubMedCrossRefGoogle Scholar
  10. 10.
    Hsu JL, Wang HC, Hsu WC. Hyperglycemia-induced unilateral basal ganglion lesions with and without hemichorea A PET study. J Neurol. 2004;251:1486–90.PubMedCrossRefGoogle Scholar
  11. 11.
    Shan DE. Hemichorea-hemiballism associated with hyperintense putamen on T1-weighted MR images: an update and a hypothesis. Acta Neurol Taiwan. 2004;13:170–7.PubMedGoogle Scholar
  12. 12.
    Ohara S, Nakagawa S, Tabata K, Hashimoto T. Hemiballism with hyperglycemia and striatal T1-MRI hyperintensity: an autopsy report. Mov Disord. 2001;16:521–5.PubMedCrossRefGoogle Scholar
  13. 13.
    Maeda K, Katayama Y, Sugimoto T, et al. Activated microglia in the subthalamic nucleus in hyperglycaemic hemiballism: a case report. J Neurol Neurosurg Psychiatry. 2010;81(10):1175–7. Epub 2010 Jun 14.PubMedCrossRefGoogle Scholar
  14. 14.
    Nath J, Jambhekar K, Rao C, Armitano E. Radiological and pathological changes in hemiballism-hemichorea with striatal hyperintensity. J Magn Reson Imaging. 2006;23:564–8.PubMedCrossRefGoogle Scholar
  15. 15.
    Galea I, Norwood F, Phillips MJ, Shearman C, McMonagle P, Gibb WR. Pearls & Oy-sters: resolution of hemichorea following endarterectomy for severe carotid stenosis. Neurology. 2008;71:e80–2.PubMedCrossRefGoogle Scholar
  16. 16.
    Carpenter MB, Whittier JR, Mettler FA. Analysis of choroid hyperkinesia in the rhesus monkey: surgical and pharmacological analysis of hyperkinesia resulting from lesions in the subthalamic nucleus of luys. J Comp Neurol. 1950;92:293–331.PubMedCrossRefGoogle Scholar
  17. 17.
    Crossman AR, Sambrook MA, Jackson A. Experimental hemichorea/hemiballismus in the monkey. Studies on the intracerebral site of action in a drug-induced dyskinesia. Brain. 1984;107(Pt 2):579–96.PubMedCrossRefGoogle Scholar
  18. 18.
    DeLong MR. Primate models of movement disorders of basal ganglia origin. Trends Neurosci. 1990;13:281–5.PubMedCrossRefGoogle Scholar
  19. 19.
    Guridi J, Obeso JA. The subthalamic nucleus, hemiballismus and Parkinson’s disease: reappraisal of a neurosurgical dogma. Brain. 2001;124:5–19.PubMedCrossRefGoogle Scholar
  20. 20.
    Lenz FA, Suarez JI, Metman LV, et al. Pallidal activity during dystonia: somatosensory reorganisation and changes with severity. J Neurol Neurosurg Psychiatry. 1998;65:767–70.PubMedCrossRefGoogle Scholar
  21. 21.
    Suarez JI, Metman LV, Reich SG, Dougherty PM, Hallett M, Lenz FA. Pallidotomy for hemiballismus: efficacy and characteristics of neuronal activity. Ann Neurol. 1997;42:807–11.PubMedCrossRefGoogle Scholar
  22. 22.
    Vitek JL, Chockkan V, Zhang JY, et al. Neuronal activity in the basal ganglia in patients with generalized dystonia and hemiballismus. Ann Neurol. 1999;46:22–35.PubMedCrossRefGoogle Scholar
  23. 23.
    Hyland HH, Forman DM. Prognosis in hemiballismus. Neurology. 1957;7:381–91.PubMedCrossRefGoogle Scholar
  24. 24.
    Klawans HL, Moses III H, Nausieda PA, Bergen D, Weiner WJ. Treatment and prognosis of hemiballismus. N Engl J Med. 1976;295:1348–50.PubMedCrossRefGoogle Scholar
  25. 25.
    Johnson WG, Fahn S. Treatment of vascular hemichorea and hemiballismus with perphenazine. Trans Am Neurol Assoc. 1974;99:222–4.PubMedGoogle Scholar
  26. 26.
    Sitburana O, Ondo WG. Tetrabenazine for hyperglycemic-induced hemichorea-hemiballismus. Mov Disord. 2006;21:2023–5.PubMedCrossRefGoogle Scholar
  27. 27.
    Ranawaya R, Lang AE. Neurological emergencies in movement disorders. In: Weiner W, editor. Emergent and urgent neurology. 1st ed. Philadelphia: J.B. Lippincott; 1992. p. 277–319.Google Scholar
  28. 28.
    Marchione P, Vento C, Marianetti M, Romeo T, Amabile GA, Giacomini P. Hemiballismus in subthalamic haemorrhage: efficacy of levetiracetam. Eur J Neurol. 2009;16:e112–3.PubMedCrossRefGoogle Scholar
  29. 29.
    Obeso JA, Marti-Masso JF, Astudillo W, De La PE, Carrera N. Treatment with hemiballism with reserpine. Ann Neurol. 1978;4:581.PubMedCrossRefGoogle Scholar
  30. 30.
    Driver-Dunckley E, Evidente VG. Hemichorea-hemiballismus may respond to topiramate. Clin Neuropharmacol. 2005;28:142–4.PubMedCrossRefGoogle Scholar
  31. 31.
    Kothare SV, Pollack P, Kulberg AG, Ravin PD. Gabapentin treatment in a child with delayed-onset hemichorea/hemiballismus. Pediatr Neurol. 2000;22:68–71.PubMedCrossRefGoogle Scholar
  32. 32.
    Krauss JK, Pohle T, Borremans JJ. Hemichorea and hemiballism associated with contralateral hemiparesis and ipsilateral basal ganglia lesions. Mov Disord. 1999;14:497–501.PubMedCrossRefGoogle Scholar
  33. 33.
    Riley D, Lang AE. Hemiballism in multiple sclerosis. Mov Disord. 1988;3:88–94.PubMedCrossRefGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2013

Authors and Affiliations

  1. 1.Department of NeurologyMcGill University, Montreal General HospitalMontrealCanada
  2. 2.Morton and Gloria Shulman Movement Disorders Center and the Edmond J. Safra Program in Parkinson’s Disease, Toronto Western HospitalUniversity of TorontoTorontoCanada

Personalised recommendations