Dystonic storm

  • “Status dystonicus” or “dystonic storm” can occur in patients with idiopathic or symptomatic generalized dystonia
  • Unremitting spasms lead to hyperpyrexia, dehydration, respiratory failure, rhabdomyolysis and renal failure
  • The spasms are typically refractory to common medication used to treat dystonia

Differential

  • Neuroleptic malignant syndrome
  • Serotonin syndrome
  • Malignant hyperthermia
  • Intrathecal baclofen withdrawal
  • Bacterial meningitis
  • Paraneoplastic encephalitis
  • Generalized tetanus
  • Stiff-person syndrome

Conditions that may cause dystonic storm

  • Infections
  • Medication adjustments
  • Hyperthermia
  • Trauma
  • Dehydration
  • Respiratory compromise
  • Acute exposure to neuroleptics
  • Neurodegenerative disorders

Management

  • Admit to ICU and monitor airway and cardiopulmonary status
  • Provide supportive care, hydrate, maintain euthermia with antipyretics and passive cooling
  • Provide mild sedation. Midazolam drip has direct muscle relaxant effect and is a good initial choice
  • General anesthesia, paralyzing agents and intrathecal baclofen may be required in refractory cases
  • Dystonia-specific medical treatment may be unsuccessful in controlling the spasms
  • Begin with monoamine-depleting agents (tetrabenazine) and gradually add anticholinergics and/or dopamine receptor blocking agents
  • Baclofen and benzodiazepines may also be effective, however benzodiazepines have been reported to precipitate dystonic storm. Use as second or third line agents
  • Pallidotomy and deep brain stimulation have been used in the treatment of dystonic storm



References

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  • A critical review of the pathophysiology of dysautonomia following traumatic brain injury. Neurocrit Care. 2008;8:293–300.doi: 10.1007/s12028-007-9021-3
  • Paroxysmal autonomic instability with dystonia after brain injury. Arch Neurol. 2004 Mar;61(3):321-8. doi: 10.1001/archneur.61.3.321
  • Paroxysmal sympathetic storms (“diencephalic seizures”) after severe diffuse axonal head injury. Mayo Clin Proc. 1998;73:148–52 doi: 10.1016/S0025-6196(11)63647-1
  • Dystonic storm due to Batten’s disease treated with pallidotomy and deep brain stimulation. Mov Disord. 2009;24:1048–53. doi: 10.1002/mds.22515
  • Emergency department presentations of patients with Parkinson’s dis- ease. Am J Emerg Med. 2000;18:209–15 doi: 10.1016/s0735-6757(00)90023-8
  • Movement Disorder Emergencies Diagnosis and Treatment, Second edition Humana Press (2013) ISBN 978-1-60761-834-8
  • Status dystonicus: the syndrome and its management. Brain 1998;121(Pt 2):243-52. doi: 10.1093/brain/121.2.243
  • Movement disorder emergencies. J Neurol 2008;255 Suppl4:2-13.doi: 10.1007/s00415-008-4002-9
  • Status dystonicus: study of five cases. Arq Neuropsiquiatr. 2005;63:26–9. doi: 10.1590/s0004-282x2005000100005