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Semin Neurol 2020; 40(05): 485-491
DOI: 10.1055/s-0040-1713845
Review Article

Paroxysmal Sympathetic Hyperactivity

Rachael A. Scott
1   Department of Pharmacy, Mayo Clinic, Rochester, Minnesota
,
Alejandro A. Rabinstein
2   Department of Neurology, Mayo Clinic, Rochester, Minnesota
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Abstract

Paroxysmal sympathetic hyperactivity (PSH) is a relatively common, but often unrecognized, complication of acute diffuse or multifocal brain diseases, most frequently encountered in young comatose patients with severe traumatic brain injury. It is presumed to be caused by loss of cortical inhibitory modulation of diencephalic and brain stem centers and possible additional maladaptive changes in the spinal cord that combine to produce exaggerated sympathetic responses to stimulation. The syndrome consists of repeated sudden episodes of tachycardia, tachypnea, hypertension, sweating, and sometimes fever and dystonic posturing. The diagnosis is clinical. Treatment includes reducing any external stimulation that can trigger the episodes, and starting abortive (e.g., intravenous morphine) and preventive medications (e.g., gabapentin, propranolol, clonidine). Prompt and adequate treatment of PSH may reduce the likelihood of secondary complications, such as dehydration, weight loss and malnutrition, and muscle contractures.

Keywords

paroxysmal sympathetic hyperactivity - dysautonomia - autonomic storms - hypertension - tachycardia


Publication History

Article published online:
09 September 2020

© 2020. Thieme. All rights reserved.

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  • References

  • 1 Meyfroidt G, Baguley IJ, Menon DK. Paroxysmal sympathetic hyperactivity: the storm after acute brain injury. Lancet Neurol 2017; 16 (09) 721-729
    CrossrefPubMedGoogle Scholar
  • 2 Hughes JD, Rabinstein AA. Early diagnosis of paroxysmal sympathetic hyperactivity in the ICU. Neurocrit Care 2014; 20 (03) 454-459
    CrossrefPubMedGoogle Scholar
  • 3 Mathew MJ, Deepika A, Shukla D, Devi BI, Ramesh VJ. Paroxysmal sympathetic hyperactivity in severe traumatic brain injury. Acta Neurochir (Wien) 2016; 158 (11) 2047-2052
    CrossrefPubMedGoogle Scholar
  • 4 Rabinstein AA, Benarroch EE. Treatment of paroxysmal sympathetic hyperactivity. Curr Treat Options Neurol 2008; 10 (02) 151-157
    CrossrefPubMedGoogle Scholar
  • 5 Perkes I, Baguley IJ, Nott MT, Menon DK. A review of paroxysmal sympathetic hyperactivity after acquired brain injury. Ann Neurol 2010; 68 (02) 126-135
    CrossrefPubMedGoogle Scholar
  • 6 Blackman JA, Patrick PD, Buck ML, Rust Jr RS. Paroxysmal autonomic instability with dystonia after brain injury. Arch Neurol 2004; 61 (03) 321-328
    CrossrefPubMedGoogle Scholar
  • 7 Baguley IJ, Perkes IE, Fernandez-Ortega JF, Rabinstein AA, Dolce G, Hendricks HT. Consensus Working Group. Paroxysmal sympathetic hyperactivity after acquired brain injury: consensus on conceptual definition, nomenclature, and diagnostic criteria. J Neurotrauma 2014; 31 (17) 1515-1520
    CrossrefPubMedGoogle Scholar
  • 8 Rabinstein AA. Paroxysmal sympathetic hyperactivity in the neurological intensive care unit. Neurol Res 2007; 29 (07) 680-682
    CrossrefPubMedGoogle Scholar
  • 9 Godoy DA, Di Napoli M, Rabinstein AA. Cerebral fat embolism: recognition, complications, and prognosis. Neurocrit Care 2018; 29 (03) 358-365
    CrossrefPubMedGoogle Scholar
  • 10 Mittal MK, Rabinstein AA, Hocker SE, Pittock SJ, Wijdicks EF, McKeon A. Autoimmune encephalitis in the ICU: analysis of phenotypes, serologic findings, and outcomes. Neurocrit Care 2016; 24 (02) 240-250
    CrossrefPubMedGoogle Scholar
  • 11 Hinson HE, Schreiber MA, Laurie AL, Baguley IJ, Bourdette D, Ling GSF. Early fever as a predictor of paroxysmal sympathetic hyperactivity in traumatic brain injury. J Head Trauma Rehabil 2017; 32 (05) E50-E54
    CrossrefPubMedGoogle Scholar
  • 12 Lv LQ, Hou LJ, Yu MK. et al. Prognostic influence and magnetic resonance imaging findings in paroxysmal sympathetic hyperactivity after severe traumatic brain injury. J Neurotrauma 2010; 27 (11) 1945-1950
    CrossrefPubMedGoogle Scholar
  • 13 Laxe S, Terré R, León D, Bernabeu M. How does dysautonomia influence the outcome of traumatic brain injured patients admitted in a neurorehabilitation unit?. Brain Inj 2013; 27 (12) 1383-1387
    CrossrefPubMedGoogle Scholar
  • 14 van Eijck MM, Sprengers MOP, Oldenbeuving AW, de Vries J, Schoonman GG, Roks G. The use of the PSH-AM in patients with diffuse axonal injury and autonomic dysregulation: a cohort study and review. J Crit Care 2019; 49: 110-117
    CrossrefPubMedGoogle Scholar
  • 15 Baguley IJ, Slewa-Younan S, Heriseanu RE, Nott MT, Mudaliar Y, Nayyar V. The incidence of dysautonomia and its relationship with autonomic arousal following traumatic brain injury. Brain Inj 2007; 21 (11) 1175-1181
    CrossrefPubMedGoogle Scholar
  • 16 Alofisan TO, Algarni YA, Alharfi IM. et al. Paroxysmal sympathetic hyperactivity after severe traumatic brain injury in children: prevalence, risk factors, and outcome. Pediatr Crit Care Med 2019; 20 (03) 252-258
    CrossrefPubMedGoogle Scholar
  • 17 Baguley IJ. The excitatory:inhibitory ratio model (EIR model): an integrative explanation of acute autonomic overactivity syndromes. Med Hypotheses 2008; 70 (01) 26-35
    CrossrefPubMedGoogle Scholar
  • 18 Baguley IJ, Heriseanu RE, Nott MT, Chapman J, Sandanam J. Dysautonomia after severe traumatic brain injury: evidence of persisting overresponsiveness to afferent stimuli. Am J Phys Med Rehabil 2009; 88 (08) 615-622
    CrossrefPubMedGoogle Scholar
  • 19 Huang P, Lin WC, Huang PK, Khor GT. Susceptibility weighted imaging in a patient with paroxysmal sympathetic storms. J Neurol 2009; 256 (02) 276-278
    CrossrefPubMedGoogle Scholar
  • 20 Fernández-Ortega JF, Prieto-Palomino MA, Quesada-García G, Barrueco-Francioni J. Findings in the magnetic resonance of paroxysmal sympathetic hyperactivity. J Neurotrauma 2011; 28 (07) 1327-1328
    CrossrefPubMedGoogle Scholar
  • 21 Fernández-Ortega JF, Prieto-Palomino MA, Muñoz-López A, Lebron-Gallardo M, Cabrera-Ortiz H, Quesada-García G. Prognostic influence and computed tomography findings in dysautonomic crises after traumatic brain injury. J Trauma 2006; 61 (05) 1129-1133
    CrossrefPubMedGoogle Scholar
  • 22 Hinson HE, Puybasset L, Weiss N. , et al; Neuro Imaging for Coma Emergence, Recovery (NICER) Consortium. Neuroanatomical basis of paroxysmal sympathetic hyperactivity: a diffusion tensor imaging analysis. Brain Inj 2015; 29 (04) 455-461
    CrossrefPubMedGoogle Scholar
  • 23 Perkes IE, Menon DK, Nott MT, Baguley IJ. Paroxysmal sympathetic hyperactivity after acquired brain injury: a review of diagnostic criteria. Brain Inj 2011; 25 (10) 925-932
    CrossrefPubMedGoogle Scholar
  • 24 Fernandez-Ortega JF, Baguley IJ, Gates TA, Garcia-Caballero M, Quesada-Garcia JG, Prieto-Palomino MA. Catecholamines and paroxysmal sympathetic hyperactivity after traumatic brain injury. J Neurotrauma 2017; 34 (01) 109-114
    CrossrefPubMedGoogle Scholar
  • 25 Samuel S, Lee M, Brown RJ, Choi HA, Baguley IJ. Incidence of paroxysmal sympathetic hyperactivity following traumatic brain injury using assessment tools. Brain Inj 2018; 32 (09) 1115-1121
    CrossrefPubMedGoogle Scholar
  • 26 Lump D, Moyer M. Paroxysmal sympathetic hyperactivity after severe brain injury. Curr Neurol Neurosci Rep 2014; 14 (11) 494
    CrossrefPubMedGoogle Scholar
  • 27 Caldwell SB, Smith D, Wilson FC. Impact of paroxysmal sympathetic hyperactivity on nutrition management after brain injury: a case series. Brain Inj 2014; 28 (03) 370-373
    CrossrefPubMedGoogle Scholar
  • 28 Mehta NM, Bechard LJ, Leavitt K, Duggan C. Severe weight loss and hypermetabolic paroxysmal dysautonomia following hypoxic ischemic brain injury: the role of indirect calorimetry in the intensive care unit. JPEN J Parenter Enteral Nutr 2008; 32 (03) 281-284
    CrossrefPubMedGoogle Scholar
  • 29 Thomas A, Greenwald BD. Paroxysmal sympathetic hyperactivity and clinical considerations for patients with acquired brain injuries: a narrative review. Am J Phys Med Rehabil 2019; 98 (01) 65-72
    CrossrefPubMedGoogle Scholar
  • 30 Baguley IJ, Cameron ID, Green AM, Slewa-Younan S, Marosszeky JE, Gurka JA. Pharmacological management of Dysautonomia following traumatic brain injury. Brain Inj 2004; 18 (05) 409-417
    CrossrefPubMedGoogle Scholar
  • 31 Baguley IJ, Heriseanu RE, Gurka JA, Nordenbo A, Cameron ID. Gabapentin in the management of dysautonomia following severe traumatic brain injury: a case series. J Neurol Neurosurg Psychiatry 2007; 78 (05) 539-541
    CrossrefPubMedGoogle Scholar
  • 32 Rose MA, Kam PC. Gabapentin: pharmacology and its use in pain management. Anaesthesia 2002; 57 (05) 451-462
    CrossrefPubMedGoogle Scholar
  • 33 Pucks-Faes E, Hitzenberger G, Matzak H, Verrienti G, Schauer R, Saltuari L. Intrathecal baclofen in paroxysmal sympathetic hyperactivity: impact on oral treatment. Brain Behav 2018; 8 (11) e01124
    CrossrefPubMedGoogle Scholar
  • 34 Morinaga Y, Nii K, Sakamoto K, Inoue R, Mitsutake T, Hanada H. Efficacy of trazodone for treating paroxysmal sympathetic hyperactivity presenting after thalamic hemorrhage: a case report. Drug Discov Ther 2019; 13 (03) 168-171
    CrossrefPubMedGoogle Scholar
  • 35 Pozzi M, Conti V, Locatelli F. et al. Paroxysmal sympathetic hyperactivity in pediatric rehabilitation: clinical factors and acute pharmacological management. J Head Trauma Rehabil 2015; 30 (05) 357-363
    CrossrefPubMedGoogle Scholar
  • 36 Fernandez-Ortega JF, Prieto-Palomino MA, Garcia-Caballero M, Galeas-Lopez JL, Quesada-Garcia G, Baguley IJ. Paroxysmal sympathetic hyperactivity after traumatic brain injury: clinical and prognostic implications. J Neurotrauma 2012; 29 (07) 1364-1370
    CrossrefPubMedGoogle Scholar
  • 37 Hendricks HT, Geurts AC, van Ginneken BC, Heeren AJ, Vos PE. Brain injury severity and autonomic dysregulation accurately predict heterotopic ossification in patients with traumatic brain injury. Clin Rehabil 2007; 21 (06) 545-553
    CrossrefPubMedGoogle Scholar