Abstract
Paroxysmal sympathetic hyperactivity develops following acute brain insult from diverse pathological conditions. The episodic sympathetic hyperactivity results in hypertension, tachycardia, tachypnea, rise in body temperature, excessive diaphoresis, and dystonia. Its prevalence is more common in males, and is generally identified during patient’s liberation from mechanical ventilator and withdrawal of sedation/muscle relaxant. The episode may follow various stimuli, including nonnoxious stimuli such as touch or even sound. Its onset is usually after a week of acute brain insult and may last up to 1 year. There are no specific signs on magnetic resonance imaging (MRI) of brain but it is suspected that patients of diffuse axonal injury on MRI are more prone to get this syndrome. Its definite etiology remains elusive. The most popular theory of excitatory–inhibitory ratio model tends to explain the sympathetic over-reactive nature of response of these patients even to nonallodynic stimuli. Its management is symptomatic and supportive; the mainstay of management relies on morphine and propranolol. Typically, beta-blockers are used to minimize the frequency of the spells, whereas morphine is administered to abort the episodes. Many second-line agents have also been used in refractory cases with variable success rate. Patients who suffer from this entity have higher incidence of various systemic complications. Early recognition is highly essential to avoid unnecessary, costly investigations and for proper management.
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References
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):1175–81.
Jennett B, Teasdale G, Galbraith S, Pickard J, Grant H, Braakman R, et al. Severe head injuries in three countries. J Neurol Neurosurg Psychiatry. 1977;40(3):291–8.
Choi HA, Jeon SB, Samuel S, Allison T, Lee K. Paroxysmal sympathetic hyperactivity after acute brain injury. Curr Neurol Neurosci Rep. 2013;13(8):370.
Perkes I, Baguley IJ, Nott MT, Menon DK. A review of paroxysmal sympathetic hyperactivity after acquired brain injury. Ann Neurol. 2010;68(2):126–35.
Baguley IJ, Perkes IE, Fernandez-Ortega JF, Rabinstein AA, Dolce G, Hendricks HT. Paroxysmal sympathetic hyperactivity after acquired brain injury: Consensus on conceptual definition, nomenclature, and diagnostic criteria. J Neurotrauma. 2014;31:1515–20.
Hinson HE, Sheth KN. Manifestations of the hyperadrenergic state after acute brain injury. Curr Opin Crit Care. 2012;18(2):139–45.
Lv LQ, Hou LJ, Yu M, Qi XO, Chen JX, Hu GH, et al. Risk factors related to dysautonomia after severe traumatic brain injury. J Trauma. 2011;71(3):538–42.
Baguley IJ, Nicholls JL, Felmingham KL, Crooks J, Gurka JA, Wade LD. Dysautonomia after traumatic brain injury: a forgotten syndrome? J Neurol Neurosurg Psychiatry. 1999;67(1):39–43.
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(5):539–41.
Penfield W. Diencephalic autonomic epilepsy. Arch Neurol Psychiatry. 1929;22:358–74.
Rabinstein AA. Paroxysmal sympathetic hyperactivity in the neurological intensive care unit. Neurol Res. 2007;29(7):680–2.
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(7):1364–70.
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):925–32.
Rabinstein AA, Benarroch EE. Treatment of paroxysmal sympathetic hyperactivity. Curr Treat Options Neurol. 2008;10(2):151–7.
Dolce G, Quintieri M, Leto E, Milano M, Pileggi A, Lagani V, et al. Dysautonomia and clinical outcome in vegetative State. J Neurotrauma. 2008;50:502–4.
Fearnside MR, Cook RJ, McDougall P, McNeil RJ. The Westmead head injury project outcome in severe head injury. A comparative analysis of pre-hospital, clinical and CT variables. Br J Neurosurg. 1993;7(3):267–79.
Fernandez-Ortega JF, Prieto-Palomino MA, Munoz-Lopez A. Lebron- Gallardo M, Cabrera-Ortiz H, Quesada-Garcia G. Prognostic influence and computed tomography findings in dysautonomic crises after traumatic brain injury. J Trauma. 2006;61(5):1129–33.
Hendricks HT, Geurts AC, van Ginnken 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(6):545–53.
Moeller RF, Carpenter JL, Dean N, Wells EM. Paroxysmal Sympathetic hyperactivity in critically ill Children with encephalitis and meningoencephalitis. Neurocrit Care. 2015;23(3):380–5.
Dalmau J, Tuzun E, Wu HY, Masjuan J, Rossi JE, Voloschin A, et al. Paraneoplastic anti-N-methyl- D-aspartate receptor encephalitis associated with ovarian teratoma. Ann Neurol. 2007;61(1):25–36.
Nazif TM, Vázquez J, Honig LS, Dizon JM. Anti-N-methyl-D-aspartate receptor encephalitis: an emerging cause of centrally mediated sinus node dysfunction. Europace. 2012;14(8):1188–94.
Gao B, Jeffrey A, Holly P, Hinson E. Paroxysmal sympathetic hyperactivity in hemispheric intraparenchymal haemorrhage. Annals of Clinical and Translational Neurology. 2014;1(3):215–9.
Liu Y, Jolly S, Pokala K. Prolonged Paroxysmal Sympathetic Storming Associated withSpontaneous Subarachnoid Hemorrhage. Case Reports in Medicine. 2013;1-4
Ramdhani NA, Sikma MA, Witkamp TD, Slooter AJC, de Lange DW. Paroxysmal autonomic instability with dystonia in a patient with tuberculous meningitis: a case report. J Medical Case Reports. 2010;4:304.
Baguley IJ, Heriseanu RE, Felmingham KL, Cameron ID. Dysautonomia and heart rate variability following severe traumatic brain injury. Brain Inj. 2006;20(4):437–44.
Boeve BF, Wijdicks EF, Benarroch EE, Schmidt KD. Parxysmal sympathetic storms (diencephalic seizure) after severe diffuse axonal head injury. Mayo Clin Proc. 1998;73(2):148–52.
Bullard DE. Diencephalic seizures: responsiveness to bromocriptine and morphine. Ann Neurol. 1987;21(6):609–11.
Dalmau J, Gleichman AJ, Hughes EG, Rossi JE, Peng X, Lai M, et al. Anti-NMDA-receptor encephalitis: case series and analysis of the effects of antibodies. Lancet Neurol. 2008;7(12):1091–8.
Pranzatelli MR, Pavlakis SG, Gould RJ, De Vivo DC. Hypothalamic-midbrain dysregulation syndrome: hypertension, hyperthermia, hyperventilation, and decerebration. J Child Neurol. 1991;6(2):115–22.
Thorley RR, Wertsch JJ, Klingbeil GE. Acute hypothalamic instability in traumatic brain injury: a case report. Arch Phys Med Rehabil. 2001;82(2):246–9.
Carmel PW. Vegetative dysfunctions of the hypothalamus. Acta Neurochir (Wien). 1985;75(1-4):113–21.
Doshi R, Neil-Dwyer G. Hypothalamic and myocardial lesions after subarachnoid haemorrhage. J Neurol Neurosurg Psychiatry. 1977;40(8):821–6.
Reynolds RW. Pulmonary Edema as a consequence of hypothalamic lesions in rats. Science. 1963;141(3584):930–2.
Baguley IJ. The excitatory:inhibitory ratio model (EIR model): An integrative explanation of acute autonomic overactivity syndromes. Med Hypotheses. 2008;70(1):26–35.
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(8):615–22.
Baguley IJ, Nott MT, Slewa-Younan S, Heriseanu RE, Perkes IE. Diagnosing dysautonomia after acute traumatic brain injury: evidence for overresponsiveness to afferent stimuli. Arch Phys Med Rehabil. 2009;90(4):580–6.
Baguley IJ, Heriseanu RE, Cameron ID, Nott MT. Slewa-Younan S.A critical review of the pathophysiology of dysautonomia following traumatic brain injury. Neurocrit Care. 2008;8(2):293–300.
Lv LQ, Hou LJ, Yu MK, Qi XQ, Chen HR, Chen JX, et al. 'Prognostic influence and magnetic resonance imaging findings in paroxysmal sympathetic hyperactivity after severe traumatic brain injury. J Neurotrauma. 2010;27(11):1945–50.
Cuny E, Richer E, Castel JP. Dysautonomia syndrome in the acute recovery phase after traiumatic brain injury: relief with intrathecal baclofen therapy. Brain Inj. 2001;15(10):917–25.
Krach LE, Kriel RL, Morris WF, Warhol BL, Luxenberg MG. Central autonomic dysfunction following acquired brain injury in children. Neuroreahabil Neural Repair. 1997;11:41–5.
Becker R, Benes L, Sure U, Hellwig D, Bertalaeffy H. Intrathecal baclofen alleviates autonomic dysfunction in severe brain injury. J ClinNeurosci. 2000;7(4):316–9.
Ko S-B, Kim CK, Lee S-H, et al. Morphine-sensitive paroxysmal sympathetic storm in pontine intracerebral hemorrhage. Neurologist. 2010;16(6):384–5.
Rossitch Jr E, Bullard DE. The autonomic dysfunction syndrome: aetiology and treatment. Br J Neurosurg. 1988;2(4):471–8.
Siu G, Marino M, Desai A, Nissley F. Sympathetic storming in a patient with intracranial basal ganglia hemorrhage. Am J Phys Med Rehabil. 2011;90(3):243–6.
Tong C, Konig MW, Roberts PR, Tatter SB, Li XH. Autonomic dysfunction secondary to intracerebral hemorrhage. Anesth Analg. 2000;91(6):1450–1.
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(5):409–17.
Hendricks HT, Heeren AH, Vos PE. Dysautonomia after severe traumatic brain injury. Eur J Neurol. 2010;17(9):1172–7.
Zhang J, Mitsis EM, Chu K, Newmark RE, Hazlett EA, Buchsbaum MS. Statistical parametric mapping and cluster counting analysis of [18F] FDG-PET imaging in traumatic brain injury. J Neurotrauma. 2010;27(1):35–49.
Blackman JA, Patrick PD, Buck ML, Rust Jr RS. Paroxysmal autonomic instability with dystonia after brain injury. Arch Neurol. 2004;61(3):321–8.
Goddeau Jr RP, Silverman SB, Sims JR. Dexmedetomidine for the treatment of paroxysmal autonomic instability with dystonia. Neurocrit Care. 2007;7(3):217–20.
Rabinstein AA. Paroxysmal autonomic instability after brain injury. Arch Neurol. 2004;61(10):1625.
Kelly AB, Zimmerman RD, Snow RB, Gandy SE, Heier LA, Deck MD. Head trauma: comparison of MR and CT--experience in 100 patients. AJNR. 1988;9(4):699–708.
Diesing TS, Wijdicks EF. Arc de cercle and dysautonomia from anoxic injury. Mov Disord. 2006;21(6):868–9.
Huang P, Lin WC, Huang PK, Khor GT. Susceptibility weighted imaging in a patient with paroxysmal sympathetic storms. J Neurol. 2009;256(2):276–8.
Wang VY, Manley G. Recognition of paroxysmal autonomic instability with dystonia (PAID) in a patient with traumatic brain injury. J Trauma. 2008;64(2):500–2.
Clifton GL, Ziegler MG, Grossman RG. Circulating catecholamines and sympathetic activity after head injury. Neurosurgery. 1981;8(1):10–4.
Hughes JD, Rabinstein AA. Early diagnosis of paroxysmal sympathetic hyperactivity in the ICU. Neurocrit Care. 2014;20:454–9.
Srinivasan S, Lim CC, Thirugnanam U. Paroxysmal autonomic instability with dystonia. ClinAuton Res. 2007;17(6):378–81.
Oddo M, Carrera E, Claassen J, Mayer SA, Hirsch LJ. Continuous electroencephalography in the medical intensive care unit. Crit Care Med. 2009;37(6):2051–6.
Meyer KS. Understanding paroxysmal sympathetic hyperactivity after traumatic brain injury. SurgNeurol Int. 2014;5(Suppl 13):S490–2.
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(3):281–4.
Ott L, Young B, Phillips R, McClain C, Adams L, Dempsey R, Tibbs P, Ryo UY. Altered gastric emptying in the head-injured patient: relationship to feeding intolerance. J Neurosurg. 1991;74(5):738–42.
Scott JS, Ockey RR, Holmes GE, Varghese G. Autonomic dysfunction associated with locked-in syndrome in a child. Am J Phys Med Rehabil. 1997;76(3):200–3.
Ryan JB, Hicks M, Cropper JR, Garlick SR, Kesteven SH, Wilson MK, et al. Functional evidence of reversible ischemic injury immediately after the sympathetic storm associated withexperimental brain death. J Heart Lung Transplant. 2003;22(8):922–8.
Woiciechowsky C, Asadullah K, Nestler D, Eberhardt B, Platzer C, Schöning B, et al. Sympathetic activation triggers systemic interleukin-10 release in immunodepression induced by brain injury. Nat Med. 1998;4(7):808–13.
Baguley IJ. Autonomic complications following central nervous system injury’. Semin Neurol. 2008;28(5):716–25.
Do D, Sheen VL, Bromfield E. Treatment of paroxysmal sympathetic storm with labetalol. J Neurol Neurosurg Psychiatry. 2000;69(6):832–3.
Chioléro RL, Breitenstein E, Thorin D, Christin L, de Tribolet N, Freeman J, et al. Effects of propranolol on resting metabolic rate after severe head injury. Crit Care Med. 1989;17(4):328–34.
Meythaler JM, Stinson 3rd AM. Fever of central origin in traumatic brain injury controlled with propranolol. Arch Phys Med Rehabil. 1994;75(7):816–8.
Head GA, Chan CK, Burke SL. Relationship between imidazoline and alpha2-adrenoceptors involved in the sympatho-inhibitory actions of centrally acting antihypertensive agents. J Auton Nerv Syst. 1998;72(2-3):163–9.
Payen D, Quintin L, Plaisance P, Chiron B, Lhoste F. Head injury: clonidine decreases plasma catecholamines. Crit Care Med. 1990;18(4):392–5.
Kang SH, Kim MJ, Shin IY, Park DW, Sohn JW, Yoon YK. Bromocriptine for control of hyperthermia in a patient with mixed autonomic hyperactivity after neurosurgery: a case report. J Korean Med Sci. 2012;27(8):965–8.
Russo RN, O'Flaherty S. Bromocriptine for the management of autonomic dysfunction after severe traumatic brain injury. J Paediatr Child Health. 2000;36(3):283–5.
Cunningham JA, Jelic S. Baclofen withdrawal: a cause of prolonged fever in the intensive care unit. Anaesth Intensive Care. 2005;33(4):534–7.
Lazar RM, Fitzsimmons BF, Marshall RS, Mohr JP, Berman MF. Midazolam challenge reinduces neurological deficits after transient ischemic attack. Stroke. 2003;34(3):794–6.
Chan CH. Dantrolene sodium and hepatic injury. Neurology. 1990;40(9):1427–32.
Lv LQ, Hou LJ, Yu MK, Ding XH, Qi XQ, Lu YC. Hyperbaric oxygen therapy in the management of paroxysmal sympathetic hyperactivity after severe traumatic brain injury: a report of 6 cases. Arch Phys Med Rehabil. 2011;92(9):1515–8.
Greer DM, Funk SE, Reaven NL, Ouzounelli M, Uman GC. Impact of fever on outcome in patients with stroke and neurologic injury: a comprehensive meta-analysis. Stroke. 2008;39(11):3029–35.
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Bithal, P.K., Goyal, K. (2017). Paroxysmal Sympathetic Hyperactivity Following Acute Acquired Brain Injury. In: Khan, Z. (eds) Challenging Topics in Neuroanesthesia and Neurocritical Care. Springer, Cham. https://doi.org/10.1007/978-3-319-41445-4_5
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