ABSTRACT
Severe sympathetic overactivity occurs in several conditions that are recognized as
medical emergencies. Following central nervous system injury, a small proportion of
individuals develop severe paroxysmal sympathetic and motor overactivity. These individuals
have a high attendant risk of unnecessary secondary morbidity. Following acquired
brain injury, the syndrome is known by a number of names including dysautonomia and
sympathetic storm. Dysautonomia is currently a diagnosis of exclusion and often goes
unrecognized. The evidence base for management is almost entirely anecdotal in nature;
there has been little structured or prospective research. In contrast, the evidence
base for autonomic dysreflexia following spinal cord injury is much stronger, with
level 1 evidence for many treatment interventions. This review presents a current
understanding of each condition and suggests simple management protocols. With the
marked disparity in the literature for the two conditions, the main focus is on the
literature for dysautonomia. The similarity between these two conditions and the other
autonomic emergency conditions is discussed.
KEYWORDS
Dysautonomia - sympathetic storms - autonomic dysreflexia - brain injury - spinal
cord injury
REFERENCES
- 1
Baguley I J, Nicholls J L, Felmingham K L et al..
Dysautonomia after traumatic brain injury: a forgotten syndrome?.
J Neurol Neurosurg Psychiatry.
1999;
67(1)
39-43
- 2
Penfield W.
Diencephalic autonomic epilepsy.
Arch Neurol Psychiatry.
1929;
22
358-374
- 3
Carmel P W.
Vegetative dysfunctions of the hypothalamus.
Acta Neurochir (Wien).
1985;
75(1–4)
113-121
- 4
Strich S J.
Diffuse degeneration of the cerebral white matter in severe dementia following head
injury.
J Neurol Neurosurg Psychiatry.
1956;
19
163-185
- 5
Bricolo A, Turazzi S, Alexandre A, Rizzuto A.
Decerebrate rigidity in acute head injury.
J Neurosurg.
1977;
47
680-698
- 6
Wortsman J, Burns G, Van Beek A L, Couch J.
Hyperadrenergic state after trauma to the neuroaxis.
JAMA.
1980;
243(14)
1459-1460
- 7
Hortnagl H, Hammerle A F, Hackl J M, Brucke T, Rumpl E, Hortnagl H.
The activity of the sympathetic nervous system following severe head injury.
Intensive Care Med.
1980;
6(3)
169-177
- 8 Cartlidge N EF, Shaw D A. Head Injury. London; WB Saunders 1981
- 9
Davis R A, Davis L.
Decerebrate rigidity in humans.
Neurosurgery.
1982;
10(5)
635-642
- 10
Rosner M J, Newsome H H, Becker D P.
Mechanical brain injury: the sympathoadrenal response.
J Neurosurg.
1984;
61(1)
76-86
- 11
Rossitch E, Bullard D E.
The autonomic dysfunction syndrome: aetiology and treatment.
Br J Neurosurg.
1988;
2
471-478
- 12
Pranzatelli M R, Pavlakis S G, Gould R G, De Vivo D C.
Hypothalamic-midbrain dysregulation syndrome: hypertension, hyperthermia, hyperventilation,
and decerebration.
J Child Neurol.
1991;
6
115-122
- 13
Fearnside M R, Cook R J, McDougall P, McNeil R J.
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)
267-279
- 14
Sneed R C.
Hyperpyrexia associated with sustained muscle contractions: an alternative diagnosis
to central fever.
Arch Phys Med Rehabil.
1995;
76(1)
101-103
- 15 Ropper A H.
Acute autonomic emergencies and autonomic storm. In: Low PA Clinical Disorders of Autonomic Function. Boston; Little Brown 1992:
747-760
- 16
Blackman J A, Patrick P D, Buck M L, Rust Jr R S.
Correction: paroxysmal autonomic instability with dystonia after brain injury.
Arch Neurol.
2004;
61(6)
980
- 17
Rabinstein A A.
Paroxysmal autonomic instability after brain injury.
Arch Neurol.
2004;
61(10)
1625
- 18
Oh S J, Hong Y K, Song E.
Paroxysmal autonomic dysregulation with fever that was controlled by propanolol in
a brain neoplasm patient.
Korean J Intern Med.
2007;
22(1)
51-54
- 19
Boeve B F, Wijdicks E F, Benarroch E E, Schmidt K D.
Paroxysmal sympathetic storms (“diencephalic seizures”) after severe diffuse axonal
head injury.
, [see comments]
Mayo Clin Proc.
1998;
73(2)
148-152
- 20
Klug N, Hoffman O, Zierski J, Buss K, Laun A, Agnoli A L.
Decerebrate rigidity and vegetative signs in the acute midbrain syndrome with special
regard to motor activity and intracranial pressure.
Acta Neurochir (Wien).
1984;
72(3-4)
219-233
- 21
Meythaler J M, Stinson A M.
Fever of central origin in traumatic brain injury controlled with propanolol.
Arch Phys Med Rehabil.
1994;
75(7)
816-818
- 22
Sandel M E, Abrams P L, Horn L J.
Hypertension after brain injury: case report.
Arch Phys Med Rehabil.
1986;
67
469-472
- 23
Russo R N, O'Flaherty S.
Bromocriptine for the management of autonomic dysfunction after severe traumatic brain
injury.
J Paediatr Child Health.
2000;
36(3)
283-285
- 24
Baguley I J, Cameron I D, Green A M, Slewa-Younan S, Marosszeky J E, Gurka J A.
Pharmacological management of dysautonomia following traumatic brain injury.
Brain Inj.
2004;
18(5)
409-417
- 25
Rabinstein A A.
Paroxysmal sympathetic hyperactivity in the neurological intensive care unit.
Neurol Res.
2007;
29(7)
680-682
- 26 Penfield W, Jasper H. Somatic motor seizures. In: Epilepsy and the Functional Anatomy
of the Human Brain. London; McClelland and Stewart Ltd 1954: 350-388
- 27 Dunne S L. Clonidine for the treatment of paroxysmal autonomic instability with
dystonia following traumatic brain injury. Paper presented at: Neonatal Paediatric
Pharmacists Group Conference Bournemouth, UK; November 2007
- 28
Anderson V L, Ahmed G, Duraski S A, Senno R G.
Alternative treatment in the management of combined hyperadrenergia and spasticity
in the adult with a severe traumatic brain injury: a case report.
Arch Phys Med Rehabil.
2004;
85(9)
e15
- 29
Turner M S.
Early use of intrathecal baclofen in brain injury in pediatric patients.
Acta Neurochir Suppl.
2003;
87
81-83
- 30
Srinivasan S, Lim C CT, Thirugnanam U.
Paroxysmal autonomic instability with dystonia.
Clin Auton Res.
2007;
17(6)
378-381
- 31
Francois B, Vacher P, Roustan J et al..
Intrathecal baclofen after traumatic brain injury: early treatment using a new technique
to prevent spasticity.
J Trauma.
2001;
50(1)
158-161
- 32
Lemke D M.
Sympathetic storming after severe traumatic brain injury.
Crit Care Nurse.
2007;
27(1)
30-37
- 33
Thorley R R, Wertsch J J, Klingbeil G E.
Acute hypothalamic instability in traumatic brain injury: a case report.
Arch Phys Med Rehabil.
2001;
82(2)
246-249
- 34
Becker R, Benes L, Sure U, Hellwig D, Bertalanffy H.
Intrathecal baclofen alleviates autonomic dysfunction in severe brain injury.
J Clin Neurosci.
2000;
7(4)
316-319
- 35
Mazey R M, Kotchen T A, Ernst C B.
A syndrome resembling pheochromocytoma following a stroke. Report of a case.
JAMA.
1974;
230(4)
575-577
- 36
Tong C, Konig M W, Roberts P R, Tatter S B, Li X.
Autonomic dysfunction secondary to intracerebral haemorrhage.
Anesth Analg.
2000;
91(6)
1450-1451
- 37
Leow M KS, Loh K C, Kwek T K, Ng P Y.
Catecholamine and metanephrine excess in intracerebral haemorrhage: revisiting an
obscure yet common “pseudophaeochromocytoma”.
J Clin Pathol.
2007;
60(5)
583-584
- 38
Senno R G, Anderson V, Ahmed G, Duraski S A.
Intrathecal baclofen administration in the management of hyperadrenergic state in
an adult with a severe anoxic brain injury: a case report.
Arch Phys Med Rehabil.
2004;
85(9)
e15
- 39
Bullard D E.
Diencephalic seizures: Responsiveness to bromocriptine and morphine.
Ann Neurol.
1987;
21(6)
609-611
- 40
Baguley I J.
Nomenclature of “paroxysmal sympathetic storms”.
Mayo Clin Proc.
1999;
74(1)
105
- 41
Diesing T S, Wijdicks E F.
Arc de Cercle and dysautonomia from anoxic injury.
Mov Disord.
2006;
21(6)
868-869
- 42
Talman W T, Florek G, Bullard D E.
A hyperthermic syndrome in two patients with acute hydrocephalus.
Arch Neurol.
1988;
45(9)
1037-1040
- 43
Bhigjee A I, Ames F R, Rutherford G S.
Adult aqueduct stenosis and diencephalic epilepsy. A case report.
J Neurol Sci.
1985;
71(1)
77-89
- 44
Lu C S, Ryu S J.
Neuroleptic malignant-like syndrome associated with acute hydrocephalus.
Mov Disord.
1991;
6(4)
381-383
- 45
Do D, Sheen V L, Broomfield E.
Treatment of paroxysmal sympathetic storm with labetalol.
J Neurol Neurosurg Psychiatry.
2000;
69(6)
832-833
- 46
McLean A J.
Autonomic epilepsy: reports of a case with observations at necropsy.
Arch Neurol Psychiatry.
1934;
32
189-197
- 47
Evans C H, Westfall V, Atuk N O.
Astrocytoma mimicking the features of pheochromocytoma.
N Engl J Med..
1972;
286(26)
1397-1399
- 48
Solomon G E.
Diencephalic autonomic epilepsy caused by a neoplasm.
J Pediatr.
1973;
83
277-280
- 49
Scott J S, Ockey R R, Holmes G E, Varghese G.
Autonomic dysfunction associated with locked-in syndrome in a child.
Am J Phys Med Rehabil.
1997;
76(3)
200-203
- 50
Rey M, Borrallo J M, Vogel C M, Pereira M A, Varela M A, Diz J C.
Paroxysmal sympathetic storms after type a dissection of the aorta.
J Cardiothorac Vasc Anesth.
2005;
19(5)
654-655
- 51
Baguley I J.
The excitatory:inhibitory model (EIR model): an integrative explanation of acute autonomic
overactivity syndromes.
Med Hypotheses.
2008;
70
26-35
- 52
Kipps C M, Fung V S, Grattan-Smith P, de Moore G M, Morris J G.
Movement disorder emergencies.
Mov Disord.
2005;
20(3)
322-334
- 53
Macrokanis C J, Hall N L, Mein J K.
Irukandji syndrome in northern Western Australia: an emerging health problem.
Med J Aust.
2004;
181(11–12)
699-702
- 54
Reed M J, Clegg G R.
Paroxysmal sympathetic surge associated with gamma hydroxybutyrate.
Eur J Emerg Med.
2006;
13(1)
41-42
- 55
Howie M B, Black H A, Zvara D, McSweeney T D, Martin D J, Coffman J A.
Esmolol reduces autonomic hypersensitivity and length of seizures induced by electroconvulsive
therapy.
Anesth Analg.
1990;
71
384-388
- 56
Fernandez-Ortega J F, Prieto-Palomino M A, 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)
1129-1133
- 57
Hendricks H T, Guerts A CH, van Ginnekin B C, Heeren A J.
Brain injury severity and autonomic dysregulation accurately predict heterotopic ossification
in patients with traumatic brain injury.
Clin Rehabil.
2007;
21(6)
545-553
- 58
Baguley I J, Slewa-Younan S, Heriseanu R E, Nott M T, Mudaliar Y, Nayyar V.
The incidence of dysautonomia and its relationship with autonomic arousal following
traumatic brain injury.
Brain Inj.
2007;
21(11)
1175-1182
- 59 Jennett B, Teasdale G. Management of Head Injuries. Philadelphia; FA Davis Company
1981: 84-326
- 60
Jennett B, Snoek J, Bond M R, Brooks D N.
Disability after head injury: observations on the use of the Glasgow Outcome Scale.
J Neurol Neurosurg Psychiatry.
1981;
44
285-292
- 61 Uniform Data System for Medical Rehabilitation. Guide for the Uniform Data Set
for Medical Rehabilitation (including the FIMTM instrument). Version 5.1 Buffalo, NY; Uniform Data System for Medical Rehabilitation
- 62
Baguley I J, Heriseanu R E, Felmingham K L, Cameron I D.
Dysautonomia and heart rate variability following severe traumatic brain injury.
Brain Inj.
2006;
20(4)
437-444
- 63
Figa-Talamanca L, Gualandi C, Di Meo L, Di Battista G, Neri G, Lo R F.
Hyperthermia after discontinuance of levodopa and bromocriptine therapy: impaired
dopamine receptors a possible cause.
Neurology.
1985;
35(2)
258-261
- 64
Lemke D M.
Riding out the storm: sympathetic storming after traumatic brain injury.
J Neurosci Nurs.
2004;
36(1)
4-9
- 65 Strum S. Post head injury autonomic complications. http://Available at: www.emedicine.com Accessed April 8, 2004
- 66
Baguley I J, Heriseanu R E, Cameron I D, Nott M T, Slewa-Younan S.
A critical review of the pathophysiology of dysautonomia following traumatic brain
injury.
Neurocrit Care.
2008;
8(2)
293-300
- 67
Hackl J M, Gottardis M, Wieser C et al..
Endocrine abnormalities in severe traumatic brain injury–a cue to prognosis in severe
craniocerebral trauma?.
Intensive Care Med.
1991;
17(1)
25-29
- 68
Cuny E, Richer E, Castel J P.
Dysautonomia syndrome in the acute recovery phase after traumatic brain injury: relief
with intrathecal baclofen therapy.
Brain Inj.
2001;
15(10)
917-925
- 69
Blackman J A, Patrick P D, Buck M L, Rust Jr R S.
Paroxysmal autonomic instability with dystonia after brain injury.
Arch Neurol.
2004;
61(3)
321-328
- 70
Baguley I J, Heriseanu R E, Gurka J A, Nordenbo A, Cameron I D.
Gabapentin in the management of dysautonomia following severe traumatic brain injury:
a case series.
J Neurol Neurosurg Psychiatry.
2007;
78(5)
539-541
- 70a
Baguley I J, Nott M T, Slewa-Younan S, Heriseanu R E, Perkes I E.
Diagnosing dysautonomia following acute traumatic brain injury: evidence for over-responsiveness
to afferent stimuli. APMR.
, In press
- 71
Baena R C, Busto R, Dietrich W D, Globus M Y, Ginsberg M D.
Hyperthermia delayed by 24 hours aggravates neuronal damage in rat hippocampus following
global ischemia.
Neurology.
1997;
48(3)
768-773
- 72
Ryan J B, Hicks M, Cropper J R et al..
Functional evidence of reversible ischaemic injury immediately after the sympathetic
storm associated with experimental brain death.
J Heart Lung Transplant.
2003;
22(8)
922-928
- 73
McLeod A A, Neil-Dwyer G, Meyer C H, Richardson P L, Cruickshank J, Bartlett J.
Cardiac sequelae of acute head injury.
Br Heart J.
1982;
47(3)
221-226
- 74
Hamill R W, Woolf P D, McDonald J V, Lee L A, Kelly M.
Catecholamines predict outcome in traumatic brain injury.
Ann Neurol.
1987;
21(5)
438-443
- 75 Kishner S, Augustin J. Post head injury autonomic complications. http://Available at: www.emedicine.com Accessed March 10, 2008
- 76
Follett K A, Burchiel K, Deer T et al..
Prevention of intrathecal drug delivery catheter-related complications.
Neuromodulation.
2003;
6(1)
32-41
- 77 Baguley I J, Heriseanu R E, Gurka J A, Slewa-Younan S. Gabapentin for the management
of dysautonomia following severe traumatic brain injury: a cautionary tale. Paper
presented at: 4th World Congress of the International Society of Physical and Rehabilitation
Medicine June 10–14, 2007 Seoul, Korea;
- 78
Robertson C S, Clifton G L, Taylor A A, Grossman R G.
Treatment of hypertension associated with head injury.
J Neurosurg.
1983;
59
455-460
- 79
Feibel J H, Baldwin C A, Joynt R J.
Catecholamine-associated refractory hypertension following acute intracranial hemorrhage:
control with propanolol.
Ann Neurol.
1981;
9(4)
340-343
- 80
Salim A, Hadjizacharia P, Brown C et al..
Significance of troponin elevation after severe traumatic brain injury.
J Trauma.
2008;
64(1)
46-52
- 81
Payen D, Quintin L, Plaisance P, Chiron B, Lhoste F.
Head injury: clonidine decreases plasma catecholamines.
Crit Care Med.
1990;
18(4)
392-395
- 82
Metz S A, Halter J B, Porte Jr D, Robertson R P.
Autonomic epilepsy: clonidine blockade of paroxysmal catecholamine release and flushing.
Ann Intern Med.
1978;
88(2)
189-193
- 83
Baguley I J, Heriseanu R E, Slewa-Younan S.
Clonidine in the management of dysautonomia following severe traumatic brain injury.
Neurorehabil Neural Repair.
2006;
20(1)
98-99
- 84
Ditunno J F, Little J W, Tessler A, Burns A S.
Spinal shock revisited: a four-phase model.
Spinal Cord.
2004;
42(7)
383-395
- 85
Guly H R, Bouamra O, Lecky F E.
The incidence of neurogenic shock in patients with isolated spinal cord injury in
the emergency department.
Resuscitation.
2008;
76(1)
57-62
- 86 Krassioukov A, Claydon V E.
The clinical problems in cardiovascular control following spinal cord injury: an overview. In: Weaver LC, Polosa C Progress in Brain Research: Autonomic Dysfunction After
Spinal Cord Injury. Amsterdam; Elsevier 2005: 223-229
- 87 Middleton J. Treatment of Autonomic Dysreflexia for Adults with Spinal Cord Injuries. Sydney,
Australia; Motor Accidents Authority of New South Wales 2002
- 88
Hiersemenzel L P, Curt A, Dietz V.
From spinal shock to spasticity: neuronal adaptations to a spinal cord injury.
Neurology.
2000;
54(8)
1574-1582
- 89
Teasell R W, Arnold J M, Krassioukov A, Delaney G A.
Cardiovascular consequences of loss of supraspinal control of the sympathetic nervous
system after spinal cord injury.
Arch Phys Med Rehabil.
2000;
81(4)
506-516
- 90
Weaver L C, Verghese P, Bruce J C, Fehlings M G, Krenz N R, Marsh D R.
Autonomic dysreflexia and primary afferent sprouting after clip-compression injury
of the rat spinal cord.
J Neurotrauma.
2001;
18(10)
1107-1119
- 91
Rabchevsky A G.
Segmental organization of spinal reflexes mediating autonomic dysreflexia after spinal
cord injury.
Prog Brain Res.
2006;
152
265-274
- 92
Silver J R.
Early autonomic dysreflexia.
Spinal Cord.
2000;
38(4)
229-233
- 93
Krassioukov A V, Furlan J C, Fehlings M G.
Autonomic dysreflexia in acute spinal cord injury: an under-recognized clinical entity.
J Neurotrauma.
2003;
20(8)
707-716
- 94 Krassioukov A, Warburton D ER, Teasell R W, Eng J J.
Autonomic dysreflexia following spinal cord injury. In: Eng JJ, Teasell RW, Miller WC, et al Spinal Cord Injury Rehabilitation Evidence. http://Available at: www.lulu.com (online publisher)
- 95 Campagnolo D I. Autonomic dysreflexia in spinal cord injury. http://Available at: www.emedicine.com Accessed March 10, 2008
- 96
Karlsson A K.
Autonomic dysreflexia.
Spinal Cord.
1999;
37(6)
383-391
- 97
Karlsson A K, Friberg P, Lonnroth P, Sullivan L, Elam M.
Regional sympathetic function in high spinal cord injury during mental stress and
autonomic dysreflexia.
Brain.
1998;
121(9)
1711-1719
- 98
Weaver L C, Marsh D R, Gris D, Brown A, Dekaban G A.
Autonomic dysreflexia after spinal cord injury: central mechanisms and strategies
for prevention.
Prog Brain Res.
2006;
152
245-263
- 99
Singh S, Fried G W, Sawhney J K.
Boosting in spinal cord injured wheelchair athletes: a case report.
Arch Phys Med Rehabil.
2006;
87(11)
e48-e49
- 100
Elliott S, Krassioukov A.
Malignant autonomic dysreflexia in spinal cord injured men.
Spinal Cord.
2006;
44(6)
386-392
- 101
Silver J K, Lux W E.
Early onset dystonia following traumatic brain injury.
Arch Phys Med Rehabil.
1994;
75(8)
885-888
- 102
Chiolero R L, Breitenstein E, Thorin D et al..
Effects of propanolol on resting metabolic rate after severe head injury.
Crit Care Med.
1989;
17(4)
328-334
Ian J BaguleyM.B.B.S. Ph.D.
Research Team Leader, Department of Rehabilitation Medicine, Westmead Hospital
PO Box 533, Wentworthville, NSW 2145, Australia
eMail: ianb@biru.wsahs.nsw.gov.au
