Neurocritical Care

, Volume 23, Supplement 2, pp 23–47 | Cite as

Emergency Neurological Life Support: Acute Non-traumatic Weakness

  • Oliver FlowerEmail author
  • Mark S. Wainwright
  • Anna Finley Caulfield
Review Article


Acute non-traumatic weakness may be life threatening if it involves the respiratory muscles or is associated with autonomic dysfunction. Most patients presenting with acute muscle weakness have a worsening neurological disorder that requires a rapid, systematic evaluation, and detailed neurological exam to localize the disorder. Urgent laboratory tests and neuroimaging are needed in many patients to make the diagnosis. Because acute weakness is a common presenting sign of neurological emergencies, it was chosen as an Emergency Neurological Life Support protocol. Causes of acute non-traumatic weakness are discussed by both presenting clinical signs and anatomical location. For each diagnosis, key features of the history, examination, investigations, and treatment are outlined in the included tables.


Neuromuscular weakness Acute weakness Respiratory failure 


  1. 1.
    Mehta S. Neuromuscular disease causing acute respiratory failure. Respir Care. 2006;51:1016–21 discussion 1021–3.PubMedGoogle Scholar
  2. 2.
    Lawn ND, Fletcher DD, Henderson RD, Wolter TD, Wijdicks EF. Anticipating mechanical ventilation in Guillain–Barre syndrome. Arch Neurol. 2001;58:893–8.CrossRefPubMedGoogle Scholar
  3. 3.
    Abel M, Eisenkraft JB. Anesthetic implications of myasthenia gravis. Mt Sinai J Med. 2002;69:31–7.PubMedGoogle Scholar
  4. 4.
    Orebaugh SL. Succinylcholine: adverse effects and alternatives in emergency medicine. Am J Emerg Med. 1999;17:715–21.CrossRefPubMedGoogle Scholar
  5. 5.
    Zuccoli G, Panigrahy A, Bailey A, Fitz C. Redefining the Guillain–Barre spectrum in children: neuroimaging findings of cranial nerve involvement. Am J Neuroradiol. 2011;32:639–42.CrossRefPubMedGoogle Scholar
  6. 6.
    Pidcock FS, Krishnan C, Crawford TO, Salorio CF, Trovato M, Kerr DA. Acute transverse myelitis in childhood: center-based analysis of 47 cases. Neurology. 2007;68:1474–80.CrossRefPubMedGoogle Scholar
  7. 7.
    Chen L, Li J, Guo Z, Liao S, Jiang L. Prognostic indicators of acute transverse myelitis in 39 children. Pediatric Neurol. 2013;49:397–400.CrossRefGoogle Scholar
  8. 8.
    Wolf VL, Lupo PJ, Lotze TE. Pediatric acute transverse myelitis overview and differential diagnosis. J Child Neurol. 2012;27:1426–36.CrossRefPubMedGoogle Scholar
  9. 9.
    Bernard T, Rivkin M, Scholz K, deVeber G, Kirton A, Gill JC, Chan A, Hovinga C, Ichord R, Grotta J, Jordan L, Benedict S, Friedman N, Dowling M, Elbers J, Torres M, Sultan S, Cummings D, Grabowski E, McMillan H, Beslow L, Amlie-Lefond C. Emergence of the primary pediatric stroke center: impact of the thrombolysis in pediatric stroke trial. Stroke. 2014;45:2018–23.PubMedCentralCrossRefPubMedGoogle Scholar
  10. 10.
    Novy J, Carruzzo A, Maeder P, Bogousslavsky J. Spinal cord ischemia: clinical and imaging patterns, pathogenesis, and outcomes in 27 patients. Arch Neurol. 2006;63:1113–20.CrossRefPubMedGoogle Scholar
  11. 11.
    Brinar VV, Habek M, Brinar M, Malojcic B, Boban M. The differential diagnosis of acute transverse myelitis. Clin Neurol Neurosurg. 2006;108:278–83.CrossRefPubMedGoogle Scholar
  12. 12.
    DeRosa MA, Cryer PE. Hypoglycemia and the sympathoadrenal system: neurogenic symptoms are largely the result of sympathetic neural, rather than adrenomedullary, activation. Am J Physiol Endocrinol Metab. 2004;287:E32–41.CrossRefPubMedGoogle Scholar
  13. 13.
    Eddleston M, Buckley NA, Eyer P, Dawson AH. Management of acute organophosphorus pesticide poisoning. Lancet. 2008;371:597–607.PubMedCentralCrossRefPubMedGoogle Scholar
  14. 14.
    Roberts DM, Aaron CK. Management of acute organophosphorus pesticide poisoning. BMJ. 2007;334:629–34.PubMedCentralCrossRefPubMedGoogle Scholar
  15. 15.
    White J. Venomous animals: clinical toxinology. EXS. 2010;100:233–91.PubMedGoogle Scholar
  16. 16.
    Warrell DA. Snake bite. Lancet. 2010;375:77–88.CrossRefPubMedGoogle Scholar
  17. 17.
    Hagan PG, Nienaber CA, Isselbacher EM, et al. The international registry of acute aortic dissection (IRAD): new insights into an old disease. JAMA. 2000;283:897–903.CrossRefPubMedGoogle Scholar
  18. 18.
    Gaul C, Dietrich W, Friedrich I, Sirch J, Erbguth FJ. Neurological symptoms in type A aortic dissections. Stroke. 2007;38:292–7.CrossRefPubMedGoogle Scholar
  19. 19.
    Tsai TT, Nienaber CA, Eagle KA. Acute aortic syndromes. Circulation. 2005;112:3802–13.CrossRefPubMedGoogle Scholar
  20. 20.
    Aggarwal M, Khan IA. Hypertensive crisis: hypertensive emergencies and urgencies. Cardiol Clin. 2006;24:135–46.CrossRefPubMedGoogle Scholar
  21. 21.
    Vaughan CJ, Delanty N. Hypertensive emergencies. Lancet. 2000;356:411–7.CrossRefPubMedGoogle Scholar
  22. 22.
    Grob D, Brunner N, Namba T, Pagala M. Lifetime course of myasthenia gravis. Muscle Nerve. 2008;37:141–9.CrossRefPubMedGoogle Scholar
  23. 23.
    Meriggioli MN, Sanders DB. Advances in the diagnosis of neuromuscular junction disorders. Am J Phys Med Rehabil. 2005;84:627–38.CrossRefPubMedGoogle Scholar
  24. 24.
    Headache Classification Subcommittee of the International Headache, Society. The International Classification of Headache Disorders: 2nd edition. Cephalalgia. 2004;24(1):9–160.Google Scholar
  25. 25.
    Russell MB, Ducros A. Sporadic and familial hemiplegic migraine: pathophysiological mechanisms, clinical characteristics, diagnosis, and management. Lancet Neurol. 2011;10:457–70.CrossRefPubMedGoogle Scholar
  26. 26.
    Guisado R, Arieff AI. Neurologic manifestations of diabetic comas: correlation with biochemical alterations in the brain. Metabolism. 1975;24:665–79.CrossRefPubMedGoogle Scholar
  27. 27.
    Kitabchi AE, Umpierrez GE, Miles JM, Fisher JN. Hyperglycemic crises in adult patients with diabetes. Diabetes Care. 2009;32:1335–43.PubMedCentralCrossRefPubMedGoogle Scholar
  28. 28.
    Gallmetzer P, Leutmezer F, Serles W, Assem-Hilger E, Spatt J, Baumgartner C. Postictal paresis in focal epilepsies—incidence, duration, and causes: a video-EEG monitoring study. Neurology. 2004;62:2160–4.CrossRefPubMedGoogle Scholar
  29. 29.
    Rolak LA, Rutecki P, Ashizawa T, Harati Y. Clinical features of Todd’s post-epileptic paralysis. J Neurol Neurosurg Psych. 1992;55:63–4.CrossRefGoogle Scholar
  30. 30.
    DeAngelis LM. Brain tumors. N Engl J Med. 2001;344:114–23.CrossRefPubMedGoogle Scholar
  31. 31.
    Muzumdar D, Jhawar S, Goel A. Brain abscess: an overview. Int J Surg. 2011;9:136–44.CrossRefPubMedGoogle Scholar
  32. 32.
    Sayer FT, Vitali AM, Low HL, Paquette S, Honey CR. Brown-Sequard syndrome produced by C3–C4 cervical disc herniation: a case report and review of the literature. Spine. 2008;33:E279–82.CrossRefPubMedGoogle Scholar
  33. 33.
    Antich PA, Sanjuan AC, Girvent FM, Simo JD. High cervical disc herniation and Brown-Sequard syndrome. A case report and review of the literature. J Bone Joint Surg Br. 1999;81:462–3.CrossRefPubMedGoogle Scholar
  34. 34.
    Bruno MA, Pellas F, Schnakers C, et al. Blink and you live: the locked-in syndrome. Rev Neurol (Paris). 2008;164:322–35.CrossRefGoogle Scholar
  35. 35.
    Puetz V, Sylaja PN, Coutts SB, et al. Extent of hypoattenuation on CT angiography source images predicts functional outcome in patients with basilar artery occlusion. Stroke. 2008;39:2485–90.CrossRefPubMedGoogle Scholar
  36. 36.
    Ropper AH. The Guillain–Barre syndrome. N Engl J Med. 1992;326:1130–6.CrossRefPubMedGoogle Scholar
  37. 37.
    Seneviratne U. Guillain–Barre syndrome. Postgrad Med J. 2000;76:774–82.PubMedCentralCrossRefPubMedGoogle Scholar
  38. 38.
    Meena AK, Khadilkar SV, Murthy JM. Treatment guidelines for Guillain–Barre Syndrome. Ann Indian Acad Neurol. 2011;14:S73–81.PubMedCentralCrossRefPubMedGoogle Scholar
  39. 39.
    Alshekhlee A, Hussain Z, Sultan B, Katirji B. Guillain–Barre syndrome: incidence and mortality rates in US hospitals. Neurology. 2008;70:1608–13.CrossRefPubMedGoogle Scholar
  40. 40.
    Ropper AH. Further regional variants of acute immune polyneuropathy. Bifacial weakness or sixth nerve paresis with paresthesias, lumbar polyradiculopathy, and ataxia with pharyngeal-cervical-brachial weakness. Arch Neurol. 1994;51:671–5.CrossRefPubMedGoogle Scholar
  41. 41.
    Hughes RA, Swan AV, van Koningsveld R, van Doorn PA. Corticosteroids for Guillain–Barre syndrome. Cochrane Database Syst Rev. 2006:CD001446.Google Scholar
  42. 42.
    Varma JK, Katsitadze G, Moiscrafishvili M, et al. Signs and symptoms predictive of death in patients with foodborne botulism—Republic of Georgia, 1980–2002. Clin Infect Dis. 2004;39:357–62.CrossRefPubMedGoogle Scholar
  43. 43.
    Long SS. Infant botulism. Pediatr Infect Dis J. 2001;20:707–9.CrossRefPubMedGoogle Scholar
  44. 44.
    Chalk C, Benstead TJ, Keezer M. Medical treatment for botulism. Cochrane Database Syst Rev. 2011:CD008123.Google Scholar
  45. 45.
    Graeme KA, Pollack CV Jr. Heavy metal toxicity, Part I: arsenic and mercury. J Emerg Med. 1998;16:45–56.CrossRefPubMedGoogle Scholar
  46. 46.
    Graeme KA, Pollack CV Jr. Heavy metal toxicity, part II: lead and metal fume fever. J Emerg Med. 1998;16:171–7.CrossRefPubMedGoogle Scholar
  47. 47.
    Marx A, Glass JD, Sutter RW. Differential diagnosis of acute flaccid paralysis and its role in poliomyelitis surveillance. Epidemiol Rev. 2000;22:298–316.CrossRefPubMedGoogle Scholar
  48. 48.
    Venance SL, Cannon SC, Fialho D, et al. The primary periodic paralyses: diagnosis, pathogenesis and treatment. Brain. 2006;129:8–17.CrossRefPubMedGoogle Scholar
  49. 49.
    Iorizzo LJ 3rd, Jorizzo JL. The treatment and prognosis of dermatomyositis: an updated review. J Am Acad Dermatol. 2008;59:99–112.CrossRefPubMedGoogle Scholar
  50. 50.
    Anderson KE, Bloomer JR, Bonkovsky HL, et al. Recommendations for the diagnosis and treatment of the acute porphyrias. Ann Intern Med. 2005;142:439–50.CrossRefPubMedGoogle Scholar
  51. 51.
    Grattan-Smith PJ, Morris JG, Johnston HM, et al. Clinical and neurophysiological features of tick paralysis. Brain. 1997;120(Pt 11):1975–87.CrossRefPubMedGoogle Scholar
  52. 52.
    Edlow JA, McGillicuddy DC. Tick paralysis. Infect Dis Clin North Am. 2008;22:397–413.CrossRefPubMedGoogle Scholar
  53. 53.
    Kuncl RW. Agents and mechanisms of toxic myopathy. Curr Opin Neurol. 2009;22:506–15.CrossRefPubMedGoogle Scholar
  54. 54.
    Wirtz PW, Smallegange TM, Wintzen AR, Verschuuren JJ. Differences in clinical features between the Lambert-Eaton myasthenic syndrome with and without cancer: an analysis of 227 published cases. Clin Neurol Neurosurg. 2002;104:359–63.CrossRefPubMedGoogle Scholar
  55. 55.
    Keogh M, Sedehizadeh S, Maddison P. Treatment for Lambert-Eaton myasthenic syndrome. Cochrane Database Syst Rev. 2011:CD003279.Google Scholar
  56. 56.
    Barohn RJ, Sahenk Z, Warmolts JR, Mendell JR. The Bruns-Garland syndrome (diabetic amyotrophy). Revisited 100 years later. Arch Neurol. 1991;48:1130–5.CrossRefPubMedGoogle Scholar
  57. 57.
    Tracy JA, Dyck PJ. The spectrum of diabetic neuropathies. Phys Med Rehabil Clin N Am. 2008;19:1–26.PubMedCentralCrossRefPubMedGoogle Scholar
  58. 58.
    Davies L, Spies JM, Pollard JD, McLeod JG. Vasculitis confined to peripheral nerves. Brain. 1996;119(Pt 5):1441–8.CrossRefPubMedGoogle Scholar
  59. 59.
    Mathew L, Talbot K, Love S, Puvanarajah S, Donaghy M. Treatment of vasculitic peripheral neuropathy: a retrospective analysis of outcome. QJM. 2007;100:41–51.CrossRefPubMedGoogle Scholar
  60. 60.
    London Z, Albers JW. Toxic neuropathies associated with pharmaceutic and industrial agents. Neurol Clin. 2007;25:257–76.CrossRefPubMedGoogle Scholar
  61. 61.
    Fox IK, Mackinnon SE. Adult peripheral nerve disorders: nerve entrapment, repair, transfer, and brachial plexus disorders. Plast Reconstr Surg. 2011;127:105e–18e.CrossRefPubMedGoogle Scholar
  62. 62.
    Neal S, Fields KB. Peripheral nerve entrapment and injury in the upper extremity. Am Fam Physician. 2010;81:147–55.PubMedGoogle Scholar
  63. 63.
    Racaniello VR. One hundred years of poliovirus pathogenesis. Virology. 2006;344:9–16.CrossRefPubMedGoogle Scholar
  64. 64.
    Reynolds RM, Padfield PL, Seckl JR. Disorders of sodium balance. BMJ. 2006;332:702–5.PubMedCentralCrossRefPubMedGoogle Scholar
  65. 65.
    Adrogue HJ, Madias NE. Hypernatremia. N Engl J Med. 2000;342:1493–9.CrossRefPubMedGoogle Scholar
  66. 66.
    Schrier RW, Bansal S. Diagnosis and management of hyponatremia in acute illness. Curr Opin Crit Care. 2008;14:627–34.PubMedCentralCrossRefPubMedGoogle Scholar
  67. 67.
    Riggs JE. Neurologic manifestations of electrolyte disturbances. Neurol Clin. 2002;20:227–39.CrossRefPubMedGoogle Scholar
  68. 68.
    Sterns RH, Hix JK, Silver S. Treating profound hyponatremia: a strategy for controlled correction. Am J Kidney Dis. 2010;56:774–9.CrossRefPubMedGoogle Scholar
  69. 69.
    Ravid M, Robson M. Proximal myopathy caused by latrogenic phosphate depletion. JAMA. 1976;236:1380–1.CrossRefPubMedGoogle Scholar
  70. 70.
    Sebastian S, Clarence D, Newson C. Severe hypophosphataemia mimicking Guillain–Barre syndrome. Anaesthesia. 2008;63:873–5.CrossRefPubMedGoogle Scholar
  71. 71.
    Rowland JP, Shneider NA. Amyotrophic lateral sclerosis. NEJM. 2001;344:1688–700.CrossRefPubMedGoogle Scholar
  72. 72.
    Kiernan MC, Vucic S, Cheah BC, Turner MR, et al. Amyotrophic lateral sclerosis. Lancet. 2011;377:942–55.CrossRefPubMedGoogle Scholar
  73. 73.
    Miller RG, Jackson CE, Kasarskis EJ, England JD, et al. Practice Parameter update: the care of the patient with amyotrophic lateral sclerosis: multidisciplinary care, symptom management, and cognitive/behavioral impairment (an evidence-based review). Neurology. 2009;73:1227–33.PubMedCentralCrossRefPubMedGoogle Scholar

Copyright information

© Springer Science+Business Media New York 2015

Authors and Affiliations

  • Oliver Flower
    • 1
    Email author
  • Mark S. Wainwright
    • 2
  • Anna Finley Caulfield
    • 3
  1. 1.Department of Intensive CareRoyal North Shore HospitalSydneyAustralia
  2. 2.Departments of Neurology and PediatricsNorthwestern University Feinberg School of MedicineChicagoUSA
  3. 3.Department of NeurologyStanford UniversityStanfordUSA

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