Status Epilepticus, Guillain-Barré and Myasthenia Gravis

Also known as Status epilepticus · Seizures · Guillain-Barre syndrome · Myasthenia gravis · Neuromuscular respiratory failure · Non-convulsive status

Status epilepticus: ILAE t1 5 min / t2 30 min for convulsive SE. RAMPART IM midazolam 73.4 vs 63.4%. ESETT each ~half at 60 min. Kantanen SRSE 1-year mortality 36%. Guillain-Barre and myasthenia gravis are the two neuromuscular diseases that most commonly cause the respiratory failure needing the ICU. This topic builds the examiner's framework on the SE management (the benzodiazepine, the fosphenytoin/levetiracetam, the RAMPART and the ESETT evidence), the GBS (the IVIG, the plasma exchange, the respiratory monitoring), and the myasthenia (the IVIG, the plasma exchange, the crisis).

medium24 referencesUpdated 18 Aug 202623 min readSource-verified · Sept 2026

Checked against its sources on 3 Sept 2026

Practise this topic

16 MCQs

The one-paragraph exam answer

Three neurological emergencies share one ICU door. Status epilepticus is a benzodiazepine first — IM midazolam by autoinjector when there is no line (RAMPART), IV lorazepam 4 to 8 mg when there is (EFNS; 0.1 mg/kg in the VA Cooperative Study) — then a full-dose second-line antiepileptic once the benzodiazepine has failed (levetiracetam 60 mg/kg, fosphenytoin 20 mg PE/kg or valproate 40 mg/kg — equivalent in ESETT), then anaesthetic coma with barbiturate, midazolam or propofol titrated to burst suppression on continuous EEG for at least 24 hours in the refractory case. Guillain-Barré is ascending flaccid paralysis with areflexia — watch the vital capacity and intubate before the crash, then IVIG 0.4 g/kg daily for five days or five plasma exchanges of 200 to 250 mL/kg (Dutch trial: 53 vs 34% one-grade improvement at four weeks; corticosteroids do not work). Myasthenic crisis is fatigable weakness — IVIG 0.4 g/kg daily for 3 or 5 days or three plasma exchanges (Gajdos), withdraw the precipitant, and start long-term immunosuppression.^[1]^[2]^[3]^[8]^[14]^[15]^[16]^[17]

Meet the patient

Bed 4 is mid-seizure at eight minutes — generalised, tonic-clonic, jaw clenched, no IV access yet, oxygen on a non-rebreather. Across the bay, bed 7 is a quieter emergency: four days of ascending weakness, now too weak to cough, the bedside vital capacity drifting down overnight. One brain is firing when it should not; one set of nerves is failing when they should fire.^[1]^[23]

The question that decides both patients has the same shape — is this the brain, or is it the nerve? The seizing brain wants a benzodiazepine now and a clock-driven ladder; the failing nerve wants the vital capacity watched before the blood gas, and immunomodulation, never a steroid. Hold that fork and the rest of this topic slots into place.^[16]

References24Show

[1]Silbergleit R, Durkalski V, Lowenstein D, et al. Intramuscular versus intravenous therapy for prehospital status epilepticus N Engl J Med, 2012.PMID 22335736
[2]Kapur J, Elm J, Chamberlain JM, et al. Randomized Trial of Three Anticonvulsant Medications for Status Epilepticus N Engl J Med, 2019.PMID 31774955
[3]Treiman DM, Meyers PD, Walton NY, et al. A comparison of four treatments for generalized convulsive status epilepticus. Veterans Affairs Status Epilepticus Cooperative Study Group N Engl J Med, 1998.PMID 9738086
[4]Trinka E, Cock H, Hesdorffer D, et al. A definition and classification of status epilepticus--Report of the ILAE Task Force on Classification of Status Epilepticus Epilepsia, 2015.PMID 26336950
[5]Brophy GM, Bell R, Claassen J, et al. Guidelines for the evaluation and management of status epilepticus Neurocrit Care, 2012.PMID 22528274
[6]Claassen J, Mayer SA, Kowalski RG, Emerson RG, Hirsch LJ. Detection of electrographic seizures with continuous EEG monitoring in critically ill patients Neurology, 2004.PMID 15159471
[7]Benbadis SR, Tatum WO 4th Prevalence of nonconvulsive status epilepticus in comatose patients Neurology, 2000.PMID 11087805
[8]Vossler DG, Bainbridge JL, Boggs JG, et al. Treatment of Refractory Convulsive Status Epilepticus: A Comprehensive Review by the American Epilepsy Society Treatments Committee Epilepsy Curr, 2020.PMID 32822230
[9]Hirsch LJ, Gaspard N, van Baalen A, et al. Proposed consensus definitions for new-onset refractory status epilepticus (NORSE), febrile infection-related epilepsy syndrome (FIRES), and related conditions Epilepsia, 2018.PMID 29399791
[10]Thakur KT, Probasco JC, Hocker SE, et al. Ketogenic diet for adults in super-refractory status epilepticus Neurology, 2014.PMID 24453083
[11]Rosati A, L'Erario M, Ilvento L, et al. Efficacy and safety of ketamine in refractory status epilepticus in children Neurology, 2012.PMID 23197747
[12]Kantanen AM, Reinikainen M, Parviainen I, et al. Incidence and mortality of super-refractory status epilepticus in adults Epilepsy Behav, 2015.PMID 26141934
[13]Ferlisi M, Hocker S, Grade M, et al. Preliminary results of the global audit of treatment of refractory status epilepticus Epilepsy Behav, 2015.PMID 25952268
[14]Meierkord H, Boon P, Engelsen B, et al. EFNS guideline on the management of status epilepticus in adults Eur J Neurol, 2010.PMID 20050893
[15]van der Meché FG, Schmitz PI; Dutch Guillain-Barré Study Group A randomized trial comparing intravenous immune globulin and plasma exchange in Guillain-Barré syndrome N Engl J Med, 1992.PMID 1552913
[16]Hughes RA, Brassington R, Gunn AA, van Doorn PA Corticosteroids for Guillain-Barré syndrome Cochrane Database Syst Rev, 2016.PMID 27775812
[17]Gajdos P, Chevret S, Clair B, et al. Clinical trial of plasma exchange and high-dose intravenous immunoglobulin in myasthenia gravis Ann Neurol, 1997.PMID 9189040
[18]Rossetti AO, Reichhart MD, Schaller MD, et al. Propofol treatment of refractory status epilepticus: a study of 31 episodes Epilepsia, 2004.PMID 15230698
[19]Cremer OL, Moons KG, Bouman EA, et al. Long-term propofol infusion and cardiac failure in adult head-injured patients Lancet, 2001.PMID 11197401
[20]Martyn JA, Richtsfeld M Succinylcholine-induced hyperkalemia in acquired pathologic states: etiologic factors and molecular mechanisms Anesthesiology, 2006.PMID 16394702
[21]Sparr HJ, Vermeyen KM, Beaufort AM, et al. Early reversal of profound rocuronium-induced neuromuscular blockade by sugammadex in a randomized multicenter study: efficacy, safety, and pharmacokinetics Anesthesiology, 2007.PMID 17457124
[22]Durand MC, Porcher R, Orlikowski D, et al. Clinical and electrophysiological predictors of respiratory failure in Guillain-Barré syndrome: a prospective study Lancet Neurol, 2006.PMID 17110282
[23]Mehta S Neuromuscular disease causing acute respiratory failure Respir Care, 2006.PMID 16934165
[24]Misra UK, Kalita J A comparison of four antiepileptic drugs in status epilepticus: experience from India Int J Neurosci, 2016.PMID 26456955