Now onset refractory status epilepticus (NORSE)

Evidence-based neurology checklist on now onset refractory status epilepticus (norse): Clinical features This is the occurrence of persistent seizures with no identifiable cause There is no pre-existing epilepsy The seizures last longer than 24 hours despite optimal treatment They usually start as…

Clinical features

  • This is the occurrence of persistent seizures with no identifiable cause
  • There is no pre-existing epilepsy
  • The seizures last longer than 24 hours despite optimal treatment
  • They usually start as repeated focal seizures with secondary lateralization
  • Most cases evolve to super-refractory status epilepticus (NOSRSE)
  • There is a high risk of long-term disability and mortality

Risk factors

Features of cryptogenic NORSE

Investigations

Paraneoplastic screening tests

Magnetic resonance imaging (MRI) brain: claustrum sign

Treatment: first line immunotherapy

Treatment: second line immunotherapy

Treatment: others

Acronym

References

  1. Khawaja AM, DeWolfe JL, Miller DW, Szaflarski JP. New-onset refractory status epilepticus (NORSE)-the potential role for immunotherapy. Epilepsy Behav 2015; 47:17-23. 
  2. 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; 59:739-744.
  3. Matthews E, Alkhachroum A, Massad N, et al. New-onset super-refractory status epilepticus: a case series of 26 patients. Neurology 2020; 95:e2280-e2285. 
  4. Gaspard N, Foreman BP, Alvarez V, et al; Critical Care EEG Monitoring Research Consortium (CCEMRC). New-onset refractory status epilepticus: etiology, clinical features, and outcome. Neurology 2015; 85:1604-1613.
  5. Meierkord H, Boon P, Engelsen B, et al. EFNS guideline on the management of status epilepticus in adults. Eur J Neurol 2010; 17:348-355.
  6. And 10 more. Subscribe to see the full list

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