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The Nursing Challenges of Caring for Patients with NORSE Syndrome Melissa V Moreda RN BSN CNRN. disclosures. Merz . KS is a 28 yo Korean American female Admitted to OSH flu-like symptoms for 4-5 days. nausea/vomiting and 105.0 Fever.

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The Nursing Challenges of Caring for Patients with NORSE Syndrome Melissa V Moreda RN BSN CNRN

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The Nursing Challenges of Caring for Patients with NORSE SyndromeMelissa V Moreda RN BSN CNRN


  • Merz

KS is a 28 yo Korean American female

Admitted to OSH

flu-like symptoms for 4-5 days.

nausea/vomiting and 105.0 Fever.

She became disoriented and confused & had a witnessed tonic clonic seizure.

Recurrent seizures continued.

Case #1 K.S.

Case #1 K.S.

Transferred to Duke in Status Epilepticus

Workup unremarkable

NO past hx of childhood epilepsy, febrile seizure, no head trauma, no meningitis

NO family hx of sz, no predisposing evidence

Case #1 K.S.

Social hx: ANY exposure--- what do they work with? any unusual circumstances or any family/friends with recent sickness?

Habits: drinking/drugs/etc

Allergies: none

Medications (any herbals/supplements)

Extensive negative workup

Case #1 K.S.

Initial lines of anticonvulsants started.

Quick escalation of medication dosages and additional anticonvulsants added.

Seizures continued.

Paralytics added, max therapies reached.

Case #1 K.S. - Hospital Course

Day 1--Dilantin, Topamax, Pentobarbgtt, Versed

Day 10– Dilantin, Topamax, Pentobarbgtt, Keppra, Zonisamide

Day 20– Lidocaine/Magnesium gtt

Day 30— Dilantin, Topamax, Clonazepam, Keppra,Phenobarb,Valproate, Ketamine

Case #1 K.S.

Day 40--Ativan gtt, MgSo4, Keppra, Topamax, Valproate, Phenobarb

Day 50 -- Ativan,Keppra,Topamax, Phenobarb, Memantine, Ketamine gtt

Day 62 -- Status continuing

Uroseptic - death within 1 hour of foley exchange

Electrical activity of the brain


Abnormal electrical signal

What is the difference between

Seizures and Status Epilepticus

What is seizure?

A single (finite) event of abnormal discharge in the brain that results in an abrupt and temporary altered state of cerebral function.

What is Status Epilepticus?

  • Continuous seizures lasting at least 5 minutes.

  • 2 or morediscrete seizures between which there is incomplete recovery of consciousness.


SE=Medical Emergency

Morbidity & Mortality

SE=Medical Emergency

Status Epilepticus

  • Repeated partial seizures manifesting as focal motor convulsions, focal sensory symptoms, or focal impairment of function (ie: aphasia not associated with LOC)

  • Tonic-clonic most common type.

  • Convulsive more easily seen clinically. Partial less obvious and more difficult to identify. Subclinical only identifiable on cEEG.

  • Wittman & Hirsch--Neurocritical Care 2005: increasing awareness of nonconvulsive seizures in critically ill

What is refractory SE?


of max

doses of







Safety & Nursing Care: Convulsive vs Anticonvulsive

External Safety:

padded rails

suction present

bed in low position

pulse ox/ vitals

staying with the patient

obtaining benzos & AEDS

Family needs education and reassurance

Safety & Nursing Care: Convulsive vs Anticonvulsive

A seizure represents actual danger to brain tissue

Time is BRAIN

Internal Safety:

Returning to Case Study #1K.S.

What’s going on here???

KS has the flu, spikes a fever, starts seizing and doesn’t stop despite multiple line anticonvulsants. Full life support.

Completely negative workup.

Family, Nurses, MD’s, PRM’s, want to know….







What is it?????

*2005 Wilder-Smith ,Lim, Teoh, Sharma, Tan, Chen, et al documented and claimed this phenomenon in Singapore.











  • Of the 7 identified:

    • Shortest stay: 7days resulting in death

      -11 days: 1 patient survived, but in persistent vegetative state with frequent seizures.

      • The other one died.

      • Longest stay: 92 days survived but in persistent vegetative state with frequent seizures as well.

NORSE-EEG Relationships

Initial eeg captured ictal discharges in all.

In 3, eeg showed ictal discharges from bilateral fronto-temporal regions with no side preference.

In 1, continuous parasagital ictal discharge.

In 3, fronto-temporal discharges originating from the right on 2 occasions and once on the left

Why use these meds???

  • Status Epilepticus Clinical Guidelines 2003

    • Benzodiazepines

    • Phenytoin loads + maintenance doses

    • Barbiturates

Wilder-Smith et al

  • Treatments included:

    • BenzodiazepinesLevetiracetam

    • PhenytoinIVIG

    • Valproate

    • Propofol

    • Thiopental

    • Topiramate





  • Lorazepam (Ativan ):

    • increases action of GABA which inhibits neurotransmission, depressing all levels of CNS

      Problem: hypotension, caution in renal or hepatic impairment, MG



  • Diazepam (Valium):

  • Short acting , ½ life 16-90 minutes, the later ½ eliminated slower.

  • High does and accumulation of active diazepam metabolites = respiratory depression and hypotension

  • 5-20mg IV slowly at 1-2 mg/min Repeat 5-10 mg every 5-10 minutes to a maximum of 100mg/24 hours


  • Midazolam(Versed):

    • Large number of trials reflecting that this medication works

    • Rapid absorption into brain

    • Benign from a hemodynamic standpoint

    • Peds: success of breaking seizures without intubation

    • Load:0.2mg/kg, maintenance: up to 2 mcg/kg/min, most effective as a gtt

    • Problem: tachyphylaxis (wears off), accumulates in critically ill.

Phenytoin /Fosphenytoin

  • may work in motor cortex, may stop spread of activity

  • Brain stem centers stop tonic phase of grand mal sz

  • 18-20mg/kg, if refractory – may use 30mg/kg

  • Problem: hypotension, purple glove syndrome, fever, cardiac death from widened QRS

Refractory Status Epilepticus

  • 30 minutes to 1 hour of seizure activity not broken

  • No interval of time between failed treatment and next therapy

  • Start infusions of Benzodiazepines, Propofol, Barbitruates

  • Keep giving bolus doses


  • Pentobarbital:

    • Extremely effective halting seizures on eeg by inducing coma

    • Load: 5-12 mg/kg, maintenance: 1-10 mg/kg

      Problem: high doses suppress cardiac function,difficult to monitor levels, poor chemotaxis of wbc, paralysis of resp cilia, poikilothermia



  • Phenobarbital:

    • Introduced 1912

    • CNS depressant

    • elevates seizure threshold by decreasing postsynaptic excitation, possibly stimulating postsynaptic GABA inhibitor responses.

    • Sometimes given in combo with dilantin

    • Rapidly absorbed by all routes, 3-4 weeks to reach steady state

Medicinal Management

Traditional goal of therapy: achieve burst suppression on EEG 12-24 hours to control or prevent recurrent seizures.

Termination of the seizures should occur rapidly to minimize neural damage, correction of metabolic defects, and resuscitation

SE=Medical Emergency

When your clinical guidelines fail

in stopping seizures…

What do you do???

Medicinal Management of Refractory Status Epilepticus

1st Maximize Phenytoin & Benzodiazepine loads. This usually controls 70% of patients.

2ndMidazolam boluses infusion, Propofol, Barbiturates

3rdTopiramate, phenobarbital, midazolam, pentobarbital, valproate, levetiracetam, lidocaine, ketamine, thiopental, or isoflurane

Medicinal Management

  • Other treatments we have tried

    • Antivirals upon arrival

    • Magnesium drips

    • Memantine

    • Ketamine drips

    • Prn ativan, phenobarb, pentobarb, versed

    • Giving “holidays” to reintroduce the medications, hopefully to “break through” the seizures.

Wilder-Smith et al

Thiopental: anesthetic that is barbiturate based.

IVIG: ?autoimmune, does not cross the BBB

Propofol: difficult to suppress sz while titrating/ balancing hemodynamics

  • Rosetti et al 2004 burst suppression 31 patients/ 21 successfully suppressed

  • 1 interesting side effect: EPILEPTIFORM ACTIVITY!!!!

Alternative Therapies

Robakis, Hirch 2006 lit review + expert neurointensivists/ epileptologists

Tx included: ketamine, gammaglobulin, plasmapheresis, steroids, adrenocorticotropic hormone, high dose phenobarb, isoflurane, lidocaine, ect, ketogenic diet, hypothermia, mag


  • CNS depression with cessation of convulsions

  • Biphasic:

    • Blocks inhibitory CNS pathways resulting in stimulation

    • Blocks inhibitory/excitatory impulses resulting in CNS inhibition (Peralta 2007)

  • Walker, Slovis 1997 effective in peds not responding to barbs

  • Bolus + maintenance dose

  • Toxicity Rare


  • Good Stops Seizures through anesthesia: NMDA antagonist with intrinsic sympathomimetic properties

    • 1-4.5 mg/kg

    • Borris et al 2000

  • Bad Neurotoxicity

    • Diffuse cerebellar atrophy

    • 44yo male, tx for status 3 months later- consistent with animal models N-methyl-D-aspartate antagonist –mediated neurotoxicity

    • Ubogu et al2003

    • SE: Pyschotic

Using Anesthesia for Treatment of Refractory Status Epilepticus

  • Rosetti in Epilepsia 2007

    • Each anesthetic has advantages/risks

    • Depends on the protocols regarding duration and depth of sedation

    • The biological background of the patient remains the prognostic determinant of SE

Alternative Therapies

Hypothermia (31-35C)

Corry, Dhar, Murphy, Diringer 2006

Alternative Therapies

  • ECT: -1 second seizure is induced while anesthetized, wakes 10-15 minutes later

    • 3x week for up to 15 treatments, return to baseline, 1-2 more treatments

Alternative Therapies

  • Ketogenic Diet:

    • High fats, low carbs

    • Strict diet requiring medical supervision

    • Body burns fat instead of glucose

    • Kids usually on for 2 years

    • Should we consider this in tube feeds?

Nursing Considerations

  • Airway/Breathing:vented, full support, possiblity of VAP, need for aggressive pulmonary toileting

  • Circulation: hemodynamically unstable due to AEDs/coma inducing meds requiring vasoactive agents, increased risk of DVTS

  • Disability: (Neuro exam): pupils sometimes work. Can’t see more damage due to coma

Nursing Considerations

  • Expose: what does their skin/lines look like? Usually generally edematous, pressure sores common. Med rashes.

  • Fahrenheit: Poikilothermic, immune

    system suppressed, may need routine pan cultures

  • Family Finances…Increased anxiety

  • Gadgets: Scds, afo boots, hand splints, various machines for life support, eeg electrodes

Nursing Considerations

  • Head to Toe: Assessment of physical appearance. Oh, how I wish I could wash their hair.

  • IV’s: site appearance, correct iv doses, do you have enough access? Do you need to make more pentobarb or levophed? Are you waiting for more depakote or an abx?

  • JP/Drains:hopefully, not needed.

Nursing Considerations

  • Keep Family Informed: This is the tricky part. I usually want to cry at this point.

  • Labs: Are you therapeutic? Have any of the weirdo labs come back from Mayo or the state?

  • Legal: Have we discussed DNR?

Nursing Considerations

  • Meds:Besides the AEDs, anticoagulants, GI motility agents, ABX, acid reduction agents, blood products, SSI… Do you have any more room on your Medication Administration Record?

  • Movement: PROM

  • Nutrition: Are they actually getting any with their gut shut down? High residuals? Have they developed an ileus? Are they on a bowel regimen with daily stimulation?




  • In the last 10 years at Duke, we can identify at least 8 patients

    • All young (<35)

    • Previous good health

    • Initially Flu-like symptoms or pyschotic behavior, then status

    • Negative workup

    • 4 AA female, 1 Asian female, 1 Lebanese male, 1 caucasian male and female


By exclusion only


Workup Includes:

*Radiographic Imagery (ct,mri)

*Continuous eeg

*Serum samples: Heavy Metals, Complex Virus, RMSF, Arbovirus, Rabies, Leptospiral Abs, Autoimmune

*Brain Biopsy

Workup includes:

  • *CSF(culture,gram stains, cryptococcal antigens, herpes PCR polymerase chain reactions- replicate DNA)

  • *Stool (organisms/parasites)

  • *Infection Disease Consult

does having an actual diagnosis matter?



Difference b/w TBI &NORSE?

Why does having an actual diagnosis matter?

Merle Mishel PhD, FAAN- UNC Chapel Hill

Uncertainty in Illness Theory






Case Study #2 K.C.

  • 19 yo female with URI and fever 101.0

  • Z pack started

  • 2 days later, in car with family, started seizing and turning blue, incontinent of urine.

  • OSH- tonic clonic sz, intubated

  • Negative primary workup

Case Study #2 K.C.

  • 2 days later extubated

  • Another sz, meds given, intubated

  • Sz continues, bradycardic and cyanotic

  • Transfer to Duke

  • 3 weeks after initial sz, off pentobarb, and smiling at dad

Another Survivor!


  • Jirsch J. Hirsch LJ. Nonconvulsive seizures: developing a rational approach to the diagnosis and management in the critically ill population. Clin Neurophysiol.2007118(8): 1660-70.

  • Robakis TK, Hirsch LJ. Literature review, case report, and expert discussion of prolonged refractory status epilepticus. Neurocrit Care. 2006. 4(1):35-46.

  • Khaled KJ, Hirsch LJ. Advances in the Management of Seizures and Status Epilepticus in Critically Ill Patients. Crit Care Clin. 2007. (22):637-659.

  • Wilder-Smith EPV, Lim ECH, Teoh HL, Sharma VK, Tan JJH, Chan BPL, Ong BKC. The NORSE (New Onset Refractory Status Epilepticus) Syndrome: Defining a Disease Entity. Ann Acad Med Singapore. 2005, 34:417-20.

  • Hirsch LJ., Kull L. Continuous EEG Monitoring in the Intensive Care Unit. Am. J. END Technol. 2004 (44):137-158.

  • Hickey JV. The Clinical Practice of Neurological and Neurosurgical Nursing.Philadelphia: Lippincott, 1997.

  • Corry JJ, Dhar R, Murphy T, Diringer MN. Hypothermia for Refractory Status Epilepticus. Neurocrit Care 2008: 9(2):189-97.

  • Rosetti AO. Anesthesia for Treatment of Refractory Status Epilepticus. Epilepsia 2007. 48 (8):52-55

Please contact me with questions or insight…

[email protected]

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