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Electrical (non-convulsive) status epilepticus
Paediatric Neurology update 2014
28 August 2014
Ahmad Rithauddin bin Mohamed
Paediatric Neurologist, IPHKL
Non-convulsive status epilepticus (NCSE)
a state of ongoing (or non-recovery between) seizures without convulsions, usually for more
than 30 min
Intensive care setting
‘comatose’, ‘encephalopathic’ post convulsive status
Out-patient setting
‘not quite right’ neuroregression
Case example 1
• 5 yr old, unwell with fever and diarrhoea
• Had 2-3 brief seizures initially, then convulsive status epilepticus after several hours
• Continued to have intermittent seizures while ventilated (2/52)
• Post extubation – brief eye deviation, remained encephalopathic
after 20 seconds
NCSE in febrile illness related epilepsy syndrome (FIRES)
How common is NCSE in ICU setting?
% of electrographic seizures and status epilepticus in PICU
ove
rall
IPH
KL
North American Paediatric Critical Care EEG Group, 50 consecutive EEG monitoring at each centre
ove
rall
Sanchez et al, 2013
% of electrographic seizures and status epilepticus in PICU
ove
rall
IPH
KL
ove
rall
IPH
KL
Khoo TB, 2014
Prevalence of NCS/NCSE
• Sanchez 2013 (11 centres, N=550)
– 162/550 (29%) had electrographic seizures
– 61/550 (11%) had electrographic status epilepticus
– 114/550 (21%) had non-convulsive (+/- convulsive) seizures
• Khoo 2014 (IPHKL, N=50)
– 11/50 (22%) had electrographic seizures
– 4/50 (8%) had electrographic status epilepticus
– 8/50 (16%) had non-convulsive (+/- convulsive) seizures; 4/50 (8%) had NCSE
Prevalence of NCS/NCSE
• Sanchez 2013 (11 centres, N=550)
– 162/550 (29%) had electrographic seizures
– 61/550 (11%) had electrographic status epilepticus
– 114/550 (21%) had non-convulsive (+/- convulsive) seizures
• Khoo 2014 (IPHKL, N=50)
– 11/50 (22%) had electrographic seizures
– 4/50 (8%) had electrographic status epilepticus
– 8/50 (16%) had non-convulsive (+/- convulsive) seizures; 4/50 (8%) had NCSE
4 FIRES, 1 NMDA encephalitis, 1 viral encephalitis, 2 epilepsy exacerbation
Predictors of NCSE
• Greiner 2012 – witnessed seizure, abnormal brain imaging
• McCoy 2011 - epilepsy, witnessed seizure, acute structural brain injury, interictal discharges on EEG
• IPHKL – witnessed seizure
Predictors of NCSE
• Greiner 2012 – witnessed seizure, abnormal brain imaging
• McCoy 2011 - epilepsy, witnessed seizure, acute structural brain injury, interictal discharges on EEG
• IPHKL – witnessed seizure
ADC
DWI
Acute stroke
Predictors of NCSE
• Greiner 2012 – witnessed seizure, abnormal brain imaging
• McCoy 2011 - epilepsy, witnessed seizure, acute structural brain injury, interictal discharges on EEG
• IPHKL – witnessed seizure
HSV encephalitis
Non-convulsive status epilepticus (NCSE)
a state of ongoing (or non-recovery between) seizures without convulsions, usually for more
than 30 min
Intensive care setting
‘comatose’, ‘encephalopathic’ post convulsive status
Out-patient, epilepsy setting
‘not quite right’, neuroregression
Case example 2
• 10 yr old boy
• Fever provoked convulsions at 7 years
• Unprovoked nocturnal seizures from 8 years, EEG showed GSW, started on Epilim
• Old brother with GTCs, on Epilim
• Now presenting with abnormal behaviour, less responsive, drooling
3 Hz spike waves during spells
Absence SE, underlying idiopathic generalised epilepsy
Case example 3
• 10 yr old boy, underlying mild developmental delay
• Infrequent nocturnal seizures from 4 years, EEG showed centrotemporal spikes, treated as BRE
• From 6 years – had more seizures, unsteady gait, cognitive regression
• EEG encephalopathic, unchanged despite treatment
EEG awake
EEG sleep
BRE with atypical evolution to Electrical Status Epilepticus or Continuous Spikes and Waves during
Slow wave sleep (ESES or CSWS)
language delay/regression no / rare seizures
frequent, bisynchronous CTS
language delay / regression oromotor problems / ataxia
many + & - rolandic seizures frequent, bisynchronous CTS
severe global delay / regression motor and behavioural deficits many rolandic & other seizures
continuous CTS
seizures
development normal
ESES CSWS
delayed development
no or rare seizures
“normal” child no seizures
uni or independent CTS normal (2%)
“normal” child rare rolandic seizures
uni or independent CTS
BRE
Atypical BRE
LKS
Other examples of epilepsies with frequent occurences of NCSE
Syndrome Etiology or clinical context
Clinical form EEG
Benign occipital epilepsy
Idiopathic Autonomic status epilepticus
Occipital ictal rhythms
NCSE in Lennox-Gastaut syndrome
Various, often cryptogenic
Atypical absence status epilepticus
2-2.5 Hz GSW
NCSE in other syndromes (eg ring chromosome 20, Angelman, myoclonic–astatic epilepsy)
Various, usually genetic or cryptogenic
Atypical absence and other nonspecific forms
Various
Non-convulsive status epilepticus
(NCSE)
When to suspect
Intensive care setting
Suspect in ‘comatose’ & ‘encephalopathic’ patients,
especially following witnessed seizures & when imaging is
abnormal
Out-patient, epilepsy setting
Suspect in certain epilepsy syndromes when patient is ‘not
quite right’ or shows neuroregression
Do NCS/NCSE in ICU cause any harm?
Midline shift after intracranial haemorrhage
Vespa 2003 Presented by Hirsch, AES meeting 2010
NCS in TBI: effect on ICP
Vespa 2007 Presented by Hirsch, AES meeting 2010
Presence of NCS/NCSE is an independent predictor of worse outcome
De Lorenzo 1998
Variable Mortality OR (95% CI)
p-value Worsened PCPC OR (95% CI)
p-value
Seizure Category
No Seizures
Ref Ref Ref Ref
Electrographic Seizures
1.3 (0.3, 5.1) 0.74 1.2 (0.4, 3.9) 0.77
Electrographic Status
5.1 (1.4, 18) 0.01 17.3 (3.7, 80) <0.001
Presence of NCSE but not NCS is associated with worse outcome
Topjian 2013
Treatment of NCS/ NCSE
• Same AEDs as for convulsive status
• Balance between side effects and benefits
Do NCS/NCSE in outpatient setting cause any harm?
Neurospychiatric evolution of patients with ESES
typ
ical
n
ega
tive
Bra
in le
sio
ns
Mo
tor
man
ife
stat
ion
s
Pera 2013
Preferred drugs for treatment of CSWS
RCH/ IPHKL
Valproate
Benzodiazepine
Ethosuximide
Sulthiame
Steroids
IVIG
N=196 N=187
Fernandez 2014
Conclusion
• NCSE is an important cause of encephalopathy in the ICU setting, especially in patients with witnessed seizures and abnormal brain imaging
• NCSE may complicate certain epilepsy syndromes, anticipating such complication will assist early detection and effective treatment
• Successful treatment of NCSE may impact positively on mortality and long term neurodevelopmental outcome
Electrical (non-convulsive) status epilepticus
Thank you
Acknowledgment
Dr Khoo
IPHKL EEG unit
IPHKL Paed Neuro team
Patients and families