ARTICLE IN PRESS

Current Paediatrics (2005) 15, 324–332

www.elsevier.com/locate/cupe

Status epilepticus: Beyond guidelines

Helen Basu, Finbar O’Callaghan

Department of Paediatric Neurology, Bristol Royal Hospital for Children, Upper Maudlin Street,
Bristol BS2 8BJ, UK

KEYWORDS Summary Status epilepticus is the most common neurological emergency in

Convulsive status paediatric practice. If convulsive status epilepticus is prolonged (i.e. greater than
epilepticus; 30 min) then there is a loss of cerebral autoregulation that results in cerebral
Refractory status oedema and ischaemic injury to the brain. There may be permanent neurological
epilepticus; sequelae. The aims of treatment are to terminate the seizure as rapidly as possible
Non-convulsive and to identify and treat any underlying causes. Treatment guidelines for established
status epilepticus convulsive status epilepticus have recently been developed by the Status Epilepticus
Working Party, have been endorsed by the National Institute of Clinical Excellence
(NICE) and are recommended here. The treatment of refractory convulsive status
epilepticus and the less well recognised non-convulsive status epilepticus is
problematic but management options are discussed in this article.
& 2005 Elsevier Ltd. All rights reserved.

Introduction status epilepticus can reduce mortality and

morbidity.
Status epilepticus encompasses a range of epileptic
seizure types with different aetiologies, clinical
presentations and outcomes. Convulsive status Types of status epilepticus
epilepticus is the most common neurological
emergency in paediatric practice and carries a risk Status epilepticus can be considered in two broad
of permanent neurological sequelae. Data have groups: convulsive and non-convulsive. Convulsive
suggested that between 4 and 8 children per 1000 status epilepticus can be physiologically or electro-
may experience an episode of convulsive status graphically generalised (affecting the whole brain)
epilepticus before the age of 15 years.1 The or focal (partial—affecting part of the brain).
outcome may be dependent on aetiology but Generalised status epilepticus includes tonic, to-
prompt and appropriate treatment of convulsive nic–clonic, clonic and myoclonic seizure types.
Generalised convulsive (tonic–clonic) status epi-
lepticus has been defined as a generalised convul-
Corresponding author. Tel.: +44 117 342 0187. sion lasting 30 min or longer, or repeated
E-mail address: [email protected] tonic–clonic convulsions occurring over a 30 min
(F. O’Callaghan). period without recovery of consciousness between

0957-5839/$ - see front matter & 2005 Elsevier Ltd. All rights reserved.
doi:10.1016/j.cupe.2005.04.003
 ARTICLE IN PRESS
Status epilepticus: Beyond guidelines 325

each convulsion.2 As status epilepticus continues normal children. It may present as a first seizure or
the tonic phase may become less obvious and the as part of an established seizure disorder.
seizure evolves into continuous clonic status epi- Remote symptomatic status epilepticus occurs in
lepticus. Consciousness is impaired. Afterwards the children with a known neurological insult such as
child is usually drowsy but gradually regains full cerebral palsy, previous stroke or previous head
consciousness. If this does not occur after a trauma. They may or may not have had previous
reasonable time the possibility of continuing brain seizures.
epileptic activity without ongoing motor signs must There are many causes of acute symptomatic
be considered. This electroclinical dissociation may epilepsy including CNS infection, head trauma,
constitute ‘subtle’ convulsive or non-convulsive cerebrovascular disturbance, e.g. arterial ischae-
status epilepticus manifested by just twitching, mic stroke and intracranial haemorrhage. Other
blinking or no movement at all. An electroence- causes include hypoxia, metabolic and electrolyte
phalogram (EEG) may be needed to help with disturbances (hypoglycaemia and hyperglycaemia)
diagnosis. and drug intoxication, e.g. cocaine, theophylline,
In tonic status epilepticus there is continuous tricyclic antidpressants, amphetamines and insulin.
contraction usually of the extensor muscles and Acute withdrawal of antiepileptic drugs may pre-
it may be associated with hypoventilation and cipitate seizures. Status epilepticus also occurs in
cyanosis. This rare type of status epilepticus some progressive neurological disorders, e.g. brain
may be seen in children with Lennox–Gastaut tumours and neurodegenerative diseases.
syndrome.
Myoclonic status epilepticus is characterised
by persistent myoclonic jerks. It may occur in
children with a previous history of myoclonic Pathophysiology
epilepsy or it may be associated with acute brain
injury and anoxia in which case the prognosis is In generalised convulsive status epilepticus there
much worse. are widespread systemic and metabolic changes.3
In focal convulsive status epilepticus there is Tachycardia, hypertension and increased cardiac
usually rhythmic jerking of one part of the body, output are the results of increased sympathetic
often the upper limb. The convulsive activity does outflow. Hyperthermia and hyperglycaemia also
not spread to the whole of the affected side. There occur. Initially the increased metabolic demands
may be more subtle movements such as eye of the brain are met by increased cerebral blood
blinking or trunk rotation. Secondary generalisation flow. As the lactic acid level rises a metabolic
may occur. acidosis develops. After about 30–60 min decom-
Epilepsia partialis continua is a type of focal pensation occurs and cerebral autoregulation is
motor status, notably seen in Rasmussen’s ence- impaired. Cerebral perfusion falls as hypotension
phalitis. There is regular or irregular jerking of a develops. Serum glucose and bicarbonate decrease
body part, most commonly the face or hand. It may and serum potassium rises. Antiepileptic drugs may
continue for hours, days or weeks. become less effective and cardiac arrhythmias may
Non-convulsive status epilepticus will be consid- ensue. Hypoventilation and respiratory failure may
ered later. result from the status epilepticus and from the
medications given to treat it. Pulmonary oedema
and aspiration may contribute to hypoxaemia and
hypercarbia. Venous stasis and cerebral vein
Aetiology thrombosis can result from hypotension and volume
depletion. In prolonged status epilepticus, rhabdo-
Four aetiological groups can be identified: febrile, myolysis and myoglobinuria with renal failure may
idiopathic, remote symptomatic and acute sympto- occur.
matic. Loss of cerebral autoregulation may result in
Febrile status epilepticus occurs in children aged ischaemic injury and cerebral oedema.
6 months to 5 years. It is precipitated by fever in In addition to the loss of autoregulation neuronal
children with no history of previous afebrile hyperexcitability probably also contributes to
seizures and no evidence of central nervous system neuronal damage, which tends to occur in selec-
(CNS) infection or other cause. There is a genetic tively vulnerable areas such as the hippocampus.
predisposition. The neuronal hyperexcitability may be mediated by
Idiopathic status epilepticus occurs without a failure of the inhibitory neurotransmitter gamma-
CNS or systemic insult in otherwise neurologically aminobutyric acid (GABA). The failure of the
 ARTICLE IN PRESS
326 H. Basu, F. O’Callaghan

inhibitory effects of the GABA system is partly Management of convulsive status

responsible for the initiation and continuation of epilepticus
status epilepticus. Excitatory amino acids such as
glutamate probably also contribute to the increase
Aims
in excitation and prolongation of status epilepticus
in addition to contributing to neuronal injury.
The aim of treatment is to terminate the seizure as
Activation of the N-methyl-D aspartate (NMDA)
rapidly as possible and to identify and treat any
receptor by glutamate results in calcium influx
underlying cause. A child who presents with a
and intracellular calcium increases. Destructive tonic–clonic convulsion lasting more than 5 min
calcium-dependent enzymes are activated result-
should be treated in the same way as a child who is
ing in neuronal damage.4
in established status epilepticus. A treatment
Since the loss of cerebral autoregulation and the
guideline for an acute tonic–clonic convulsion
shift towards brain hyperexcitability seems to
including established convulsive status epilepticus
occur approximately 30 min after seizure onset,
was developed by the Status Epilepticus Working
treatment protocols are directed towards termi-
Party and has recently been endorsed by NICE.2,6
nating the seizure before this time in an attempt to
The guideline is shown in Fig. 1.
prevent irreversible neurological damage. During the initial evaluation of a child with
potential or suspected status epilepticus a history,
brief clinical examination and some basic investi-
gations should be performed in order to confirm the
Differential diagnosis of convulsive diagnosis and if possible identify any underlying
status epilepticus cause. However, any necessary support for the
airway, breathing and circulation should not be
Before any treatment is instituted for status delayed.
epilepticus it is important to be certain of the
diagnosis. Several conditions can mimic convulsive
History
status epilepticus. A brief history and clinical
examination should help to differentiate these.
The history should include enquiry about recent
trauma, infection, ingestion, previous seizures and
1. Decerebrate or decorticate posturing in a child medication.
with increased intracranial pressure and im-
pending herniation may be mistaken for status Examination
epilepticus as may an acute dystonic reaction in
response to drugs such as phenothiazines and The examination should include assessment of
butyrophenones. Consciousness is usually pre- airway, breathing, circulation, temperature, ex-
served in acute dystonic reactions. ternal signs of trauma, skin rashes, neurocutaneous
2. Movement disorders including chorea, hemibal- stigmata and neurological status. Close monitoring
lismus or a paroxysmal dyskinesia may be of oxygen saturation, pulse, blood pressure, re-
difficult to distinguish from status epilepticus spiratory rate and depth and electrocardiogram
especially if they develop in the context of an (ECG) should be established. The type of ictal
acute encephalopathic illness or following head behaviour and its duration should be observed and
trauma. documented.
3. Benign neonatal sleep myoclonus may also
mimic status epilepticus.5 This is a self-limiting
Investigations
movement disorder characterised by neonatal-
onset myoclonic jerks only during sleep with an
The probable aetiology of the status epilepticus
absence of concomitant electrographic changes
may determine the investigations but all children
suggestive of seizures.
should have blood tests for glucose, urea, electro-
4. Non-epileptic seizures or psychogenic seizures lytes, calcium, magnesium and blood gas analysis.
may take the form of prolonged pseudo-status
Blood tests for antiepileptic drug levels may be
epilepticus.
appropriate. If infection is suspected a full blood
count and blood culture should be performed.
In cases where there is doubt about the diagnosis Further tests such as plasma amino acids, urine
of status epilepticus an EEG is necessary for organic acids, blood lactate and ammonia may be
confirmation. considered if a metabolic disorder is suspected.
 ARTICLE IN PRESS
Status epilepticus: Beyond guidelines 327

Airway Breathing Circulation

Give high flow oxygen

Measure blood glucose

Confirm epileptic seizure

IMMEDIATE IV ACCESS NO IV ACCESS

1. LORAZEPAM 0.1 mg/kg IV 1. DIAZEPAM 0.5 mg/kg PR

(Give over 30–60 seconds)

Seizure continuing at 10 min IV ACCESS Seizure continuing at 10 min

2. LORAZEPAM 0.1 mg/kg IV 2. PARALDEHYDE 0.4 ml/kg PR

(Give over 30–60 seconds) (Give with same volume of olive oil)

Seizure continuing at 10 min Seizure continuing at 10 min

after commencing Step 2 after commencing Step 2

CALL FOR SENIOR HELP

3. PHENYTOIN 18 mg/kg IV over 20 min

OR

If already on PHENYTOIN give PHENOBARBITONE 20 mg/kg IV over 10 min

(use intraosseous route if still no IV access)

AND

PARALDEHYDE 0.4 ml/kg PR + same volume of olive oil if not already given

AND

CALL ON-CALL ANAESTHETIST OR INTENSIVE CARE PHYSICIAN

Seizure continues 20 min after commencing Step 3

4. RAPID SEQUENCE INDUCTION OF ANAESTHESIA USING THIOPENTAL 4 mg/kg IV

TRANSFER TO INTENSIVE CARE UNIT

Figure 1 Treatment guideline for an acute tonic–clonic convulsion including established convulsive status epilepticus.
IV, intravenous (modified from Appleton et al.2).

Blood and urine for toxicological screening may be the status epilepticus has been effectively treated
necessary if the cause of the status epilepticus is and the level of consciousness improved or until
unclear. Lumbar puncture should be deferred until cerebral oedema and a mass lesion have been
 ARTICLE IN PRESS
328 H. Basu, F. O’Callaghan

excluded. Indications for urgent brain imaging with first dose of lorazepam can be given. If intravenous
CT scan include status epilepticus associated with access still has not been achieved and the child is
head trauma, focal seizures, focal neurological still convulsing at 10 minutes after an initial dose of
signs or focal EEG abnormalities. Children with a rectal diazepam a dose of rectal paraldehyde
previous history of epilepsy who have been thor- 0.4 ml/kg mixed with an equal volume of olive oil
oughly investigated do not need repeat brain can be given.
imaging with every episode of status epilepticus.
However each child should be carefully evaluated Step 3
as new pathology may occur. EEG may be necessary If the seizure is continuing 10 minutes later, a
if the diagnosis of status epilepticus is uncertain. longer-acting anti-epileptic drug such as phenytoin
or phenobarbital is used. Phenytoin is preferred as
General management it causes less respiratory depression and CNS
depression.2 Side effects include cardiac arrhyth-
Initially 100% oxygen should be administered and mias and hypotension. A dose of 18 mg/kg should be
the airway should be supported as necessary. administered intravenously over 20 min. The infu-
Breathing should be assessed and supported with sion rate should not exceed 1 mg/kg/min and ECG
ventilation via a face mask or endotracheal tube as monitoring should be performed.
appropriate. A nasogastric or orogastric tube may If a child is on oral maintenance therapy with
aid stomach decompression and reduce the risk of phenytoin there may be a risk of phenytoin toxicity
aspiration. Circulation may need support with if intravenous phenytoin is used. Therefore, in this
intravenous fluids but overhydration should be situation, intravenous phenobarbitone 20 mg/kg
avoided. If infection is suspected antibiotics and infused over 10 min is recommended. Barbiturates
acyclovir should be commenced and antipyretics potentiate the inhibitory action of GABA. Respira-
given. tory and CNS depression are common and may be
severe enough to warrant mechanical ventilation.
Drug treatment in status epilepticus If intravenous access has not been achieved the
intraosseous route can be used.
The drug treatment of convulsive status epilepticus
in children is outlined in Fig. 1. Step 4
Benzodiazepines are potent and effective for the If convulsive status epilepticus is continuing 20 min-
initial management of status epilepticus and have a utes after commencement of Step 3 then rapid
rapid onset of action. They bind to the benzodia- sequence induction of anaesthesia should be
zepine binding site on the GABA receptor complex performed using intravenous thiopentone (the
increasing GABAergic transmission. This results in dosage will depend on the cardiovascular status of
inhibition of sustained and repetitive neuronal the child). If neuromuscular blockade is necessary,
discharges. a short-acting agent should be used in order not to
mask ongoing convulsive activity. The child should
Step 1 be transferred to a paediatric intensive care unit
If rapid intravenous access is not achieved, rectal (PICU) for further management.
diazepam should be administered in a dose of
0.5 mg/kg. This has a rapid onset of action but as it
is highly lipid-soluble it quickly redistributes to Refractory status epilepticus
other fatty tissues causing brain and blood con-
centrations to fall rapidly. If intravenous access can If convulsive status epilepticus is continuing despite
be achieved rapidly, the first line agent to be the measures outlined in Steps 1–4 above it is
administered is lorazepam (0.1 mg/kg). Lorazepam considered to be refractory. There are no clear
has a rapid onset of action and a longer duration of guidelines for further drug treatment since rando-
action than diazepam as it is less lipophilic. Both mised clinical trials have not been done in
diazepam and lorazepam can cause respiratory refractory status epilepticus, but a number of
depression and hypotension. different agents have been used. These include
midazolam, propofol, phenobarbitone, pentobarbi-
Step 2 tal, lidocaine, ketamine, intravenous chlormethia-
After 10 minutes if the seizure has not stopped a zole, inhalational agents such as isoflurane and
second dose of intravenous lorazepam 0.1 mg/kg desflurane, high-dose intravenous lorazepam, in-
should be given. If diazepam was given initially, a travenous valproate and topiramate. At this stage
 ARTICLE IN PRESS
Status epilepticus: Beyond guidelines 329

care should be ongoing on a PICU under the joint is prolonged. Side-effects include sedation, re-
supervision of a paediatric neurologist. Supportive spiratory depression and hypotension. Since seda-
therapy should be continued in order to maintain tion can be prolonged clinical assessment may be
and support the airway, breathing and circulation difficult.
and further diagnostic tests may need to be
considered if not already completed. Serum crea- Pentobarbital
tinine kinase and urine myoglobin levels should be
monitored due to the risk of rhabdomyolosis in Pentobarbital is a barbiturate with a shorter half-
refractory convulsive status epilepticus. life than phenobarbital and, therefore, it may be
more suitable than phenobarbitone in refractory
Thiopentone status epilepticus as it allows more rapid clinical
assessment. Pentobarbital may be an effective
Thiopentone is a barbiturate anaesthetic agent treatment in some cases14,15 but may be associated
traditionally used in the treatment of status with significant hypotension requiring vasopressor
epilepticus. It has antiepileptic effects but has a therapy.16 It is not licensed in the UK.
tendency to accumulate and cause hypotension,
which may require vasopressor therapy. Sodium Valproate

Midazolam Intravenous loading with sodium valproate has been

found to be safe and effective in refractory status
Midazolam has been reported to be an effective epilepticus.17 It has fewer cardiovascular side
and safe agent for the treatment of refractory effects than some other agents18 and is relatively
status epilepticus.7–9 It is a potent fast-acting non-sedating.
benzodiazepine with a short elimination half-life.
Its action is short-lived and therefore a loading dose Lignocaine
is usually administered followed by a continuous
infusion, the rate titrated to achieve seizure Intravenous lignocaine has been used in refractory
suppression. Tachyphylaxis may occur necessitating status epilepticus.19 Sodium channel blockade and
large dose increases after 24–48 h in order to decreased membrane excitability are thought to be
maintain seizure control. Midazolam accumulates responsible for its antepileptic properties. At high
with prolonged infusion and this may result in a levels, lignocaine may cause CNS toxicity including
long time to awakening. Side effects include confusion, drowsiness, tremulousness, tinnitus and
hypotension and respiratory suppression. psychosis.

Propofol Inhalational agents

Propofol is an intravenous anaesthetic agent with Isoflurane and desflurane, inhalational anaesthetic
antiepileptic properties. It is highly lipophilic and agents, have been reported to be effective in
has a rapid onset of action. Recovery is rapid when stopping epileptic discharges in adult patients with
it is discontinued. It has been found to be effective refractory status epilepticus and prolonged treat-
in the treatment of refractory status epilepti- ment was well tolerated.20
cus.10,11 However, propofol has been associated
with a higher mortality rate than treatment with High dose intravenous lorazepam
midazolam.12 Long-term propofol infusion may
cause lipaemia, acidosis and rhabdomyolosis and High dose intravenous lorazepam given as either a
the risks may be higher in children than in adults.13 continuous infusion or frequent intermittent bo-
It is currently not routinely used in paediatric luses has been reported to be effective in stopping
practice. clinical or electrographic seizures in patients with
refractory status epilepticus.21
Phenobarbitone
Topiramate
Phenobarbital may be effective in refractory status
epilepticus. It has a rapid onset of action but is Topiramate administered via a nasogastric tube
eliminated slowly and there is a risk of accumula- was reported to be effective in terminating
tion necessitating blood level monitoring if therapy refractory status epilepticus within 24 h of
 ARTICLE IN PRESS
330 H. Basu, F. O’Callaghan

initiating maintenance therapy in three children.22 epilepticus with sensory symptomatology, focal
These children had failed to respond to treatment status epilepticus with affective symptomatology,
with benzodiazepines, phenytoin, phenobarbitone, complex partial status epilepticus, continuous
midazolam or pentobarbital. spike and wave during slow-wave sleep and status
epilepticus in coma.
Pyridoxine Typical absence status epilepticus is rare in
children. It is characterised by a prolonged period
A trial of intravenous pyridoxine in a dose of 100 mg of diminished responsiveness or confusion asso-
should be given to all children presenting up to the ciated with generalised spike and slow wave
age of 2 years with refractory status epilepticus discharges on the EEG.25 There may be associated
that has not responded to other antiepileptic drugs. movements including eyelid flutter and rhythmical
Vitamin B6-dependent seizures are due to an facial or upper extremity movements.
autosomal recessive disorder and typically present Atypical absence epilepsy occurs more fre-
in the neonatal period. However they may begin as quently in childhood and is associated with Len-
late as 2 years of age and usually respond quickly to nox–Gastaut syndrome, myoclonic astatic epilepsy
intravenous pyridoxine. and severe myoclonic epilepsy of infancy. Children
may present with a change in personality, apparent
loss of contact with their environment, decreased
EEG monitoring
cognition, drooling, feeding difficulties, impaired
speech and unsteady gait.
The goal of treatment of refractory status epilepti-
Simple focal non-convulsive status epilepticus
cus is the termination of seizures, but by this stage
may present with sensory or affective symptoms
convulsive activity may be very subtle. Ideally all
including auditory sensations and fear.
children with refractory status epilepticus should
Complex partial status epilepticus has been
have continuous EEG monitoring in a PICU setting.
defined as a ‘prolonged epileptic episode in which
Continuous EEG monitoring has several advantages
focal fluctuating or frequently recurring electro-
including reasonable spatial resolution (i.e. activity
graphic epileptic discharges, arising in temporal or
in different brain regions can be assessed simulta-
extratemporal regions, result in a confusional state
neously) and good temporal resolution (2–4 ms).23
with variable clinical symptoms.’26 These include
At the very least, daily intermittent EEG recordings
fluctuating level of consciousness, impaired inter-
should be performed but there is a danger of over-
action with familiar people, staring, automatisms
or under-treatment.
and speech arrest.
There is no general consensus on the goal
Electrical status epilepticus in slow-wave sleep is
regarding EEG activity. The traditional aim has
characterised by spike and wave discharges in at
been the attainment of a burst-suppression pattern
least 85% of non-rapid eye movement (REM) sleep.
but seizure control is achieved in some patients
It is associated with Landau–Kleffner syndrome and
with a background of continuous slow wave
Lennox–Gastaut syndrome. The child may present
activity.24
with language regression, behavioural changes and
neuropsychological problems.
Long-term antiepileptic drug therapy Non-convulsive status epilepticus can occur in
coma either following convulsive status epilepticus
The need for long-term antiepileptic drug therapy or associated with other pathologies in which the
after convulsive status epilepticus has been con- significance of the EEG abnormalities may be
trolled depends on the underlying aetiology and an difficult to interpret as they may represent wide-
assessment of the risk of recurrence. For example, spread cortical damage rather than true epileptic
patients with known idiopathic generalised epi- activity.27
lepsy or a structural brain lesion are likely to need The diagnosis of non-convulsive status epilepti-
long-term therapy unlike those in whom status cus is based on the clinical presentation together
epilepticus occurred in the context of a transient with a supportive EEG. The clinical features may be
metabolic disturbance. subtle and difficult to interpret especially in
children with existing learning disability.
The differential diagnosis of non-convulsive
Non-convulsive status epilepticus status epilepticus includes a postictal state,
metabolic encephalopathy, psychogenic or conver-
Types of non-convulsive status epilepticus include sion disorder, alcohol or drug intoxication, psycho-
typical absence, atypical absence, focal status sis, hyperglycaemia and diabetic ketoacidosis,
 ARTICLE IN PRESS
Status epilepticus: Beyond guidelines 331

psychogenic mutism, depression, stroke, emotional ciated with acute medical illness has a poor
lability and psychosis. prognosis with a high mortality rate.32

Management of non-convulsive status Conclusions

epilepticus
Status epilepticus is a common paediatric neurolo-
Typical absence status epilepticus usually responds gical emergency. There is an established protocol
to benzodiazepines, which may be given orally or for the treatment of convulsive status epilepticus in
intravenously, e.g. lorazepam, clonazepam, diaze- the UK but the management of refractory status
pam. Intravenous valproate has also been used epilepticus and non-convulsive status epilepticus is
successfully.28 more difficult. The outcome of status epilepticus
Atypical absence status epilepticus may be more depends to a large extent on the aetiology, but
difficult to treat and intravenous benzodiazepines early and aggressive treatment of convulsive status
may not only be less effective but may precipitate epilepticus reduces morbidity and mortality.
tonic status epilepticus. Oral treatment may be
preferred initially and valproate, lamotrigine,
topiramate, clonazepam and clobazam have all
been used effectively. Oral ketamine has also been References
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