NEUROLOGIC & MUSCULAR DISORDERS

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Table 23–10. Status epilepticus: clinical types. Table 23–11. Status epilepticus treatment.

Generalized seizure (common) 1. ABCs

Convulsive (tonic, clonic, myoclonic): 90% a. Airway: maintain oral airway; intubation may be necessary.
Nonconvulsive (absence, atypical absence, atonic): 10% b. Breathing: oxygen.
Focal (partial) seizures (rare) c. Circulation: assess pulse, blood pressure; support with IV fluids,
Simple partial drugs. Monitor vital signs.
Complex partial 2. Start glucose-containing IV; evaluate serum glucose; electrolytes,
Neonatal HCO3–, CBC, BUN, anticonvulsant levels.
Many clinical varieties 3. May need arterial blood gases, pH.
May show electroclinical dissociation (on EEG with no visible clinical 4. Give 50% glucose if serum glucose low (1–2 mL/kg).
correlate) 5. Begin IV drug therapy; goal is to control status epilepticus in 20–60 min.
Other (very rare) a. Diazepam, 0.3–0.5 mg/kg over 1–5 min (20 mg maximum); may
Continuous epileptiform activity in sleep (Landau-Kleffner syndrome repeat in 5–20 min; or, lorazepam, 0.05–0.2 mg/kg (less
and syndrome of continuous spike-wave in slow wave sleep) effective with repeated doses, longer-acting than diazepam).
a
Midazolam: IV, 0.1–0.2 mg/kg; intranasally, 0.2 mg/kg.
b. Phenytoin, 10–20 mg/kg IV (not IM) over 5–20 min; 1000 mg
maximum); monitor with blood pressure and ECG. Fosphenytoin
because patients sometimes appear merely stuporous and may be given more rapidly in the same dosage; order 10–20
lack typical convulsive movements. mg/kg of “phenytoin equivalent.”
c. Phenobarbital, 5–20 mg/kg (sometimes higher in newborns or
refractory status in intubated patients).
" Clinical Findings 6. Correct metabolic perturbations (eg, low-sodium, acidosis).
A child with status epilepticus may have a high fever with or 7. Other drug approaches in refractory status:
a. Repeat phenytoin, phenobarbital (10 mg/kg). Monitor blood
without intracranial infection. Studies show that 25–75% of
levels. Support respiration, blood pressure as necessary.
children with status epilepticus experience it as their initial b. aMidazolam drip: 1–5 mcg/kg/min (even to 20 kg/min).
seizure. Often it is a reflection of a acute or remote insult. Valproate sodium, available as 100 mg/mL for IV use; give 15–
Tumor and stroke, which are common causes of status 30 mg/kg over 5–20 min.
epilepticus in adults, are uncommon causes in childhood. c. Pentobarb coma. Propofol. General anesthetic.
Fifty percent of pediatric status epilepticus is due to acute 8. Consider underlying causes:
(25%) or chronic (25%) CNS disorders. Infection and met- a. Structural disorders or trauma: MRI or CT scan.
b. Infection: lumbar puncture, blood culture, antibiotics.
abolic disorders are common causes of status epilepticus in
c. Metabolic disorders: consider lactic acidosis, toxins, uremia. May
children. The cause is unknown in 50% of patients, but need to evaluate medication levels. Toxin screen. Judicious fluid
many of these patients will be febrile. In the child with administration.
known epilepsy, medication nonadherence should be con- 9. Give maintenance drug (if diazepam only was sufficient to halt status
sidered. Status epilepticus occurs most commonly in chil- epilepticus): phenytoin (10 mg/kg); phenobarbital (5 mg/kg); daily
dren aged 5 years and younger (85%). The most common dose IV (or by mouth) divided every 12 h.
age is 1 year or younger (37%); the distribution is even for a
Much supportive data.
each year thereafter (approximately 12% per year). BUN, blood urea nitrogen; CBC, complete blood count; CT, computed
tomography; ECG, electrocardiogram; IM, intramuscularly; IV, intravenously;
" Treatment MRI, magnetic resonance imaging.

For treatment options, see Table 23–11.

Rarely status epilepticus may occur during a febrile sei-
3. Febrile Seizures zure. Febrile seizures rarely (1–3%) lead to recurrent unpro-
Criteria for febrile seizures are (1) age 3 months to 5 years voked seizures (epilepsy) in later childhood and adult life
(most occur between ages 6 and 18 months), (2) fever of (risk is increased two- to fivefold compared with children
greater than 38.8°C, and (3) non-CNS infection. More than who do not have febrile seizures). The chance of later
90% of febrile seizures are generalized, last less than 5 epilepsy is higher if the febrile seizures have complex fea-
minutes, and occur early in the illness causing the fever. tures, such as duration longer than 15 minutes, more than
Febrile seizures occur in 2–3% of children. Acute respiratory one seizure in the same day, or focal features. Other adverse
illnesses are most commonly associated with febrile sei- factors are an abnormal neurologic status preceding the
zures. Gastroenteritis, especially when caused by Shigella or seizures (eg, cerebral palsy or mental retardation), early
Campylobacter, and urinary tract infections are less common onset of febrile seizure (before age 1 year), and a family
causes. Roseola infantum is a rare but classic cause. One history of epilepsy. Even with adverse factors, the risk of
study implicated viral causes in 86% of cases. Immunizations epilepsy after febrile seizures is still only in the range of 15–
may be a cause. 20%, although it is increased if more than one risk factor is
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present. Recurrent febrile seizures occur in 30–50% of cases show to be ineffective. Newer antiepileptic drugs have not
but in general do not worsen the long-term outlook. been studied. Diazepam started at the first onset of fever for
Generalized epilepsy with febrile seizures plus (GEFS+) is the duration of the febrile illness (0.5 mg/kg two or three
an autosomal dominant form of epilepsy first described in times per day orally or rectally) may be effective but will
1997. The most frequently observed GEFS+ phenotype sedate a child. Prophylactic diazepam is also limited by the
includes childhood onset of multiple febrile seizures persist- fact that a seizure is often the first evidence of fever associ-
ing beyond the age of 6 years, and unprovoked (afebrile) ated with an acute illness. Diastat (rectal diazepam gel) can
seizures, including absence, myoclonic, or atonic seizures, be used to prevent febrile status epilepticus in the child with
and rarely, myoclonic-astatic epilepsy. Originally associated a prolonged febrile seizure (one lasting over 5 minutes),
with a point mutation in SCN1B, GEFS+ is now known to often the greatest concern.
have other channelopathies. Phenobarbital, 3–5 mg/kg/d as a single bedtime dose, is
an inexpensive long-term prophylaxis. However, significant
" Clinical Findings behavioral disturbance is seen in about one third of toddlers
treated with phenobarbital. Often, increasing the dosage
A. Diagnostic Evaluation gradually (eg, starting with 2 mg/kg/d the first week, increas-
The child with a febrile seizure must be evaluated for the ing to 3 mg/kg/d the second week, and so on) decreases side
source of the fever, in particular to exclude CNS infection. effects and nonadherence. A plasma phenobarbital level in
Routine studies such as serum electrolytes, glucose, calcium, the range of 15–40 mg/mL is desirable.
skull radiographs, or brain imaging studies are seldom help- Valproate sodium is potentially more hazardous. In
ful. A white count above 20,000/µL or an extreme left shift infants, the commonly used liquid suspension has a short
may correlate with bacteremia. Complete blood count and half-life and causes more gastrointestinal upset than do the
blood cultures may be appropriate. Serum sodium is often sprinkle capsules. The dosage is 15–60 mg/kg/d in divided
slightly low but not low enough to require treatment or to doses. Precautionary laboratory studies are necessary. In the
cause the seizure. Meningitis and encephalitis must be con- infant younger than age 2 years, there is an increased risk of
sidered. Signs of meningitis (eg, bulging fontanelle, stiff hepatic toxicity. Thrombocytopenia may occur, particularly
neck, stupor, and irritability) may all be absent, especially in in the face of an acute illness.
a child younger than age 18 months. Measures to control fever such as sponging or tepid
baths, antipyretics, and the administration of antibiotics for
B. Lumbar Puncture proven bacterial illness are reasonable but unproven to
prevent recurrent febrile seizures.
After controlling the fever and stopping an ongoing seizure, Simple febrile seizures do not have any long-term adverse
the physician must decide whether to do a lumbar puncture. consequences. An EEG may be considered if the febrile
The fact that the child has had a previous febrile seizure does seizure is complicated, focal, or otherwise unusual. In
not rule out meningitis as the cause of the current episode. uncomplicated febrile seizures, the EEG is usually normal.
The younger the child, the more important is the procedure, Ideally the EEG should be done at least a week after the
because physical findings are less reliable in diagnosing illness to avoid transient changes due to fever or the seizure
meningitis. Although the yield is low, a lumbar puncture itself. In older children, 3/s spike-wave discharges, suggestive
should probably be done if the child is younger than age 18 of a genetic propensity to epilepsy, may occur. In the young
months, if recovery is slow, if no other cause for the fever is infant, EEG findings seldom aid in assessing the chance of
found, or if close follow-up will not be possible. Occasionally recurrence of febrile seizures or in long-term prognosis.
observation in the emergency department for several hours
obviates the need for a lumbar puncture. A negative finding
does not exclude the possibility of emergence of CNS infec- Aldenkamp AP et al: Optimizing therapy of seizures in children
and adolescents with ADHD. Neurology 2006;67(Suppl 4):S49
tion during the same febrile illness. Sometimes a second [PMID: 17190923].
procedure must be done. Barkovich AJ et al: A developmental and genetic classification for
malformations of cortical development. Neurology 2005;65:1873
" Treatment & Prognosis [PMID: 16192428].
Bartha AI et al: Neonatal seizures: Multicenter variability in
Prophylactic anticonvulsants are not recommended after an current treatment practices. Pediatr Neurol 2007;37:85 [PMID:
uncomplicated febrile seizure. 17675022].
If febrile seizures are complicated or prolonged, or if Berg AT et al: How long does it take for epilepsy to become
intractable? A prospective investigation. Ann Neurol 2006;60:73
medical reassurance fails to relieve family anxiety, anticon- [PMID: 16685695].
vulsant prophylaxis may be indicated and can reduce the Berg AT et al. Longitudinal assessment of adaptive behavior in
incidence of recurrent febrile seizures. Only phenobarbital infants and young children with newly diagnosed epilepsy:
and valproic acid have demonstrated efficacy in preventing Influences of etiology, syndrome, and seizure control. Pediat-
febrile seizures; phenytoin and carbamazepine have been rics 2004;114:645 [PMID: 15342834].