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Seizures and Epilepsy

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38 ■ Section 2: Common Pediatric Neurologic Problems<br />

Syndromes<br />

Benign familial neonatal seizures<br />

Benign familial neonatal seizures occur as a genetic<br />

disorder with an autosomal dominant inheritance pattern<br />

associated with chromosome 20q. The seizures<br />

typically start on day of life 2 or 3. The neonate may<br />

have as many as 10 to 20 seizures per day. The syndrome<br />

is usually self-limited <strong>and</strong> benign, but approximately<br />

10% of cases progress to an antiepileptic<br />

drug-requiring seizure disorder. Neurological development<br />

is normal. 58–61<br />

Fifth-day fits<br />

Fifth-day fits usually begin on day of life 4 to 6. The<br />

seizures are typically multifocal clonic seizures <strong>and</strong> are<br />

frequently associated with apnea. The seizures usually<br />

last for less than 24 hours. Fifth-day fits progress to status<br />

epilepticus in 80% of cases. 58–61<br />

Benign neonatal sleep myoclonus<br />

Benign neonatal sleep myoclonus begins during the first<br />

week of life. The seizures are usually bilateral myoclonic<br />

jerks that last for several minutes <strong>and</strong> occur only during<br />

NREM sleep. The EEG is normal or slow. The seizures<br />

worsen with the administration of benzodiazepines. The<br />

seizures usually resolve within 2 months <strong>and</strong> neurological<br />

outcome is normal. 58–61<br />

Benign myoclonus of early infancy<br />

Benign myoclonus of early infancy has an onset at age 3<br />

to 9 months but it can be much earlier. The seizures<br />

resemble infantile spasms but the EEG is normal. The<br />

seizures usually occur while the patient is awake. The<br />

seizures disorder may continue for 1 to 2 years but neurological<br />

outcome is normal. 58–61<br />

Treatment<br />

The clinician should first search for underlying etiologies<br />

producing the seizures <strong>and</strong> treat (hypoglycemia,<br />

hypocalcemia, sepsis). If the clinician cannot find a<br />

readily identifiable <strong>and</strong> treatable etiology, the frontline<br />

agent of choice for treating seizures is phenobarbital<br />

(see Table 3-14 for dosing suggestions). 62 Phenobarbital<br />

as a single agent will stop seizure activity in 42% of<br />

patients. When the seizure does not respond to a single<br />

agent, phenytoin is added with an increase in efficacy to<br />

65%. Currently, fosphenytoin, the salt ester of phenytoin,<br />

is preferred in the neonate because it is an aqueous<br />

solution that is soluble in glucose-containing solutions,<br />

can be administered more quickly than phenytoin, <strong>and</strong><br />

will not cause “purple glove syndrome.” 62 Purple glove<br />

syndrome is necrosis or injury of the soft tissue that can<br />

occur with intravenous infusion of the highly alkaline<br />

phenytoin. The drug of choice for neonates in status is<br />

lorazepam. This agent has several properties that make<br />

it ideal—a long half-life <strong>and</strong> a small volume of distribution,<br />

which prolongs its retention at high levels in the<br />

brain.<br />

REFERENCES<br />

Table 3-14.<br />

Neonatal AED Dosing Suggestions<br />

Phenobarbital—20 mg/kg load over 10 to 15 min.<br />

If necessary add more phenobarbital in 5-mg/kg boluses<br />

Fosphenytoin—20 mg/kg at 1 mg/kg/min<br />

Ativan—0.1 mg/kg<br />

From Rennie J, Boylan G. Treatment of neonatal seizures. Arch Dis Child Fetal<br />

Neonatal 2007;92:F148–F150.<br />

1. Hauser WA, Beghi E. First seizure definitions <strong>and</strong> worldwide<br />

incidence <strong>and</strong> mortality. Epilepsia. 2008; 49(suppl 1):<br />

8-12.<br />

2. Teasell R, Bayona N, Lippert C, Villamere J, Hellings C.<br />

Post-traumatic seizure disorder following acquired brain<br />

injury. Brain Inj. 2007;21:201-214.<br />

3. Statler KD. Pediatric posttraumatic seizures: epidemiology,<br />

putative mechanisms of epileptogenesis <strong>and</strong> promising<br />

investigational progress. Dev Neurosci. 2006;28:<br />

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4. Agrawal A, Timothy J, P<strong>and</strong>it L, Manju M. Post-traumatic<br />

epilepsy: an overview. Clin Neurol Neurosurg. 2006;<br />

108:433-439.<br />

5. Weber YG, Lerche H. Genetic mechanisms in idiopathic<br />

epilepsies. Dev Med Child Neurol. 2008;50:648-654.<br />

6. Leventer RJ, Guerrini R, Dobyns WB. Malformations of<br />

cortical development <strong>and</strong> epilepsy. Dialogues Clin Neurosci.<br />

2008;10:47-62.<br />

7. Steinlein OK. Genetics <strong>and</strong> epilepsy. Dialogues Clin Neurosci.<br />

2008;10:29-38.<br />

8. Griffith JF, Ch’ien LT. Herpes simplex virus encephalitis.<br />

Diagnostic <strong>and</strong> treatment considerations. Med Clin North<br />

Am. 1983;67:991-1008.<br />

9. Geyer J, Keating J, Potts D, Carney P, eds. Neurology for the<br />

Boards. 3rd ed. Philadelphia: Lippincott Williams &<br />

Wilkins;2006.<br />

10. Riviello JJ. Classification of seizures <strong>and</strong> epilepsy. Curr<br />

Neurol Neurosci Rep. 2003;3:325-331.<br />

11. Durón RM, Medina MT, Martínez-Juárez IE, et al.<br />

<strong>Seizures</strong> of idiopathic generalized epilepsies. Epilepsia.<br />

2005;46:34-47.<br />

12. Gardiner M. Genetics of idiopathic generalized epilepsies.<br />

Epilepsia. 2005;46(suppl 9):15-20.<br />

13. Jallon P, Latour P. Epidemiology of idiopathic generalized<br />

epilepsies. Epilepsia. 2005;46(suppl 9):10-14.

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