Epilepsies with Neonatal Onset (Birth to 2 Months): Focal and Generalized Epilepsies



Epilepsies with Neonatal Onset (Birth to 2 Months): Focal and Generalized Epilepsies





Both focal and generalized seizures (see Chapter 1) are described in this chapter. Five syndromes have onset in the neonatal period; they are listed in Table 4-1.


I. NEONATAL SEIZURES

Children are at high risk for seizures during the first months and years of life. Because of the birthing process, the infant is at risk for a number of insults such as trauma, hypoxic-ischemic insults, intracranial hemorrhages, and infection. In addition, a large number of pathologic processes occurring in neonates may be seen initially with seizures. For example, congenital brain anomalies, inborn errors of metabolism, and genetic conditions may lead to recurrent seizures during the neonatal period. Because neonatal seizures are often associated with serious neurologic disorders, they are the most ominous neurologic signs in newborns. Because seizures may be the first and only sign of a central nervous system disorder, their recognition is extremely important. Despite advances made in the areas of obstetrics and perinatal care, seizures continue to be a significant predictor of poor neurologic outcome.

A multitude of changes occur in neuronal and glial growth and differentiation, myelination, and neurochemical composition of the brain during the third trimester of pregnancy and postnatal months. Because of immaturities in anatomic, chemical, and bioelectric connections in and between cortical and subcortical structures, it is not surprising that neonatal seizures differ markedly from those occurring in older subjects. They differ mainly in their clinical manifestations, in their etiologies, and in their short- and long-term prognosis.


A. Definition

Neonatal seizures refer to seizures that occur between birth and 2 months of age.


B. Seizure Phenomena

A considerable difference is apparent in the behavior observed during seizures in neonates and the behaviors seen in older children and adults. Infants are unable to sustain organized generalized epileptiform discharges, and generalized tonic-clonic and absence seizures do not occur. The age-dependent clinical and electroencephalographic (EEG) features of seizures in neonates are a result of the immaturity of cortical organization and myelination.









Table 4-1. Groups of epilepsy syndromes and specific epilepsy syndromes with neonatal onset (birth to 2 months) and accompanying seizure types

















Neonatal seizures syndrome (CLON, MYO, TON)


Benign familial neonatal convulsions syndrome (CLON)


Benign neonatal seizures (nonfamilial) syndrome (CLON)


Benign partial epilepsy of infancy (CPS, TCS)


Early myoclonic encephalopathy syndrome (MYO)


Otahara syndrome (CLON)


CLON, clonic seizure; CPS, complex partial seizure; TCS, tonic-clonic seizure; MYO, myoclonic seizure; TON, tonic seizure.


Neonatal seizures are classified as clonic, tonic, and myoclonic. Clonic seizures consist of rhythmic jerking of groups of muscles and occur in either a focal or a multifocal pattern. In multifocal clonic seizures, movements may migrate from one part of the body to another. Although focal seizures may be seen with localized brain insults, such as neonatal strokes, they may also be seen in disorders that diffusely affect the brain, such as asphyxia, subarachnoid hemorrhage, hypoglycemia, and infection. In tonic seizures, asymmetric posturing of the trunk or deviation of the eyes to one side develop in infants. Myoclonic seizures are similar to those seen in older children, consisting of rapid jerks of muscles. The myoclonic seizures can consist of bilateral jerks, although occasionally unilateral or focal myoclonus can occur.

Sick neonates often display repetitive, stereotyped behavior that may be confused with seizures. These behaviors include repetitive sucking and other oral-buccal-lingual movements, assumption of an abnormal posture, pedaling movements of the legs or paddling movements of the arms, blinking, momentary fixation of gaze with or without eye deviation, nystagmus, and apnea. However, when these behaviors are observed during EEG recordings, epileptiform activity is usually not recorded. Likewise, when tonic posturing involves all four extremities and the trunk, an associated EEG-epileptiform discharge rarely appears. Myoclonus not associated with epileptiform discharges also can be seen in sick neonates.


C. Electroencephalographic Phenomena

Whereas the diagnosis of seizures relies primarily on clinical observation, the EEG may be extremely valuable in confirming the presence of epileptic seizures. In addition, the EEG is very useful in the detection of electrographic seizures in paralyzed infants or in assessing response to antiepileptic medications.


1. Interictal Abnormalities

Background EEG patterns are very helpful in determining prognosis after neonatal seizures. Electroencephalograms demonstrating isoelectric, low-voltage burst suppression and excessive discontinuity are associated with poor prognoses, whereas records with normal backgrounds are associated with excellent outcomes.
The prognostic value of the EEG improves when similar findings are found on serial studies.

As with older children and adults, interictal spikes are seen more frequently in infants with seizures than in those without seizures. However, differentiating “normal” spikes and sharp waves from those with pathologic significance may be difficult. Strict criteria for differentiating normal sharp waves and spikes from those that are pathologic have yet to be established. For example, frontal sharp waves that shift from hemisphere to hemisphere (often termed “frontal sharp transients”) and multifocal spikes and sharp waves that occur only during the burst phase of quiet sleep (tracé alternant or tracéé discontinue) are considered normal by most electroencephalographers. In addition, normal infants, both term and preterm, may have infrequent, sporadic spikes and sharp waves.

Criteria used to classify spikes and sharp waves as abnormal include spikes and sharp waves that are focal and persist through all sleep states, rolandic-positive sharp waves, and focal or multifocal spikes during the low-amplitude phase of discontinuous sleep. Pathologic spikes often occur in bursts. As with older children, spikes and sharp waves can be seen in neonates who never have detected seizures.

Positive spikes occurring over the rolandic area are often associated with underlying white matter disease, such as periventricular leukomalacia or intraventricular hemorrhages. Positive rolandic spikes typically are not associated with seizures.


2. Ictal Discharges

Epileptiform activity that is rhythmic and has a distinct beginning and ending is considered an ictal event. In addition, most ictal discharges have some degree of evolution in frequency or morphology of the waveforms. Although no consensus yet exists on the minimal length of time required for the discharge to be considered ictal, we define an EEG ictus as 10 seconds of rhythmic epileptiform activity.

Ictal epileptiform activity can be divided into four basic types: focal spike or sharp-wave discharges, focal low-frequency discharges, focal rhythmic discharges, and multifocal discharges. Although the type of ictal discharge has not been shown to be specific to etiology, this classification system is useful for the purposes of description. Focal spike or sharp-wave discharges consist of rhythmic spikes or sharp waves that originate focally. The frequency of the discharge usually exceeds 2 Hz, and spread of the focal discharge to other regions occasionally occurs. The spread of ictal discharges in the immature brain is usually much slower than in older children and adults. Focal low-frequency discharges consist of focal spikes or sharp waves that occur at a low frequency (approximately 1 Hz). Differentiation of this ictal discharge from periodic lateralized epileptiform discharges is based primarily on evolution and duration. Periodic lateralized epileptiform discharges demonstrate no evolution, typically last longer than 10 minutes, and are considered nonepileptic activity.

Focal low-frequency ictal discharges usually demonstrate an evolution during the discharge in frequency, amplitude, or waveform
morphology (Fig. 4-1). Focal rhythmic patterns consist of rhythmic, monomorphic waves varying from 0.5 to 15.0 Hz. The ictal patterns often vary in frequency during the course of the discharge. In some patients, the ictal discharges “migrate” from one area of the cortex to another. The discharge may have a resemblance to “normal” activity and has been referred to as focal pseudo-beta-alpha-theta-delta discharges. However, unlike the normal background activity seen on the neonatal EEG, ictal betaalpha-theta-delta discharges are paroxysmal, rhythmic, and usually monomorphic in character (Fig. 4-2). Multifocal patterns consist of EEG discharges originating independently or, rarely, simultaneously from two or more foci.






Fig. 4-1. Focal low-frequency discharge arising from right central region. EKG, electrocardiogram; RESP, respiration.

Focal epileptiform activity does not necessarily imply focal pathology. Infants may have focal epileptiform discharges in the face of systemic disorders such as hypoglycemia or hypoxic-ischemic injuries. Cerebral infarctions are frequently associated with focal seizures and lateralized epileptiform discharges in term infants.

Electroencephalographic seizures often occur without any clear clinical accompaniment. In general, EEG seizures without clinical accompaniment have a poorer prognosis than electrical seizures with behavioral changes.


D. Differential Diagnosis

A challenge to the clinician in evaluating the neonate with seizures is differentiating seizures from other stereotyped repetitive behavior in the neonate. Many infants with central nervous system disorders have episodes of chewing, repetitive sucking, and other oral-buccal-lingual movements; assumption of an abnormal posture; pedaling movements of the legs or paddling movements of the arms; blinking; momentary fixation of gaze with or without eye deviation; or nystagmus that is not seizure activity. The EEG is enormously helpful in distinguishing seizure from nonseizure activity.

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Jun 17, 2016 | Posted by in GENERAL & FAMILY MEDICINE | Comments Off on Epilepsies with Neonatal Onset (Birth to 2 Months): Focal and Generalized Epilepsies

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