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Seizures and Epilepsy
Paul R. Carney and James D. Geyer
DEFINITIONS AND EPIDEMIOLOGY
A general simpliﬁed deﬁnition of a seizure is a sudden temporary change in brain function caused by an abnormal rhythmic electrical discharge. Epilepsy is, simply put, a state of recurrent seizure activity. The mechanism whereby a seizure turns into epilepsy, a process known as epileptogenesis, is controversial. Seizures are common in humans, with an incidence of approximately 80/100,000 per year and an overall risk of epilepsy of 1% to 3%.1 Status epilepticus is a less common form of severe prolonged seizure activity with a high morbidity and mortality.
Seizures arise secondary to a number of etiologies. Idiopathic seizures, or “cryptogenic” seizures, are fairly common. Contrary to what many patients and families might think, the inability to ﬁnd a cause for the seizure is not necessary “bad.” In fact, this may portend a somewhat better prognosis for long-term seizure control. Febrile seizures are common in children and are covered in detail in Chapter 4. Trauma contributes to the risk of seizures in two fairly distinct fashions. Early posttraumatic seizures are typically associated with intracranial hemorrhage, focal neurological deﬁcits, posttraumatic amnesia exceeding 24 hours, and linear skull fractures. Late posttraumatic seizures are also associated with intracranial hemorrhage and posttraumatic amnesia exceeding 24 hours, but are usually seen in patients with depressed skull fractures and with the injury after age 16 years.2–4 A number of congenital malformations increase the risk for epilepsy. Disorders associated with migrational
disorders and structural anomalies often increase the risk of subsequent seizures. The genetic diseases listed in Table 3-1 also increase the risk of epilepsy whether or not they are associated with structural malformations.5–7 Infections are also common causes of seizure activity in the pediatric population. Meningitis and encephalitis can result in seizures either related to the fever or to the direct effects of the infection. These are covered in detail in the chapters on infectious disease. Bacterial infections can result in meningitis, encephalitis, and abscess formation. Herpes simplex virus (HSV) is a well known cause of seizures and can be catastrophic.8 Other viral infections including cytomegalovirus (CMV) infection and various viral encephalitides can result in seizures. Fungal infections and toxoplasmosis also raise the risk of developing seizures. A wide array of toxic and metabolic disorders can result in seizures. These derangements can cause seizures to occur de novo but can also worsen a preexisting epilepsy. The common metabolic and toxic causes of seizures are listed in Table 3-2.
Table 3-1. Genetic Causes of Epilepsy
Amino acidurias Channelopathies Lysosomal storage diseases Phakomatoses—tuberous sclerosis, von Hippel–Lindau disease, neuroﬁbromatosis Phenylketonuria (PKU) Sturge–Weber syndrome
The EEG serves as a vitally important tool in the correct diagnosis of the various epilepsy subtypes and syndromes. Toxic/Metabolic Causes of Seizures Drug Intoxication Amphetamines Cocaine Lidocaine Theophylline Tricyclic antidepressants Drug withdrawal Antiepileptic drugs (AEDs) Barbiturates Benzodiazepines Ethanol Electrolyte—hypo/hypernatremia. and cyanosis. hypomagnesemia Heavy metals—lead. These seizures may be divided as follows10: Both focal and generalized Situation-related epilepsy Febrile convulsions Isolated seizure Isolated status epilepticus Toxic/metabolic In each of these cases. Rolandic epilepsy. The interictal EEG is highly variable with a normal background in some patients and slowing present in others. DIAGNOSIS Seizure Types Generalized tonic-clonic seizures Generalized tonic-clonic seizures typically have no preceding aura but may have a prodrome of apathy or irritability. Several subtypes of generalized seizures have also been identiﬁed. the electroencephalographic (EEG) findings may be different. The ictal EEG usually consists of generalized spike and wave or polyspike activity. Cerebral ischemia is a common cause of seizures in the neonate and in the older adult but is relatively uncommon in the older pediatric population. the jaw snaps shut followed by 10 to 15 seconds or longer of tonic spasms. Seizures usually occur in the more slowly growing tumors. with confusion. Epileptic Syndromes and Related Seizure Disorders groups these disorders in the “undetermined” category. Localizationrelated epilepsy or partial epilepsy has a primary focus from which the electrical discharges arise. the seizure affects the entire cortex electrically. The postictal phase lasts for minutes to hours. and frontal lobe epilepsy are all examples of partial epilepsy. The Revised International Classiﬁcation of Epilepsies.CHAPTER 3 Seizures and Epilepsy ■ 31 Table 3-2. generalized tonic-clonic seizures. Epileptic Syndromes and Related Seizure Disorders divides the localization-related epilepsies as follows:10 Idiopathic localization-related epilepsy Symptomatic or secondary localization-related epilepsy Cryptogenic localization-related epilepsy . The Revised International Classiﬁcation of Epilepsies. The clonic phase usually consists of 1 to 2 minutes of rhythmic generalized muscle contractions and increased blood pressure. hypocalcemia. apnea. Jacksonian motor seizures. In this category of epilepsy. mercury Hyperosmolarity Hypoxia Liver failure Porphyria Pyridoxine deﬁciency Thyroid storm Uremia (usually following 3 days of anuria) Generalized seizures are the other major seizure type. and progressive myoclonic epilepsy. Epileptic Syndromes and Related Seizure Disorders divides the generalized epilepsies as follows10: Primary generalized epilepsy Symptomatic generalized epilepsy Cryptogenic epilepsy A number of seizures and epilepsies may be very difﬁcult to categorize. juvenile myoclonic epilepsy. and possibly agitation. Generalized seizures are rare in newborns. somnolence. During the tonic phase. CLINICAL PRESENTATION Epilepsy is divided into several categories with signiﬁcant differences in the characteristics of the electrical discharges as well as the clinical manifestations. Tumors located in the supratentorial region cause seizures more frequently than do cerebellar or brainstem tumors. hypo/hyperglycemia. temporal lobe epilepsy. Generalized seizures occur most frequently in children secondary to fevers and metabolic derangements. The Revised International Classification of Epilepsies. including absence epilepsy with 3-Hz spike and wave activity. Complex partial seizures occur with alteration of awareness while simple partial seizures have no alteration of awareness.
There is a sudden interruption of consciousness. The EEG consists of a hypsarrhythmia pattern with bursts of asynchronous slow waves. vertebral anomalies. Atypical absence epilepsy usually occurs in children who are neurologically or developmentally abnormal. Most of these disorders have a genetic basis.13 sharp waves alternate with a suppressed EEG. Patients are usually developmentally and neurologically normal. Atypical absence seizures have generalized spike and wave activity but usually have a frequency less than 3 Hz.11 The ictal EEG usually consists of generalized polyspike and slow wave activity. There is no alteration in consciousness. but postictal aphasia may occur if the primary epileptogenic zone involves the dominant hemisphere.17 The clinical features of West syndrome include infantile spasms and mental retardation. The EEG associated with these disorders is variable. staring.14–16 The seizures associated with West syndrome consist of a jack-knifing movement and myoclonus. which varies according to the etiology of the spasms. The interictal EEG usually has a normal background. The seizures are described as infantile spasms. Aicardi syndrome is an X-linked disorder present from birth that is associated with infantile spasms.32 ■ Section 2: Common Pediatric Neurologic Problems Absence seizures Absence seizures typically have no preceding aura or prodrome.13 Table 3-3. Febrile seizures are covered in detail in Chapter 4. agenesis of the corpus callosum. but alternating hemiconvulsions may also be seen. The background is often slow. The interictal EEG is typically unremarkable. A complicated febrile seizure has prolonged seizure activity or focal seizure activity. spikes and Complex partial seizures Benign Rolandic epilepsy. These seizures begin with tonic contractions of the face. motor.18 Lennox–Gastaut syndrome Lennox–Gastaut syndrome typically begins between 1 and 10 years of age.11 Partial seizures: localization-related epilepsy Jacksonian motor seizures are simple partial seizures with no alteration of consciousness. There is no postictal confusion.19 Progressive myoclonic epilepsy The family of disorders known as the progressive myoclonic epilepsies (Table 3-3) consists of a number of loosely related disorders. multifocal spikes. associated with variable degrees of mental retardation. Infantile spasms West syndrome typically begins between 3 months and 3 years of age.11 The ictal EEG usually consists of 3-Hz generalized spike and wave activity with some slowing of the discharge frequency during the seizure. Benign Rolandic epilepsy usually begins between ages 5 and 10 years and is transmitted in an autosomal dominant pattern with variable penetrance. Progressive Myoclonic Epilepsies Dentorubral-pallidoluysian atrophy Juvenile neuroaxonal atrophy Lafora disease Late infantile and juvenile GM2 gangliosidosis Myoclonic epilepsy and ragged red ﬁbers (MERRF) Neuronal ceroid lipofuscinosis (NCL) (also known as Batten disease) Noninfantile Gaucher disease Sialidosis Unverricht–Lundborg disease (Baltic myoclonus) Febrile seizures Febrile seizures occur with a prodromal fever. 3-Hz blinking. Juvenile myoclonic epilepsy The seizures associated with juvenile myoclonic epilepsy typically have no preceding aura but may have a prodrome of morning myoclonus. and less frequently automatisms. sensory. however. A simple febrile seizure occurs as a brief generalized tonic clonic seizure occurring after the onset of fever. Simple partial seizures may involve autonomic (Table 3-5). It is fairly common. The clinical features of Aicardi syndrome include coloboma.12 The age of onset of juvenile myoclonic epilepsy is typically 10 to 20 years. with an incidence of . There are multiple seizure types. These epilepsy subtypes are quite rare and have complex presentations and diagnostic ﬁndings. The seizures may consist of generalized tonic-clonic activity. and generalized paroxysmal fast activity (GPFA). or psychic functions. or feet and transform into clonic movements that march to other muscle groups on the ipsilateral hemibody.5 Hz. ﬁngers. though sporadic cases have occurred in some cases (Table 3-4). The seizures are typically generalized. The EEG reveals a slow spike wave complex with a frequency of 1 to 2. absence seizures may also occur. and seizures. chorioretinal lacunae.12 Absence seizures typically start between ages 4 and 10 years and resolve by age 20 years. The postictal phase is variable depending on the seizure type. An absence seizure usually lasts for only several seconds to minutes.
Alteration in consciousness. Postictal confusion is rare. The ictal EEG usually consists of a focal central or mid-temporal ictal onset. Auras are also common but not universal and include an array of ﬁndings such as déjà vu. Auras are unusual.21. The postictal phase consists of minutes to hours of confusion and somnolence. Right temporal lobe seizures are often hypermobile. Left temporal lobe seizures often result in behavior arrest. Frontal seizures often have atypical presentations and vary widely depending on the region of the frontal lobe from which the seizures arise (Table 3-6). Frontal lobe epilepsy accounts for approximately 20% of partial seizures. contralateral twitching or tonic–clonic movements.23. and posturing. Distinguishing Characteristics of the Progressive Myoclonic Epilepsies Clinical Features Ch o r e a Dentorubral-pallidoluysian atrophy Juvenile neuroaxonal dystrophy Juvenile Gaucher disease Deafness Biotin-responsive encephalopathy MERRF Sialidosis type II F o ca l O c c i p i t a l S p i k e s MERRF Unverricht–Lundborg disease Little or No D ement i a Biotin-responsive encephalopathy Noninfantile Gaucher disease Myoclonus—nal failure Sialidosis type I Unverricht–Lundborg disease S ev e r e D e m e n t i a GM2 gangliosidosis Juvenile neuroaxonal dystrophy Lafora disease Late infantile NCL S ev e r e M y o c l o n u s Lafora’s disease MERRF Sialidosis Table 3-5.24 Temporal lobe epilepsy. head and eye deviation. The ictal findings or semiology include oral or motor automatisms. The seizures resolve by age 16 years. aura. Versive head movements are relatively common.000 children. Ipsiversive movements are less common but occur most commonly in patients with temporal foci. A prodrome consisting of lethargy is common.31–36 Genetics Au tosomal D o m i n a nt Dentatorubral-pallidoluysian atrophy Kuf disease Geography Ca n a d a Myoclonus—renal failure Fi n l a n d Santavori disease Unverricht–Lundborg disease Japan Dentatorubral pallidoluysian atrophy Sialidosis type II S we d e n Gaucher disease Maternal Inheritance MERRF 21/100.24–30 Frontal lobe epilepsy. with the possibility of secondary generalization. and postictal confusion are rare. A prodrome is rare. Secondary generalization is common. 22 .CHAPTER 3 Seizures and Epilepsy ■ 33 Table 3-4. The seizures typically consist of combinations of behavior alteration and automatisms of very brief duration.20 The clinical features include a single nocturnal seizure with clonic movement of the mouth and gurgling. Temporal lobe epilepsy accounts for approximately 70% of partial seizures. Possible Autonomic Seizure Clinical Features Abdominal sensations Apnea Arrhythmia Chest pain Cyanosis Erythema Flushing Genital sensations Hyperventilation Incontinence Miosis Perspiration Vomiting The interictal EEG consists of central and midtemporal high-amplitude spike and wave with a characteristic dipole. and 90% of patients with versive head movements had a primary epileptogenic zone in the contralateral hemisphere. Many patients have a prior history of febrile seizures or head trauma. alteration of consciousness.
The disorder is usually unilateral. there are typically elemental visual hallucinations. however. but over 2/3 of children are left with signiﬁcant language or behavioral deﬁcits. When the striate cortex is involved.40-42 Landau–Kleffner syndrome. accounting for less than 10% of partial seizures. music Autoscopy Objects. The may be seen as simple partial seizures but they will often propagate. The initial features can include contralateral paresthesias. jamais vu. predominating in the temporal lobes. Occipital lobe epilepsy. EEG is a vital component of the evaluation to categorize the seizure type and assist with planning of the treatment strategy. invariably progressive. blurred Hallucination Flashbacks Voices. pulsing lights. Rasmussen encephalitis is a syndrome of diffuse lymphocytic inﬁltration of the brain associated with partial seizures and progressive neurological deterioration with hemiparesis. EVALUATION As with many facets of neurology. the history is the most important diagnostic tool and should include information on each of the items in Table 3-7. This disorder typically affects children 1 to 14 years old. if possible. contralateral pain. The MRI sequences are much more sensitive to the causes of epilepsy than is CT imaging.47 Occipital (%) 0 5 5 5 10 5 10 15 0 50 0 Aura Type Auditory Cephalic Epigastric General Gustatory None Olfactory Psychical Somatosensory Visual Vertiginous Frontal (%) 0 15 15 15 0 40 0 5 15 5 2 Rasmussen encephalitis. clearer Depersonalization. Involvement of the lateral occipital lobe results in twinkling. Many patients are unable to provide accurate descriptions of the seizure and the postictal period. faces. far.45 Sometimes corticosteroids and IV Ig or even surgery with subpial transection46 are used in refractory Table 3-7.44 Treatment is usually with valproic acid and benzodiazepines.34 ■ Section 2: Common Pediatric Neurologic Problems Table 3-6. softer. The child then develops a verbal auditory agnosia and infrequent nocturnal partial or secondarily generalized seizures.37-39 Parietal lobe epilepsy. The outcome for overall language and cognitive function depends in part on how early the syndrome is recognized and treated. CT can. and antibodies to the glutamate subunit GluR3 are commonly identiﬁed. be of help in the emergency department setting. Occipital lobe epilepsy is rare. Rasmussen encephalitis is very difﬁcult to treat and frequently requires surgical management with hemispherectomy. The syndrome has a pathognomonic EEG pattern consisting of high-voltage multifocal spikes. the seizure characteristics are dependent on the area of the occipital lobe involved. rushing. slowing Macropsia. The history should be obtained from family and eyewitnesses. and limb movement sensations. idiomotor apraxia. louder. The syndrome is associated with perivascular cufﬁng on pathologic sections. near. Frequency of Aura Types by Location Temporal (%) 10 5 50 10 10 15 10 15 5 10 10 cases. Prodromes are rare with occipital lobe seizures and auras are unusual. Initial evaluation with ﬂuid balance proﬁle (FBP). micropsia. idiopathic acquired aphasia related to a focal epileptic disturbance in the area of the brain responsible for verbal processing. scenes . Aura Types Psychical Auras Memory Sound Self-image Time Vision Illusion Déjà vu. Parietal lobe seizures are also relatively uncommon. receding. Ca++. As with the frontal lobe seizures. As the seizure progresses and propagates. remoteness Stand-still. The need for laboratory testing is highly variable depending on the history.43 The syndrome begins between ages 3 and 10 in a child with normally acquired language abilities. Seizures arising from the temporo-occipital are usually associated with formed visual hallucinations. strangeness Advancing. MRI of the head with temporal lobe protocol (thin coronal slices through hippocampi) is the preferred imaging modality for most patients. asymmetric tonic posturing and automatisms may develop. Landau–Kleffner syndrome is a rare.
emotion. Guidelines for the selection of antiepileptic drugs. The patient selection criteria for treatment after a single seizure are listed in Table 3-8. and liver insufficiency should be considered. In the absence of a structural cause for the seizures or a typical syndrome of epilepsy. and liver function tests (LFTs) is important for both the search for a potential cause of the seizures and for medication selection. chewing. menstrual cycle. genital manipulation Seizures include gustatory sensation. gagging Orbitofrontal Blinking or staring Complex automatisms Probably Not Acute febrile illness Drug withdrawal or intoxication Electrolye imbalance EtOH withdrawal Hyper/hypoglycemia Immediate posttraumatic seizure Severe sleep deprivation–related seizure Supplementary Motor Area Contralateral tonic posture Simple motor seizure Somatosensory aura Tonic eye and head contraversion Vocalizes . stress. the concomitant medical disorders such as headache. Cingular Amnesia Facial expressions of fear and anger “Psychotic” appearance Dorsolateral frontal Simple partial Tonic eye and head contraversion Table 3-10. However. Urine drug screening should be obtained for patients with new-onset seizures. Table 3-9. Components of a Seizure History Aura Birth and developmental history CNS infections Exacerbating factors (sleep. and potential interactions are reviewed in Tables 3-10 to 3-13. bone marrow dysfunction. salivation.50-53 Surgery for epilepsy is an important but often underutilized treatment option. The prognostic factors used for making the decision regarding discontinuation are listed in Table 3-9. most patients do not require long-term treatment with an antiepileptic medication. substance abuse) Family history of epilepsy Head trauma Postictal state Seizure description by an eyewitness TREATMENT Physicians and families often agonize over the decision about whether to initiate therapy after a single seizure.CHAPTER 3 Seizures and Epilepsy ■ 35 Mg++. Characteristics of Frontal Lobe Seizures by Region of Onset Anteromedial Frontal Contralateral eye and head version Frequent generalization Somatosensory aura Tonic posture The selection of a particular antiepileptic medication for a given subtype of epilepsy has long been the subject of controversy. When selecting a given drug.49 Stopping the antiepileptic medication can also be a challenge.48. Patient Selection Criteria for Treatment After a Single Seizure Probably AVM Brain tumor CNS infection Immediate family history of epilepsy Frontopolar Loss of tone Rapid generalization Opercular/Insular Complex seizures include gagging. amnesia. The surgical option provides an opportunity for some patients to become seizure free. the pharmacology of the common antiepileptic drugs. swallowing.49 Table 3-8. this is a complex discussion and beyond the scope of this book.
and neurons in such regions as the hippocampus are excited rather than inhibited by the neurotransmitter GABA (normally the primary inhibitory neurotransmitter in the brain). malingering. Several categories of non-epileptic events (thrashing. Keating J. 3rd ed. Clonic. The imbalance occurs anatomically and physiologically by an overexpression of NMDA receptor in the hippocampus and neocortical regions of the neonatal brain. 2006. Philadelphia: Lippincott Williams & Wilkins. and the infant is not clearly unconscious. accounting for 50% to 60% of patients with neonatal seizures.36 ■ Section 2: Common Pediatric Neurologic Problems Table 3-11. 1b 2 2 2 2 2 2 1 1 2 2 1b 1b Numbers refer to order of preference for use in speciﬁc seizure types. These seizures may have limited EEG changes correlating with the seizure activity. Carney P. 2006. Etiologies include conversion disorder.57 The neonatal brain is particularly vulnerable to seizure activity as a result of an imbalance of excitatory to inhibitory circuitry. Potts D. and often difﬁcult to treat. From Geyer J. Symptoms suggesting non-epileptic events include closed eyes. As the name suggests. especially in sick neonates. Keating J. Table 3-12. Focal clonic seizures involve one side of the body. 58-61 Neonatal Seizures Neonatal seizures are poorly classiﬁed. Infants. especially cardiac disorders. progressive integration of circuitry. 3rd ed. a delay in the maturation of the inhibitory system. hypoxic ischemic encephalopathy is the most common etiology. chewing.54-56 Neonatal seizures are often the presenting clinical manifestation of underlying neurological conditions such as hypoxic-ischemic encephalopathy. slow movements. Clonic seizures typically present as rhythmic. lack of cyanosis. especially in the younger child. Neurology for the Boards. etc. Potts D. eds. non-physiologic progression. apoptosis. staring. The movements have a frequency of 1 to 3 Hz. In some cases there may be “boxing” movements. sustained eye opening. intraventricular or intraparenchymal hemorrhages. eds. and rapid reorientation following the event. Non-epileptic Events Non-epileptic events are unusual in the pediatric population. Clinical presentation Subtle. meningitis. crying. pelvic thrusting. Carney P. Subtle seizures are more common in premature infants. Antiepileptic Drug Selectiona Seizure Type Infantile spasms Absence Tonic-clonic Myoclonic Atypical absence Simple partial Complex partial a VPA 2 1 1 1 3 2 LTG 2 2 2 2 2 TPM LVT ETX 1 ACTH 1 GBP CBZ PHT 2 PHB 2 2 3 2 4.) have different natural histories and variable prognosis. the seizures may be difﬁcult to identify with only tonic horizontal eye movements. stroke. Prognostic Factors for Stopping AEDs Favorable Primary generalized epilepsy Idiopathic epilepsy Childhood onset Easy to control Normal neurological examination Normal intelligence More than 2–3 years seizure-free Normal EEG Unfavorable Partial epilepsy Identiﬁable lesions Adult onset Difﬁcult to control Abnormal neurologic examination Mental retardation Less than 3 years seizurefree Epileptiform EEG From Geyer J. sepsis. and medical conditions. lack of tongue biting. and does so to facilitate important developmental processes that occur during the neonatal period (synaptogenesis. under-recognized. The imbalance favors excitation. Neurology for the Boards. variable semiology. Of these. or metabolic disorders. synaptic pruning). or apnea. b . Philadelphia: Lippincott Williams & Wilkins. resisted eyelid opening.
Focal tonic seizures result in sustained posturing of a limb. These seizures are usually accompanied by EEG changes. increase. Often. Conversely. Keating J. eds. Generalized clonic seizures are rarely observed in newborn because of the incomplete myelination of the brain. 2006.58–61 Tonic.58–61 upper limbs and tonic extension of lower limbs (mimicking decerebrate posturing). the trunk. or the neck.CHAPTER 3 Seizures and Epilepsy ■ 37 Table 3-13. These generalized seizures are more likely to have EEG changes.58–61 . 3rd ed. or no change No change Decrease Increased concentration of derived phenobarbital No change No change Increased concentration of derived phenobarbital Increase Decrease. Carney P. Drug–Drug Interactions: Effects on Serum Concentration of Adding a Second Antiepileptic Drug to First Antiepileptic Drug Effects of Added Drug on Serum Concentration of Original Drug No change Decrease Decrease Decrease Decrease Decrease No change Decrease Increase No change No change No change Increase or no change No change Data conﬂicting Increase Increase Increase Increase or decrease Data conﬂicting No change Increase Decrease. generalized myoclonic seizures exhibit bilateral jerks of both upper and lower limbs. Generalized tonic seizures exhibit tonic extension of all limbs (mimicking decorticate posturing) or tonic ﬂexion of Myoclonic. increase. often in a migrating pattern. Neurology for the Boards. or no change No change No change Decrease Decrease Decrease Original Drug Carbamazepine Added Drug Clonazepam Phenobarbital Phenytoin Primidone Phenobarbital Phenytoin Valproate Carbamazepine Methylphenobarbital Phenobarbital Phenytoin Primidone Valproate Carbamazepine Clonazepam Methsuximide Phenytoin Valproate Carbamazepine Clonazepam Ethosuximide Methsuximide Phenobarbital Primidone Valproate Carbamazepine Clonazepam Ethosuximide Phenytoin Valproate Carbamazepine Clonazepam Ethosuximide Phenobarbital Phenytoin Primidone Clonazepam Ethosuximide Phenobarbital Phenytoin Primidone Valproate From Geyer J. Multifocal clonic seizures involve several body parts. Philadelphia: Lippincott Williams & Wilkins. and may resemble infantile spasms. Focal myoclonic seizures usually involve ﬂexor muscles of an upper extremity. there are no EEG changes. There are no EEG changes in 85% of cases. Potts D.
Epilepsia. sepsis). 2. 49(suppl 1): 8-12. Lippert C.46:34-47. Hauser WA. Guerrini R. The seizures usually resolve within 2 months and neurological outcome is normal. Classiﬁcation of seizures and epilepsy. Weber YG. Currently. Epilepsia. 11. Gardiner M. 2006. Martínez-Juárez IE. putative mechanisms of epileptogenesis and promising investigational progress. Timothy J. 6. Dialogues Clin Neurosci. Pandit L.21:201-214. Grifﬁth JF. The syndrome is usually self-limited and benign.1 mg/kg From Rennie J. Herpes simplex virus encephalitis. Leventer RJ. the frontline agent of choice for treating seizures is phenobarbital (see Table 3-14 for dosing suggestions). Epidemiology of idiopathic generalized epilepsies. The seizures are usually bilateral myoclonic jerks that last for several minutes and occur only during NREM sleep.58–61 occur with intravenous infusion of the highly alkaline phenytoin. Benign myoclonus of early infancy Benign myoclonus of early infancy has an onset at age 3 to 9 months but it can be much earlier.58–61 Table 3-14. Geyer J. 8. Agrawal A. Clin Neurol Neurosurg. Potts D. fosphenytoin. Seizures of idiopathic generalized epilepsies. 2005. Neurological development is normal. 13. Lerche H. 108:433-439. Philadelphia: Lippincott Williams & Wilkins. can be administered more quickly than phenytoin. Manju M. Neonatal AED Dosing Suggestions Phenobarbital—20 mg/kg load over 10 to 15 min. eds.46(suppl 9):15-20. If the clinician cannot find a readily identiﬁable and treatable etiology.62 Phenobarbital as a single agent will stop seizure activity in 42% of patients. Dev Neurosci. Keating J.10:47-62. Villamere J.10:29-38. When the seizure does not respond to a single agent. 2003. The seizures worsen with the administration of benzodiazepines. Latour P. 10.”62 Purple glove syndrome is necrosis or injury of the soft tissue that can . Epilepsia. 2008. First seizure deﬁnitions and worldwide incidence and mortality. Beghi E. 1983. The seizures usually last for less than 24 hours. Med Clin North Am. 2005. Post-traumatic seizure disorder following acquired brain injury. Brain Inj. If necessary add more phenobarbital in 5-mg/kg boluses Fosphenytoin—20 mg/kg at 1 mg/kg/min Ativan—0. which prolongs its retention at high levels in the brain. Post-traumatic epilepsy: an overview. The seizures are typically multifocal clonic seizures and are frequently associated with apnea. 4.38 ■ Section 2: Common Pediatric Neurologic Problems Syndromes Benign familial neonatal seizures Benign familial neonatal seizures occur as a genetic disorder with an autosomal dominant inheritance pattern associated with chromosome 20q. Statler KD. Diagnostic and treatment considerations.46(suppl 9):10-14. The seizures usually occur while the patient is awake. The neonate may have as many as 10 to 20 seizures per day. Bayona N.50:648-654. Ch’ien LT.28: 354-363. Epilepsia. Pediatric posttraumatic seizures: epidemiology. 9. Teasell R. 12.58–61 REFERENCES 1. The seizures typically start on day of life 2 or 3. Medina MT. This agent has several properties that make it ideal—a long half-life and a small volume of distribution.67:991-1008. Fifth-day ﬁts Fifth-day ﬁts usually begin on day of life 4 to 6. 2005. and will not cause “purple glove syndrome. Riviello JJ. Dev Med Child Neurol.92:F148–F150. 3rd ed. Hellings C. Fifth-day ﬁts progress to status epilepticus in 80% of cases. Jallon P. 2008. Genetic mechanisms in idiopathic epilepsies. The EEG is normal or slow. et al.2006. Curr Neurol Neurosci Rep. 2006. 5. Treatment of neonatal seizures. 2008. 3. 7. The drug of choice for neonates in status is lorazepam. Genetics and epilepsy. Boylan G. but approximately 10% of cases progress to an antiepileptic drug-requiring seizure disorder. Benign neonatal sleep myoclonus Benign neonatal sleep myoclonus begins during the ﬁrst week of life. Dobyns WB. Arch Dis Child Fetal Neonatal 2007.3:325-331. Neurology for the Boards. Genetics of idiopathic generalized epilepsies. Carney P. hypocalcemia. The seizures disorder may continue for 1 to 2 years but neurological outcome is normal. Dialogues Clin Neurosci. The seizures resemble infantile spasms but the EEG is normal. is preferred in the neonate because it is an aqueous solution that is soluble in glucose-containing solutions. Steinlein OK.58–61 Treatment The clinician should ﬁrst search for underlying etiologies producing the seizures and treat (hypoglycemia. 2007. Durón RM. Malformations of cortical development and epilepsy. the salt ester of phenytoin. 2008. phenytoin is added with an increase in efﬁcacy to 65%.
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