An Update in Pediatric Seizure Disorders Linda P

An update in pediatric seizure disorders Linda P. Nelson, DMD, MScD Steven D. Ureles, DMD, MS Gregory Holmes, MD

Abstract can be identified while in the idiopathic group, no Recent advancesin knowledgeof pediatric seizure disor- etiologic factors can be detected. Etiological agents in ders, including classification of seizure types and febrile children differ from those in adults. Seizures in adults convulsions, have been the topics of major symposia.In light most frequently are caused by brain tumors, cerebro- of these recent developments,an in-depth review is presented vascular accidents, and trauma, while in children, con- to aid the pediatric dentist in the treatmentof these children. genital brain malformations, infections, metabolic disease, hypoxic-ischemic injuries, and inherited disorders Introduction are more common. Contrary to the belief of the lay Epilepsy is so common,that it is likely that pediatric public, brain tumors rarely cause seizures in children. dentists will have children in their practices with the Classification disorder. Pediatric dentists must be aware of the clinical features of this disorder and the behavior management Seizures first were classified accordingto their clinical problems that may occur in this population. This paper features. Our understanding that seizures can be symptomsof disease either within or outside the nervous will review and update the behavior manifestations and current treatment of childhood epilepsy. system, and that seizures may reflect the location of Seizures are among the most frequently observed abnormal neuronal activity, has caused a continuous neurologic disorders in children, with as manyas 4% of reclassification of seizures and epilepsy (Farmer and all children having had at least one seizure during the Greenwood 1983). Table 2 shows an overview of the first 15 years of life. Recurrent seizures affect about 0.5% classification system. Seizures are partial, generalized, of children (Dodsonet al. 1976, Part I). By definition, or unclassified, according to the electrophysiologic seizure is the clinical manifestation of abnormalneuronal source of discharge and video monitoring techniques. hyperactivity, and its location determines the type of seizure. Epilepsy is defined as recurrent, unprovoked Table1. Glossaryof Terms seizures. One seizure does not constitute epilepsy. Term Definition Similarly, recurrent provoked seizures, i.e. febrile seizures, do not mean that the child has epilepsy. While Epilepsy Recurrent unprovoked seizures epilepsy means recurrent, unprovoked seizures, it Convulsions Seizures with motor manifestations needn’t be a lifelong disorder. Approximately 60-70% Myoclonic Brief body jerks of children with epilepsy outgrow the disorder. Seizures are symptomsof underlying disease and as such can be Tonic Continuouscontraction(s) the modeof expression for manydiseases. Tables 1 and Clonic Alternating contraction and relaxation 2 (next page) give the pediatric dentist a glossary Atonic Loss of tone useful terms. Akinetic Motionless The features of seizure disorders in childhood are Typical Absences sufficiently distinctive to deserve separate discussion. Neonatal seizures, infantile spasms, febrile convulsions, Simple staring episodes with impairmentof consciousness and the absence seizure syndrome are specific childhood seizure types. The etiology of pediatric seizures Complex staring episodes, impairmentof can be divided into two groups: symptomatic and id- consciousness, automatisms, clonic iopathic. In symptomaticepilepsy, an etiological factor activity, changesin postural tone

128 PEDIATRICDENTISTRY: MAY/JUNE, 1991- VOLUME13, NUMBER3 Table2. Classificationof seizures(Dreifuss 1989)

Generalized Partial Unclassified

Begins simultaneously in both begins in one cerebral hemisphere Anyseizure which cannot be cerebral hemispherescharacter- / ~ classified because of inadequate or ized by loss of consciousness incompletedata. This includes and motor changes which are y ~ someneonatal seizures, i.e.: generalizedor bilateral. Simple Complex rhythmic eye movements, chewing ...... consciousness ~mpa~red- mov emen st ana- ’ swimming’ ktocal, jacKsonlan} aoerrat~onsor nenaviori.e.: movements automatisms can occur. .,~~consciousness retained(temporal lobe,.... psychomotor partial) PrimaryGeneralized Epilepsy ~ ~ Both types of partial seizures mayspread and become Idiopathic Epilepsy - \ "xsecondarily generalized. not associated with neurological abnormalities and \ \ SecondaryGeneralized Epilepsy °nft~na~PolP~ari~eC~deh°°d ~ These a~e associated wi’ th’ne’urologically abnormaland delayed psychomotor a . scen ~ ..se \ develovmentand indicative of difuse cerebral ~atholo~¢ i.e.: Lennox-Gestaut se~.zures resvonawell to \ treatment and often ~ ~ynarome, west 5ynarome. ,~u are common~n cnn~noo~Put can occur at diminish in frequency or \ any age. disappear after adolescence Gener~dizedSeizures Furtl~,r Subclassified (Masland 1974). Simpleand Complextypical absence (petit mal) i.e.: pyknolepsy,juvenile absence epilepsy, juvenile myoclonicepi.lepsy; atypical absence;clonic; tonic; tonic-clonic (grand real); atonic; myoclonic(akinetic, minor motor); infantile spasm- symptomatic cryptogenic;reflex seizures; and febrile seizures.

Partial seizures begin in one cerebral hemisphere and Generalized Seizures are subclassified as simple (if consciousness is retained) or complex (if consciousness is altered). Generalized Tonic-Clonic Seizures (Formerly Termed Grand Mal seizures begin simultaneously in both hemispheres and Seizures) are characterized by loss of consciousness and motor The generalized tonic-clonic seizure involves all ex- changes which are generalized or bilateral. The gener- tremities and has both tonic and clonic features. Ten per alized seizures are subdivided into primary and sec- cent of adolescent and adult patients with epilepsy ondary generalized epilepsies. Primary generalized suffer from tonic-clonic seizures (Gastaut et al 1974). epilepsies, called idiopathic epilepsy, are not associated These seizures usually are not present in infants due to with neurological abnormalities. These seizures respond relatively unmyelinated neurons and reduced neuronal well to treatment and often diminish in frequency or excitability of the CNS (Vernadakis and Woodbury disappear after adolescence (Masland 1974). Secondary 1969). Most generalized tonic-clonic seizures begin as generalized epilepsy is associated with neurologically partial seizure and then becomegeneralized secondarily. abnormal and delayed psychomotor development and Anaura is, in fact, a simple partial seizure. If a patient is indicative of diffuse cerebral pathology (i.e. Lennox- has an aura before a generalized tonic-clonic seizure it Gastaut syndrome and West syndrome). These seizures means that the seizure began partially and then gener- are commonlyseen in childhood but can occur at any alized. age. Generalized seizures are further subclassified as Tonic-clonic seizures usually begin with a bilateral simple and complex typical absence (petit mal), atypi- generalized massive myoclonic muscular jerk, which cal absence, tonic, clonic, tonic-clonic (grand mal), atonic lasts for several seconds and is often accompaniedby a seizures, akinetic seizures, bilateral myoclonic, and in- jerky cry. The person is propelled forward or backward fantile spasms. The nomenclature can becomevery con- and mayfall to the ground. fusing, especially when a simple partial seizure The next phase is a series of both tonic and clonic progresses to a secondarily generalized seizure. motor and autonomic changes. The tonic phase consists An unclassified category includes seizures which of a sustained muscular contraction lasting 20-30 sec cannot be classified because of inadequate or incom- whichusually consists of a brief phase of flexion followed plete data (Bresnan and Konkol 1987). by a longer period of extension. During the tonic flexion

PEDIATRICDENTISTRY: MAY/JUNE, 1991N VOLUME"13, NUMBER3 129 phase, consciousness is lost abruptly. The tonic contrac- lying structural pathology. The hallmark of the typical tion of the elevator and depressor muscles of mastica- simple absence seizure is suppression of mental func- tion occurs equally and keeps the mouth open rigidly. tion, usually to the point of complete abolition of The extension tonic phase then begins with the mouth sponsiveness, awareness, and memory (Holmes et al opening wide and snapping shut, the arms and legs 1987). The seizures always begin abruptly, without an extending, and a prolonged steady expiration forcing aura or warning, and generally last a few seconds. air across the spasmodic glottis as the thoracic and Atypical absence seizures may last a minute or more. abdominal muscles contract. This can lead to apnea, Activity suddenly is interrupted, and the patient changes and the patient may not begin respiration again until facial expression and becomes transfixed with a mo- the postictal phase (Masland 1974). tionless, distant expression. The absence seizure ends The clonic phase consists of 30 sec of alternating suddenly, and the child returns to the gesture, sentence, rhythmic contractions and relaxations of the muscles, or other interrupted activity. Postictal impairment never appearing clinically as a succession of brief and violent occurs although the child may be confused momentarily, flexor spasms of the entire body. Tongue biting can due to time loss. This time loss mayserve as a clue to the occur. The jerks gradually subside until the end of the child that a seizure had occurred. seizure, as the period of muscular atonia becomes pro- In simple absence seizures, the child changes facial gressively longer (Farmer and Greenwood 1983). expression, stares and has a momentary impairment of Immediate postictal features lasting several seconds consciousness, while in complex seizures, a variety of to 4 rain include tonic contractions of the facial muscles other activities, such as, automatisms, myoclonus, and and muscles of mastication, producing an intense changes in postural tone occur. Complex absence sei- trismus and frothy saliva (Masland 1974). Postictal fea- zures are much more common than simple absence tures include complete atonia (flaccidity) and inconti- seizures (Penry et al. 1975). nence. The patient awakens confused and then usually Absence seizures may be precipitated by hyperven- falls into a deep sleep, emerging with a headache and tilation and photic stimulation. complete retrograde amnesia. Atypical Absences The onset of idiopathic primary generalized epilepsy is around 5 years of age, but generalized seizures caused Atypical absences differ from typical absences by a longer duration and more changes in postural tone. The by CNSdamage may have an earlier onset. Generalized children often have other seizure types in addition to tonic-clonic seizures with focal origin usually stem from the atypical absence seizures. These seizures are much cerebral damagedue to infection, birth injury, dysgenesis more difficult to treat than typical absences (Holmes et (defective embryonic development), metabolic and al. 1987). degenerative disease, or tumors (Bresnan and Konkol Someof these seizures can be associated with dete- 1987). rioration of intellectual performance and eventual No attempt should be made to move the patient who has a tonic-clonic seizure in the dental chair. The chair psychomotor retardation. Most children with atypical absences have the Lennox-Gastaut syndrome, charac- should be tilted back into the reclined position, and the terized by mental deterioration and a mixed seizure patient’s head placed to one side to prevent aspiration of saliva. Clothing around the neck should be loosened disorder (Gastaut et al. 1966; Markand1977; O’Donohoe 1979; Bresnan and Konkol 1987). These seizures are and oxygen administered if signs of anoxia occur. Al- difficult to control with medication. Ketogenic diet us- though tongue biting is a concern, more damage is ing medium chain triglycerides is tried sometimes caused by forcing objects between the teeth; this prac- (Dodsonet al. 1976). tice is not recommended. If one is cognizant of an The dental implication of absence seizures is the impending seizure, then a towel can be placed between need to reacclimate the child to the treatment being the dental arches at the beginning of the tonic phase, rendered after the seizure. when the mouth is open. If a patient bites on an in- strument or rubber dam clamp lodging between the Infantile Spasms teeth, wait until the seizure subsides before attempting West first described infantile spasms in 1841 as to remove the object. The general rule to follow if a massive myoclonic jerks beginning in infancy (West tonic-clonic seizure occurs is to prevent the factitional 1841). Infantile spasms can be divided into flexor, ex- injury (Brahamet al 1977). tensor, or mixed types. Flexor spasms are characterized Absence Seizures (Formerly Termed by sudden flexion of the trunk, neck, hips, and arms, Petit Mal Seizures) while in extensor spasms the legs and arms extend. In mixed spasms, elements of the flexor and extensor Absence seizures are generalized seizures that are spasms combine. Infantile spasms are brief, lasting 1-5 nonconvulsive and usually not associated with under-

130 PEDIATRICDENTISTRY: MAY/JUNE, 1991~ VOLUME13, NUMBER3 sec, and usually occur in clusters. While the clusters (Emerson 1981), but age of onset appears to be typically consist of 10-15 spasms over several minutes, important factor in determining the frequency of recur- some infants can have a hundred or more spasms in a rences. Fifty per cent of children whohave a seizure by cluster. The untreated child usually has multiple clus- one year of age will have a another one (Dreifuss 1983; ters daily. Infantile spasms are characterized by recur- Weineret al 1988). ring attacks and often associated with mental retarda- The goal of treatment is to prevent recurrence of tion and hypsarrhythmia (chaotic rhythm EEG, Lacy febrile seizures. Typical managementof a febrile seizure and Penry 1976). This triad of spasms, retardation, and includes antipyretics to decrease temperature and fluid hypsarrhythmia is known as West syndrome. replacement therapy if the patient is dehydrated. Most Infantile spasms may be symptomatic (occurring in febrile seizures do not need medication, but infants with a history of CNSdefects and disorders of anticonvulsant therapy is considered if there is an ab- prenatal, perinatal, and postnatal origin) or classified as normal neurological exam, prolonged focal seizure cryptogenic (occurring in infants without neurological (greater than 15 min), or association with a neurological symptoms). Ultimately, 90%of all children with infan- deficit. Phenobarbital taken daily prevents recurrent tile spasms become mentally retarded and usually have febrile seizures. However, it has been demonstrated a mixed seizure disorder of myoclonic-akinetic and recently that phenobarbital lowers the IQ in children other generalized seizures (Weiner et al. 1988). treated for febrile seizures, and is ineffective in pre- It is generally thought that early diagnosis and venting recurrences of febrile or afebrile seizures treatment leads to a better prognosis. A normal infant (Farwell et al. 1990). with a normal head size who begins having spasms has Status Epilepticus the best prognosis. Although the incidence is low, 0.24/ Most investigators define status epilepticus as con- 1000 live births (Nelson 1972), an early diagnosis tinued seizure activity lasting longer than 30 min and extremely important, as prompt treatment with intra- resulting in a fixed epileptic condition. Status epilepticus muscular long acting adrenocorticotropic hormone is an emergencysituation, regardless of its clinical form. (ACTH) may prevent permanent encephalopathy, se- If it lasts untreated for an hour, the patient may never vere retardation, and generalized seizures (Gastaut regain consciousness (Gastaut 1970; Dodsonet al. 1976; 1974). Infantile spasms continue to be one of the more Abramawicz 1983). Diazepam, diazepam with simul- resistant forms of epilepsy to antiepileptic therapy. taneous administration of phenytoin, or phenobarbital Febrile Convulsions are first-line medications in treatment. Febrile convulsions are seizures that occur with fever and infections. Within the first 5 years of life, 7%of all Partial Seizures children will have a seizure, and 50%or more of these Simple Partial Seizures will be febrile seizures (Bresnan and Konkol 1987). Partial seizures with simple symptoms(focal seizures) These seizures typically occur between 6-24 months of consist of motor movements or sensory phenomena age. Ninety per cent of these seizures are generalized, of limited to one limb, or they may involve both limbs on short duration (15 min or less) and predominantly tonic. one side of the body (Dreifuss 1983). There may be As the temperature exceeds 102°F (39°C), there is spread of motor activity (jacksonian march). increased risk for convulsion. A positive family history jacksonian seizure may begin, for example, with of convulsions and genetic studies suggest autosomal twitching of the thumb followed by twitching of the dominant inheritance with incomplete penetrance (Bresnan and Konkol 1987). hand, forearm, upper arm, and shoulder. The most commoncauses are focal damage(cerebral palsy), tumor, Three factors increase the risk of subsequently de- arteriovenous malformation, and abscess. In neonates veloping epilepsy after febrile seizures: and infants, metabolic seizures, may occasionally be 1. Duration longer than 15 min, focal in character, focal (Hoekelmanet al. 1987). or more than one seizure in 24 hr ComplexPartial Seizures 2. Preexisting neurological abnormality 3. Family history of afebrile seizures (epilepsy in This focal seizure disorder may or may not be sec- parents or siblings). ondary to a lesion in the temporal lobe. Complexpartial seizures frequently begin in the temporal lobe, but may Twoof these risk factors present a 10% chance of start in frontal, occipital, or other cortical regions. Causes developing epilepsy. The recurrence of simple febrile include birth trauma, prolonged febrile convulsions, seizures also increases the risk of subsequent afebrile tumor, and hamartomas. Partial seizures as a part of a seizures to 4%(Nelson and Ellenberg 1978). The overall mixed seizure disorder secondary to a diffuse fixed recurrence rate of simple febrile seizures is 30-40% encephalopathy are probably the most common. There

PEDIATRICDENTISTRY: MAY/JUNE, 1991- VOLUME13, NUMBER3 131 frequently is an aura, often described by children as Table3. Commonetiologies of neonatalseizures "funny feelings," fearful feelings, "butterflies," or ab- Day 1 dominal pain. The seizure may take the form of Hypoxia automatisms, automatic movements and tasks such as Hypoglycemia swallowing or lip smacking, a repetitive motion with the hands (picking at one’s clothes), behavior abnor- Hyperglycemia malities (walking to another part of the room), or lack Drug withdrawal (from mother) responsiveness. Amnesia, confusion, and drowsiness Pyridoxine dependency accompany the episodes. Complex partial seizures may Infection (meningitis, sepsis, TORCH) becomegeneralized to a tonic-clonic seizure (Weiner et Trauma, severe al. 1988; Bresnan and Konkol 1987). Days 2-3 Benign Rolandi¢ Epilepsy Simple partial seizures are unusual in childhood. Infection (sepsis, meningitis) One notable exception is Benign Rolandic Epilepsy Drug withdrawal (BRE), also knownas Sylvian seizures and midtemporal Developmental malformations spikes, or benign epilepsy of childhood with Intracranial hemorrhage centrotemporal EEGfoci. These seizures tend to begin Metabolic disorders (urea cycle, hyperglycinemia) late in childhood and disappear at or just after adolescence. BRE makes up 16-24% of childhood seizures Hyperglycemia (Cavazzuti 1980). They are quite recognizable by the Hypoglycemia clinical characteristics of onset during sleep of brief Hyponatremia or hypernatremia facial clonus, salivary pooling, and anarthria (loss of Day 4 on power of articulate speech). It has been concluded that BREis the result of autosomal dominant inheritance Developmental malformations with an age-dependent penetrance. Almost all children Hypocalcemiaand hyperphosphatemia (dietary) outgrow these seizures with age (Bresnan and Konkol Hyponatremia 1987; Farmer and Greenwood 1983). Metabolic(urea cycle or aminoacid disorders, Neonatal Seizures hyperglycinemia) Neonatal seizures are a major prognostic indicator of Infection (sepsis, meningitis, herpes simplex) children who will later have both severe mental retar- Drug withdrawal dation and cerebral palsy (Nelson and Broman 1977). Seizures with an onset during the neonatal period have different characteristics, causes, treatment, and prog- survived following neonatal seizures were develop- nosis than seizures that occur in an older infant. This is mentally normal at age 7 years (Myers and Cassady related to the immaturity of myelination and synaptic 1983; Volpe 1977). development. Seizures are seen in fewer than 1%of full- term and in more than 5% of premature neonates, and Diagnostic Aids for the Pediatric Dentist are the most commonmanifestation of neurologic dif- To Evaluate Patients With Seizures ficulties during the neonatal period. These seizures are related to underlying abnormalities as listed in Table 3. Physical Exam These seizures are confusing because of difficulty in The pediatric dentist rarely has the opportunity to distinguishing them from jitteriness. Jitteriness differs observe a seizure at the time of onset; so a careful from seizures by being stimulus sensitive, ceasing when history is important. Included in the review of past and the extremity is held, and not being associated with present medical history should be a discussion con- abnormal eye movements. The most effective treatment cerning family history of seizures, complications at of neonatal seizures is prevention. Prenatal and birth, prewarning signs of seizures (auras) experienced, intrapartum monitoring and appropriate resuscitation the manner in which seizure progresses, whether con- following delivery may prevent brain damage. Once sciousness is lost, postictal behavior, the age seizures seizures start, intravenous solutions are started to cor- began, how often seizures occurs, whether seizures rect any metabolic disorder. If anticonvulsants are have changed in character, the medication currently necessary, phenobarbital is the drug of choice in the used, and its effectiveness. neonate. The prognosis is good. In the follow-up of the If the child has no seizure history and has one seizure Collaborative Perinatal Project, 70% of the 181 who in the dental office, the child should be examined by a

132 PEDIATRICDENTISTRY: MAY/JUN G 1991N VOLUME13, NUMBER3 physician following the seizure. The physical examina- Pharmacotherapy tion should include a thorough neurological exam and Children are not placed on antiepileptic drugs until evaluation for injury. These children often will require they have two or more unprovoked seizures. Frequently, an electroencephalogram (EEG), and the pediatric a child will only have a single seizure, and treatment dentist should understand its usefulness. The EEGis a with potentially harmful drugs is not required. Some diagnostic aid for patients with suspected severe seizure neurologists do not treat children with more than two disorders because it frequently shows evidence of a seizures if they are brief, non life-threatening, and occur paroxysmal electrical dysrhythmia (Farmer and at a frequency of less than two per year. The chief reason Greenwood 1983). The EEG can reveal the nature and for treating a child after a second unprovokedseizure is location of brain cell overactivity, identify structural to prevent the associated emotional and physical injury. abnormality, provide clues as to the clinical seizure Children who have frequent or prolonged seizures re- type, and may predict recurrences (1985). A normal quire anticonvulsant therapy. EEGdoes not rule out epilepsy, and many children with The managementof the child with a seizure disorder a normal waking EEGcan have an abnormality induced can best be summarized by "attempt to cause no harm." by hyperventilation, photic stimulation, or a spontane- Anticonvulsant therapy should preserve vital functions ous arousal during natural sleep or sedation. Almost all while controlling seizure activity. If possible, treatment children, with the exception of children with typical of the underlying disease is a goal of therapy while absence seizures, will have computerized tomography preventing damageto the brain. Therapeutic approaches (CT) or magnetic resonance imaging (MRI) scans to convulsive seizures are summarizedin Table 4. In the tained. The "yield" of the MRI over CT is about 10%, majority of the children with seizure disorders, no makingit a superior test that eventually will replace the specific cause is found. Difficulties of drug treatment CT. include poor prognosis, prolonged multidrug treatment,

Table4. Therapeuticapproaches to convulsive seizures

Major Drug Typeof Sefzure ToxicSide Effects Doses DrugInteractions of Interest to the PediatricDentist

Carbamazepine partial seizures Leu.kopenia _< 8 years 100 mgBID Erythromycinwill elevate (Tegretol) hepatic dysfunction or TID tegretol levels and thus its use (drug of choice) rashes v > 8 years 100-200mg must be monitoredcarefullv. TID or QID Therapeutic levels in 15--30hr. Valproic acid absence mal hepatotoxicity 15-60 mg/kg/day Valproic acid maycause (Depakote) pancreatitis medication administered prolonged bleeding times. every6-12 hr to ff~aintain blood levels. Therapeuticlevels in 6-15 hr. Clonazepam atypical absences behavioral ~hanges < 10 years (30 kg) Do not stop Clonazepam (Klonopin) infantile spasms hyperactivity, 0.05 mg/kg/day then therapy suddenly or status irritability, aggressive increased by 0.25 to 0.5 epilepticus maybe precipitated. behavior in 15%of mg/kg/day to a patients. Thickspeech, maximumof 0.2 mg/kg/ salivation, and day in 3 divided doses. bronchial hypersecretion Phenobarbital febrile seizures behavioral side 30 mg BID(children) Sedatives and phenobarbital can (although effects mayinterfere 60 mg BID(teenagers) lead to over-sedation. Also true rarely treated) with cognition Therapeuticlevels in for primidone(mysoline). hyperactivity 1-3 weeks. Phenytoin tonic-clonic maculo pappular 6 mg/kg/day up to Increasedincidence of cleft lip/ (Dilantin) or focal seizures rash, Stevens- 30kg in children palate in children of mothers Johnson syndrome, 200-300 mg/day in taking phenytoin. hirsutism, gingival teenagers hypertrophy Therapeuticlevels in

PEDIATRICDENTISTRY: MAY/JUNE, 1991~ VOLUME13, NUMBER3 133 chronic toxicity, and uncertainty of the relative efficacy Primidone is a drug which must be introduced in and toxicity of individual anticonvulsants. Compliance small increments to avoid oversedation. It is felt that is a major problem. Because of the number of drugs primidone has little to offer over phenobarbital alone. prescribed, their unwanted side effects, and the pro- Primidone is metabolized to a large extent to longed treatment, some patients omit some or all of phenobarbital; thus, the combination of barbiturates their medications (Reynolds 1978). and primidone can lead to oversedation. Diazepam Numerousanticonvulsant medications are available, plus primidone may also cause oversedation (Weiner et but multidrug regimens (polypharmacy) are usually al. 1988). avoided if possible. In children, carbamazepine is the Ethosuximide is the drug of choice for absence sei- current drug of choice of pediatric neurologists for zures. A lupus-like syndrome can occur as a toxic side partial seizures and valproic acid for primarily general- effect. (Bresnan and Konkol 1987; Weiner et al. 1988). ized seizures. Acetazolamide is a useful adjunct medication for Phenobarbital is used widely by pediatricians. absence and myoclonic seizures, and occasionally in Phenobarbital is no longer the first drug used in the generalized tonic-clonic seizures. ACTHis a useful treatment of seizures in children by most neurologists adjunct medication for infantile spasms. although pediatricians, by habit, continue to use A ketogenic diet has been advocated to control phenobarbital as their drug of choice. Although safe, myoclonic, tonic, atonic, atypical absence, and gener- phenobarbitol’s main disadvantages are behavioral side alized tonic-clonic seizures. This diet is designed to effects and interference with cognition (Farwell 1990). produce ketosis with a high fat intake, thus raising the Phenytoin is another effective anticonvulsant. A convulsive threshold (Dodson et alo 1976). maculopapular rash (resembling measles) is a side effect In general, children who have not had a seizure for which may lead to Stevens-Johnson syndrome if medi- three to four years, whoare of normal intelligence, and cation continues. Other side effects include hirsutism, who have normal findings on EEGand neurologic ex- gingival hypertrophy, blood dyscrasias secondary to amination have a 74%chance of being free of seizures bone marrow toxicity, megaloblastic anemia (folate with discontinuation of medication. Fifty per cent of deficiency), and rickets or osteomalacia (low calcium relapses after discontinuation of medication are likely and high alkaline phosphatase). Although nystagmus to occur within three months, and 80%within one year on lateral gaze is a good clinical sign in teenagers that (Emersonet al. 1981). they are taking the medication, it is not present with It is important for the pediatric dentist to be aware of regularity in children. Ataxia of gait is a common lidocaine-induced seizures. Seizures from oral viscous manifestation of toxicity in all age groups (Weiner et al. lidocaine prescribed for herpetic gingivostomatitis are 1988). The method of action of phenytoin is probably documented (Hess and Walson 1988). As well, toxic related to preventing the spread of seizure activity doses of lidocaine given for dental procedures can in- rather than abolishing the primary focus of seizure duce seizure activity (Hellstr6m-Westas et al. 1988). discharges (Farmer and Greenwood 1983). Carbamazepine is becoming increasingly popular Social Aspects for partial seizures and generalized seizures because of The majority of people with epilepsy are intelligent, its lack of side effects. Toxic side effects include and historically have included great political leaders, leukopenia, hepatic dysfunction and rashes. The pedi- artists, musicians, and scientists. Patients with seizures atric dentist should be aware that erythromycin will encompass a variety of people; ranging from successful elevate carbamazepine levels (Weiner et al. 1988). businesspeople, to wheelchair-bound people with Valproic acid is most effective in absence, myoclonic, mental retardation and quadriplegi .ao However,all share tonic, atonic and primary generalized tonic-clonic sei- the commonfear of an unpredictable seizure ending zures. The pediatric dentist should be aware that valproic with public embarrassment and humiliation acid may lead to bleeding abnormalities (Browne 1980; (Niedermeyer 1990). Bresnan and Konkol 1987; Weiner et al. 1988). Epilepsy is a commondisorder, but patients have Clonazepam is primarily used in atypical absences great difficulty being accepted in schools, communities, and infantile spasms. Hyperactivity, irritability, and and social activities. Their problems of acceptance can aggressive behavior occur in about 15% of patients. be attributed to ignorance, false illusions of what seizures Other toxic symptomsinclude thick speech, salivation, are, and superstitions. "Epilepsy" is derived from the and bronchial hypersecretion. It is important that the Greek word epilambanein: to take, to seize. In other pediatric dentist does not suddenly stop clonazepam words, an evil spirit "takes over" or "seizes" the patient therapy as status epilepticus may be precipitated (Niedermeyer 1990). (Browne 1978; Weiner et al. 1988). In the past, people with epilepsy were institutional-

134 PEDIATRICDENTISTRY: MAY/JUNE, 1991~ VOLUME13, NUMBER3 ized and labelled mentally retarded, but today, they Epilepsy: An Overview of Legal Issues, Federal Laws, and State cannot be hospitalized against their will merely because Laws Affecting Persons with Epilepsy. 5th ed. Landover, MD, 1987, pp 1-89. they have epilepsy. People with epilepsy are still dis- Farmer TW, Greenwood RS: Paroxysmal disorders, in Pediatric criminated against. Obtaining health, life, or automobile Neurology 3rd ed. Farmer TWed. Philadelphia: Harper and Row, insurance continues to be a major problem, and if cov- 1983, pp 205-63. erage is available at all, the premiumsare too high for Farwell JR, Lee YJ, Hirtz DG, Sulzbacher SI, Ellenberg JH, Nelson KB: Phenobarbital for febrile seizures -- effects on intelligence and on most people to afford. Driving restrictions for these seizure recurrence. N Engl J Med322:364-9, 1990. patients vary from state to state but most states require Gastaut H: Clinical and electroencephalographical classification of medical verification of seizure control and usually re- epileptic seizures. Epilepsia 11:102-19, 1970. quire a seizure-free period ranging from six months to Gastaut H, Broughton R, Roger J, Tassinari CA: Generalized convulsive seizures without local onset, in Magnus O, Lorentz de Haas Am two years. Somepeople deny their condition and do not eds: Handbookof Clinical Neurology, Vol. 15. The epilepsies. seek medical care (Epilepsy Foundation 1987). NewYork: American Elsevier Publishing, 1974, pp 107-29. At present, few laws protect patients with epilepsy Gastaut H, Roger H, Soulayrol R et al: Childhood epilepsia against employment discrimination. The Rehabilitation emphalopathy with diffuse slow spike waves (otherwise known as "petit mal variant") or Lennox syndrome. Epilepsia 7:139-79, Act of 1973 only requires an employer who is a federal 1966. agency, or contracts with the federal government for Hauser WA:Evaluation of the newly identified seizure patient. New over $2500,or is a recipient of federal financial assistance York: Columbia University, 1985. not to discriminate against people with seizures. The Hellstr6m-Westas L, Westgren U, Ros6n I, Svenningsen NW:Lidocaine for treatment of severe seizures in newborn infants. I. Clinical accident rate for people with epilepsy in industry is as effects and cerebral electrical activity monitoring. Acta Paediatr low as that of other employees, but few corporations or Scand 77:79-84, 1988. companies appreciate this data (Epilepsy Foundation Hess GP, Walson PD: Seizures secondary to oral viscous lidocaine. 1987). Education of the public and participation in public Ann Emerg Med 17: 725-27, 1988. Holmes GL, McKeever M, Adamson M: Absence seizures in children: activities by those with seizure disorders can change clinical and electroencephalographic features. Ann Neuro121:268- the misconceptions and attitudes toward the disorder. 73, 1987. Lacy JR, Penry JK: Infantile spasms. NewYork: Raven Press, 1976, pp Drs. Nelson and Ureles are associates in pediatric dentistry, Children’s 1-124. Hospital, and clinical instructors in pediatric dentistry, Harvard School Markand ON: Slow spike-wave activity in EEGand associated clini- of Dental Medicine, Boston, MA. Dr. Holmes is director, Neuro- cal features: often called ’Lennox’ or ’Lennox-Gastaut’ syndrome. physiology Laboratory, Children’s Hospital and associate professor Neurology 27:746-57, 1977. of neurology, Harvard School of Medicine, Boston, MA. Reprint MaslandRL: The classification of the epilepsies: a historical review. In requests should be sent to Dr. Linda P. Nelson, Dental Division, Magnus O, Lorentz de Haas Ameds. Handbook of Clinical Neu- Children’s Hospital, 300 LongwoodAve., Boston, MA02115. rology, Vol. 5, The Epilepsies. NewYork: American Elsevier Publishing, 1974, pp 1-24. Abramowicz M: Drugs for epilepsy. Med Let Drugs Ther 25:81-84, Myers GJ, Cassady G: Neonatal seizures. Pediatr in Rev 5:67-72,1983. 1983. Niedermeyer E: The Epilepsies: Diagnosis and Management. Balti- Braham RL, Casamassimo PS, NowakAJ, Posnick WR, Steinberg AD: more, MD:Urban and Schwarzenberg, Inc, 1990, pp 1-7. The dental implications of epilepsy. US Department of Health, Nelson KB: Discussion, Alter M, Hauser WAeds. In The Epidemiol- Education, and Welfare. Rockville, MD: DHEWPublication No. ogy of Epilepsy: A Workshop NINDS monograph, No. 14. (HSA) 78-5217, 1977, pp 1-13. Washington, DC: U.S. Government Printing Office, 1972. Bresnan MJ, Konkol RJ: Seizure disorders, in Primary Pediatric Care. Nelson KB, BromanSH: Perinatal risk factors in children with serious Hoekelman RA, Blatman S, Friedman SB, Nelson NM, Seidel HM motor and mental handicaps. Ann Neurol 2:371-77, 1977. eds. St. Louis: CVMosby Co, 1987, pp 1467-77. Nelson KB, Ellenberg JH: Prognosis in children with febrile seizures. Browne TR: Clonazepam. N Engl J Med 299:812-16, 1978. Pediatrics 61:720-27, 1978. BrowneTR: Valproic acid. N Engl J Med 302:661-65, 1980. O’DonohoeNV: Epilepsies of childhood. London: Butterworths, 1979. Dodson WE, Prensky AL, DeVivo DC, Goldring S, Dodge PR: Man- Penry JK, Dean JC, Riela AR: Juvenile myoclonic epilepsy: Long-term agement of seizure disorders: selected aspects. Part I. J Pediatr response to therapy. Epilepsia 30:(Suppl 4) $19-27, 1975. 89:527-40, 1976. Reynolds EH: Drug treatment of epilepsy. Lancet 2:721-25, 1978. Dreifuss FE: Pediatric Epileptology: Classification and Management Vernadakis A, Woodbury DM: Maturational factors in development of Seizures in the Child. Boston: John Wright, PSG, Inc, 1983, pp of seizures. In Jasper HH, Ward AA, Pope A eds. Basic Mechanisms 129-44. of Epilepsies. Boston: Little, Brownand Co, 1969, pp 535-41. Dreifuss, FE: Seizure disorders. Classification of epileptic seizures Volpe JJ: Neonatal seizures. Clin Perinatol 4:43~3, 1977. and the epilepsies. Pediatr Clin North Am36(2):265-79, 1989. Weiner HL, Urion DK, Levitt LP: Pediatric Neurology for the House Emerson R, D’Souza BJ, Vining EP, Holden KR, Mellits ED, Freeman Officer, 3rd ed. Baltimore, MD:Williams and Eilkens, 1988, pp 33- JM: Stopping medication in children with epilepsy. N Engl J Med 61. 304:1125-29, 1981. West WJ: On a peculiar form of infantile convulsions. Lancet 1:724-25, Epilepsy Foundation of America. The Legal Rights of Persons with 1841.

PEDIATRICDENTISTRY: MAY/JUNE, 1991 - VOLUME13, NUMBER3 135