One of the most dramatic medical emergencies that confronts the pediatrician is the convulsing child. In this situation, etiology should be thoroughly understood before treatment is considered. And it is imperative that the physician develop a well-organized diagnostic and therapeutic approach to the problem. Table 1 provides an outline that we have found successful.
The first step in managing the convulsing patient is to ensure that the airway is adequate and the patient is not in shock. We then ask everyone to step back. An understanding of what may cause a seizure allows specific questions to be asked and a pertinent physical examination to be performed quickly so that emergency action that might prevent a catastrophe can be taken.
ACUTE MANAGEMENT OF SEIZURES
Table 2 categorizes the causes of seizures under seven general headings. The immediate history should determine whether trauma has occurred. A child with convulsions secondary to head trauma might show skin manifestations of such trauma. Further, retinal hemorrhages should lead to the diagnosis of subdural or subarachnoid hemorrhage; nuchal rigidity should be found in the latter. Obviously, neurosurgical intervention has to be considered in these cases. Observation of eye deviation is extremely important, as this may be the only manifestation of a focal lesion, deviation to the right suggesting a left-sided focus. The pupils are usually dilated while the child is convulsing; asymmetry of pupil size will occasionally be noted. However, this does not necessarily establish the existence of an expanding intracranial lesion.1 Pupillary constriction, on the other hand, would suggest a narcotic overdose.
ETIOLOGY OF SEIZURES
The presence of fever should arouse suspicions of bacterial meningitis. While the seizure is being stopped, a temperature should be obtained and preparations made for a spinal tap. This article will not discuss the controversies that surround the diagnosis and management of the simple febrile convulsion.
Metabolic causes of a convulsion also require immediate and very specific action. This category of seizure can also be suspected on the basis of a brief history and rapidly performed physical examination. Questions regarding pre-existing illnesses are very helpful, as seizures may occur in patients with chronic renal disease. A pale-appearing child with a cold sweat should arouse suspicions of hypoglycemia. The presence of sunken eyes would suggest dehydration.2 Blood should be drawn for sugar, electrolyte, calcium, and urea nitrogen analysis before initiation of anticonvulsant treatment. In fact, some recommend routine intravenous administration of glucose before treatment with anticonvulsants.
Toxic causes of seizures do not require immediate, specific action, although stomach aspiration should be considered. However, an appropriate history and laboratory studies should be obtained to determine the presence of lead intoxication (pica, flat plate of the abdomen, blood lead level). Ferric chloride testing of the urine is an adequate screening test for phenothiazine intoxication. Thallium is used in rat poison. Usually, there will be a history of vomiting for a few days before the convulsion. The treatment of camphor-induced seizures is similar to that for other central nervous system stimulantinduced seizures - namely, with anticonvulsants.
Vascular lesions constitute the fifth general category in our etiologic approach to the convulsing child. Unless nuchal rigidity is present, suggesting subarachnoid bleeding, symptomatic treatment is provided in advance of a more definitive evaluation. Sickle cell disease must be considered in a black child.3
Structural lesions - such as porencephalic cysts, agenesis of the corpus callosum, microcephaly, and brain tumors - have been associated with convulsions. The initial approach consists of stopping the seizure; this is followed by the more definitive evaluation.
Genetic epilepsy, on the other hand, is characterized by the absence of a demonstrable structural or metabolic problem and implies that an inherited hyperexcitable population of neuron exists. Metrakos and Metrakos4 have shown that genetic epilepsy most commonly occurs between the ages of five and 12.
Table 3 outlines the most likely causes of seizures, depending on the age of the child. During the first two years of life, metabolic, infectious, and congenital lesions are the most common causes of seizures. During childhood, trauma, toxins, and genetic predispositions are the most likely causes. Acute or chronic renal disease is a rare cause of seizure, but it must be considered. The most common causes of convulsions in the adolescent age group are trauma, genetic predisposition, and occasionally brain tumors.
The specific causes of seizures that require immediate action have been considered. Diazepam (Valium®), 0.1-0.3 mg./kg. administered intravenously over a two-to-three-minute period, is now given. This can be repeated 20 minutes later. Once the seizure has been terminated, an intramuscular injection of phenobarbital, 5 mg./kg., is then given, as the effect of Valium® lasts no more than 30 to 45 minutes. Potential respiratory distress must be anticipated at all times.
A detailed history, with special attention to potential causes, can now be obtained. Not only should the causes outlined in Table 2 again be considered, but a history of personality change over the past several months or a drop in school marks should alert the physician to the possibility of degenerative brain disease, such as subacute sclerosing panencephalitis.
The physical examination most commonly reveals a normal child. However, one should look for some very specific abnormalities. Head circumference should be a routine part of the evaluation, and cranial bruits should be listened for. An examination of the skin might reveal a portwine stain of the face (Sturge-Weber disease), depigmented spots on the trunk (tuberous sclerosis), or cafe-aulait spots (neurofibromatosis),
The EEG is absolutely necessary as part of the total evaluation. Frequently, the EEG will help differentiate between petit mal and psychomotor epilepsy. Further, the EEG can provide clues as to whether there is a metabolic basis for the problem or whether a degenerative brain disease might be present. It is uncommon for a brain tumor to manifest itself as a cause of seizures in children; the possibility certainly exists, however, and an EEG is a valuable screening test for this diagnosis. Finally, it must be realized that an abnormal EEG does noi establish the diagnosis of epilepsy unless the EEG happens to be obtained during the actual seizure.
Unless an infection, such as encephalitis or meningitis, is suspected, a spinal tap is not a routine part of the evaluation in our hands.
Skull films are routinely obtained in all children with a diagnosis of epilepsy. This is the only way calcifications can be found that might lead to a more specific diagnosis.
ETIOLOGY OF SEIZURES BY AGE
Technetium scanning is not routinely ordered unless the seizure had a focal onset or was focal in nature or the EEG shows a definite focus.5
CONVULSION NOT WITNESSED BY THE PHYSICIAN
Unfortunately, more often than not, the physician does not have the opportunity to witness the actual convulsion. In this case, the problem is not only to determine the cause but also to decide whether the paroxysmal event was, in fact, a seizure that might be recurrent or another type of paroxysmal behavior that would not require anticonvulsant therapy. A carefully obtained history is the only way to establish the diagnosis of a seizure under these circumstances. The description of the seizure should be obtained in such detail that one would conclude that the physician was the actual witness.
Before discussing the questions to ask, it is necessary to establish a working definition of the term "seizure" and to describe the different types of seizures.
The term seizure refers to a sudden onset of abnormal behavior resulting from an excessive discharge of a hyperexcitable neuronal population.6 This behavior should be disorderly and transient. Other manifestations of a seizure include some, but not all, of the following:
1. Loss of or derangement in consciousness
2. Excess or loss of muscle tone or movement
3. Alteration of sensations
4. Alterations of autonomie nervous system function, including paleness, sweating, etc.
5. Emotional changes, including fright, anger, and aggression.
As it is commonly understood, the term epilepsy refers to chronic, recurrent seizures; the term convulsion is synonymous with grand mal epilepsy.
Table 4 lists the five types of sei' zures. Grand mal epilepsy usually starts with an aura. The aura most frequently consists of dizziness and/or abdominal pains. If the patient is of an appropriate age, he should be asked about unusual sounds, smells, visions, or feelings prior to "passing out." The ictus follows shortly and usually starts with the tonic phase, which consists of increased tone of the extensor muscles of the four extremities as well as the neck muscles. This is followed by the clonic phase: contraction of the antigravity muscles, the biceps of the upper extremities, and the hamstrings of the lower extremities causes the patient to go into flexion. Commonly, with muscle relaxion, there is incontinence of urine or stool. After the patient goes through a series of tonic and clonic phases, fatigue occurs. The fatigue or postictal state can last from five minutes to several hours.
Focal seizures may be sensory or motor (Jacksonian), depending on the location of the abnormal discharge. Focal epilepsy is considered in a separate category, as a focal onset to a seizure should alert the physician to the possibility of an arteriovenous malformation or brain tumor.
CLINICAL CLASSIFICATION OF SEIZURES
Psychomotor epilepsy is the third clinical category.7 Clinically, this is one of the most difficult types of epilepsies to diagnose. If one approaches it according to the schema outlined for grand mal seizures, it can be more readily understood. The aura for the psychomotor seizures is quite varied but does not differ significantly from that of grand mal seizures. It precedes the seizure by a few seconds to minutes. The ictus, on the other hand, is quite complex. There are sensory, psychic, and motor phenomena, although not all three are necessarily present. The seizure may consist merely of a feeling of numbness or paresthesia in the extremities. Psychic symptoms include hallucinations, partial or complete loss of awareness, and feelings of depression, anger, and fear. The motor component may consist entirely of purposeless movements of the extremities. The postictal state includes complaints of headache, fatigue, and hunger.
Petit mal epilepsy is the fourth clinical type. This is an uncommon type of epilepsy, occurring in only 5 per cent of children with epilepsy. Further, petit mal does not occur under the age of three.8 The attacks start suddenly and without warning. The episodes last less than 15 seconds. The patients are unaware of their environment during the episodes, and the attacks stop suddenly. Occasionally, purposeless motor movements of the extremities and face are noted.
Finally, minor motor seizures, the most difficult type of seizure to control, are the fifth clinical type. These can be further subdivided into akinetic seizures, which consist of loss of muscle tone (drop attacks), and myoclonic seizures, which consist of a sudden jerk of muscle groups (head drop, unilateral or bilateral extremity, or body flexion or extension). These seizures are usually observed in the younger child and very rarely occur after age 12.
As has already been stated, the history is the only way to establish the diagnosis. The history must be obtained from the person who observed the actual seizures. This may mean contacting a child's schoolteacher or speaking to a sibling or a peer who observed the episode. The parent must be interviewed, and the child should not be neglected during this aspect of the diagnostic process.
Just as a seizure can be divided into the aura, ictus, and postictal phases, so the history should approach the problem with questions regarding each of these stages. Precipitating factors - such as fatigue, emotional upset, and nonspecific illnesses - should be determined, as seizure treatment might be altered depending on the presence of one of these variables.9
When the history is taken, the differential diagnosis of unexplained episodic behavior must be considered (Table 5). Hysterical "seizures" can sometimes be very difficult to differentiate from grand mal or psychomotor seizures. The aura of hysterical "seizures" may consist of choking; consciousness is impaired but not lost. The motor movements are commonly bizarre rather than of the typical tonic-clonic type. The patient almost never hurts himself, and incontinence rarely occurs. The hysterical "seizure" usually stops almost as rapidly as it starts, with no postictal state; however, the patient will occasionally appear or act fatigued.
DIFFERENTIAL DIAGNOSIS OF PAROXYSMAL BEHAVIOR
Syncope (fainting spells) must also be considered in the differential diagnosis of epilepsy. Syncopal attacks almost invariably occur in the upright position. The "aura" commonly consists of vertigo or Iightheadedness. During the attack, the patient may develop brief tonicclonic movements, a result of transient cerebral anoxia. The postictal symptoms are minimal and consist of irritability or fright. In my experience, many children use the term "dizziness" when, in fact, they mean light-headedne ss .
Breath holding10 also has to be considered in the differential diagnosis of paroxysmal behavior with a loss of consciousness. These attacks always occur following a cry or a fright. In association with the apnea and perioral cyanosis, some clonic-tonic movements as well as opisthotonic posturing may be observed. Occasionally, pallor rather than cyanosis will be noted. Postictal symptoms, which may consist of irritability and fatigue, are short-lived.
Abnormal behavior during sleep is commonly confused with epilepsy.11 Night terrors consist of unexplained screaming, staring, and incomprehensive rambling associated with bizarre motor movements. The patient will have no recollection of the episode the following morning. Also, the patient may sleep-walk as part of the attack.
Migraine headaches are common in children and can be very difficult to differentiate from epilepsy. The major symptom in migraine is headache. The migraine phenomenon also consists of an aura that commonly comprises visual symptoms. Headache then occurs, followed by nausea, vomiting, and sleep. In epilepsy, the headache usually occurs when the patient awakens from the postictal state. However, a sudden or transient headache may be the only manifestation of epilepsy. If the history does not allow one to differentiate between these two entities, a trial of anticonvulsants may be the only alternative.
IMPORTANCE OF FOLLOW-UP CARE
Once the diagnosis of epilepsy has been established, a prescription is written. This should not be the end of the consultation, however. Whitehouse12 found that approximately 56 per cent of children with epilepsy had learning problems. Rodin et al.13 have established that it is very difficult for some people with epilepsy to obtain employment. This difficulty relates not to the frequency, severity, or type of epilepsy but, rather, to learning problems experienced by the child when in school. It has also been noted that the attitudes of school personnel are still archaic, suggesting that the physician should have some school contact once the diagnosis has been established.14
Epilepsy is a symptom and not a disease. An etiologjc and therapeutic approach to the acutely convulsing child has been outlined. The necessity of attempting to determine the cause while in the process of stopping the seizure has been emphasized. Suggestions as to what to evaluate on physical examination and what laboratory tests to order have been discussed.
An approach to the child whose seizure was not witnessed by a physician is also presented. Emphasis is placed on obtaining an accurate history. Finally, the treatment of a child with epilepsy involves more than prescribing appropriate medication. The child's performance in school and the concerns of parents and patient must be considered.
1. Parrt, S.S., Benton, J, W., and Dodge, P. R, Unilateral pupillary dilatation during and immediately following seizures. Neurology 17 (1967), 837.
2. Reynolds, E. H. Water, electrolytes and epilepsy. J. Neurol. Scl. Ti (1970). 327.
3. Portnoy. B. A., and Herion, J. C. Neurological manifestations in sickle cell disease. Ann, int. Med. 76 (1972), 643.
4. Metrakos. J. D.. and Metrakos, K. Genetics of convulsive disorders. Neurology 10 (1960), 228.
5. Yalaz. K., and Trêves, S. Brain scanning and cerebral radioisótopo angiography in children. Pediatrics 54(1974), 696.
6. Lennox, W. G. Epilepsy and Related Disorders. Boston: Little, Brown & Company. 1960.
7. Gold, A. P. Psychomotor epilepsy in childhood. Pediatrics 53 (1974), 540.
8. Charlton, M. H., and Yahr, M. D. Long-term follow-up ol patients with petit mai. Arch. Neuroi. 16 (1967), 595.
9. Sibley, W. A. Diagnosis and treatment of epilepsy: Overview and general principles. Pediatrics 53 (1 974), 531.
10. Lombroso, C. T., and Lerman, P. Breathholding spells (cyanotic and pallid infantile syncope). Ped/airics 39 (1967), 563.
11. Anders, T, F., and Weinstein, P. Sleep and its disorders in infants and children: A review. Pediatrics 50 (1972), 312.
12. Whitehouse, D. Psychological and neurological correlates of seizure disorders. Joins Hopkins Med. J. 729(1971). 36.
13. Rodin, E. . et al. Vocational and educational problems of epileptic patients. Epilepsia T3 (1972). 149.
14. Martin, J. W. Attitudes toward epileptic children in a city high school system. J. Sch. H. 44 (1 974), 1 44.
ACUTE MANAGEMENT OF SEIZURES
ETIOLOGY OF SEIZURES
ETIOLOGY OF SEIZURES BY AGE
CLINICAL CLASSIFICATION OF SEIZURES
DIFFERENTIAL DIAGNOSIS OF PAROXYSMAL BEHAVIOR