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Febrile Seizures Febrile convulsions, the most common seizure disorder during childhood.Febrile seizures are age dependent and are rare before 9 mo and after 5 yr of ageIt is initially generalized and tonic-clonic in nature, lasts a few seconds and rarely up to 15 min A febrile seizure is described as complex or complicated when the duration is >15 min,when repeated convulsions occur within 24 hr, or when focal seizure activity or focal findings are present during the postictal period.During the acute evaluation, a physician's most important responsibility is to determine the cause of the fever and to rule out meningitis or encephalitis. If any doubt exists about the possibility of meningitis, a lumbar puncture with examination of the cerebrospinal fluid (CSF) is indicated. A lumbar puncture should be strongly considered in children <12 mo of age and considered in those 12–18 mo of age, especially if seizures are complex or sensorium remains clouded after a short postictal periodAside from glucose determination, laboratory testing such as serum electrolytes and toxicology screening should be ordered based on individual clinical circumstances such as evidence of dehydration Partial Seizures Partial seizures account for a large proportion of childhood seizures, up to 40% in some series. Partial seizures may be classified as simple or complex; consciousness is maintained with simple seizures and is impaired in patients with complex seizures. SIMPLE PARTIAL SEIZURES (SPS). Motor activity is the most common symptom of SPS. The movements are characterized by asynchronous clonic or tonic movements, and they tend to involve the face, neck, and extremities. Versive seizures consisting of head turning and conjugate eye movements are particularly common in SPS. Automatisms do not occur with SPS, but some patients complain of aura (chest discomfort, headache), which may be the only manifestation of a seizure. COMPLEX PARTIAL SEIZURES (CPS). A CPS may begin with a simple partial seizure with or without an aura, followed by impaired consciousness; conversely, the onset of the CPS may coincide with an altered state of consciousness. An aura consisting of vague, unpleasant feelings, epigastric discomfort, or fear is present in approximately one third of children with SPS and CPS. The presence of an aura always indicates a focal onset of the seizure. BENIGN PARTIAL EPILEPSY WITH CENTROTEMPORAL SPIKES (BPEC). BPEC is a common type of partial epilepsy in childhood and has an excellent prognosis. The clinical features, EEG findings (rolandic foci), and lack of a neuropathologic lesion are characteristic and readily separate BPEC from CPS. BPEC occurs between the ages of 2 and 14 yr and has a peak age of onset of 9–10 yr Generalized Seizures ABSENCE SEIZURES. Simple (typical) absence (petit mal) seizures are characterized by a sudden cessation of motor activity or speech with a blank facial expression and flickering of the eyelids. These seizures, which are uncommon before age 5 yr, are more prevalent in girls, are never associated with an aura, rarely persist longer than 30 sec, and are not associated with a postictal state. These features tend to differentiate absence seizures from complex partial seizures. Children with absence seizures may experience countless seizures daily, whereas complex partial seizures are usually less frequent. Patients do not lose body tone, but their head may fall forward slightly. Immediately after the seizure, patients resume preseizure activity with no indication of postictal impairment. Automatic behavior frequently accompanies simple absence seizures. Hyperventilation for 3–4 min routinely produces an absence seizure. The EEG shows a typical 3/sec spike and generalized wave discharge. Complex (atypical) absence seizures have associated motor components consisting of myoclonic movement of the face, fingers, or extremities and, on occasion, loss of body tone. These seizures produce atypical EEG spike and wave discharges at 2–2.5/sec. GENERALIZED TONIC-CLONIC SEIZURES. These seizures are common and may follow a partial seizure with a focal onset (second generalization) or occur de novo. They may be associated with an aura, suggesting a focal origin of the epileptiform discharge. It is important to inquire about the presence of an aura, because its presence and site of origin may indicate the area of pathology. Patients suddenly lose consciousness and, in some cases, emit a shrill, piercing cry. Their eyes roll back, their entire body musculature undergoes tonic contractions, and they rapidly become cyanotic in association with apnea. The clonic phase of the seizure is heralded by rhythmic clonic contractions alternating with relaxation of all muscle groups. The clonic phase slows toward the end of the seizure, which usually persists for a few minutes, and patients often sigh as the seizure comes to an abrupt stop. During the seizure, children may bite their tongue but rarely vomit. Loss of sphincter control, particularly the bladder, is common during a generalized tonic-clonic seizure. Tight clothing and jewelry around the neck should be loosened, the patient should be placed on one side, and the neck and jaw should be gently hyperextended to enhance breathing. The mouth should not be opened forcibly by an object or by a finger because the patient's teeth may be dislodged and aspirated, or significant injury to the oropharyngeal cavity may result. Postictally, children are initially semicomatose and typically remain in a deep sleep from 30 min to 2 hr. If patients are examined during the seizure or immediately postictally, they may demonstrate truncal ataxia, hyperactive deep tendon reflexes, clonus, and a Babinski reflex. The postictal phase is often associated with vomiting and an intense bifrontal headache. Idiopathic seizure is a term applied when the cause of a generalized seizure cannot be ascertained. Many factors are known to precipitate generalized tonic-clonic seizures in children, including low-grade fever associated with non– central nervous system infections, excessive fatigue or emotional stress, and various drugs including psychotropic medications, theophylline, and methylphenidate, particularly if the seizures are poorly controlled by anticonvulsant drugs. MYOCLONIC EPILEPSIES OF CHILDHOOD. This disorder is characterized by repetitive seizures consisting of brief, often symmetric muscular contractions with loss of body tone and falling or slumping forward, which has a tendency to cause injuries to the face and mouth. Myoclonic epilepsies include a heterogeneous group of conditions with multiple causes and variable outcomes. At least five distinct subgroupings can be identified; these represent the broad spectrum of myoclonic epilepsies in the pediatric population. BENZODIAZEPINES. The benzodiazepines exert anticonvulsant activity by binding to a specific GABA site that enhances the opening frequency of the chloride channel without affecting open or burst duration (see Fig. 593-4 ). The drugs diazepam and lorazepam IV are used for initial management of status epilepticus. Rectal diazepam gel has been demonstrated to be an effective and safe treatment to abort episodes of acute repetitive seizures in children and is available as Diastat gel in 2.5, 5, and 10 mg doses for children. Buccal or nasal midazolam is also effective acutely. Clonazepam is useful for the management of the Lennox-Gastaut syndrome, myoclonic, akinetic, and absence seizures. CARBAMAZEPINE. This drug is effective for the management of generalized tonicclonic and partial seizures Significant leukopenia (<1,000 neutrophils/mL3) and hepatotoxicity may rarely develop, particularly during the initial 3–4 mo of therapy. Therefore, a CBC with differential and AST and ALT levels should be obtained on a monthly basis during this period, although serious idiosyncratic drug reactions may develop despite normal liver function tests results and routine blood work. ETHOSUXIMIDE. Ethosuximide provides its anticonvulsant action by blocking calcium channels associated with thalamocortical circuitry. Ethosuximide is an effective drug for the management of typical absence epilepsy and has a half-life of 60 hr GABAPENTIN. This anticonvulsant is used as an add-on drug for patients with refractory complex partial and secondarily generalized tonicclonic seizures LAMOTRIGINE. Lamotrigine is a phenyltriazine compound used as an add-on drug for the management of complex partial and generalized tonic-clonic seizures. Lamotrigine is effective as monotherapy for some children with the Lennox-Gastaut syndrome and generalized absence seizures PHENOBARBITAL AND PRIMIDONE. These are relatively safe anticonvulsants that are particularly useful for generalized tonic-clonic seizures. Approximately 25% of children undergo severe behavioral changes on these drugs. Neurologically abnormal children are at greater risk. Furthermore, there is evidence that phenobarbital may adversely affect the cognitive performance of children treated on a long-term basis PHENYTOIN. Phenytoin acts by decreasing the sustained repetitive firing of single neurons by blocking sodium-dependent channels and decreasing depolarization-dependent calcium uptake. Phenytoin is used for primary and secondary generalized tonic-clonic seizures, partial seizures, and status epilepticus VALPROIC ACID. Valproic acid is a broad-spectrum anticonvulsant. It acts by blocking voltage-dependent sodium channels and increases calcium-dependent potassium conductance. The elimination half-life is 6–16 hr. This drug is useful for the management of many seizure types, including generalized tonic-clonic, absence, atypical absence, and myoclonic seizures. It rarely induces behavioral changes but is associated with mild gastrointestinal disturbances, alopecia, tremor, and hyperphagia. Two rare but serious side effects of valproate are a Reye-like syndrome and irreversible hepatotoxicity. ACTH. This is the preferred drug for the management of infantile spasms, although the dose and duration of therapy are not uniform. Prednisone is equally effective