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Yibian
 Shen Yaozi 
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diseasePediatric Epilepsy
aliasEpilepey, Epilepsy
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bubble_chart Overview

Epilepsy is a syndrome of brain dysfunction caused by various disease factors, characterized by paroxysmal, sudden, and temporary brain dysfunction due to abnormal hypersynchronous discharges of brain cell groups. Clinical manifestations can take various forms, the most common being altered consciousness or loss of consciousness, localized or generalized muscle tonic or clonic spasms, and sensory abnormalities. It may also present with behavioral abnormalities, emotional and perceptual disturbances, memory changes, or autonomic nervous system dysfunction.

bubble_chart Clinical Manifestations

According to clinical seizure types, it is classified as: {|###|}

  1. Tonic-clonic seizure: Also known as grand mal seizure. During the seizure, consciousness is suddenly lost, with generalized muscle tonic contraction, eyes wide open, upward rolling of the eyeballs, apnea, cyanosis, dilated pupils, and loss of light reflex. This lasts for several seconds or dozens of seconds before transitioning to the clonic phase. The clonic phase manifests as rhythmic limb convulsions, typically lasting 1–5 minutes. After the clonic phase ends, there is a brief period of muscle weakness lasting a few seconds, during which urinary incontinence may occur. Post-seizure, there is transient confusion or drowsiness before returning to a clear state. Patients often feel fatigued, experience headaches, and sometimes vomiting or generalized muscle pain afterward. {|###|}
  2. Absence seizure (petit mal): Sudden, brief loss of consciousness without aura, lasting a few seconds and rarely exceeding 30 seconds. During the seizure, speech and activity halt without falling, maintaining a fixed posture with a blank stare. There may be slight tremors in the hands, lips, or head. Post-seizure, normal activities resume, with no recollection of the episode. These seizures may occur several to dozens of times daily. {|###|}
  3. Myoclonic seizure: Characterized by sudden, rapid, and forceful contractions of a muscle or muscle group, causing unilateral or bilateral limb jerks. Objects held in the hand may drop or be flung. Trunk muscle contractions may result in sudden forceful nodding, bending forward, or arching backward. If standing, the person may fall violently to the ground, often injuring the head, forehead, jaw, lips, or teeth. {|###|}
  4. Tonic seizure: Sudden tonic contraction of certain muscles, such as trunk flexion, neck extension, head tilting forward, shoulder elevation, forearm pronation, elbow flexion or extension, fixed in a posture for seconds or longer. After the seizure ends, muscle tone normalizes, and the original posture is restored. Brief loss of consciousness occurs during the episode. {|###|}
  5. Atonic seizure: Sudden, transient loss of muscle tone, leading to inability to maintain posture. The seizure lasts 1–3 seconds with loss of consciousness. If standing, the person may suddenly drop their head, slightly abduct their arms, open their fingers, let their upper arms droop, bend their knees, and then fall. Consciousness quickly returns, allowing immediate standing. Sometimes, these seizures may occur consecutively. {|###|}
  6. Clonic seizure: Rhythmic flexion and extension of limbs during the seizure, with varying speed and amplitude of movements. {|###|}
  7. Focal motor seizure: Rhythmic twitching of a specific body part, such as one arm or facial muscles. It may start locally and gradually spread to other areas. Consciousness is preserved during the seizure. {|###|}
  8. Focal sensory seizure: Abnormal somatic or special sensory experiences (e.g., visual, auditory, olfactory, or gustatory) without limb spasms. {|###|}
  9. Complex partial seizure: Symptoms involving mental, consciousness, motor, sensory, and autonomic functions during the seizure. Automatisms may occur—a series of purposeless, inappropriate, and bizarre repetitive stereotyped movements. Consciousness is impaired, preventing understanding of the environment or execution of simple commands or normal actions. This type may generalize, leading to loss of consciousness. {|###|}
Epileptic syndromes specific to childhood {|###|}
  1. Ohtahara syndrome: Onset in neonates and young infants, often presenting with tonic spasms, sometimes in clusters, or partial seizures. Prognosis is poor, often accompanied by severe intellectual disability. EEG shows periodic, diffuse burst suppression. {|###|}
  2. Infantile spasms: Onset in infancy, characterized by clusters of tonic spasms, often associated with intellectual decline and developmental delay. EEG exhibits "hypsarrhythmia." {|###|}
  3. Lennox-Gastaut syndrome: Onset in early childhood, with diverse seizure types including myoclonic, tonic, atonic, and atypical loss of vitality seizures, often accompanied by intellectual disability. EEG shows bursts of 2-2.5 Hz spike-and-slow-wave or polyspike-and-slow-wave complexes.
  4. Benign epilepsy with centrotemporal spikes: The onset mostly occurs in preschool to school-age children. Seizures often occur during sleep, starting with facial or unilateral limb spasms and quickly progressing to generalized convulsions. The prognosis is favorable. EEG findings show spikes or spike-and-wave complexes in the central or mid-temporal regions, appearing singly or in clusters.
  5. Epilepsia partialis continua (Kojewnikow syndrome): This is a partial motor seizure caused by lesions in the motor cortex of the brain, characterized by continuous focal clonic seizures in the face or limbs without impaired consciousness.
  6. Acquired epileptic aphasia (Landau-Kleffner syndrome): Sudden onset of aphasia in preschool or elementary school-age children, often accompanied by convulsions and behavioral disturbances. EEG background activity is normal, with multifocal spikes or spike-and-wave complexes.

bubble_chart Auxiliary Examination

Electroencephalography (EEG) Examination

EEG is an important method for diagnosing epilepsy. It not only confirms the diagnosis but also helps differentiate the types of epilepsy. During generalized seizures, the EEG shows simultaneous discharges across the entire brain, whereas during partial seizures, it reveals localized abnormalities. When an EEG is performed during the interictal period of epilepsy, the positive rate is only 50–60%, so a normal EEG does not rule out epilepsy. EEG abnormalities include nonspecific findings, such as increased slow waves, asymmetry, and poor regulation, which alone cannot diagnose epilepsy. However, epileptiform discharges—such as spikes, spike-and-slow waves, polyspike-and-slow waves, sharp waves, sharp-and-slow waves, and hypsarrhythmia—are highly significant for epilepsy diagnosis. To confirm the diagnosis, sleep EEG or 24-hour EEG monitoring may sometimes be necessary.

Other Laboratory Tests

These are primarily aimed at identifying the disease cause of epilepsy.

  1. Cerebrospinal Fluid (CSF) Examination: This is generally unnecessary unless an infection (viral, bacterial, parasitic, or Chinese Taxillus Herb-related), hemorrhage, or other factors are suspected as contributing to the condition.
  2. CT and Magnetic Resonance Imaging (MRI): These tests help detect structural abnormalities in the brain and are valuable for identifying the disease cause of epilepsy. However, the presence or absence of abnormalities on CT or MRI cannot definitively confirm epilepsy.
  3. Single-Photon Emission Computed Tomography (SPECT): Combining radionuclide imaging with CT technology, SPECT displays the distribution of radioisotopes across different brain layers, reflecting regional cerebral blood flow and metabolic changes. During the interictal period of epilepsy, blood perfusion decreases at the lesion site, whereas during a seizure, perfusion increases significantly. SPECT can detect epileptic foci that manifest only as functional brain abnormalities.

bubble_chart Diagnosis

The diagnosis of epilepsy first requires determining whether it is an epileptic seizure. Epileptic seizures are characterized by sudden onset, self-termination, and a tendency to recur, with each episode being similar. Next, it is necessary to identify the type of seizure, which is diagnosed based on clinical manifestations and changes in the electroencephalogram (EEG) during or (and) between seizures. Finally, the cause of epilepsy should be investigated. Epilepsy with an identifiable cause is classified as secondary epilepsy; cases without a known cause are primary epilepsy. Additionally, some epilepsies, although currently without a clear cause, are presumed to be related to certain factors and are termed cryptogenic epilepsy.

bubble_chart Treatment Measures

Status epilepticus should be treated as an emergency, with the following principles:

  1. Use potent anticonvulsant drugs to promptly control seizures;
  2. Maintain vital functions and prevent or manage complications, with special attention to addressing cerebral edema, acidosis, respiratory or circulatory failure, and hyperthermia;
  3. Actively identify the disease cause and treat the underlying condition;
  4. After seizure cessation, long-term antiepileptic therapy should be initiated.

(1) Anticonvulsant Drugs

  1. Diazepam (Valium): Acts rapidly, with effects occurring within 1–3 minutes after intravenous injection. The intravenous dose is 0.25–0.5 mg/kg per administration. For young children, a single dose should not exceed 5 mg, and for infants, it should not exceed 2 mg. If necessary, the dose may be repeated after 20 minutes, up to 2–4 times within 24 hours. Intravenous injection should not be too rapid; it should be administered at a rate of 1 mg per minute. Intramuscular injection of diazepam is absorbed more slowly than oral administration, so it is not suitable for treating status epilepticus via this route.
  2. Phenytoin Sodium: Reaches peak concentration in the brain within 15 minutes after intravenous administration. A single loading dose of phenytoin sodium is 15–20 mg/kg, diluted in 0.9% saline for intravenous infusion, administered at a rate of 1 mg/kg per minute. Maintenance doses are given 12 hours later, calculated at 5 mg/kg per day, administered once every 24 hours.
  3. Clonazepam: The usual single dose is 1–4 mg, not exceeding 10 mg, administered intravenously or intramuscularly.
  4. Phenobarbital: Administered as its sodium salt at 5–10 mg/kg per dose, intramuscularly. However, this drug acts slowly, taking 20–60 minutes to reach peak concentration in the brain, so it cannot immediately stop seizures. Nonetheless, after seizures are controlled by drugs like diazepam, phenobarbital remains a fundamental anticonvulsant for long-term use.
  5. Paraldehyde: This drug has strong anticonvulsant effects and good efficacy, but it is excreted through the respiratory tract and can be irritating. Caution is advised in infants and patients with pneumonia. The intramuscular dose is 0.2 ml/kg or 1 ml per year of age, not exceeding 5 ml per dose. Paraldehyde can react with plastic tubing and produce toxic substances, so plastic syringes (disposable syringes) should not be used.
  6. Thiopental Sodium or Amobarbital Sodium: These are fast-acting barbiturates that can be administered intramuscularly or by slow intravenous injection. Thiopental sodium (0.25 g) is diluted in 10 ml of sterile water for injection and administered intravenously at a rate of 0.5 mg/kg per minute until seizures stop. The remaining solution should not be injected further, with a maximum dose of 5 mg/kg per administration.
(2) Maintaining Vital Functions and Preventing Complications

Children with status epilepticus require close monitoring to maintain normal respiration, circulation, blood pressure, and body temperature. Ensure airway patency. Monitor fluid intake and output, initially limiting intravenous fluids to 1,000–1,200 ml/m² per day. To prevent hypoglycemia, intravenous glucose may be administered to maintain blood glucose at around 8.4 mmol/L. Cerebral edema and secondary increased intracranial pressure often occur during status epilepticus; dexamethasone and mannitol may be used as dehydrating agents.

(3) Identifying the Disease Cause and Treating the Underlying Condition

In children previously diagnosed with epilepsy, the most common cause of status epilepticus is sudden discontinuation of antiepileptic drugs. Other triggers may include infection, poisoning, severe stress, or sleep deprivation. The cause should be identified and symptomatic treatment initiated immediately. For children without a history of depressive psychosis, the disease cause should be identified based on medical history, physical examination, and laboratory tests.

(4) Long-Term Use of Anticonvulsants

For children with status epilepticus, regardless of whether they have a history of epilepsy, oral antiepileptic drugs should be administered after the current episode is controlled. If the primary condition (such as infection or high fever) has not completely subsided, the dosage should be slightly higher, then reduced to a maintenance dose after a few days, and continued for 1 to several months. Follow-up (including EEG examination) should be conducted to determine whether to continue antiepileptic medication.

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