Indian Write- Ups

Paediatrics
 
Epilepsy in Children
- Dr Susan D’Souza,Jaslok Hospital Mumba
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Introduction

Very often the term seizures (convulsions) and epilepsy are incorrectly used interchangeably. While seizure is a symptom, defined as a paroxysmal involuntary disturbance of the brain function, which may manifest as an abnormal motor activity, behavioural abnormalities, sensory or autonomic dysfunction with or without loss of consciousness, epilepsy manifests as recurrent seizures unrelated to fever or an acute cerebral insult.

Classification of Epilepsy

The ILAE (International League Against Epilepsy) in 1958 had classified epilepsy as a partial, generalized and unclassified seizures. Partial seizures account for up to 40% of the seizures in childhood and are further subdivided as simple partial, during which consciousness is retained , and complex partial , in which consciousness is impaired (Table-1).

With clinical observations and interictal EEGs, seizures in infants cannot be reliably classified by the current ILAE criteria. So, in contrast a new classification scheme based on semiology has been proposed for infantile seizures (astatic, behavioural, clonic, tonic and versive epileptic spasms).1

Approach to a Child with Epilepsy

The first step to ensure that the child has epilepsy, with a detailed history and corroborative examination. A generalized tonic-clonic convulsion is easy to diagnose. But an astute clinician should be able to detect seizures and differentiate infantile spasms from colic.

On history one must attempt to define factors that have precipitated the convulsion, details of seizure type and the post-ictal state. Most parents would be able to vividly recall and imitate their child’s seizure.

The second step is to rule out non-epileptic paroxysmal events that may be confused with epilepsy, like breath holding spasms, migraine, syncope, recurrent abdominal pain, narcolepsy, cataplexy, apnoea in the newborn etc. Situation related to seizures are also common in children due to factors like dyselectrolytemia, meningoencephalitis, tumours, infracts etc.

The hallmarks of epileptic seizures are

  • Recurrent
  • Brief
  • Spontaneous , unprovoked and sudden
The examination of a child with a seizure disorder should focus on trying to rule out an organic cause.

Clues that point to neurocutaneous syndromes should be sought after, like café au lait spots, Shagreen patch, adenoma sebaceum etc. Localising neurological signs like subtle Hemiparesis and Babinski’s response may point towards a contralateral hemisphere structural lesion. A fundoscopic evaluation must look for the presence of papilloedema, chorioretinitis, choroidal tubercles, retinal phakomas etc.

Table 1

ILAE revised classification of seizures (1989)

  • Localised- Epilepsies and Syndromes

    • Idiopathic (with age related onset)

      • Benign childhood epilepsy with centerotemporal spike.
      • Childhood epilepsy with occipital paroxysms
    • Symptomatic

      • Chronic progressive epilepsia partialis continua childhood
      • Syndromes characterized by seizures with specific modes of presentation
    • Cryptogenic: (presumed symptomatic but no etiology known).
  • Generalised epilepsies and syndromes

    • Idiopathic (with age related onset, listed in order of age)

      • Benign neonatal familiar convulsions
      • Benign neonatal convulsions
      • Benign myoclonic epilepsy in infancy
      • Juvenile absence epilepsy
      • Epilepsy with grand mal (GTC seizures) on awakening
    • Cryptogenic or Symptomatic

      • West syndrome
      • Lennos gastaut syndrome
      • Epilepsy with myoclonic-astatic seizures(Dose Syndrome)
      • Epilepsy with myoclonic absences
    • Symptomatic

      Non-specific etiology
      • Early myoclonic encephalopathy
      • Early infantile epileptic encephalopathy with suppression burst

      Specific syndromes/ etiologies
      • Cerebral malformations
      • Inborn errors of metabolism including pyridoxine dependency
  • Epilepsy and syndromes undetermined whether focal or generalized

    With both generalized and focal seizures
    • Neonatal seizures
    • Severe myoclonic epilepsy in infancy
    • Epilepsy with continous spike-waves during slow wave sleep
    • Acquired epileptic aphasia(Landau-Kleffner Syndrome)
    Without unequivocal generalized or focal features

  • Special Syndromes
    • Isolated seizures or isolated status epileptics
    • Seizure occurring only when acute metabolic/ toxic even
    • t Non-ketotic hyperglycameia
    • Reflex epilepsy
 
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