Indian Write- Ups

Paediatrics
 
Epilepsy in Children
- Dr Susan D’Souza,Jaslok Hospital Mumba
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  • Benign epilepsy with occipital paroxysms (BEOP)

    This occurs usually in neurologically normal children and seizures include a wide array of unusual symptoms like hemianopia, visual hallucinations, illusions,motor amaurosis etc. Prognosis is usually good and seizures cease by adult life.
  • Landau Kleffner

    This epilepsy syndrome is associated with acquired aphasia. Defect in language function and verbal auditory agnosis with an inability to comprehend language and reduction in spontaneous expression is present. Behavioural and intellectual difficulties are present. These children usually present at 3-9 years and remit by 15 years.4

Adolescence

  • Juvenile absence epilepsy

    In contrast to childhood absence these seizures are frequent and have less impairment of consciousness. Patients have generalised tonic-clonic seizures on awakening or myoclonic seizures.
  • Juvenile myoclonic epilepsy

    This occurs more often in females between 12-16 years. Patients have more frequent myoclonic jerks upon awakening. A few years later early morning generalised tonic-clonic seizures develop in association with the myoclonus. EEG shows a 4-6 cycles/sec irregular spike and wave pattern, which is enhanced by photic stimulation. The majority of these patients respond dramatically to valproate, which is required life-long. Discontinuance of the drug causes a high rate of recurrence of seizures.

Miscellaneous syndrome

  • Chronic progressive epilepsia partialis continuia (EPC) Rasmussen’s syndrome

    In 1958,Rasmussen,et al described this syndrome characterized by association of chronic encephalitis with intractable epilepsy. Clinical course occurs in three stages. The first stage is a simple partial seizure. Complex partial seizure can occur but no motor deficit is seen. Seizures are unresponsive and gradually increase. Second stage consists of neuropsychological deterioration, progressive hemiparesis, hemianopia and hemisensory loss and if dominant hemisphere is involved, it then results in dysphasia. In the third stage, arrest of neuropsychological deterioration occurs and seizures decrease in frequency. Arrest occurs in 2 months to 10 years after onset. MRI shows progressive brain atrophy and areas of increased 12 weighted signals in the affected hemisphere. Treatment comprises of high dose steroids, interferons, gancyclovir and functional hemispherectomy.5
  • Photosensitive epilepsy

    This is an interesting and rare form of epilepsy in which tonic-clonic (84%) absence (61%) and partial (2.5%) seizures occur following exposure to flicker lights, T. V. and videogames. The treatment is to view TV under bright light from a distance of 2 m, view TV with one eye closed (monocular vision) and use dark glasses or polaroid lense. If required sodium valproate needs to be tried.
  • Diagnosis of seizures

    Investigations of seizures depend on many factors like the age, type of seizure and the presence or absence of neurological findings. All children with the first aferbile seizure merit a minimum work up consistency of blood sugar, Ca+2, Mg+2, and serum electrolytes.

    EEG is the non-invasive gold standard in epilepsy. It not only helps in diagnosis but also helps in classifying the seizure type. Nowadays, a simultaneous video EEG recording is useful in determining the type of seizure, the classification of syndrome and to rule out to pseudoseizures or in the pre-surgical evaluation of patients. It may be normal in 2-3% of epileptics especially simple partial seizures. If a structural lesion is suspected then neuro-imaging is a must. MRI has now almost replaced the CT scan in epilepsy because lesions like mesial temporal sclerosis, focal cortical dysplasias, small angiomas and white matter lesions are better appreciated on an MRI scan, however still play as role in detecting calcified lesions.

Treatment of Epilepsy

The risk of occurrence after a first unprovoked seizure is between 25-80% depending on the risk factors. Seizure aetiology and EEG are the strongest predictors of recurrence, the risk being low (24%) in idiopathic epilepsy and normal EEG, and high (65%) in symptomatic epilepsy and abnormal EEG. The decision to treat should be always individualized. Initiation of an antiepileptic drug after a first unprovoked seizure may at times be considered in children with a constellation of risk factors like remote symptomatic seizure, children with partial seizure, sibling with epilepsy or prior acute seizure.

Now a whole new range of antiepileptic drugs is available to choose from like Colbazam, Clonazepam, Nitrazepam, Lamotrigine, Vigabatrine, Topirmate and Tiagabine. While a few of them like Vigabatrine are used specifically for infantile spasms7 seen in patients with tuberous sclerosis, the other drugs should be used judiciously weighing the pros and cons.

Choice of an antiepileptic drug according to seizure type and epilepsy syndrome
Type of seizure
First choice
Second choice
1. Partial seizure without generalization CBZ, PHT VPA, PB
2 GTC CBZ, VPA, PHT PB
3. Childhood absence
VPA, ESM -
4. Juvenile myoclonic epilepsy
VPA PB,CBZ
5.Progressive myoclonic epilepsy
VPA -
6. Infantile spasms
Oral steroids or ACTH
VPA
7. Rolandic epilepsy
CBZ, VPA
PHT
8. Tuberous sclerosis
Vigabatrine
-
CBZ-catbamazaepine PHT- phenytoin
VPA-sodium valproate PB-phenobabitone
ESM-enthousuximide

 

References

  • Douglas R. Nordle, Jr.; Carl W. et al. Recognition and classification of seizures in infants, Epilepsia 1997; July vol 2; 7-14.
  • Fusco L. Vigevaro F. Ictal Clinical Electroencephalographic findings of spasms In West syndrome, Epilipsi 1993; 34:371-8.
  • Berg AT, Shinnar S. Predictors of recurrent febrile seizures. Arch Pediatr Adolesc Med. 1997; 151:371.
  • Paquler P F, Van-Dongen H R, Loonen C B, The Landau Kleffner syndrome of acquired aphasia with convulsive disorder, Arch Neurolo, 1992; 42 : 354-359.
  • G. Cappovilla , F Paldin et al. Rasmussen’s syndrome; Longitudinal; EEG study from the first seizure to Epilepsia partialis continua. Epilepsia 1997; April 38 : 483-488.
  • Pratibho Singhi, Sudeshna Mitro Approach to management of a child with Epilepsy Indian Pediatrics 1997; 34:27-38.
  • R E Appleton, ACB Peters, Randomised placebo-controlled study of Vigabatrine as first line treatment of infantile spasms 2000: Jan:32.
 
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