儿童原发性癫痫治疗指南(12)

儿童癫痫指南

However a large number become resistant toAEDs and there is a progressive cognitive,behavioural and memory impairment, if theepilepsy remains uncontrolled. Early surgery in theform of anterior temporal lobectomy is significantlymore effective than best medical treatment inadults(95).

Epilepsia Partialis Continua

Epilepsia partialis continua should be suspectedwhen focal, fairly constant myoclonic/clonic jerksinvolve one or more parts of the body (face/limb/tongue) only unilateraly(96). In most children,progressive, presumably immune-mediatedencephalitis, Rasmussen’s encephalitis underliesthis disorder. Over time, a progressive hemiplegiawith deterioration in cognition and behaviour isusual, as the epilepsy is resistant to all AEDs(96).Investigation should include a MRI which isinitially often normal but shows progressive hemi-atrophy and unilateral signal changes. EEGs arealso helpful though they may be deceptively showabsence of abnormalities. Only focal backgrounddisturbances may be seen. Till a hemiplegiadevelops immune therapies like steroids and IVIGcan be used.

Treatment is again primarily surgical(96) and ahemispheric resection/disconnection are the pro-cedure that seems to benefit a large number. Theonly drawback is that a permanent motor/visualfield defect is invariant after a hemispherectomy.Hence, this procedure becomes difficult in childrenwho still have good function of the limbs.17. Epilepsies and Cognition

Cognitive deterioration, academic underachieve-ment and behavioral problems are common comorbidities in children with chronic epilepsy(97-99). Uncontrolled seizures and worsening of theEEG with increasing epileptiform abnormalities aremore likely to be responsible for cognitivedeterioration, than any AED(100).

All children with epilepsy should be screenedwith a simple child behavior checklist(101)consisting of questions directed towards mood,behavior and school performance. Some childrenmerit a more formal neuropsychologicalexamination.

Sensitization of parents and teachers regardingassociated co-morbidities and early referral forpsycho-educational evaluation and specialeducation is useful.

All attempts to switch to monotherapy should bedone to reduce AED induced behaviour / cognitiveeffects.

It is important to recognize rare but importantsyndromes like the Landau-Kleffner syndrome(LKS) and Continuous Spike-Wave in Slow-WaveSleep (CSWS)(102,103). LKS presents aspredominant language deterioration in a previouslynormal child who may or may not have clinicalseizures. Initially the child may stop respondingwhen called and may appear to be deaf; there maybe school performance as well as behaviordeterioration. CSWS is a more global disturbancewith a frank dementia and autism. Both thesesyndromes are presumably causally associated witha continuous epileptic discharges in non-REM sleep– electrical status in slow wave sleep (ESES).Hence, any child with or without epilepsy, who hascognitive, language and behavioural deteriorationshould have an awake and more importantly sleepEEG to establish the diagnosis. Evaluation andmanagement of these complex syndromes needreferral to specialized epilepsy centers.18. Status Epilepticus

Children with seizure clusters are at increased riskof SE. Use of oral CLB or DZP for 2 days may bebeneficial in decreasing this risk. A child who isbrought to the physician from an extramural settingstill convulsing should be considered to be in SE;the minimum time for this definition of SE isregarded as >5 minutes(104). There is an increasedrisk of irreversible neuronal injury after 30 minutesof convulsive status(105). A management algori-thm(106-110) is provided for status epilepticus inFig 2.

Competing interests: None stated.

Funding: Sanofi Aventis India Ltd provided the fundingfor conducting the meeting.

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