儿童原发性癫痫治疗指南(11)

儿童癫痫指南

Steroids are the drugs of first choice in all casesof West syndrome(90,91), especially so incryptogenic WS, except in tuberous sclerosis. ACTHis preferred over oral steroids(90,91). Oralprednisolone is given in doses of 2-4 mg/kg(91) ornatural ACTH in 30-40 units/day (3-6 U/kg) for 2weeks, with rapid taper over the next 2 weeks. Rapidcontrol of the spasms within 1 month of onset isassociated with rapid developmental gains (VPAcould be continued after steroids in symptomaticWest syndrome).

Vigabatrin is preferred in TS as firstchoice(90,91) and in steroid failures in the others(taking into account availability/affordabilityissues). It should be used for a period of 3-6 monthsonly, due to the fear of visual field defects.Nitrazepam/high dose VPA/Topiramate can beused as alternatives. The ketogenic diet and surgeryin selected lesional cases are other alternatives.Lennox Gastaut syndrome

Any toddler, who has epileptic drop attacks and isdelayed or has arrest in development, should beconsidered to have LGS(89). Usually other types ofseizures are also present (like atypical absence andbrief tonic seizures in sleep).

An MRI is essential in case no obvious cause isidentified on history and examination. An EEGshould be performed to confirm the diagnosis thoughthe typical slow spike-wave paroxysms are notmandatory for diagnosis(89). It is mandatory fordiagnosis of non-convulsive status epilepticus,which often occurs in LGS and manifests asdecreased responsiveness, drooling and regressionof milestones lasting hours to days(89).

VPA and CLB should be used initially. LTG(92)or TPM(93) should be added in case of continuingseizures. CBZ and OXZ should be avoided.The ketogenic diet should be used early, ifavailable. Helmets should be worn to prevent headinjury. It is best to refer such children to a tertiaryepilepsy center to manage these complicatedpatients.

Dravet’s syndrome (Severe myoclonic epilepsy ofinfancy)

One should think of this syndrome in normal infantswith onset of refractory febrile/afebrile, focal/generalized seizures in the first year of life(94).They often present as refractory febrile or afebrilestatus epilepticus (SE)(94) which often lasts severalhours. Over the next 2-3 years, delayed languagedevelopment, autistic features and later, gaitdifficulties become evident. Myoclonic andabsence seizures, often photosensitive usuallybecome prominent after the first year, though theyare not mandatory for diagnosis (93). VPA, CLBand TPM(71) are drugs most likely to help preventSE, though a full remission is unlikely to occur.LTG and CBZ regularly worsen these seizures andtherefore should be avoided in all febrile seizures ininfancy, even those which are clinically focal. Oncethis condition is considered, it is best to refer to atertiary care center for further evaluation andmanagement.

16. Refractory Epilepsies in Older Childrenand Adolescents

Mesial Temporal Lobe Epilepsy

Clinical recognition of mesial temporal lobeepilepsy (MTLE) is important as it is the mostcommon refractory epilepsy syndrome in the olderchild/teenager. It is often caused by hippocampalsclerosis, though other etiologies like corticaldysplasia, tumors and vascular malformations mayalso underlie it. It presents as complex partialseizures with an aura of fear or epigastric sensationfollowed by unresponsiveness, automatisms andlater secondary generalization. The patients haveoften had febrile seizures (often febrile statusepilepticus) in the past. The complex partial seizurespresent several years later and over time becomerefractory.

Sleep deprived EEGs with special electrodes areoften needed in these cases and should be also donein specialized centers. High quality MRIs need to bedone to diagnose hippocampal sclerosis and whereMTLE is suspected, it is better to refer the child foran MRI through a specialized center to avoid dualcosts. In many children, conventional AEDs likeCBZ, PHT and newer AEDs like OXZ and TPM areeffective for a while in controlling seizures.

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