儿童原发性癫痫治疗指南(10)

儿童癫痫指南

Discrete lesions without involvement offunctional motor, visual and language cortex,where a lesionectomy will often result in acomplete cure. Common lesions would includedevelopmental tumors, cortical dysplasias,AVMs etc. Sometimes lesions like largedysplasias/infarcts may need lobectomies/multi-lobar resections.

Mesial temporal lobe epilepsy caused often byhippocampal sclerosis is not uncommon inteenagers and is amenable to an anterior temporallobectomy.

Drop attacks with injuries respond well to corpuscallosotomy and should be offered as a palliativeprocedure.

15. Catastrophic Epilepsies in Infancy andEarly Childhood

West syndrome, symptomatic generalized epilepsieslike the Lennox-Gastaut syndrome and many otherlesional partial epilepsies starting in infancy, whichmay have fairly rapid effects on the developing brainwith high risk are appropriately labeled as thecatastrophic epilepsies. These often require fairlydetailed knowledge and expertise in both evaluationand management and are best referred to aspecialized centre where pediatric neurologist orepileptologist is available for specialized care.West syndrome (WS)

Early recognition needs taking a detailed history ofthe jerks with an emphasis on when they occur(usually on awakening in clusters lasting fewminutes)(89). Typically, they are described as“jhatka” in Hindi, “dachakte” in Marathi and“chamke” in Gujrathi. There is often loss of eyecontact and social smile, which should be carefullylooked for. This may sometimes precede spasm onsetby days or weeks.

A detailed history of preceding perinatal events(hypoxic-ischemic encephalopathy, neonatal hypo-glycemia etc), developmental milestones,examination of the skin for stigmata of tuberoussclerosis, head circumference measurement andcareful neurologic/developmental examinationlooking for deficits/delays will help to differentiatecryptogenic vs. symptomatic spasms.

An EEG should be done to confirm the diagnosis,though the characteristic pattern of hypsarrhythmiais not mandatory for diagnosis(89,90). Moreover, anexperienced electroencephalographer should beavailable for interpretation.

If the history and examination do not reveal anobvious etiology, an MRI preferably with specialtechniques to look for malformations of corticaldevelopment should be undertaken. Metabolic testsare usually unhelpful and should be done only inselected infants(90), where there is a high suspicionfor a neurometabolic condition e.g. consanguinity,positive family history, etc.

14. Refractory Epilepsy

Refractory epilepsy in childhood can be defined asepilepsy which is uncontrolled despite adequatetrials of three first line AEDs(82) and when itdisrupts developmental progress or normalchildhood activity(85). When faced with a child withuncontrolled epilepsy, always try and confirmwhether the diagnosis is correct. Often non-epilepticconditions may be confused as seizures (see above).Also the type of seizure and if possible, a correctdiagnosis of the specific epilepsy syndrome mayfacilitate correct choice of drug.

Errors in management must be looked for aspseudo-intractability often results from aninadequate dose, irrational polytherapy or wrongchoice of AED e.g. carbamazepine for absenceseizures(86). Every effort should be made to keep aseizure diary and see if a specific AED is actuallyhelping or in some cases worsening the seizures e.g.carbamazepine/oxcarbazine may worsen andsometimes even induce absence/myoclonicseizures(87,88).

It is best to refer intractable epilepsy early to atertiary center for appropriate evaluation (includinghigh-end MRI using standardized epilepsy proto-cols, video EEG etc) as well as to get guidance onmanagement options like newer AEDs, the ketogenicdiet and surgery.

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