Abstract
the role of LCM in patients withidiopathic generalized epilepsy presenting with absence statusepilepticus (ASE) is currently unknown.A 57-year-old woman came to be under our observation afterexperiencing confusion and drowsiness, immediately afterwaking up. Her family history was unremarkable. Apart frommild mental retardation and epilepsy, she had no other medicalproblems. Raretonic-clonic seizures,andepisodesoflost contactlasting for a few seconds or minutes had occurredmonthly sincethe age of 6 yearsold despite havingbeen treatedwith 2000 mg/day valproate(VPA),300 mg/daylamotrigine(LTG),3000mg/daylevetiracetam (LEV), 300 mg/day topiramate, 100 mg/day phe-nobarbital. The interictal EEG showed generalized spike-waveand polyspike-wave discharges at 3–4 Hz, lasting from 1 to 3 s.Brain MRI and genetic analysis (karyotypeand array-CGH) werenormal. After 6 h from the onset of confusion, the patient wasadmitted to our centre and at that timeshe was on 2000mg/dayVPA (plasma VPA was 87 mg/L, range 50–100 mg/L) and3000 mg/day LEV (plasma LEV was 25 mg/L, range 10–37 mg/L). A long-term video-EEG monitoring was performed and ASEwas diagnosed. In particular, she was slow, vague, inattentive,with no verbal contact and displayed rare, small myoclonictwitches of the eyelids and facial muscles.The ictal EEG revealeda continuous, generalized, 2.5–4Hz spike, polispike-wavepattern and brief trains of polispikes during confusional state(Fig. 1a). Intravenous (IV) diazepam (10 mg in bolus) induced arapid but transient effect (Fig. 1b), with ASE reappearing after5 min. The administration of IVdiazepam (10 mg bolus) was alsorepeated withouteffect.Wethereforeadministeredan IVloadingdose of200mgLCMoveraperiodof15 min,througha peripheralline, diluted in 50 ml of normal saline. During and at the end ofinfusion, the clinical picture and EEG pattern were unchanged(Fig. 1c). After 10 min, an additional 200 mg of LCM wasadministered for 15 min without effect (Fig. 1d). No adverseeffects were observed during the administration of LCM and noECG andlaboratoryvalues-changesweredocumented.After24 hthe ASE spontaneously resolved. The patient was then given2000 mg/day VPA and 100 mg/day LTG, while LEV was graduallydiscontinued. She has now been seizure-free during the 12months of follow-up.Althoughthe most commonly used bolus dose of LCM is 200–400 mg over 3–5 min,
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