PMID: 16525920Mar 10, 2006Paper

Characteristics of status epilepticus in MELAS. Analysis of four cases

Neurología : publicación oficial de la Sociedad Española de Neurología
R RibacobaA Astudillo

Abstract

Clinical characteristics of status epilepticus (SE) as a first manifestation in patients with MELAS who had not previously epileptic episode has been studied little in the literature. Our aim was to analyse precipitating factors, clinical characteristics, EEG and difficulties in the treatment of SE in MELAS. We studied four cases with ages between 27 an 41 years who began with SE and they had been diagnosed with MELAS during the episode. Case 3 was confirmed by autopsy. Cases 1, 2 and 4 showed a 3243 mtDNA mutation in the lymphocytes. Epileptic seizures had not been present in any previous status case. The precipitating factor in cases 1 and 3 was fever and in case 2 and 4 stress by headache. Moreover in case 2 second status was caused by stress in hyperglycaemic ketoacidosis. All cases were studied with EEG and a brain CT or MRI. All patients started with epilepsia partialis continua that began with partial motor simple seizures, but sometimes progressed to partial complex seizures or secondary tonic clonic seizures. In two cases the initial symptom was migraine with aura, in two cases fever with cephalalgia and in one case diabetes mellitus decompensation. The EEG during a seizure presented a complex pseudoperiodic complex in...Continue Reading

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