Occipital status epilepticus in patients with MELAS-like picture

Stroke-like episodes have been described in MELAS: usually they are associated with hemiparesis, hemianopsia and cortical blindness, seldom with seizures or Status Epilepticus. We describe a 12 years-old boy with Leber's disease, that at 10 yrs of age presented partial seizures. Five months later atypical absences appeared, treated with VPA. After 2 months a picture of acute encephalopathy associated with coagulation disease occurred, recovered after 10 days. One month later partial seizures, characterized only by right deviation of eyes, related on the EEG with fast, low amplitude discharge on the left occipital region, appeared. MRI showed a left parieto-occipital lesion, similar to stroke-like lesions. All the investigation appropriate for the Mithocondrial Myopathy (e.g.: lactate, DNA mt analysis, muscular biopsy) are normal. However the Authors hypothesize the diagnosis of MELAS.