A case of Melas (A3243G) on chronic dichloroacetate treatment.

eralized epileptic dysfunction, more marked in the right regions, and phenytoin was started. Mild perceptive deafness was also present. CSF and plasma routine examination was normal. The EMG suggested a moderate axonal neuropathy, mainly involving the lower limbs. Since the muscular biopsy was compatible with mitochondrial involvement, leukocyte mitochondrial DNA was sequenced, showing an A3243G mutation. In 1999, D.F. experienced generDear Sir, Mitochondrial myopathy, encephalopathy, lactic acidosis and stroke-like episodes (MELAS) is a severe mitochondrial disease for which the currently available therapeutic options are largely supportive and the prognosis remains substantially poor. Interestingly, sodium dichloroacetate (DCA) has been consistently shown to reduce lactic acidemia, present in mitochondrial disorders, by increasing the activity of the pyruvate dehydrogenase complex [1] . Here we describe a MELAS patient treated for almost seven years with DCA. Although further recurrence of headache, abdominal pain and stroke-like episodes was prevented, DCA exacerbated a preexistent peripheral neuropathy. In 1996, D.F., a 17-year-old male, developed a right temporo-occipital ischemic stroke, characterized by a migraine attacklike onset and left lateral homonymous hemianopsia, followed by generalized seizures. Although the CT scan of the brain evidenced a right temporo-occipital lesion, the neurological defi cits recovered completely during the next days. In fact, 6 months later the lesion was not any more documented at the brain MRI scan. Two years later, the patient experienced a migraine attack with visual aura, followed by left hemianopsia and weakness in his left hand. A brain MRI evidenced a wide right ischemic lesion involving both cortical and subcortical matters; the EEG showed a genReceived: July 21, 2005 Accepted: October 12, 2005 Published online: February 9, 2006