Abstract
We report a case with MELAS(mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes)which was diagnosed after repeated stroke-like attacks in adulthood. A 29-year-old female had her first attack at age 23 when she presented with headache and high fever associated with a diffuse lesion in the right temporal lobe as revealed on her MRI. The symptoms and radiological abnormalities disappeared in a short time period. Her second attack was at age 27 when she had left homonymous hemianopia and generalized tonic convulsion associated with a right occipital lesion observed on a CT scan and MRI. Laboratory serum data showed in normal and angiographical studies demonstrated no pathological findings. The hemianopia improved progressively following medical treatment. The third attack was at age 29, when she had right homonymous hemianopia assoiated with a left occipital diffuse lesion depicted via CT scan and MRI. Angiography demonstrated early venous filling in the lesion. After the third attack, the serum level of lactic acid was studied and was found to be at a pathologically high level. Finally, blood screening was ordered, which revealed an A to G point mutation of mtDNA. Retrospectively, she had a history of diagnosis of WPW(Wolff-Parkinson-White)syndrome at age 6 and diabetes mellitus since she was twenty-five. MELAS is commonly diagnosed in childhood and adolescence by a variety of symptoms in the systemic organs. MELAS with only stroke-like onset, in particular in adulthood, is potentially misdiagnosed as a cerebrovascular disease. This entity is rare for neurosurgeons to encounter but should be considered as one of the possible differential diagnosis in casess with unknown cause of stroke-like episodes.
