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- W622751334 abstract "OBJECTIVE:To report a case of MELAS of late adult onset presenting with non-convulsive status and atypical strokes.BACKGROUND:MELAS is a mitochondrial disorder, characterized by stroke-like episodes, seizures, encephalopathy and muscle weakness [1]. The reported age of onset ranges from 3 to 40 years [2]. The majority of cases are due to A3G mutation at position 3243 of the mitochondrial DNA. [2]METHODS:A 58 year old Hispanic woman presented with acute mental status change. Exam was significant for a Wernicke’s aphasia without any motor or sensory deficits. MRI Brain showed left temporal lobe cortical edema and laminar necrosis. Video EEG showed non-convulsive seizures with left central-temporal focus. Initial blood workup showed lactic acidosis. CSF showed no cells, an elevated lactate level of 4.6. HSV-PCR was negative. Her sister had died after two strokes at the age of 53.RESULTS:The patient was successfully treated with Valproate and Levetiracetam for non-convulsive status epilepticus. However, her neurologic condition worsened and she became lethargic and unresponsive. Repeat EEG and CSF analysis were normal. Repeat MRI showed new areas of abnormality in the right insular cortex and bilateral hippocampi. Cerebral angiogram was normal. Brain biopsy showed laminar necrosis and muscle biopsy showed COX-negative Ragged Red Fibers. Patient eventually became alert and was discharged to a rehabilitation facility with persistent Wernicke’s aphasia. Genetic analysis of muscle biopsy specimen showed heteroplasmic A3243G mutation in the mitochondrial tRNA. Ten weeks after discharge, patient remained seizure free with a persistent Wernicke’s aphasia and memory difficulty.CONCLUSIONS:Adult-onset strokes due to MELAS have rarely been described and are usually suspected in the setting of recurrent strokes. Our patient was diagnosed at her initial attack because of her unique presentation. We believe that MELAS should be considered in all adult patients presenting with atypical strokes and seizures. Disclosure: Dr. Raval has nothing to disclose. Dr. El-Ghanem has nothing to disclose. Dr. Hillen has nothing to disclose." @default.
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- W622751334 date "2015-04-06" @default.
- W622751334 modified "2023-09-23" @default.
- W622751334 title "Late adult onset MELAS, presenting as non-convulsive status and atypical strokes. (P2.257)" @default.
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