Abstract
Objective: To discuss the acute neuroimaging findings of stroke like episodes (SLEs) in patients with MELAS syndrome (Mitochondrial myopathy, Encephalopathy, Lactic acidosis, and Stroke-like episodes).
Background: The pathophysiology, neuroimaging and treatment of SLEs are thought to be different from that of arterial ischemic stroke in patients with MELAS syndrome. An overlap in acute neuroimaging characteristics have been reported between SLEs and arterial ischemic stroke in terms of diffusion weighted Imaging (DWI) sequences, but the SLEs do not conform to a vascular territory. Here we report a case of MELAS with recurrent arterial strokes.
Design/Methods: Case report study and review of the neuroimaging literature in MELAS.
Results: A 50 year-old right-handed Hispanic man with a history of primary hypoparathyroidism, diabetes mellitus, chronic kidney disease, sensorineural deafness, and short stature presented with global aphasia and unresponsiveness. He had a positive DWI lesion (high DWI/low ADC signal) in the inferior division of the left middle cerebral artery (MCA) in keeping with an acute ischemic stroke. Apart from lactic acidosis, extensive stroke workup was inconclusive for a stroke etiology. He was treated with Aspirin. He presented one month later with recurrent lactic acidosis, right gaze deviation and altered mental status. Repeat MRI showed a new DWI positive lesion in the same vascular territory as the first event. Muscle biopsy revealed ragged red fibers and histology consistent with mitochondrial dysfunction. The diagnosis of MELAS syndrome was made and the patient was treated coenzyme Q10.
Conclusions: Patients with MELAS syndrome may present with recurrent strokes in the arterial territory rather than SLEs. Clinicians should entertain the possibility of a mitochondrial disease in young patients with cryptogenic stroke and pursue appropriate diagnostic evaluations and treatment.
Disclosure: Dr. Liaw has nothing to disclose. Dr. Lewis has nothing to disclose. Dr. Saini has nothing to disclose. Dr. Gultekin has nothing to disclose. Dr. Koch has nothing to disclose. Dr. Asdaghi has nothing to disclose.
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