Muscle Study Group
J. Hamel, E. Ciafaloni (Rochester, NY)
Title: Temporal and spatial evolution of stroke-like lesions on MRI in MELAS
Introduction: Stroke-like lesions in MELAS (Mitochondrial encephalomyopathy, lactic acidosis, stroke-like episodes) can evolve over time. The pathogenesis is not well understood.
Objectives/Case Report: A 34-year-old man with MELAS (3243A>G mutation) presented with cortical blindness and lactic acidosis after 2 weeks of treatment with Metformin and discontinuation of arginine and coenzyme Q10.
Methods: Description of the clinical progression and evolution on MR-imaging.
Results: 14 hours after symptom onset, the MRI was normal. Serial imaging over 2-3 days revealed development of restricted diffusion and hyperperfusion in the bilateral occipital lobes. Treatment with IV arginine resulted in some initial improvement. 10 days later, he developed status epilepticus with progression of the lesion to the right temporal lobe.
Conclusions: Stroke-like episodes differ clinically and radiologically from ischemic strokes. Normal early imaging should not delay arginine treatment and sufficient seizure management is important. The role of metformin as a potential trigger in this case will be discussed.
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