l-Arginine prevents stroke-like episodes but not brain atrophy: a 20-year follow-up of a MELAS patient
Mitochondrial encephalomyopathy with lactic acidosis and stroke-like episodes (MELAS) usually presents with stroke-like episodes (SLEs), progressive encephalopathy, lactic acidosis, migraine headaches, exercise intolerance, and multisystem involvement . SLEs represent the clinical signature of MELAS with a relapsing-remitting pattern . The prognosis of MELAS is determined by the frequency and severity of SLEs, the progression of diffuse encephalopathy, and the involvement of multiple systems. The pathogenesis of SLEs has not been fully elucidated, but recent investigations have found that SLEs are metabolic stroke driven by seizure activity, and other non-ischemic cellular mechanisms may be involved . Meanwhile, growing evidence has indicated that nitric oxide (NO) deficiency occurs in MELAS syndrome and can contribute significantly to endothelial cell dysfunction . Arginine supplementation has been reported to decrease the frequency and severity of SLEs ....
We thank the patient and her family, as well as the medical staff of the Peking Union Medical College Hospital.
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Conflict of interest
The authors declare that they have no conflict of interest.
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