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Migraine-like headache is the most common presenting symptom of stroke-like episode in patients with mitochondrial myopathy, encephalopathy, lactic acidosis, and stroke-like episodes (MELAS).1,2⇓ This study was designed to investigate the mechanism of headache in patients with MELAS and based on our recent report that neuronal hyperexcitability might initiate stroke-like episodes in MELAS.2
Methods.
After obtaining informed consent, sumatriptan 3 mg was subcutaneously injected for intractable headache under serial EKG monitoring in two patients with MELAS, who carried the A3243G transition in transfer RNALeu(UUR) gene, when headache intensity exceeded 8/10 of the Headache Scale. The clinical data obtained from a patient, who received an injection during the stroke-like episode, were compared with those in our series of 15 consecutive stroke-like episodes of six patients with MELAS, who did not receive injections during the episodes.2,3⇓
Results.
The first patient was a 40-year-old woman with incomplete phenotype of MELAS who had never had stroke-like episode but otherwise had typical manifestations. Since age 31, she had headaches characterized by episodic, bilateral, and throbbing pain with moderate or severe intensity and …
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