We report two patients with fatal mitochondrial myopathy, encephalopat
hy, lactic acidosis, and stroke-like episodes (MELAS). Single-photon e
mission computed tomography (SPECT) with I-123-N-iso-propyl-p-iodoamph
etamine was more sensitive to the lesions than CT or MRI. SPECT showed
focal hyperperfusion before or during the stroke and diffuse hypoperf
usion of the brain, sparing the basal ganglia in the terminal stages.
These findings support the theory that metabolic disturbance in the br
ain causes the ''stroke'' in MELAS.