Immunohistochemical study of intracranial arteries.

<p>A and D: immunoreactivity indicating that α-SMA is detected in the thickened intima of MCA of patients with MMD (black arrows), <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g005" target="_blank">Figure 5D</a> is an enlarged region (box) of <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g005" target="_blank">Figure 5A</a>; B and E: immunoreactivity indicating that S100A4 protein is detected in the thickened intima and media smooth muscle cells (black arrows), <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g005" target="_blank">Figure 5E</a> is an enlarged region (box) of <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g005" target="_blank">Figure 5B</a>; F: vascular smooth muscle cells, especially the damaged internal elastic lamina, showed intense immunoreactivity of IgG (black arrows); C: the same area of <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g005" target="_blank">Figure 5F</a>, black arrows indicate the stratified internal elastic lamina (Ponceau/Victoria blue staining). The red stars indicate lumen of the blood vessels. (A, B) ×40, (C, D, E, F) ×200.</p

Photomicrographs of brain vessels in MMD patients.

<p>A and B: narrow lumen with intimal thickening and attenuation of media in middle cerebral artery (Ponceau/Victoria blue staining, A: ×40, B: ×100), black arrows indicate stratified internal elastic lamina, <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g004" target="_blank">Figure 4B</a> is an enlarged region (box) of <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g004" target="_blank">Figure 4A</a>; C and D: vacuolar degeneration (black arrows) in cerebrovascular smooth muscle cells (H&E staining, C: ×40, D: ×100), <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g004" target="_blank">Figure 4D</a> is an enlarged region (box) of <a href="http://www.plosone.org/article/info:doi/10.1371/journal.pone.0036386#pone-0036386-g004" target="_blank">Figure 4C</a>.</p

Macroscopical and microscopical findings at autopsy.

<p>A: malformation of the vascular network around the proximal middle cerebral artery (white arrow); B: fine vascular network indicated by black arrows (Ponceau/Victoria blue staining, ×100); C: subarachnoid hemorrhage in the right parieto-temporal area; D: subarachnoid hemorrhage in right parietal lobe (H&E staining, ×100); yellow stars indicate the hemorrhagic area.</p

Summary of patients with MMD in Chaoshan District.^a

a<p>No: number.</p

Digital subtraction angiogram (DSA)showing stenosis of the distal portion of the ICA (white arrow).

<p>Digital subtraction angiogram (DSA)showing stenosis of the distal portion of the ICA (white arrow).</p

Kaplan-Meier plot for stroke-free survival after treatment.

<p>Kaplan-Meier plot for stroke-free survival after treatment.</p

Comparison of MMD in different districts.^a

a<p>No: number; F: female; M: male; NA: not available;</p>*<p>including 65 MMD patients in our institutions.</p

Onset age distribution of MMD in different districts.

<p>Onset age distribution of MMD in different districts.</p