Presentation
Acute confusion and agitation. A known case of diabetes mellitus type I.
Patient Data
The MR study shows the following:
right frontal multiple cortical and subcortical patchy areas of diffusion restriction consistent with acute infarcts
smaller multifocal lesions of diffusion restriction are seen involving the left frontal cortical and peri ventricular regions and right caudate nucleus head consistent with acute infarcts
bilateral cerebral small vessel disease with left frontal cortical gliotic changes
TOF study without contrast revealed asymmetry of the flow-related signal of the internal carotid, anterior, and middle cerebral arteries suggesting underlying vascular disease
motion artifacts couldn't be avoided due to acute confusion and agitation
CTA shows the following
marked stenosis of the terminal right internal carotid artery immediately beyond the origin of the PCOM branch
long segmental marked stenosis of the M1 segment of the right MCA. A collateral network refills the terminal M1 and the rest of the right MCA segments
stenotic A1 segment of the right ACA with ACOM refills the A2 segment
moderate stenosis of the terminal left internal carotid artery at the origin of the left MCA
short segmental marked stenosis of the M1 segment of the left MCA. A collateral network refills the M1 and the rest of the left MCA segments
Case Discussion
CT evidence of vasculo-occlusive disease involving the terminal internal carotid arteries, proximal, middle cerebral arteries, and proximal right anterior cerebral artery with variable degrees of stenosis and collaterals formation.
This CT picture suggests Moyamoya syndrome (a term used to describe when Moyamoya disease occurs in conjunction with other systemic disorders, one of the associations is type 1 diabetes).
The reason for this association is yet to be understood, with many hypotheses pointing toward an autoimmune etiology 1,2.
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