Irreversible Vasculopathy Proceeds Rapidly in POEMS Syndrome

Abstract

A 48-year-old woman with polyneuropathy, organomegaly, endocrinopathy, M-protein, skin change (POEMS) syndrome suddenly presented with numbness of her right upper limb. Magnetic resonance imaging showed multiple acute infarctions in her left cerebrum, and magnetic resonance angiography (MRA) showed multiple intra-cranial vascular lesions, which contrasted with previously normal MRA results obtained eight months prior to the stroke. After completing successful treatment for POEMS syndrome, there were no recurrent stroke episodes. A six-month follow-up scan showed that although the vascular lesions did not progress, they did not improve much either. POEMS syndrome is associated with the rapid extension of large blood vessels-vasculopathy-resulting in nearly irreversible brain lesions.

Keywords: POEMS syndrome; ischemic stroke; vasculopathy.

Figure.

A: Brain magnetic resonance angiography (MRA) scans taken eight months prior to the first admission. No specific vascular lesions can be seen. B: Diffusion-weighted magnetic resonance imaging (MRI) scans obtained on the day of the stroke onset. Multiple acute ischemic stroke lesions can be seen in the left cerebrum (arrow). C: Brain MRA scans taken on the day of the stroke onset. The right internal carotid artery (ICA), right middle cerebral artery (MCA), and bilateral anterior cerebral artery (ACA) were found to be occluded. D: Brain-carotid MRA scan taken three days after the stroke onset. The right ICA is not depicted from its origin (arrowhead), and a tandem stenotic lesion in left ICA was detected (arrow). The proximal vascular stenosis of the tandem lesion was not detected by brain MRA taken on the day of the stroke onset. E, F: Brain MRA scans taken six months after the stroke onset. The bilateral ACAs, left MCA, and proximal stenosis of the tandem lesion (arrow) showed mild improvement.