A case of POEMS syndrome presenting craniocervical vascular stenosis

A 54-years-old Japanese man visited local hospital with six-month history of progressive numbness and muscle weakness in his lower limbs. He was diagnosed with POEMS syndrome based on positive serum M-protein, elevated serum VEGF, and splenomegaly. MRI showed multiple cerebral infarcts in the watershed area, and MRA showed complete vascular occlusion of the right internal carotid artery and severe vascular stenosis of the left common carotid artery, suggesting hemodynamic cerebral infarction. He was referred to our department and treated with three courses of Daratumumab-Bortezomib-Dexamethasone (DBd) therapy. After normalization of VEGF was confirmed, right superficial temporal artery-middle cerebral bypass surgery was performed. He has been kept in good condition with improved muscle strength and walking ability, and normalized VEGF level under the maintenance Daratumumab-Lenalidomide-Dexamethasone (DLd) therapy. Because POEMS syndrome-associated vasculopathy can develop even in the very early stage of the disease, intensive evaluation of craniocervical vessels before treatment initiation is very important for treatment selection and risk assessment.