Rezidivierende zerebrale Ischämien bei zerebraler Vaskulitis im Rahmen eines inkompletten POEMS-Syndroms mit M. Castleman

Abstract

The POEMS syndrome is a multisystem disorder characterised by polyneuropathy, organomegaly, endocrinopathy, plasma cell dyscrasia and skin changes. A coincidence with angiofollicular lymphoid hyperplasia (Castleman's disease) exists in about 60% of all cases. The POEMS syndrome may be associated with macroangiopathy and acute vascular obliterations. Most case reports refer to involvement of the coronary and lower limb arteries. There are only few reports dealing with cerebral strokes and POEMS syndrome. We report on a 32-year-old female with an incomplete form of POEMS syndrome with Castleman's disease and associated cerebral vasculitis. After sufficient treatment of the plasma cell dyscrasia, recurrent cerebral ischemias occurred. A stable state was finally reached after primary treatment of the vasculitis with cyclophosphamide.

Ischemic stroke in patients with POEMS syndrome: a case report and comprehensive analysis of literature

“…However, the frequencies of stroke risk factors such as diabetes mellitus, hypertension and atrial fibrillation occurred in our study are similar to or even lower than those in stroke registries [ 54 , 55 ]. In accordance with previous literature, the preponderance of multiple cerebral vessel lesions and coexistence of extracerebral vessel disease in patients of POEMS without stroke risk factors are also more common than those reported in stroke registries [ 13 - 14 , 22 - 24 , 27 - 28 , 38 ]. This phenomenon may suggest that POEMS syndrome has a predisposition towards large-vessel vasculopathy which could only be confirmed by biopsy.…”

“…Moreover, pathologically confirmed vasculitis involving small-sized and medium-sized muscular artery are reported to be directly associated with POEMS syndrome [ 57 , 58 ]. In addition, larger-sized vessels are also suspected to be affected in POEMS syndrome [ 8 , 10 , 22 , 59 ]. Co-existing quasi-moyamoya disease and POEMS syndrome were observed in a patient with sudden-onset intracranial hemorrhage [ 60 ].…”

“…Hori et al [ 38 ] described a patient with Castleman’s disease and POEMS syndrome who presented hypertrophic cranial pachymeningitis and bilateral internal carotid artery occlusion due to suspected vasculitis. Rössler and colleague [ 22 ] reported a patient with Castleman’s disease and POEMS syndrome who presented recurrent ischemic stroke secondary to clinical diagnosed cerebral vasculitis. Garcia et al [ 13 ] described two patients with diffuse stenosis of the intracranial arteries, being suggestive of a non-atherosclerotic cerebral vasculopathy.…”

Cerebral angiitis associated with subarachnoid hemorrhage in Castleman’s disease: report of two cases

“…Rössler M et al reported on a case of young female with POEMS syndrome presented recurrent ischemic stroke due to cerebral vasculitis. In this case, DSA showed the segmental stenosis of proximal ACA [ 9 ]. Garcia T et al described two cases of POEMS syndrome and MCD accompanied with recurrent ischemic stroke, in which DSA showed diffuse non-atherosclerotic segmental stenosis of the intracranial arteries.…”

“…Among these inflammatory biomarkers, IL-6 is well known to play an important role in acute inflammatory response triggered by cerebral ischemia, and an elevated blood IL-6 concentration is associated with poor outcome after stroke [ 11 ]. Moreover, a higher level of serum IL-6 is also associated with an increased risk of recurrent vascular events after stroke [ 9 ]. Despite these conclusive findings in both acute and chronic phases that clearly indicate an important role of IL-6 in stroke patients, these associations are not fully understood.…”