Abstract:
BACKGROUND AND PURPOSE:We aimed to describe the clinical and imaging features of patients with tumor-like presentation of primary angiitis of the central nervous system. METHODS:We retrospectively analyzed 10 patients enrolled in the French primary angiitis of the central nervous system cohort, who initially presented tumor-like brain lesions and compared them with other patients within the cohort. RESULTS:The 10 patients with tumor-like presentation in the cohort were younger and had more seizures at diagnosis than the other 75 patients (median of 37 [30-48] years versus 46 [18-79] years; P=0.008; 9 [90%] with seizures versus 22 [29%], P<0.001; respectively). All 10 patients had a biopsy (stereotactic procedure in 7 and open-wedge surgery in 3). Histological findings suggestive of vasculitis were observed in 9 patients in whom conventional cerebral angiography and magnetic resonance angiography were negative. In the remaining patient, vascular imaging demonstrated diffuse bilateral large- and medium-sized vessel involvement (biopsy did not reveal vasculitis). All patients with tumor-like presentation received glucocorticoids, combined with cyclophosphamide in 9 cases. With a median follow-up of 27 (12-130) months, 5 (50%) patients relapsed, but achieved remission again after treatment intensification. CONCLUSIONS:Patients with tumor-like presentation of primary angiitis of the central nervous system represent a subgroup characterized with mainly small-sized vessel disease that requires histological confirmation because vascular imaging is often normal. Although relapses are not uncommon, global outcomes are good under treatment with glucocorticoids and cyclophosphamide.
journal_name
Strokejournal_title
Strokeauthors
de Boysson H,Boulouis G,Dequatre N,Godard S,Néel A,Arquizan C,Detante O,Bloch-Queyrat C,Zuber M,Touzé E,Bienvenu B,Aouba A,Guillevin L,Naggara O,Pagnoux C,French Vasculitis Study Group.doi
10.1161/STROKEAHA.116.013917subject
Has Abstractpub_date
2016-09-01 00:00:00pages
2401-4issue
9eissn
0039-2499issn
1524-4628pii
STROKEAHA.116.013917journal_volume
47pub_type
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