title: Isolated PACNS-like presentation of a systemic vasculitis complicating a myelodysplastic syndrome.
abstract: Myelodysplastic syndromes (MDS) are a series of haematological malignancies ranging from chronic refractory anaemia to leukaemia. There is increasing recognition of immunological abnormalities in patients with MDS, including few reports of cutaneous vasculitis; in no instance, a cerebral localization has been ascertained. Here, the case of a patient with MDS who presented exclusively with neurological signs that were considered indicative of a primary, isolated central nervous system vasculitis (PACNS) is reported. Although histological findings on brain tissue confirmed a small-vessel vasculitis, this had to be considered in the context of a systemic vasculitis. In fact, at autopsy, an involvement of skin, myocardium, lungs, liver, kidney and bone marrow was also found. An autoimmune vasculitis should be included in the differential diagnosis of acute-onset, isolated, cerebral symptoms complicating the course of MDS.
authors.affiliation: Dipartimento di Scienze Gerontologiche, Geriatriche e Fisiatriche, Centro di Medicina dell'Invecchiamento, Università Cattolica del Sacro Cuore, Rome, Italy.
authors.forename: R A
authors.initials: RA
authors.lastname: Incalzi
authors.forename: V
authors.initials: V
authors.lastname: Arena
authors.forename: A
authors.initials: A
authors.lastname: Capelli
authors.forename: G
authors.initials: G
authors.lastname: Gambassi
mesh_terms: D000368:Aged; D003331:Coronary Vessels / Q000473:pathology; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D009190:Myelodysplastic Syndromes / Q000150:complications* / Q000188:drug therapy / Q000473:pathology; D020293:Vasculitis, Central Nervous System / Q000150:complications* / Q000188:drug therapy / Q000473:pathology
chemical_list: D007166:Immunosuppressive Agents