Catastrophic primary central nervous system vasculitis.
Primary central nervous system vasculitis (PCNSV) is an uncommon condition that affects the brain and the spinal cord. It is heterogeneous in presenting characteristics and outcomes. We report a patient with a catastrophic rapidly progressive course refractory to intensive treatment with pulses of methylprednisolone and iv cyclophosphamide. The condition rapidly deteriorated and the patient died 6 weeks after presentation. Rapidly progressive PCNSV represents the worst end of the clinical spectrum of PCNSV. These patients are characterised by bilateral, multiple, large cerebral vessel lesions on angiograms and multiple bilateral cerebral infarctions.
Unità Operativa di Reumatologia, Azienda Ospedaliera ASMN, IRCCS di Reggio Emilia, Italy. salvarani.carlo@asmn.re.it.
Carlo
C
Salvarani
Robert D
RD
Brown
Jonathan M
JM
Morris
John
J
Huston
Gene G
GG
Hunder
D020520:Brain Infarction / Q000175:diagnosis / Q000209:etiology; D002533:Cerebral Angiography / Q000379:methods; D003520:Cyclophosphamide / Q000008:administration & dosage; D018450:Disease Progression; D017809:Fatal Outcome; D006801:Humans; D007166:Immunosuppressive Agents / Q000008:administration & dosage; D018810:Magnetic Resonance Angiography / Q000379:methods; D008297:Male; D008775:Methylprednisolone / Q000008:administration & dosage; D008875:Middle Aged; D018696:Neuroprotective Agents / Q000008:administration & dosage; D049268:Positron-Emission Tomography / Q000379:methods; D020293:Vasculitis, Central Nervous System / Q000150:complications / Q000188:drug therapy
D007166:Immunosuppressive Agents; D018696:Neuroprotective Agents; D003520:Cyclophosphamide; D008775:Methylprednisolone