Uveitis associated with Primary Angiitis of the Central Nervous System.
BACKGROUND
In Primary Angiitis of the Central Nervous System (PACNS), disease is typically limited to the brain and spinal cord although other organs may be affected. Uveitis is occasionally seen in systemic vasculitides but is not a recognized manifestation of PACNS. We describe two patients who developed PACNS following the onset of uveitis.


METHODS
Case 1--a 47-year-old male suffered multiple TIAs and left pontine stroke shortly after two episodes of diffuse uveitis. A cerbral angiogram demonstrated multiple caliber changes within several intracranial vessels. Cyclophosphamide was added after his stroke occurred during pulse methylprednisolone therapy. Case 2--a 35-year-old male suffered a spinal cord TIA followed by hemispheric and brainstem infarctions two months after an episode of uveitis and Bell's palsy treated with oral prednisone. A cerebral angiogram demonstrated multiple caliber changes within several intracranial vessels. He was successfully treated with oral prednisone and cyclophosphamide.


CONCLUSIONS
Uveitis should be considered a recognized feature of PACNS. Combination immunosuppressive therapy with prednisone and cyclophosphamide may be necessary for successful treatment.
Woolfenden
A R
AR
Division of Neurology, Department of Medicine, University of British Columbia, Vancouver, Canada.
Wade
N K
NK
Tang
P
P
Chalmers
A
A
Reid
G
G
Teal
P A
PA
D000328:Adult; D020526:Brain Stem Infarctions / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D002533:Cerebral Angiography; D002536:Cerebral Arteries / Q000000981:diagnostic imaging / Q000473:pathology* / Q000503:physiopathology; D002544:Cerebral Infarction / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D003520:Cyclophosphamide / Q000627:therapeutic use; D006801:Humans; D007166:Immunosuppressive Agents / Q000627:therapeutic use; D002546:Ischemic Attack, Transient / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D008297:Male; D008875:Middle Aged; D020760:Spinal Cord Ischemia / Q000188:drug therapy / Q000209:etiology / Q000503:physiopathology; D013256:Steroids / Q000627:therapeutic use; D020521:Stroke / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D016896:Treatment Outcome; D014605:Uveitis / Q000188:drug therapy / Q000209:etiology* / Q000503:physiopathology; D020293:Vasculitis, Central Nervous System / Q000150:complications* / Q000188:drug therapy / Q000503:physiopathology
D007166:Immunosuppressive Agents; D013256:Steroids; D003520:Cyclophosphamide