PRIMARY ANGIITIS OF THE CNS DIAGNOSED BY ANGIOGRAPHY

Sixteen patients (8 female, 8 male) with primary angiitis of the CNS ( PACNS), were followed prospectively in a vasculitis clinic. Diagnosis was by angiography in patients without underlying disease. Median age at diagnosis was 36.5 years, and median duration of follow-up was 28 m onths. Onset was acute in 14 patients (88%), with 3.5 weeks (median) f rom onset symptoms to diagnosis. Three women developed symptoms within 3 weeks postpartum. The most frequent symptoms were severe headaches (12, 75%), stroke (6, 30%), transient ischaemic attack (TIA) (4, 28%), seizures (7, 44%), visual aberration (3, 19%), and cognitive impairme nt (5, 31%). Laboratory data included high ESR (2, 13%), leucocytosis (8, 80%), thrombocytosis (1, 6%), positive antinuclear antibody titre (3, 15%), and high levels of complement (5, 31%). Lumbar puncture was performed in 12 patients (75%). CSF analysis was abnormal in five pati ents (42%). EEC was abnormal in 5/9 patients. The major CT/MRI scan fi ndings were cerebral haemorrhage (4, 25%), brain infarcts (5, 31%), br ain atrophy (2, 13%) and nonspecific lesions (2, 13%). Four patients h ad normal studies. All patients received corticosteroids (CS), and fiv e were treated with oral cyclophosphamide, Two patients relapsed despi te CS and cyclophosphamide therapy. All patients are alive, and at the last assessment, eight had a permanent neurological deficit, which in cluded paresis (3, 19%), neurocognitive abnormalities (2, 13%), visual loss (2, 13%) and seizure activity (5, 31%). Our data suggest a non-p rogressive, non-fatal course in those PACNS patients diagnosed angiogr aphically and treated with CS with or without cyclophosphamide.