TY - JOUR T1 - A Remarkable Pattern of Subcortical Vessel Wall Enhancement in Granulomatous Angiitis of the Central Nervous System JF - The Journal of Rheumatology JO - J Rheumatol SP - 2051 LP - 2053 DO - 10.3899/jrheum.120543 VL - 39 IS - 10 AU - NIELS SCHOENMAKER AU - MENNO R. GERMANS AU - DIRK TROOST AU - EDO RICHARD Y1 - 2012/10/01 UR - http://www.jrheum.org/content/39/10/2051.abstract N2 - To the Editor:Primary angiitis of the central nervous system (PACNS) is an idiopathic disorder. Clinical as well as radiological characteristics are nonspecific and heterogenic, making diagnosis delicate. We report an unusual pattern of extensive radial enhancement of the deep cerebral vasculature on magnetic resonance imaging (MRI) in a previously healthy 64-year-old woman.Our patient presented with a 6-month history of seizures, weight loss, difficulty walking, and bilateral deterioration of vision. She and her partner had noticed progressive cognitive decline with decreased mental speed and lack of initiative. She scored 29/30 on the Mini Mental State Examination with remarkable mental slowness. Visual acuity was 20/40 (right) and 20/32 (left) with bilateral optic atrophy. She had asymmetrical parkinsonism with myoclonus of both hands and presence of primitive reflexes.MRI of the brain demonstrated symmetrical white matter abnormalities (Figure 1A) with … Address correspondence to Dr. N. Schoenmaker, Department of Neurology, Academic Medical Centre, PO Box 22660, 1100 DD Amsterdam, The Netherlands. E-mail: n.schoenmaker{at}amc.uva.nl ER -