TY - JOUR
T1 - Aβ-related angiitis
T2 - Comparison with CAA without inflammation and primary CNS vasculitis
AU - Salvarani, Carlo
AU - Hunder, Gene G.
AU - Morris, Jonathan M.
AU - Brown, Robert D.
AU - Christianson, Teresa
AU - Giannini, Caterina
PY - 2013/10/29
Y1 - 2013/10/29
N2 - Objective: To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-b (Ab) deposition with and without inflammatory vascular infiltration. Methods: We report 78 consecutive patients with cerebral vascular Ab deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Ab peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Ab-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Ab seen over 25 years (1983 through 2007). Results: Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRAwere older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar. Conclusions: ABRA appears to represent a distinct subset of PCNSV.
AB - Objective: To analyze the clinical findings, response to therapy, and outcomes of patients with cerebral vascular amyloid-b (Ab) deposition with and without inflammatory vascular infiltration. Methods: We report 78 consecutive patients with cerebral vascular Ab deposition examined at Mayo Clinic Rochester over 25 years (1987 through 2011). Specimens reviewed by a neuropathologist showed 40 with vascular Ab peptide without inflammation (cerebral amyloid angiopathy [CAA]), 28 with granulomatous vasculitis (Ab-related angiitis or ABRA), and 10 with perivascular CAA-related inflammation. We also matched findings in 118 consecutive patients with primary CNS vasculitis (PCNSV) without Ab seen over 25 years (1983 through 2007). Results: Compared to the 40 with CAA, the 28 with ABRA were younger at diagnosis (p = 0.05), had less altered cognition (p = 0.02), fewer neurologic deficits (p = 0.02), and fewer intracranial hemorrhages (<0.001), but increased gadolinium leptomeningeal enhancement (p = 0.01) at presentation, and less mortality and disability at last follow-up (p < 0.001). Compared with PCNSV, the 28 patients with ABRAwere older at diagnosis (p < 0.001), had a higher frequency of altered cognition (p = 0.05), seizures/spells (p = 0.006), gadolinium leptomeningeal enhancement (p < 0.001), and intracerebral hemorrhage (p = 0.02), lower frequency of hemiparesis (p = 0.01), visual symptoms (p = 0.04), and MRI evidence of cerebral infarction (p = 0.003), but higher CSF protein levels (p = 0.03). Results of treatment and outcomes in ABRA and PCNSV were similar. Conclusions: ABRA appears to represent a distinct subset of PCNSV.
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U2 - 10.1212/WNL.0b013e3182a9f545
DO - 10.1212/WNL.0b013e3182a9f545
M3 - Article
C2 - 24078731
AN - SCOPUS:84888274155
SN - 0028-3878
VL - 81
SP - 1596
EP - 1603
JO - Neurology
JF - Neurology
IS - 18
ER -