Aβ-related angiitis

Comparison with CAA without inflammation and primary CNS vasculitis

Carlo Salvarani, Gene G. Hunder, Jonathan M. Morris, Robert D Jr. Brown, Teresa Christianson, Caterina Giannini

Research output: Contribution to journalArticle

62 Citations (Scopus)

Abstract

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.

Original languageEnglish (US)
Pages (from-to)1596-1603
Number of pages8
JournalNeurology
Volume81
Issue number18
DOIs
StatePublished - Oct 29 2013

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Cerebral Amyloid Angiopathy
Central Nervous System Vasculitis
Vasculitis
Amyloid
Blood Vessels
Inflammation
Gadolinium
Cognition
Intracranial Hemorrhages
Cerebral Infarction
Cerebral Hemorrhage
Paresis
Neurologic Manifestations
Seizures
Central Nervous System
Peptides
Mortality
Proteins
Deposition
Enhancement

ASJC Scopus subject areas

  • Clinical Neurology
  • Arts and Humanities (miscellaneous)
  • Medicine(all)

Cite this

Aβ-related angiitis : Comparison with CAA without inflammation and primary CNS vasculitis. / Salvarani, Carlo; Hunder, Gene G.; Morris, Jonathan M.; Brown, Robert D Jr.; Christianson, Teresa; Giannini, Caterina.

In: Neurology, Vol. 81, No. 18, 29.10.2013, p. 1596-1603.

Research output: Contribution to journalArticle

Salvarani, Carlo ; Hunder, Gene G. ; Morris, Jonathan M. ; Brown, Robert D Jr. ; Christianson, Teresa ; Giannini, Caterina. / Aβ-related angiitis : Comparison with CAA without inflammation and primary CNS vasculitis. In: Neurology. 2013 ; Vol. 81, No. 18. pp. 1596-1603.
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