Primary central nervous system vasculitis mimicking brain tumor: Comprehensive analysis of 13 cases from a single institutional cohort of 191 cases.
J Autoimmun
; 97: 22-28, 2019 02.
Article
in En
| MEDLINE
| ID: mdl-30528173
ABSTRACT
OBJECTIVE:
To describe the clinical, laboratory, and imaging features and course of patients with primary central nervous system vasculitis (PCNSV) presenting with an intracranial tumor-like mass (TLM).METHODS:
We retrospectively studied a cohort of 191 consecutive patients with PCNSV seen at the Mayo Clinic, Rochester, MN over a 35-year period (1982-2017). 13/191 patients presented with a TLM. We compared the findings in these 13 patients with those from the 178 without this presentation.RESULTS:
In 13 of 191 (6.8%) patients with TLM the diagnosis of PCNSV was established by cerebral biopsy. Granulomatous vasculitis was found in 11/13 patients, accompanied by vascular deposits of ß-amyloid peptide in 7. Compared to the 178 patients without TLM, the patients with TLM were more likely to be male (pâ¯=â¯0.04), and less likely to have a transient ischemic attack (pâ¯=â¯0.023), bilateral cerebral infarcts (pâ¯=â¯0.018), or vasculitic lesions on angiography (pâ¯=â¯0.045). They were more likely to have seizures (pâ¯=â¯0.022), gadolinium-enhanced lesions (pâ¯=â¯0.007), and amyloid angiopathy (pâ¯=â¯0.046). All 13 patients responded to therapy and 8/13 (61.5%) had a Rankin disability score of 0â¯at last visit. Overall, high disability scores (Rankin scores 4-6) at last follow-up were associated with increasing age (odds ratio, OR, 1.49) and cerebral infarction (OR, 3.47), but were less likely in patients with gadolinium-enhanced lesions (OR, 0.36) and amyloid angiopathy (OR, 0.21).CONCLUSION:
In PCNSV a TLM at presentation represents a definable subgroup of patients with a favourable treatment response.Key words
Full text:
1
Collection:
01-internacional
Database:
MEDLINE
Main subject:
Brain Neoplasms
/
Vasculitis, Central Nervous System
Type of study:
Diagnostic_studies
/
Etiology_studies
/
Observational_studies
/
Prognostic_studies
/
Risk_factors_studies
Limits:
Adult
/
Aged
/
Aged80
/
Female
/
Humans
/
Male
/
Middle aged
Language:
En
Journal:
J Autoimmun
Journal subject:
ALERGIA E IMUNOLOGIA
Year:
2019
Type:
Article