The University of Toledo Translation Journal of Medical Sciences 
Rheumatology Abstract, Department of Medicine Research Symposium UTJMS 2023 May 05; 11(1):e1-e1 

Clinical Characteristics, Management, and 
Outcomes of CLIPPERS: A Comprehensive 
Systematic Review of 140 patients from 100 
Studies 
Nicholas R. DelCimmuto1*, Mustafa Al-Chalabi2, Azizullah Beran, MD3; Pratyush 
Pavan Devarasetty1, Asmaa Mhanna, MD3, Ajaz Sheikh, MD2 

1College of Medicine and Life Sciences, The University of Toledo, Toledo, OH 
43614 
2Department of Neurology, The University of Toledo, Toledo, OH 43614 
3Division of Internal Medicine, Department of Medicine, The University of Toledo, 
Toledo, OH 43614 

*Corresponding author: nicholas.delcimmuto@rockets.utoledo.edu

Published: 05 May 2023 

Introduction: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to 
steroids (CLIPPERS) is a rare inflammatory disorder of the central nervous system, characterized by 
symptoms referable to the brainstem and cerebellum such as, diplopia, gait ataxia and cerebellar 
dysarthria. The features and outcomes of CLIPPERS remains uncertain. Wwe conducted this 
comprehensive systematic review to summarize the existing studies that described CLIPPERS in the 
literature and to provide a quantitative assessment on the clinical characteristics, management, and 
outcomes of this rare syndrome. 

Methods: A search of PubMed and Web of Science databases was conducted from inception until 
January 15, 2022, was conducted. We included the cases that reported probable or definite diagnosis of 
CLIPPERS based on Taieb et al.’s criteria. The quality of the included studies was assessed using the 
JBI Critical Appraisal Tool. Descriptive statistics were performed to analyze the studies.   

Results: We identified 140 patients with CLIPPERS (mean age: 46±18 years and males were 60%). The 
average follow-up duration was 32.27±57.8 months. Ataxia was the most common presenting symptom.  
Sixteen percent of the cases were associated with malignancy, mostly hematologic malignancies. The 
overall relapse rate was 59.2%, and the duration of steroid therapy was considerably shorter in the 
relapsed cases than in the non-relapsed (mean 6.19±7.9 vs. 10.14±12.1 days, respectively, P=0.04). The 
overall mortality rate was 10%, but mortality in patients with malignancy was 30% and it was 12% in 
patients with relapses. 

https://dx.doi.org/10.46570/utjms.vol11-2023-523 

https://dx.doi.org/10.46570/utjms.vol11-2023-523
mailto:nicholas.delcimmuto@rockets.utoledo.edu