Nicholas L Zalewski1, W Oliver Tobin2,3. 1. Department of Neurology, Mayo Clinic, Rochester, MN, USA. 2. Department of Neurology, Mayo Clinic, Rochester, MN, USA. Tobin.Oliver@mayo.edu. 3. Mayo Clinic College of Medicine, Rochester, MN, USA. Tobin.Oliver@mayo.edu.
Abstract
PURPOSE OF REVIEW: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described treatable, inflammatory, brainstem predominant encephalomyelitis. The diagnosis of CLIPPERS is challenging without a specific biomarker, and thus it is important to consider if both the clinical and radiographic features are consistent with the diagnosis, or rather a disease mimicker. RECENT FINDINGS: Many patients with CLIPPERS-like lesions have been described in the literature with follow-up revealing a range of alternative diagnoses, such as malignancies, vasculitis, and other specific inflammatory diseases. As a result, some have proposed that CLIPPERS might represent a pre-malignancy state or simply an initial clinical syndrome of a variety of possible etiologies. We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. A recommended diagnostic evaluation and initial treatment plan is provided.
PURPOSE OF REVIEW: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described treatable, inflammatory, brainstem predominant encephalomyelitis. The diagnosis of CLIPPERS is challenging without a specific biomarker, and thus it is important to consider if both the clinical and radiographic features are consistent with the diagnosis, or rather a disease mimicker. RECENT FINDINGS: Many patients with CLIPPERS-like lesions have been described in the literature with follow-up revealing a range of alternative diagnoses, such as malignancies, vasculitis, and other specific inflammatory diseases. As a result, some have proposed that CLIPPERS might represent a pre-malignancy state or simply an initial clinical syndrome of a variety of possible etiologies. We describe the typical clinical, radiographic, and pathological features of CLIPPERS and emphasize consideration for alternative diagnoses when findings are not classic. A recommended diagnostic evaluation and initial treatment plan is provided.
Authors: Joanne L Jones; Andrew F Dean; Nagui Antoun; Daniel J Scoffings; Neil G Burnet; Alasdair J Coles Journal: Brain Date: 2011-06-07 Impact factor: 13.501
Authors: Hjalmar J De Graaff; Mike P Wattjes; Annemieke J Rozemuller-Kwakkel; Axel Petzold; Joep Killestein Journal: JAMA Neurol Date: 2013-07 Impact factor: 18.302