Contributions
Abstract: P283
Type: Poster
Abstract Category: Clinical aspects of MS - MS Variants
Objective: The aim of this study is to propose revised criteria for the diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Background: CLIPPERS is an inflammatory disorder of unknown origin defined by
1) subacute brainstem signs and symptoms; accompanied by
2) punctate and curvilinear enhancing lesions predominantly involving the pons; with a
3) prompt steroid sensitivity; in
4) absence of alternative diagnosis. When the brain biopsy is performed,
5) perivascular lympho-histiocytic infiltrates are seen. However, since its first description in 2010, multiple sclerosis, primary arteritis of the CNS, primary CNS lymphoma and lymphomatoid granulomatosis could fulfil, at least initially, all these CLIPPERS criteria including histological features.
Methods: In our previously reported French study, included 12 patients, CLIPPERS had a relapsing-remitting course in absence of immunosuppressive therapy. Relapses fitted always the four first criteria and occurred only when steroid dosage was below 20 mg/day. Therefore we propose to add this sixth criterion into the CLIPPERS definition, in order to evaluate its ability to distinguish CLIPPERS from its related diseases.
Results: Among our 22 patients fulfilling the first diagnostic criteria set for CLIPPERS, 4 progressed eventually to a well-defined disease: multiple sclerosis (n=1), primary arteritis of the CNS (n=1), primary CNS lymphoma (n=1), and lymphomatoid granulomatosis (n=1). Except in the patient having lymphomatoid granulomatosis, a brain biopsy was performed during the first attack and fitted the fifth criterion. However, within 2 years after the initial diagnosis of CLIPPERS, relapses did never fit the sixth criterion in all of these four patients.
Conclusions: If we consider that CLIPPERS is new disorder and not a pre stage of well-defined diseases, our results suggest that this sixth criterion may be useful to distinguish CLIPPERS from its related diseases, and should be added in the CLIPPERS criteria.
References:
1. Taieb G, Duflos C, Renard D, et al. (2012) Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 69:847-55.
Disclosure:
Guillaume Taieb: nothing to disclosure
Pierre Labauge: nothing to disclosure
and the French CLIPPERS group: nothing to disclosure
Abstract: P283
Type: Poster
Abstract Category: Clinical aspects of MS - MS Variants
Objective: The aim of this study is to propose revised criteria for the diagnosis of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).
Background: CLIPPERS is an inflammatory disorder of unknown origin defined by
1) subacute brainstem signs and symptoms; accompanied by
2) punctate and curvilinear enhancing lesions predominantly involving the pons; with a
3) prompt steroid sensitivity; in
4) absence of alternative diagnosis. When the brain biopsy is performed,
5) perivascular lympho-histiocytic infiltrates are seen. However, since its first description in 2010, multiple sclerosis, primary arteritis of the CNS, primary CNS lymphoma and lymphomatoid granulomatosis could fulfil, at least initially, all these CLIPPERS criteria including histological features.
Methods: In our previously reported French study, included 12 patients, CLIPPERS had a relapsing-remitting course in absence of immunosuppressive therapy. Relapses fitted always the four first criteria and occurred only when steroid dosage was below 20 mg/day. Therefore we propose to add this sixth criterion into the CLIPPERS definition, in order to evaluate its ability to distinguish CLIPPERS from its related diseases.
Results: Among our 22 patients fulfilling the first diagnostic criteria set for CLIPPERS, 4 progressed eventually to a well-defined disease: multiple sclerosis (n=1), primary arteritis of the CNS (n=1), primary CNS lymphoma (n=1), and lymphomatoid granulomatosis (n=1). Except in the patient having lymphomatoid granulomatosis, a brain biopsy was performed during the first attack and fitted the fifth criterion. However, within 2 years after the initial diagnosis of CLIPPERS, relapses did never fit the sixth criterion in all of these four patients.
Conclusions: If we consider that CLIPPERS is new disorder and not a pre stage of well-defined diseases, our results suggest that this sixth criterion may be useful to distinguish CLIPPERS from its related diseases, and should be added in the CLIPPERS criteria.
References:
1. Taieb G, Duflos C, Renard D, et al. (2012) Long-term outcomes of CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) in a consecutive series of 12 patients. Arch Neurol 69:847-55.
Disclosure:
Guillaume Taieb: nothing to disclosure
Pierre Labauge: nothing to disclosure
and the French CLIPPERS group: nothing to disclosure