ArticleNeurology(R) neuroimmunology & neuroinflammation2016
Widespread inflammation in CLIPPERS syndrome indicated by autopsy and ultra-high-field 7T MRI.
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Article in Neurology(R) neuroimmunology & neuroinflammation, 2016. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 27 papers.
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27 citing papers in PubMed, 48 citations in OpenAlex.
- From CLIPPERS to SLIPPERS: a case report supporting the expanding spectrum of steroid-responsive perivascular CNS inflammation.Acta neurologica Belgica · 2026Article
- An Autopsied Case of Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids for Over Four Years.Internal medicine (Tokyo, Japan) · 2025Article
- Role of advanced MRI in diagnosing and evolutive follow-up to treatment in a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).Radiology case reports · 2025Article
- KLHL11-associated rhomboencephalitis presenting as clippers-like syndrome.Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology · 2025Article
- Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies.Journal of neurology · 2024Review
- Supratentorial Lymphocytic Inflammation with Parenchymal Perivascular Enhancement Responsive to Steroids (SLIPPERS)-Does it Really Exist?Brain sciences · 2023Article
- CLIPPERS manifesting with dizziness: Recognizable and treatable.SAGE open medical case reports · 2023Article
- A rare case report of primary Sjögren's syndrome with clinical characteristics similar to those of CLIPPERS.BMC neurology · 2022Article
- Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids: An Acute Presentation.Cureus · 2022Article
- Brain biopsy in patients with CLIPPERS syndrome: why and when.Therapeutic advances in neurological disorders · 2022Article
- CLIPPERS, chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: A challenge in neurological practice, clinical landmarks (Review).Experimental and therapeutic medicine · 2021Review
- [Inflammatory diseases of the central nervous system].Der Radiologe · 2021Article
- CLIPPERS With Exclusive Extra Pontine Involvement.Neurology(R) neuroimmunology & neuroinflammation · 2021Article
- Iron Rims as an Imaging Biomarker in MS: A Systematic Mapping Review.Diagnostics (Basel, Switzerland) · 2020Review
- Two New Cases and Literature Review of CLIPPERS Syndrome with Long-Term Follow-up.Noro psikiyatri arsivi · 2020Article
- Pathology of inflammatory diseases of the nervous system: Human disease versus animal models.Glia · 2020Review
- Autoimmune glial fibrillary acidic protein astrocytopathy with lesions distributed predominantly in the entire spinal cord.Therapeutic advances in neurological disorders · 2020Article
- Suggestions for improving the design of clinical trials in multiple sclerosis-results of a systematic analysis of completed phase III trials.The EPMA journal · 2019Review
- [CLIPPERS syndrome with supratentorial and spinal cord involvement and histological anomalies].Der Nervenarzt · 2019Article
- Assessment of lesions on magnetic resonance imaging in multiple sclerosis: practical guidelines.Brain : a journal of neurology · 2019Review
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Authors and funding
11 authors at 5 institutions in 4 countries.
Morten BlaabjergDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Klemens RuprechtDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Tim SinneckerDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Daniel KondziellaDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Thoralf NiendorfDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Bjørg Morell Kerrn-JespersenDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Mette LindelofDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Hans LassmannDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Bjarne Winther KristensenDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Friedemann PaulDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Zsolt IllesDepartments of Neurology (M.B., Z.I.) and Pathology (B.W.K.), Odense University Hospital, Denmark; Clinical and Experimental Multiple Sclerosis Research Center (K.R., T.S., F.P.), Department of Neurology (K.R., F.P.), NeuroCure Clinical Research Center (T.S., F.P.), and Experimental and Clinical Research Center (T.N., F.P.), Charité-Universitätsmedizin Berlin, Germany; Department of Neurology (T.S.), Universitatsspital Basel, Switzerland; Department of Neurology (D.K.), Rigshospitalet, Copenhagen University Hospital, Denmark; Berlin Ultrahigh Field Facility (B.U.F.F.) (T.N.), Max Delbrueck Center for Molecular Medicine (F.P.), Berlin, Germany; Department of Neurology (B.M.K.-J., M.L.), Herlev Hospital, Denmark; Center for Brain Research (H.L.), Medical University of Vienna, Austria; and Institute of Clinical Research (M.B., B.W.K., Z.I.), University of Southern Denmark, Odense.
Max Delbrück Center · DEMedical University of Vienna · ATHerlev Hospital · DKRigshospitalet · DKUniversity Hospital of Basel · CH
Funding
No grant is acknowledged in the PubMed record.
Abstract
objectiveTo examine if there is widespread inflammation in the brain of patients with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome by using histology and ultra-high-field MRI at 7.0T.
methodsWe performed a detailed neuropathologic examination in 4 cases, including 1 autopsy case, and studied 2 additional patients by MRI at 7.0T to examine (1) extension of inflammation to areas appearing normal on 3.0T MRI, (2) potential advantages of 7.0T MRI compared to 3.0T MRI in reflecting widespread inflammation, perivascular pathology, and axonal damage, and (3) the possibility of lymphoma.
resultsIn the autopsy case, perivascular inflammation dominated by CD4+ T cells was not only detected in the brainstem and cerebellum but also in brain areas with normal appearance on 3.0T MRI, including supratentorial regions and cranial nerve roots. There was no evidence of lymphoma in any of the 4 patients. The 7.0T MRI in clinical remission also revealed supratentorial lesions and perivascular pathology in vivo with contrast-enhancing lesions centered around a small venous vessel. Ultra-high-field MRI at 7.0T disclosed prominent T1 hypointensities in the brainstem, which were not seen on 3.0T MRI. This corresponded to neuropathologic detection of axonal injury in the autopsy case.
conclusionOur findings suggest more widespread perivascular inflammation and postinflammatory axonal injury in patients with CLIPPERS.
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