CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases

B M Kerrn-Jespersen, M Lindelof, Zsolt Illes, Morten Blaabjerg, E L Lund, C Klausen, I Christiansen, F Sellebjerg, D Kondziella

Research output: Contribution to journalJournal articleResearchpeer-review

Abstract

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is an inflammatory CNS disorder characterized by 1) subacute onset of cerebellar and brainstem symptoms, 2) peripontine contrast-enhancing perivascular lesions with a "salt-and-pepper" appearance on MRI, and 3) angiocentric, predominantly T-lymphocytic infiltration as revealed by brain biopsy. Inflammatory diseases including neuroinfections, CNS lymphoma and neurosarcoidosis must be excluded. Since CLIPPERS was described in 2010, many patients might have been misdiagnosed in the past. We therefore searched medical records from a large tertiary neurological center, the Department of Neurology at Rigshospitalet, Copenhagen University Hospital, for patients discharged between 1999 and 2013 with a diagnosis of "sarcoidosis with other localization", "other acute disseminating demyelination", "other demyelinating disease in the CNS" or "encephalitis, myelitis or encephalomyelitis". Of 206 identified patients, 24 had been examined by brain biopsy and were included for further evaluation. Following clinical, neuroradiological and neuropathological review, 3 patients (12.5%) were reclassified as having CLIPPERS. Median long-term follow-up was 75 months. The present results suggest that clinical re-evaluation of patients previously diagnosed with unspecified inflammatory demyelinating CNS disease or atypical neurosarcoidosis may increase the detection rate of CLIPPERS. Further, potentially severe neurological deficits and progressive parenchymal atrophy on MRI may suggest neurodegenerative features, which emphasizes the need for early immunomodulatory treatment.

Original languageEnglish
JournalJournal of the Neurological Sciences
Volume343
Issue number1-2
Pages (from-to)224-7
ISSN0022-510X
DOIs
Publication statusPublished - 15. Aug 2014

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Central Nervous System Diseases
Demyelinating Diseases
Sarcoidosis
Neurology
Diagnostic Errors
Medical Records
Lymphoma
Salts

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Kerrn-Jespersen, B M ; Lindelof, M ; Illes, Zsolt ; Blaabjerg, Morten ; Lund, E L ; Klausen, C ; Christiansen, I ; Sellebjerg, F ; Kondziella, D. / CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases. In: Journal of the Neurological Sciences. 2014 ; Vol. 343, No. 1-2. pp. 224-7.
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abstract = "Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is an inflammatory CNS disorder characterized by 1) subacute onset of cerebellar and brainstem symptoms, 2) peripontine contrast-enhancing perivascular lesions with a {"}salt-and-pepper{"} appearance on MRI, and 3) angiocentric, predominantly T-lymphocytic infiltration as revealed by brain biopsy. Inflammatory diseases including neuroinfections, CNS lymphoma and neurosarcoidosis must be excluded. Since CLIPPERS was described in 2010, many patients might have been misdiagnosed in the past. We therefore searched medical records from a large tertiary neurological center, the Department of Neurology at Rigshospitalet, Copenhagen University Hospital, for patients discharged between 1999 and 2013 with a diagnosis of {"}sarcoidosis with other localization{"}, {"}other acute disseminating demyelination{"}, {"}other demyelinating disease in the CNS{"} or {"}encephalitis, myelitis or encephalomyelitis{"}. Of 206 identified patients, 24 had been examined by brain biopsy and were included for further evaluation. Following clinical, neuroradiological and neuropathological review, 3 patients (12.5{\%}) were reclassified as having CLIPPERS. Median long-term follow-up was 75 months. The present results suggest that clinical re-evaluation of patients previously diagnosed with unspecified inflammatory demyelinating CNS disease or atypical neurosarcoidosis may increase the detection rate of CLIPPERS. Further, potentially severe neurological deficits and progressive parenchymal atrophy on MRI may suggest neurodegenerative features, which emphasizes the need for early immunomodulatory treatment.",
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Kerrn-Jespersen, BM, Lindelof, M, Illes, Z, Blaabjerg, M, Lund, EL, Klausen, C, Christiansen, I, Sellebjerg, F & Kondziella, D 2014, 'CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases', Journal of the Neurological Sciences, vol. 343, no. 1-2, pp. 224-7. https://doi.org/10.1016/j.jns.2014.06.002

CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases. / Kerrn-Jespersen, B M; Lindelof, M; Illes, Zsolt; Blaabjerg, Morten; Lund, E L; Klausen, C; Christiansen, I; Sellebjerg, F; Kondziella, D.

In: Journal of the Neurological Sciences, Vol. 343, No. 1-2, 15.08.2014, p. 224-7.

Research output: Contribution to journalJournal articleResearchpeer-review

TY - JOUR

T1 - CLIPPERS among patients diagnosed with non-specific CNS neuroinflammatory diseases

AU - Kerrn-Jespersen, B M

AU - Lindelof, M

AU - Illes, Zsolt

AU - Blaabjerg, Morten

AU - Lund, E L

AU - Klausen, C

AU - Christiansen, I

AU - Sellebjerg, F

AU - Kondziella, D

N1 - Copyright © 2014 Elsevier B.V. All rights reserved.

PY - 2014/8/15

Y1 - 2014/8/15

N2 - Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is an inflammatory CNS disorder characterized by 1) subacute onset of cerebellar and brainstem symptoms, 2) peripontine contrast-enhancing perivascular lesions with a "salt-and-pepper" appearance on MRI, and 3) angiocentric, predominantly T-lymphocytic infiltration as revealed by brain biopsy. Inflammatory diseases including neuroinfections, CNS lymphoma and neurosarcoidosis must be excluded. Since CLIPPERS was described in 2010, many patients might have been misdiagnosed in the past. We therefore searched medical records from a large tertiary neurological center, the Department of Neurology at Rigshospitalet, Copenhagen University Hospital, for patients discharged between 1999 and 2013 with a diagnosis of "sarcoidosis with other localization", "other acute disseminating demyelination", "other demyelinating disease in the CNS" or "encephalitis, myelitis or encephalomyelitis". Of 206 identified patients, 24 had been examined by brain biopsy and were included for further evaluation. Following clinical, neuroradiological and neuropathological review, 3 patients (12.5%) were reclassified as having CLIPPERS. Median long-term follow-up was 75 months. The present results suggest that clinical re-evaluation of patients previously diagnosed with unspecified inflammatory demyelinating CNS disease or atypical neurosarcoidosis may increase the detection rate of CLIPPERS. Further, potentially severe neurological deficits and progressive parenchymal atrophy on MRI may suggest neurodegenerative features, which emphasizes the need for early immunomodulatory treatment.

AB - Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is an inflammatory CNS disorder characterized by 1) subacute onset of cerebellar and brainstem symptoms, 2) peripontine contrast-enhancing perivascular lesions with a "salt-and-pepper" appearance on MRI, and 3) angiocentric, predominantly T-lymphocytic infiltration as revealed by brain biopsy. Inflammatory diseases including neuroinfections, CNS lymphoma and neurosarcoidosis must be excluded. Since CLIPPERS was described in 2010, many patients might have been misdiagnosed in the past. We therefore searched medical records from a large tertiary neurological center, the Department of Neurology at Rigshospitalet, Copenhagen University Hospital, for patients discharged between 1999 and 2013 with a diagnosis of "sarcoidosis with other localization", "other acute disseminating demyelination", "other demyelinating disease in the CNS" or "encephalitis, myelitis or encephalomyelitis". Of 206 identified patients, 24 had been examined by brain biopsy and were included for further evaluation. Following clinical, neuroradiological and neuropathological review, 3 patients (12.5%) were reclassified as having CLIPPERS. Median long-term follow-up was 75 months. The present results suggest that clinical re-evaluation of patients previously diagnosed with unspecified inflammatory demyelinating CNS disease or atypical neurosarcoidosis may increase the detection rate of CLIPPERS. Further, potentially severe neurological deficits and progressive parenchymal atrophy on MRI may suggest neurodegenerative features, which emphasizes the need for early immunomodulatory treatment.

U2 - 10.1016/j.jns.2014.06.002

DO - 10.1016/j.jns.2014.06.002

M3 - Journal article

C2 - 24954086

VL - 343

SP - 224

EP - 227

JO - Journal of the Neurological Sciences

JF - Journal of the Neurological Sciences

SN - 0022-510X

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ER -