Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the CNS with a predilection for the hindbrain and responsive to immunotherapy. Five further cases are described with detailed pathology and long te...
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| Veröffentlicht in: | Journal of Neurology, Neurosurgery and Psychiatry Neurosurgery and Psychiatry, 2012-01, Vol.83 (1), p.15-22 |
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| container_title | Journal of Neurology, Neurosurgery and Psychiatry |
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| creator | Simon, Neil G Parratt, John D Barnett, Michael H Buckland, Michael E Gupta, Ruta Hayes, Michael W Masters, Lynette T Reddel, Stephen W |
| description | Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the CNS with a predilection for the hindbrain and responsive to immunotherapy. Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent a benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. We propose that the title of the syndrome be amended to chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids to more accurately reflect the distribution of the radiological findings. |
| doi_str_mv | 10.1136/jnnp-2011-301054 |
| format | Article |
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Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent a benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. We propose that the title of the syndrome be amended to chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids to more accurately reflect the distribution of the radiological findings.</description><identifier>ISSN: 0022-3050</identifier><identifier>EISSN: 1468-330X</identifier><identifier>DOI: 10.1136/jnnp-2011-301054</identifier><identifier>PMID: 22056964</identifier><identifier>CODEN: JNNPAU</identifier><language>eng</language><publisher>London: BMJ Publishing Group Ltd</publisher><subject>Adrenal Cortex Hormones - therapeutic use ; Adult ; Aged ; Basal Ganglia - pathology ; Biological and medical sciences ; Brain - pathology ; Brain Diseases - drug therapy ; Brain Diseases - immunology ; Brain Diseases - pathology ; Brain Stem - pathology ; Cerebellum - pathology ; Diseases of striated muscles. 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For permission to use (where not already granted under a licence) please go to http://group.bmj.com/group/rights-licensing/permissions.</rights><lds50>peer_reviewed</lds50><woscitedreferencessubscribed>false</woscitedreferencessubscribed><citedby>FETCH-LOGICAL-b462t-e917ebe942f75e0abdb77fc3b753389d94f5b4abe7494cc6a4cc55c7231579443</citedby></display><links><openurl>$$Topenurl_article</openurl><openurlfulltext>$$Topenurlfull_article</openurlfulltext><thumbnail>$$Tsyndetics_thumb_exl</thumbnail><linktopdf>$$Uhttps://jnnp.bmj.com/content/83/1/15.full.pdf$$EPDF$$P50$$Gbmj$$H</linktopdf><linktohtml>$$Uhttps://jnnp.bmj.com/content/83/1/15.full$$EHTML$$P50$$Gbmj$$H</linktohtml><link.rule.ids>114,115,313,314,780,784,792,3196,4024,23571,27922,27923,27924,27925,77600,77631</link.rule.ids><backlink>$$Uhttp://pascal-francis.inist.fr/vibad/index.php?action=getRecordDetail&idt=25289553$$DView record in Pascal Francis$$Hfree_for_read</backlink><backlink>$$Uhttps://www.ncbi.nlm.nih.gov/pubmed/22056964$$D View this record in MEDLINE/PubMed$$Hfree_for_read</backlink></links><search><creatorcontrib>Simon, Neil G</creatorcontrib><creatorcontrib>Parratt, John D</creatorcontrib><creatorcontrib>Barnett, Michael H</creatorcontrib><creatorcontrib>Buckland, Michael E</creatorcontrib><creatorcontrib>Gupta, Ruta</creatorcontrib><creatorcontrib>Hayes, Michael W</creatorcontrib><creatorcontrib>Masters, Lynette T</creatorcontrib><creatorcontrib>Reddel, Stephen W</creatorcontrib><title>Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)</title><title>Journal of Neurology, Neurosurgery and Psychiatry</title><addtitle>J Neurol Neurosurg Psychiatry</addtitle><description>Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory disease of the CNS with a predilection for the hindbrain and responsive to immunotherapy. Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent a benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. 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Five further cases are described with detailed pathology and long term evaluation. CLIPPERS does not represent a benign condition, and without chronic immunosuppression the disease may relapse. The radiological distribution is focused not only in the pons but also in the brachium ponti and cerebellum. Pontocerebellar atrophy occurred early, even in cases treated promptly. Significant cognitive impairment was seen in some cases and was associated with additional cerebral atrophy. The pathology included distinctive histiocytic as well as lymphocytic components and evidence of neuro-axonal injury. Additional subclinical systemic findings on investigation were identified. Relapse was associated with withdrawal of corticosteroids, and disability was least marked in cases where both the presentation and relapses were treated promptly. We propose that the title of the syndrome be amended to chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids to more accurately reflect the distribution of the radiological findings.</abstract><cop>London</cop><pub>BMJ Publishing Group Ltd</pub><pmid>22056964</pmid><doi>10.1136/jnnp-2011-301054</doi><tpages>8</tpages></addata></record> |
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| subjects | Adrenal Cortex Hormones - therapeutic use Adult Aged Basal Ganglia - pathology Biological and medical sciences Brain - pathology Brain Diseases - drug therapy Brain Diseases - immunology Brain Diseases - pathology Brain Stem - pathology Cerebellum - pathology Diseases of striated muscles. Neuromuscular diseases Humans Immunosuppressive Agents - therapeutic use Inflammation - pathology Lymphocytes - pathology Magnetic Resonance Imaging Medical sciences Middle Aged Neuroimaging Neurology Young Adult |
| title | Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) |
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