Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
Identifieur interne : 001647 ( Main/Exploration ); précédent : 001646; suivant : 001648Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS)
Auteurs : Sean J. Pittock [États-Unis] ; Jan Debruyne [Belgique] ; Karl N. Krecke [États-Unis] ; Caterina Giannini [États-Unis] ; Jelle Van Den Ameele [Belgique] ; Veerle De Herdt [Belgique] ; Andrew Mckeon [États-Unis] ; Robert D. Fealey [États-Unis] ; Brian G. Weinshenker [États-Unis] ; Allen J. Aksamit [États-Unis] ; Bruce R. Krueger [États-Unis] ; Elizabeth A. Shuster [États-Unis] ; B. Mark Keegan [États-Unis]Source :
- Brain [ 0006-8950 ] ; 2010.
Abstract
The classification and pathological mechanisms of many central nervous system inflammatory diseases remain uncertain. In this article we report eight patients with a clinically and radiologically distinct pontine-predominant encephalomyelitis we have named ‘chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids’ (CLIPPERS). The patients were assessed clinically, radiologically and pathologically at Mayo Clinic, USA and Ghent University Hospital, Belgium from 1999 to 2009. Median follow-up duration from clinical onset was 22 months (range 7–144 months). Patients underwent extensive laboratory (serum and cerebrospinal fluid), radiological and pathological testing (conjunctival, transbronchial and brain biopsies) to search for causes of an inflammatory central nervous system disorder. All eight patients (five female, three male) presented with episodic diplopia or facial paresthesias with subsequent brainstem and occasionally myelopathic symptoms and had a favourable initial response to high dose glucocorticosteroids. All patients had symmetric curvilinear gadolinium enhancement peppering the pons and extending variably into the medulla, brachium pontis, cerebellum, midbrain and occasionally spinal cord. Radiological improvement accompanied clinical response to glucocorticosteroids. Patients routinely worsened following glucocorticosteroid taper and required chronic glucocorticosteroid or other immunosuppressive therapy. Neuropathology of biopsy material from four patients demonstrated white matter perivascular, predominantly T lymphocytic, infiltrate without granulomas, infection, lymphoma or vasculitis. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a definable, chronic inflammatory central nervous system disorder amenable to immunosuppressive treatment. The T cell predominant inflammatory pathology in affected central nervous system lesions and the clinical and radiological response to immunosuppressive therapies is consistent with an immune-mediated process.
Url:
DOI: 10.1093/brain/awq164
Affiliations:
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Mark Keegan</name><affiliation wicri:level="2"><country xml:lang="fr">États-Unis</country><wicri:regionArea>1 Department of Neurology, Mayo Clinic College of Medicine, Rochester, MN 55905</wicri:regionArea><placeName><region type="state">Minnesota</region></placeName></affiliation><affiliation wicri:level="1"><country wicri:rule="url">États-Unis</country></affiliation></author></analytic><monogr></monogr><series><title level="j" type="main">Brain</title><idno type="ISSN">0006-8950</idno><idno type="eISSN">1460-2156</idno><imprint><publisher>Oxford University Press</publisher><date when="2010-09">2010</date><date type="e-published" when="2010-07-17">2010</date><biblScope unit="vol">133</biblScope><biblScope unit="issue">9</biblScope><biblScope unit="page" from="2626">2626</biblScope><biblScope unit="page" to="2634">2634</biblScope></imprint><idno type="ISSN">0006-8950</idno></series></biblStruct></sourceDesc><seriesStmt><idno type="ISSN">0006-8950</idno></seriesStmt></fileDesc><profileDesc><textClass></textClass></profileDesc></teiHeader><front><div type="abstract">The classification and pathological mechanisms of many central nervous system inflammatory diseases remain uncertain. In this article we report eight patients with a clinically and radiologically distinct pontine-predominant encephalomyelitis we have named ‘chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids’ (CLIPPERS). The patients were assessed clinically, radiologically and pathologically at Mayo Clinic, USA and Ghent University Hospital, Belgium from 1999 to 2009. Median follow-up duration from clinical onset was 22 months (range 7–144 months). Patients underwent extensive laboratory (serum and cerebrospinal fluid), radiological and pathological testing (conjunctival, transbronchial and brain biopsies) to search for causes of an inflammatory central nervous system disorder. All eight patients (five female, three male) presented with episodic diplopia or facial paresthesias with subsequent brainstem and occasionally myelopathic symptoms and had a favourable initial response to high dose glucocorticosteroids. All patients had symmetric curvilinear gadolinium enhancement peppering the pons and extending variably into the medulla, brachium pontis, cerebellum, midbrain and occasionally spinal cord. Radiological improvement accompanied clinical response to glucocorticosteroids. Patients routinely worsened following glucocorticosteroid taper and required chronic glucocorticosteroid or other immunosuppressive therapy. Neuropathology of biopsy material from four patients demonstrated white matter perivascular, predominantly T lymphocytic, infiltrate without granulomas, infection, lymphoma or vasculitis. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids is a definable, chronic inflammatory central nervous system disorder amenable to immunosuppressive treatment. The T cell predominant inflammatory pathology in affected central nervous system lesions and the clinical and radiological response to immunosuppressive therapies is consistent with an immune-mediated process.</div></front></TEI><affiliations><list><country><li>Belgique</li><li>États-Unis</li></country><region><li>Floride</li><li>Minnesota</li></region></list><tree><country name="États-Unis"><region name="Minnesota"><name sortKey="Pittock, Sean J" sort="Pittock, Sean J" uniqKey="Pittock S" first="Sean J." last="Pittock">Sean J. Pittock</name></region><name sortKey="Aksamit, Allen J" sort="Aksamit, Allen J" uniqKey="Aksamit A" first="Allen J." last="Aksamit">Allen J. Aksamit</name><name sortKey="Fealey, Robert D" sort="Fealey, Robert D" uniqKey="Fealey R" first="Robert D." last="Fealey">Robert D. Fealey</name><name sortKey="Giannini, Caterina" sort="Giannini, Caterina" uniqKey="Giannini C" first="Caterina" last="Giannini">Caterina Giannini</name><name sortKey="Keegan, B Mark" sort="Keegan, B Mark" uniqKey="Keegan B" first="B. Mark" last="Keegan">B. Mark Keegan</name><name sortKey="Keegan, B Mark" sort="Keegan, B Mark" uniqKey="Keegan B" first="B. Mark" last="Keegan">B. Mark Keegan</name><name sortKey="Krecke, Karl N" sort="Krecke, Karl N" uniqKey="Krecke K" first="Karl N." last="Krecke">Karl N. Krecke</name><name sortKey="Krueger, Bruce R" sort="Krueger, Bruce R" uniqKey="Krueger B" first="Bruce R." last="Krueger">Bruce R. Krueger</name><name sortKey="Mckeon, Andrew" sort="Mckeon, Andrew" uniqKey="Mckeon A" first="Andrew" last="Mckeon">Andrew Mckeon</name><name sortKey="Mckeon, Andrew" sort="Mckeon, Andrew" uniqKey="Mckeon A" first="Andrew" last="Mckeon">Andrew Mckeon</name><name sortKey="Pittock, Sean J" sort="Pittock, Sean J" uniqKey="Pittock S" first="Sean J." last="Pittock">Sean J. Pittock</name><name sortKey="Shuster, Elizabeth A" sort="Shuster, Elizabeth A" uniqKey="Shuster E" first="Elizabeth A." last="Shuster">Elizabeth A. Shuster</name><name sortKey="Weinshenker, Brian G" sort="Weinshenker, Brian G" uniqKey="Weinshenker B" first="Brian G." last="Weinshenker">Brian G. Weinshenker</name></country><country name="Belgique"><noRegion><name sortKey="Debruyne, Jan" sort="Debruyne, Jan" uniqKey="Debruyne J" first="Jan" last="Debruyne">Jan Debruyne</name></noRegion><name sortKey="De Herdt, Veerle" sort="De Herdt, Veerle" uniqKey="De Herdt V" first="Veerle" last="De Herdt">Veerle De Herdt</name><name sortKey="Van Den Ameele, Jelle" sort="Van Den Ameele, Jelle" uniqKey="Van Den Ameele J" first="Jelle" last="Van Den Ameele">Jelle Van Den Ameele</name></country></tree></affiliations></record>Pour manipuler ce document sous Unix (Dilib)
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