A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with initial normal magnetic resonance imaging

Taïeb, Guillaume; Wacongne, Anne; Renard, Dimitri; Figarella‐Branger, Dominique; Castelnovo, Giovanni; Labauge, Pierre

doi:10.1093/brain/awq390

Cited by 30 publications

(33 citation statements)

References 2 publications

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“…These abnormalities were seen from the first relapse in all patients except patient 5, in whom typical MR imaging features of CLIPPERS were observed at 9 years after the onset of disease (during a 12th relapse). 2 The mean size of the gadolinium-enhancing lesions was 1 to 3 mm (eFigure 1); few lesions exceeded 3 mm. Gadoliniumenhancing lesions exceeding 3 mm had a typical nodular aspect ( Figure 4A).…”

Section: Neuroimagingmentioning

confidence: 96%

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Long-term Outcomes of CLIPPERS (Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids) in a Consecutive Series of 12 Patients

Taïeb¹,

Duflos²,

Renard³

et al. 2012

Arch Neurol

113

133

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Background: Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a central nervous system inflammatory disease.Objective: To describe the disease course of CLIPPERS.Design: A nationwide study was implemented to collect clinical, magnetic resonance imaging, cerebrospinal fluid, and brain biopsy specimen characteristics of patients with CLIPPERS.

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Section: Neuroimagingmentioning

confidence: 96%

“…2 At the end of the follow-up period, additional MR imaging features were seen in some patients, primarily extensive spinal cord involvement, followed by spinal cord atrophy and black holes. Pontocerebellar atrophy was also seen, confirming recently reported cases of CLIPPERS.…”

Section: Commentmentioning

confidence: 97%

Long-term Outcomes of CLIPPERS (Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids) in a Consecutive Series of 12 Patients

Taïeb¹,

Duflos²,

Renard³

et al. 2012

Arch Neurol

113

133

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“…[14] MRI may show no abnormalities at the onset of the disease. [30] Serial MRI examinations may be necessary when CLIPPERS is suspected. The hallmark feature of CLIIPERS on MRI is punctate and curvilinear gadolinium enhancement in a “pepper-like appearance” with a perivascular pattern centered on the pons.…”

Section: Discussionmentioning

confidence: 99%

Horizontal eyeball akinesia as an initial manifestation of CLIPPERS

et al. 2016

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Background:Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare chronic inflammatory disorder in the central nervous system (CNS), which is characterized by magnetic resonance imaging (MRI) appearance with punctate and curvilinear gadolinium enhancement “peppering” the pons. Lesions of CLIPPERS mainly involve the pons and the cerebellum. Adjacent structures such as the medulla and the midbrain may also be involved. It is proposed that CLIPPERS is an immune-mediated inflammatory condition characteristic of T-cell-predominant infiltrates and good responsiveness to corticosteroids.Methods and Results:We report a 46-year-old woman who presented with horizontal eyeball akinesia and gait ataxia with characteristic MRI features of CLIPPERS. The possible pathogenesis, clinical manifestations, imaging features, treatment, and prognosis of this peculiar disorder are summarized.Conclusion:This report contributes to the clinical understanding of CLIPPERS which may present with horizontal eyeball akinesia as an initial manifestation. The characteristic presentation of a subacute cerebellar and brainstem syndrome and pepper-like gadolinium enhancement was confirmed in this report. Long-term immunosuppressive treatment seems to be mandatory to sustain improvement. Azathioprine alone may be capable of maintaining remission.

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“…Von allen 17 aktuell beschriebenen Patienten entwickelten nur 3 eine Paraparese, vermutlich ausgelöst durch eine Dissemination der Läsionen in das obere Halsmark [6,10]. Ein weiterer Patient entwickelte nach einer Krankheitsdauer von 30 Jahren eine Tetraparese, die sich nach Gabe von Glukokortikosteroiden rasch besserte [12].…”

Section: Abb 2 8 Cmrt Des Patienten Nach Prednisolonbehandlung Es Zunclassified

“…Bei 8 Patienten, bei denen eine Biopsie in der Pons (3 Patienten), im Thalamus (1 Patient) oder im Kleinhirn (5 Patienten) durchgeführt wurde, zeigte die pathologische Untersuchung ein ausgeprägtes lymphozytäres Infiltrat in der weißen Substanz mit perivaskulä-rer Ausbreitung, das hauptsächlich aus CD3-reaktiven T-Lymphozyten, einigen CD20-positiven B-Lymphozyten und vereinzelt Histiozyten und Mikroglia bestand [6,10,12]. Die neuropathologischen Befunde und die klinische Besserung auf Steroide lassen die Annahme einer autoimmunvermittelten Entzün-dung zu.…”

Section: Abb 2 8 Cmrt Des Patienten Nach Prednisolonbehandlung Es Zunclassified

Chronische steroidresponsive lymphozytäre perivaskuläre pontine Entzündung

et al. 2012

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Pittock und Kollegen formulierten 2010 anhand einer Fallserie von 8 Patienten erstmalig klinische und magnetresonanztomographische diagnostische Kriterien für das von ihnen neu definierte Syndrom einer chronischen steroidresponsiven lymphozytären perivaskulären pontin betonten Entzündung ("chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids"/CLIPPERS) [10]. Seit Veröffent-lichung dieser ersten Fallserie sind 9 weitere Fälle in der Literatur beschrieben worden [3, 4, 5, 6, 7, 11, 12].

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A new case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with initial normal magnetic resonance imaging

Cited by 30 publications

References 2 publications

Long-term Outcomes of CLIPPERS (Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids) in a Consecutive Series of 12 Patients

Long-term Outcomes of CLIPPERS (Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids) in a Consecutive Series of 12 Patients

Horizontal eyeball akinesia as an initial manifestation of CLIPPERS

Chronische steroidresponsive lymphozytäre perivaskuläre pontine Entzündung

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