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CLIPPERS: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids
  1. Damien Biotti1,
  2. Romain Deschamps1,
  3. Eimad Shotar1,
  4. Elisabeth Maillart1,
  5. Mickaël Obadia1,
  6. Ivan Mari1,
  7. Julien Savatovsky2,
  8. Olivier Gout1
  1. 1Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
  2. 2Department of Radiology, Fondation Ophtalmologique Adolphe de Rothschild, Paris, France
  1. Correspondence to Dr D Biotti, Department of Neurology, Fondation Ophtalmologique Adolphe de Rothschild, 25 Rue Manin, 75019 Paris, France; dbiotti{at}hotmail.com

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A 56-year-old previously healthy man developed progressive cerebellar ataxia and binocular horizontal diplopia, worsening over several weeks. An MR scan of brain showed multiple punctate hyperintensities in T2 weighted images, confined to the pons, medulla oblongata and cerebellum, with curvilinear gadolinium enhancement in T1 weighted images. The clinical and radiological features suggested the CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids). The symptoms and MR findings improved dramatically with high dose parenteral corticosteroids.

Introduction

CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a recently described CNS inflammatory disorder.1 Since 2010, several new possible cases have been published.2,,5 We report a further patient with features consistent with this syndrome.

Case report

A previously healthy 56-year-old man was admitted with a several week history of progressive ataxia and binocular horizontal diplopia. On initial neurological examination, he showed dysarthria, left sixth nerve palsy, a right-sided extensor plantar response, right-sided sensory loss and cerebellar limb and gait ataxia; ophthalmoscopy was normal. MR scan of the brain (T2 weighted images) showed multiple punctate hyperintensities in the pons, medulla oblongata and cerebellum (figure 1A), with curvilinear gadolinium enhancement in T1 weighted images (figure 1B, C). Diffusion weighted MR images gave no additional information. MR …

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