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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.
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.
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 …
Competing interests None.
Patient consent Obtained.
Provenance and peer review Not commissioned; externally peer reviewed.
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