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The expanding phenotype of CLIPPERS: is it a disease or a syndrome?
  1. Jun-ichi Kira
  1. Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Japan
  1. Correspondence to Professor Jun-ichi Kira, Department of Neurology, Neurological Institute, Graduate School of Medical Sciences, Kyushu University, Fukuoka 812-8582, Japan; kira{at}neuro.med.kyushu-u.ac.jp

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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a newly named pontine-centric inflammatory disorder.1 The cardinal feature of the disease is punctate gadolinium enhancement ‘peppering’ the pons on MRI. The unique MRI features of this disorder have attracted many neurologists' attention leading to the publication of several case reports recently.2–5 The biopsied pontine pathology from the original study revealed a marked perivascular and parenchymal CD3-postive T-cell inflammation without any specific pathology.1 However, because of the lack of a specific biomarker and long-term follow-up, the nosological position of CLIPPERS is still to be established.

The paper by Simon and colleagues6 (see page 15) reports five additional cases of CLIPPERS with detailed pathology and long-term evaluation, expanding the clinical, neuroimaging and pathological phenotype of this disorder: (1) cognitive impairment was seen in four of five cases along with cerebral atrophy in three of them; (2) MRI lesions were distributed not only in the pons but also in the brachium ponti and cerebellum, which later culminated in severe atrophy of the …

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