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Il Giornale Italiano di Radiologia Medica 2019 Settembre-Ottobre;6(5):464-70

DOI: 10.23736/S2283-8376.19.00227-4

Copyright © 2019 EDIZIONI MINERVA MEDICA

language: English, Italian

A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome: clinical-radiological correlation and potential role of diffusion tensor imaging

Irene GRAZZINI 1 , Silvia PRADELLA 2, Benedetta CALCHETTI 2, Amedeo BIANCHI 2, Gianluca CUNEO 3

1 Department of Radiology, Santa Maria alla Gruccia Hospital, Montevarchi, Arezzo, Italy; 2 Unit of Neurology, Department of Cardiology and Neurology, San Donato Hospital, Arezzo, Italy; 3 Section of Neuroradiology, Department of Radiology, San Donato Hospital, Arezzo, Italy


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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described inflammatory central nervous system disorder, prominently involving the brainstem and in particular the pons, with a favorable response to high dose corticosteroids. The disease has characteristic clinical symptoms and magnetic resonance imaging (MRI) appearance, with punctate and curvilinear gadolinium enhancement “peppering” the pons. The radiological distribution is focused in the pons and adjacent rhombencephalic structures such as the cerebellar peduncles, cerebellum, medulla and the midbrain. Additionally, lesions may also occur in the cervico-thoracic spine cord and supratentorial structures such as the cerebral white matter and the deep cerebral nuclei. Diagnosis of CLIPPERS is challenging, and requires careful exclusion of alternative diagnoses, given that CLIPPERS constitutes a treatable condition and that patients may benefit from an early introduction of glucocorticoids ensued by long-term immunosuppression. In this paper we report the case of a 27-year-old woman with multiple infratentorial contrast enhancing lesions, initially diagnosed as multiple sclerosis. We discuss the symptoms, imaging and treatment of CLIPPERS, and potential application of diffusion tensor imaging to demonstrate cortico-spinal fibers rarefaction in the pons, according with the prevalent structural damage in this region. Early recognition on MRI and radiological follow-up are important to optimize the treatment.


KEY WORDS: Magnetic resonance imaging; Diffusion tensor imaging; Brain stem; Steroids

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