National Center for Advancing and Translational Sciences Genetic and Rare Diseases Information Center, a program of the National Center for Advancing and Translational Sciences

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids


Other Names:
CLIPPERS
Categories:
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a treatable inflammatory disease of the central nervous system. Specifically, it is a type of encephalomyelitis, which is a general term describing inflammation of the brain and spinal cord.[1] CLIPPERS predominantly affects the cerebellum, spinal cord, and brainstem - the part of the brain that directly connects to the spinal cord.[1][2]

Symptoms usually develop over weeks or months, and only rarely over days.[1] The most common symptoms of CLIPPERS include uncoordinated walking (gait ataxia), double vision (diplopia), difficulty speaking (dysarthria), cognitive impairment, and abnormal facial sensations (such as numbness or tingling).[1][2][3] Other symptoms may include pseudobulbar affect, ringing in the ears (tinnitis), shaking muscles (tremor), uncontrolled eye movements (nystagmus), distortion of taste, nausea, or paraparesis (partial loss of function of the legs).[1] 

The underlying cause of CLIPPERS currently is unclear.[1] There is no one laboratory or imaging test that confirms the diagnosis, but various laboratory and imaging tests may be needed to support the diagnosis, and to confirm or rule out many other conditions that cause overlapping symptoms.[1] A brain MRI with contrast typically has characteristic findings.[1][2] An important part of the ultimate diagnosis, and a classic feature of CLIPPERS, is that symptoms and MRI findings markedly improve with corticosteroid therapy.[1][2]

Corticosteroids may first be taken intravenously (by IV) for several days, and then orally. Most people with CLIPPERS recover significantly within weeks of starting corticosteroids. However, depending on symptom severity and how long the disease was present before treatment, some people will not recover completely. Prolonged treatment with corticosteroids or other immunosuppressant medications (for at least one year) usually is necessary to prevent symptoms from recurring or worsening again.[1]
Last updated: 3/28/2018

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In-Depth Information

  • The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
  • Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
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Selected Full-Text Journal Articles

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  1. Zalewski NL, Tobin WO. CLIPPERS. Curr Neurol Neurosci Rep. September, 2017; 17(9):65. https://www.ncbi.nlm.nih.gov/pubmed/28726127.
  2. Tobin WO, Guo Y, Krecke KN et al. Diagnostic criteria for chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. September, 2017; 140(9):2415-2425. https://academic.oup.com/brain/article-abstract/140/9/2415/4096699?redirectedFrom=fulltext.
  3. Pittock SJ, Debruyne J, Krecke KN, Giannini C, van den Ameele J, De Herdt V, et al. Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Brain. Sept 2010; 133(9):2626-34. http://brain.oxfordjournals.org/content/133/9/2626.long.