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PubMed This is a summary of 14 peer-reviewed journal articles Updated
Neurology · Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

Symptoms and Emergency Warning Signs

At a Glance

CLIPPERS symptoms often develop over weeks to months and may relapse. Gradual worsening should be reported promptly to a neurologist, while sudden severe changes in breathing, alertness, swallowing, speech, movement, or vision require emergency care.

Because CLIPPERS primarily affects the brainstem (the area at the base of the brain that connects to the spinal cord), the symptoms it causes often involve the coordination and “automatic” functions of the body [1][2]. The condition typically develops over a period of weeks to months, a timeline doctors call subacute [3][4].

Common Symptoms of CLIPPERS

The most frequent symptoms of CLIPPERS relate to pontocerebellar dysfunction—a disruption in the communication between the pons (the brainstem relay station) and the cerebellum (the part of the brain controlling movement and balance) [1][5].

  • Gait Ataxia: This is often the first and most common sign [6]. It feels like a loss of balance or unsteadiness while walking, sometimes requiring a “wide-based” walk to stay upright [2][7].
  • Diplopia (Double Vision): Inflammation can disrupt the nerves that control eye movements, leading to seeing two of everything [1]. This may be accompanied by nystagmus, where the eyes make involuntary, jerky movements [1][8].
  • Dysarthria (Slurred Speech): You may find it difficult to articulate words clearly, or your speech may sound thick or slow [5][9].
  • Facial Sensory Changes: Some people experience numbness, tingling (paresthesia), or unusual sensations in the face [5][7].
  • Dysphagia (Difficulty Swallowing): This can manifest as coughing while eating or drinking, or a feeling that food is getting “stuck” [5][9].

How Symptoms Progress

In most cases, these symptoms emerge and worsen gradually [2]. While the initial onset is subacute, the disease often follows a relapsing pattern, where symptoms improve significantly with steroid treatment but can return if the medication is lowered too quickly or if the underlying inflammation flares up again [2][3].

When to Seek Emergency Care

While most symptoms of CLIPPERS are managed through regular outpatient care, the brainstem also controls vital functions like breathing and consciousness [10]. In rare cases, severe inflammation in the brainstem can lead to life-threatening complications. Sudden, severe neurological changes could also indicate a stroke, severe infection, or another emergency.

Call your local emergency number (e.g., 911) or go to the nearest emergency room immediately if you or a loved one experiences:

  • New or severe trouble breathing, including extreme shortness of breath or bluish lips [11][12].
  • An inability to wake up or extreme confusion (a sudden state of being unable to stay awake or answer questions appropriately) [10][13].
  • Severe choking or a sudden inability to swallow your own saliva (which carries a high risk of inhaling fluid into the lungs, known as aspiration) [5][9].
  • Sudden inability to speak, move, or see [10][14].

For symptoms that are worsening gradually (over days) rather than suddenly: Do not wait for your next scheduled appointment. Contact your neurologist or specialist promptly to discuss your symptoms and determine if your treatment plan needs adjustment.

Common questions in this guide

What symptoms are common with CLIPPERS?
Common symptoms include unsteady walking or loss of balance, double vision, involuntary eye movements, slurred speech, facial numbness or tingling, and trouble swallowing. Symptoms occur when inflammation affects brainstem areas involved in movement, eye control, speech, sensation, and swallowing.
How quickly do CLIPPERS symptoms develop?
CLIPPERS symptoms usually develop and worsen over weeks to months rather than appearing all at once. This gradual pattern is called subacute, although symptoms can later improve and return in relapses.
Which CLIPPERS symptoms require emergency care?
Call your local emergency number or go to an emergency room for new or severe trouble breathing, inability to wake, extreme confusion, severe choking, or sudden inability to swallow saliva. Sudden inability to speak, move, or see also requires immediate evaluation because it may signal a stroke or another emergency.
What should I do if CLIPPERS symptoms gradually get worse?
Do not wait for your next appointment if symptoms worsen over days. Contact your neurologist or another specialist promptly so they can assess whether inflammation is flaring or your treatment plan needs adjustment.
Can CLIPPERS cause swallowing or breathing problems?
Yes. Brainstem inflammation can interfere with swallowing and, in severe cases, with breathing or alertness; coughing or choking while eating, trouble swallowing saliva, or new breathing difficulty should be taken seriously. Severe breathing trouble or inability to swallow saliva requires emergency care.
How can I recognize a CLIPPERS relapse?
A relapse means symptoms return or worsen after improvement, often when inflammation becomes active again or steroid treatment is reduced too quickly. Gradual return of balance, speech, vision, or swallowing problems should be reported promptly to your neurologist, while sudden severe changes require emergency care.

Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.Are my current symptoms considered 'subacute' or 'progressive,' and how does that affect my care plan?
  2. 2.How will we monitor my swallowing function to prevent issues like aspiration pneumonia?
  3. 3.Given my specific symptoms, what is the best way to contact your office if I notice a sudden change in my balance or speech?
  4. 4.What specific signs should I look for that would indicate a 'relapse' requiring a clinic visit versus an emergency requiring immediate care?
  5. 5.Are there specific breathing or sleep issues we should be screening for?

Questions For You

Tap a prompt to share your answer — we'll use it plus this page's context to start a tailored conversation.

References

References (14)
  1. 1

    Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids Presenting With Predominantly Neuro-Ophthalmic Features.

    McDonald HM, Handzic A, Mandell DM, et al.

    Journal of neuro-ophthalmology : the official journal of the North American Neuro-Ophthalmology Society 2025; (45(4)):460-465 doi:10.1097/WNO.0000000000002271.

    PMID: 39774846
  2. 2

    Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids, a mimicker of malignancy: a case report and review of the literature.

    Zhuang E, Shane L, Ramezan N, et al.

    Journal of medical case reports 2021; (15(1)):246 doi:10.1186/s13256-021-02814-5.

    PMID: 34001259
  3. 3

    Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS): contemporary advances and current controversies.

    Li EC, Lai QL, Cai MT, et al.

    Journal of neurology 2024; (271(4)):1747-1766 doi:10.1007/s00415-024-12189-4.

    PMID: 38286842
  4. 4

    Two New Cases and Literature Review of CLIPPERS Syndrome with Long-Term Follow-up.

    Kamişli Ö, Tecellioğlu M, Erbay MF, et al.

    Noro psikiyatri arsivi 2020; (57(2)):160-164 doi:10.5152/npa.2017.22732.

    PMID: 32550784
  5. 5

    Increased Number of Perivascular CD20-Positive B Lymphocytes in the Neuropathology of CLIPPERS: Findings of 6 Patients from Mainland China.

    Yang B, Liu F, Li J, Wen Y

    European neurology 2021; (84(1)):22-30 doi:10.1159/000512406.

    PMID: 33498046
  6. 6

    Clinical characteristics, management, and outcomes of CLIPPERS: A comprehensive systematic review of 140 patients from 100 studies.

    Al-Chalabi M, DelCimmuto NR, Beran A, et al.

    Multiple sclerosis and related disorders 2022; (68()):104112 doi:10.1016/j.msard.2022.104112.

    PMID: 36029706
  7. 7

    Clinical and imaging characteristics of corticosteroid-responsive chronic lymphocytic inflammation with Pontine perivascular enhancement syndrome (CLIPPERS): an analysis of 9 cases.

    Sun X, Li X, Raza HK, et al.

    Neurological sciences : official journal of the Italian Neurological Society and of the Italian Society of Clinical Neurophysiology 2025; (46(8)):3819-3826 doi:10.1007/s10072-025-08208-5.

    PMID: 40335832
  8. 8

    Navigating the Mysteries of CLIPPERS Syndrome: A Case Report of Long-Term Follow-Up.

    Etemadifar M, Alaei SA, Mirian ZS, Norouzi M

    Clinical case reports 2024; (12(11)):e9572 doi:10.1002/ccr3.9572.

    PMID: 39540001
  9. 9

    Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) with intracranial Epstein-Barr virus infection: A Case Report.

    Ma Y, Sun X, Li W, et al.

    Medicine 2016; (95(46)):e5377 doi:10.1097/MD.0000000000005377.

    PMID: 27861371
  10. 10

    Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids with seizures and central pyrexia, in a patient requiring tracheal intubation and mechanical ventilation: A case report.

    Wall RJ

    Journal of the Intensive Care Society 2018; (19(2)):167-170 doi:10.1177/1751143717719644.

    PMID: 29796076
  11. 11

    Hemidysgeusia, phantosmia and respiratory arrest: a case of CLIPPERS.

    Nguyen PB, Prentice D, Brazel R, Leong WK

    BMJ case reports 2019; (12(5)) doi:10.1136/bcr-2019-230094.

    PMID: 31151979
  12. 12

    Is chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in children the same condition as in adults?

    Sa M, Green L, Abdel-Mannan O, et al.

    Developmental medicine and child neurology 2019; (61(4)):490-496 doi:10.1111/dmcn.13997.

    PMID: 30146710
  13. 13

    CLIPPERS Responsive to Cladribine as a Durable Steroid-Sparing Agent.

    Thebault S, Bergman H, Atkins HL, et al.

    Neurology(R) neuroimmunology & neuroinflammation 2023; (10(1)) doi:10.1212/NXI.0000000000200060.

    PMID: 36396449
  14. 14

    Co-occurrence of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and posterior reversible encephalopathy syndrome: a case report.

    Song Y, Bai M, Chen H, et al.

    Multiple sclerosis and related disorders 2021; (49()):102791 doi:10.1016/j.msard.2021.102791.

    PMID: 33517177

This page is for informational purposes only and does not constitute medical advice. Ask your neurologist about your symptoms, and seek emergency care for sudden or severe changes in breathing, alertness, swallowing, speech, movement, or vision.

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