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

Al-Chalabi, Mustafa; Delcimmuto, Nicholas; Beran, Azizullah; Devarasetty, Pratyush Pavan; Mhanna, Asmaa; Mahfooz, Naeem; Sheikh, Ajaz

doi:10.1016/j.msard.2022.104112

Cited by 13 publications

(15 citation statements)

References 97 publications

Supporting

Mentioning

Contrasting

Order By: Relevance

“…Age at onset has shown large variation, ranging from 16 to 86 years 1. A slight male predominance is noted 11. Though the typical clinical presentation of symptoms is localised to the involvement of the brain stem, symptoms related to long tract and spinal cord involvement, cognitive dysfunction, and pseudo-bulbar affect have all been reported 4.…”

Section: Discussionmentioning

confidence: 99%

See 1 more Smart Citation

Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome

John,

Kankara,

Palasamudram Kumaran

et al. 2024

BMJ Case Rep

View full text Add to dashboard Cite

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described chronic inflammatory central nervous system disease. This case report describes a young female patient presenting with weakness in bilateral upper and lower limbs and tinnitus for 2 months. A neurological examination revealed signs of brainstem and cerebellar involvement. MRI brain showed characteristic features of CLIPPERS, with punctate and nodular enhancement in the pons and cerebellum. Differential diagnoses were systematically considered and excluded. The patient showed significant clinical and radiological improvement with steroid therapy. No clinical or radiological red flags occurred during the follow-up. This case underscores the critical role of integrating clinical and radiological findings to effectively diagnose and manage CLIPPERS. It emphasises the importance of ruling out alternative diagnoses through a thorough evaluation.

show abstract

Section: Discussionmentioning

confidence: 99%

“…The treatment of CLIPPERS includes an initial regimen of glucocorticosteroids (GCS), followed by GCS-sparing immunosuppressants or long-term steroid therapy 11. Marked symptomatic improvement can be noted within days, though some irreversible deficits may persist.…”

Section: Discussionmentioning

confidence: 99%

Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome

John,

Kankara,

Palasamudram Kumaran

et al. 2024

BMJ Case Rep

View full text Add to dashboard Cite

show abstract

“…In fact, methotrexate (15 mg/week), azathioprine (150 mg/day), hydroxychloroquine (400 mg/day), or cyclophosphamide (1 g/month) are the currently recommended immunosuppressants that should be initiated before tapering steroids [15]. Furthermore, cyclophosphamide and methotrexate have been reported to prolong the time to relapse [16].…”

Section: Discussionmentioning

confidence: 99%

“…Furthermore, cyclophosphamide and methotrexate have been reported to prolong the time to relapse [16]. et al [3] have presented the only report on IVIg administration for the treatment of CLIPPERS; they stated that IVIg was not as effective as azathioprine.…”

Section: Statement Of Ethicsmentioning

confidence: 99%

Efficacy of Intravenous Immunoglobulins against Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids: A Case Report

2023

View full text Add to dashboard Cite

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disease of the central nervous system that predominantly affects the brainstem. Apart from corticosteroids, there are few reported treatment options for CLIPPERS, and there is no standard therapy. A 77-year-old man presented with diplopia that had persisted for 5 months. Dysarthria and numbness of the distal right upper extremity and right lips were also observed. Brain magnetic resonance imaging (MRI) revealed a hyperintense area around the brainstem. Symptoms were relieved immediately following intravenous methylprednisolone (IVMP) administration. However, after gradual tapering of oral prednisolone to 5 mg/day, the symptoms relapsed, and brain imaging revealed that the condition had worsened. Intravenous immunoglobulins (IVIg) were administered for recurrence, with no clinical improvement. After each IVMP treatment, the patient recovered promptly. Based on the patient’s symptoms and characteristic MRI findings, exclusion of other diseases, and the significant efficacy of corticosteroids, he was diagnosed with CLIPPERS. There was no recurrence at a maintenance prednisolone dose of 8 mg/day. IVIg had a poor effect on the acute phase of CLIPPERS symptoms. Compared with other immunosuppressants, IVIg is less effective in suppressing the relapse of CLIPPERS.

show abstract

“…The etiology of the primary infiltration of lymphocytes into the perivascular space of the brain stem remains unclear, and at the time of writing this report, there have been no controlled trials of potential treatments or recommendations for treatment. Although the disease is usually a primary relapsing disorder without typical progressive demyelination, in a few cases various malignancies, especially hematologic, have been reported before or after the diagnosis or relapse of CLIPPERS, leading to a chronic disease with a higher mortality rate 4 . In this article, in addition to presenting a patient who had Hodgkin's lymphoma (HL) years before the onset of CLIPPERS, we briefly review the previous lymphoma‐associated cases and discuss the possible relationship between these conditions.…”

Section: Introductionmentioning

confidence: 99%

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids triggered by Hodgkin's lymphoma: A case report and brief literature review

Shervedani,

Tabibian,

Khotbesara

et al. 2023

Clinical Case Reports

View full text Add to dashboard Cite

Key Clinical MessageWe presented a patient, diagnosed with lymphoma‐associated CLIPPERS, 11 years after lymphoma treatment. Therefore, CLIPPERS may be paraneoplastic neurological syndrome of lymphoma, which needs to be considered in the follow‐up of lymphoma cases.AbstractChronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare central nervous system disorder with a recent increase in incidence. There are few reports of lymphoma‐associated CLIPPERS, although the relationship between these two diseases and the pathophysiology of CLIPPERS in general need further investigation. Here, we present a patient with a history of Hodgkin's lymphoma (HL) more than 10 years before the onset of CLIPPERS, in contrast to the majority of previously reported lymphoma‐associated cases, and discuss the possibility that CLIPPERS is the paraneoplastic neurological syndrome of HL. This highlights the need to consider CLIPPERS as a differential diagnosis during follow‐up of patients with a history of lymphoma.

show abstract

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

Cited by 13 publications

References 97 publications

Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome

Clinicoradiological features of probable chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome

Efficacy of Intravenous Immunoglobulins against Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids: A Case Report

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids triggered by Hodgkin's lymphoma: A case report and brief literature review

Contact Info

Product

Resources

About