Brain biopsy in patients with CLIPPERS syndrome: why and when? A case report and literature review

Abstract

CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an inflammatory disorder of the central nervous system (CNS), predominantly involving the brainstem with a characteristic magnetic resonance imaging (MRI) appearance and clinical and radiological responsiveness to glucocorticosteroids. Yet diagnostic biomarkers are missing and other immune-mediated, (para-) infectious and malignant causes mimic CLIPPERS-like MRI presentations. We report the case of a 51-year-old male patient with CLIPPERS who repeatedly responded well to high-dose corticosteroids. After 7 months, however, treatment failed, and he had a biopsy-confirmed diagnosis of a CNS B-cell lymphoma. Clinical and MRI signs of CLIPPERS include a wide spectrum of differential diagnoses which often arise only later during the course of disease. Similar to the case presented here, delayed diagnosis and specific therapy may contribute to an unfavorable outcome. Hence, we propose that in the absence of other diagnostic markers, brain biopsy should be performed as early as possible in CLIPPERS patients.

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

“…The transmural infiltration of lymphocytes, mainly CD3-positive or CD4-positive cells, into the perivascular region is the key pathological hallmark of CLIPPERS [1, 7, 8]. Recent reports suggest that the perivascular infiltration of CD20-positive B lymphocytes is another pathological characteristic of CLIPPERS and is the first sign of its possible transformation into a lymphoma [9, 10]. The detailed mechanism of how white blood cells (mainly T cells) infiltrate the perivascular space of the brainstem is currently unknown.…”

“…a lymphoma [9,10]. The detailed mechanism of how white blood cells (mainly T cells) infiltrate the perivascular space of the brainstem is currently unknown.…”

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

“…3,5 To find previous lymphoma-associated cases up to November 2023, PubMed and Scopus online databases were searched using the keywords "CLIPPER" and "Lymphoma". To the best of our knowledge, 20 patients with lymphoma-associated CLIPPERS have been reported [6][7][8][9][10][11][12][13][14][15][16] since the first introduction of CLIPPERS. 2 A review of 10 patients by Zhang et al showed that patients with lymphoma-associated CLIPPERS had worse clinical outcomes and higher risk of mortality compared to those without lymphoma.…”

Glioblastoma mimicking Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) syndrome – case Report

“…Despite considering all the studies and cases, there are still no clear guidelines for diagnostics, in order to ensure correct diagnosis in every patient with red flags or atypical presentation. Therefore, these patients should have a stereotactic brain biopsy performer [15,16]. Also, patients in whom lesions did not respond to treatment should have a biopsy conducted [16].…”