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Co-occurrence of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and posterior reversible encephalopathy syndrome: a case report.
Mult Scler Relat Disord. 2021 Apr; 49:102791.MS

Abstract

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder, mainly involving the brainstem. Posterior reversible encephalopathy syndrome (PRES) is a syndrome that affects the bilateral parietal-occipital region. Here, we describe a rare case of CLIPPERS with PRES. A 47-year-old woman presented with subacute, progressive ataxia symptoms, and radiological and pathological findings were consistent with CLIPPERS. In addition, she had acute convulsions and was unconscious, and brain magnetic resonance imaging fluid-attenuated inversion recovery showed patchy high-intensity posterior cerebral white matter signals, with imaging lesions showing vasogenic oedema, a typical manifestation of PRES. The imaging lesions showed vasogenic oedema in bilateral parietal and occipital lobes and typical 'pepper-like' punctate gadolinium enhancement in pons and bilateral cerebellar hemispheres, which were considered to be caused by merger of CLIPPERS and PRES. Clinical manifestations and imaging lesions disappeared after two months of steroids and symptomatic treatment, supporting the diagnosis of CLIPPERS with PRES. When patients with CLIPPERS show unusual symptoms or atypical vasogenic oedema lesions in the posterior cerebral white matter, the coexistence of PRES should be considered.

Authors+Show Affiliations

Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China. Electronic address: syn19940820@163.com.Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China.Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China.Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China.Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China. Electronic address: drzhaoyang123456@163.com.Department of Neurology and Neuroscience Center, The First Hospital of Jilin University, Changchun, China. Electronic address: kangdingliu@163.com.

Pub Type(s)

Case Reports
Letter

Language

eng

PubMed ID

33517177

Citation

Song, Yanna, et al. "Co-occurrence of Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids and Posterior Reversible Encephalopathy Syndrome: a Case Report." Multiple Sclerosis and Related Disorders, vol. 49, 2021, p. 102791.
Song Y, Bai M, Chen H, et al. Co-occurrence of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and posterior reversible encephalopathy syndrome: a case report. Mult Scler Relat Disord. 2021;49:102791.
Song, Y., Bai, M., Chen, H., Liu, S., Zhao, Y., & Liu, K. (2021). Co-occurrence of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and posterior reversible encephalopathy syndrome: a case report. Multiple Sclerosis and Related Disorders, 49, 102791. https://doi.org/10.1016/j.msard.2021.102791
Song Y, et al. Co-occurrence of Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids and Posterior Reversible Encephalopathy Syndrome: a Case Report. Mult Scler Relat Disord. 2021;49:102791. PubMed PMID: 33517177.
* Article titles in AMA citation format should be in sentence-case
TY - JOUR T1 - Co-occurrence of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids and posterior reversible encephalopathy syndrome: a case report. AU - Song,Yanna, AU - Bai,Miaoshui, AU - Chen,Huaqiu, AU - Liu,Shan, AU - Zhao,Yang, AU - Liu,Kangding, Y1 - 2021/01/22/ PY - 2020/11/11/received PY - 2021/01/17/revised PY - 2021/01/19/accepted PY - 2021/2/1/pubmed PY - 2021/5/15/medline PY - 2021/1/31/entrez KW - Autoimmunity KW - CLIPPERS KW - Lymphoma KW - PRES SP - 102791 EP - 102791 JF - Multiple sclerosis and related disorders JO - Mult Scler Relat Disord VL - 49 N2 - Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare inflammatory disorder, mainly involving the brainstem. Posterior reversible encephalopathy syndrome (PRES) is a syndrome that affects the bilateral parietal-occipital region. Here, we describe a rare case of CLIPPERS with PRES. A 47-year-old woman presented with subacute, progressive ataxia symptoms, and radiological and pathological findings were consistent with CLIPPERS. In addition, she had acute convulsions and was unconscious, and brain magnetic resonance imaging fluid-attenuated inversion recovery showed patchy high-intensity posterior cerebral white matter signals, with imaging lesions showing vasogenic oedema, a typical manifestation of PRES. The imaging lesions showed vasogenic oedema in bilateral parietal and occipital lobes and typical 'pepper-like' punctate gadolinium enhancement in pons and bilateral cerebellar hemispheres, which were considered to be caused by merger of CLIPPERS and PRES. Clinical manifestations and imaging lesions disappeared after two months of steroids and symptomatic treatment, supporting the diagnosis of CLIPPERS with PRES. When patients with CLIPPERS show unusual symptoms or atypical vasogenic oedema lesions in the posterior cerebral white matter, the coexistence of PRES should be considered. SN - 2211-0356 UR - https://www.unboundmedicine.com/medline/citation/33517177/Co_occurrence_of_chronic_lymphocytic_inflammation_with_pontine_perivascular_enhancement_responsive_to_steroids_and_posterior_reversible_encephalopathy_syndrome:_a_case_report_ L2 - https://linkinghub.elsevier.com/retrieve/pii/S2211-0348(21)00057-2 DB - PRIME DP - Unbound Medicine ER -