Unusual CLIPPERS presentation and role of MRI examination in the proper diagnostic assessment: A case report

Vola, Elena Augusta; Russo, Camilla; Macera, Antonio; Vecchio, Walter Del; Puoti, Gianfranco; Caranci, Ferdinando; Elefante, Andrea

doi:10.1016/j.ejro.2019.05.005

Cited by 11 publications

(6 citation statements)

References 15 publications

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“…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. There have also been reports of patients presenting with depression, dementia, headaches and confusion 12–14…”

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

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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.

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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

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“…CLIPPERS syndrome was first described in 2010 by Pittock et al [ 4 ]. It often presents with a triad of neurological signs and symptoms, a dense perivascular CD4+ T cell infiltrate, and clinical improvement after initiating glucocorticoid therapy [ 5 ]. The brainstem findings seen in CLIPPERS include punctate or “salt and pepper” perivascular enhancement of the MRI in the pons, cerebellum, or brachium pontis [ 6 , 7 ].…”

Section: Discussionmentioning

confidence: 99%

Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids: An Acute Presentation

Ambia¹,

Al-Zahrani²,

Almakadma³

et al. 2022

Cureus

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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a rare disease with an unknown etiology which most commonly results in subacute diplopia and ataxia. Diagnosis is achieved through a triad of the following findings: lymphocytic pleocytosis with increased CD4+ T cells on cerebrospinal fluid (CSF) analysis; perivascular punctate and curvilinear hemorrhages in the pons, medulla, or cerebellum on magnetic resonance imaging (MRI) with contrast; and the cessation of symptoms after the initiation of corticosteroids. Here, we report the case of a 23-year-old male who presented with non-specific signs and symptoms, including diffuse weakness in all limbs, ataxia, and slurred speech. The diagnosis was achieved through a contrast MRI of the brain, suggestive of brainstem encephalitis, and a CSF analysis, which revealed elevated glucose and protein levels. Intravenous methylprednisolone was administered for five days and resulted in acute improvement of the patient's clinical status. Repeat CSF analysis and MRI of the brain with contrast two weeks later showed resolution of previous findings. CLIPPERS syndrome is a newly identified disease thought to cause a predominantly inflammatory reaction in the pons, medulla, cerebellum, and supratentorial region. MRI with contrast tends to reveal a "salt and pepper appearance" in a punctate and curvilinear fashion. The hallmark of treatment is corticosteroid therapy, and discontinuation of therapy should be done with caution as relapse of the syndrome with corticosteroid withdrawal has been documented.

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“…Typical offset occurs during midlife, but there is no documented preference for either age or gender of the patients [5,6] . The inflammation typically includes perivascular spaces in the pons or cerebellum, but it may occur in other localizations, such as the spinal cord, midbrain and medulla [7,8]. Pathogenesis of CLIPPERS is still not clear and many theories are being investigated for verification [6].…”

Section: Discussionmentioning

confidence: 99%

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

Szklener¹,

Kuryło²,

Anna³

et al. 2022

J Pre Clin Clin Res.

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Non-specific focal lesions in the central nervous system pose diagnostic and therapeutic challenges. Moreover, the prognosis, depending on the lesion etiology, can be extremely varied from mild and potentially curable to extremely severe and untreatable lesions. Interestingly, due to the lack of a definitive diagnosis, the patient frequently remains in observation until the disease progresses. This case study presents a patient in whom the radiological image of a non-specific brainstem lesion was consistent with glioblastoma mimicking Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids syndrome (CLIPPERS). Radiologically compatible CLIPPERS may conceal a number of different pathologies. Reports of CLIPPERS mimickers highlight the importance of considering alternative diagnoses in patients presenting with the CLIPPERS phenotype. The clinical and radiologic presentation of glioblastoma with features mimicking CLIPPERS are described.

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Unusual CLIPPERS presentation and role of MRI examination in the proper diagnostic assessment: A case report

Cited by 11 publications

References 15 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

Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids: An Acute Presentation

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

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