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

ABSTRACT Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a rare central nervous system inflammatory disorder with an unknown pathophysiology. We present the case of a 51‐year‐old female exhibiting clinical and radiological features...

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Bibliographic Details
Published in:Clinical case reports 2024-11, Vol.12 (11), p.e9572-n/a
Main Authors: Etemadifar, Masoud, Alaei, Seyyed‐Ali, Mirian, Zahra‐Sadat, Norouzi, Mahdi
Format: Article
Language:English
Subjects:
Ataxia
Case Report
Case reports
CLIPPERS
CNS autoimmune disease
corticosteroid therapy
Diplopia
Gait
Glycoproteins
Hypotheses
Immunoglobulins
Inflammation
Lymphocytes
Magnetic resonance imaging
Nervous system
Neurology
Neuropathology
Pathogenesis
Pathophysiology
Patients
Steroids
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Summary:ABSTRACT Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) syndrome is a rare central nervous system inflammatory disorder with an unknown pathophysiology. We present the case of a 51‐year‐old female exhibiting clinical and radiological features consistent with CLIPPERS syndrome. She manifested diplopia, vertigo, gait ataxia, and lower limb asthenia, accompanied by impaired tandem gait, right sixth nerve palsy, and coarse horizontal nystagmus during the physical examination. Magnetic resonance imaging (MRI) revealed punctate and curvilinear lesions centered in the pons. Treatment with glucocorticoids resulted in significant clinical improvement, and the patient was discharged with a maintenance dose of prednisolone. Throughout a four‐year follow‐up period, the patient remained symptom‐free without any relapses using the low‐dose corticosteroid. The CLIPPERS syndrome diagnosis remains challenging, but recent evidence supports an autoimmune pathogenesis. Although corticosteroid treatment has shown significant clinical improvement, there are no established guidelines or clinical trials on the optimal therapeutic regimen. Despite its rarity, clinicians should consider CLIPPERS syndrome in patients with compatible clinical and radiological features to prevent irreversible neurological damage.
ISSN:2050-0904
2050-0904
DOI:10.1002/ccr3.9572