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Aim. In this paper, we aim to analyse our own clinical observation of a patient with CLIPPERS syndrome on the basis of available literature data. Materials and methods. The medical record of a patient treated at the Neurology Unit of the S.V. Ochapovsky Regional Clinical Hospital No. 1 (Kradnodarsy Krai) was investigated, along with available national and foreign literary sources. Results. The patient was diagnosed with autoimmune brainstem encephalitis (CLIPPERS syndrome) on the basis of the diagnostic CLIPPERS syndrome criteria modified and adapted by W. Tobin and co-authors in 2017. Among them are: 1) subacute development of symptoms for the pons and cerebellum lesions along with other signs of CNS damage (onset is characterised by diplopia and ataxia); 2) good response to glucocorticosteroid (GCS) therapy; 3) lack of involvement of the peripheral nervous system; 4) enhanced MRI lesions homogeneously accumulating contrast agents without a mass effect; 5) a significant decrease in the accumulation of contrast material during GCS therapy; 6) absence of myelin loss. Conclusion. Considering that the GCS therapy produces a rapid and significant effect, it is important to focus on early detection and treatment of this disease through the development of more accurate markers and evaluation criteria for its diagnosis.

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Paraproteinemias or monoclonal gammopathies constitute a broad spectrum of heterogeneous clonal disorders of plasma cells characterized by the secretion of monoclonal proteins of heavy or light chains and the development of symptoms associated with them through mechanisms independent of tumor burden. Specifically, peripheral neuropathies represent an increasingly recognized manifestation of these paraproteinemias. We report a case of a 71-year-old female who presented to the emergency department with clinical symptoms of perioral paresthesias associated with an ataxic gait that progressively compromised her functionality, eventually completely limiting her ability to walk. Initially diagnosed with chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS syndrome), management with corticosteroids was initiated, leading to partial improvement. After comprehensive etiological studies ruled out common causes of peripheral neuropathy (PN), a monoclonal peak of immunoglobulin M (IgM) was detected. With the initiation of appropriate treatment, the patient progressively regained her ability to walk. Unfortunately, due to prolonged corticosteroid use, she developed osteoporosis and multiple fragility fractures, which again limited her mobility. CLIPPERS syndrome coexisting with monoclonal gammopathy is extremely rare, highlighting the importance of this report., Competing Interests: Human subjects: Consent was obtained or waived by all participants in this study. Conflicts of interest: In compliance with the ICMJE uniform disclosure form, all authors declare the following: Payment/services info: All authors have declared that no financial support was received from any organization for the submitted work. Financial relationships: All authors have declared that they have no financial relationships at present or within the previous three years with any organizations that might have an interest in the submitted work. Other relationships: All authors have declared that there are no other relationships or activities that could appear to have influenced the submitted work., (Copyright © 2024, Prieto-Torres et al.)

CLIPPERS syndrome (Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids) is a recently described rare disease affecting the central nervous system. It is characterized by subacute development of symptoms of lesions predominantly in the brain stem and cerebellum, by specific magnetic resonance imaging (MRI) changes, perivascular lymphocytic infiltration in the brain substance and a good response to glucocorticoid (GC) therapy. The paper describes CLIPPERS syndrome in a patient who has been followed up in a clinic for 10 years. During this period, different variants of clinical diagnosis have been considered. The final diagnosis was made only when comparing the clinical course and manifestations of the disease, MRI data, as well as the reaction to GC therapy and its discontinuation. Literature data and diagnostic criteria of this disease are presented.

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is an inflammatory disorder of the central nervous system. The pathophysiology of CLIPPERS is unknown. The disease has characteristic radiological lesions located in the pons, bulbus, and cerebellum. Here we report two new cases and review the literature on CLIPPERS syndrome. A 35-year-old woman presented with a 2-month history of progressive double vision, vertigo, gait ataxia, nausea, and vomiting. The second case was that of a 40-year-old Iraqi man who presented with a 3-month history of vertigo, headache, and gait ataxia. Diagnosis of CLIPPERS was established based on findings of punctate, nodular enhancing lesions in the pons and bulbus in the first case and in the cerebellum in the second. Our patients responded well to steroid therapy and remained relapse-free for 2 years. CLIPPERS is a rare autoimmune disorder with characteristic radiological findings. Long-term immunosuppressive therapy is necessary for treatment. [ABSTRACT FROM AUTHOR]

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently identified diagnosis that can cause a variety of severe symptoms, including ataxia, dysarthria, diplopia, paraparesis, and vertigo. These symptoms rarely present in isolation but often accompany one another in various combinations. Magnetic Resonance Imaging (MRI) of the brain is critical for making the diagnosis and typically reveals scattered enhancement within the pons and adjacent structures. The syndrome responds well to high-dose steroids, and maintenance therapy is required to prevent a recurrence. In this report, we present a case of a 62-year-old man who developed CLIPPERS syndrome. The patient presented with hemiparesis and dysarthria, which developed over four months and then acutely worsened within 24 hours. After diagnosing CLIPPERS, the patient was placed on high-dose steroids and experienced rapid clinical improvement, as well as improvement on repeat MRI. The patient's treatment was complicated by an incidental diagnosis of tuberculosis, which required simultaneous management with isoniazid., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2023, Coggins et al.)

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., Competing Interests: The authors have declared that no competing interests exist., (Copyright © 2022, Ambia et al.)