Growing the phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in children

Tobin, W. Oliver

doi:10.1111/dmcn.14043

Cited by 3 publications

(2 citation statements)

References 5 publications

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“…18 These features were most prominent in the scans when he was young, and the scans at older age show more characteristic neuroimaging findings. Although these atypical features do not fulfill the recently proposed MRI diagnostic criteria, 2,18 similar imaging findings have been reported in pediatric case series by Sa et al 6 Given that there are so few reports of pediatric-onset CLIPPERS in literature, 3,6 -8 we believe these atypical features could either be expanding the spectrum of CLIPPERS or represent differences in early-onset cases as compared to imaging findings in adults. With the evolving clinical and radiological phenotypes, it needs to be seen whether we are looking at a new pediatric-onset inflammatory disorder.…”

Section: Discussionmentioning

confidence: 93%

CLIPPERS Spectrum Disorder: A Rare Pediatric Neuroinflammatory Condition

Nemani

Udwadia-Hegde

Karnavat

et al. 2019

Child Neurology Open

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CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a recently described, rare neuroinflammatory disorder diagnosed by clinical symptoms involving the brain stem with a distinct pattern on neuroimaging and a perivascular T-lymphocyte infiltrate on brain biopsy. It is a condition usually described in adults in the fourth to fifth decade. We report a case of 13-year-old Indian boy who presented with recurrent episodes of ataxia and diplopia with onset at 7 years of age. He was investigated extensively to rule out infective, neoplastic, autoimmune, and demyelinating conditions over a span of 6 years. The diagnosis of CLIPPERS was entertained on the basis of clinico-radio-pathological correlation. Treatment with steroids and steroid-sparing agents, particularly methotrexate, seems to provide a promising outcome. With very few cases in literature so far, reporting of a larger case series with pediatric onset may expand it to CLIPPERS spectrum disorder.

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Section: Discussionmentioning

confidence: 93%

CLIPPERS Spectrum Disorder: A Rare Pediatric Neuroinflammatory Condition

Nemani

Udwadia-Hegde

Karnavat

et al. 2019

Child Neurology Open

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“…3 -9 Furthermore, in numerous cases, an alternative diagnosis was ultimately reached or highly suspected. 4,5 Diagnostic clinical, radiologic, and histologic criteria have been proposed in the adult literature, 10,11 with ongoing debate as to their applicability in pediatrics in view of the small case numbers and significant variability in described clinical features. 12 Potential underlying conditions and drivers of CLIPPERS require consideration at diagnosis particularly if occurring in the absence of a steroid wean.…”

Section: Discussionmentioning

confidence: 99%

Exploring the Intersection of Isolated-CNS Hemophagocytic Lymphohistiocytosis and Pediatric Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids

et al. 2021

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CLIPPERS (chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is an extremely rare neurologic inflammatory condition. Fewer than 10 pediatric cases have been described. Debate persists as to whether it is a distinct disease or a clinical, radiologic, and histologic phenotype evolving into another disorder. We propose that CLIPPERS may be a clinical manifestation of an underlying state of immune-dysregulation. We describe the case of the youngest known report of CLIPPERS, an 18-month-old infant from Melbourne, Australia. Reviewing the literature for all reported pediatric cases, we identified that robust investigation and whole exome sequencing was underutilized and proposed diagnostic criteria were frequently unmet. Particular focus should be paid to genes known to cause familial hemophagocytic lymphohistiocytosis (HLH), with the CLIPPERS phenotype manifesting as a form of isolated central nervous system (CNS)-HLH in some patients. Curative treatment options such as hematopoietic stem cell transplantation may be appropriate for some patients and should be considered early.

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Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

Калашникова

Добрынина

Novikov

et al. 2020

Z. nevrol. psikhiatr. im. S.S. Korsakova

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Growing the phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in children

Abstract: This commentary is on the case series by Sa et al. on pages 490–496 of this issue.

Cited by 3 publications

References 5 publications

CLIPPERS Spectrum Disorder: A Rare Pediatric Neuroinflammatory Condition

CLIPPERS Spectrum Disorder: A Rare Pediatric Neuroinflammatory Condition

Exploring the Intersection of Isolated-CNS Hemophagocytic Lymphohistiocytosis and Pediatric Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids

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