Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (Q84151): Difference between revisions
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A inflamação linfocítica crônica com realce perivascular pontino responsiva a esteróides (CLIPPERS) é caracterizada por uma síndrome do tronco cerebral pontina central subaguda, incluindo ataxia, disartria, diplopia e dormência facial com uma característica de ressonância magnética marcante de realce pontilhado de gadolínio pontino central subagudo "em sal e pimenta" envolvendo a ponte ( às vezes estendendo-se para envolver estruturas adjacentes) e que, por definição, responde ao tratamento com corticosteroides. Investigações extensas são necessárias para excluir etiologias alternativas. Embora a biópsia do tronco encefálico / cerebelar seja útil e tipicamente revele um infiltrado de células T perivascular, predominantemente CD3-positivo, ela não é necessária para o diagnóstico. | |||
| description / en | description / en | ||
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is characterised by a subacute pontine-centric brainstem syndrome including ataxia, dysarthria, diplopia, and facial numbness with a hallmark MRI feature of punctate curvilinear patchy gadolinium enhancement “peppering” the pons (sometimes extending to involve adjacent structures) and which by definition responds to corticosteroid treatment. Extensive investigations are necessary to exclude alternative etiologies. Although brainstem/cerebellar biopsy is helpful and typically reveals a perivascular, predominantly CD3-positive T cell infiltrate it is not necessary for the diagnosis. | |||
Revision as of 09:30, 15 August 2026
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is characterised by a subacute pontine-centric brainstem syndrome including ataxia, dysarthria, diplopia, and facial numbness with a hallmark MRI feature of punctate curvilinear patchy gadolinium enhancement “peppering” the pons (sometimes extending to involve adjacent structures) and which by definition responds to corticosteroid treatment. Extensive investigations are necessary to exclude alternative etiologies. Although brainstem/cerebellar biopsy is helpful and typically reveals a perivascular, predominantly CD3-positive T cell infiltrate it is not necessary for the diagnosis.
| Language | Label | Description | Also known as |
|---|---|---|---|
| default for all languages | ID_913443626 |
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| English | Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids |
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is characterised by a subacute pontine-centric brainstem syndrome including ataxia, dysarthria, diplopia, and facial numbness with a hallmark MRI feature of punctate curvilinear patchy gadolinium enhancement “peppering” the pons (sometimes extending to involve adjacent structures) and which by definition responds to corticosteroid treatment. Extensive investigations are necessary to exclude alternative etiologies. Although brainstem/cerebellar biopsy is helpful and typically reveals a perivascular, predominantly CD3-positive T cell infiltrate it is not necessary for the diagnosis. |
