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CLIPPERS in danger.

CLIPPERS syndrome

Dr Sampurna Roy MD                     

 

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CLIPPERS syndrome (Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids) is a well-defined chronic inflammatory central nervous system disorder which can be controlled by immunosuppressive treatment.

Pittock et al reported this disorder in 2010. 

Clinically, the patients usually present with diplopia, facial paresthesia, dysarthria and ataxia.

In some patients there is paresthesia of the extremities with mild gait disturbance.

 

The lesion predominantly involves the pons, medulla, cerebellum, midbrain and  occasionally spinal cord that improved with immunosuppressive treatment.

Cases have been reported involving  thalamus, basal ganglia, capsula interna, corpus callosum and the cerebral white matter.

Biopsy from the patients showed perivascular, predominantly T lymphocytic infiltrate in the white matter without demyelination on neuropathological examination.

There is no evidence of granulomas, infection, lymphoma or vasculitis.

The diagnosis of Clippers Syndrome is challenging.

One has to carefully exclude alternative diagnoses.

A specific serum or cerebrospinal fluid (CSF) biomarker for the disorder is not known.

 

Further reading:

Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).

CLIPPERS: chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids. Review of an increasingly recognized entity within the spectrum of inflammatory central nervous system disorders.

Stroke mimicking relapse in a patient with CLIPPERS syndrome.

Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

A case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) in East Asia.

Expanding the clinical, radiological and neuropathological phenotype of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).

Atypical chronic lymphocytic inflammation with pontocerebellar perivascular enhancement responsive to steroids (CLIPPERS), primary angiitis of the CNS mimicking CLIPPERS or overlap syndrome? A case report.

Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.

A case of "CLIPPERS" cured by immunotherapy five years before the description of the syndrome.

 

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Dr  Sampurna Roy  MD

Consultant Histopathologist (Kolkata - India)

 

 


 

 

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