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CLIPPERS in danger.
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.
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.
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.
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