Clinical and imaging characteristics of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids: one case report / 中华神经科杂志

ABSTRACT

Objective Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS) is a recently described brainstem-predominant inflammatory disorder responsive to steroids with characteristic magnetic resonance imaging(MRI) feature.There was no case report in China.The aim of this study was to characterize the clinical and imaging patterns of CLIPPERS for early diagnosis and treatment.Method Clinical and imaging data and therapeutic methods and following up after treatment of 1 patient diagnosed CLIPPERS were reviewed retrospectively.Results The patient presented with subacute weakness of the lower extremities,gait ataxia and facial numbness.Cerebellar ataxia and pyramidal signs were the main positive signs on neurological examinations.Brain MRI showed punctate gadolinium enhancement peppering the pons and cerebellar.Treatment with steroids led to rapid clinical improvement and marked resolution of MRI lesions which accorded with characteristics of CLIPPERS.Conclusions There are no characteristic clinical manifestations of CLIPPERS.Punctate gadolinium enhancement peppering the pons is the distinct magnetic resonance imaging feature which may suggest the clinical diagnosis of CLIPPERS.Early treatment with steroids can improve the prognosis.