Headache As the Sole Symptom in Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS): A Case Report and Literature Review

TL;DR

A 22-year-old woman with CLIPPERS had only headaches, showing the condition can present atypically, requiring early diagnosis and treatment.

Contribution

This case report highlights headache as the sole symptom of CLIPPERS, an atypical presentation not previously emphasized.

Findings

Headache was the only symptom in a CLIPPERS case confirmed by imaging and biopsy.

Steroid treatment initially helped but relapsed on tapering, requiring long-term low-dose steroids and mycophenolate mofetil for remission.

The case underscores the need for prompt diagnosis of atypical CLIPPERS presentations to improve outcomes.

Abstract

Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids (CLIPPERS) is a rare central nervous system inflammatory condition usually presenting with a range of symptoms, including ataxia, diplopia, dysarthria, seizures, and headaches. We present a unique case of a 22-year-old woman exhibiting headache as the sole symptom. Imaging and biopsy confirmed the diagnosis, and initial steroid treatment provided relief, though it relapsed on tapering. Long-term management with low-dose steroids and mycophenolate mofetil achieved remission. This case highlights the importance of recognizing atypical presentations of CLIPPERS, emphasizing the need for prompt diagnosis and appropriate treatment plans to improve patient outcomes. Further research is necessary to enhance our understanding and management of CLIPPERS.

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