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

**Authors:** Omar Abdelkader, Hamza Abuzenah, Hans Shuhaiber

PMC · DOI: 10.7759/cureus.64310 · 2024-07-11

## 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.

## Key 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.

## Linked entities

- **Chemicals:** steroids (PubChem CID 139082353), mycophenolate mofetil (PubChem CID 5281078)
- **Diseases:** CLIPPERS (MONDO:0017297)

## Full-text entities

- **Diseases:** Headache (MESH:D006261), dysarthria (MESH:D004401), ataxia (MESH:D001259), Chronic Lymphocytic Inflammation With (MESH:D015451), seizures (MESH:D012640), diplopia (MESH:D004172), central nervous system inflammatory condition (MESH:D002493)
- **Species:** Homo sapiens (human, species) [taxon 9606]

## Figures

3 figures with captions in the complete paper: https://tomesphere.com/paper/PMC11316347/full.md

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Source: https://tomesphere.com/paper/PMC11316347