Case Report with Review of Literature for the Dilemma of Diagnosis of CLIPPERS

Abstract

CLIPPER is a chronic inflammatory disorder in the CNS, which is characterized by MRI appearance of punctate and curvilinear gadolinium enhancement that involve the pons and the cerebellum and exquisite response to steroid. We report a patient presented with clinical and radiological features suggestive of CLIPPERS. However, despite the initial response to steroid, there were dramatic changes in the course of his disease that were conducive to considering another diagnosis. We searched PubMed using word (CLIPPERS) till December 2018. The pathogenesis, clinical manifestations, imaging features, treatment and prognosis of this disorder are summarized. A review of the literature for cases of CLIPPERS demonstrated a subset of patients who later discovered to have an alternative pathology. Indeed, clinicians should be scrupulous to diagnose this disease based solely on the clinical and radiological findings and they should have a lower threshold of having a brain biopsy.

Keywords::

• CLIPPERS
• Erdheim–Chester disease
• brain biopsy in CLIPPERS

Financial & competing interests disclosure

The authors have no relevant affiliations or financial involvement with any organization or entity with a financial interest in or financial conflict with the subject matter or materials discussed in the manuscript. This includes employment, consultancies, honoraria, stock ownership or options, expert testimony, grants or patents received or pending, or royalties.

No writing assistance was utilized in the production of this manuscript.

Ethical consent disclosure

Written informed consent was obtained from the patient for publication of this case report and accompanying images and this was attained from the Research Ethics Committee in the Security Forces Hospital Makkah, Saudi Arabia.