Abstract Details

Title Steroid-Responsive Ecchordosis Physaliphora Diagnosed as CLIPPERS: A Systematic Review of Literature and Case Report

Topic General Neurology

Presentation(s) P1 - Poster Session 1 (9:00 AM-5:00 PM)

Poster/Presentation
Number 186

Objective To investigate and describe the frequency of neurological manifestations in symptomatic patients with ecchordosis physaliphora (EP).

Background EP, a benign notochordal remnant, is often an incidental finding on neuroimaging. However, it can rarely present with neurological symptoms.

Design/Methods PubMed and Embase were systematically searched from 1982 to June 2021 for articles reporting symptomatic EP.

Results We present the largest systematic literature review of symptomatic EP conducted to date, revealing 46 cases in 38 articles. Headache (46%) and CSF rhinorrhea (28%) were the most frequent clinical manifestations. Other common symptoms included diplopia (17%), CN VI palsy (13%), hemiparesis (9%), dizziness (7%) and tinnitus (7%). The majority of symptomatic EP lesions were located in the prepontine region (67%) and required surgical resection (80%). In addition, we report the case of a 55-year-old woman who presented with headache, extremity paresthesias, and weakness. Initially, a diagnosis of CLIPPERS was suspected based on the combination of abnormal imaging changes in the pontine region and steroid-responsiveness.

Conclusions EP should be considered in patients with neurologic symptoms in the setting of prepontine or posterior sphenoid sinus lesions. While symptomatic patients often require surgical intervention, rare cases may respond to oral corticosteroids.

Authors/Disclosures
Thejasvi A. Reddy, MD (The Ken & Ruth Davee Department of Neurology, Northwestern Memorial Hospital) PRESENTER	Ms. Reddy has nothing to disclose.
Carlos A. Perez, MD (University of Texas Health Science Center At Houston)	Dr. Perez has received personal compensation for serving as an employee of Sanofi-Genzyme.
Anam Q. Haque, MD	Miss Haque has nothing to disclose.
	No disclosure on file
Rajesh K. Gupta, MBBS (UTHealth)	Dr. Gupta has nothing to disclose.

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