AUTHOR=Zhang Weihe , Cui Lei , Dong Mingrui , Tian Zhaohui , Jiao Yujuan , Jiao Jinsong 

TITLE=Area Postrema Syndrome: A Rare Feature of Chronic Lymphocytic Inflammation With Pontine Perivascular Enhancement Responsive to Steroids

JOURNAL=Frontiers in Neurology

VOLUME=11

YEAR=2020

URL=https://www.frontiersin.org/journals/neurology/articles/10.3389/fneur.2020.00730

DOI=10.3389/fneur.2020.00730

ISSN=1664-2295

ABSTRACT=<p><bold>Background:</bold> The area postrema syndrome (APS) is a unique diagnostic criterion for neuromyelitis optica spectrum disorders (NMOSD). However, APS has rarely been reported in cases of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS).</p><p><bold>Case presentation:</bold> A 36-year-old woman presented with APS and clinical features of diffuse central nervous system involvement during the early stage of the disease. Owing to the absence of serum aquaporin 4 antibodies, she was initially misdiagnosed as a case of seronegative NMOSD. However, the distinct neuroimaging characteristics [symmetrical small punctuate gadolinium enhancing lesions (pepper-like)], typical clinical/radiological relapse, and intense steroid-dependence in this case, prompted us to correct the diagnosis as probable CLIPPERS. To prevent relapse, long-term oral steroids and an immunosuppressive agent were administered.</p><p><bold>Conclusions:</bold> CLIPPERS may present as APS, and should be considered in the differential diagnosis of NMOSD.</p>