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

Frontiers in Neurology

关键词: CLIPPERS;    neuroinflammation;    neuromyelitis optica spectrum disorders;    MRI;    area postrema;    misdiagnosis;   

DOI  :  10.3389/fneur.2020.00730

来源: DOAJ

【 摘 要 】

Background: 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).Case presentation: 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.Conclusions: CLIPPERS may present as APS, and should be considered in the differential diagnosis of NMOSD.

【 授权许可】

Unknown