title: Atypical presentation of CLIPPERS syndrome: a new entity in the differential diagnosis of central nervous system rheumatologic diseases.
abstract: Numerous autoimmune diseases can affect the central nervous system (CNS), and variable clinical presentations confound the differential diagnosis. The challenging task of properly characterizing various CNS autoimmune diseases enables patients to be rapidly triaged and appropriately treated. In this review article, we aim to explore different CNS manifestations of rheumatologic diseases with emphasis on the utility of imaging and cerebrospinal fluid findings. We review the classic physical examination findings, characteristic imaging features, cerebrospinal fluid results, and serum biomarkers. In addition, we also present a unique case of newly described autoimmune entity CLIPPERS syndrome. Our case is unique in that this is the first case which demonstrates involvement of the supratentorial perivascular spaces in addition to the classic infratentorial involvement as initially described by Pittock et al (Brain. 2010;133:2626-2634).
authors: From the *Department of Medicine, Winthrop University Hospital, Mineola; †Department of Radiology Stony Brook University Medical Center, Stony Brook; and ‡Department of Rheumatology, Clinical Immunology and Allergy, Winthrop University Hospital, Mineola, NY.
authors: Maryam
authors: M
authors: Gul
authors: Ammar A
authors: AA
authors: Chaudhry
authors: Abbas A
authors: AA
authors: Chaudhry
authors: Mubashir A
authors: MA
authors: Sheikh
authors: Steven
authors: S
authors: Carsons
mesh_terms: D000368:Aged; D001259:Ataxia / Q000175:diagnosis* / Q000188:drug therapy; D001327:Autoimmune Diseases / Q000175:diagnosis*; D002493:Central Nervous System Diseases / Q000175:diagnosis*; D015897:Comorbidity; D003937:Diagnosis, Differential; D004172:Diplopia / Q000175:diagnosis* / Q000188:drug therapy; D005260:Female; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D020336:Paraparesis, Spastic / Q000175:diagnosis* / Q000188:drug therapy; D011239:Prednisolone / Q000627:therapeutic use; D012216:Rheumatic Diseases / Q000175:diagnosis*; D013116:Spinal Cord / Q000473:pathology; D013577:Syndrome; D016896:Treatment Outcome
chemical_list: D005938:Glucocorticoids; D011239:Prednisolone