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A6873 - Clipper Disease
Author Block: A. Adial1, A. Iftikhar2, S. Beri3, P. Upadhyaya4, S. Gosai5; 1Pulmonary, NYPQ, Flushing, NY, United States, 2feinstein institute, Northwell Health, Manhasset, NY, United States, 3Critical Care, New York, NY, United States, 4Infectious Disease, Stanford University, Palo Alto, CA, United States, 5Oncology research department, OU health science center, Oklahoma, OK, United States.
Chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (Clippers disease) is the inflammatory disease of brainstem. The clinical presentation is variable making diagnosis of this disease challenging. Features of initial presentation are insidious onset and progressive brainstem, spinal and cerebellar signs. The cardinal feature of the condition is a punctate and/or curvilinear gadolinium enhancement, ‘peppering’ the pons and adjacent hindbrain structures on MRI and usually responds to steroids. We are presenting a case of a patient in whom is likely to be the diagnosis.
Case:
58 year-old African American man with h/o HTN, dyslipidemia, DM admitted due to DKA and hypoxic respiratory failure. DKA was resolved. Patient also complained of worsening gait problem, dysphagia, dysarthria, blurry vision. He was intubated given no gag reflex, persistent hypoxia and severe atelectasis with prominent secretions. Outpatient brain MRI demonstrated unusual patchy signal in mid brain, pons, and medulla without mass effect with associated T2/FLAIR hyper intensity. Work up was negative for oligo clonal bands, NMO, Lyme, VDRL, cytology/cytometry, HIV, and TB. LP was significant for elevated protein. He was given a trial of pulse dose of steroid and plasmapharesis with minimal improvement in symptoms and improvement in MRI. Patient remained ventilator dependent and referred to Tertiary center for further care.
Discussion:
is the chronic inflammatory disorder of brainstem, particular the pons defined in 2010 by Pittock and colleagues. Diagnostic criteria include episodic brainstem symptoms, characteristic punctuate and curvilinear gadolinium-enhancing lesions peppering the brainstem (mainly in the pons) on magnetic resonance (MR) imaging, and T-lymphocytic infiltrate with perivascular predominance in brain biopsy specimens. Mild to moderate elevation of protein and/or pleocytosis mainly lymphocytic or lymphomonocytic predominance on CSF analysis may be present. Presence of a perivascular and parenchymal inflammatory cell infiltrate, combined with the clinical response to immunosuppressive therapies suggests an immune-mediated pathogenesis. Though usually steroid responsive, the dose and duration of treatment is not standardized. Steroid therapy failure seems to be a very strong indicator for an alternative diagnosis and raises the possibility of a tumor or neuronal degeneration. The high risk of relapse during reduction of steroids and the chronicity of the disorder account for the reason to establish a maintenance immunosuppressive therapy, usually with steroids and a steroid-sparing immunosuppressant. Further studies to determine the exact location of the disorder, potential biomarkers, reliable diagnostic criteria’s as well as the optimal form and duration of treatment are necessary.