%0 Journal Article
%T A Case of Rare Inflammatory Brainstem Syndrome: Chronic Lymphocytic Inflammation with Pontine Perivascular Enhancement Responsive to Steroids
%A Anshu Rohatgi
%A Neha Pandita
%A Rahul Sharma
%A Samir Patel
%A Virti Shah
%A Yatin Chandrakant Sagvekar
%J Archive of "Annals of Indian Academy of Neurology".
%D 2019
%R 10.4103/aian.AIAN_370_18
%X We report a case of a 23-year-old female with subacute onset of dysarthria, ataxia, vertigo, and bulbar involvement with magnetic resonance imaging (MRI) brain [Figure 1] showing diffuse T2 and fluid-attenuated inversion recovery hyperintensities involving the pons which appeared slightly expanded, with punctate foci of restricted diffusivity manifested as hyperintense signals on diffusion-weighted imaging images, with contrast enhancement and preservation of transverse pontine fibers. She had no history of or predilection to allergies. Her laboratory screening included complete blood count, renal and liver function, creatine kinase, C-reactive protein, erythrocyte sedimentation rate, serum protein electrophoresis, immunofixation, thyroid function test, lipid profile, Vitamin B12, and urinalysis. Autoimmune serological evaluations included antinuclear, antineutrophil cytoplasmic antibody (c and p), rheumatoid factor, complement levels, lupus anticoagulant, anti-beta-2-glycoprotein-1 and anticardiolipin antibodies, angiotensin-converting enzyme, anti-aquaporin-4 antibody, thyroid autoantibodies, and onconeural antibodies. Microbiological examination included hepatitis C, hepatitis B, human immunodeficiency virus, venereal disease research laboratory, Treponema pallidum hemagglutination, and Borrelia burgdorferi serological evaluations. Results for all these laboratory tests were normal or negative. She responded dramatically to pulse followed by maintenance corticosteroid treatment. In view of clinical features, typical MRI findings, not consistent with multiple sclerosis (MS), negative vasculitis and autoimmune markers, and good response to steroids, the patient was provisionally diagnosed as a case of chronic lymphocytic inflammation with pontine perivascular enhancement responsive to steroids (CLIPPERS). Six-month follow-up showed remarkable clinical and radiological improvements
%U https://www.ncbi.nlm.nih.gov/pmc/articles/PMC6472233/