Cytotoxic CD8 + T cells and CD138 + plasma cells prevail in cerebrospinal fluid in non-paraneoplastic cerebellar ataxia
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JOURNAL OF NEUROINFLAMMATION
CASE REPORT
Open Access
Cytotoxic CD8+ T cells and CD138+ plasma cells prevail in cerebrospinal fluid in non-paraneoplastic cerebellar ataxia with contactin-associated protein-2 antibodies Nico Melzer1*, Kristin S Golombeck2, Catharina C Gross1, Sven G Meuth1 and Heinz Wiendl1
Abstract Objective: The purpose of this paper is to report a patient with otherwise unexplained cerebellar ataxia with serum antibodies against contactin-associated protein-2 (CASPR-2) and provide a detailed description of the composition of cellular infiltrates in the cerebrospinal fluid (CSF) compared to the peripheral blood (PB). CASPR-2 antibodies strongly labeling axons of cerebellar granule neurons have recently been identified in sera from nine patients with otherwise unexplained progressive cerebellar ataxia with mild to severe cerebellar atrophy. Design: This is a report of a single case. Methods: The study methods used were neurologic examination, magnetic resonance imaging, fluorodeoxyglucose positron emisson tomography, lumbar puncture and multicolor flow-cytometry. Results: A 23-year-old Caucasian male presented with a two-year history of a progressive cerebellar and brainstem syndrome. Magnetic resonance imaging (MRI) showed pronounced cerebellar atrophy, especially of the medial parts of the hemispheres and the vermis. Cerebral fluorodeoxyglucose positron emission tomography (FDG-PET) showed pronounced hypometabolism of the whole cerebellum. CASPR-2 antibodies were detected in the serum but not the CSF, and none of the staging and laboratory assessments revealed other causes of progressive cerebellar degeneration. Interestingly, flow-cytometry of the CSF as compared to the PB showed increased fractions of CD138+ plasma cells as well as human leukocyte antigen (HLA)-DR+ CD8+ T cells suggesting that both B cells and CD8+ T cells were preferentially recruited to and activated within the CSF- (and putatively central nervous system (CNS)-) compartment. Conclusion: We confirm the association of CASPR-2 serum antibodies with cerebellar ataxia and provide the first evidence for a combined humoral and cellular immune response in this novel antibody-associated inflammatory CNS disease. Keywords: CD138+ plasma cells, Cytotoxic CD8+ T cells, Contactin-2-associated protein-2, Cerebellar ataxia
Background Antibodies to the complex of voltage-gated K+ channels (VGKC) and associated neuronal membrane proteins (contactin-associated protein-2 (CASPR-2; axon); contactin-2 (ensheathing glial cells); leucine-rich glioma inactivated 1 protein (LGI-1; synapse)) are detected in * Correspondence: [email protected] 1 Department of Neurology, Inflammatory Disorders of the Nervous System and Neurooncology, University of Münster, Albert-Schweitzer Campus 1, Münster 48149, Germany Full list of author information is available at the end of the article
the sera of patients with peripheral nerve hyperexcitability (acquired neuromyotonia), Morvan's disease and limbic encephalitis [1-3]. Recently, CASPR-2 antibodies str
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