Longitudinal Analysis of Cytokines and Chemokines in the Cerebrospinal Fluid of a Patient with Neuro-Sweet Disease Presenting with Recurrent Encephalomeningitis

Abstract

Background Neuro-Sweet disease (NSD) has recently been identified as Sweet disease with central nervous system (CNS) involvement and chemokines (i.e., CCL2, CCL3, CCL5, CXCL8, in 10 CSF samples from the patient longitudinally for one year including those during two episodes of encephalomeningitis. Results The elevations of IL-6, IFN-γ, CXCL8 (IL8) and CXCL10 (IP10) were markedly higher than the levels in uninfected control subjects with neurological disorders. The levels of these cytokines and chemokines were statistically correlated with total CSF cell counts (p <0.01). Conclusion CD4+ helper T (Th) cells can be divided into the

Neuro-Sweet disease with positive modified acid-fast staining of the cerebrospinal fluid: A case report

“…The patient presented with clinical features of viral meningitis and the cutaneous manifestations of classical Sweet disease. Although the brain MRI was normal, which has rarely been demonstrated in previous patients with NSD ( 4 , 9 , 13 – 16 ), the present patient fulfilled all the essential points of the diagnostic criteria for NSD, including: i) high fever of 38–40°C; ii) painful and dull red erythematous plaques on the face, neck and upper part of the trunk; iii) neutrophilic infiltration of the dermis without uveitis; iv) positive HLA-Cw1 and negative HLA-B51; and v) good response to corticosteroid therapy ( 8 ). Combination therapy with corticosteroids and antiviral agents was initiated due to suspected viral meningitis, prior to the final diagnosis of NSD.…”

Diffuse chronic leptomeningitis with seropositive rheumatoid arthritis: report of a case successfully treated as rheumatoid leptomeningitis

“…Tumor necrosis factor (TNF)-a, IL-1b, IL-2, IL-10, and IL-17 were not detected in the same CSF samples. Although IL-4 and interferon (IFN)-c were detected in these CSF samples, any increases in values were within normal ranges[2].…”

Reversible extensive leukoencephalopathy in Sweet disease: A case report

“…In Sweet disease, dermal neutrophil infiltration is not accompanied with vasculitis or thrombophlebitis. This characteristic finding, together with HLA B51 negativity, is thought to be important in distinguishing Sweet disease from Behçet's disease [11]. The clinical features of NSD are different from those of Neuro-Behçet's disease (NBD): (1) both sexes are equally affected in NSD, while NBD predominantly affects males; (2) abnormal signals on brain MRI are observed in various CNS regions with no site predominance in NSD, while the basal ganglia and brainstem are preferentially involved in NBD; (3) some patients with NSD show ocular signs including episcleritis and conjunctivitis, whereas uveitis is common in Behçet's disease; (4) there is a strong HLA-Cw1 and B54 association in NSD, while there is a high frequency of HLA-B51 in NBD.…”