Mitochondrial carrier homolog 1 (Mtch1) antibodies in neuro-Behçet's disease

Cataloged from PDF version of article.Efforts for the identification of diagnostic autoantibodies for neuro-Behcet's disease (NBD) have failed. Screening of NBD patients' sera with protein macroarray identified mitochondrial carrier homolog 1 (Mtch1), an apoptosis-related protein, as a potential autoantigen. ELISA studies showed serum Mtch1 antibodies in 68 of 144 BD patients with or without neurological involvement and in 4 of 168 controls corresponding to a sensitivity of 47.2% and specificity of 97.6%. Mtch1 antibody positive NBD patients had more attacks, increased disability and lower serum nucleosome levels. Mtch1 antibody might be involved in pathogenic mechanisms of NBD rather than being a coincidental byproduct of autoinflammation. © 2013 Elsevier B.V

Mycophenolate mofetil as a novel immunosuppressant in the treatment of neuro-Behçet's disease with parenchymal involvement: Presentation of four cases

PubMed ID: 21968239Background. Behçet's disease is a multisystemic, relapsing, inflammatory disorder of unknown origin. Among Turkish cohorts, 5-15% of patients show involvement of the central nervous system (CNS) at some time during their disease. There are mainly two types of clinical presentation: parenchymal CNS inflammation manifesting mainly as meningoencephalitis of the brainstem, or dural sinus thrombosis. Several drugs like high-dose steroids or immunosuppressive agents, mainly azathioprine, are used in the treatment. For patients who do not respond sufficiently to these agents or are not able tolerate them, other options are needed. Patients. We are presenting 4 cases with parenchymal neuro-Behçet's disease, where commonly used immunosuppressive drugs could not be continued due to intolerance or inefflcacy. However, the patients benefited well from mycophenolate mofetil. The benefit was sustained during 3-7 years of follow-up (median 6.5 years). Conclusion. Mycophenolate mofetil seems to be an alternative drug in parenchymal neuro-Behçet's disease; however, large controlled studies should be performed for verification of our results. © Copyright CLINICAL AND EXPERIMENTAL RHEUMATOLOGY 2011

Mycophenolate mofetil as a novel immunosuppressant in the treatment of neuro-Behcet's disease with parenchymal involvement: presentation of four cases

WOS: 000295669200012PubMed ID: 21968239Background. Behcet's disease is a inultisystemic, relapsing, inflammatory disorder of unknown origin. Among Turkish cohorts, 5-15% of patients show involvement of the central nervous system (CNS) at some time during their disease. There, are mainly two types of clinical presentation: parehchymal CNS inflammation manifesting mainly as meningoencephalitis of the brainstem, or dural sinus thrombosis. Several drugs like high-dose steroids or immunosuppressive agents, mainly azathioprine, are used in the treatment. For patients who do not respond sufficiently to these agents or are not able tolerate them, other options are needed. Patients. We are presenting 4 cases with parenchymal neuro-Behcet's disease, where commonly used immunosuppressive drugs could not be continued due to intolerance or inefficacy. However, the patients benefited well from mycophenolate mofetil. The benefit was sustained during 3-7 years of follow-up (median 6.5 years). Conclusion. Mycophenolate mofetil seems to be an alternative drug in parenchymal neuro-Behcet's disease; however, large controlled studies should be performed for verification of our results

Autoantibodies to aquaporin-1 in Turkish neuromyelitis optica patients with and without aquaporin-4 antibodies

29th Congress of the European-Committee-for-Treatment-and-Research-in-Multiple-Sclerosis / 18th Annual Conference of Rehabilitation in MS -- OCT 02-05, 2013 -- Copenhagen, DENMARKWOS: 000328751401025…European Comm Treatment & Res Multiple Sclerosi

Coexistence of Guillain-Barre syndrome and Behcet's disease

WOS: 000326356700018PubMed ID: 23433066Behcet's disease (BD) is a multisystemic, recurrent and inflammatory disorder. Neurological involvement is rare and affects mainly the central nervous system (CNS) in the form of brainstem meningoencephalitis or dural sinus thrombosis. Peripheral neuropathy is usually not observed during the course of BD but some reports have shown electrophysiologic evidence of subclinical neuropathy, mononeuritis multiplex and cranial neuropathy in BD patients. The co-occurrence of Guillain-Barre syndrome (GBS), an acute inflammatory demyelinating neuropathy, with other autoimmune or systemic diseases is rare. We present a case of BD with clinical and electrophysiological diagnosis of GBS. The findings of the patient were discussed with reference to literature

Long-term MRI findings in neuromyelitis optica: Seropositive versus seronegative patients

PubMed ID: 23279782Background and purpose: Neuromyelitis optica (NMO) is a severe demyelinating inflammatory disorder associated with serum antibodies against aquaporin 4 (AQP4-Ab). A significant number of patients with NMO remain seronegative over time. Long-term observational magnetic resonance imaging (MRI) studies of the CNS in patients with NMO are rare or of limited duration. The objective of this study is to determine long-term MRI characteristics of seropositive and seronegative patients, and assess possible overlap with multiple sclerosis (MS). Methods: Clinical and radiological characteristics of 28 patients with NMO at onset and of 17 patients after an average follow-up time of 9years were recorded. Fifty percent of patients were seropositive for AQP4-Ab. Onset and final brain/spinal MRI scans were retrospectively analysed and compared. Results: Significantly more patients in the seronegative group had brain lesions at onset. Spinal lesions of seropositive patients were longer and showed increased cord swelling at onset MRI scans. After the follow-up time the differences between both groups disappeared. Patients in the seropositive group tended to develop brain lesions over time. No patient fulfilled Barkhof's or McDonald's radiological criteria for MS at onset or over time. Conclusion: Brain MRI features show differences between seropositive and seronegative patients at time of onset in NMO, but differences between groups vanish over time. None of the AQP4-negative patients fulfill radiological MS criteria on a long-term basis, suggesting that seronegative NMO constitutes an independent entity. © 2012 The Author(s) European Journal of Neurology © 2012 EFNS

Long-term MRI findings in neuromyelitis optica: seropositive versus seronegative patients

Background and purpose Neuromyelitis optica (NMO) is a severe demyelinating inflammatory disorder associated with serum antibodies against aquaporin 4 (AQP4-Ab). A significant number of patients with NMO remain seronegative over time. Long-term observational magnetic resonance imaging (MRI) studies of the CNS in patients with NMO are rare or of limited duration. The objective of this study is to determine long-term MRI characteristics of seropositive and seronegative patients, and assess possible overlap with multiple sclerosis (MS). Methods Clinical and radiological characteristics of 28 patients with NMO at onset and of 17 patients after an average follow-up time of 9years were recorded. Fifty percent of patients were seropositive for AQP4-Ab. Onset and final brain/spinal MRI scans were retrospectively analysed and compared. Results Significantly more patients in the seronegative group had brain lesions at onset. Spinal lesions of seropositive patients were longer and showed increased cord swelling at onset MRI scans. After the follow-up time the differences between both groups disappeared. Patients in the seropositive group tended to develop brain lesions over time. No patient fulfilled Barkhof's or McDonald's radiological criteria for MS at onset or over time. Conclusion Brain MRI features show differences between seropositive and seronegative patients at time of onset in NMO, but differences between groups vanish over time. None of the AQP4-negative patients fulfill radiological MS criteria on a long-term basis, suggesting that seronegative NMO constitutes an independent entity