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Sample records for chronic inflammatory demyelinating

  1. Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

    Science.gov (United States)

    ... People About NINDS NINDS Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) Information Page Table of Contents (click to jump ... en Español What is Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)? Chronic inflammatory demyelinating polyneuropathy (CIDP) is a neurological ...

  2. Chronic Inflammatory Demyelinating Polyneuropathy

    OpenAIRE

    Dimachkie, Mazen M.; Barohn, Richard J.

    2013-01-01

    Chronic Inflammatory polyneuropathies are an important group of neuromuscular disorders that present chronically and progress over more than 8 weeks, being referred to as chronic inflammatory demyelinating polyneuropathy (CIDP). Despite tremendous progress in elucidating disease pathogenesis, the exact triggering event remains unknown. Our knowledge regarding diagnosis and management of CIDP and its variants continues to expand, resulting in improved opportunities for identification and treat...

  3. Chronic inflammatory demyelinative polyneuropathy

    DEFF Research Database (Denmark)

    Said, Gérard; Krarup, Christian

    2013-01-01

    Chronic inflammatory demyelinative polyneuropathy (CIDP) is an acquired polyneuropathy presumably of immunological origin. It is characterized by a progressive or a relapsing course with predominant motor deficit. The diagnosis rests on the association of non-length-dependent predominantly motor...... deficit following a progressive or a relapsing course associated with increased CSF protein content. The demonstration of asymmetrical demyelinating features on nerve conduction studies is needed for diagnosis. The outcome depends on the amplitude of axon loss associated with demyelination. CIDP must...... be differentiated from acquired demyelinative neuropathies associated with monoclonal gammopathies. CIDP responds well to treatment with corticosteroids, intravenous immunoglobulins, and plasma exchanges, at least initially....

  4. [Chronic inflammatory demyelinating polyradiculoneuropathy].

    Science.gov (United States)

    Franques, J; Azulay, J-P; Pouget, J; Attarian, S

    2010-06-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a demyelinating chronic neuropathy of immune origin whose diagnosis is based upon clinical, biological and electrophysiological data; previously critical to the diagnosis the nerve biopsy is now restricted to the rare situations where accurate diagnosis cannot be reached using these data alone. CIDP are mainly idiopathic, but a few associated diseases must be sought for as they require specific attention. Such associated diseases must particularly be discussed when the manifestations are severe or resistant to immunomodulating or immunosuppressive agents. Indeed, idiopathic CIDP are usually responsive to these treatments. The effectiveness of these treatments is limited by the importance of the secondary axonal loss. The dependence or the resistance may sometimes justify the association of several immunomodulating treatments. A single randomized controlled trial support the use of cytotoxic drugs and none with rituximab.

  5. Chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Van den Bergh, Peter Y K; Rajabally, Yusuf A

    2013-06-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common autoimmune neuropathy. The diagnosis depends on the clinical presentation with a progressive or relapsing course over at least 2 months and electrophysiological evidence of primary demyelination. Whereas typical CIDP is quite easily recognizable because virtually no other neuropathies present with both distal and proximal motor and sensory deficit, atypical CIDP, focal and multifocal variants in particular, may represent a difficult diagnostic challenge. CIDP very likely is an underdiagnosed condition as suggested also by a positive correlation between prevalence rates and sensitivity of electrophysiological criteria. Since no 'gold standard' diagnostic marker exists, electrophysiological criteria have been optimized to be at the same time as sensitive and as specific as possible. Additional supportive laboratory features, such as increased spinal fluid protein, MRI abnormalities of nerve segments, and in selected cases nerve biopsy lead to the correct diagnosis in the large majority of the cases. Objective clinical improvement following immune therapy is also a useful parameter to confirm the diagnosis. The pathogenesis and pathophysiology of CIDP remain poorly understood, but the available evidence for an inflammatory origin is quite convincing. Steroids, intravenous immunoglobulin (IVIG), and plasma exchange (PE) have been proven to be effective treatments. IVIG usually leads to rapid improvement, which is useful in severely disabled patients. Repeat treatment over regular time intervals for many years is often necessary. The effect of steroids is slower and the side-effect profile may be problematic, but they may induce disease remission more frequently than IVIG. An important and as of yet uncompletely resolved issue is the evaluation of long-term outcome to determine whether the disease is still active and responsive to treatment.

  6. Chronic inflammatory demyelinating polyneuropathy

    Science.gov (United States)

    Polyneuropathy - chronic inflammatory; CIDP; Chronic inflammatory polyneuropathy; Guillain-Barré - CIDP ... CIDP is one cause of damage to nerves outside the brain or spinal cord ( peripheral neuropathy ). Polyneuropathy ...

  7. Management strategies in chronic inflammatory demyelinating polyradiculoneuropathy

    OpenAIRE

    Patel Kamakshi; Bhanushali Minal; Muley Suraj

    2010-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic, proximal and distal, asymmetrical or symmetrical, motor and sensory demyelinating polyneuropathy with a progressive course for at least 2 months. The accurate diagnosis is crucial as CIDP is amenable to treatment. Recent advances have provided new strategies and options for management of this syndrome. In this article, we review the clinical and diagnostic features as well as discuss recent insights and treatment s...

  8. Steroids for Chronic Inflammatory Demyelinating Polyneuropathy

    OpenAIRE

    J Gordon Millichap

    2005-01-01

    The efficacy and safety of high-dose, intermittent IV methylprednisolone (IVMP) as initial and long-term maintenance therapy for chronic inflammatory demyelinating polyneuropathy (CIDP) were analyzed by a retrospective review of outcome data derived from patients’ medical records between 1992 and 2003 at Washington University School of Medicine, St Louis, MO.

  9. HLA antigens in chronic inflammatory demyelinating polyneuropathy.

    OpenAIRE

    Feeney, D J; Pollard, J D; McLeod, J G; Stewart, G. J.; Doran, T J

    1990-01-01

    HLA typing of 71 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) showed an overall increase in frequencies of HLA-A3, -B7, -DR2 as well as concomitantly decreased frequencies of HLA-44 and DR7. The strongest associations were seen with HLA-DR2, -DR7 and -B44 in CIDP overall, although they did not reach statistical significance.

  10. Chronic inflammatory demyelinating polyneuropathy associated with carcinoma.

    OpenAIRE

    Antoine, J C; Mosnier, J. F.; Lapras, J; Convers, P.; Absi, L; Laurent, B.; Michel, D

    1996-01-01

    The association of chronic inflammatory demyelinating polyneuropathy (CIDP) and carcinoma has rarely been reported and its relevance is debated. Thirty three consecutive patients with probable or definite CIDP (idiopathic or associated with M protein) were investigated. Three patients with definite CIDP had a concomitant carcinoma. One had an IgM paraprotein. Steroids and intravenous immunoglobulins were effective.

  11. Steroids for Chronic Inflammatory Demyelinating Polyneuropathy

    Directory of Open Access Journals (Sweden)

    J Gordon Millichap

    2005-03-01

    Full Text Available The efficacy and safety of high-dose, intermittent IV methylprednisolone (IVMP as initial and long-term maintenance therapy for chronic inflammatory demyelinating polyneuropathy (CIDP were analyzed by a retrospective review of outcome data derived from patients’ medical records between 1992 and 2003 at Washington University School of Medicine, St Louis, MO.

  12. Management strategies in chronic inflammatory demyelinating polyradiculoneuropathy

    Directory of Open Access Journals (Sweden)

    Patel Kamakshi

    2010-01-01

    Full Text Available Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is a chronic, proximal and distal, asymmetrical or symmetrical, motor and sensory demyelinating polyneuropathy with a progressive course for at least 2 months. The accurate diagnosis is crucial as CIDP is amenable to treatment. Recent advances have provided new strategies and options for management of this syndrome. In this article, we review the clinical and diagnostic features as well as discuss recent insights and treatment strategies along with our experience in the management of patients with CIDP.

  13. Chronic inflammatory demyelinating polyradiculoneuropathy associated intracranial hypertension.

    Science.gov (United States)

    Altinkaya, Ayca; Topcular, Baris; Sakalli, Nazan Karagoz; Kuscu, Demet Yandim; Kirbas, Dursun

    2013-06-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired immune-mediated demyelinating neuropathy. In this report, we detail the course of a 58-year-old male patient who had headache and double vision followed by progressive paresthesia and difficulty in walking. The patient had bilateral papilledema and mild leg weakness, absent ankle jerks and loss of sensation in distal parts of his lower and upper extremities. His electromyography (EMG) was concordant with CIDP and lumbar puncture revealed high opening pressure. The polyradiculoneuropathy as well as the papilledema and elevated cerebrospinal fluid (CSF) pressure improved under steroids. The improvement in intracranial hypertension (IHT) and papilledema under steroid treatment suggests that the IHT in this patient might be associated with CIDP.

  14. Distribution patterns of demyelination correlate with clinical profiles in chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Kuwabara, S; Ogawara, K; Misawa, S; M. Mori; Hattori, T.

    2002-01-01

    Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder having a wide clinical range, and is characterised by multifocal demyelination that can involve the distal nerve terminals, intermediate nerve segments, and nerve roots.

  15. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Lehmann, Helmar C; Hughes, Richard A C; Hartung, Hans-Peter

    2013-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a sporadically occurring, acquired neuropathic condition of autoimmune origin with chronic progressive or relapsing-remitting disease course. CIDP is a treatable disorder; a variety of immunosuppressive and immunomodulatory agents are available to modify, impede, and even reverse the neurological deficits and sequelae that manifest in the course of the disease. However, in many cases CIDP is not curable. Challenges that remain in the treatment of CIDP patients are well recognized and include a remarkably individual heterogeneity in terms of disease course and treatment response as well as a lack of objective and feasible measures to predict and monitor the responsiveness to the available therapies. In this chapter an overview of the currently used drugs in the treatment of CIDP patients is given and some important and controversial issues that arise in the context of care for CIDP patients are discussed.

  16. [Acute-Onset Chronic Inflammatory Demyelinating Polyradiculoneuropathy].

    Science.gov (United States)

    Kanbayashi, Takamichi; Sonoo, Masahiro

    2015-11-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is characterized by an insidious onset showing progression over two months. However, up to 16% of CIDP patients may show acute presentation similar to Guillain-Barré syndrome (GBS). Such cases are termed acute-onset CIDP (A-CIDP). Distinguishing A-CIDP from GBS, especially the acute inflammatory demyelinating polyneuropathy (AIDP) subtype, is critical because therapeutic strategies and outcomes may differ between the two syndromes. Regarding clinical features, A-CIDP is less likely to have autonomic nervous system involvement, facial weakness, a preceding infectious illness, or the need for mechanical ventilation, in comparison with AIDP. Electrophysiological features are usually quite similar between the two, although follow-up studies may elucidate key differences. Around 8%-16% of GBS patients may show clinical deterioration shortly after improvement or stabilization following initial immunological therapy. Such a situation is termed treatment-related fluctuation (TRF; GBS-TRF). The distinction between GBS-TRF and A-CIDP is an important clinical issue because maintenance treatment is often required in CIDP. The diagnosis of A-CIDP should be considered when the condition of a patient with GBS deteriorates after nine weeks from onset, or when deterioration occurs three times or more.

  17. Chronic Inflammatory Demyelinating Polyradiculoneuropathy: From Bench to Bedside

    OpenAIRE

    Peltier, Amanda C.; Donofrio, Peter D.

    2012-01-01

    Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) is the most common treatable chronic autoimmune neuropathy. Multiple diagnostic criteria have been established, with the primary goal of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immuno-modulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulins (IVIg). This review describes th...

  18. Chronic inflammatory demyelinating polyradiculoneuropathy: from bench to bedside.

    Science.gov (United States)

    Peltier, Amanda C; Donofrio, Peter D

    2012-07-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is the most common treatable chronic autoimmune neuropathy. Multiple diagnostic criteria have been established, with the primary goal of identifying neurophysiologic hallmarks of acquired demyelination. Treatment modalities have expanded to include numerous immunomodulatory therapies, although the best evidence continues to be for corticosteroids, plasma exchange, and intravenous immunoglobulin (IVIg). This review describes the pathology, epidemiology, pathogenesis, diagnosis, and treatment of CIDP.

  19. Child Neurology: Chronic inflammatory demyelinating polyradiculoneuropathy in children

    OpenAIRE

    Markowitz, JA; Jeste, SS; Kang, PB

    2008-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder characterized by patchy demyelination of nerve roots and distal nerves. The course may be monophasic progressive or relapsing-remitting. CIDP is less common in children than in adults. As in adults, children with CIDP present with proximal and distal weakness and loss of deep tendon reflexes. Children are most often brought to medical attention due to gait disturbance and falling. As in adults, immunomo...

  20. A girl with atypical chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Chan, SHS; Mak, W; Wong, VCN

    2009-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronic, acquired immune and inflammatory disorder that targets the peripheral nerves. The cardinal features include a progressive or a relapsing-remitting course, predominant motor symptoms and signs, symmetrical involvement of arms and legs, proximal muscles involvement along with distal muscles, and decrease or absence of deep tendon reflexes. The diagnosis is confirmed by cerebrospinal fluid (CSF) protein elevation without pleo...

  1. Intractable chronic inflammatory demyelinating polyneuropathy treated successfully with ciclosporin

    OpenAIRE

    M. Odaka; Tatsumoto, M.; Susuki, K.; Hirata, K; YUKI, N

    2005-01-01

    Background: Chronic inflammatory demyelinating polyneuropathy (CIDP) is a heterogeneous disorder and both clinical course and response to treatment vary widely. Because of the propensity for relapse, CIDP requires maintenance therapy after the initial response to treatment. There is no consensus regarding this in the published literature.

  2. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy

    NARCIS (Netherlands)

    P.A. van Doorn (Pieter)

    1990-01-01

    textabstractPatients with a chronic inflammatory demyelinating polyneuropathy (CIDP) may respond to treatment with corticosteroids and to plasmapheresis, which was demonstrated in controlled clinical studies. In an uncontrolled study it was found that 13/17 CIDP patients had a rapid and clinical imp

  3. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype

    OpenAIRE

    Mathey, Emily K; Susanna B Park; Hughes, Richard A C; Pollard, John D.; Armati, Patricia J; Barnett, Michael H.; Taylor, Bruce V; Dyck, P. James B.; Kiernan, Matthew C; Lin, Cindy S-Y.

    2015-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to ...

  4. Human Endogenous Retrovirus and Neuroinflammation in Chronic Inflammatory Demyelinating Polyradiculoneuropathy

    OpenAIRE

    Faucard, Raphaël; Madeira, Alexandra; Gehin, Nadège; Authier, François-Jérôme; Panaite, Petrica-Adrian; Lesage, Catherine; Burgelin, Ingrid; Bertel, Mélanie; Bernard, Corinne; Curtin, François; Lang, Aloïs B.; Steck, Andreas J.; Perron, Hervé; Kuntzer, Thierry; Créange, Alain

    2016-01-01

    Background Human endogenous retroviruses HERV-W encode a pro-inflammatory protein, named MSRV-Env from its original identification in Multiple Sclerosis. Though not detected in various neurological controls, MSRV-Env was found in patients with chronic inflammatory demyelinating polyradiculoneuropathies (CIDPs). This study investigated the expression of MSRV in CIDP and evaluated relevant MSRV-Env pathogenic effects. Methods 50 CIDP patients, 19 other neurological controls (ONDs) and 65 health...

  5. Child neurology: chronic inflammatory demyelinating polyradiculoneuropathy in children.

    Science.gov (United States)

    Markowitz, Jennifer A; Jeste, Shafali S; Kang, Peter B

    2008-12-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disorder characterized by patchy demyelination of nerve roots and distal nerves. The course may be monophasic progressive or relapsing-remitting. CIDP is less common in children than in adults. As in adults, children with CIDP present with proximal and distal weakness and loss of deep tendon reflexes. Children are most often brought to medical attention due to gait disturbance and falling. As in adults, immunomodulatory treatment is the mainstay of therapy. Based on the small number of case series available, children with CIDP seem have a more favorable long-term course than adults.

  6. Characteristic MRI features of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Abe, Yuichi; Terashima, Hiroshi; Hoshino, Hideki; Sassa, Kaori; Sakai, Tetsuro; Ohtake, Akira; Kubota, Masaya; Yamanouchi, Hideo

    2015-10-01

    We present characteristic magnetic resonance imaging (MRI) features in a pediatric female patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Muscle weakness developed at 8 years old and fluctuated during the clinical course over 7 years. Electrophysiological studies showed a demyelination pattern with moderately delayed nerve conduction velocity, as well as dispersion phenomenon. MRI showed marked changes in thickening of the spinal nerve roots and their peripheral nerves in the lumber and brachial plexuses, as well as in the bilateral trigeminal nerves. It is suggested that these MRI features are characteristic and strongly supportive of the diagnosis of CIDP with a prolonged clinical course.

  7. Chronic inflammatory demyelinating polyradiculoneuropathy in a patient with Crohn's disease.

    Science.gov (United States)

    Ohyagi, Masaki; Ohkubo, Takuya; Yagi, Yousuke; Ishibashi, Satoru; Akiyama, Junko; Nagahori, Masakazu; Watanabe, Mamoru; Yokota, Takanori; Mizusawa, Hidehiro

    2013-01-01

    Crohn's disease (CD) is a chronic inflammatory disorder of the gastrointestinal tract that is frequently accompanied by systemic complications. Neuropathologies have not been well investigated as extraintestinal manifestations of CD. We herein report the case of a 36-year-old man with CD who presented with progressive weakness and numbness. A neurological examination and the results of a nerve conduction study and a sural nerve biopsy led to a diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Plasma exchanges were initially effective; however, the effects gradually declined starting 10 days after the plasma exchange (PE). These results suggest that humoral factors may play an important role in CIDP associated with CD.

  8. Treatment of chronic inflammatory demyelinating polyneuropathy with cyclosporin-A.

    OpenAIRE

    Mahattanakul, W; Crawford, T O; Griffin, J. W.; Goldstein, J. M.; Cornblath, D. R.

    1996-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated polyneuropathy for which there are effective therapies. However, not all patients improve with these treatments. Eight patients with CIDP, two with IgG monoclonal gammopathies, were treated with cyclosporin-A (3 to 5 mg/kg/day). In three, this treatment was successful. It was unsuccessful in four patients who were resistant to other treatments and in one who had initially received cyclosporin-A. There were no serio...

  9. Standard and escalating treatment of chronic inflammatory demyelinating polyradiculoneuropathy

    OpenAIRE

    Yoon, Min-Suk; Chan, Andrew; Gold, Ralf

    2011-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired, immune-mediated polyradiculoneuritis that is progressive or relapsing over a period of at least 8 weeks. Although the exact pathogenesis is unclear, it is thought to be mediated by both cellular and humoral immune reactions directed against the peripheral nerve myelin or axon. CIDP also involves spinal nerve roots. Early medical treatment of CIDP is important to prevent axonal loss. Only three treatment regimens for CIDP...

  10. Chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus

    OpenAIRE

    Farzad Fatehi; Shahriar Nafissi; Keivan Basiri; Mostafa Amiri; Akbar Soltanzadeh

    2013-01-01

    Various forms of neuropathy are seen diabetic patients; chronic inflammatory demyelinating polyneuropathy (CIDP) seems not to be infrequent neuropathy in patients suffering from diabetes and it seems to be more common than in the general population; on the contrary, some authorities do not support pathogenetic association between diabetes mellitus (DM) and CIDP. Also, there are some controversies on the subject of CIDP treatment in diabetic patients. Some studies showed that patients with CID...

  11. Corneal confocal microscopy in chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Stettner, Mark; Hinrichs, Lena; Guthoff, Rainer; Bairov, Silja; Petropoulos, Ioannis N.; Warnke, Clemens; Hartung, Hans‐Peter; Malik, Rayaz A.; Kieseier, Bernd C.

    2015-01-01

    Abstract Objective There is an unmet need for better diagnostic tools to further delineate clinical subsets of heterogeneous chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) to facilitate treatment decisions. Corneal confocal microscopy (CCM) is a noninvasive and reproducible nerve imaging technique. This study evaluates the potential of CCM as a diagnostic surrogate in CIDP and MMN. Methods In a cross‐sectional prospective approach, 182 p...

  12. Epidemiology of chronic inflammatory demyelinating polyneuropathy abroad and in Russia

    OpenAIRE

    T. E. Popova; N. A. Shnayder; M. M. Petrova; T. Ya. Nikolaeva; E. A. Kantimirova

    2015-01-01

    Current article provides an overview of the results of epidemiological studies of chronic inflammatory demyelinating polyneuropathy (CIDP) in Russia and abroad. It is shown that the prevalence of CIDP is different in countries, due to the use of different diagnostic criteria. It should be noted that the reliability of epidemiological prevalence and incidence is affected by difficulties of diagnosis of atypical forms of the disease.

  13. An update on the management of chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Gorson, Kenneth C.

    2012-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an immune mediated disorder of the peripheral nervous system with clinical features that include weakness, sensory loss, imbalance, pain and impaired ambulation which may lead to substantial disability. This review highlights current treatment strategies for CIDP, how best to utilize proven therapies such as intravenous immunoglobulin, oral prednisone, pulse dexamethasone, and plasma exchange, and when and how to use alternative immu...

  14. Axonal and perikaryal involvement in chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Nagamatsu, M; TERAO, S; Misu, K.; M. Li; Hattori, N; Ichimura, M.; Sakai, M; Yamamoto, H.; Watanabe, H.(Max-Planck-Institut für Kernphysik, 69117, Heidelberg, Germany); Riku, S; Ikeda, E; Hata, J; Oda, M; M. Satake; Nakamura, N

    1999-01-01

    OBJECTIVES—To assess the extent of loss of myelinated nerve fibres and spinal motor neuron loss in chronic inflammatory demyelinating polyneuropathy (CIDP), a clinicopathological study was conducted on biopsied sural nerves and necropsied spinal cords from patients with CIDP.
METHODS—The myelinated fibre pathology of 71 biopsied sural nerves and motor neuron pathology of nine necropsied spinal cords at L4 levels in patients with CIDP were quantitatively and immunohistoche...

  15. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy.

    OpenAIRE

    van Doorn, P. A.

    1994-01-01

    Intravenous immunoglobulin (IVIg) treatment is shown to be effective in a selected group of patients with a chronic inflammatory demyelinating polyneuropathy (CIDP). The proportion of patients that improve after IVIg treatment varies between studies. Because 40% of a group of IVIg treated CIDP patients needed intermittent IVIg infusions to maintain their improved clinical condition, it is expected that IVIg is effective, at least in this subgroup of patients. However, the proportion of patien...

  16. Chronic inflammatory demyelinating polyneuropathy mimicking a lumbar spinal stenosis syndrome.

    OpenAIRE

    Ginsberg, L; Platts, A. D.; Thomas, P K

    1995-01-01

    A patient with chronic inflammatory demyelinating polyneuropathy (CIDP) established by biopsy developed cauda equina symptoms due to swelling of the nerve roots in the lumbar spinal canal. Magnetic resonance imaging of the lumbar spine showed profoundly thickened nerve roots from the level of the conus medullaris, filling the caudal thecal sac. Immunosuppressant treatment produced partial clinical and radiological resolution. This case shows that spinal compressive syndromes may occur in acqu...

  17. Treatment of chronic inflammatory demyelinating polyradiculoneuropathy with methotrexate

    OpenAIRE

    Fialho, D; Chan, Y‐C; Allen, D C; Reilly, M.M.; Hughes, R A C

    2006-01-01

    We discovered many reports of other immunosuppressive drugs being used in adults with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but none of methotrexate. As weekly low dose oral methotrexate is safe, effective, and well tolerated in other diseases, we treated 10 patients with otherwise treatment resistant CIDP. Seven showed improvement in strength by at least two points on the MRC sum score and three worsened. Only two showed an improvement in disability and both were a...

  18. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Doorn, Pieter

    1990-01-01

    textabstractPatients with a chronic inflammatory demyelinating polyneuropathy (CIDP) may respond to treatment with corticosteroids and to plasmapheresis, which was demonstrated in controlled clinical studies. In an uncontrolled study it was found that 13/17 CIDP patients had a rapid and clinical important improvement after infusion of Fresh Frozen Plasma (FFP). A beneficial response was also seen after-mtravenous rmmunoglobulin (Mg) treatment. The aims of this study were: - to evaluate the cl...

  19. Epidemiology of chronic inflammatory demyelinating polyneuropathy abroad and in Russia

    Directory of Open Access Journals (Sweden)

    T. E. Popova

    2015-01-01

    Full Text Available Current article provides an overview of the results of epidemiological studies of chronic inflammatory demyelinating polyneuropathy (CIDP in Russia and abroad. It is shown that the prevalence of CIDP is different in countries, due to the use of different diagnostic criteria. It should be noted that the reliability of epidemiological prevalence and incidence is affected by difficulties of diagnosis of atypical forms of the disease.

  20. [Anesthetic Management of Three Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy].

    Science.gov (United States)

    Maruyama, Naoko; Wakimoto, Mayuko; Inamori, Noriko; Nishimura, Shinya; Mori, Takahiko

    2015-08-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronically progressing or relapsing disease caused by immune-mediated peripheral neuropathy. We report the anesthetic management of three CIDP patients who underwent elective orthopedic surgeries. Owing to the risk of neuraxial anesthetics triggering demyelination, general anesthesia was selected to avoid epidural or spinal anesthesia or other neuraxial blockade. It was also judged prudent to avoid prolonged perioperative immobilization, which might compress vulnerable peripheral nerves. For Patient 1, general anesthesia was induced with propofol, remifentanil, and sevoflurane, and was maintained with sevoflurane and remifentanil. For Patients 2 and 3, general anesthesia was induced and maintained with propofol and remifentanil. For tracheal intubation, under careful monitoring with peripheral nerve stimulators, minimal doses of rocuronium (0.6-0.7 mg x kg(-1)) were administered. When sugammadex was administered to reverse the effect of rocuronium, all patients rapidly regained muscular strength. Postoperative courses were satisfactory without sequelae.

  1. [Therapeutic responsiveness in chronic inflammatory demyelinating polyradiculoneuropathy].

    Science.gov (United States)

    Iijima, Masahiro

    2011-11-01

    CIDP is autoimmune-associated peripheral neuropathy characterized by motor and sensory disturbances in each limb. While various phenotypes have been reported in CIDP, the essential pathogenesis is not elucidated yet. Clinicopathological study indicated axonal dysfunction (muscle atrophy and decreased compound muscular action potentials) is one of the most important factors in IVIg Non-responders. Furthermore, single nucleotide polymorphism (SNP) haplotype/diplotype analysis within a linkage disequilibrium block indicates transient axonal glycoprotein 1 (TAG-1), which controls proper distribution of potassium channels in juxtaparanode, is an important factor for IVIg responsiveness. Gene expression analysis of biopsied nerves supported the hypothesis that CIDP pathogenesis is involved in humoral and cellular immune system. With respect to IVIg responsiveness, expression profiles indicate whole CIDP patients need conventional immune-modulating therapies in somewhat, while we should re-consider how to use them. From aspects of gene expression results, Non-responders need not only conventional immune-modulating therapies but also other original modalities which could intervene the pathogenesis except Schwann/inflammatory cells while Responders with IVIg dependence should need stronger and longer immune-suppression.

  2. Chronic inflammatory demyelinating polyradiculoneuropathy: from pathology to phenotype.

    Science.gov (United States)

    Mathey, Emily K; Park, Susanna B; Hughes, Richard A C; Pollard, John D; Armati, Patricia J; Barnett, Michael H; Taylor, Bruce V; Dyck, P James B; Kiernan, Matthew C; Lin, Cindy S-Y

    2015-09-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory neuropathy, classically characterised by a slowly progressive onset and symmetrical, sensorimotor involvement. However, there are many phenotypic variants, suggesting that CIDP may not be a discrete disease entity but rather a spectrum of related conditions. While the abiding theory of CIDP pathogenesis is that cell-mediated and humoral mechanisms act together in an aberrant immune response to cause damage to peripheral nerves, the relative contributions of T cell and autoantibody responses remain largely undefined. In animal models of spontaneous inflammatory neuropathy, T cell responses to defined myelin antigens are responsible. In other human inflammatory neuropathies, there is evidence of antibody responses to Schwann cell, compact myelin or nodal antigens. In this review, the roles of the cellular and humoral immune systems in the pathogenesis of CIDP will be discussed. In time, it is anticipated that delineation of clinical phenotypes and the underlying disease mechanisms might help guide diagnostic and individualised treatment strategies for CIDP.

  3. Specific features of chronic inflammatory demyelinating polyneuropathy in children

    OpenAIRE

    A. L. Kurenkov; S. S. Nikitin; B. I. Bursagova; Kuzenkova, L.M.

    2012-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune peripheral neuropathy that affects both adults and children. The basis for the paper is the analysis of 5 cases of CIDP in children (3 girls and 2 boys) aged 5 to 17 years, followed up for 3 to 6 years. The types of its clinical picture and electromyographic changes at different disease stages are considered in detail. The course of the disease is traced during therapy with corticosteroids and intravenous human immunogl...

  4. Novel immunotherapeutic strategies in chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Mathis, Stéphane; Vallat, Jean-Michel; Magy, Laurent

    2016-02-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic immune-mediated neuropathy: it is clinically heterogeneous (relapsing-remitting form, chronic progressive form, monophasic form or CIDP having a Guillain-Barré syndrome-like onset), but potentially treatable. Although its pathophysiology remains largely unknown, CIDP is considered an immune-mediated neuropathy. Therefore, many immunotherapies have been proposed in this peripheral nervous system disorder, the most known efficient treatments being intravenous immunoglobulin, corticosteroids and plasma exchange. However, these therapies remain unsatisfactory for many patients, so numerous other immunotherapeutic strategies have been evaluated, based on their immunosuppressant or immunomodulatory potency. We have performed a large review of the literature about treatment in CIDP, with a special emphasis on novel and alternative immunotherapeutic strategies. PMID:26809024

  5. Improving the management of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Allen, Jeffrey A; Bril, Vera

    2016-06-01

    This article considers several issues of current interest relating to the management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including diagnostic pitfalls, differences between CIDP patients with and without concurrent diabetes mellitus and how to best measure treatment response in daily practice. Despite the availability of diagnostic criteria, many patients diagnosed with CIDP do not meet these criteria; reasons for misdiagnosis are discussed. There are no definitive predictors of treatment response in CIDP; however, certain clinical and electrophysiological characteristics may be helpful. Patients with CIDP and concurrent diabetes present an additional diagnostic challenge; the differences between these groups, including possible differences in response predictors are discussed. Finally, the most appropriate outcome measures for use in daily practice are considered. PMID:27230584

  6. [Subcutaneous immunoglobulin. Treatment in chronic inflammatory demyelinating polyradiculo-neuropathy].

    Science.gov (United States)

    Nogués, Martín A; Varela, Francisco J; Seminario, Gisela; Insúa, María C; Bezrodnik, Liliana

    2016-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired disease that may affect nerve roots and peripheral nerves. Despite its low incidence, diagnosis is particularly important because there are different effective treatments. Human immunoglobulin is one of the mainstays of the treatment. Although there are few studies up to date, subcutaneous immunoglobulin (IgSC) has been proposed as an alternative to intravenous administration with similar efficacy. We present three cases with definite CIDP, classified according to the European Federation of Neurological Societies / Peripheral Nerve, Society (EFNS /PNS) criteria in which was used SCIgG as a treatment after success with the intravenous route. The Overall Neuropathy Limitations Scale (ONLS) was used to estimate the changes in the muscular strength before and after treatment. PMID:26826992

  7. Chronic inflammatory demyelinating polyneuropathy associated with diabetes mellitus

    Directory of Open Access Journals (Sweden)

    Farzad Fatehi

    2013-01-01

    Full Text Available Various forms of neuropathy are seen diabetic patients; chronic inflammatory demyelinating polyneuropathy (CIDP seems not to be infrequent neuropathy in patients suffering from diabetes and it seems to be more common than in the general population; on the contrary, some authorities do not support pathogenetic association between diabetes mellitus (DM and CIDP. Also, there are some controversies on the subject of CIDP treatment in diabetic patients. Some studies showed that patients with CIDP-DM considerably had recovered following treatment with immunotherapeutic modalities like (Intravenous immunoglobulin IVIG and conversely, some else have argued against the prescription of IVIG in this group and recommend treatment with corticosteroids and provided that resistant, rituximab may be beneficial. The main limitation in most studies is the inadequate number of cases and as a result, problematic decision making in treatment. This article represents an inclusive review of diabetic CIDP presentation and treatment.

  8. Subcutaneous immunoglobulin preserves muscle strength in chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Markvardsen, L H; Harbo, T; Sindrup, S H;

    2014-01-01

    BACKGROUND AND PURPOSE: Subcutaneous immunoglobulin (SCIG) is superior to placebo treatment for maintenance of muscle strength during 12 weeks in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The present study evaluated whether SCIG preserves muscle strength for 1 year...... in an open-label follow-up study. METHODS: Seventeen responders to intravenous immunoglobulin (IVIG) who had participated in the previous study of SCIG versus placebo in CIDP were included. After one IVIG infusion 2 weeks prior to baseline, all continued on SCIG treatment at weekly equal dosage and were...... remained unchanged. CONCLUSION: SCIG preserves muscle strength and functional ability in patients with CIDP who previously responded to IVIG. SCIG should be considered as an alternative in long-term treatment of CIDP patients....

  9. Improving the management of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Allen, Jeffrey A; Bril, Vera

    2016-06-01

    This article considers several issues of current interest relating to the management of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including diagnostic pitfalls, differences between CIDP patients with and without concurrent diabetes mellitus and how to best measure treatment response in daily practice. Despite the availability of diagnostic criteria, many patients diagnosed with CIDP do not meet these criteria; reasons for misdiagnosis are discussed. There are no definitive predictors of treatment response in CIDP; however, certain clinical and electrophysiological characteristics may be helpful. Patients with CIDP and concurrent diabetes present an additional diagnostic challenge; the differences between these groups, including possible differences in response predictors are discussed. Finally, the most appropriate outcome measures for use in daily practice are considered.

  10. Long-term immunoglobulin therapy for chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Rajabally, Yusuf A

    2015-05-01

    Immunoglobulins are an effective but expensive treatment for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Although the goal is to improve function, use of functional scales to monitor therapy is not widespread. Limited recent evidence suggests that doses lower than those used traditionally may be as effective. There are no proven correlations of effective dose with weight, disease severity, or duration. The clinical course of CIDP is heterogeneous and includes monophasic forms and complete remissions. Careful monitoring of immunoglobulin use is necessary to avoid overtreatment. Definitive evidence for immunoglobulin superiority over steroids is lacking. Although latest trial evidence favors immunoglobulins over steroids, the latter may result in higher remission rates and longer remission periods. This article addresses the appropriateness of first-line, high-dose immunoglobulin treatment for CIDP and reviews important clinical questions regarding the need for long-term therapy protocols, adequate monitoring, treatment withdrawal, and consideration of corticosteroids as an alternative to immunoglobulin therapy.

  11. New insights into the management of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Rajabally, Yusuf A; Blomkwist-Markens, Patricia H; Katzberg, Hans D

    2015-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and its variants can be challenging to diagnose and treat. A combination of clinical, electrophysiological and laboratory features is often required to reach a diagnosis. New data are emerging about potential biomarkers and factors that may indicate treatment needs in individual patients. High-quality evidence exists for the efficacy of intravenous immunoglobulin (IVIG) in the treatment of CIDP, including quality of life (QoL) benefits. Besides pharmacological treatment, psychological factors must also be addressed to improve patients' QoL. Home-based IVIG infusion therapy is currently a well-established approach in some countries. A 6-month pilot study conducted in Ontario, Canada, provided proof of safety and patient acceptance of home-based IVIG therapy, although some logistical issues emerged.

  12. Specific features of chronic inflammatory demyelinating polyneuropathy in children

    Directory of Open Access Journals (Sweden)

    A. L. Kurenkov

    2012-01-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is an autoimmune peripheral neuropathy that affects both adults and children. The basis for the paper is the analysis of 5 cases of CIDP in children (3 girls and 2 boys aged 5 to 17 years, followed up for 3 to 6 years. The types of its clinical picture and electromyographic changes at different disease stages are considered in detail. The course of the disease is traced during therapy with corticosteroids and intravenous human immunoglobulin and plasmapheresis. The results of the authors’ observations are compared with those of investigations conducted by other authors. The consideration of the diagnosis of CIDP and its treatment options focuses on that the international standards must be necessarily met to minimize errors in its differential diagnosis and management of these patients, and to make the prognosis for the disease.

  13. [Chronic inflammatory demyelinating polyradiculoneuropathy: clinical heterogeneity and therapeutic perspectives].

    Science.gov (United States)

    Leger, Jean-Marc; Bombelli, Francesco; Tran-Thanh, Hung; Chassande, Bénédicte; Maisonobe, Thierry; Viala, Karine

    2010-01-01

    Since the first description of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) by PJ Dyck's group at the Mayo Clinic 35 years ago, a wide range of publications have underlined the clinical, electrophysiologic and histopathologic heterogeneity of this disease. Expert consensus opinion is that CIDP should be considered in any patient with progressive symmetrical or asymmetrical polyradiculoneuropathy whose clinical course is relapsing and remitting or progresses for more than two months, especially if there are positive sensory symptoms, proximal weakness, are flexia without wasting, or preferential loss of vibration or joint-position sense. Electrophysiologic features of demyelinating polyneuropathy (especially conduction blocks) and elevated protein levels in cerebrospinal fluid may assist with the diagnosis. However, various clinical pictures have been described in patients with CIDP including pure motor or sensory impairment, and distal, multifocal or focal distribution. Two specific points have recently been emphasized:--while most CIDP patients have chronic onset, acute onset resembling Guillain-Barré syndrome may sometimes occur;--pure sensory forms may require different diagnostic strategies, including the use of somatosensory evoked potentials showing abnormal proximal sensory conduction, and nerve biopsy showing macrophage-associated demyelination, onion bulb formation, demyelinated and partially remyelinated nerve fibres, endoneurial edema, endoneurial mononuclear cell infiltration, and variation between fascicles. Several sets of diagnostic criteria for CIDP have been proposed, with different sensitivities and specificities. The European Federation of Neurological Societies/Peripheral Nerve Society criteria strike a balance between specificity, which needs to be higher for research purposes than for clinical diagnosis, and sensitivity, which, if too low, might lead to some cases being missed. CIDP patients may have a variety of

  14. Newer therapeutic options for chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Kuitwaard, Krista; van Doorn, Pieter A

    2009-05-29

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated disorder with variable symptoms and severity that can be difficult to diagnose. Intravenous immunoglobulin, plasma exchange and corticosteroids have all been proven to be beneficial in randomized controlled trials, although the proof for corticosteroids is less clear. Although these treatments are likely to be similar in efficacy, they differ in terms of their cost, availability and adverse effects. These characteristics should be taken into account when deciding which treatment to offer a patient. If there is no response to the first treatment option, one of the other treatments should be tried. Patients with a pure motor CIDP may deteriorate after corticosteroid treatment. Some patients do not respond or become refractory or intolerant to these conventional treatments. Those who become unresponsive to therapy should be checked again for the appearance of a monoclonal protein or other signs of malignancy. Over the years, small non-randomized studies have reported possible beneficial effects of various immunosuppressive agents. A Cochrane review concluded that currently there is insufficient evidence to decide whether these immunosuppressive drugs are beneficial in CIDP. When giving immunosuppressive drugs, one should be aware that some might even cause demyelinating disease. It is difficult to prove beneficial effects of these newer treatments since they have only been used in small groups of patients, who are refractory to other treatments, and often in combination with other treatments. CIDP patients can deteriorate during or after infections or improve spontaneously, making it more difficult to judge treatment efficacy. Various treatments for CIDP are described such as azathioprine, ciclosporin, cyclophosphamide, interferons, methotrexate, mycophenolate mofetil, rituximab and etanercept. An overview of these newer treatments, their mode of action, adverse effects and

  15. Randomised controlled trial comparing two different intravenous immunoglobulins in chronic inflammatory demyelinating polyradiculoneuropathy

    NARCIS (Netherlands)

    K. Kuitwaard; L.H. van den Berg; M. Vermeulen; E. Brusse; E.A. Cats; A.J. van der Kooi; N.C. Notermans; W.L. van der Pol; I.N. van Schaik; S.I. van Nes; W.C.J. Hop; P.A. van Doorn

    2010-01-01

    Background Different preparations of intravenous immunoglobulin (IVIg) are considered to have comparable clinical efficacy but this has never been formally investigated. Some patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) report that some IVIg brands are more effectiv

  16. Stance Postural Strategies in Patients with Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

    Directory of Open Access Journals (Sweden)

    Steno Rinalduzzi

    Full Text Available Polyneuropathy leads to postural instability and an increased risk of falling. We investigated how impaired motor impairment and proprioceptive input due to neuropathy influences postural strategies.Platformless bisegmental posturography data were recorded in healthy subjects and patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP. Each subject stood on the floor, wore a head and a hip electromagnetic tracker. Sway amplitude and velocity were recorded and the mean direction difference (MDD in the velocity vector between trackers was calculated as a flexibility index.Head and hip postural sway increased more in patients with CIDP than in healthy controls. MDD values reflecting hip strategies also increased more in patients than in controls. In the eyes closed condition MDD values in healthy subjects decreased but in patients remained unchanged.Sensori-motor impairment changes the balance between postural strategies that patients adopt to maintain upright quiet stance. Motor impairment leads to hip postural strategy overweight (eyes open, and prevents strategy re-balancing when the sensory context predominantly relies on proprioceptive input (eyes closed.

  17. Clinical and electrophysiological study of chronic inflammatory demyelinating polyneuropathy

    Institute of Scientific and Technical Information of China (English)

    秦绍森; 玛依努尔; 王湘

    2001-01-01

    Objective To investigate the clinical and electrophysiological features of chronic inflammatory demyelinating polyneuropathy (CIDP) . Methods The clinical symptoms and signs of 11 patients with CIDP were studied, motor conduction velocity( MCV), sensory con-duction velocity (SCV) and Electromyography (EMG) were also respectively carried out on 54 motor nerves, 28 sensory nerves and 21 musclesof these 11 cases. The amplitudes of compound muscle action potential(CAMP) obtained from distal and proximal ends were compared to as-certain the presence of conduction block (CB) by stimulating the segments starting from the distal ends. Results More than 3 nerves werefound involved in 10 out of 11 cases, slow MCV were found in 52%, prolongation of the distal latency in 64%, reduction of the amplitudes ofCAMP in 68%, CB in 26%, slow SCV in 85. 7%. EMG revealed neurogenic damage in 81%. Conclusion CIDP is a peripheral de- myelinating neuropathy involving not only the prox imal and distal segments but also the sensory and motor nerves. If there were no conditionsto perform nerve biopsy, testing of protein in CSF and electrophysiology mightbe of important diagnostic value for CIDP.

  18. Chronic inflammatory demyelinating polyradiculoneuropathy complicating anti TNF α therapy for chronic plaque psoriasis

    OpenAIRE

    Ahmed, Zahra; Powell, Robert; Llewelyn, Gareth; Anstey, Alex

    2011-01-01

    A 53-year-old woman with chronic plaque psoriasis treated with adalimumab (antitumour necrosis factor (anti TNF) α therapy) for 10 months presented with an 8 week history of hyperesthesia in a ‘glove and stocking’ distribution and clumsiness on walking. Nerve conduction studies confirmed the clinical diagnosis of a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). She was admitted and treated with intravenous immunoglobulin and oral steroids and made an excellent recovery. To ...

  19. [Treatment options for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)].

    Science.gov (United States)

    Kuntzer, T

    2006-04-01

    Limits of treatment in chronic inflammatory demyelinating poly(radiculo)neuropathies (CIDP) patients are better known thanks to recent Cochrane reviews. (1) Randomized controlled trials have only focused on short-term effects, but most patients need long-term therapy, (2) There are three proven effective treatments available (prednisone; intravenous immunoglobulin or IVIg and plasma exchange or PE) which are useful in more than 60 p. 100 of patients, (3) New open studies indicated possible efficacy for mycophenolate, rituximab, etanercept, ciclosporine and interferons, and (4) Whether CIDP variants need specific treatment is still unknown. Many CIDP patients need treatment for years. The fear of side effects during long-term steroid treatment, the high costs of IVIg, the necessity for specialized equipment and the invasive nature of PE, are important factors determining the choice for one of these treatments. In most up-to-date treatment options, patients are initially treated with IVIg at a dosage of 2 g/kg administered for 25 days, clinical improvement can be judged within 10 days. The percentage of patients responding seems to be approximately 70 percent, with a very high chance (approximately 85 percent) that repeated administration of IVIg will be necessary, explaining why most neurologists add an immunosuppressive drug at this stage, but there is no consensus concerning the best drug to be used. Combinations of drugs are most likely to be useful in the next future, using IVIg, prednisone, and a immunosuppressor agent, such as mycophenolate, rituximab, etanercept, or ciclosporine. General measures to rehabilitate patients and to manage symptoms like fatigue and other residual findings are important.

  20. Immunopathogenesis of Guillain-Barré syndrome and chronic inflammatory demyelinating polyradoculoneuropathy

    OpenAIRE

    Press, Rayomand

    2002-01-01

    Guillain-Barre syndrome (GBS) is an inflammatory polyradiculoneuropathy with acute onset and usually a spontaneous recovery. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic progressive inflammatory neuropathy. GBS and CIDP are associated with high morbidity despite treatment with immunomodulatory drugs. Both conditions are associated with inflammation of spinal nerve roots and/or distal nerves of the peripheral nervous system (PNS). GBS is assum...

  1. An atypical chronic inflammatory demyelinating polyradiculoneuropathy that radiologically mimicking neurofibromatosis: Case report

    OpenAIRE

    AKPINAR, Kursad Cetin; DOGRU, Hakan; Balci, Kemal; Terzi, Murat

    2014-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon demyelinating disorder with a relapsing and remitting or continuously progressive course. Patients may have motor and sensory involvement, but generally motor involvement may be more prominent and more severe in lower extremities. CIDP is a treatable neuropathy that is challenging to diagnose and has a broad spectrum of presentations. When ranked by the descending frequency, postural tremor in the arms, peripheral...

  2. Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

    OpenAIRE

    Narges Karimi; Athena Sharifi; Ashraf Zarvani; Hamed Cheraghmakani

    2015-01-01

    Context: Chronic inflammatory demyelinating polyradiculopathy (CIDP) is an acquired and autoimmune neuropathy, characterized by a chronic, rapidly progressive, symmetric weakness. In children, abnormal gait is as a first symptom of muscle weakness. Evidence Acquisition: The diagnosis of CIDP is on the basis of clinical characteristics, electrodiagnostic that shows the severity of the disease, lumbar puncture and spine magnetic res...

  3. Chronic inflammatory demyelinating polyneuropathy in adults: diagnostic approaches and first line therapy

    OpenAIRE

    N. А. Suponevа; E. S. Naumovа; E. V. Gnedovskaya

    2016-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is among the key reasons of chronic polyneuropathies in adults. Diagnostic algorithm of CIDP in adults is presented. Diagnosis of CIDP is based on clinical and electrodiagnostic criteria of European Federation of Neurological Societies/Peripheral Nervous System in 2010. Principles of CIDP treatment are discussed, including modern trends of standard and 10 % IVIG solutions. 

  4. Chronic inflammatory demyelinating polyradiculoneuropathy complicating anti TNF α therapy for chronic plaque psoriasis.

    Science.gov (United States)

    Ahmed, Zahra; Powell, Robert; Llewelyn, Gareth; Anstey, Alex

    2011-12-01

    A 53-year-old woman with chronic plaque psoriasis treated with adalimumab (antitumour necrosis factor (anti TNF) α therapy) for 10 months presented with an 8 week history of hyperesthesia in a 'glove and stocking' distribution and clumsiness on walking. Nerve conduction studies confirmed the clinical diagnosis of a chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). She was admitted and treated with intravenous immunoglobulin and oral steroids and made an excellent recovery. To our knowledge, this is the first published report of CIDP associated with anti TNF α therapy given to treat psoriasis.

  5. Challenges in the treatment of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Guimarães-Costa, R; Iancu Ferfoglia, R; Viala, K; Léger, J-M

    2014-10-01

    Chronic idiopathic demyelinating polyradiculoneuropathy (CIDP) is a rare disease, the most frequent one within the spectrum of the so-called "chronic immune-mediated neuropathies". Challenges in the treatment of CIDP firstly concern its diagnosis, which may be difficult, mainly for the atypical forms. Secondly, challenges encompass the choice of the first-line treatment, such as corticosteroids, intravenous immunoglobulins (IVIg), and plasma exchanges (PE) that have been proven as efficacious by several randomized controlled trials (RCT). Recent reports have focused on both different regimens of corticosteroids, and the occurrence of relapses following treatment with either corticosteroids or IVIg. These data may be helpful for the choice of the first-line treatment and may result in changing the guidelines for treatment of CIDP in clinical practice. The third and more difficult challenge is to manage long-term treatment for CIDP, since no immunomodulatory treatment has to date been proven as efficacious in this situation. Lastly, challenges in the treatment concern the choice of the best outcome measure for CIDP in RCT and clinical practice. The aim of this article is to overview the results of the more recently reported published trials for CIDP, and to give some insights for the current and future management of CIDP.

  6. Does the chronic inflammatory demyelinating polyradiculoneuropathy due to secondary cause differ from primary?

    OpenAIRE

    Vaibhav Wadwekar; Jayantee Kalita; Usha Kant Misra

    2011-01-01

    Background: The clinical presentation, neurophysiological findings, and outcome may vary between primary and secondary chronic inflammatory demyelinating polyradiculopathy (CIDP). Objective: To compare clinical and electrodiagnostic features of primary and secondary CIDP. Setting: Tertiary care teaching referral hospital. Materials and Methods: The CIDP patients who were diagnosed as per European Federation of Neurological Societies/Peripheral Nerve Society criteria were included and subjecte...

  7. Contactin 1 IgG4 associates to chronic inflammatory demyelinating polyneuropathy with sensory ataxia

    OpenAIRE

    Miura, Yumako; Devaux, Jérôme J.; Fukami, Yuki; Manso, Constance; Belghazi, Maya; Wong, Anna Hiu Yi; Yuki, Nobuhiro

    2015-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is clinically heterogeneous and shows varying responses to immunotherapy. In a cohort of 533 Japanese patients with CIDP, Miura et al. identify 13 patients with IgG4 antibodies against the axonal adhesion molecule, contactin-1. Antibodies are associated with subacute onset, sensory ataxia and good response to corticosteroids.

  8. Anaesthetic management and implications of a case of chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Babita Gupta; Pramendra Agrawal; Nita D′souza; Chhavi Sawhney

    2011-01-01

    A 60-year-old man with chronic inflammatory demyelinating polyneuropathy (CIDP) was posted for surgery of the neck femur fracture and was successfully managed. We discuss the anaesthetic considerations during regional and general anaesthesia of this patient with CIDP. A brief review of the available literature reveals no consensus on the choice of anaesthetic management.

  9. Anaesthetic management and implications of a case of chronic inflammatory demyelinating polyneuropathy

    Directory of Open Access Journals (Sweden)

    Babita Gupta

    2011-01-01

    Full Text Available A 60-year-old man with chronic inflammatory demyelinating polyneuropathy (CIDP was posted for surgery of the neck femur fracture and was successfully managed. We discuss the anaesthetic considerations during regional and general anaesthesia of this patient with CIDP. A brief review of the available literature reveals no consensus on the choice of anaesthetic management.

  10. Chronic inflammatory demyelinating polyneuropathy in children: a report of four patients with variable relapsing courses

    OpenAIRE

    Chang, Soo Jin; Lee, Ji Hyun; Kim, Shin Hye; Lee, Joon Soo; Kim, Heung Dong; Kang, Joon Won; Lee, Young Mock; Kang, Hoon-Chul

    2015-01-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is a chronically progressive or relapsing symmetric sensorimotor disorder presumed to occur because of immunologic antibody-mediated reactions. To understand the clinical courses of CIDP, we report variable CIDP courses in children with respect to initial presentation, responsiveness to medical treatment, and recurrence interval. Four patients who were diagnosed with acute-onset and relapsing CIDP courses at Severance Children's Hospita...

  11. Impaired inhibitory Fcγ receptor IIB expression on B cells in chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Tackenberg, Björn; Jelčić, Ilijas; Baerenwaldt, Anne; Wolfgang H Oertel; Sommer, Norbert; Nimmerjahn, Falk; Lünemann, Jan D.

    2009-01-01

    The inhibitory Fc-γ receptor FcγRIIB, expressed on myeloid and B cells, has a critical role in the balance of tolerance and autoimmunity, and is required for the antiinflammatory activity of intravenous Ig (IVIG) in various murine disease models. However, the function of FcγRIIB and its regulation by IVIG in human autoimmune diseases are less well understood. Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common treatable acquired chronic polyneuropathy, and IVIG is wide...

  12. Chronic inflammatory demyelinating polyneuropathy in adults: diagnostic approaches and first line therapy

    Directory of Open Access Journals (Sweden)

    N. А. Suponevа

    2016-01-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is among the key reasons of chronic polyneuropathies in adults. Diagnostic algorithm of CIDP in adults is presented. Diagnosis of CIDP is based on clinical and electrodiagnostic criteria of European Federation of Neurological Societies/Peripheral Nervous System in 2010. Principles of CIDP treatment are discussed, including modern trends of standard and 10 % IVIG solutions. 

  13. Chronic Inflammatory Demyelinating Polyneuropathy Following Anti-TNF-α Therapy With Infliximab for Crohn's Disease.

    Science.gov (United States)

    Kamel, Amir Y; Concepcion, Orestes; Schlachterman, Alexander; Glover, Sarah; Forsmark, Christopher Y

    2016-04-01

    We present a 29-year-old male with Crohn's disease who developed chronic inflammatory demyelinating polyneuropathy (CIDP) related to infliximab therapy. He developed lower extremity weakness and dysesthesia 3 weeks after a fourth infliximab dose. Laboratory examination revealed an elevated cerebrospinal fluid protein without pleocytosis. The patient initially responded to plasmapheresis therapy with marked symptomatic improvement, but relapsed and was refractory to subsequent treatments with plasmaphereisis, intravenous immunoglobulin, and glucocorticoids. While a causal relationship between infliximab and CIDP cannot be proven, clinicians should monitor Crohn's disease patients who are receiving TNF-α antagonists for neurologic symptoms suggestive of demyelinating disease. PMID:27144200

  14. Chronic Inflammatory Demyelinating Polyneuropathy Following Anti-TNF-α Therapy With Infliximab for Crohn's Disease

    Science.gov (United States)

    Concepcion, Orestes; Schlachterman, Alexander; Glover, Sarah; Forsmark, Christopher Y.

    2016-01-01

    We present a 29-year-old male with Crohn's disease who developed chronic inflammatory demyelinating polyneuropathy (CIDP) related to infliximab therapy. He developed lower extremity weakness and dysesthesia 3 weeks after a fourth infliximab dose. Laboratory examination revealed an elevated cerebrospinal fluid protein without pleocytosis. The patient initially responded to plasmapheresis therapy with marked symptomatic improvement, but relapsed and was refractory to subsequent treatments with plasmaphereisis, intravenous immunoglobulin, and glucocorticoids. While a causal relationship between infliximab and CIDP cannot be proven, clinicians should monitor Crohn's disease patients who are receiving TNF-α antagonists for neurologic symptoms suggestive of demyelinating disease. PMID:27144200

  15. Diffuse spinal and intercostal nerve involvement in chronic inflammatory demyelinating polyradiculoneuropathy: MRI findings

    Energy Technology Data Exchange (ETDEWEB)

    Oguz, B.; Oguz, K.K.; Cila, A. [Dept. of Radiology, Hacettepe Univ. Faculty of Medicine, Ankara (Turkey); Tan, E. [Dept. of Neurology, Hacettepe Univ. Faculty of Medicine, Ankara (Turkey)

    2003-12-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an uncommon demyelinating disorder with a relapsing and remitting or continuously progressive course. Hypertrophic nerve roots, sometimes associated with gadolinium enhancement, has been reported more commonly in lumbar spine and less commonly in the brachial plexus and cervical roots; however, diffuse involvement of intercostal nerves bilaterally has never been reported previously. We present MRI findings which include diffuse enlargement and mild enhancement of roots and extraforaminal segments of nerves in all segments except a short segment between T12-L2 as well as all the intercostal nerves in a case of CIPD with a 10-year history. (orig.)

  16. Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

    Directory of Open Access Journals (Sweden)

    Mohamed Mahdi-Rogers

    2010-03-01

    Full Text Available Mohamed Mahdi-Rogers, Yusuf A RajaballyNeuromuscular Clinic, Department of Neurology, University Hospitals of Leicester, Leicester, UKAbstract: Chronic inflammatory demyelinating polyneuropathy (CIDP is an acquired heterogeneous disorder of immune origin affecting the peripheral nerves, causing motor weakness and sensory symptoms and signs. The precise pathophysiology of CIDP remains uncertain although B and T cell mechanisms are believed to be implicated. Intravenous immunoglobulins (IVIg have been shown in a number of trials to be an effective treatment for CIDP. IVIg is thought to exert its immunomodulatory effects by affecting several components of the immune system including B-cells, T-cells, macrophages and complement. This article provides an overview of the pathogenesis of CIDP and of its treatment with IVIg.Keywords: chronic inflammatory demyelinating polyneuropathy, intravenous immunoglobulin, pathogenesis, treatment

  17. Diagnostic value of sural nerve biopsy in chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    Molenaar, D.S.M.; Vermeulen, M.; Haan

    1998-01-01

    OBJECTIVE—To investigate the additional diagnostic value of sural nerve biopsy of 64 patients in whom chronic inflammatory demyelinating polyneuropathy (CIDP) was considered, as sural nerve biopsy is recommended in the research criteria of an ad hoc subcommittee to diagnose CIDP.
METHODS—Firstly, the additional diagnostic value of sural nerve biopsy was analysed with multivariate logistic re- gression. Six clinical features (remitting course, symmetric sensorimotor neurop...

  18. Comparison of monoclonal gammopathy of undetermined significance-associated neuropathy and chronic inflammatory demyelinating polyneuropathy patients

    OpenAIRE

    Alkhawajah, Nuha M.; Dunnigan, Samantha K.; Bril, Vera

    2014-01-01

    Objectives There are varying reports on whether monoclonal gammopathy of undetermined significance-associated neuropathy (MGUSN) patients are distinguishable from those with chronic inflammatory demyelinating polyneuropathy (CIDP) and whether specific MGUSN subclasses are associated with specific clinical phenotypes. Methods We performed a retrospective chart review of MGUSN (n = 56) and CIDP (n = 67) patients. Data extracted included: demographics, neurological examination, and nerve conduct...

  19. Overview of the pathogenesis and treatment of chronic inflammatory demyelinating polyneuropathy with intravenous immunoglobulins

    OpenAIRE

    Mohamed Mahdi-Rogers; Yusuf A Rajabally

    2010-01-01

    Mohamed Mahdi-Rogers, Yusuf A RajaballyNeuromuscular Clinic, Department of Neurology, University Hospitals of Leicester, Leicester, UKAbstract: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an acquired heterogeneous disorder of immune origin affecting the peripheral nerves, causing motor weakness and sensory symptoms and signs. The precise pathophysiology of CIDP remains uncertain although B and T cell mechanisms are believed to be implicated. Intravenous immunoglobulins (IVIg) ...

  20. Chronic Inflammatory Demyelinating Polyneuropathy Following Anti-TNF-α Therapy With Infliximab for Crohn's Disease

    OpenAIRE

    Kamel, Amir Y.; Concepcion, Orestes; Schlachterman, Alexander; Glover, Sarah; Forsmark, Christopher Y.

    2016-01-01

    We present a 29-year-old male with Crohn's disease who developed chronic inflammatory demyelinating polyneuropathy (CIDP) related to infliximab therapy. He developed lower extremity weakness and dysesthesia 3 weeks after a fourth infliximab dose. Laboratory examination revealed an elevated cerebrospinal fluid protein without pleocytosis. The patient initially responded to plasmapheresis therapy with marked symptomatic improvement, but relapsed and was refractory to subsequent treatments with ...

  1. Unusual features in chronic inflammatory demyelinating polyneuropathy: Good outcome after prolonged ventilatory support

    OpenAIRE

    Sanjeev Jha; M K Ansari; K K Sonkar; Paliwal, V. K.

    2011-01-01

    Severe respiratory muscle paralysis and ventilatory failure is rare in chronic inflammatory demyelinating polyneuropathy (CIDP). We report a 14 year child who presented with respiratory failure, bulbar and multiple cranial nerves involvement along with bilateral phrenic nerve paralysis. He was diagnosed with CIDP after electrophysiological evaluation. He required AMBU ventilation for about 4 months (including domiciliary use), after which he recovered significantly. Along with several unusual...

  2. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating polyneuropathy: a double blind, placebo controlled study.

    OpenAIRE

    Vermeulen, M.; van Doorn, P. A.; Brand, A; Strengers, P F; Jennekens, F G; Busch, H F

    1993-01-01

    Patients with a clinical diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) were randomised in a double-blind, placebo-controlled multicentre trial to investigate whether high-dose intravenous immunoglobulin treatment (IVIg) for 5 consecutive days has a beneficial effect. Fifteen patients were randomised to IVIg and 13 to placebo. In the IVIg treatment group 4 patients improved and 3 patients in the placebo group. The degree of improvement of the patients in the IVIg treatm...

  3. Intravenous immunoglobulin treatment in patients with chronic inflammatory demyelinating neuropathy not responsive to other treatments.

    OpenAIRE

    Nemni, R; Amadio, S; Fazio, R; GALARDI, G; Previtali, S; G. Comi

    1994-01-01

    Nine patients with chronic inflammatory demyelinating poliradiculoneuropathy (CIDP) were treated with intravenous immunoglobulin. All patients had been previously treated with prednisone and/or plasma exchange without effect. Objective improvement in clinical condition occurred in six patients. One patient became refractory after two treatment courses, two patients had no response. The results indicate that intravenous immunoglobulin has beneficial effects in a high percentage of patients wit...

  4. Chronic inflammatory demyelinating polyneuropathy-like neuropathy as an initial presentation of Crohn’s disease

    OpenAIRE

    Kim, Suji; Kang, Seok-Jae; Oh, Ki-Wook; Ahn, Byung Kyu; Lee, Hang Lak; Han, Dong Soo; Jang, Kiseok; Kim, Young Seo

    2015-01-01

    Background Chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare complication of Crohn's disease (CD), and it is uncertain whether it is associated with CD itself or with its treatment. We describe a case of CIDP-like neuropathy as an initial symptom of CD. The neurologic symptoms of the patient which responded partially to intravenous immunoglobulin (IVIG) recovered after resection of the appendiceal CD. Case presentation A 17-year-old male had experienced three separate attacks...

  5. A review of the use of biological agents for chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Stübgen, Joerg-Patrick

    2013-03-15

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is a group of idiopathic, acquired, immune-mediated inflammatory demyelinating diseases of the peripheral nervous system. A majority of patients with CIDP respond to "first-line" treatment with IVIG, plasmapheresis and/or corticosteroids. There exists insufficient evidence to ascertain the benefit of treatment with "conventional" immunosuppressive drugs. The inconsistent efficacy, long-term financial burden and health risks of non-specific immune altering therapy have drawn recurrent attention to the possible usefulness of a variety of biological agents that target key aspects in the CIDP immunopathogenic pathways. This review aims to give an updated account of the scientific rationale and potential use of biological therapeutics in patients with CIDP. No specific treatment recommendations are given. The discovery, development and application of biological markers by modern molecular diagnostic techniques may help identify drug-naïve or treatment-resistant CIDP patients most likely to respond to targeted immunotherapy.

  6. Unmyelinated nerve fiber degeneration in chronic inflammatory demyelinating polyneuropathy

    NARCIS (Netherlands)

    Bosboom, WMJ; Van den Berg, LH; Dieks, HJG; Plante, E; Veldman, H; Franssen, H; Wokke, JHJ

    2000-01-01

    To determine whether unmyelinated nerve fibers escape degeneration as one might expect in an immune response exclusively directed at myelin, we performed a morphometric examination of unmyelinated axons and myelinated nerve fibers in sural nerve biopsy specimens of 14 patients with a chronic inflamm

  7. Chronic inflammatory demyelinating polyradiculoneuropathy and variants: where we are and where we should go.

    Science.gov (United States)

    Nobile-Orazio, Eduardo

    2014-03-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic and often disabling sensory motor neuropathy postulated as caused by an immune attack against peripheral nerve myelin. In addition to a classic sensory–motor polyneuropathy, other phenotypes of CIDP have been described including the Lewis- Sumner syndrome, distal acquired demyelinating symmetric (DADS) neuropathy, pure motor CIDP, pure sensory CIDP including chronic immune sensory polyradiculopathy (CISP), and focal CIDP. These phenotypes are currently considered to be variants of CIDP, even if the possibility that they represent different demyelinating neuropathies cannot be fully excluded considering differences in their response to therapy. Several data support the role of the immune system in the pathogenesis of CIDP even if the precise targets and actors (antibodies and lymphocytes) of this immune response remain uncertain. Recent studies have shown that the therapeutic response may differ in patients with peculiar clinical presentations supporting the hypothesis that different pathogenetic mechanisms may underlie the heterogeneity of CIDP. The majority of patients with CIDP show improvement after immune therapies including corticosteroids, plasma exchange, and high-dose intravenous immunoglobulin (IVIg). It remains unclear why none of the other immune therapies that were reported to be variably effective in other immune disorders proved to be effective also in CIDP.

  8. Nerve sonography in multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy

    OpenAIRE

    D. S. Druzhinin; Naumova, E. S.; S. S. Nikitin

    2016-01-01

    The quantitative ultrasound characteristics (USC) of the median, ulnar nerve at different levels and the spinal nerves in patients with multifocal motor neuropathy (MMN; n=13; 40,4 ± 12,6 years old) and chronic inflammatory demyelinating polyneuropathy (CIDP; n = 7; 47,3 ± 11,2 year old) did not reveal statistical difference in cross sectional area (CSA) between analyzed groups. Patients with MMN have more pronounced asymmetry of CSA in comparison with CIDP patients which have a symmetrical p...

  9. Relapse with Dysphagia in a Case of Chronic Inflammatory Demyelinating Polyradiculoneuropathy.

    Science.gov (United States)

    Teramoto, Hiroko; Morita, Akihiko; Hara, Makoto; Ninomiya, Satoko; Shigihara, Shuntaro; Kusunoki, Susumu; Kamei, Satoshi

    2015-01-01

    Glossopharyngeal and/or vagus nerve involvement is infrequent in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We herein report the case of a 69-year-old Japanese woman who presented with muscle weakness and numbness of the extremities with dysphagia. The serum anti-ganglioside GM1 immunoglobulin IgM antibody levels were elevated, and treatment with intravenous immunoglobulin (IVIg) resulted in a dramatic improvement; the weakness, numbness and dysphagia all resolved. However, relapse comprising dysphagia alone occurred on hospital day 26, and treatment with IVIg again proved extremely effective. IVIg therapy can be effective against cranial nerve involvement in cases of CIDP.

  10. Subcutaneous immunoglobulin in responders to intravenous therapy with chronic inflammatory demyelinating polyradiculoneuropathy

    DEFF Research Database (Denmark)

    Markvardsen, Lars Høj; Debost, J-C; Harbo, Thomas;

    2013-01-01

    BACKGROUND AND PURPOSE: We hypothesized that subcutaneous administration of immunoglobulins (SCIG) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is feasible, safe and superior to treatment with saline for the performance of muscle strength. METHODS: Thirty patients with motor...... involvement in maintenance therapy with intravenous immunoglobulin (IVIG) fulfilling the EFNS/PNS criteria for CIDP, aged 18-80 years, were randomized either to SCIG at a dose corresponding to their pre-study IVIG dose or to subcutaneous saline given twice or thrice weekly for 12 weeks at home. At the start...

  11. Unusual features in chronic inflammatory demyelinating polyneuropathy: Good outcome after prolonged ventilatory support

    Directory of Open Access Journals (Sweden)

    Sanjeev Jha

    2011-01-01

    Full Text Available Severe respiratory muscle paralysis and ventilatory failure is rare in chronic inflammatory demyelinating polyneuropathy (CIDP. We report a 14 year child who presented with respiratory failure, bulbar and multiple cranial nerves involvement along with bilateral phrenic nerve paralysis. He was diagnosed with CIDP after electrophysiological evaluation. He required AMBU ventilation for about 4 months (including domiciliary use, after which he recovered significantly. Along with several unusual features of CIDP, this report highlights good example of steady basic intensive care to save lives and rewarding outcome of prolonged respiratory support, provided by AMBU ventilation which is a rather primitive, but inexpensive device.

  12. [Diagnostic strategy for chronic inflammatory demyelinating polyradiculoneuropathy. Recommendations of the French working group].

    Science.gov (United States)

    Magy, L

    2008-12-01

    The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) requires a careful clinical and neurophysiological evaluation, often completed by CSF analysis. In numerous cases, this diagnosis is straightforward and leads to rapid initiation of an immunomodulatory treatment. However, some patients are not diagnosed because of atypical clinical and/or neurophysiological features, and do not benefit from a potentially effective treatment. In this context, a working group was composed with the task of establishing recommendations on diagnostic strategies for CIDP in the main clinical situations where this diagnosis may be suspected. We have summarized these recommendations and tried to present them in the form of a decision-making algorithm.

  13. Sural nerve biopsy in chronic inflammatory demyelinating polyneuropathy: Are supportive pathologic criteria useful in diagnosis?

    Directory of Open Access Journals (Sweden)

    Kulkarni Girish

    2010-01-01

    Full Text Available Background : According to American Academy of Neurology (AAN criteria, demonstration of demyelination in the sural nerve by teased fiber or ultrastructure is considered mandatory for diagnosis of chronic inflammatory demyelinating polyneuropathies (CIDP. In resource-restricted settings where these techniques are not freely available, it is useful to determine the utility of ′supportive′ pathologic criteria (subperineurial edema, inflammation, onion bulb formation, and demyelination proposed by AAN for diagnosis of CIDP. Settings and Design : Tertiary care hospital, retrospective study. Patients and Methods : Forty-six patients with idiopathic CIDP (32 with progressive course and 14 with relapsing-remitting course satisfying AAN clinical and electrophysiologic criteria evaluated between January 1991 and August 2004 were reviewed. Frequency of specific pathological alterations such as demyelination, inflammation, onion bulb formation, and axonal changes in sural nerve biopsies was evaluated. Statistical Analysis : SPSS statistical package was used to calculate mean, range, and standard deviation. Student′s t test, chi-square test, and ANOVA were used for determining statistical significance. Results and Conclusion : Reduction in myelinated fiber density was most frequent (93.5%, followed by demyelination (82.8%, inflammation (58.7%, and onion bulb formation (28.3%. Endoneurial inflammation was frequent in the relapsing-remitting form and epineurial inflammation and axonal changes in those with progressive course. Greater disability at presentation, poor response to immunomodulation, and lower CSF protein levels was seen in those with axonal pathology. Pathological abnormalities were demonstrable in all (100%, whereas electrophysiological abnormalities were detected in 90.8%, suggesting that supportive histologic AAN criteria are helpful in diagnosis of CIDP.

  14. Atypical idiopathic inflammatory demyelinating lesions

    DEFF Research Database (Denmark)

    Wallner-Blazek, Mirja; Rovira, Alex; Fillipp, Massimo;

    2013-01-01

    Atypical lesions of a presumably idiopathic inflammatory demyelinating origin present quite variably and may pose diagnostic problems. The subsequent clinical course is also uncertain. We, therefore, wanted to clarify if atypical idiopathic inflammatory demyelinating lesions (AIIDLs) can be class...

  15. Involvement of the central nervous system in chronic inflammatory demyelinating polyneuropathy: a clinical, electrophysiological and magnetic resonance imaging study.

    OpenAIRE

    Ormerod, I E; Waddy, H M; Kermode, A G; Murray, N M; Thomas, P K

    1990-01-01

    In a consecutive series of 30 patients with chronic inflammatory demyelinating polyneuropathy (CIDP) minor clinical evidence of CNS involvement was found in five. Cranial magnetic resonance imaging (MRI) was performed in 28 and revealed abnormalities consistent with demyelination in nine patients aged less than 50 years and abnormalities in five aged 50 years or over. Measurements of central motor conduction time (CMCT) were obtained in 18 and showed unilateral or bilateral abnormalities in s...

  16. Electrophysiological Evaluation of Chronic Inflammatory Demyelinating Polyneuropathy and Charcot-Marie-Tooth Type 1: Dispersion and Correlation Analysis

    OpenAIRE

    Kang, Ji Hyuk; Kim, Hye Jeong; Lee, Eun Ryeong

    2013-01-01

    [Purpose] The purpose of this study was to analyze and compare electrophysiological characteristics observed in nerve conduction studies (NCS) of chronic inflammatory demyelinating polyneuropathy (CIDP) and Charcot-Marie-Tooth disease type 1 (CMT 1). [Subjects] A differential diagnosis of acquired and congenital demyelinating neuropathies was based on a study of 35 patients with NCS-confirmed CIDP and 30 patients with CMT 1 genetically proven by peripheral myelin protein-22 (PMP-22) gene anal...

  17. Dispersion of compound muscle action potential in hereditary neuropathies and chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Stanton, Michael; Pannoni, Valerie; Lewis, Richard A; Logigian, Eric L; Naguib, Demian; Shy, Michael E; Cleland, James; Herrmann, David N

    2006-10-01

    Distal compound muscle action potential (DCMAP) dispersion, defined as a DCMAP duration > or = 9 ms, and proximal-distal (P-D) CMAP dispersion are considered useful in the electrodiagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP). Distal and P-D CMAP dispersion have not been fully studied in hereditary neuropathies, and it is not known whether these measures distinguish hereditary from acquired demyelination. We compared DCMAP duration and P-D CMAP dispersion in 91 genetically characterized hereditary neuropathies and 33 subjects with CIDP. DCMAP dispersion was more frequent in nerves affected by CIDP (41.5%) than in Charcot-Marie-Tooth disease (CMT)1A (24.4%), CMT1B (7.4%), hereditary neuropathy with liability to pressure palsies (HNPP) (10.5%), or CMTX (9.8%). P-D CMAP dispersion was more frequent in CIDP (27.7% of nerves) than in hereditary neuropathies (16.3%) when applying American Academy of Neurology (AAN) criteria; however, its frequency was similar in CIDP and the hereditary neuropathies using the more restrictive criteria of the American Association of Neuromuscular and Electrodiagnostic Medicine (AANEM). Although dispersion is more common in CIDP than in the hereditary neuropathies, DCMAP and P-D dispersion occur in at least one motor nerve in a significant proportion of hereditary neuropathies, and cannot be used in isolation to distinguish acquired from hereditary demyelination.

  18. Chronic Inflammatory Demyelinating Polyneuropathy in Children: A Review of Clinical Characteristics and Recommendations for Treatment

    Directory of Open Access Journals (Sweden)

    Narges Karimi

    2015-07-01

    Full Text Available Context: Chronic inflammatory demyelinating polyradiculopathy (CIDP is an acquired and autoimmune neuropathy, characterized by a chronic, rapidly progressive, symmetric weakness. In children, abnormal gait is as a first symptom of muscle weakness. Evidence Acquisition: The diagnosis of CIDP is on the basis of clinical characteristics, electrodiagnostic that shows the severity of the disease, lumbar puncture and spine magnetic resonance imaging (MRI. Results: The first-line treatments in childhood CIDP are intravenous immunoglobulin (IVIG, corticosteroids, and plasmapheresis. Response to first-line therapies is usually satisfactory; nevertheless, recommendations regarding the choice of second-line therapy can only be prepared on the basis of the existing practice described in some of the case reports. Conclusions: This review demonstrated the clinical presentation, diagnosis, and treatment of childhood CIDP.

  19. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition.

    Science.gov (United States)

    Vallat, Jean-Michel; Sommer, Claudia; Magy, Laurent

    2010-04-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic neuropathy of supposed immune origin. Understanding of its pathophysiology has recently improved, although its causes remain unclear. The classic presentation of CIDP includes sensory and motor symptoms in the distal and proximal segments of the four limbs with areflexia, evolving over more than 8 weeks. Raised protein concentrations in CSF and heterogeneous slowing of nerve conduction are typical of the condition. In addition to this usual phenotype, distribution of symptoms, disease course, and disability can be heterogeneous, leading to underdiagnosis of the disorder. Diagnosis is sometimes challenging and can require use of imaging and nerve biopsy. Steroids and intravenous immunoglobulin are effective, and plasma exchange can be helpful as rescue therapy. The usefulness of immunosuppressants needs to be established. The identification of specific diagnostic markers and new therapeutic strategies with conventional or targeted immunotherapy are needed to improve the outlook for patients with CIDP.

  20. Chronic inflammatory demyelinating polyneuropathy disease activity status: recommendations for clinical research standards and use in clinical practice

    NARCIS (Netherlands)

    K.C. Gorson; I.N. van Schaik; I.S.J. Merkies; R.A. Lewis; R.J. Barohn; C.L. Koski; D.R. Cornblath; R.A.C. Hughes; A.F. Hahn; M. Baumgarten; J. Goldstein; J. Katz; M. Graves; G. Parry; P.A. van Doorn

    2010-01-01

    Defining long-term outcomes in chronic inflammatory demyelinating polyneuropathy (CIDP) has been complicated by varying definitions of treatment response and differing scales measuring impairment or disability. An expert panel was convened to devise a CIDP Disease Activity Status (CDAS) and to class

  1. Chronic inflammatory demyelinating polyradiculoneuropathy in a boy with systemic lupus erythematosus.

    Science.gov (United States)

    Zoilo, Morel Ayala; Eduardo, Benadón; Enrique, Faugier; del Rocio, Maldonado V M

    2010-05-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired, autoimmune peripheral neuropathy. Systemic lupus erythematosus (SLE) is a multisystemic, autoimmune disease that can affect the central nervous system in about 40% of patients, with prevalence and incidence unknown in the pediatric population due to lack of multicenter studies. We report the case of a 13-year-old Mexican boy, diagnosed with CIDP at the onset of SLE, beginning with progressive muscle weakness of lower and upper limbs, without affection of the central nervous system. The patient had positive ANA, antiDNAdc, antiBeta2glycoprotein, anti-cardiolipin, ANCA-C and X. He received intravenous immunoglobulin, cyclophosphamide, steroids, and azathioprine and showed clinical improvement. It is important to take into account the presence of peripheral neurological disorders in patients with pediatric SLE, considering CIDP as an uncommon presentation, making the diagnosis important for better treatment and evolution.

  2. Treatment of Chronic Inflammatory Demyelinating Polyneuropathy: From Molecular Bases to Practical Considerations

    Directory of Open Access Journals (Sweden)

    Paolo Ripellino

    2014-01-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is an autoimmune disease of the peripheral nervous system, in which both cellular and humoral immune responses are involved. The disease is clinically heterogeneous with some patients displaying pure motor form and others also showing a variable degree of sensory dysfunction; disease evolution may also differ from patient to patient, since monophasic, progressive, and relapsing forms are reported. Underlying such clinical variability there is probably a broad spectrum of molecular dysfunctions that are and will be the target of therapeutic strategies. In this review we first explore the biological bases of current treatments and subsequently we focus on the practical management that must also take into account pharmacoeconomic issues.

  3. Nerve sonography in multifocal motor neuropathy and chronic inflammatory demyelinating polyneuropathy

    Directory of Open Access Journals (Sweden)

    D. S. Druzhinin

    2016-01-01

    Full Text Available The quantitative ultrasound characteristics (USC of the median, ulnar nerve at different levels and the spinal nerves in patients with multifocal motor neuropathy (MMN; n=13; 40,4 ± 12,6 years old and chronic inflammatory demyelinating polyneuropathy (CIDP; n = 7; 47,3 ± 11,2 year old did not reveal statistical difference in cross sectional area (CSA between analyzed groups. Patients with MMN have more pronounced asymmetry of CSA in comparison with CIDP patients which have a symmetrical pattern of diffuse nerve involvement. Quantitative USC has shown to be not informative enough in differentiation of MMN and CIDP. The qualitative analysis (QA according to 3 described types of nerve changes has shown that CIDP is characterized by the prevalence of type 3 pattern (85.8 % while MMN – by type 2 (69.2 %. The sensitivity and specificity of proposed QA patterns in nerve USC need to be analyzed in additional investigations. 

  4. Treatment of pediatric chronic inflammatory demyelinating polyneuropathy: Challenges, controversies, and questions

    Directory of Open Access Journals (Sweden)

    Jay Desai

    2015-01-01

    Full Text Available Pediatric chronic inflammatory demyelinating polyneuropathy (CIDP is an uncommon acquired disorder of unknown cause, presumed to have an immunological basis. We report 20 patients seen at Children′s Hospital Los Angeles over a period of 10 years. The outcome of our patients was favorable in a vast majority with good response to various treatments instituted. However, residual neurologic deficit was common. The choice of treatment modality was empirical and selected by the treating neurologist. Intravenous immunoglobulin (IVIG and corticosteroids were most commonly utilized for treatment. Plasmapheresis, mycophenolate mofetil, rituximab, cyclophosphamide, azathioprine, and abatacept were added if the patients were refractory to IVIG or became corticosteroid dependent. The spectrum of disease severity ranged from a single monophasic episode, to multiphasic with infrequent relapses with good response to IVIG, to progressive disease refractory to multiple therapies.

  5. Recurrent hypogeusia in a patient with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

    Science.gov (United States)

    Kawaguchi, Norihiko; Sugeno, Naoto; Endo, Kaoru; Miura, Emiko; Misu, Tatsuro; Nakashima, Ichiro; Itoyama, Yasuto

    2012-04-01

    Hypogeusia, a condition with diminished sense of taste, is caused by several conditions, including zinc deficiency and as a side-effect of drugs, but is not common in neurological disorders. A 55-year-old Japanese man with a 30-year history of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) presented with hypogeusia during hospitalization for a recurrence of CIDP. The hypogeusia improved after treatment with high-dose intravenous methylprednisolone (HIMP). Two years later, hypogeusia developed again. A complete taste deficit was revealed by a filter paper test. Brain MRI showed enhancement of the bilateral facial nerve ganglia. Hypogeusia was partially ameliorated after extensive immunosuppressive therapy with repeated HIMP and plasma exchange. Improvement was more prominent in the area innervated by the chorda tympani nerve than that innervated by the glossopharyngeal nerve. To our knowledge, this is the first report of recurrent hypogeusia, which might be caused by cranial nerve injury associated with CIDP.

  6. Does the chronic inflammatory demyelinating polyradiculoneuropathy due to secondary cause differ from primary?

    Directory of Open Access Journals (Sweden)

    Vaibhav Wadwekar

    2011-01-01

    Full Text Available Background: The clinical presentation, neurophysiological findings, and outcome may vary between primary and secondary chronic inflammatory demyelinating polyradiculopathy (CIDP. Objective: To compare clinical and electrodiagnostic features of primary and secondary CIDP. Setting: Tertiary care teaching referral hospital. Materials and Methods: The CIDP patients who were diagnosed as per European Federation of Neurological Societies/Peripheral Nerve Society criteria were included and subjected to detailed history and examinations. The clinical disability was graded on a 0-10 scale. Neurophysiology included motor and sensory nerve conductions and F wave studies of all four limbs. Based on investigations for underlying diseases, the patients were categorized into primary or secondary CIDP. Prednisolone was prescribed in all and azathioprine added in resistant cases. The secondary CIDP group received specific treatment in addition. The outcome was assessed at 3 months, 6 months, and last follow-up. Results: A total of 65 patients aged 17 to 72 years were included and 20 were females. Twenty-five patients had secondary CIDP and include diabetes mellitus (16, POEMS (polyneuropathy, organomegaly, endocrinopathy, M protein, and skin changes (4, monoclonal gammopathy of undetermined significance (2, myeloma (1, lymphoma (1, and malignancy (1. The secondary CIDP patients were older (48.35 vs 41.0 years, had less relapsing remitting (0 vs 6 and more frequent dysautonomia (7 vs 1. The demyelinating features were more marked in primary CIDP group and had better outcome compared with secondary CIDP. Conclusions: Of the total patients with CIDP, 38.5% of patients had secondary CIDP which was associated with progressive course, less demyelinating features, and worse prognosis.

  7. Alemtuzumab in the treatment of IVIG-dependent chronic inflammatory demyelinating polyneuropathy.

    LENUS (Irish Health Repository)

    Marsh, E A

    2010-06-01

    Chronic inflammatory demyelinating polyneuropathy (CIDP) is an idiopathic immune mediated neuropathy causing demyelination and conduction block thought to occur as the result of an aberrant autoimmune response resulting in peripheral nerve inflammation mediated by T cells and humoral factors. Diagnosis commonly prompts initial treatment with steroids or intravenous immunoglobulin (IVIG) on which 5-35% subsequently become dependent to maintain function. Despite a number of small scale trials, the role for alternative long-term immunosuppression remains unclear. Alemtuzumab is a humanised monoclonal antibody targeting the CD52 antigen present on the surface of lymphocytes and monocytes. A single intravenous infusion results in rapid and profound lymphopoenia lasting >12 months. We report its use and clinical outcome in a small series of patients with severe IVIG-dependent CIDP. Seven patients (4 Males; 3 Females) who had failed to respond to conventional immunosuppression were treated in 5 centres receiving 9 courses of alemtuzumab (dose range 60-150 mg). Following treatment, mean monthly IVIG use fell 26% from 202 to 149 g and IVIG administration frequency from 22 to 136 days. Two patients had prolonged remission, two patients had a partial response and no clear benefit was observed in the remaining three patients (2 Males, 1 Females). Responding patients had a younger age at onset (19.5 years) and shorter disease duration than non-responders. Three patients developed autoimmune disease following treatment. Alemtuzumab may offer an alternative treatment for a subset of early onset IVIG dependent CIDP patients failing conventional immunosuppressive agents, but concerns about toxicity may limit its use.

  8. [A case of chronic inflammatory demyelinating polyradiculoneuropathy concomitant with acquired von Willebrand syndrome].

    Science.gov (United States)

    Ueda, Maki; Kawamura, Nobutoshi; Tateishi, Takahisa; Shigeto, Hiroshi; Ohyagi, Yasumasa; Kira, Jun-ichi

    2011-05-01

    We report a case of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) concomitant with acquired von Willebrand syndrome. A 33-year-old man developed motor and sensory polyneuropathy with electrophysiological conduction slowing. At this time, M-protein was absent He was diagnosed with CIDP and received intravenous immunoglobulin and subsequent oral corticosteroids, which resulted in almost complete remission for over 10 years. At the age of 44, he presented with chronic anemia. Laboratory tests and colonoscopy revealed that he had acquired von Willebrand syndrome with monoclonal gammopathy of undetermined significance (IgG lambda type) and colon cancer. Bleeding symptoms were.resolved with intravenous immunoglobulin, but not with supplementation of factor VIII. Shortly after successful excision of the cancer, CIDP and acquired von Willebrand syndrome simultaneously recurred. Intravenous immunoglobulin produced rapid improvement of both neurological and hematological abnormalities. Concurring CIDP and acquired von Willebrand syndrome in the present case may indicate that the conditions have a partly common immunological background including monoclonal gammopathy and a potential common autoantibody-mediated mechanism. Alternatively, dysfunction of von Willebrand factor may increase blood-nerve barrier permeability, inducing the recurrence of CIDP.

  9. Childhood chronic inflammatory demyelinating polyradiculoneuropathy: combined analysis of a large cohort and eleven published series.

    Science.gov (United States)

    McMillan, Hugh J; Kang, Peter B; Jones, H Royden; Darras, Basil T

    2013-02-01

    The clinical presentation, disease course, response to treatment, and long-term outcome of thirty childhood chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients are presented representing the largest cohort reported to date. Most children (60%) presented with chronic (>8-weeks) symptom-onset while a smaller proportion showed sub-acute (4-8 weeks) or acute (''GBS-like''; CIDP series providing a comprehensive review of 143 childhood CIDP cases. The combined initial or first-line treatment response across all studies was favourable for IVIG (79% patients) and corticosteroids (84% patients). Response to first-line plasma exchange was poor (only 14% patients improved) although it may offer some transient or partial benefit as an adjuvant or temporary therapy for selected patients. The combined long-term outcome of our cohort and the literature reveals a favourable prognosis for most patients. The combined modified Rankin scale decreased from 3.7 (at presentation) to 0.7 (at last follow-up). This review provides important data pertaining to clinical course, treatment response and long-term outcome of this relatively uncommon paediatric autoimmune disease.

  10. What's new in chronic inflammatory demyelinating polyradiculoneuropathy in 2007-2008?

    Science.gov (United States)

    van Schaik, Ivo N

    2008-12-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP)-related research has made progress in the field of pathogenesis, genetics, and treatment. The number of circulating CD4(+) CD25(+) T-regulatory cells was shown to be reduced in CIDP patients. Increased frequency of genotype GA13-16 of the SH2D2A gene encoding for a T-cell-specific adapter protein in CIDP patients may result in a defective control and elimination of autoreactive T cells. IVIg treatment has been shown to increase numbers and function of peripheral CD4(+) CD25(+) T-regulatory cell in a mouse model. These findings shed new light on the understanding of why peripheral tolerance is breached in CIDP patients and why the disease becomes chronic and adds another possible mechanism of action of intravenous immunoglobulin to the already long list. Long-term effectiveness of IVIg has now been proven. Subcutaneous immunoglobulin could be an alternative for IVIg, but this has to be explored further in well-designed trials. Autologous stem cell transplantation has been tried in refractory patients, but larger trials are necessary to assess safety and effect of this treatment.

  11. Surgical treatment and intraoperative spinal cord monitoring in scoliosis associated with chronic inflammatory demyelinating polyneuropathy: A case report

    OpenAIRE

    Kudo, Daisuke; Miyakoshi, Naohisa; Hongo, Michio; Kasukawa, Yuji; Ishikawa, Yoshinori; MISAWA, Akiko; Shimada, Yoichi

    2013-01-01

    There has been only one reported case of neuromuscular scoliosis following chronic inflammatory demyelinating polyneuropathy (CIDP). However, no cases of scoliosis that were treated with surgery secondary to CIDP have been previously described. A 16-year-old boy with CIDP was consultant due to the progression of scoliosis with the coronal curve of 86° from T8 to T12. Posterior correction and fusion with segmental pedicle screws were performed under intraoperative spinal cord monitoring with t...

  12. Chronic inflammatory demyelinating polyneuropathy (CIDP): change of serum IgG dimer levels during treatment with intravenous immunoglobulins

    OpenAIRE

    Ritter, Christian; Bobylev, Ilja; Helmar C Lehmann

    2015-01-01

    Background Intravenous immunoglobulin (IVIg) is an effective treatment in chronic inflammatory demyelinating polyneuropathy (CIDP). In most patients, the optimal IVIg dose and regime is unknown. Polyvalent immunoglobulin (Ig) G form idiotypic/anti-idiotypic antibody pairs in serum and IVIg preparations. We determined IgG dimer levels before and after IVIg treatment in CIDP patients with the aim to explore their utility to serve as a surrogate marker for treatment response. Methods IgG was pur...

  13. Immunoadsorption in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with unsatisfactory response to first-line treatment

    OpenAIRE

    Galldiks, N; Burghaus, L.; Klingel, R; Benzing, T.; Fink, G R; Haupt, W. F.; Dohmen, C.; Teschner, S; Pollok, M.; Leebmann, J.; Frischmuth, N.; Hollinger, P.; Nazli, N.; Fassbender, C.

    2011-01-01

    First-line treatment options for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are corticosteroids, intravenous immunoglobulin, and plasma exchange. In a significant number of patients, first-line therapy fails, and long-term maintenance treatment still remains a therapeutic challenge. Immunoadsorption (IA) may be an alternative to classical plasma exchange in the therapy of immune-mediated neurologic diseases. The aim of this investigation was to evaluate efficacy and safe...

  14. Peripheral Nerve Ultrasonography in Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy: Correlations with Clinical and Neurophysiological Data

    OpenAIRE

    Aristide Merola; Michela Rosso; Alberto Romagnolo; Erdita Peci; Dario Cocito

    2016-01-01

    Objective. This cross-sectional study analyzes the pattern of ultrasound peripheral nerve alterations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) at different stages of functional disability. Material and Methods. 22 CIDP and 10 MMN patients and a group of 70 healthy controls were evaluated with an ultrasound scan of the median, ulnar, peroneal, tibial, and sural nerves. Results were correlated with clinical disabilit...

  15. The Characteristics of Chronic Inflammatory Demyelinating Polyneuropathy in Patients with and without Diabetes – An Observational Study

    OpenAIRE

    Dunnigan, Samantha K.; Hamid Ebadi; Ari Breiner; Katzberg, Hans D.; Carolina Barnett; Perkins, Bruce A.; Vera Bril

    2014-01-01

    INTRODUCTION: We aimed to determine whether the clinical characteristics and electrodiagnostic classification of nerve injury, and response to treatment differed in patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP) with and without diabetes. METHODS: CIDP patients with diabetes (CIDP+DM) (n = 67) and without diabetes (CIDP-DM) (n = 67) underwent clinical examination and nerve conduction studies (NCS). CIDP-DM patients were selected using age and gender matching ...

  16. Chronic inflammatory demyelinating polyneuropathy: quality of life, sociodemographic profile and physical complaints

    Directory of Open Access Journals (Sweden)

    Patricia Leila dos Santos

    2014-03-01

    Full Text Available Whereas an evaluation of quality of life and possible impacts on the mental state of a patient may help to evaluate the evolution of chronic inflammatory demyelinating polyneuropathy (CIDP, the aim of this study was to study the psychological profile of patients, and evaluate quality of life associated with the disease. Method 41 patients were evaluated using a Mini-Mental State Examination (MMSE and a Short-Form Health Survey (SF-36. Results The mean age of the patients was 50.6 years, 63.4% men. Of the participants, 65.9% had other health problems, 39% reported needing help with activities of daily living, 49% slept less than 8 hours per night, and 34.1% complained of some memory deficit. The average MMSE score was 26. Impairment of functional capacity and pain were the more important altered health states. Conclusion CIDP has important social and economic impacts, owing to functional impairments that can lead to professional and personal limitations.

  17. Long-Lasting Cranial Nerve III Palsy as a Presenting Feature of Chronic Inflammatory Demyelinating Polyneuropathy

    Directory of Open Access Journals (Sweden)

    Rossella Spataro

    2015-01-01

    Full Text Available We describe a patient with chronic inflammatory demyelinating polyneuropathy (CIDP in which an adduction deficit and ptosis in the left eye presented several years before the polyneuropathy. A 52-year-old man presented with a 14-year history of unremitting diplopia, adduction deficit, and ptosis in the left eye. At the age of 45 a mild bilateral foot drop and impaired sensation in the four limbs appeared, with these symptoms showing a progressive course. The diagnostic workup included EMG/ENG which demonstrated reduced conduction velocity with bilateral and symmetrical sensory and motor involvement. Cerebrospinal fluid studies revealed a cytoalbuminologic dissociation. A prolonged treatment with corticosteroids allowed a significant improvement of the limb weakness. Diplopia and ptosis remained unchanged. This unusual form of CIDP presented as a long-lasting isolated cranial nerve palsy. A diagnostic workup for CIDP should therefore be performed in those patients in which an isolated and unremitting cranial nerve palsy cannot be explained by common causes.

  18. Diffusion tensor imaging of peripheral nerve in patients with chronic inflammatory demyelinating polyradiculoneuropathy: a feasibility study

    Energy Technology Data Exchange (ETDEWEB)

    Kakuda, Takako; Fukuda, Hiroshi; Tanitame, Keizo; Takasu, Miyuki; Date, Shuji; Awai, Kazuo [Hiroshima University, Department of Diagnostic Radiology, Graduate School of Biomedical Sciences, Hiroshima (Japan); Ochi, Kazuhide; Ohshita, Tomohiko; Matsumoto, Masayasu [Hiroshima University, Department of Clinical Neuroscience and Therapeutics, Graduate School of Biomedical Science, Hiroshima (Japan); Kohriyama, Tatsuo [Department of Neurology, Hiroshima City Hospital, Hiroshima (Japan); Ito, Katsuhide [Department of Radiology, Onomichi General Hospital, Onomichi, Hiroshima-ken (Japan)

    2011-12-15

    The purpose of this study was to assess the clinical feasibility of diffusion tensor imaging (DTI) for the evaluation of peripheral nerves in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Using a 3-T magnetic resonance imaging scanner, we obtained DTI scans of the tibial nerves of 10 CIDP patients and 10 sex- and age-matched healthy volunteers. We prepared fractional anisotropy (FA) maps, measured the FA values of tibial nerves, and compared these values in the two study groups. In nine patients, we also performed tibial nerve conduction studies and analyzed the correlation between the FA values and parameters of the nerve conduction study. The tibial nerve FA values in CIDP patients (median 0.401, range 0.312-0.510) were significantly lower than those in healthy volunteers (median 0.530, range 0.469-0.647) (Mann-Whitney test, p < 0.01). They were significantly correlated with the amplitude of action potential (Spearman correlation coefficient, p = 0.04, r = 0.86) but not with nerve conduction velocity (p = 0.79, r = 0.11). Our preliminary data suggest that the noninvasive DTI assessment of peripheral nerves may provide useful information in patients with CIDP. (orig.)

  19. Variations of the perforin gene in patients with chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Buttini, S; Cappellano, G; Ripellino, P; Briani, C; Cocito, D; Osio, M; Cantello, R; Dianzani, U; Comi, C

    2015-01-01

    Perforin (PRF) has a key role in the function of cytotoxic T and natural killer cells. Rare variations of PRF1 predispose to autoimmunity. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune disease of the peripheral nervous system, involving defective lymphocyte apoptosis. The aim of this study was to investigate the role of PRF1 in CIDP. The entire coding region of PRF1 was sequenced in 94 patients and 158 controls. We found three missense variations leading to amino acid substitutions and one nonsense variation resulting in a premature stop codon. All variations would decrease PRF activity. Their overall frequency was significantly higher in patients than in controls (odds ratio (OR)=4.47). The most frequent variation was p.Ala91Val (OR=3.92) previously associated with other autoimmune diseases. Clinical analysis showed that PRF1 variations were more frequent in relapsing patients and in patients displaying axonal damage. These data suggest that PRF1 variations may influence CIDP development and course.

  20. Spinal cord involvement in chronic inflammatory demyelinating polyradiculoneuropathy: a clinical and MRI study.

    Science.gov (United States)

    Ioannidis, Panagiotis; Parissis, Dimitris; Karapanayiotides, Theodoros; Maiovis, Pantelis; Karacostas, Dimitris; Grigoriadis, Nikolaos

    2015-06-01

    Concomitant central nervous system (CNS) involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is rare. Although the spinal nerve roots may present MRI abnormalities in CIDP, hitherto, the spinal cord has been investigated in a single study. We retrospectively investigated clinically and with MRI a cohort of patients with definite CIDP diagnosis (EFNS/PNS criteria) for evidence of brain and spinal cord involvement, who were initially admitted in our department during the last 4 years. Among 12 patients with CIDP (men: 8, mean age: 59.3 years, mean disease duration: 3.8 years), nine patients had their MRI scan during a clinical relapse and three during remission. Brain MRI did not document typical multiple sclerosis lesions in any patient. We did not identify any MRI abnormalities in ten patients without clinical evidence of spinal cord involvement. Conversely, MRI disclosed extensive lesions of the thoracic cord in two patients with an overt spinal cord syndrome, whom we describe. This represents the biggest MRI study of CIDP patients who have been investigated for spinal cord involvement. Our data support earlier observations that a minority of CIDP patients may additionally develop CNS involvement of variable degree.

  1. Circulating subsets and CD4(+)CD25(+) regulatory T cell function in chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Sanvito, Lara; Makowska, Anna; Gregson, Norman; Nemni, Raffaello; Hughes, Richard A C

    2009-01-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an inflammatory disease of the peripheral nervous system that is probably autoimmune in origin. Different components of the adaptive and innate immunity may be responsible for the aberrant response towards nerve antigens. To investigate this, we examined lymphocyte subsets and regulatory T cell (Treg) function in the blood of CIDP patients, healthy controls (HC) and subjects with non-immune mediated neuropathies (other neuropathies, ON). We used flow cytometry to determine the frequency of monocytes, B cells, natural killer (NK) and NK-T cells, total and activated CD4(+) and CD8(+) T cells, effector memory and central memory CD4(+) and CD8(+) T cells, and CD4(+)CD25(high)Foxp3(+) Tregs. Treg function was studied after polyclonal stimulation and antigen specific stimulation with myelin protein peptides in CIDP and HC. There was an increased frequency of monocytes (p = 0.02) and decreased frequency of NK cells (p = 0.02) in CIDP compared with HC but not ON. There were no significant differences in other populations. Treg function was impaired in CIDP compared to HC (p = 0.02), whilst T cell proliferation to myelin protein peptides before and after depletion of Tregs was not different between patients and controls. This study shows increased circulating monocytes and reduced NK cells in CIDP. Although Treg frequency was not altered, we confirm that Tregs display a defect of suppressive function. Myelin protein peptides were not the target of the altered peripheral regulation of the immune response. The mechanisms of peripheral immune tolerance in CIDP and their relevance to the pathogenesis deserve further exploration.

  2. Chronic Inflammatory Demyelinating Polyneuropathy With Diabetes Mellitus Is Responsive To Intravenous Immune Globulin; Case Report

    OpenAIRE

    Koca, Süleyman Serdar; YOLDAŞ, Tahir K.; ÖZKAN, Yusuf; GÜNAY, İzzettin; DÖNDER, Emir

    2006-01-01

    Chronic demyelinating polyneuropathy (CIDP) is a disease which has different treatment modality like immunomodulatory method and have good response to treatment than the other peripheral neuropathy. We have established a patient with CIDP female 68 years old and had a type 2 diabetes mellitus diagnosis for 16 years. She treated with intravenous immunoglobuline (0.5 mg/kg/day) for five days and four weeks intervals at six months. This case has showed that the autoimmune neuropathy should keep ...

  3. Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: evidence base and clinical practice.

    Science.gov (United States)

    Press, R; Hiew, F L; Rajabally, Y A

    2016-04-01

    Evidence-based therapies for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consist of corticosteroids, intravenous immunglobulins (IVIg), and plasma exchange. Steroids represent the oldest treatment used historically. In countries where readily available and affordable, IVIg tends to be favored as first-line treatment. The reason for this preference, despite substantially higher costs, is the perception that IVIg is more efficacious and safer than corticosteroids. However, the unselected use of IVIg as a first-line treatment option in all cases of CIDP raises issues of cost-effectiveness in the long-term. Furthermore, serious although rare, particularly thromboembolic side effects may result from their use. Recent data from randomized trials suggest pulsed corticosteroids to have a higher potential in achieving therapy-free remission or longer remission-free periods compared with IVIg, as well as relatively low rates of serious side effects when given as pulsed intravenous infusions during short periods of time. These specific advantages suggest that pulsed steroids could in many cases be used, as the first, rather than second choice of treatment when initiating immunomodulation in CIDP, primarily in hopes of achieving a remission after the short-term use. This article reviews the evidence base for the use of corticosteroids in its various forms in CIDP and factors that may influence clinicians' choice between IVIg and pulsed steroid treatment. The issue of efficacy, relapse rate and time, and side effect profile are analyzed, and some aspects from the authors' experience are discussed in relation to the possibility of using the steroid option as first-line therapy in a large proportion of patients with CIDP. PMID:26437234

  4. Steroids for chronic inflammatory demyelinating polyradiculoneuropathy: evidence base and clinical practice.

    Science.gov (United States)

    Press, R; Hiew, F L; Rajabally, Y A

    2016-04-01

    Evidence-based therapies for chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) consist of corticosteroids, intravenous immunglobulins (IVIg), and plasma exchange. Steroids represent the oldest treatment used historically. In countries where readily available and affordable, IVIg tends to be favored as first-line treatment. The reason for this preference, despite substantially higher costs, is the perception that IVIg is more efficacious and safer than corticosteroids. However, the unselected use of IVIg as a first-line treatment option in all cases of CIDP raises issues of cost-effectiveness in the long-term. Furthermore, serious although rare, particularly thromboembolic side effects may result from their use. Recent data from randomized trials suggest pulsed corticosteroids to have a higher potential in achieving therapy-free remission or longer remission-free periods compared with IVIg, as well as relatively low rates of serious side effects when given as pulsed intravenous infusions during short periods of time. These specific advantages suggest that pulsed steroids could in many cases be used, as the first, rather than second choice of treatment when initiating immunomodulation in CIDP, primarily in hopes of achieving a remission after the short-term use. This article reviews the evidence base for the use of corticosteroids in its various forms in CIDP and factors that may influence clinicians' choice between IVIg and pulsed steroid treatment. The issue of efficacy, relapse rate and time, and side effect profile are analyzed, and some aspects from the authors' experience are discussed in relation to the possibility of using the steroid option as first-line therapy in a large proportion of patients with CIDP.

  5. Brachial and lumbar plexuses in chronic inflammatory demyelinating polyradiculoneuropathy: MRI assessment including apparent diffusion coefficient

    Energy Technology Data Exchange (ETDEWEB)

    Adachi, Yuko; Sato, Noriko; Yamashita, Fumio; Kida, Jiro; Takahashi, Tomoyuki [National Center Hospital of Neurology and Psychiatry, Department of Radiology, Kodaira, Tokyo (Japan); Okamoto, Tomoko [National Center Hospital of Neurology and Psychiatry, Department of Neurology, Kodaira, Tokyo (Japan); Sasaki, Masayuki; Komaki, Hirofumi [National Center Hospital of Neurology and Psychiatry, Department of Child Neurology, Kodaira, Tokyo (Japan); Matsuda, Hiroshi [Saitama Medial University Hospital, Department of Nuclear Medicine, Iruma-gun, Saitama (Japan)

    2011-01-15

    Our purpose was to clarify the magnetic resonance (MR) imaging characteristics of the brachial and lumbar plexuses in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) using various kinds of sequences, including diffusion-weighted images (DWI). We evaluated the MR imaging findings for lumbar and/or brachial nerve plexuses in 13 CIDP patients and 11 normal volunteers. The nerve swelling was evaluated in comparison with normal controls by coronal short tau inversion recovery (STIR), and signal abnormalities were evaluated by coronal STIR, T1-weighted images, and DWIs. The degrees of contrast enhancement and apparent diffusion coefficient (ADC) values of the plexus were also assessed. In the patient group, diffuse enlargement and abnormally high signals were detected in 16 out of 24 plexuses (66.7%) on STIR, a slightly high signal was detected in 12 of 24 plexuses (50%) on T1-weighted images, and a high-intensity signal was detected in 10 of 18 plexuses (55.6%) on DWIs with high ADC values. Contrast enhancement of the plexuses was revealed in 6 of 19 plexuses (31.6%) and was mild in all cases. There were statistically significant differences between the ADC values of patients with either swelling or abnormal signals and those of both normal volunteers and patients without neither swelling nor abnormal signals. There were no relationships between MR imaging and any clinical findings. STIR is sufficient to assist clinicians in diagnosing CIDP. T1-weighted images and DWIs seemed useful for speculating about the pathological changes in swollen plexuses in CIDP patients. (orig.)

  6. Severity and patterns of blood-nerve barrier breakdown in patients with chronic inflammatory demyelinating polyradiculoneuropathy: correlations with clinical subtypes.

    Directory of Open Access Journals (Sweden)

    Fumitaka Shimizu

    Full Text Available OBJECTIVE: Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is currently classified into clinical subtypes, including typical and atypical forms (multifocal acquired demyelinating sensory and motor neuropathy (MADSAM and distal acquired demyelinating symmetric neuropathy (DADS. The aim of this study was to elucidate the patterns and severity of breakdown of the blood-nerve barrier (BNB in each CIDP subtype. METHODS: We evaluated the effects of sera obtained from patients with typical CIDP, MADSAM and DADS and control subjects on the expression levels of tight junction proteins and transendothelial electrical resistance (TEER value in human peripheral nerve microvascular endothelial cells (PnMECs. RESULTS: The sera obtained from the patients with the three clinical phenotypes of CIDP decreased the amount of claudin-5 protein levels and TEER values in the PnMECs. In addition, the sera obtained from typical CIDP patients more prominently reduced claudin-5 protein levels and TEER values in the PnMECs than did that obtained from the MADSAM and DADS patients. Furthermore, the severity of BNB disruption after exposure to the sera was associated with higher Hughes grade, lower MRC score, more pronounced slowing of motor nerve conduction in the median nerve and higher frequency of abnormal temporal dispersion. CONCLUSIONS: Sera derived from typical CIDP patients destroy the BNB more severely than those from MADSAM or DADS patients. The extent of BNB disruption in the setting of CIDP is associated with clinical disability and demyelination in the nerve trunk. These observations may explain the phenotypical differences between CIDP subtypes.

  7. Peripheral Nerve Ultrasonography in Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy: Correlations with Clinical and Neurophysiological Data

    Directory of Open Access Journals (Sweden)

    Aristide Merola

    2016-01-01

    Full Text Available Objective. This cross-sectional study analyzes the pattern of ultrasound peripheral nerve alterations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP and multifocal motor neuropathy (MMN at different stages of functional disability. Material and Methods. 22 CIDP and 10 MMN patients and a group of 70 healthy controls were evaluated with an ultrasound scan of the median, ulnar, peroneal, tibial, and sural nerves. Results were correlated with clinical disability scales and nerve conduction studies. Results. Patients with intermediate functional impairment showed relatively larger cross-sectional areas than subjects with either a milder (p<0.05 or more severe impairment (p<0.05, both in CIDP and in MMN. In addition, MMN was associated with greater side-to-side intranerve variability (p<0.05, while higher cross-sectional areas were observed in CIDP (p<0.05 and in nerve segments with predominantly demyelinating features (p<0.05. Higher CSA values were observed in nerves with demyelinating features versus axonal damage (p<0.05 for CIDP; p<0.05 for MMN. Discussion and Conclusions. Greater extent of quantitative and qualitative US alterations was observed in patients at intermediate versus higher functional disability and in nerves with demyelinating versus axonal damage. CIDP and MMN showed differential US aspects, with greater side-to-side intranerve variability in MMN and higher cross-sectional areas in CIDP.

  8. Distribution of Th17 cells and Th1 cells in peripheral blood and cerebrospinal fluid in chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Chi, Li Jun; Xu, Wan Hai; Zhang, Zong Wen; Huang, Hui Tao; Zhang, Li Ming; Zhou, Jin

    2010-12-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated demyelinating disease of the peripheral nervous system. Th17 and Th1 cells contribute to the pathogenesis of most autoimmune diseases, but little is known about their distribution and reciprocal relationship in CIDP. In this study, we analyzed the distribution of Th17, Th1, and Th17/Th1 cells in the peripheral blood and cerebrospinal fluid (CSF). The results showed that the frequency of Th17 cells was significantly higher in the peripheral blood mononuclear cell (PBMCs) and CSF of active CIDP in comparison with remitting CIDP or to other non-inflammatory neurological diseases (ONDs), accompanied by similar findings for Th17/Th1 cells. Both active and remitting CIDP have higher percentage of Th1 cells in the CSF than OND. CSF protein levels positively correlated with the frequencies of Th17 cells either in the PBMCs or CSF of active CIDP, while there was no significant correlation with Th1 cells. In line with these observations, the levels of interleukin-17 (IL-17) in plasma and transcript factors retinoic acid receptor-related orphan receptor (ROR)γt expressed by PBMCs were significantly higher in the active CIDP than remitting CIDP or OND. In summary, our preliminary findings suggest that elevated numbers of inflammatory T cells, especially for Th17 cells, might be an important determinant in the evolution of CIDP.

  9. Acute inflammatory demyelinating polyneuropathy associated with pegylated interferon 2a therapy for chronic hepatitis C virus infection

    Institute of Scientific and Technical Information of China (English)

    Vijay Khiani; Thomas Kelly; Adeel Shibli; Donald Jensen; Smruti R Mohanty

    2008-01-01

    The combination of pogylated interferon (Peg-IFN) and ribavirin is the standard of care for chronic hepatitis C virus (HCV) infection treatment. In general, common side effects related to this combination therapy are mild and are very well tolerated. However, peripheral neuropathy including demyelinating polyneuropathy related to Peg-IFN is extremely rare. We present the first case of an acute inflammatory demyelinating polyneuropathy (AIDP)associated with Peg-IFN-α 2a (Pegasys) after 16 wk of a combination therapy with Pegasys and ribavirin in a 65-year-old woman with chronic HCV infection.She developed tingling, numbness, and weakness of her upper and lower extremities and was hospitalized for acute neurological deficits. Her clinical course,neurological findings, an electromyogram (EHG), nerve conductions studies (NCS), muscle biopsy, and a sural nerve biopsy were all consistent with AIDP likely related to Pegasys use. The patient recovered completely with the use of intravenous immunoglobulin (IVIG) including physical therapy and neurological rehabilitation. It is very important that gastroenterologists and/or hepatologists recognize this rare neurological complication related to Peg-IFN treatment very early, since it requires a prompt discontinuation of therapy including an immediate referral to a neurologist for the confirmation of diagnosis, management, and the prevention of long-term neurological deficits.

  10. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial

    NARCIS (Netherlands)

    R.A.C. Hughes (Richard); P. Donofrio (Peter); V. Bril (Vera); M.C. Dalakas (Marinos); C. Deng (Chunqin); K. Hanna (Kim); H.P. Hartung; N. Latov (Norman); I.S.J. Merkies (Ingemar); P.A. van Doorn (Pieter)

    2008-01-01

    textabstractBackground: Short-term studies suggest that intravenous immunoglobulin might reduce disability caused by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but long-term effects have not been shown. We aimed to establish whether 10% caprylate-chromatography purified immune

  11. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial.

    NARCIS (Netherlands)

    Schaik, I.N. van; Eftimov, F.; Doorn, P.A. van; Brusse, E.; Berg, L.H. van den; Pol, W.L. van der; Faber, C.G.; Oostrom, J.C. van; Vogels, O.J.M.; Hadden, R.D.; Kleine, B.U.; Norden, A.G.W. van; Verschuuren, J.J.; Dijkgraaf, M.G.; Vermeulen, M.

    2010-01-01

    BACKGROUND: Pulsed high-dose dexamethasone induced long-lasting remission in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a pilot study. The PREDICT study aimed to compare remission rates in patients with CIDP treated with high-dose dexamethasone with rates in pa

  12. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study) : a double-blind, randomised, controlled trial

    NARCIS (Netherlands)

    van Schaik, Ivo N.; Eftimov, Filip; van Doorn, Pieter A.; Brusse, Esther; van den Berg, Leonard H.; van der Pol, W. Ludo; Faber, Catharina G.; van Oostrom, Joost C. H.; Vogels, Oscar J. M.; Hadden, Rob D. M.; Kleine, Bert U.; van Norden, Anouk G. W.; Verschuuren, Jan J. G. M.; Dijkgraaf, Marcel G. W.; Vermeulen, Marinus

    2010-01-01

    Background Pulsed high-dose dexamethasone induced long-lasting remission in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a pilot study. The PREDICT study aimed to compare remission rates in patients with CIDP treated with high-dose dexamethasone with rates in pat

  13. Pulsed high-dose dexamethasone versus standard prednisolone treatment for chronic inflammatory demyelinating polyradiculoneuropathy (PREDICT study): a double-blind, randomised, controlled trial

    NARCIS (Netherlands)

    I.N. van Schaik; F. Eftimov; P.A. van Doorn; E. Brusse; L.H. van den Berg; W.L. van der Pol; C.G. Faber; J.C. van Oostrom; O.J. Vogels; R.D. Hadden; B.U. Kleine; A.G. van Norden; J.J. Verschuuren; M.G. Dijkgraaf; M. Vermeulen

    2010-01-01

    Background Pulsed high-dose dexamethasone induced long-lasting remission in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) in a pilot study. The PREDICT study aimed to compare remission rates in patients with CIDP treated with high-dose dexamethasone with rates in pat

  14. Peripheral Nerve Ultrasonography in Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy: Correlations with Clinical and Neurophysiological Data.

    Science.gov (United States)

    Merola, Aristide; Rosso, Michela; Romagnolo, Alberto; Peci, Erdita; Cocito, Dario

    2016-01-01

    Objective. This cross-sectional study analyzes the pattern of ultrasound peripheral nerve alterations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) at different stages of functional disability. Material and Methods. 22 CIDP and 10 MMN patients and a group of 70 healthy controls were evaluated with an ultrasound scan of the median, ulnar, peroneal, tibial, and sural nerves. Results were correlated with clinical disability scales and nerve conduction studies. Results. Patients with intermediate functional impairment showed relatively larger cross-sectional areas than subjects with either a milder (p CIDP and in MMN. In addition, MMN was associated with greater side-to-side intranerve variability (p CIDP (p CIDP; p CIDP and MMN showed differential US aspects, with greater side-to-side intranerve variability in MMN and higher cross-sectional areas in CIDP. PMID:27313890

  15. Intravenous immunoglobulin treatment in chronic inflammatory demyelinating polyradiculoneuropathy, a time to start and a time to stop.

    Science.gov (United States)

    Adrichem, Max E; Eftimov, Filip; van Schaik, Ivo N

    2016-09-01

    Intravenous immunoglobulin (IVIg) is often used as preferred treatment in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Several studies highlighted the short-term efficacy of IVIg for CIDP yet many patients need maintenance therapy. Notwithstanding the fact IVIg has been used for over 30 years in CIDP, there is only limited evidence to guide dosage and interval during maintenance treatment. The variation in disease course, lack of biomarkers, and fear of deterioration after stopping IVIg makes long-term treatment challenging. Recent studies suggest a proportion of patients receive unnecessary IVIg maintenance treatment. This review provides an overview of the use of IVIg for CIDP treatment, focusing on evidence for long-term IVIg use.

  16. Peripheral Nerve Ultrasonography in Chronic Inflammatory Demyelinating Polyradiculoneuropathy and Multifocal Motor Neuropathy: Correlations with Clinical and Neurophysiological Data.

    Science.gov (United States)

    Merola, Aristide; Rosso, Michela; Romagnolo, Alberto; Peci, Erdita; Cocito, Dario

    2016-01-01

    Objective. This cross-sectional study analyzes the pattern of ultrasound peripheral nerve alterations in patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) at different stages of functional disability. Material and Methods. 22 CIDP and 10 MMN patients and a group of 70 healthy controls were evaluated with an ultrasound scan of the median, ulnar, peroneal, tibial, and sural nerves. Results were correlated with clinical disability scales and nerve conduction studies. Results. Patients with intermediate functional impairment showed relatively larger cross-sectional areas than subjects with either a milder (p CIDP and in MMN. In addition, MMN was associated with greater side-to-side intranerve variability (p CIDP (p CIDP; p CIDP and MMN showed differential US aspects, with greater side-to-side intranerve variability in MMN and higher cross-sectional areas in CIDP.

  17. Genetics of Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP): current knowledge and future directions.

    Science.gov (United States)

    Blum, Stefan; McCombe, Pamela A

    2014-06-01

    Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are thought to be autoimmune diseases. There have been many attempts to find a human leukocyte antigen (HLA) association with GBS and CIDP with little success. There have been studies of other plausible genes in GBS and CIDP and the role of these genes in GBS and CIDP and the data from these genetic studies is reviewed. Some of the genes that have been studied are immune related and some others have nervous system effects. The studies are limited by small numbers. Some of the genes show association with disease severity rather than disease susceptibility. The need for more detailed molecular studies of the role of HLA molecules and the need for modern genetic approaches to GBS and CIDP are explained.

  18. Subcutaneous versus intravenous immunoglobulin in drug-naïve patients with chronic inflammatory demyelinating polyneuropathy (CIDP)

    DEFF Research Database (Denmark)

    Markvardsen, L H; Sindrup, S H; Christiansen, I;

    2016-01-01

    BACKGROUND AND PURPOSE: Subcutaneous immunoglobulin (SCIG) is superior to placebo treatment for maintenance of muscle strength during 12 weeks in patients with chronic inflammatory demyelinating polyneuropathy (CIDP). The present study evaluated whether SCIG preserves muscle strength for 1 year...... in an open-label follow-up study. METHODS: Seventeen responders to intravenous immunoglobulin (IVIG) who had participated in the previous study of SCIG versus placebo in CIDP were included. After one IVIG infusion 2 weeks prior to baseline, all continued on SCIG treatment at weekly equal dosage and were...... remained unchanged. CONCLUSION: SCIG preserves muscle strength and functional ability in patients with CIDP who previously responded to IVIG. SCIG should be considered as an alternative in long-term treatment of CIDP patients....

  19. Intravenous immune globulin (10% caprylate-chromatography purified) for the treatment of chronic inflammatory demyelinating polyradiculoneuropathy (ICE study): a randomised placebo-controlled trial

    OpenAIRE

    Hughes, Richard; Donofrio, Peter; Bril, Vera; Dalakas, Marinos; Deng, Chunqin; Hanna, Kim; Hartung, H. P.; Latov, Norman; Merkies, Ingemar; Doorn, Pieter

    2008-01-01

    textabstractBackground: Short-term studies suggest that intravenous immunoglobulin might reduce disability caused by chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) but long-term effects have not been shown. We aimed to establish whether 10% caprylate-chromatography purified immune globulin intravenous (IGIV-C) has short-term and long-term benefit in patients with CIDP. Methods: 117 patients with CIDP who met specific neurophysiological inflammatory neuropathy cause and treat...

  20. Disease-modifying therapy in multiple sclerosis and chronic inflammatory demyelinating polyradiculoneuropathy: common and divergent current and future strategies.

    Science.gov (United States)

    Melzer, N; Meuth, S G

    2014-03-01

    Multiple sclerosis (MS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) represent chronic, autoimmune demyelinating disorders of the central and peripheral nervous system. Although both disorders share some fundamental pathogenic elements, treatments do not provide uniform effects across both disorders. We aim at providing an overview of current and future disease-modifying strategies in these disorders to demonstrate communalities and distinctions. Intravenous immunoglobulins (IVIG) have demonstrated short- and long-term beneficial effects in CIDP but are not effective in MS. Dimethyl fumarate (BG-12), teriflunomide and laquinimod are orally administered immunomodulatory drugs that are already approved or likely to be approved in the near future for the basic therapy of patients with relapsing-remitting MS (RRMS) due to positive results in Phase III clinical trials. However, clinical trials with these drugs in CIDP have not (yet) been initiated. Natalizumab and fingolimod are approved for the treatment of RRMS, and trials to evaluate their safety and efficacy in CIDP are now planned. Alemtuzumab, ocrelizumab and daclizumab respresent monoclonal antibodies in advanced stages of clinical development for their use in RRMS patients. Attempts to study the safety and efficacy of alemtuzumab and B cell-depleting anti-CD20 antibodies, i.e. rituximab, ocrelizumab or ofatumumab, in CIDP patients are currently under way. We provide an overview of the mechanism of action and clinical data available on disease-modifying immunotherapy options for MS and CIDP. Enhanced understanding of the relative effects of therapies in these two disorders may aid rational treatment selection and the development of innovative treatment approaches in the future.

  1. A diagnosis challenge-L4 nerve root compression as the initial presentation of chronic inflammatory demyelinating polyneuropathy.

    Science.gov (United States)

    Cojocaru, Inimioara Mihaela; Alexianu, Marilena; Bastian, Alexandra; Sapira, Violeta; Herţea, Cristina; Cojocaru, M

    2012-01-01

    The authors present the case of a 65-year-old woman who was admitted for paraparesis and paresthesias in the inferior limbs. The neurological examination revealed the difficulty in extension of the right foot and of the right toe, accompanied by paresthesias located in the anterolateral area of the right leg, dorsum and plantar area of the foot, the reduction of the right knee jerk, and of the ankle tendon jerk both sides. The vertebro-spinal MRI showed lumbar canal stenosis with L4 intraforaminal compression on the right, and L2-L3 on the left. CSF examination revealed mild increase in protein concentration. The morphological picture of the sural nerve biopsy was compatible with a chronic inflammatory neuropathy and severe muscular lesions of neurogenic origin were observed on right gastrocnemius muscle biopsy. The diagnosis of chronic inflammatory demyelinating polyneuropathy (CIDP) was established. Solu-medrol (0.5 g/d)-5 days, then medrol (prednisolone) was done, followed by improving of the symptomatology. For the relapse of the disease intravenous immunoglobulins (IVIG)-0.4 g/kg/d-5 days was the elective treatment. Six months later she presented a new relapse. IVIG were administered with the remission of the sensitive symptoms. A chronic treatment with medrol was recommended. The diagnosis of L4 disc herniation was obvious in the studied case, but the electroneurographic examination brought extra data for the associated diagnosis of CIDP whose onset was asymmetrical and initially paucisymptomatic. Neither the electroneurographic examination nor the CSF examination were total relevant for CIDP, imposing the sural nerve biopsy. The diagnosis of CIDP involves a team-work composed of neurologist, electroneurophysiologist and neuropathologist. PMID:23610977

  2. Acute inflammatory demyelinating polyneuropathy after treatment with pegylated interferon alfa-2a in a patient with chronic hepatitis C virus infection: a case report

    Directory of Open Access Journals (Sweden)

    Lahbabi Mounia

    2012-09-01

    Full Text Available Abstract Introduction The combination of polyethylene glycol (PEGylated interferon (pegylated interferon and ribavirin has been shown to be an effective treatment for chronic hepatitis C virus. In general, common side effects related to this combination therapy are mild and are well tolerated. However, peripheral neuropathy including demyelinating polyneuropathy related to PEG-interferon α2a (pegylated interferon alfa-2a is extremely rare. In the literature, only one case of acute inflammatory demyelinating polyneuropathy related to PEG-interferon α2a has been published previously. Case presentation To the best of our knowledge we present only the second case of acute inflammatory demyelinating polyneuropathy related to PEG-interferon α2a, occurring in a 63-year-old Caucasian man. He developed tingling, numbness, and weakness of his upper and lower extremities with acute neurological deficits after five weeks of a combination therapy with PEG-interferon α2a and ribavirin for chronic hepatitis C virus infection. His clinical course, neurological findings, and his electromyogram results were all consistent with acute inflammatory demyelinating polyneuropathy. Our patient recovered completely after interferon was stopped and symptomatic treatment and a further electromyogram showed a disappearance of neuropathy. Four weeks later, PEG-interferon α2a was reintroduced with a gradually increasing dose without any reappearance of neurological symptoms allowing hepatitis C seroconversion. Conclusions Recognition of this rare yet possible presentation is important for early and accurate diagnosis and treatment. This case report also suggests that the reintroduction of PEGylated interferon in patients who had presented with acute inflammatory demyelinating polyneuropathy related to interferon α may be safe, but this must be confirmed by further studies.

  3. A randomised, double-blinded, placebo-controlled trial of the effect of subcuta-neous immunoglobulin on muscular performance in chronic inflammatory de-myelinating polyneuropathy

    DEFF Research Database (Denmark)

    Harbo, Thomas; Markvardsen, Lars Høj; Sindrup, Søren Hein;

    Objectives: Subcutaneous treatment with large amounts of immunoglobulins is feasible and effective in multifocal motor neuropathy and has been reported in a few cases in chronic inflammatory demyelinating polyneuropathy (CIDP). We hypothesized that the effect of subcutaneous treatment with immuno......Objectives: Subcutaneous treatment with large amounts of immunoglobulins is feasible and effective in multifocal motor neuropathy and has been reported in a few cases in chronic inflammatory demyelinating polyneuropathy (CIDP). We hypothesized that the effect of subcutaneous treatment...... with immunoglobulins (SCIG) on muscular performance is superior to placebo and equals the effect of intravenous infusion (IVIG). Methods: Subjects with motor involvement in maintenance therapy with IVIG fulfilling the EFNS/PNS criteria for CIDP, aged 18 - 80 years were considered for participation. Exclusion criteria...

  4. A randomized, double-blind, placebo-controlled trial of the effect of subcutaneous immunoglobulin on muscular performance in chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Markvardsen, Lars Høj; Harbo, Thomas; Sindrup, Søren Hein;

    We hypothesized that subcutaneous administration of immunoglobulins (SCIG) in chronic inflammatory demyelinating polyneuropathy (CIDP) is feasible and safe and superior to treatment with saline for the performance of muscle strength. Patients with motor involvement in maintenance therapy with int......We hypothesized that subcutaneous administration of immunoglobulins (SCIG) in chronic inflammatory demyelinating polyneuropathy (CIDP) is feasible and safe and superior to treatment with saline for the performance of muscle strength. Patients with motor involvement in maintenance therapy...... with intravenous immunoglobulin (IVIg) fulfilling the EFNS/PNS criteria for CIDP, aged 18-80 years, were randomised either to SCIG at a dose determined from their pre-study IVIg dose or to subcutaneous saline given twice or thrice weekly for 12 weeks at home. At the start and end of the trial, as well as two weeks...... of immunoglobulins in CIDP is feasible, safe and effective and seems an attractive alternative to IVIg....

  5. European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: Report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - First Revision

    NARCIS (Netherlands)

    P.Y.K. van den Bergh; R.D.M. Hadden; P. Bouche; D.R. Cornblath; A. Hahn; I. Illa; C.L. Koski; J.M. Leger; E. Nobile-Orazio; J. Pollard; C. Sommer; P.A. van Doorn; I.N. van Schaik

    2010-01-01

    Background: Consensus guidelines on the definition, investigation, and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) have been previously published in European Journal of Neurology and Journal of the Peripheral Nervous System. Objectives: To revise these guidelines. M

  6. T cell reactivity to P0, P2, PMP-22, and myelin basic protein in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyradiculoneuropathy

    OpenAIRE

    Csurhes, P; Sullivan, A.; Green, K.; Pender, M.; McCombe, P

    2005-01-01

    Objectives: It has been suggested that autoimmunity to peripheral myelin proteins is involved in the pathogenesis of Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). We aimed to compare reactivity of peripheral blood mononuclear cells (PBMC) to antigens of peripheral myelin proteins in patients with GBS and patients with CIDP with that of healthy controls and patients with other non-immune mediated neuropathies (ON).

  7. Isolation and characterisation of T lymphocytes from sural nerve biopsies in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy.

    OpenAIRE

    Ben-Smith, A.; Gaston, J S; Barber, P. C.; Winer, J B

    1996-01-01

    OBJECTIVES: To characterise cultured T lymphocytes from nerve biopsies in patients with Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy (CIDP). METHODS: Sural nerve biopsies, obtained from six patients with Guillain-Barré syndrome, four with CIDP, and six controls with other neuropathies, were cultured with 20 U/ml recombinant interleukin-2 (IL-2) for eight weeks. Flow cytometry was used to determine the phenotype of cultured T lymphocytes. Their proliferative re...

  8. MRI of the cervical nerve roots in the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy: a single-institution, retrospective case–control study

    OpenAIRE

    Tanaka, Kanta; Mori, Nobuyuki; Yokota, Yusuke; Suenaga, Toshihiko

    2013-01-01

    Objective To systematically evaluate the usefulness of assessing the cervical nerve roots by MRI for the diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Design Single-institution, retrospective case–control study. Setting A regional referral hospital. Participants We retrospectively enrolled 15 consecutive patients with CIDP who satisfied the European Federation of Neurological Societies/Peripheral Nerve Society (EFNS/PNS) typical and definite criteria and under...

  9. Cost-utility of Intravenous Immunoglobulin (IVIG) compared with corticosteroids for the treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP) in Canada

    OpenAIRE

    Campbell Kaitryn; Xie Feng; Gaebel Kathryn; Blackhouse Gord; Assasi Nazila; Tarride Jean-Eric; O'Reilly Daria; Chalk Colin; Levine Mitchell; Goeree Ron

    2010-01-01

    Abstract Objectives Intravenous immunoglobulin (IVIG) has demonstrated improvement in chronic inflammatory demyelinating polyneuropathy (CIDP) patients in placebo controlled trials. However, IVIG is also much more expensive than alternative treatments such as corticosteroids. The objective of the paper is to evaluate, from a Canadian perspective, the cost-effectiveness of IVIG compared to corticosteroid treatment of CIDP. Methods A markov model was used to evaluate the costs and QALYs for IVI...

  10. A current view of the diagnosis, clinical variants, response to treatment and prognosis of chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Viala, Karine; Maisonobe, Thierry; Stojkovic, Tanya; Koutlidis, Régine; Ayrignac, Xavier; Musset, Lucile; Fournier, Emmanuel; Léger, Jean-Marc; Bouche, Pierre

    2010-03-01

    We retrospectively analyzed 146 patients fulfilling the European Federation of Neurological Societies and the Peripheral Nerve Society (EFNS/PNS) criteria for definite chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) to (1) evaluate the relevance of these criteria, (2) assess the frequency of CIDP variants, and (3) determine the response to treatment and the prognosis. We found that 75% of these patients fulfilled the main EFNS/PNS clinical and electrophysiological criteria (type I). The remaining patients were diagnosed using laboratory tools as supportive criteria. The common form of CIDP represented 51% of patients. We observed a high frequency of the sensory variant (35% of patients) and the rapid onset form (18%). A positive response to treatment was observed in 87% of patients, with a similar efficacy of prednisone and IVIg. However, in the long term, 40% of treated patients remained dependent on treatment. The IVIg dependency rate was higher than the prednisone or plasma exchange dependency rate (55%, 18%, and 23%, respectively; p = 0.0054). Severe handicap was observed in 24% of patients.

  11. Impairment of circulating CD4+CD25+ regulatory T cells in patients with chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Chi, Li-Jun; Wang, Hua-Bing; Wang, Wei-Zhi

    2008-03-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an immune-mediated peripheral nervous system disease. CD4+CD25+ T regulatory cells (Tregs) have been unequivocally shown to be critical in maintaining immune tolerance and preventing auto-immune diseases by suppressing self-reactive T cells. Thus, we hypothesized that the numbers and/or the function of Tregs would be deranged during the progressive or relapse phases of CIDP. The number of Tregs was determined by flow cytometry according to their characteristic CD4+CD25(high) membrane phenotype. Functional characterization of Tregs was analyzed by suppression of proliferation and secretion of cytokines by co-cultured effector CD4+CD25- T cells. FOXP3 message expression level was assessed by quantitative real-time polymerase chain reaction. The results showed significant reduction in both the number and the suppressive function of Tregs in the patients with CIDP compared with healthy controls. Also, Tregs isolated from CIDP patients expressed lower levels of FoxP3 mRNA. During the progressive or the relapsing phases of CIDP, the number of Tregs was reduced, and the suppressive function of them decreased. These findings may be helpful to our understanding of the possible role of Tregs in the pathogenesis of CIDP.

  12. Contribution of plexus MRI in the diagnosis of atypical chronic inflammatory demyelinating polyneuropathies.

    Science.gov (United States)

    Lozeron, Pierre; Lacour, Marie-Christine; Vandendries, Christophe; Théaudin, Marie; Cauquil, Cécile; Denier, Christian; Lacroix, Catherine; Adams, David

    2016-01-15

    Nerve enlargement has early been recognized in CIDP and plexus MRI hypertrophy has been reported in typical CIDP cases. Our aim is to determine plexus MRI value in the diagnosis of CIDP with an initial atypical presentation, which, up to now, has not been demonstrated. Retrospective study of 33 consecutive patients suspected of CIDP. Plexus MRI was performed on the most affected territory (brachial or lumbar). Were assessed: plexus trophicity, T2-STIR signal intensity and gadolinium enhancement. Final CIDP diagnosis was made after comprehensive workup. A histo-radiological correlation was performed. Final CIDP diagnosis was made in 25 (76%) including 21 with initial atypical clinical presentation. Eleven CIDP patients (52%) with initial atypical clinical presentation had abnormal plexus MRI including 9 suggestive of CIDP (43%) and none of the patients with an alternative diagnosis. Hypertrophy of the proximal plexus and/or extraforaminal roots was found in 8 cases and Gadolinium enhancement in 2 cases. Abnormalities were more frequent on brachial (86%) than lumbosacral MRIs (29%) and asymmetrical (72%) and most often associated with histological signs of demyelination. The nerve biopsy was suggestive of CIDP in 9/13 patients with normal MRI. Plexus MRI seems useful in the diagnostic strategy of patients with suspicion of CIDP with atypical presentation. Nerve biopsy remains important when other investigations are inconclusive. PMID:26723995

  13. [Topics of chronic inflammatory demyelinating polyradiculoneuropathy--diagnosis and treatment situation in Japan].

    Science.gov (United States)

    Iijima, Masahiro

    2013-05-01

    CIDP is a motor and sensory neuropathy characterized by chronic, step-wised, or relapsing progression. Both cellular and humoral autoimmunity targeting the myelin sheath is assumed as the main mechanism of CIDP pathogenesis. While the AAN diagnostic criteria have been the main method in Japan, the EFNS/PNS criteria recently replaced them because of their good diagnostic sensitivity and clinical superiority. The first-line therapy for CIDP patients in Japan is intravenous immunoglobulin (IVIg), corticosteroids, and phasmapheresis, the same as in other countries. Regarding therapeutics, two major differences between Japan and other countries exist. Firstly, while half-dose IVIg (1 g/kg body weight) every three weeks was established as maintenance therapy as a result of the ICE study in 2008, full-dose IVIg (2 g/kg body weight over five days) once a month is still accepted in Japan for highly recurrent patients. Secondly, Japanese clinicians prefer immune adsorption plasmapheresis (IAPP) instead of plasma exchange (PE) among three types of plasmapheresis (IAPP, PE, and double-filtered plasmapheresis [DFPP]). These differences could be due to the characteristic and independent health insurance system in Japan. Using recent knowledge and diagnostic criteria, clinical trials have been based on these global platforms. Recently, efforts have been made to share these platforms with a worldwide vision.

  14. The characteristics of chronic inflammatory demyelinating polyneuropathy in patients with and without diabetes--an observational study.

    Directory of Open Access Journals (Sweden)

    Samantha K Dunnigan

    Full Text Available INTRODUCTION: We aimed to determine whether the clinical characteristics and electrodiagnostic classification of nerve injury, and response to treatment differed in patients diagnosed with chronic inflammatory demyelinating polyneuropathy (CIDP with and without diabetes. METHODS: CIDP patients with diabetes (CIDP+DM (n = 67 and without diabetes (CIDP-DM (n = 67 underwent clinical examination and nerve conduction studies (NCS. CIDP-DM patients were selected using age and gender matching with the existing CIDP+DM cohort. Patients treated with immunotherapies were classified as responders (R (n = 46 or non-responders (NR (n = 54 based on clinical response to treatment. The groups were compared using analysis of variance, contingency tables and Kruskal-Wallis analyses. RESULTS: CIDP+DM subjects had more severe neuropathy based on higher lower limb vibration potential thresholds (VPT(p = 0.004, higher Toronto Clinical Neuropathy Score (TCNS (p = 0.0009, more proximal weakness (p = 0.03, more gait abnormality (p = 0.03 and more abnormal NCS. CIDP+DM subjects had more abnormal sural NCS with lower sural sensory nerve action potential amplitudes (2.4±3.0 µV, 6.6±6.0 µV, p<0.0001 and slower sural nerve conduction velocities (38.6±5.4 m/s, 41.0±5.3 m/s, p = 0.04. CIDP-DM subjects were more likely to receive immune therapies (93% vs 57%, p = <0.0001, despite no significant differences in treatment responder rates (p = 0.71. Patients who responded to therapy had shorter duration of CIDP than non-responders (8.0±6.0 y vs 11.9±7.6 y, p = 0.004. DISCUSSION: The clinical phenotype and electrophysiological profile of CIDP patients differs according to the presence or absence of diabetes. Despite CIDP+DM patients having more severe clinical and electrophysiological neuropathy, they are less likely to receive disease-modifying/specific therapy, yet have similar response rates to treatment as those without

  15. Transcriptome Analysis of Peripheral Blood in Chronic Inflammatory Demyelinating Polyradiculoneuropathy Patients Identifies TNFR1 and TLR Pathways in the IVIg Response.

    Science.gov (United States)

    Richard, Alexandra; Corvol, Jean-Christophe; Debs, Rabab; Reach, Pauline; Tahiri, Khadija; Carpentier, Wassila; Gueguen, Justine; Guillemot, Vincent; Labeyrie, Céline; Adams, David; Viala, Karine; Cohen Aubart, Fleur

    2016-05-01

    We have studied the response to intravenous immunoglobulins (IVIg) by a transcriptomic approach in 11 chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients (CIDP duration = 6 [0.83-6.5] years). RNA was extracted from cells in whole blood collected before and 3 weeks after IVIg treatment, and hybridized on Illumina chips. After RNA quality controls, gene expression was analyzed using statistical tests fitted for microarrays (R software, limma package), and a pathway analysis was performed using DAVID software. We identified 52 genes with expression that varied significantly after IVIg (fold change [FC] > 1.2, P CIDP pathophysiology and the response to IVIg. We conclude that responder patients have stronger inflammatory activity that is lessened by IVIg. PMID:27175635

  16. Transcriptome Analysis of Peripheral Blood in Chronic Inflammatory Demyelinating Polyradiculoneuropathy Patients Identifies TNFR1 and TLR Pathways in the IVIg Response.

    Science.gov (United States)

    Richard, Alexandra; Corvol, Jean-Christophe; Debs, Rabab; Reach, Pauline; Tahiri, Khadija; Carpentier, Wassila; Gueguen, Justine; Guillemot, Vincent; Labeyrie, Céline; Adams, David; Viala, Karine; Cohen Aubart, Fleur

    2016-05-01

    We have studied the response to intravenous immunoglobulins (IVIg) by a transcriptomic approach in 11 chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) patients (CIDP duration = 6 [0.83-6.5] years). RNA was extracted from cells in whole blood collected before and 3 weeks after IVIg treatment, and hybridized on Illumina chips. After RNA quality controls, gene expression was analyzed using statistical tests fitted for microarrays (R software, limma package), and a pathway analysis was performed using DAVID software. We identified 52 genes with expression that varied significantly after IVIg (fold change [FC] > 1.2, P CIDP pathophysiology and the response to IVIg. We conclude that responder patients have stronger inflammatory activity that is lessened by IVIg.

  17. [Autopsy case of a patient with Charcot-Marie-Tooth disease type 1A and suspected chronic inflammatory demyelinating polyradiculoneuropathy, which was later diagnosed as amyotrophic lateral sclerosis].

    Science.gov (United States)

    Higuchi, Yujiro; Sakiyama, Yusuke; Nishihira, Yasushi; Endo, Kazuhiro; Suwazono, Shugo; Suehara, Masahito

    2012-01-01

    We report an autopsy case of a 74-year-old man with late onset Charcot-Marie-Tooth disease type 1A (CMT1A) diagnosed by genetic screening, later associated with amyotrophic lateral sclerosis (ALS). At the age of 70 years, the patient was admitted to our hospital because of progressive weakness and dysesthesia in the right upper limb. In the early stages of the illness, he was diagnosed with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), and transient improvement was achieved with intravenous immunoglobulin. However, the symptoms progressively worsened and became refractory. Gene analysis revealed PMP22 gene duplication, which confirmed CMT1A. On sural nerve biopsy, severe demyelinating neuropathy and abundant onion-bulb formations with endoneurial infiltration of inflammatory cells were observed. Thereafter, pseudo-bulbar palsy and respiratory muscle weakness developed insidiously and progressed rapidly along with muscle weakness in the limbs and trunk. The patient died about four years after the onset of this disease. Postmortem examination showed moderate neuronal cell loss, Bunina bodies, and TDP-43-positive inclusions in the anterior horn cells. The spinal cord revealed axonal loss and extensive macrophage permeation in the corticospinal tracts. On the basis of these findings, the final neuropathological diagnosis was ALS. This is the first report of an autopsy case of CMT1A complicated with ALS. We here discuss the significant clinical and neuropathological findings of this case.

  18. Treatment of chronic inflammatory neuropathies

    NARCIS (Netherlands)

    F. Eftimov

    2015-01-01

    This thesis focuses on the efficacy of existing and alternative treatments in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) and explores predictors of treatment response in patients with CIDP treated with corticosteroids. The efficacy of intra

  19. 慢性炎性脱髓鞘性多发性神经病的药物治疗现状%Current Medical Treatment of Chronic Inflammatory Demyelinating Polyradiculopathy

    Institute of Scientific and Technical Information of China (English)

    陈远春

    2010-01-01

    @@ 慢性炎性脱髓鞘性多发性神经病(Chronic inflammatory demyelinating polyradiculopathy,CIDP)是一种获得性的周围神经脱髓鞘性疾病,以反复发作的肌无力为特征,可伴感觉缺失和腱反射消失等.

  20. A randomised, double-blinded, placebo controlled trial of the effect of subcutaneous immunoglobulin on muscular performance in chronic inflammatory demyelinating polyneuropathy

    DEFF Research Database (Denmark)

    Jakobsen, Johannes Klitgaard; Markvardsen, Lars Høj; Harbo, Thomas;

    Objective: We hypothesised that the effect of subcutaneous infusion of immunoglobulins(SCIG) on muscular performance in chronic inflammatory demyelinating polyneuropathy(CIDP) is superior to that of placebo and equals the therapeutic effect of intravenous infusion(IVIG). Background Subcutaneous...... treatment with large amounts of immunoglobulins in multifocal motor neuropathy is feasible, safe and effective. In CIDP case reports indicate its therapeutic usefullness as well. Design/Methods: Subjects in IVIG maintenace therapy full-filling the EFNS/PNS criteria for CIDP were considered for participation....... Exclusion criteria were pregnancy, malignancies, dementia, severe medical disorders and psychiatric disturbances. Thirty CIDP patients(n,30) characterized as IVIG-responders by their treating physicians were included in the study.Participants were randomised to receive either subcutaneous Ig...

  1. Studies of HLA associations in male and female patients with Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

    Science.gov (United States)

    McCombe, Pamela A; Csurhes, Peter A; Greer, Judith M

    2006-11-01

    HLA associations are found to differ with the gender of the patient in some autoimmune diseases. Here we have investigated whether there are gender-related HLA associations in Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), both of which occur more frequently in male patients than in females. In GBS, no particular HLA associations were noted, except for a slight negative association in both males and females for carriage of HLA-DR5. In CIDP, the gene frequency and the frequency of individuals positive for HLA-DR2 were greater in female patients than female controls, although this was statistically significant only for the gene frequency. Furthermore more female CIDP patients were homozygous for DR2, than male CIDP patients, or male or female controls and patients with GBS. This suggests that sex-related factors may interact with the risk associated with carriage of HLA-DR2 for development of CIDP.

  2. Epstein-Barr virus antibodies in serum and cerebrospinal fluid from multiple sclerosis, chronic inflammatory demyelinating polyradiculoneuropathy and amyotrophic lateral sclerosis.

    Science.gov (United States)

    Nociti, V; Frisullo, G; Marti, A; Luigetti, M; Iorio, R; Patanella, A K; Bianco, A; Tonali, P A; Grillo, R L; Sabatelli, M; Batocchi, A P

    2010-08-25

    Elevated anti-Epstein-Barr virus (EBV) antibody levels are present in serum of Multiple sclerosis (MS) patients but literature lacks of studies comparing anti-EBV antibody levels between MS and other neurological diseases. We evaluate anti-VCA IgG and IgM, anti-EBNA1 IgG, anti-Cytomegalovirus IgG and IgM titres in serum and cerebrospinal fluid (CSF) of 267 MS, 50 Chronic Inflammatory Demyelinating Polyradiculoneuropathy (CIDP) and 88 Amyotrophic Lateral Sclerosis (ALS) patients. We found increased titres of anti-EBV-IgG in serum and CSF of MS subjects as compared to CIDP and ALS patients thus providing additional evidence for a possible involvement of EBV in MS.

  3. A case of a 17-year-old male with neurofascin-155 antibody-positive chronic inflammatory demyelinating polyradiculoneuropathy presenting with tremor and ataxia.

    Science.gov (United States)

    Itaya, Kazuhiro; Inoue, Manabu; Iizuka, Natsuko; Shimizu, Yuki; Yuki, Nobuhiro; Ichikawa, Hiroo

    2016-09-29

    A 17-year-old male with no medical history noticed weakness of his limbs with imbalance and subsequent finger tremors. Physical examination revealed features of polyneuropathy, including diffuse weakness, distal symmetrical numbness with impaired deep sensation and areflexia in all limbs. Postural tremor was present in fingers. Ataxia was apparent in both lower limbs, causing a wide-based gait with a positive Romberg sign. Cerebrospinal fluid contained elevated total protein without pleocytosis. A nerve conduction study disclosed demyelinating features with prolonged terminal latencies, slow velocities with delayed F-wave latencies, and prominent temporal dispersion. These findings led to diagnosis of typical chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) with notable feature of postural finger tremor and ataxia of unknown cause. These atypical features prompted us to examine neurofascin-155 (NF155) antibodies, which were positive. No significant improvement occurred after initial administration of intravenous immunoglobulin and subsequent plasma exchange. However, corticosteroids with intravenous pulse therapy followed by oral prednisolone significantly improved the symptoms. Patients with CIDP with anti-NF155 antibodies may have similar clinical features and constitute a CIDP subgroup. In such patients, corticosteroids may be more effective than intravenous immunoglobulin. Further studies are needed to define the features of this subgroup and determine effective therapy for CIDP.

  4. Chronic dysimmune neuropathies: Beyond chronic demyelinating polyradiculoneuropathy

    Directory of Open Access Journals (Sweden)

    Khadilkar Satish

    2011-01-01

    Full Text Available The spectrum of chronic dysimmune neuropathies has widened well beyond chronic demyelinating polyradiculoneuropathy (CIDP. Pure motor (multifocal motor neuropathy, sensorimotor with asymmetrical involvement (multifocal acquired demylinating sensory and motor neuropathy, exclusively distal sensory (distal acquired demyelinating sensory neuropathy and very proximal sensory (chronic immune sensory polyradiculopathy constitute the variants of CIDP. Correct diagnosis of these entities is of importance in terms of initiation of appropriate therapy as well as prognostication of these patients. The rates of detection of immune-mediated neuropathies with monoclonal cell proliferation (monoclonal gammopathy of unknown significance, multiple myeloma, etc. have been facilitated as better diagnostic tools such as serum immunofixation electrophoresis are being used more often. Immune neuropathies associated with malignancies and systemic vasculitic disorders are being defined further and treated early with better understanding of the disease processes. As this field of dysimmune neuropathies will evolve in the future, some of the curious aspects of the clinical presentations and response patterns to different immunosuppressants or immunomodulators will be further elucidated. This review also discusses representative case studies.

  5. Understanding the consequences of chronic inflammatory demyelinating polyradiculoneuropathy from impairments to activity and participation restrictions and reduced quality of life: the ICE study.

    Science.gov (United States)

    Merkies, Ingemar S J; Hughes, Richard A C; Donofrio, Peter; Bril, Vera; Dalakas, Marinos C; Hanna, Kim; Hartung, Hans-Peter; Latov, Norman; van Doorn, Pieter A; Deng, Chunqin

    2010-09-01

    A randomized trial (ICE trial) in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) demonstrated significantly more improvement with intravenous immunoglobulin (Gamunex(®), Talecris Biotherapeutics, Inc., Research Triangle Park, NC) than placebo. To understand the relationship between CIDP impairments, activity and participation restrictions, and quality of life (QoL) in this trial, we investigated the association between scales representing these outcome levels. Gamunex or placebo was given every 3 weeks for up to 24 weeks to 117 patients in an initial treatment period after which treatment failures were crossed over (alternative treatment). We assessed impairments, activity and participation, and SF-36 component mental (MCS) and physical summaries (PCS). Regression analyses of baseline data were performed (all subjects) and change from baseline to endpoint (Gamunex-treated group only) to determine correlations between outcomes. Grip strength, medical research council (MRC) sum score, and inflammatory neuropathy cause and treatment (INCAT) sensory sum score were the strongest explanatory variables of disability (at baseline: r(2) = 0.46; change from baseline: r(2) = 0.66). Only up to half of the variance in QoL scores (PCS at baseline: r(2) = 0.30; change from baseline: r(2) = 0.41; MCS: at baseline: r(2) = 0.10; change from baseline: r(2) = 0.24) was explained by impairment and activity and participation measures. Future studies are required to elucidate the impact of CIDP on disability and QoL changes, because the obtained correlations provide only partial explanation.

  6. European Federation of Neurological Societies Peripheral Nerve Society guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society (Reprinted from Journal of the Peripheral Nervous System, vol 10, pg 220-228, 2005)

    NARCIS (Netherlands)

    R.A.C. Hughes; P. Bouche; D.R. Cornblath; E. Evers; R.D.M. Hadden; A. Hahn; I. Illa; C.L. Koski; J.M. Leger; E. Nobile-Orazio; J. Pollard; C. Sommer; P. van den Bergh; P.A. van Doorn; I.N. van Schaik; M.M. Mehndiratta; R. Hughes; J.B. Winer; R. de Haan; M. Vermeulen; P. Agarwal

    2006-01-01

    Numerous sets of diagnostic criteria have sought to define chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and randomized trials and systematic reviews of treatment have been published. The objective is to prepare consensus guidelines on the definition, investigation and treatment o

  7. pSTAT1, pSTAT3, and T-bet as markers of disease activity in chronic inflammatory demyelinating polyradiculoneuropathy.

    Science.gov (United States)

    Madia, Francesca; Frisullo, Giovanni; Nociti, Viviana; Conte, Amelia; Luigetti, Marco; Del Grande, Alessandra; Patanella, Agata Katia; Iorio, Raffaele; Tonali, Pietro Attilio; Batocchi, Anna Paola; Sabatelli, Mario

    2009-06-01

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered an auto-immune disorder. We evaluated expression of pSTAT1, T-bet, and pSTAT3 in circulating T-cells, B-cells, and monocytes and spontaneous production of interleukin-17 (IL17), interferon-gamma (IFN gamma), and interleukin-10 (IL10) by peripheral blood mononuclear cells (PBMCs) from 14 active CIDP patients compared with 6 patients with long-lasting remission and 20 controls. Active disease patients showed higher pSTAT1, T-bet, and pSTAT3 in CD4(+) T-cells than controls (p CIDP patients than controls (p = 0.0011, p = 0.0041, p = 0.0413, respectively) and remission patients (p = 0.0073, p = 0.0274, p = 0.0251, respectively). Moreover in CD8(+) T-cells, pSTAT3 expression was higher in active CIDP patients than in remission patients (p = 0.0345) and in controls (p = 0.0023). IL17 and IFN gamma production were significantly higher in active CIDP patients than in controls (p CIDP patients (p = 0.0073). IL10 levels were higher in active phase patients than in controls (p = 0.0334). Our data suggest that pSTAT1, T-bet, and pSTAT3 can be considered putative markers of disease activity and potential targets for specific therapies.

  8. Cost-utility of Intravenous Immunoglobulin (IVIG compared with corticosteroids for the treatment of Chronic Inflammatory Demyelinating Polyneuropathy (CIDP in Canada

    Directory of Open Access Journals (Sweden)

    Campbell Kaitryn

    2010-06-01

    Full Text Available Abstract Objectives Intravenous immunoglobulin (IVIG has demonstrated improvement in chronic inflammatory demyelinating polyneuropathy (CIDP patients in placebo controlled trials. However, IVIG is also much more expensive than alternative treatments such as corticosteroids. The objective of the paper is to evaluate, from a Canadian perspective, the cost-effectiveness of IVIG compared to corticosteroid treatment of CIDP. Methods A markov model was used to evaluate the costs and QALYs for IVIG and corticosteroids over 5 years of treatment for CIDP. Patients initially responding to IVIG could remain a responder or relapse every 12 week model cycle. Non-responding IVIG patients were assumed to be switched to corticosteroids. Patients on corticosteroids were at risk of a number of adverse events (fracture, diabetes, glaucoma, cataract, serious infection in each cycle. Results Over the 5 year time horizon, the model estimated the incremental costs and QALYs of IVIG treatment compared to corticosteroid treatment to be $124,065 and 0.177 respectively. The incremental cost per QALY gained of IVIG was estimated to be $687,287. The cost per QALY of IVIG was sensitive to the assumptions regarding frequency and dosing of maintenance IVIG. Conclusions Based on common willingness to pay thresholds, IVIG would not be perceived as a cost effective treatment for CIDP.

  9. Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) associated to hereditary neuropathy with liability to pressure palsies (HNPP) and revealed after influenza AH1N1 vaccination.

    Science.gov (United States)

    Remiche, Gauthier; Abramowicz, Marc; Mavroudakis, Nicolas

    2013-12-01

    Neurological complications of AH1N1 vaccination such as Guillain-Barré syndrome were described in the previous years. Several reports suggest that hereditary neuropathies may be a predisposing factor for immune-mediated neuropathies. We report the case of a 54-year-old female who developed chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) 5 weeks after AH1N1 vaccination. She had no previous neurological history, but neurophysiological features led us to suspect an underlying hereditary neuropathy. PMP22 gene analysis showed a typical deletion, confirming the diagnosis of hereditary neuropathy with liability to pressure palsies (HNPP). We observed a significant clinical and neurophysiological improvement of the neuropathy after intravenous immunoglobulin treatment. This is, to our knowledge, the first reported case of CIDP potentially triggered by AH1N1 vaccination. This and previous observations suggest that genetic-determined neuropathies could predispose to the occurrence of immune-mediated neuropathies. One must recall the possibility of a superimposed hereditary neuropathy like HNPP in patients with a clinical presentation of CIDP, especially when positive family history or unexpected neurophysiological features are present.

  10. Aggregation of MBP in chronic demyelination

    Science.gov (United States)

    Frid, Kati; Einstein, Ofira; Friedman-Levi, Yael; Binyamin, Orli; Ben-Hur, Tamir; Gabizon, Ruth

    2015-01-01

    Objectives Misfolding of key disease proteins to an insoluble state is associated with most neurodegenerative conditions, such as prion, Parkinson, and Alzheimer’s diseases. In this work, and by studying animal models of multiple sclerosis, we asked whether this is also the case for myelin basic protein (MBP) in the late and neurodegenerative phases of demyelinating diseases. Methods To this effect, we tested whether MBP, an essential myelin component, present prion-like properties in animal models of MS, as is the case for Cuprizone-induced chronic demyelination or chronic phases of Experimental Autoimmune Encephalomyelitis (EAE). Results We show here that while total levels of MBP were not reduced following extensive demyelination, part of these molecules accumulated thereafter as aggregates inside oligodendrocytes or around neuronal cells. In chronic EAE, MBP precipitated concomitantly with Tau, a marker of diverse neurodegenerative conditions, including MS. Most important, analysis of fractions from Triton X-100 floatation gradients suggest that the lipid composition of brain membranes in chronic EAE differs significantly from that of naïve mice, an effect which may relate to oxidative insults and subsequently prevent the appropriate insertion and compaction of new MBP in the myelin sheath, thereby causing its misfolding and aggregation. Interpretation Prion-like aggregation of MBP following chronic demyelination may result from an aberrant lipid composition accompanying this pathological status. Such aggregation of MBP may contribute to neuronal damage that occurs in the progressive phase of MS. PMID:26273684

  11. Polirradiculoneuropatia desmielinizante inflamatória crônica: estudo de 18 pacientes Chronic inflammatory demyelinating polyradiculoneuropathy: study of 18 patients

    Directory of Open Access Journals (Sweden)

    Leandro C. Calia

    1997-01-01

    Full Text Available Neste estudo prospectivo, analisamos as características clínicas, evolução e resposta terapêutica de 18 pacientes com a forma idiopática de polirradiculoneuropatia desmielinizante inflamatória crônica, que foram acompanhados por período que variou de 4 a 127 meses. O sexo masculino predominou sobre o feminino (1,25:1 e a idade de início dos sintomas variou de 6 a 85 anos. Observamos a preponderância da forma de evolução progressiva (61,1% sobre a forma recidivante (38,9%, bem como a baixa ocorrência de fatores predisponentes (16,7%. Todos os pacientes apresentavam comprometimento sensitivo e motor, associado a hipo ou arreflexia, enquanto apenas três (16,7% apresentavam comprometimento de nervos cranianos. No exame do liquor, as taxas de proteínas estavam elevadas em 88,9% dos pacientes, com média de 203,4 mg/dl. A eletroneuromiografia mostrou alterações desmielinizantes em todos os pacientes, associadas a alterações axonais em 94,4% deles. Em todos os sete pacientes submetidos a biopsia de nervo sural encontramos alterações compatíveis com desmielinização/remielinização. A análise com imunofluorescência, realizada em três pacientes foi normal em um e evidenciou depósito de anticorpos anti-CD3 em dois e anti-HLA-Dr em um. Optamos pela prednisona como tratamento inicial em todos os pacientes, sendo mantida posteriormente em doses reduzidas e em dias alternados em 72,2% deles. Dois pacientes (11,1% estão assintomáticos mesmo após retirada total da medicação e introduzimos azatioprína, associada ou não ao corticóide, nos quatro pacientes com má resposta à prednisona. Até a última avaliação, 16 pacientes (88,9% evoluíram com melhora funcional.This is a prospective study that describes 18 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP, idiopathic type. The patients have been followed for a period of 4 to 127 months. We evaluated the clinical characteristics, the evolution

  12. Inhibition of System Xc(-) Transporter Attenuates Autoimmune Inflammatory Demyelination.

    Science.gov (United States)

    Evonuk, Kirsten S; Baker, Brandi J; Doyle, Ryan E; Moseley, Carson E; Sestero, Christine M; Johnston, Bryce P; De Sarno, Patrizia; Tang, Andrew; Gembitsky, Igor; Hewett, Sandra J; Weaver, Casey T; Raman, Chander; DeSilva, Tara M

    2015-07-15

    T cell infiltration into the CNS is a significant underlying pathogenesis in autoimmune inflammatory demyelinating diseases. Several lines of evidence suggest that glutamate dysregulation in the CNS is an important consequence of immune cell infiltration in neuroinflammatory demyelinating diseases; yet, the causal link between inflammation and glutamate dysregulation is not well understood. A major source of glutamate release during oxidative stress is the system Xc(-) transporter; however, this mechanism has not been tested in animal models of autoimmune inflammatory demyelination. We find that pharmacological and genetic inhibition of system Xc(-) attenuates chronic and relapsing-remitting experimental autoimmune encephalomyelitis (EAE). Remarkably, pharmacological blockade of system Xc(-) 7 d after induction of EAE attenuated T cell infiltration into the CNS, but not T cell activation in the periphery. Mice harboring a Slc7a11 (xCT) mutation that inactivated system Xc(-) were resistant to EAE, corroborating a central role for system Xc(-) in mediating immune cell infiltration. We next examined the role of the system Xc(-) transporter in the CNS after immune cell infiltration. Pharmacological inhibitors of the system Xc(-) transporter administered during the first relapse in a SJL animal model of relapsing-remitting EAE abrogated clinical disease, inflammation, and myelin loss. Primary coculture studies demonstrate that myelin-specific CD4(+) Th1 cells provoke microglia to release glutamate via the system Xc(-) transporter, causing excitotoxic death to mature myelin-producing oligodendrocytes. Taken together, these studies support a novel role for the system Xc(-) transporter in mediating T cell infiltration into the CNS as well as promoting myelin destruction after immune cell infiltration in EAE.

  13. Fampridine-PR (prolonged released 4-aminopyridine) is not effective in patients with inflammatory demyelination of the peripheral nervous system.

    Science.gov (United States)

    Leussink, Verena-Isabell; Stettner, Mark; Warnke, Clemens; Hartung, Hans-Peter

    2016-06-01

    Fampridine-PR is a voltage-gated potassium channel inhibitor potentially improving nerve conduction in demyelinated axons. Based on its established clinical efficacy in patients with demyelination in the central nervous system, we assessed if fampridine-PR is also effective in patients with inflammatory demyelination of the peripheral nerve. In this small open-label study, 10 patients with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) were treated with fampridine-PR 10 mg BID for 28 days and assessed clinically as well as by nerve conduction studies. In this study, Fampridine-PR failed to improve CIDP based on clinical measures and nerve conduction studies. Our findings suggest that Fampridine-PR appears to be ineffective in demyelinating polyneuropathies. These observations may indicate a more complex mode of action beyond improving action potential conduction in demyelinated axons. PMID:26968589

  14. Inflammatory demyelinating pseudotumor with hemorrhage masquerading high grade cerebral neoplasm

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    Amit Agrawal

    2015-03-01

    Full Text Available Demyelinating pseudotumors are rare, benign, solitary intracranial space occupying lesions which masquerade cerebral neoplasms. Contrast MRI shows open ring enhancement which is fairly specific for this entity. Advanced MRI techniques like MR spectroscopy and magnetizing transfer techniques can help differentiating these lesions. NAA/Cr ratio is significantly elevated in central regions of demyelinating pseudotumors than in gliomas and other lesions. Presence of abundant foamy macrophages, lymphoid inflammatory infiltrates around blood vessels, sheets of gemistocytic astrocytes with well-developed processes, well defined border of the lesion absence of neovascularity and necrosis should help us diagnose demyelinating pseudotumor fairly confidently on histopathology.

  15. Astrogliosis during acute and chronic cuprizone demyelination and implications for remyelination

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    Tuan Q. Le

    2012-10-01

    Full Text Available In multiple sclerosis, microglia/macrophage activation and astrocyte reactivity are important components of the lesion environment that can impact remyelination. The current study characterizes these glial populations relative to expression of candidate regulatory molecules in cuprizone demyelinated corpus callosum. Importantly, periods of recovery after acute or chronic cuprizone demyelination are examined to compare conditions of efficient versus limited remyelination, respectively. Microglial activation attenuates after early demyelination. In contrast, astrocyte reactivity persists throughout demyelination and a 6-week recovery period following either acute or chronic demyelination. This astrocyte reaction is characterized by (a early proliferation, (b increased expression of GFAP (glial fibrillary acidic protein, Vim (vimentin, Fn1 (fibronectin and CSPGs (chondroitin sulphate proteoglycans and (c elaboration of a dense network of processes. Glial processes elongated in the axonal plane persist throughout lesion areas during both the robust remyelination that follows acute demyelination and the partial remyelination that follows chronic demyelination. However, prolonged astrocyte reactivity with chronic cuprizone treatment does not progress to barrier formation, i.e. dense compaction of astrocyte processes to wall off the lesion area. Multiple candidate growth factors and inflammatory signals in the lesion environment show strong correlations with GFAP across the acute cuprizone demyelination and recovery time course, yet there is more divergence across the progression of chronic cuprizone demyelination and recovery. However, differential glial scar formation does not appear to be responsible for differential remyelination during recovery in the cuprizone model. The astrocyte phenotype and lesion characteristics in this demyelination model inform studies to identify triggers of non-remyelinating sclerosis in chronic multiple sclerosis

  16. The frequencies of Killer immunoglobulin-like receptors and their HLA ligands in chronic inflammatory demyelinating polyradiculoneuropathy are similar to those in Guillian Barre syndrome but differ from those of controls, suggesting a role for NK cells in pathogenesis.

    Science.gov (United States)

    Blum, Stefan; Csurhes, Peter; McCombe, Pamela

    2015-08-15

    Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an acquired inflammatory neuropathy, which has similar clinical and pathological features to Guillain-Barré Syndrome (GBS), but differs in time course. We investigated the frequency of genes encoding Killer immunoglobulin-like receptors and their HLA ligands in subjects with CIDP, in subjects with GBS and in healthy controls. There were no differences in KIR gene frequency among the 3 groups. The gene frequencies for HLA-B Bw4-I were significantly greater in CIDP than HC, but did not differ from GBS. The frequency of the combination of 3DL1/HLA-B Bw4I was greater in CIDP than HC, but did not differ from that of GBS. These data raise the possibility of NK cell function being an important factor in the pathogenesis of CIDP.

  17. Clinical trials in CIDP and chronic autoimmune demyelinating polyneuropathies.

    Science.gov (United States)

    Dalakas, Marinos C

    2012-05-01

    The main chronic autoimmune neuropathies include chronic inflammatory demyelinating polyneuropathy (CIDP), multifocal motor neuropathy (MMN), and anti-myelin-associated glycoprotein (MAG) demyelinating neuropathy. On the basis of randomized controlled studies, corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis provide short-term benefits in CIDP. MMN responds only to IVIg. Because in MMN and CIDP, IVIg infusions are required every 3-6 weeks to sustain benefits or long-term remissions, there is a need for "IVIg-sparing" agents. In CIDP, immunosuppressive drugs, such as azathioprine, cyclosporine, methotrexate, mycophenolate, and cyclophosphamide, are used, but controlled trials have not shown that they are effective. Controlled trials have also not shown benefit to any agents in anti-MAG neuropathy. However, clinicians use many immunosuppressive drugs in both settings, but all have potentially serious side effects and are only effective in some patients. Thus, there is a need for new therapies in the inflammatory and paraproteinemic neuropathies. New agents targeting T cells, B cells, and transmigration and transduction molecules are discussed as potential treatment options for new trials. The need for biomarkers that predict therapeutic responses or identify patients with active disease is emphasized, and the search for better scoring tools that capture meaningful changes after response to therapies is highlighted.

  18. Treatment of chronic inflammatory neuropathies

    OpenAIRE

    Schaik, van, I.N.; Eftimov, F.

    2015-01-01

    This thesis focuses on the efficacy of existing and alternative treatments in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) and multifocal motor neuropathy (MMN) and explores predictors of treatment response in patients with CIDP treated with corticosteroids. The efficacy of intravenous immunoglobulin (IVIg) in CIDP and MMN was confirmed in meta-analyses. In CIDP, IVIg efficacy was similar to the efficacy of plasma exchange, prednisolone and intravenous methylprednisolone. ...

  19. Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: case report Polirradiculoneuropatia desmielinizante inflamatória crônica na doença do enxerto contra o hospedeiro após transplante de células hematopoiéticas alogênicas: relato de caso

    OpenAIRE

    Paulo José Lorenzoni; Rosana Herminia Scola; Ana Lucila Moreira Carsten; Ana Paula Trentin; Hélio A.G. Teive; Ricardo Pasquini; Lineu C. Werneck

    2007-01-01

    The chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an unusual but important complication of hematopoietic stem cell transplantation (HSCT) rarely reported to date. We describe a 17-year-old woman with a diagnosis of acute myeloid leukemia due to Fanconi's anemia who was submitted to allogeneic HSCT and developed CIDP as part of graft-versus-host disease. Investigation showed high cerebrospinal fluid protein; electrophysiological studies revealed sensory-motor demyelinatin...

  20. Hereditary And Acquired Chronic Demyelination Neuropathies : A Clinical, electrophysiological And Histopathological Study

    Directory of Open Access Journals (Sweden)

    Menon A

    1999-01-01

    Full Text Available Differentiating hereditary motor sensory neuropathy (HMSN from chronic inflammatory demyelinating polyneuropathy (CIDP is often difficult especially when the disease starts at an early age and has protracted course. This study compares the clinical, electro, physiological and histopathological features of hereditary and acquired chronic demyelinating neuropathies. Records of 26 patients of chronic demyelinating neuropathy who underwent sural nerve biopsy were reviewed; HMSN 9, CIDP 13, chronic relapsing demyelinating polyneuropathy (CRDP-4, Salient features of the HMSN group were: Consanguineous parentage-4, onset in first decade-9, skeletal markers-7, absence of positive sensory symptoms- 7 and clinically thickened nerves-6. None of the patients with acquired neuropathy had skeletal markers, 11 had positive sensory symptoms and only 4 had nerve thickening. Electrophysiological evaluation in 22 motor nerves in the HMSN group revealed: inexcitable nerves -13, prolonged distal latency - 6, slow conduction velocity-8 and prolonged f wave latency-3. The 44 motor nerves in patients with acquired neuropathy showed: inexcitable nerves- 7, prolonged distal latency-35, slow conduction velocity-34, f wave prolongation-30 and conduction block 9. Elevated CSF protein was noticed only in acquired group (77%. Pathologically in HMSN the fibre loss was always diffuse and onion bulb formation was frequent while endoneural edema and inflammatory infiltration were absent in this group. Selection of patients with chronic demyelinating neuropathies for therapeutic modulation needs comprehensive clinical and laboratory evaluation.

  1. Interleukin-10 overexpression promotes Fas-ligand-dependent chronic macrophage-mediated demyelinating polyneuropathy.

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    Dru S Dace

    Full Text Available BACKGROUND: Demyelinating polyneuropathy is a debilitating, poorly understood disease that can exist in acute (Guillain-Barré syndrome or chronic forms. Interleukin-10 (IL-10, although traditionally considered an anti-inflammatory cytokine, has also been implicated in promoting abnormal angiogenesis in the eye and in the pathobiology of autoimmune diseases such as lupus and encephalomyelitis. PRINCIPAL FINDINGS: Overexpression of IL-10 in a transgenic mouse model leads to macrophage-mediated demyelinating polyneuropathy. IL-10 upregulates ICAM-1 within neural tissues, promoting massive macrophage influx, inflammation-induced demyelination, and subsequent loss of neural tissue resulting in muscle weakness and paralysis. The primary insult is to perineural myelin followed by secondary axonal loss. Infiltrating macrophages within the peripheral nerves demonstrate a highly pro-inflammatory signature. Macrophages are central players in the pathophysiology, as in vivo depletion of macrophages using clodronate liposomes reverses the phenotype, including progressive nerve loss and paralysis. Macrophage-mediate demyelination is dependent on Fas-ligand (FasL-mediated Schwann cell death. SIGNIFICANCE: These findings mimic the human disease chronic idiopathic demyelinating polyneuropathy (CIDP and may also promote further understanding of the pathobiology of related conditions such as acute idiopathic demyelinating polyneuropathy (AIDP or Guillain-Barré syndrome.

  2. Polarization of macrophages and microglia in inflammatory demyelination

    Institute of Scientific and Technical Information of China (English)

    Li Cao; Cheng He

    2013-01-01

    Multiple sclerosis (MS) is an autoimmune demyelinating disease of the central nervous system,and microglia and macrophages play important roles in its pathogenesis.The activation of microglia and macrophages accompanies disease development,whereas depletion of these cells significantly decreases disease severity.Microglia and macrophages usually have diverse and plastic phenotypes.Both pro-inflammatory and antiinflammatory microglia and macrophages exist in MS and its animal model,experimental autoimmune encephalomyelitis.The polarization of microglia and macrophages may underlie the differing functional properties that have been reported.In this review,we discuss the responses and polarization of microglia and macrophages in MS,and their effects on its pathogenesis and repair.Harnessing their beneficial effects by modulating their polarization states holds great promise for the treatment of inflammatory demyelinating diseases.

  3. Acute Inflammatory Demyelinating Neuropathy : Immunoglobulin And Immune Complex Profile

    OpenAIRE

    Shripad A; Patil; Taly Arun B; Puttaram Sowbhagya; Rao Shivaji; Menon Ashok; Nair KPS

    2003-01-01

    Serum immunoglobulins (IgG, IgA and IgM) and immune complexes IgG (IcG) were measured in 58 cases of acute inflammatory demyelinating neuropathy, popularly known as Guillian Barre′ syndrome, and in 30 healthy controls using single radial immunodiffusion assay. Immunoglobulin and immune complex levels were significantly elevated in patients as compared to controls. The increased levels of immunoglobulins and immune complexes may contribute to the pathogenesis of the disease and provid...

  4. Supramaximal Stimulus Intensity as a Diagnostic Tool in Chronic Demyelinating Neuropathy.

    Science.gov (United States)

    Parker, Vivien; Warman Chardon, Jodi; Mills, Julie; Goldsmith, Claire; Bourque, Pierre R

    2016-01-01

    Objective. The ability to correctly identify chronic demyelinating neuropathy can have important therapeutic and prognostic significance. The stimulus intensity value required to obtain a supramaximal compound muscle action potential amplitude is a commonly acquired data point that has not been formally assessed as a diagnostic tool in routine nerve conduction studies to identify chronic neuropathies. We postulated that this value was significantly elevated in chronic demyelinating neuropathy. Methods. We retrospectively reviewed electrophysiology laboratory records to compare the stimulus intensity values recorded during median and ulnar motor nerve conduction studies. The groups studied included normal controls (n = 42) and the following diagnostic categories: chronic inflammatory demyelinating neuropathy (CIDP) (n = 20), acquired inflammatory demyelinating neuropathy (AIDP) (n = 13), Charcot Marie Tooth (CMT) type 1 or 4C (n = 15), carpal tunnel syndrome (CTS) (n = 11), and amyotrophic lateral sclerosis (ALS) (n = 18). Results. Supramaximal intensities were significantly higher in patients with CMT (median nerve: 43.4 mA) and CIDP (median nerve: 38.9 mA), whereas values similar to normal controls (median nerve: 25.3 mA) were obtained in ALS, CTS, and AIDP. Conclusions. Supramaximal stimulus intensity may be used as an additional criterion to identify the pathophysiology of neuropathy. We postulate that endoneurial hypertrophic changes may increase electrical impedance and thus the threshold of excitation at nodes of Ranvier. PMID:27413732

  5. Supramaximal Stimulus Intensity as a Diagnostic Tool in Chronic Demyelinating Neuropathy

    Directory of Open Access Journals (Sweden)

    Vivien Parker

    2016-01-01

    Full Text Available Objective. The ability to correctly identify chronic demyelinating neuropathy can have important therapeutic and prognostic significance. The stimulus intensity value required to obtain a supramaximal compound muscle action potential amplitude is a commonly acquired data point that has not been formally assessed as a diagnostic tool in routine nerve conduction studies to identify chronic neuropathies. We postulated that this value was significantly elevated in chronic demyelinating neuropathy. Methods. We retrospectively reviewed electrophysiology laboratory records to compare the stimulus intensity values recorded during median and ulnar motor nerve conduction studies. The groups studied included normal controls (n=42 and the following diagnostic categories: chronic inflammatory demyelinating neuropathy (CIDP (n=20, acquired inflammatory demyelinating neuropathy (AIDP (n=13, Charcot Marie Tooth (CMT type 1 or 4C (n=15, carpal tunnel syndrome (CTS (n=11, and amyotrophic lateral sclerosis (ALS (n=18. Results. Supramaximal intensities were significantly higher in patients with CMT (median nerve: 43.4 mA and CIDP (median nerve: 38.9 mA, whereas values similar to normal controls (median nerve: 25.3 mA were obtained in ALS, CTS, and AIDP. Conclusions. Supramaximal stimulus intensity may be used as an additional criterion to identify the pathophysiology of neuropathy. We postulate that endoneurial hypertrophic changes may increase electrical impedance and thus the threshold of excitation at nodes of Ranvier.

  6. Supramaximal Stimulus Intensity as a Diagnostic Tool in Chronic Demyelinating Neuropathy

    Science.gov (United States)

    Parker, Vivien; Warman Chardon, Jodi; Mills, Julie; Goldsmith, Claire; Bourque, Pierre R.

    2016-01-01

    Objective. The ability to correctly identify chronic demyelinating neuropathy can have important therapeutic and prognostic significance. The stimulus intensity value required to obtain a supramaximal compound muscle action potential amplitude is a commonly acquired data point that has not been formally assessed as a diagnostic tool in routine nerve conduction studies to identify chronic neuropathies. We postulated that this value was significantly elevated in chronic demyelinating neuropathy. Methods. We retrospectively reviewed electrophysiology laboratory records to compare the stimulus intensity values recorded during median and ulnar motor nerve conduction studies. The groups studied included normal controls (n = 42) and the following diagnostic categories: chronic inflammatory demyelinating neuropathy (CIDP) (n = 20), acquired inflammatory demyelinating neuropathy (AIDP) (n = 13), Charcot Marie Tooth (CMT) type 1 or 4C (n = 15), carpal tunnel syndrome (CTS) (n = 11), and amyotrophic lateral sclerosis (ALS) (n = 18). Results. Supramaximal intensities were significantly higher in patients with CMT (median nerve: 43.4 mA) and CIDP (median nerve: 38.9 mA), whereas values similar to normal controls (median nerve: 25.3 mA) were obtained in ALS, CTS, and AIDP. Conclusions. Supramaximal stimulus intensity may be used as an additional criterion to identify the pathophysiology of neuropathy. We postulate that endoneurial hypertrophic changes may increase electrical impedance and thus the threshold of excitation at nodes of Ranvier. PMID:27413732

  7. Supramaximal Stimulus Intensity as a Diagnostic Tool in Chronic Demyelinating Neuropathy

    Science.gov (United States)

    Parker, Vivien; Warman Chardon, Jodi; Mills, Julie; Goldsmith, Claire; Bourque, Pierre R.

    2016-01-01

    Objective. The ability to correctly identify chronic demyelinating neuropathy can have important therapeutic and prognostic significance. The stimulus intensity value required to obtain a supramaximal compound muscle action potential amplitude is a commonly acquired data point that has not been formally assessed as a diagnostic tool in routine nerve conduction studies to identify chronic neuropathies. We postulated that this value was significantly elevated in chronic demyelinating neuropathy. Methods. We retrospectively reviewed electrophysiology laboratory records to compare the stimulus intensity values recorded during median and ulnar motor nerve conduction studies. The groups studied included normal controls (n = 42) and the following diagnostic categories: chronic inflammatory demyelinating neuropathy (CIDP) (n = 20), acquired inflammatory demyelinating neuropathy (AIDP) (n = 13), Charcot Marie Tooth (CMT) type 1 or 4C (n = 15), carpal tunnel syndrome (CTS) (n = 11), and amyotrophic lateral sclerosis (ALS) (n = 18). Results. Supramaximal intensities were significantly higher in patients with CMT (median nerve: 43.4 mA) and CIDP (median nerve: 38.9 mA), whereas values similar to normal controls (median nerve: 25.3 mA) were obtained in ALS, CTS, and AIDP. Conclusions. Supramaximal stimulus intensity may be used as an additional criterion to identify the pathophysiology of neuropathy. We postulate that endoneurial hypertrophic changes may increase electrical impedance and thus the threshold of excitation at nodes of Ranvier.

  8. Chronic inflammatory demyelinating polyradiculoneuropathy: two cases with cervical spinal cord compression Polirradiculoneuropatia desmielinizante inflamatória crônica: dois casos com síndrome de compressão medular

    Directory of Open Access Journals (Sweden)

    Marcos R.G. de Freitas

    2005-09-01

    Full Text Available Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is a peripheral nerve disorder probably due to an immunological disturb. It evolves either in a steadily progressive or in a relapsing and fluctuating course. Weakness is mainly in the lower limbs proximally and distally. The electromyography is demyelinating. The cerebral spinal fluid protein is most of times elevated. Sometimes enlarged nerves are found. There are few cases described with spinal cord compression due to hypertrophic spinal nerve roots. Two patients (females, 66 and 67 years old with diagnosis of a long standing CIDP are described. In the first one, the evolution was characterized by remission and relapsing course. The second patient had a chronic and progressive course. These patients presented after a long evolution a cervical spinal cord compression syndrome due to hypertrophic cervical roots. Neurologists must be aware of the possibility of development of spinal cord compression by enlarged spinal roots in patients with a long standing CIDP.A polirradiculoneuropatia desmielinizante inflamatória crônica (PDIC é uma afecção dos nervos periféricos de natureza autoimune, com evolução por surtos de exacerbação e remissão ou de evolver progressivo. O acometimento motor é predominante, com fraqueza proximal e distal nos membros inferiores. A eletroneuromiografia é do tipo desmielinizante com bloqueio de condução nervosa em dois ou mais nervos. Há aumento de proteínas do líquor. Com a evolução da doença pode haver espessamento dos nervos distal e/ou proximalmente. Excepcionalmente ocorre compressão da medula espinhal em qualquer segmento por raízes próximas hipertrofiadas. Foram estudadas duas mulheres de 66 e 67 anos respectivamente com quadro de PDIC de longa evolução. A primeira tinha evolução por surtos e na segunda o evolver era progressivo. Nos dois casos o espessamento proximal dos nervos provocou síndrome de compressão medular alta

  9. The value of electromyography in differentiating intramedullary tumor from inflammatory demyelinating disease of cervical region

    Institute of Scientific and Technical Information of China (English)

    王红芬

    2014-01-01

    Objective To investigate the value of needle electromyography(EMG)in differentiating intramedullary tumor from inflammatory demyelinating disease of cervical region.Methods Patients hospitalized in the Chinese PLA General Hospital from March 2008 to June 2013 with abnormalities on MRI of cervical vertabra and preliminary diagnosed as intramedullary tumor or inflammatory demyelinating disease of cervical region were enrolled in the

  10. Interferon beta-1a in chronic inflammatory demyelinating polyneuropathy: case report Interferon beta en polineuropatía crónica inflamatoria desmienlinizante: caso clínico

    Directory of Open Access Journals (Sweden)

    Andrés Maria Villa

    2004-09-01

    Full Text Available Chronic inflammatory demyelinating polyneuropathy (CIDP is an acquired immune-mediated neuropathy. It presents with a course of progression which may be slow and steady or step-wise or relapsing. Sensory ataxic polyneuropathy may be the only clinical manifestation of this disease. Treatment with interferon beta1a (INF beta1a has been tried with different results in patients who were refractory to other, more conventional, immunomodulatory therapies. Here we report on a patient who had a relapsing form of pure sensory ataxic CIDP and who failed to respond to intravenous human immunoglobulin. He was put on INF beta1a for 3 years. During this period he suffered no relapses while his condition stabilized.La polineuropatía crónica inflamatoria desmielinizante (PCID es una neuropatía inmuno-mediada, que presenta un curso clínico primariamente progresivo o en forma de recaídas. Las manifestaciones sensoriales pueden ser su unica forma de expresión clínica. El tratamiento con interferon beta 1a (IFN beta1a ha sido ensayado en varias oportunidades, con diferentes respuestas terapéuticas, en pacientes refractarios a las terapias inmunomoduladoras convencionales. Nosotros comunicamos un paciente con una forma ataxica recurrente de PCID, que no respondió al tratamiento con inmunoglobulina endovenosa. Posteriormente fue tratado con IFN beta 1 a por tres años. Durante el período de seguimiento no mostró nuevas recaídas y su cuadro neurológico se estabilizó.

  11. Inflammatory demyelinating polyneuropathy in a haemophiliac associated with human immunodeficiency virus infection, responding to high dose intravenous immunoglobulin.

    OpenAIRE

    Panicker, R.; Bloom, A. L.; Compston, D A

    1988-01-01

    We describe what we believe to be the first case of chronic inflammatory demyelinating polyneuropathy (CIDP) occurring in a haemophiliac infected with the human immunodeficiency virus (HIV), and the first patient to show a clinical response to treatment with high dose i.v. immunoglobulin. A 55 year old, severe haemophiliac, known to be positive for HIV antibody, presented with a short history of motor weakness and variable sensory loss in both lower limbs. Clinical examination, electrophysiol...

  12. Chronic inflammatory demyelinating polyradiculoneuropathy in chronic graft-versus-host disease following allogeneic hematopoietic stem cell transplantation: case report Polirradiculoneuropatia desmielinizante inflamatória crônica na doença do enxerto contra o hospedeiro após transplante de células hematopoiéticas alogênicas: relato de caso

    Directory of Open Access Journals (Sweden)

    Paulo José Lorenzoni

    2007-09-01

    Full Text Available The chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is an unusual but important complication of hematopoietic stem cell transplantation (HSCT rarely reported to date. We describe a 17-year-old woman with a diagnosis of acute myeloid leukemia due to Fanconi's anemia who was submitted to allogeneic HSCT and developed CIDP as part of graft-versus-host disease. Investigation showed high cerebrospinal fluid protein; electrophysiological studies revealed sensory-motor demyelinating polyradiculoneuropathy; muscle and nerve biopsy were compatible with CIDP.A polirradiculoneuropatia desmielinizante inflamatória crônica (CIDP é uma incomum, porém, importante complicação do transplante de células hematopoiéticas (HSCT raramente relatada até a data. Nós descrevemos uma mulher de 17 anos com diagnóstico de leucemia mielóide aguda por anemia de Fanconi que foi submetida à HSCT e desenvolveu CIDP como parte da doença do enxerto contra o hospedeiro. A investigação mostrou elevação na proteína no líquor; estudo eletrofisiológico revelando polirradiculoneuropatia desmielinizante sensitivo-motora; e biópsia de músculo e nervo compatível com CIDP.

  13. Axonal damage in myelin oligodendrocyte glycoprotein peptide-induced experimental autoimmune encephalomyelitis in a C57BL/6 mouse model may be not secondary to inflammatory demyelination

    Institute of Scientific and Technical Information of China (English)

    Boting Gao; Juan Chen; Qiong Wang; Wei Wang; Zhouping Tang

    2011-01-01

    The present study established a chronic experimental autoimmune encephalomyelitis model in C57BL/6 mice induced by myelin oligodendrocyte glycoprotein peptides and complete Freund's adjuvant. Onset latency was 12 days, with an incidence rate of 100%. Neuropathological characteristics included perivascular inflammatory cell infiltration, demyelination, neuronal degeneration, and axonal damage within cerebral and myelic white matter. Electron microscopy revealed swollen mitochondria, complete organ disappearance, and fused or broken myelin sheath structure, which were accompanied by myelin sheath reconstruction. Moreover, axonal damage was not consistent with demyelination distribution, and severity of axonal damage did not correlate with demyelination. Results suggested that axonal damage in an experimental autoimmune encephalomyelitis model is not secondary to inflammatory demyelination.

  14. Progesterone and nestorone promote myelin regeneration in chronic demyelinating lesions of corpus callosum and cerebral cortex.

    Science.gov (United States)

    El-Etr, Martine; Rame, Marion; Boucher, Celine; Ghoumari, Abdel M; Kumar, Narender; Liere, Philippe; Pianos, Antoine; Schumacher, Michael; Sitruk-Ware, Regine

    2015-01-01

    Multiple Sclerosis affects mainly women and consists in intermittent or chronic damages to the myelin sheaths, focal inflammation, and axonal degeneration. Current therapies are limited to immunomodulators and antiinflammatory drugs, but there is no efficient treatment for stimulating the endogenous capacity of myelin repair. Progesterone and synthetic progestins have been shown in animal models of demyelination to attenuate myelin loss, reduce clinical symptoms severity, modulate inflammatory responses and partially reverse the age-dependent decline in remyelination. Moreover, progesterone has been demonstrated to promote myelin formation in organotypic cultures of cerebellar slices. In the present study, we show that progesterone and the synthetic 19-nor-progesterone derivative Nestorone® promote the repair of severe chronic demyelinating lesions induced by feeding cuprizone to female mice for up to 12 weeks. Progesterone and Nestorone increase the density of NG2(+) oligodendrocyte progenitor cells and CA II(+) mature oligodendrocytes and enhance the formation of myelin basic protein (MBP)- and proteolipid protein (PLP)-immunoreactive myelin. However, while demyelination in response to cuprizone was less marked in corpus callosum than in cerebral cortex, remyelination appeared earlier in the former. The remyelinating effect of progesterone was progesterone receptor (PR)-dependent, as it was absent in PR-knockout mice. Progesterone and Nestorone also decreased (but did not suppress) neuroinflammatory responses, specifically astrocyte and microglial cell activation. Therefore, some progestogens are promising therapeutic candidates for promoting the regeneration of myelin.

  15. Idiopathic inflammatory-demyelinating diseases of the central nervous system

    Energy Technology Data Exchange (ETDEWEB)

    Rovira Canellas, A. [Vall d' Hebron University Hospital, Magnetic Resonance Unit (I.D.I.), Department of Radiology, Barcelona (Spain); Rovira Gols, A. [Parc Tauli University Institute - UAB, UDIAT, Diagnostic Centre, Sabadell (Spain); Rio Izquierdo, J.; Tintore Subirana, M.; Montalban Gairin, X. [Vall d' Hebron University Hospital, Neuroimmunology Unit, Department of Neurology, Barcelona (Spain)

    2007-05-15

    Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Balo's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures. (orig.)

  16. Idiopathic inflammatory-demyelinating diseases of the central nervous system

    International Nuclear Information System (INIS)

    Idiopathic inflammatory-demyelinating diseases (IIDDs) include a broad spectrum of central nervous system disorders that can usually be differentiated on the basis of clinical, imaging, laboratory and pathological findings. However, there can be a considerable overlap between at least some of these disorders, leading to misdiagnoses or diagnostic uncertainty. The relapsing-remitting and secondary progressive forms of multiple sclerosis (MS) are the most common IIDDs. Other MS phenotypes include those with a progressive course from onset (primary progressive and progressive relapsing) or with a benign course continuing for years after onset (benign MS). Uncommon forms of IIDDs can be classified clinically into: (1) fulminant or acute IIDDs, such as the Marburg variant of MS, Balo's concentric sclerosis, Schilder's disease, and acute disseminated encephalomyelitis; (2) monosymptomatic IIDDs, such as those involving the spinal cord (transverse myelitis), optic nerve (optic neuritis) or brainstem and cerebellum; and (3) IIDDs with a restricted topographical distribution, including Devic's neuromyelitis optica, recurrent optic neuritis and relapsing transverse myelitis. Other forms of IIDD, which are classified clinically and radiologically as pseudotumoral, can have different forms of presentation and clinical courses. Although some of these uncommon IIDDs are variants of MS, others probably correspond to different entities. MR imaging of the brain and spine is the imaging technique of choice for diagnosing these disorders, and together with the clinical and laboratory findings can accurately classify them. Precise classification of these disorders may have relevant prognostic and treatment implications, and might be helpful in distinguishing them from tumoral or infectious lesions, avoiding unnecessary aggressive diagnostic or therapeutic procedures. (orig.)

  17. 老年人慢性炎症性脱髓鞘性多发性神经病临床病理分析%Clinicopathological analysis of chronic inflammatory demyelinating polyneuropathy in the elderly

    Institute of Scientific and Technical Information of China (English)

    张宁; 李刚; 肖波; 刘运海; 蔡艳; 梁静慧

    2008-01-01

    目的 研究老年人慢性炎症性脱髓鞘性多发性神经病(CIDP)的临床和病理特征.方法对11例老年CIDP患者的临床表现、脑脊液检查、肌电图检查及腓肠神经活检病理结果进行总结分析. 结果 本组患者发病前有上呼吸道感染2例;首发症状为四肢远端麻木无力3例,双下肢远端麻木无力5例.双上肢远端麻木无力2例,抬头困难1例;患者均有运动障碍,伴感觉障碍8例.肢体肌肉萎缩3例,伴有肌肉压痛1例.腱反射减弱或消失8例,颅神经损害5例,自主神经受累3例,累及呼吸肌1例,复发3例;发病高峰期改良Rankin评分平均3.02分;脑脊液检查有明显蛋白细胞分离5例;肌电图示神经原性损害10例,肌原性伴神经原性损害1例;病理检查结果示髓鞘脱失6例,炎性细胞浸润6例,明显髓鞘再生2例,轴索肿胀变性2例;激素治疗8例有效. 结论 老年CIDP患者首发症状多为肢体远端麻木无力;大部分有感觉障碍;可有颅神经和自主神经损害;腓肠神经活检有助于老年CIDP诊断;激素治疗大部分有效.%Objective To study the clinical and pathological features in the elderly patients with chronic inflammatory demyelinating polyneuropathy (CIDP). Methods The features of the clinical manifestation, cerebrospinal fluid, electromyogram(EMG) and the biopsy results of sural nerve were presented and analyzed in 11 elderly patients with CIDP. Results Two cases had history of upper respiratory tract infection before the onset. As the initial symptoms , there were three cases with distal limb numbness, five cases with both distal lower extremities numbness, two cases with both distal upper extremities numbness and one case with difficulties to raise his head. Motor disorder was common to all the patients. There were eight patients with sensory dysfunction, three with limb muscle atrophy, one with muscle tenderness, eight with tendon reflexes weakened or disappeared, five with cranial nerve

  18. 糖尿病合并慢性炎性脱髓鞘性多发性神经病一例报告与文献复习%Diabetes mellitus combined with chronic inflammatory demyelinating polyneuropathy:A case report and ;literature review

    Institute of Scientific and Technical Information of China (English)

    杨茜; 赵志刚; 马跃华; 杨俊朋; 马媛媛

    2015-01-01

    Diabetes mellitus (DM ) combined with chronic inflammatory demyelinating polyneuropathy (CIDP) is rarely occurred and is difficult to distinguish from diabetic peripheral neuropathy (DPN). Here we reported a case of DM‐CIDP who was misdiagnosed as DPN in the initial treatment. Lumbar puncture , electrophysiological and other relevant examinations were helpful to timely and accurately dignose DM‐C ID P.%糖尿病合并慢性炎性脱髓鞘性多发性神经病(DM‐CIDP)临床少见。就诊时,本例误诊为糖尿病周围神经病变(DPN),在治疗过程中完善腰椎穿刺术、神经电生理等相关检查后最终确诊为DM‐CIDP。

  19. Chronic inflammatory systemic diseases

    OpenAIRE

    Straub, Rainer H.; Schradin, Carsten

    2016-01-01

    It has been recognized that during chronic inflammatory systemic diseases (CIDs) maladaptations of the immune, nervous, endocrine and reproductive system occur. Maladaptation leads to disease sequelae in CIDs. The ultimate reason of disease sequelae in CIDs remained unclear because clinicians do not consider bodily energy trade-offs and evolutionary medicine. We review the evolution of physiological supersystems, fitness consequences of genes involved in CIDs during different life-history sta...

  20. Erythromelalgia-like presentation of chronic acquired demyelinating polyneuropathy in a setting of past alcohol abuse.

    Science.gov (United States)

    Chuquilin, Miguel; Dhand, Upinder K

    2016-02-01

    Erythromelalgia may be primary or secondary to an underlying medical condition. Association with small fiber neuropathy and axonal large fiber peripheral neuropathy has been described. Erythromelalgia in the setting of acquired demyelinating neuropathy has not been reported. We report a 52-year-old woman with severe erythromelalgia, pain and burning, progressive weakness, hyporeflexia and distal pan-sensory deficits. Cerebrospinal fluid protein was 219 mg/dL. Nerve conduction study revealed extreme (ten-fold) prolongation of distal motor latencies, markedly slow motor nerve conduction, reduced terminal latency index, reduced distal compound muscle action potential (CMAP) amplitude, possible conduction blocks, and distal denervation. Treatment with intravenous immunoglobulin, prednisone and azathioprine resulted in marked clinical and electrophysiological improvement. Our patient fulfills the diagnostic criteria for chronic inflammatory demyelinating polyneuropathy (CIDP); however, the unique electrodiagnostic features and presentation with erythromelalgia may represent a CIDP variant or a novel dysimmune neuropathy, or may partly be related to neurotoxic effects of prior alcohol abuse. PMID:26804376

  1. Multifocal inflammatory demyelination in a patient with rheumatoid arthritis and treatment complications.

    Science.gov (United States)

    Lu, Jian-Qiang; Ringrose, Jennifer; Gross, Donald; Emery, Derek; Blevins, Gregg; Power, Christopher

    2016-08-15

    Rheumatoid arthritis (RA) and multiple sclerosis (MS) are both autoimmune diseases that share similar pathogenesis, but the development of MS in RA patients without the treatment of anti-tumor necrosis factor-alpha is rarely reported, which might be attributed to the use of other medications with potential immunosuppressive effects in the treatment of RA. Since MS can be clinically silent and autopsy examination of the central nervous system in RA patients is rarely described, the association of MS with RA may be possibly under-recognized. We report an autopsy case revealing multifocal inflammatory demyelination in a RA patient who had a prolonged use of methotrexate and hydroxychloroquine resulting in hydroxychloroquine-induced myopathies and heart failure. The neuropathological features of this case are consistent with MS, although there are some altered inflammatory demyelinating features such as relatively smaller lesions and less infiltration of inflammatory cells, particularly T-cells. Our present case, in combination with literature review, suggests that the RA treatment especially with hydroxychloroquine and methotrexate is likely to alter the characteristics of inflammatory demyelination and disease course. PMID:27423608

  2. Autoimmune Demyelinating Polyneuropathy as a Manifestation of Chronic Graft-versus-Host Disease after Adult Cord Blood Transplantation in a Patient with Chronic Lymphocytic Leukemia

    Directory of Open Access Journals (Sweden)

    Fredrick Hogan

    2014-01-01

    Full Text Available Immune mediated demyelinating disease after allogeneic stem cell transplantation is a rare entity with unclear etiology. Acute inflammatory demyelinating polyneuropathy (AIDP has been reported after related and adult unrelated allogeneic stem cell transplantation but no such case has been reported after unrelated cord blood transplantation. We hereby present the first case of AIDP after double umbilical cord blood transplantation (DUCBT. A 55-year-old man with chronic lymphocytic leukemia (CLL received a cord blood transplant for relapsed refractory disease with high risk cytogenetics. On day 221, patient presented with skin rash, tingling in both lower extremites, and ascending paralysis that progressed rapidly over the course of 2 days. The workup resulted in a diagnosis of AIDP and administration of intravenous immunoglobulins plus steroids was initiated. Motor and sensory powers were fully recovered and his chronic GVHD was managed for several months with single agent sirolimus.

  3. Hyaluronan oligosaccharides perturb lymphocyte slow rolling on brain vascular endothelial cells: Implications for inflammatory demyelinating disease

    OpenAIRE

    Winkler, Clayton W.; Foster, Scott C.; Itakura, Asako; Matsumoto, Steven G.; Asari, Akira; McCarty, Owen J. T.; Sherman, Larry S.

    2013-01-01

    Inflammatory demyelinating diseases like multiple sclerosis are characterized by mononuclear cell infiltration into the central nervous system. The glycosaminoglycan hyaluronan and its receptor, CD44, are implicated in the initiation and progression of a mouse model of multiple sclerosis, experimental autoimmune encephalomyelitis (EAE). Digestion of hyaluronan tethered to brain vascular endothelial cells by a hyaluronidase blocks the slow rolling of lymphocytes along activated brain vascular ...

  4. Human immunodeficiency virus seroconversion presenting with acute inflammatory demyelinating polyneuropathy: a case report

    Directory of Open Access Journals (Sweden)

    Sloan Derek J

    2008-12-01

    Full Text Available Abstract Introduction Acute Human Immunodeficiency Virus infection is associated with a range of neurological conditions. Guillain-Barré syndrome is a rare presentation; acute inflammatory demyelinating polyneuropathy is the commonest form of Guillain-Barré syndrome. Acute inflammatory demyelinating polyneuropathy has occasionally been reported in acute Immunodeficiency Virus infection but little data exists on frequency, management and outcome. Case presentation We describe an episode of Guillain-Barré syndrome presenting as acute inflammatory demyelinating polyneuropathy in a 30-year-old man testing positive for Immunodeficiency Virus, probably during acute seroconversion. Clinical suspicion was confirmed by cerebrospinal fluid analysis and nerve conduction studies. Rapid clinical deterioration prompted intravenous immunoglobulin therapy and early commencement of highly active anti-retroviral therapy. All symptoms resolved within nine weeks. Conclusion Unusual neurological presentations in previously fit patients are an appropriate indication for Immunodeficiency-Virus testing. Highly active anti-retroviral therapy with adequate penetration of the central nervous system should be considered as an early intervention, alongside conventional therapies such as intravenous immunoglobulin.

  5. CD8+ T cells in inflammatory demyelinating disease

    DEFF Research Database (Denmark)

    Weiss, Hanne A; Millward, Jason M; Owens, Trevor

    2007-01-01

    We review the contribution made by CD8+ T cells to inflammation in the central nervous system (CNS) in Multiple Sclerosis (MS), and discuss their role in the animal model Experimental Autoimmune Encephalomyelitis (EAE). We show that the inflammatory cytokines interferon-gamma and interleukin-17...... are differentially regulated in CNS-infiltrating CD4+ and CD8+ T cells in EAE, and that CD8+ T cells regulate disease. In MS, CD8+ T cells appear to play a role in promotion of disease, so cytokine regulation is likely different in CD8+ T cells in MS and EAE...

  6. Accelerated remyelination during inflammatory demyelination prevents axonal loss and improves functional recovery

    Science.gov (United States)

    Mei, Feng; Lehmann-Horn, Klaus; Shen, Yun-An A; Rankin, Kelsey A; Stebbins, Karin J; Lorrain, Daniel S; Pekarek, Kara; A Sagan, Sharon; Xiao, Lan; Teuscher, Cory; von Büdingen, H-Christian; Wess, Jürgen; Lawrence, J Josh; Green, Ari J; Fancy, Stephen PJ; Zamvil, Scott S; Chan, Jonah R

    2016-01-01

    Demyelination in MS disrupts nerve signals and contributes to axon degeneration. While remyelination promises to restore lost function, it remains unclear whether remyelination will prevent axonal loss. Inflammatory demyelination is accompanied by significant neuronal loss in the experimental autoimmune encephalomyelitis (EAE) mouse model and evidence for remyelination in this model is complicated by ongoing inflammation, degeneration and possible remyelination. Demonstrating the functional significance of remyelination necessitates selectively altering the timing of remyelination relative to inflammation and degeneration. We demonstrate accelerated remyelination after EAE induction by direct lineage analysis and hypothesize that newly formed myelin remains stable at the height of inflammation due in part to the absence of MOG expression in immature myelin. Oligodendroglial-specific genetic ablation of the M1 muscarinic receptor, a potent negative regulator of oligodendrocyte differentiation and myelination, results in accelerated remyelination, preventing axonal loss and improving functional recovery. Together our findings demonstrate that accelerated remyelination supports axonal integrity and neuronal function after inflammatory demyelination. DOI: http://dx.doi.org/10.7554/eLife.18246.001 PMID:27671734

  7. Interferon-gamma in progression to chronic demyelination and neurological deficit following acute EAE

    DEFF Research Database (Denmark)

    Renno, T; Taupin, V; Bourbonnière, L;

    1998-01-01

    The cytokine interferon-gamma (IFNgamma) is implicated in the induction of acute CNS inflammation, but it is less clear what role if any IFNgamma plays in progression to chronic demyelination and neurological deficit. To address this issue, we have expressed IFNgamma in myelinating oligodendrocytes....... In contrast to control mice, which remit from EAE with resolution of glial reactivity and leukocytic infiltration, transgenics showed chronic neurological deficits. While activated microglia/macrophages persisted in demyelinating lesions for over 100 days, CD4(+) T lymphocytes were no longer present in CNS...

  8. Acute Inflammatory Demyelinating Polyneuropathy in Children; Clinical and Electrophysiologic Findings

    Directory of Open Access Journals (Sweden)

    Seyed-Hasan Tonekaboni

    2009-03-01

    Full Text Available Objective:The aim of this study was to evaluate the electrophysiologic findings of Guillain Barre Syndrome (GBS in children and their relation with clinical progress of the disease. Methods:Twenty-three children with GBS were evaluated between 2005 and 2007. Electrophysiologic evaluations were performed at admission and one month later. Findings: Five patients needed respirator, 15 were bedridden, 1 developed recurrence 6 months later, and 2 experienced chronic GBS. The most common findings included: decreased amplitude of muscle action potential (CMAP (96%, increased distal latency (74%, increased F wave latency (69%, and decreased nerve conduction velocity (NCV (61%. Sensory nerve conduction (evaluating sural nerve was normal in 78% of the cases. These measures did not significantly change after 1 month. Conclusion:Electrodiagnostic evaluations are helpful at the primary stages of GBS for diagnosis. Fibrillation potentials and positive sharp waves showing denervation and axonal injury are presentative of longer duration of the disease and a worse prognosis.

  9. Cerebellar white matter inflammation and demyelination in chronic relapsing experimental allergic encephalomyelitis

    DEFF Research Database (Denmark)

    Wanscher, B.; Sørensen, P. S.; Juhler, M.;

    1993-01-01

    Experimental allergic encephalomyelitis, demyelination, inflammation, immunology, neuropathology......Experimental allergic encephalomyelitis, demyelination, inflammation, immunology, neuropathology...

  10. 大剂量静脉人免疫球蛋白联合激素治疗慢性格林-巴利综合症效果分析%Curative Effects of High-dose Intravenous Immunoglobulins Combining with Steroids for Chronic Inflammatory Demyelinating Polyneruropathoes

    Institute of Scientific and Technical Information of China (English)

    郭蓉

    2007-01-01

    目的 研究大剂量静脉用人血免疫球蛋白(IVIg)联合类固醇激素与单独应用类固醇激素对慢性炎症性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyneruropathoes,CIDP)的治疗效果对比.方法 CIDP患者共32例,应用IVIg联合糖皮质激素治疗15例设为实验组,年龄17~67岁;仅用糖皮质激素治疗17例,年龄21~69岁,设为对照组.治疗前和治疗后分别测定患者的肌力(Fugl-Meyer运动积分)、日常生活能力(Barthel指数).结果 治疗前后Fugl-Meyer运动积分和Barthel指数的对比发现实验组与对照组比较差异有统计学意义(P<0.01),肌力恢复和日常生活能力恢复程度明显增加.结论 IVIg联合激素治疗CIDP的效果比单独用激素治疗效果要好,且越早越好.

  11. Tumefactive Brain Demyelination Accompanying MADSAM Neuropathy

    Directory of Open Access Journals (Sweden)

    Şefik Evren Erdener

    2015-09-01

    Full Text Available Multifocal acquired demyelinating sensory and motor (MADSAM neuropathy is characterized by asymmetric multifocal motor and sensory loss and conduction blocks in peripheral nerves. Peripheral demyelinating diseases may be accompanied by demyelination in central nervous system (CNS. In this report, a MADSAM patient with a solitary tumefactive demyelinating lesion in brain is presented. Neuroimaging due to a visual field defect revealed a right parietooccipital lesion, which was initially misdiagnosed as a tumor. Pathological examination showed that it was demyelinating in nature. Peripheral nervous symptoms of the patient developed two years later and she was then diagnosed with MADSAM. There was prominent clinical and electrophysiological response to steroid treatment. Tumefactive brain involvement was not previously reported for MADSAM neuropathy, although it was documented in a single case with typical chronic inflammatory demyelinating polyneuropathy (CIDP. CNS involvement should therefore be considered in MADSAM patients.

  12. Electrophysiologic study of chronic inflammatory demyelinating polyneuropathy by using segmental stimulation in the median nerve and ulnar nerve%正中神经和尺神经分段刺激在慢性炎性脱髓鞘性多发性神经病中的电生理研究

    Institute of Scientific and Technical Information of China (English)

    王晋荣; 王进华; 叶憬; 杨伟丽

    2013-01-01

    目的 探讨运动神经传导速度(MCV)、复合肌肉动作电位(CMAP)与肌力减退的关系和传导阻滞(CB)在慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyradiculoneuritis,CIDP)中的表现特点.方法 30例CIDP患者在进行常规MCV、远端潜伏期(DML)、F波、感觉神经传导速度(SCV)、肌电图(EMG)测定的基础上,在正中神经采用由远到近的“腕-肘-腋-Erb's点”4点3段刺激,尺神经采用由远到近的“腕-肘下-肘上-腋-Erb's点”5点4段刺激,记录各段刺激后CMAP各参数及MCV的变化.结果 CMAP波幅衰减、面积衰减、时程增加以及MCV减慢与临床肌力减退无相关性,dCMAP波幅与上肢远端肌力呈正相关;患者中80.00%在正中神经、73.33%在尺神经发现了1个或多个节段的CB,且出现节段无明显选择性.结论 dCMAP波幅降低与CIDP患者肌力减退有相关性.在CIDP中CB出现率高,且较为弥散地在各节段中出现.%Objective To investigate the relationship between motor conduction velocity (MCV) and compound muscle action potential (CMAP) and muscle strength impairment; and to study the characteristics of conduction block (CB) in chronic inflammatory demyelinating polyneuropathy (CIDP). Methods 30 patients with CIDP formed the study population. All patients were examined by MCV, distal motor latency (DML), F wave, sensory nerve conduction velocity (SCV) and electromyography (EMG). Stimulations were perfomed at 4 sites of the median nerve from distal to proximal (wrist, elbow, axilla, Erb' s point) and 5 sites of the ulnar nerve (wrist, below elbow, above elbow, axilla, Erb' s point), while all the parameters of CMAP and MCV were recorded. Results There were no correlations between the CAMP amplitude attenuation, area, duration increase, MCV reduction and the clinical muscle strength. However, there was positive correlation between the amplitude of the dCMAP and the distal muscle strength in the upper

  13. 糖尿病合并慢性炎症性脱髓鞘性多发性神经病-4例临床分析并文献回顾%Diabetic chronic inflammatory demyelinating neuropathy-4 cases of clinical analysis and literature review

    Institute of Scientific and Technical Information of China (English)

    阳柏凤; 文延斌; 李静; 周文斌; 谢仁明

    2014-01-01

    目的 探讨糖尿病(diabetic mellitus,DM)合并慢性炎性脱髓鞘性多发性神经病(chronic inflammatory demyelinating polyneuropathy,CIDP)的临床、电生理特点,并与糖尿病周围神经病(diabetic peripheral neuropathy,DPN)进行早期鉴别诊断.方法 回顾性分析4例DM合并CIDP患者的临床表现、电生理检查及诊疗特征.结果 4例DM合并CIDP患者中,1例仅表现为对称性肢体乏力,其余3例均伴有对称性的麻木或疼痛,仅1例患者伴有颅神经损害;4例患者均存在腱反射均减弱或消失,病程均超过2个月,且均有脑脊液蛋白-细胞分离现象;4例患者肌电图检查均提示脱髓鞘病变为主,使用激素冲击治疗后症状均好转,其中2例复发患者分别采用丙种球蛋白和血浆置换术治疗后症状好转,4例患者目前均恢复良好.结论 当糖尿病患者出现周围神经病变时,早期根据其临床特征及辅助检查,诊断其是否合并CIDP,并对DM合并CIDP患者合理使用免疫抑制治疗效果良好.

  14. Systemic Lupus Erythematosus With Acute Inflammatory Demyelinating Polyneuropathy: A Case Report and Review of the Literature.

    Science.gov (United States)

    Li, Xiangling; Wang, Yanqiang

    2016-07-01

    We recently encountered a patient with acute inflammatory demyelinating polyneuropathy (AIDP) that was associated with systemic lupus erythematosus (SLE). A 34-year-old Chinese female with a 3-year history of SLE presented with acute bilateral leg weakness and paraparesis, and lost the ability to walk 1 day after noticing bilateral leg numbness and pain for 12 days. Physical examination revealed bilateral facial muscle paralysis, muscle strength in the legs with graded 1/5 proximally and 2/5 distally bilaterally and absence of deep tendon reflex in both knees and ankles. Paresthesia was observed in distal limbs with glove and stocking distribution. Cerebrospinal fluid analysis demonstrated albuminocytologic dissociation. Electrophysiologic survey also indicated sensory-motor demyelinating polyneuropathy. The diagnosis of SLE was established based on her initial symptoms including intermittent fevers, hair loss, oral ulcers, malar rash and arthritis affecting the elbow, wrist and hand joints; positive immunologic findings for antinuclear antibody (ANA), anti-DNA antibody, anti-Smith (anti-Sm) antibody, low serum complement levels, and the kidney biopsy specimen showed glomerular mesangial proliferation with focal endothelial cell proliferation (ISN/PPS 2004 classification lupus nephritis, class III). Treatment with intravenous immunoglobulin, methylprednisolone and cyclophosphamide resulted in clinical and electrophysiological improvement. PMID:27298667

  15. 远端潜伏期指数在POEMS综合征和慢性炎症性脱髓鞘性多发性神经根神经病鉴别诊断中的价值研究%Role of Terminal Latency Index in Differentiation between POEMS Syndrome and Chronic Inflammatory Demyelinating Polyradiculoneuropathy

    Institute of Scientific and Technical Information of China (English)

    乔凯; 黄俊; 陈向军; 王毅

    2014-01-01

    Aim To determine the role of terminal latency index (TLI) in differentiation between POEMS syndrome and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Methods Median and ulnar nerve conduction studies including motor conduction velocity (MCV), distal motor latency (DML) and terminal latency index (TLI) of 18 POEMS patients were compared between 58 matched CIDP patients and 30 normal controls. Results In 18 POEMS patients, the average age at evaluation was 51.56±8.77 years old and that of 58 CIDP patients was (46.34±16.38) years old. Except the ulnar terminal latency index in CIDP, POEMS and CIDP patients demonstrated prolonged distal latencies, low conduction velocities and increased terminal latency indexes compared with the normal group. POEMS had reduced conduction velocities and higher terminal latency indexes than CIDP. Increased TLI was found in 55.6%(median nerve) and 52.9%(ulnar nerve) POEMS and that in CIDP patients was 25.9%(median nerve) and 22.4%(ulnar nerve). Decreased TLI was found in 24.1%(median) and 20.7%(ulnar) CIDP patients and none in POEMS. Temporal dispersion (TD) and conduction block (CB) were more often seen in CIDP patients with increased TLI than that in POEMS. Conclusion Compared with CIDP, POEMS showed greater slowing of the intermediate nerve segments and relatively more uniform demyelination. About 1/4 CIDP demonstrated more distal conduction slowing and more TD and CB especially in those with increased TLI. Terminal latency index combined with TD and CB may be helpful in differentiating POEMS from CIDP.%目的:探讨远端潜伏期指数(TLI)在鉴别POEMS综合征和慢性炎症性脱髓鞘性多发性神经根神经病(CIDP)中的应用价值。方法分析18例POEMS综合征(POEMS组)、58例CIDP患者(CIDP组)和30名正常者(对照组)的正中神经和尺神经运动传导参数,包括远端潜伏期、传导速度和TLI。结果 POEMS组与CIDP组正中神经和尺神经传导速度以及TLI存在差

  16. Clinical Implication of Antibody Against Sulfatide in Guillain-Barré Syndrome Associated Chronic Inflammatory Demyelinating Polyradiculoneuropathy%炎性周围神经病患者血清和脑脊液中抗硫脂抗体的临床意义

    Institute of Scientific and Technical Information of China (English)

    吴德云; 李晓光; 郭玉璞; 陈琳

    2003-01-01

    目的通过测定炎性周围神经病患者血清和脑脊液(CSF)中抗硫脂抗体水平,探讨其临床意义和可能的致病机制. 方法应用ELISA法检测30例急性吉兰-巴雷(Guillain-Barré syndrome,GBS)患者、24例慢性吉兰-巴雷(chronic inflammatory demyelinating polyradiculoneuropathy,CIDP)患者血清和CSF中抗硫脂抗体水平. 结果 (1)GBS患者血清中高滴度抗硫脂抗体与疾病组和正常对照组比较差异无显著性 (P>0.05);CSF中IgM-抗硫脂抗体阳性率与各对照组比较差异有极显著性 (P<0.01);(2)CIDP患者血清中高滴度抗硫脂抗体与正常对照组比较差异有显著性 (P<0.05),CSF中IgM-抗硫脂抗体阳性率与各对照组比较差异有显著性(P<0.05);(3)抗硫脂抗体阳性的GBS患者多有主观感觉障碍,差异有显著性(P<0.05);抗硫脂抗体阳性的CIDP患者多为感觉轴索性损害,差异有显著性(P<0.05);(4)轻、重型组GBS患者血清和CSF中抗硫脂抗体水平之间差异无显著性(P>0.05);(5)GBS组、CIDP组血清中抗体水平与配对的CSF中抗体水平无相关性. 结论 (1)GBS患者CSF中IgM-抗硫脂抗体有可能作为感觉神经受累的一项临床辅助参考指标,抗硫脂抗体的水平与疾病的临床严重程度及预后无明显关系;(2)CIDP患者CSF中IgM-抗硫脂抗体可作为感觉轴索型CIDP的临床辅助参考指标.

  17. 异基因造血干细胞移植后慢性炎症性脱髓鞘性多发神经病变一例并文献复习%Chronic inflammatory demyelinating polyneuropathy after allogeneic hematopoietic stem cell transplantation: a case report and review of the literature

    Institute of Scientific and Technical Information of China (English)

    胡凯; 王继军; 万伟; 克晓燕

    2011-01-01

    目的 提高对异基因造血干细胞移植( allo-HSCT)后并发慢性炎症性脱髓鞘性多发神经病变( CIDP)的认识,探讨其临床特点、诊断及治疗.方法 报道1例慢性粒细胞白血病患者allo-HSCT 后发生CIDP的临床和实验室检查特征及治疗经过.结果 患者在移植后发生急性及慢性移植物抗宿主病(GVHD),在第+105天起出现慢性迁延反复的多发部位神经系统症状,以面瘫、四肢肌力减退、排尿困难为主,经多次腰椎穿刺脑脊液检查以及神经电生理检查,除外其他神经系统疾病后诊断为CIDP.经静脉丙种球蛋白、糖皮质激素、免疫抑制剂治疗及功能锻炼,GVHD及CIDP有所改善,但终因长期免疫抑制继发感染而死亡.结论 allo-HSCT后CIDP是一种罕见的、诊治困难的神经系统并发症,为移植相关的多种因素所致,GVHD及免疫系统紊乱是主要原因,应及时诊断,合理治疗.%Objective To study chronic inflammatory demyelinating polyneuropathy (CIDP) after allogeneic hematopoietic stem cell transplantation (allo-HSCT) and the clinical manifestation,diagnosis and treatment.Methods The clinical manifestation,laboratory examination,treatment and outcome of a patient with chronic myeloid leukemia after allo-HSCT were studied.Results Acute and chronic graft-versus-host disease(GVHD) were occurred in the patient followed by chronic multiple nervous system symptoms from +105 day including facioplegia,decreased muscle strength and dysuria.According to clinical manifestation,results of cerebrospinal fluid exam and electroneurophysiology exam,CIDP was diagnosed.The clinical condition was improved after treatment with intravenous immunoglobulin,glucocorticoid, immunosuppressive agents and functional exercises,but the patient died of secondary infection finally.Conclusion CIDP after allo-HSCT is a rare complication of nervous system and difficult to diagnose and treat.Numerous transplant-related causes are probably

  18. 节段性运动神经传导测定在慢性炎性脱髓鞘性多发性神经根神经病和腓骨肌萎缩症1型之间的差异%Difference of segmental motor nerve conduction study between chronic inflammatory demyelinating polyradiculoneuropathy and Clarcot-Marie-Tooth type 1

    Institute of Scientific and Technical Information of China (English)

    刘明生; 崔丽英; 冯新红; 管宇宙; 李本红; 杜华

    2010-01-01

    目的 探讨节段性运动神经传导测定在慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyradiculoneuropathy,CIDP)和腓骨肌萎缩症1型(Charcot-MarieTooth type1,CMT1)鉴别诊断中的价值.方法 收集16例CIDP和13例CMT1患者,进行节段性运动神经传导测定,比较两组远端运动潜伏期、运动神经传导速度,以及近端和远端比较复合肌肉动作电位波幅、面积和时限变化的差异.结果 CIDP和CMT1患者远端运动潜伏期分别为(5.6±3.4)、(9.3±2.1)ms(t=5.347,P=0.000),运动传导速度分别为(31.1±14.3)、(22.2±5.8)m/s(t=6.369,P=0.000),近端和远端比较波幅下降百分比M5o分别为29.7%和4.9%(Z=7.141,P=0.000).在CIDP患者,所有测定神经中40.3%(25/62)远端潜伏期正常,18.1%(26/144)的神经节段传导速度正常,而在CMT1中所有测定神经的远端潜伏期均延长,所有测定节段的传导速度均减慢.在CIDP患者29.2%的神经节段可见传导阻滞或异常波形离散,而在CMT1仅有3.0%的节段可见传导阻滞(x2=20.829,P=0.000).结论 当针对CIDP和CMT1进行鉴别时,如果节段性运动神经传导测定发现传导阻滞和异常波形离散、不同神经节段传导速度下降程度差别较大,可以支持 CIDP的诊断.%Objective to assess the utility of segmental motor nerve conduction study in differential diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy(CIDP)and Charcot-Marie-Tooth type 1(CMT1).Methods A segmental motor nerve conduction study was performed on 16 patients with CIDP and 13 patients with CMT1.Distal motor latency,motor nerve conduction velocity,the changes of amplitude,area and duration of compound motor action potential over conventional segment were compared between the groups.Results Distal motor latency was (5.6±3.4) ms in CIDP and (9.3±2.1) ms in CMT1(t=5.347 P=0.000),motor nerve conduction velocity was (31.1±14.3) m/s in CIDP and(22.2±5.8)m/s(t=6.369,P=0

  19. 慢性炎性脱髓鞘性多发性神经根神经病周围神经细胞免疫与临床研究%An immunopathological study on biopsied sural nerves of chronic inflammatory demyelinating polyradiculoneuropathy(CIDP)

    Institute of Scientific and Technical Information of China (English)

    李放; 贾建平

    2007-01-01

    目的 研究慢性炎性脱髓鞘性多发性神经根神经病(chronic inflammatory demyelinating polyradic-uloneuropathy,CIDP)细胞免疫染色结果与临床、电生理和病理的关系.方法 经周围神经活检确诊的12例CIDP神经活检标本和10例其它神经系统疾病患者的周围神经标本,用免疫组织化学染色的方法标记神经内膜的淋巴细胞、巨噬细胞和表达鼠抗人白细胞DR抗原(HLA-DR)的细胞,并分别计数,比较2组患者阳性细胞数量;分析CIDP患者3种阳性细胞数与临床、电生理和病理的关系.结果 CIDP组与对照组比较,鼠抗人白细胞共同抗原(LCA)单克隆抗体、鼠抗人巨细胞(CD68)单克隆抗体、HDL-DR单克隆抗体的计数均有明显差异,P值分别为0.001、0.006和0.002;CIDP组HLA-DR阳性计数与CD68阳性计数之间有明显差异,P值为0.04,神经内膜水肿的LCA计数和无水肿的LCA计数比较有明显差异,P值为0.03,CD68阳性细胞在感觉神经传导速度减慢、神经纤维中重度减少的患者较相应的亚组有明显增高,且有显著差异,P值均为0.01,HLA-DR阳性计数在神经纤维中重度减少的患者也较相应的亚组有明显增高,有统计学差异,P值为0.01.结论 CIDP患者神经内膜的炎性细胞浸润是较多见的病理特点,并与神经内膜水肿有关,巨噬细胞的浸润与感觉神经传导速度减慢以及神经纤维数量减少有关,病程较长时巨噬细胞和雪旺氏细胞都可能为HLA-Ⅱ类抗原的抗原提呈细胞,雪旺氏细胞可能不仅为抗原提呈细胞,还可能同时参与对髓鞘的吞噬与破坏.

  20. Application of Contact Heat Evoked Potentials in chronic inflammatory demyelinating polyradiculoneuritis%接触性热痛诱发电位在慢性炎性脱髓鞘性多发性周围神经病中的应用

    Institute of Scientific and Technical Information of China (English)

    易敏; 姚源蓉; 谢炳玓

    2011-01-01

    Objective To study the characteristics of nociceptive conduction system in chronic inflammatory demyelinating polyradiculoneuritis (CIDP) hy Contact Heat Evoked Potentials (CHEPs) , to evaluate its application value in the diagnosis of CIDP. Methods Twenty-one patients diagnosed as CIDP and thirty-two heathy controls were included in this study , stimulated by CHEP stimulator. The latency of Cz/N was recorded.The A8fibers of peripheral nerves and N-wave latencies were analyzed and compared, while nervous conduction velocities were tested and the positive rates were compared. Results (1) CHEPs wave eduction rates in control were significantly higher than CIDP group. (2) The VAS scores in CIDP were significantly lower than control group (P < 0.05). (3) The Aδfihers conduction velocities in CIDP were significantly lower than control group (P <0.05). (4) The reduced rates of Aδfiher conduction velocity in upper limbs were lower than lower limbs (P < 0.05 ).(5) N-wave latencies of upper proximal and lower proximal limbs in CIDP group were not signiricantly different from control group (P > 0.05) , while N-wave latencies of upper distal and lower distal limbs in CIDP group were significantly longer than control group (P < 0.05). (6) The abnormality rates of Aδfibers in CHEPs were higher than MCV and SCV (P < 0.05). Conclusions CHEPs can present the ahnormal regions and damaged degree of nociceptive conduction system in CIDP patients and is more sensitive than traditional nervous conduction velocities. CHEPs may be used as an electrophysiology reference index for the clinical diagnosis of CIDP.%目的:应用接触性热痛诱发电位(CHEPs)技术研究慢性炎性脱髓鞘性多发性周围神经病(CIDP)患者的痛觉传导通路病变特点,探讨CHEPs在CIDP诊断中的应用价值.方法:选取确诊为CIDP的患者21例及对照组32例,应用CHEP刺激器进行刺激,记录Cz/N的潜伏期,分析比较外周神经Aδ纤维及N波峰潜伏期,同时

  1. Hypertrophic osteoarthropathy of chronic inflammatory bowel disease

    Energy Technology Data Exchange (ETDEWEB)

    Oppenheimer, D.A.; Jones, H.H.

    1982-12-01

    The case of a 14-year old girl with painful periostitis and ulcerative colitis is reported. The association of chronic inflammatory bowel disease with osteoarthropathy is rare and has previously been reported in eight patients. The periosteal reaction found in association with inflammatory bowel disease is apparently related to a chronic disease course and may cause extreme localized pain.

  2. [Chronic inflammatory bowel diseases in cats].

    Science.gov (United States)

    Ghermai, A K

    1989-01-01

    The aetiology of chronic idiopathic intestinal inflammation is unknown. It is characterized by a diffuse infiltration with inflammatory cells into the intestinal mucosa and sometimes submucosa. Cats with chronic intermittent vomiting and diarrhoea, later on accompanied by anorexia and weight loss, are presented. Definitive diagnosis can be obtained by intestinal biopsy only. An immune pathogenesis is suspected, which is supported by the fact, that chronic inflammatory bowel disease responds to steroid therapy.

  3. Sildenafil (Viagra Protective Effects on Neuroinflammation: The Role of iNOS/NO System in an Inflammatory Demyelination Model

    Directory of Open Access Journals (Sweden)

    Catarina Raposo

    2013-01-01

    Full Text Available We recently demonstrated that sildenafil reduces the expression of cytokines, COX-2, and GFAP in a demyelinating model induced in wild-type (WT mice. Herein, the understandings of the neuroprotective effect of sildenafil and the mediation of iNOS/NO system on inflammatory demyelination induced by cuprizone were investigated. The cerebella of iNOS−/− mice were examined after four weeks of treatment with cuprizone alone or combined with sildenafil. Cuprizone increased GFAP, Iba-1, TNF-α, COX-2, IL-1β, and IFN-γ expression, decreased expression of glutathione S-transferase pi (GSTpi, and damaged myelin in iNOS−/− mice. Sildenafil reduced Iba-1, IFN-γ, and IL-1β levels but had no effect on the expression of GFAP, TNF-α, and COX-2 compared to the cuprizone group. Sildenafil elevated GSTpi levels and improved the myelin structure/ultrastructure. iNOS−/− mice suffered from severe inflammation following treatment with cuprizone, while WT mice had milder inflammation, as found in the previous study. It is possible that inflammatory regulation through iNOS-feedback is absent in iNOS−/− mice, making them more susceptible to inflammation. Sildenafil has at least a partial anti-inflammatory effect through iNOS inhibition, as its effect on iNOS−/− mice was limited. Further studies are required to explain the underlying mechanism of the sildenafil effects.

  4. Sildenafil (Viagra) Protective Effects on Neuroinflammation: The Role of iNOS/NO System in an Inflammatory Demyelination Model

    Science.gov (United States)

    Raposo, Catarina; Nunes, Ana Karolina de Santana; Luna, Rayana Leal de Almeida; Araújo, Shyrlene Meiry da Rocha; da Cruz-Höfling, Maria Alice; Peixoto, Christina Alves

    2013-01-01

    We recently demonstrated that sildenafil reduces the expression of cytokines, COX-2, and GFAP in a demyelinating model induced in wild-type (WT) mice. Herein, the understandings of the neuroprotective effect of sildenafil and the mediation of iNOS/NO system on inflammatory demyelination induced by cuprizone were investigated. The cerebella of iNOS−/− mice were examined after four weeks of treatment with cuprizone alone or combined with sildenafil. Cuprizone increased GFAP, Iba-1, TNF-α, COX-2, IL-1β, and IFN-γ expression, decreased expression of glutathione S-transferase pi (GSTpi), and damaged myelin in iNOS−/− mice. Sildenafil reduced Iba-1, IFN-γ, and IL-1β levels but had no effect on the expression of GFAP, TNF-α, and COX-2 compared to the cuprizone group. Sildenafil elevated GSTpi levels and improved the myelin structure/ultrastructure. iNOS−/− mice suffered from severe inflammation following treatment with cuprizone, while WT mice had milder inflammation, as found in the previous study. It is possible that inflammatory regulation through iNOS-feedback is absent in iNOS−/− mice, making them more susceptible to inflammation. Sildenafil has at least a partial anti-inflammatory effect through iNOS inhibition, as its effect on iNOS−/− mice was limited. Further studies are required to explain the underlying mechanism of the sildenafil effects. PMID:23970812

  5. IFN-beta gene deletion leads to augmented and chronic demyelinating experimental autoimmune encephalomyelitis

    DEFF Research Database (Denmark)

    Teige, Ingrid; Treschow, Alexandra; Teige, Anna;

    2003-01-01

    Since the basic mechanisms behind the beneficial effects of IFN-beta in multiple sclerosis (MS) patients are still obscure, here we have investigated the effects of IFN-beta gene disruption on the commonly used animal model for MS, experimental autoimmune encephalomyelitis (EAE). We show that IFN......-beta knockout (KO) mice are more susceptible to EAE than their wild-type (wt) littermates; they develop more severe and chronic neurological symptoms with more extensive CNS inflammation and demyelination. However, there was no discrepancy observed between wt and KO mice regarding the capacity of T cells...... to proliferate or produce IFN-gamma in response to recall Ag. Consequently, we addressed the effect of IFN-beta on encephalitogenic T cell development and the disease initiation phase by passive transfer of autoreactive T cells from KO or wt littermates to both groups of mice. Interestingly, IFN-beta KO mice...

  6. The neuregulin, glial growth factor 2, diminishes autoimmune demyelination and enhances remyelination in a chronic relapsing model for multiple sclerosis

    OpenAIRE

    Cannella, Barbara; Hoban, Carolyn J; Gao, Yan-Ling; Garcia-Arenas, Renee; Lawson, Deborah; Marchionni, Mark; Gwynne, David; Raine, Cedric S.

    1998-01-01

    Glial growth factor 2 (GGF2) is a neuronal signal that promotes the proliferation and survival of the oligodendrocyte, the myelinating cell of the central nervous system (CNS). The present study examined whether recombinant human GGF2 (rhGGF2) could effect clinical recovery and repair to damaged myelin in chronic relapsing experimental autoimmune encephalomyelitis (EAE) in the mouse, a major animal model for the human demyelinating disease, multiple sclerosis. Mice with EAE were treated with ...

  7. Endothelial Dysfunction in Chronic Inflammatory Diseases

    Directory of Open Access Journals (Sweden)

    Curtis M. Steyers

    2014-06-01

    Full Text Available Chronic inflammatory diseases are associated with accelerated atherosclerosis and increased risk of cardiovascular diseases (CVD. As the pathogenesis of atherosclerosis is increasingly recognized as an inflammatory process, similarities between atherosclerosis and systemic inflammatory diseases such as rheumatoid arthritis, inflammatory bowel diseases, lupus, psoriasis, spondyloarthritis and others have become a topic of interest. Endothelial dysfunction represents a key step in the initiation and maintenance of atherosclerosis and may serve as a marker for future risk of cardiovascular events. Patients with chronic inflammatory diseases manifest endothelial dysfunction, often early in the course of the disease. Therefore, mechanisms linking systemic inflammatory diseases and atherosclerosis may be best understood at the level of the endothelium. Multiple factors, including circulating inflammatory cytokines, TNF-α (tumor necrosis factor-α, reactive oxygen species, oxidized LDL (low density lipoprotein, autoantibodies and traditional risk factors directly and indirectly activate endothelial cells, leading to impaired vascular relaxation, increased leukocyte adhesion, increased endothelial permeability and generation of a pro-thrombotic state. Pharmacologic agents directed against TNF-α-mediated inflammation may decrease the risk of endothelial dysfunction and cardiovascular disease in these patients. Understanding the precise mechanisms driving endothelial dysfunction in patients with systemic inflammatory diseases may help elucidate the pathogenesis of atherosclerosis in the general population.

  8. Patient with neuromyelitis optica and inflammatory demyelinating lesions comprising whole spinal cord from C2 level till conus: case report

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    Pavlisa Goran

    2009-10-01

    Full Text Available Abstract Background Neuromyelitis optica (NMO is an idiopathic, severe, inflammatory demyelinating disease of the central nervous system, that causes severe optic neuritis and myelitis attacks. Early discrimination between multiple sclerosis (MS and NMO is important, as optimum treatment for both diseases may differ considerably. Case Presentation We report a case of a patient who initially presented as longitudinally extensive transverse myelitis (LETM, having spastic upper extremities diparesis and spastic paraplegia, C2/C3 sensory level and urinary incontinence, as well as extensive inflammatory spinal cord lesions from C2 level to conus. After 5 months the patient had another attack of transverse myelitis, had electrophysiological findings consistent with optic neuritis, was seropositive for NMO-IgG (aquaporin-4 IgG and thus fulfilled NMO diagnostic criteria. Following treatment of disease attacks with pulse corticosteroid therapy and intravenous immunoglobulins, we included oral azathioprine in a combination with oral prednisone in the therapy. Since there was no significant clinical improvement, we decided to use cyclophosphamide therapy, which resulted in good clinical improvement and gradual decrease of cord swelling. Conclusion In this NMO case report we wanted to emphasize the extensiveness of inflammatory spinal cord changes in our patient, from C2 level to conus. In the conclusion it is important to say that accurate, early diagnosis and distinction from MS is critical to facilitate initiation of immunosuppressive therapy for attack prevention.

  9. 神经超声在Chacot-Marie-Tooth1型和慢性炎性脱髓鞘性多发性神经根神经病鉴别诊断中的价值%The utility of peripheral nerve ultrasound in differentiating Charcot-Marie-Tooth type 1 from chronic inflammatory demyelinating polyradiculoneuropathy

    Institute of Scientific and Technical Information of China (English)

    刘明生; 牛婧雯; 李亦; 吴双; 管宇宙; 崔丽英

    2016-01-01

    CharcotMarie-Tooth type 1 (CMT1) from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Methods Eighteen patients with CIDP,13 patients with CMT1 and 16 healthy controls were recruited prospectively from Peking Union Medical College Hospital between January 2014 and July 2015 for this study.Ultrasonographic tests were performed via nerve tracing from wrist to axilla on median and ulnar nerve with a 10 MHz linear array probe.The cross sectional areas (CSAs) were measured at 10 defined sites of the nerves,respectively.Results CSAs (mm2) at all sites of median nerve were significantly increased in CMT1 than in CIDP (10.5 ±5.3 vs7.8 ±2.4,10.9 ±3.6 vs 6.8 ±1.9,11.5 ±5.0 vs7.3 ±1.8,13.5 ± 4.4vs7.2±2.5,16.0±4.5vs7.2±2.1,17.1±5.1vs7.0±2.8,21.0±4.5vs9.5±4.8,24.3±6.9 vs 9.5 ±4.3,23.9 ±6.0 vs 10.2 ±4.3,22.4 ±6.7 vs 9.8 ±2.1;t=2.141,4.766,2.935,4.858,6.715,6.602,7.148,7.100,8.078,6.498,respectively,all P < 0.05).CSAs (mm2) at all sites of ulnar nerve were significantly increased in CMT1 than in CIDP (7.9 ± 1.8 vs 4.0 ± 1.3,8.9 ± 2.0 vs 4.9 ± 1.3,13.5±1.9 vs6.5±2.4,15.0±4.3 vs 6.5 ±1.5,15.8 ±4.4 vs 6.8 ±3.3,11.6±2.3 vs6.9± 3.1,10.2±3.2vs7.6±2.8,14.0±3.0vs6.6±2.1,19.2±3.7vs7.6±4.4,18.1±3.6vs6.3± 2.5;t =7.652,7.414,9.194,6.893,6.443,4.766,2.561,7.897,8.113,11.554,respectively,all P < 0.05).CSAs at 8 sites of median nerve and 8 sites of ulnar nerve were significantly increased in CIDP than in healthy controls.Receiver operation characteristic curve analysis revealed that CSA was suited for differentiating CMT1 from CIDP,and the area under curve in 8 sites of median nerve and 9 sites in ulnar nerve was more than 0.9.Conclusions CSAs measured at different sites by peripheral nerve ultrasound in CMT1 were significantly increased than in CIDP.Measurement of CSAs by peripheral nerve ultrasound can be used for differentiating CMTI from CIDP.

  10. Aquaporin-4 Immuneglobulin G testing in 36 consecutive Jamaican patients with inflammatory central nervous system demyelinating disease

    Directory of Open Access Journals (Sweden)

    Sherri Sandy

    2014-08-01

    Full Text Available Epidemiological studies of neuromyelitis optica (NMO in Jamaica are lacking. Here we reviewed the clinical records of 700 patients undergoing neurological evaluation at the Kingston Public Hospital, the largest tertiary institution in Jamaica over a 4 month period. We investigated the diagnostic utility of Aquaporin-4 ImmuneglobulinG (AQP4-IgG testing in 36 consecutive patients with a diagnosis of an inflammatory demyelinating disorder (IDD of the central nervous system (CNS. Patients were classified into 3 categories: i NMO, n=10; ii multiple sclerosis (MS, n=14 and iii unclassified IDD (n=12. All sera were tested for AQP-IgG status by cell binding assay (Euroimmun. No MS cases were positive. Ninety per cent of NMO cases were positive. Four of 12 patients with unclassified IDD tested positive for AQP4-IgG. AQP4-IgG seropositivity was associated with a lower socioeconomic status, higher EDSS (P=0.04 and lower pulmonary function than the seronegative cases (P=0.007. Aquaporin-4 autoimmunity may account for a significant proportion of Jamaican CNS IDDs.

  11. Selective accumulation of pro-inflammatory T cells in the intestine contributes to the resistance to autoimmune demyelinating disease.

    Directory of Open Access Journals (Sweden)

    Kerstin Berer

    Full Text Available Myelin-specific, pro-inflammatory TH17 cells are widely regarded as the drivers of experimental autoimmune encephalomyelitis (EAE, an animal model for Multiple sclerosis (MS. The factors, responsible for the generation and maintenance of TH17 cells as well as their participation in the pathogenic cascade leading to the demyelinating disease, have been studied extensively. However, how these harmful autoreactive cells are controlled in vivo remains unclear. By comparing TCR transgenic mice on a disease susceptible and a disease resistant genetic background, we show here that pathogenic TH17 cells are sequestered within the intestine of spontaneous EAE resistant B10.S mice. Disease resistant B10.S mice harbored higher frequencies of TH17 cells in the intestine compared to EAE susceptible SJL/J mice. Moreover, transferred TH17 cells selectively migrated to intestinal lymphoid organs of B10.S mice. The sequestration of TH17 cells in the gut was partially dependent on the gut homing receptor α4β7-mediated adhesion to the intestine. Administration of α4β7 blocking-antibodies increased the peripheral availability of TH17 cells, resulting in increased EAE severity after immunization in B10.S mice. Together, these results support the concept that the intestine is a check-point for controlling pathogenic, organ-specific T cells.

  12. Chronic progressive external ophthalmoplegia with inflammatory myopathy.

    Science.gov (United States)

    Chen, Ting; Pu, Chuanqiang; Shi, Qiang; Wang, Qian; Cong, Lu; Liu, Jiexiao; Luo, Hongyu; Fei, Lingna; Tang, Wei; Yu, Shanshan

    2014-01-01

    Chronic progressive external ophthalmoplegia is one of mitochondrial disorders, characterized by ptosis, limitation of eye movement, variably severe bulbar muscle weakness and proximal limb weakness. Chronic progressive external ophthalmoplegia complicated with acquired disease is extremely rare. We report a 44 years old male patient with more than 20 years of chronic progressive bilateral ptosis and limitation of eye movements manifested dysarthria, dysphagia and neck muscle weakness for 3 years. The first muscle biopsy showed red-ragged fibers and cytochrome c oxidase negative fibers as well as inflammatory cells infiltration. Electron microscopy revealed paracrystalline inclusions. Mitochondrial genetic analysis demonstrated a large-scale mtDNA deletion of m.8470_13446del4977. The patient was treated with prednisone. In a three-year follow-up study, the second biopsy was performed. Before the treatment, except bilateral ptosis and external ophthalmopelgia, this patient presented bulbar muscle weakness and neck muscle weakness. After treated with prednisone, the symptoms of dysphagia, dysarthria and neck muscle weakness were significantly improved, and the second biopsy showed only mitochondrial myopathy pathology but the inflammations disappeared. Here, we report a patient with chronic progressive external ophthalmoplegia complicated with inflammatory myopathy and after treated with prednisone as myositis, he had a significant therapeutic effect. PMID:25674260

  13. Transcriptional changes in canine distemper virus-induced demyelinating leukoencephalitis favor a biphasic mode of demyelination.

    Directory of Open Access Journals (Sweden)

    Reiner Ulrich

    Full Text Available Canine distemper virus (CDV-induced demyelinating leukoencephalitis in dogs (Canis familiaris is suggested to represent a naturally occurring translational model for subacute sclerosing panencephalitis and multiple sclerosis in humans. The aim of this study was a hypothesis-free microarray analysis of the transcriptional changes within cerebellar specimens of five cases of acute, six cases of subacute demyelinating, and three cases of chronic demyelinating and inflammatory CDV leukoencephalitis as compared to twelve non-infected control dogs. Frozen cerebellar specimens were used for analysis of histopathological changes including demyelination, transcriptional changes employing microarrays, and presence of CDV nucleoprotein RNA and protein using microarrays, RT-qPCR and immunohistochemistry. Microarray analysis revealed 780 differentially expressed probe sets. The dominating change was an up-regulation of genes related to the innate and the humoral immune response, and less distinct the cytotoxic T-cell-mediated immune response in all subtypes of CDV leukoencephalitis as compared to controls. Multiple myelin genes including myelin basic protein and proteolipid protein displayed a selective down-regulation in subacute CDV leukoencephalitis, suggestive of an oligodendrocyte dystrophy. In contrast, a marked up-regulation of multiple immunoglobulin-like expressed sequence tags and the delta polypeptide of the CD3 antigen was observed in chronic CDV leukoencephalitis, in agreement with the hypothesis of an immune-mediated demyelination in the late inflammatory phase of the disease. Analysis of pathways intimately linked to demyelination as determined by morphometry employing correlation-based Gene Set Enrichment Analysis highlighted the pathomechanistic importance of up-regulated genes comprised by the gene ontology terms "viral replication" and "humoral immune response" as well as down-regulated genes functionally related to "metabolite and energy

  14. Intraventricular injections of mesenchymal stem cells activate endogenous functional remyelination in a chronic demyelinating murine model

    Science.gov (United States)

    Cruz-Martinez, P; González-Granero, S; Molina-Navarro, M M; Pacheco-Torres, J; García-Verdugo, J M; Geijo-Barrientos, E; Jones, J; Martinez, S

    2016-01-01

    Current treatments for demyelinating diseases are generally only capable of ameliorating the symptoms, with little to no effect in decreasing myelin loss nor promoting functional recovery. Mesenchymal stem cells (MSCs) have been shown by many researchers to be a potential therapeutic tool in treating various neurodegenerative diseases, including demyelinating disorders. However, in the majority of the cases, the effect was only observed locally, in the area surrounding the graft. Thus, in order to achieve general remyelination in various brain structures simultaneously, bone marrow-derived MSCs were transplanted into the lateral ventricles (LVs) of the cuprizone murine model. In this manner, the cells may secrete soluble factors into the cerebrospinal fluid (CSF) and boost the endogenous oligodendrogenic potential of the subventricular zone (SVZ). As a result, oligodendrocyte progenitor cells (OPCs) were recruited within the corpus callosum (CC) over time, correlating with an increased myelin content. Electrophysiological studies, together with electron microscopy (EM) analysis, indicated that the newly formed myelin correctly enveloped the demyelinated axons and increased signal transduction through the CC. Moreover, increased neural stem progenitor cell (NSPC) proliferation was observed in the SVZ, possibly due to the tropic factors released by the MSCs. In conclusion, the findings of this study revealed that intraventricular injections of MSCs is a feasible method to elicit a paracrine effect in the oligodendrogenic niche of the SVZ, which is prone to respond to the factors secreted into the CSF and therefore promoting oligodendrogenesis and functional remyelination. PMID:27171265

  15. CNS expression of B7-H1 regulates pro-inflammatory cytokine production and alters severity of Theiler's virus-induced demyelinating disease.

    Directory of Open Access Journals (Sweden)

    D'Anne S Duncan

    Full Text Available The CNS is a unique organ due to its limited capacity for immune surveillance. As macrophages of the CNS, microglia represent a population originally known for the ability to assist neuronal stability, are now appreciated for their role in initiating and regulating immune responses in the brain. Theiler's murine encephalomyelitis virus (TMEV-induced demyelinating disease is a mouse model of multiple sclerosis (MS. In response to TMEV infection in vitro, microglia produce high levels of inflammatory cytokines and chemokines, and are efficient antigen-presenting cells (APCs for activating CD4(+ T cells. However, the regulatory function of microglia and other CNS-infiltrating APCs in response to TMEV in vivo remains unclear. Here we demonstrate that microglia increase expression of proliferating cell nuclear antigen (PCNA, and phenotypically express high levels of major histocompatibility complex (MHC-Class I and II in response to acute infection with TMEV in SJL/J mice. Microglia increase expression of the inhibitory co-stimulatory molecule, B7-H1 as early as day 5 post-infection, while CNS-infiltrating CD11b(+CD11c(-CD45(HIGH monocytes/macrophages and CD11b(+CD11c(+CD45(HIGH dendritic cells upregulate expression of B7-H1 by day 3 post-infection. Utilizing a neutralizing antibody, we demonstrate that B7-H1 negatively regulates TMEV-specific ex vivo production of interferon (IFN-γ, interleukin (IL-17, IL-10, and IL-2 from CD4(+ and CD8(+ T cells. In vivo blockade of B7-H1 in SJL/J mice significantly exacerbates clinical disease symptoms during the chronic autoimmune stage of TMEV-IDD, but only has minimal effects on viral clearance. Collectively, these results suggest that CNS expression of B7-H1 regulates activation of TMEV-specific T cells, which affects protection against TMEV-IDD.

  16. Mechanisms of action of IVIg and therapeutic considerations in the treatment of acute and chronic demyelinating neuropathies.

    Science.gov (United States)

    Dalakas, Marinos C

    2002-12-24

    Intravenous immunoglobulin (IVIg) is an immunomodulating agent that has multiple activities, including modulation of complement activation products, suppressing idiotypic antibody, saturating Fc receptors on macrophages, and suppressing various inflammatory mediators including cytokines, chemokines, and metalloproteinases. Because all these factors are implicated to various degrees in the pathogenesis of immune-mediated demyelination of the PNS, administration of IVIg could be beneficial in treating neuropathies by suppressing the immune-mediated processes that are directed against myelin or axonal antigenic targets. This article outlines the actions of IVIg in CIDP and other autoimmune neuropathies based on data derived from in vivo and in vitro studies. The predominant mechanisms by which IVIg exerts its action on these neuropathies appear to be a combined effect on complement inactivation, neutralization of idiotypic antibodies, cytokine inhibition, and saturation of Fc receptors on endoneurial macrophages.

  17. Increased severity of experimental autoimmune encephalomyelitis, chronic macrophage/microglial reactivity, and demyelination in transgenic mice producing tumor necrosis factor-alpha in the central nervous system

    DEFF Research Database (Denmark)

    Taupin, V; Renno, T; Bourbonnière, L;

    1997-01-01

    are a target of immune attack. TNF-alpha also regulates macrophage activity which could contribute to autoimmune inflammation. We have expressed TNF-alpha at disease-equivalent levels in the central nervous system of transgenic mice, using a myelin basic protein (MBP) promoter. These mice were normal....../microglial reactivity was evident in demyelinating lesions in spinal cord, but T cells were not detected during chronic disease. The participation of TNF-alpha in the demyelinating process is thus more probably due to the perpetuation of macrophage/microglial activation than to direct cytotoxicity of myelin...

  18. Quantifying Demyelination in NK venom treated nerve using its electric circuit model.

    Science.gov (United States)

    Das, H K; Das, D; Doley, R; Sahu, P P

    2016-01-01

    Reduction of myelin in peripheral nerve causes critical demyelinating diseases such as chronic inflammatory demyelinating polyneuropathy, Guillain-Barre syndrome, etc. Clinical monitoring of these diseases requires rapid and non-invasive quantification of demyelination. Here we have developed formulation of nerve conduction velocity (NCV) in terms of demyelination considering electric circuit model of a nerve having bundle of axons for its quantification from NCV measurements. This approach has been validated and demonstrated with toad nerve model treated with crude Naja kaouthia (NK) venom and also shows the effect of Phospholipase A2 and three finger neurotoxin from NK-venom on peripheral nerve. This opens future scope for non-invasive clinical measurement of demyelination. PMID:26932543

  19. Quantifying Demyelination in NK venom treated nerve using its electric circuit model

    Science.gov (United States)

    Das, H. K.; Das, D.; Doley, R.; Sahu, P. P.

    2016-03-01

    Reduction of myelin in peripheral nerve causes critical demyelinating diseases such as chronic inflammatory demyelinating polyneuropathy, Guillain-Barre syndrome, etc. Clinical monitoring of these diseases requires rapid and non-invasive quantification of demyelination. Here we have developed formulation of nerve conduction velocity (NCV) in terms of demyelination considering electric circuit model of a nerve having bundle of axons for its quantification from NCV measurements. This approach has been validated and demonstrated with toad nerve model treated with crude Naja kaouthia (NK) venom and also shows the effect of Phospholipase A2 and three finger neurotoxin from NK-venom on peripheral nerve. This opens future scope for non-invasive clinical measurement of demyelination.

  20. Head mass in chronic pancreatitis: Inflammatory ormalignant

    Institute of Scientific and Technical Information of China (English)

    Amit K Dutta; Ashok Chacko

    2015-01-01

    Chronic pancreatitis increases the risk of developingpancreatic cancer. This often presents as a mass lesionin the head of pancreas. Mass lesion in the head ofpancreas can also occur secondary to an inflammatorylesion. Recognising this is crucial to avoid unnecessarysurgery. This is sometimes difficult as there is an overlapin clinical presentation and conventional computedtomography (CT) abdomen findings in inflammatory andmalignant mass. Advances in imaging technologies likeendoscopic ultrasound in conjunction with techniqueslike fine needle aspiration, contrast enhancement andelastography as well as multidetector row CT, magneticresonance imaging and positron emission tomographyscanning have been shown to help in distinguishinginflammatory and malignant mass. Research is ongoingto develop molecular techniques to help characterisefocal pancreatic mass lesions. This paper reviews thecurrent status of imaging and molecular techniquesin differentiating a benign mass lesion in chronicpancreatitis and from malignancy.

  1. Machine learning approach identifies new pathways associated with demyelination in a viral model of multiple sclerosis

    Science.gov (United States)

    Ulrich, Reiner; Kalkuhl, Arno; Deschl, Ulrich; Baumgärtner, Wolfgang

    2010-01-01

    Abstract Theiler’s murine encephalomyelitis is an experimentally virus-induced inflammatory demyelinating disease of the spinal cord, displaying clinical and pathological similarities to chronic progressive multiple sclerosis. The aim of this study was to identify pathways associated with chronic demyelination using an assumption-free combined microarray and immunohistology approach. Movement control as determined by rotarod assay significantly worsened in Theiler’s murine encephalomyelitis -virus-infected SJL/J mice from 42 to 196 days after infection (dpi). In the spinal cords, inflammatory changes were detected 14 to 196 dpi, and demyelination progressively increased from 42 to 196 dpi. Microarray analysis revealed 1001 differentially expressed genes over the study period. The dominating changes as revealed by k-means and functional annotation clustering included up-regulations related to intrathecal antibody production and antigen processing and presentation via major histocompatibility class II molecules. A random forest machine learning algorithm revealed that down-regulated lipid and cholesterol biosynthesis, differentially expressed neurite morphogenesis and up-regulated toll-like receptor-4-induced pathways were intimately associated with demyelination as measured by immunohistology. Conclusively, although transcriptional changes were dominated by the adaptive immune response, the main pathways associated with demyelination included up-regulation of toll-like receptor 4 and down-regulation of cholesterol biosynthesis. Cholesterol biosynthesis is a rate limiting step of myelination and its down-regulation is suggested to be involved in chronic demyelination by an inhibition of remyelination. PMID:19183246

  2. Osmotic demyelination syndrome in a normonatremic patient of chronic kidney disease

    Directory of Open Access Journals (Sweden)

    Atul Abhishek Jha

    2014-01-01

    Full Text Available Osmotic Demyelination Syndrome (ODS is associated with rapid correction of hyponatremia or fluid shifts, and is characterized by neurological involvement related to pons, brainstem or other areas of the brain. All possible measures should be taken to prevent this serious disorder. Diagnosing this condition early is very important and requires a high index of suspicion. The treatment is purely supportive and most patients may show dramatic recovery. ODS occurring in normonatremic and hypernatremic patients is very rare. We report a case of an 18-year-old boy of end-stage renal disease who presented with an episode of acute gastroenteritis. He was managed with aggressive intravenous fluids, hemodialysis and other supportive therapy. But, he developed altered sensorium and seizures that progressed to features of spastic quadriparesis and lower cranial nerve palsy. Neuroimaging showed hyperintensities in pons and midbrain suggestive of ODS. The patient had normal sodium levels at all times and had no evidence of hyponatremia. The patient was managed with hemodialysis, physiotherapy and other conservative measures and had a gradual clinical and radiological recovery.

  3. Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study

    Science.gov (United States)

    Papais-Alvarenga, Regina Maria; Vasconcelos, Claudia Cristina Ferreira; Carra, Adriana; de Castillo, Ibis Soto; Florentin, Sara; Diaz de Bedoya, Fernando Hamuy; Mandler, Raul; de Siervi, Luiza Campanella; Pimentel, Maria Lúcia Vellutini; Alvarenga, Marina Papais; Papais Alvarenga, Marcos; Grzesiuk, Anderson Kuntz; Gama Pereira, Ana Beatriz Calmon; Gomes Neto, Antonio Pereira; Velasquez, Carolina; Soublette, Carlos; Fleitas, Cynthia Veronica; Diniz, Denise Sisteroli; Armas, Elizabeth; Batista, Elizabeth; Hernandez, Freda; Pereira, Fernanda Ferreira Chaves da Costa; Siqueira, Heloise Helena; Cabeça, Hideraldo; Sanchez, Jose; Brooks, Joseph Bruno Bidin; Gonçalves, Marcus Vinicius; Barroso, Maria Cristina Del Negro; Ravelo, Maria Elena; Castillo, Maria Carlota; Ferreira, Maria Lúcia Brito; Rocha, Maria Sheila Guimarães; Parolin, Monica Koncke Fiuza; Molina, Omaira; Marinho, Patricia Beatriz Christino; Christo, Paulo Pereira; Brant de Souza, Renata; Pessanha Neto, Silvio; Camargo, Solange Maria das Graças; Machado, Suzana Costa; Neri, Vanderson Carvalho; Fragoso, Yara Dadalti; Alvarenga, Helcio; Thuler, Luiz Claudio Santos

    2015-01-01

    The idiopathic inflammatory demyelinating disease (IIDD) spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO) among multiple sclerosis (MS) cases in whites (1.2%-1.5%), increasing in Mestizos (8%) and Africans (15.4%-27.5%) living in areas of low MS prevalence. South America (SA) was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients’ demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian). The main disease categories and their associated frequencies were MS (76.9%), NMO (11.8%), other NMO syndromes (6.5%), CIS (3.5%), ADEM (1.0%), and acute encephalopathy (0.4%). Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS) score (r=0.374; p=<0.001). This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs). Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect

  4. Central Nervous System Idiopathic Inflammatory Demyelinating Disorders in South Americans: A Descriptive, Multicenter, Cross-Sectional Study.

    Directory of Open Access Journals (Sweden)

    Regina Maria Papais-Alvarenga

    Full Text Available The idiopathic inflammatory demyelinating disease (IIDD spectrum has been investigated among different populations, and the results have indicated a low relative frequency of neuromyelitis optica (NMO among multiple sclerosis (MS cases in whites (1.2%-1.5%, increasing in Mestizos (8% and Africans (15.4%-27.5% living in areas of low MS prevalence. South America (SA was colonized by Europeans from the Iberian Peninsula, and their miscegenation with natives and Africans slaves resulted in significant racial mixing. The current study analyzed the IIDD spectrum in SA after accounting for the ethnic heterogeneity of its population. A cross-sectional multicenter study was performed. Only individuals followed in 2011 with a confirmed diagnosis of IIDD using new diagnostic criteria were considered eligible. Patients' demographic, clinical and laboratory data were collected. In all, 1,917 individuals from 22 MS centers were included (73.7% female, 63.0% white, 28.0% African, 7.0% Mestizo, and 0.2% Asian. The main disease categories and their associated frequencies were MS (76.9%, NMO (11.8%, other NMO syndromes (6.5%, CIS (3.5%, ADEM (1.0%, and acute encephalopathy (0.4%. Females predominated in all main categories. The white ethnicity also predominated, except in NMO. Except in ADEM, the disease onset occurred between 20 and 39 years old, early onset in 8.2% of all cases, and late onset occurred in 8.9%. The long-term morbidity after a mean disease time of 9.28±7.7 years was characterized by mild disability in all categories except in NMO, which was scored as moderate. Disease time among those with MS was positively correlated with the expanded disability status scale (EDSS score (r=0.374; p=<0.001. This correlation was not observed in people with NMO or those with other NMO spectrum disorders (NMOSDs. Among patients with NMO, 83.2% showed a relapsing-remitting course, and 16.8% showed a monophasic course. The NMO-IgG antibody tested using indirect

  5. Association Between the Single Nucleotide Polymorphism and the Level of Aquaporin-4 Protein Expression in Han and Minority Chinese with Inflammatory Demyelinating Diseases of the Central Nervous System.

    Science.gov (United States)

    Chu, Lan; Dai, Qingqing; Xu, Zhu; He, Dian; Wang, Hao; Wang, Qingsong; Zhang, Yifan; Zhu, Yingwu; Li, Yuan; Cai, Gang; Slavica, Krantic; Allan, Kermode

    2016-07-01

    The purpose of this study was to determine whether or not aquaporin-4 (AQP4) gene mutations are related to the pathogenesis of inflammatory demyelinating diseases in the central nervous system. Polymorphisms of AQP4 exons 1-5 were determined by sequencing DNA from 67 patients with central nervous system inflammatory demyelinating diseases, including neuromyelitis optica (NMO), multiple sclerosis, recurrent or simultaneous bilateral optic neuritis, and longitudinally extensive transverse myelitis. A plasmid with the identified new missense mutation was constructed, and human embryonic kidney cells (HEK293A) were transfected with either the pEGFP-N1-AQP4-M23 vector (bearing the identified mutated cDNA sequence) or with the plasmid bearing the wild-type AQP4 gene sequence. AQP4 protein expression was analyzed in both experimental groups using Western Blot analysis following protein extraction from transfected cells. A synonymous mutation (rs1839318) was detected on exon 3, and an additional synonymous mutation was detected on the exon 2-2 (rs72557968). Most importantly, a new missense mutation was detected on exon 2-1. According to Western blot analysis, the mutated cDNA sequence yielded increased AQP4 protein expression in comparison with the wild-type cDNA sequence (P < 0.05). AQP4 gene mutations are uncommon, occurring in only 3 out of 67 patients. Although it is possible that the mutations contributed to an increased risk of inflammatory central nervous system disease in these individuals, it is unlikely that mutations are a significant contributor to most patients with NMO spectrum disorders in China. PMID:25895050

  6. The topograpy of demyelination and neurodegeneration in the multiple sclerosis brain.

    Science.gov (United States)

    Haider, Lukas; Zrzavy, Tobias; Hametner, Simon; Höftberger, Romana; Bagnato, Francesca; Grabner, Günther; Trattnig, Siegfried; Pfeifenbring, Sabine; Brück, Wolfgang; Lassmann, Hans

    2016-03-01

    Multiple sclerosis is a chronic inflammatory disease with primary demyelination and neurodegeneration in the central nervous system. In our study we analysed demyelination and neurodegeneration in a large series of multiple sclerosis brains and provide a map that displays the frequency of different brain areas to be affected by these processes. Demyelination in the cerebral cortex was related to inflammatory infiltrates in the meninges, which was pronounced in invaginations of the brain surface (sulci) and possibly promoted by low flow of the cerebrospinal fluid in these areas. Focal demyelinated lesions in the white matter occurred at sites with high venous density and additionally accumulated in watershed areas of low arterial blood supply. Two different patterns of neurodegeneration in the cortex were identified: oxidative injury of cortical neurons and retrograde neurodegeneration due to axonal injury in the white matter. While oxidative injury was related to the inflammatory process in the meninges and pronounced in actively demyelinating cortical lesions, retrograde degeneration was mainly related to demyelinated lesions and axonal loss in the white matter. Our data show that accumulation of lesions and neurodegeneration in the multiple sclerosis brain does not affect all brain regions equally and provides the pathological basis for the selection of brain areas for monitoring regional injury and atrophy development in future magnetic resonance imaging studies.

  7. Pneumonectomy for chronic inflammatory lung disease: indications and complications

    Institute of Scientific and Technical Information of China (English)

    NIE Gang; LIU Guo-jun; Jean Deslauriers; FAN Zhi-min

    2010-01-01

    @@ Chronic inflammatory lung disease is a common health problem and often treated with potent antibiotics, anti-tuberculosis drugs, and antifungal agents. However, in case of medical therapy failure, surgical treatment has been often considered as an effective procedure.

  8. Alcoholism with central pontine demyelination: a case report

    Directory of Open Access Journals (Sweden)

    Rohit Arora

    2014-02-01

    Full Text Available Central pontine myelinolysis is a non-inflammatory demyelinating disease characterized by loss of myelin with relative neuron sparing, associated with rapid correction of hyponatremia and sometimes hypernatremia or chronic alcoholism. We are reporting a case of 52 year old male patient who was chronic alcoholic from past 20 years, presented to us with complaints of altered sensorium and dysarthria of 5 days duration .He was investigated and diagnosed as case of central pontine myelinosis associated with chronic alcoholism. [Int J Basic Clin Pharmacol 2014; 3(1.000: 230-232

  9. Management of Cardiovascular Risk in Patients with Chronic Inflammatory Diseases

    DEFF Research Database (Denmark)

    Lindhardsen, Jesper; Kristensen, Søren Lund; Ahlehoff, Ole

    2016-01-01

    An increased risk of cardiovascular disease (CVD) has been observed in a range of chronic inflammatory diseases (CID), including rheumatoid arthritis (RA), psoriasis, inflammatory bowel diseases (IBD), and systemic lupus erythematosus (SLE). The increased risk of CVDs and reduced life expectancy...

  10. Pathophysiology and treatment of inflammatory anorexia in chronic disease

    OpenAIRE

    Braun, Theodore P.; Marks, Daniel L.

    2010-01-01

    Decreased appetite and involuntary weight loss are common occurrences in chronic disease and have a negative impact on both quality of life and eventual mortality. Weight loss in chronic disease comes from both fat and lean mass, and is known as cachexia. Both alterations in appetite and body weight loss occur in a wide variety of diseases, including cancer, heart failure, renal failure, chronic obstructive pulmonary disease and HIV. An increase in circulating inflammatory cytokines has been ...

  11. A case of chronic inflammatory demyelinating polyneuropathy with reversible alternating diaphragmatic paralysis: case study

    OpenAIRE

    Haji, Kavi; Butler, Ernest; Royse, Colin

    2015-01-01

    Respiratory failure requiring mechanical ventilation has been reported in patients with bilateral diaphragmatic paralysis due to CIDP. We report a case of CIDP that progressed to respiratory failure with normal chest radiography despite unilateral diaphragmatic paralysis. This manifestation would have been missed if ultrasound was not employed. Electronic supplementary material The online version of this article (doi:10.1186/s13089-015-0033-5) contains supplementary material, which is availab...

  12. A case of chronic inflammatory demyelinating polyneuropathy with reversible alternating diaphragmatic paralysis: case study.

    Science.gov (United States)

    Haji, Kavi; Butler, Ernest; Royse, Colin

    2015-12-01

    Respiratory failure requiring mechanical ventilation has been reported in patients with bilateral diaphragmatic paralysis due to CIDP. We report a case of CIDP that progressed to respiratory failure with normal chest radiography despite unilateral diaphragmatic paralysis. This manifestation would have been missed if ultrasound was not employed. PMID:26490681

  13. Trigeminal Inflammatory Compression (TIC) injury induces chronic facial pain and susceptibility to anxiety-related behaviors.

    Science.gov (United States)

    Lyons, D N; Kniffin, T C; Zhang, L P; Danaher, R J; Miller, C S; Bocanegra, J L; Carlson, C R; Westlund, K N

    2015-06-01

    Our laboratory previously developed a novel neuropathic and inflammatory facial pain model for mice referred to as the Trigeminal Inflammatory Compression (TIC) model. Rather than inducing whole nerve ischemia and neuronal loss, this injury induces only slight peripheral nerve demyelination triggering long-term mechanical allodynia and cold hypersensitivity on the ipsilateral whisker pad. The aim of the present study is to further characterize the phenotype of the TIC injury model using specific behavioral assays (i.e. light-dark box, open field exploratory activity, and elevated plus maze) to explore pain- and anxiety-like behaviors associated with this model. Our findings determined that the TIC injury produces hypersensitivity 100% of the time after surgery that persists at least 21 weeks post injury (until the animals are euthanized). Three receptive field sensitivity pattern variations in mice with TIC injury are specified. Animals with TIC injury begin displaying anxiety-like behavior in the light-dark box preference and open field exploratory tests at week eight post injury as compared to sham and naïve animals. Panic anxiety-like behavior was shown in the elevated plus maze in mice with TIC injury if the test was preceded with acoustic startle. Thus, in addition to mechanical and cold hypersensitivity, the present study identified significant anxiety-like behaviors in mice with TIC injury resembling the clinical symptomatology and psychosocial impairments of patients with chronic facial pain. Overall, the TIC injury model's chronicity, reproducibility, and reliability in producing pain- and anxiety-like behaviors demonstrate its usefulness as a chronic neuropathic facial pain model.

  14. Macrolides in Chronic Inflammatory Skin Disorders

    Directory of Open Access Journals (Sweden)

    Abdullateef A. Alzolibani

    2012-01-01

    Full Text Available Long-term therapy with the macrolide antibiotic erythromycin was shown to alter the clinical course of diffuse panbronchiolitis in the late 1980s. Since that time, macrolides have been found to have a large number of anti-inflammatory properties in addition to being antimicrobials. These observations provided the rationale for many studies performed to assess the usefulness of macrolides in other inflammatory diseases including skin and hair disorders, such as rosacea, psoriasis, pityriasis rosea, alopecia areata, bullous pemphigoid, and pityriasis lichenoides. This paper summarizes a collection of clinical studies and case reports dealing with the potential benefits of macrolides antibiotics in the treatment of selected dermatoses which have primarily been classified as noninfectious and demonstrating their potential for being disease-modifying agents.

  15. Delayed functional expression of neuronal chemokine receptors following focal nerve demyelination in the rat: a mechanism for the development of chronic sensitization of peripheral nociceptors

    Directory of Open Access Journals (Sweden)

    Monahan Patrick E

    2007-12-01

    Full Text Available Abstract Background Animal and clinical studies have revealed that focal peripheral nerve axon demyelination is accompanied by nociceptive pain behavior. C-C and C-X-C chemokines and their receptors have been strongly implicated in demyelinating polyneuropathies and persistent pain syndromes. Herein, we studied the degree to which chronic nociceptive pain behavior is correlated with the neuronal expression of chemokines and their receptors following unilateral lysophosphatidylcholine (LPC-induced focal demyelination of the sciatic nerve in rats. Results Focal nerve demyelination increased behavioral reflex responsiveness to mechanical stimuli between postoperative day (POD 3 and POD28 in both the hindpaw ipsilateral and contralateral to the nerve injury. This behavior was accompanied by a bilateral increase in the numbers of primary sensory neurons expressing the chemokine receptors CCR2, CCR5, and CXCR4 by POD14, with no change in the pattern of CXCR3 expression. Significant increases in the numbers of neurons expressing the chemokines monocyte chemoattractant protein-1 (MCP-1/CCL2, Regulated on Activation, Normal T Expressed and Secreted (RANTES/CCL5 and interferon γ-inducing protein-10 (IP-10/CXCL10 were also evident following nerve injury, although neuronal expression pattern of stromal cell derived factor-1α (SDF1/CXCL12 did not change. Functional studies demonstrated that acutely dissociated sensory neurons derived from LPC-injured animals responded with increased [Ca2+]i following exposure to MCP-1, IP-10, SDF1 and RANTES on POD 14 and 28, but these responses were largely absent by POD35. On days 14 and 28, rats received either saline or a CCR2 receptor antagonist isomer (CCR2 RA-[R] or its inactive enantiomer (CCR2 RA-[S] by intraperitoneal (i.p. injection. CCR2 RA-[R] treatment of nerve-injured rats produced stereospecific bilateral reversal of tactile hyperalgesia. Conclusion These results suggest that the presence of chemokine

  16. Chronic inflammatory diseases and cardiovascular risk: a systematic review.

    Science.gov (United States)

    Roifman, Idan; Beck, Paul L; Anderson, Todd J; Eisenberg, Mark J; Genest, Jacques

    2011-01-01

    Despite recent advancements in the treatment of coronary artery disease (CAD), it remains the number one cause of death in the world. While traditional risk factors partially account for the development of CAD, other novel risk factors have recently been implicated. Specifically, chronic inflammation has been postulated to play a role in the development and propagation of this disease. The purpose of this systematic review is to examine the available evidence to determine if patients with chronic inflammatory diseases have higher rates of cardiovascular disease. A MEDLINE search was conducted for articles published between 1980-2009. We focused on studies that assessed hard cardiovascular endpoints in subjects with chronic inflammatory conditions such as systemic lupus erythematosus, rheumatoid arthritis, ankylosing spondylitis, psoriatic arthritis, polymyositis/dermatomyositis, and inflammatory bowel disease. Although largely based on small studies, our review indicates that patients with chronic inflammatory conditions are likely at elevated risk for the development of CAD. Further research consisting of prospective cohort studies is needed to better quantify this risk.

  17. Prevalence of chronic diseases at the onset of inflammatory arthritis.

    NARCIS (Netherlands)

    Ursum, J.; Korevaar, J.C.; Twisk, J.W.R.; Peters, M.J.L.; Schellevis, F.G.; Nurmohamed, M.T.; Nielen, M.M.J.

    2012-01-01

    Background: To explore the prevalence of chronic diseases at the onset of inflammatory arthritis (IA) in the general practice and compare this to a group of control patients without IA. Methods: In this nested-case-control study, data were used from the Netherlands Information Network of eneral Pra

  18. Chronic Inflammatory Periodontal Disease in Patients with Human Immunodeficiency Virus.

    Directory of Open Access Journals (Sweden)

    Vania López Rodríguez

    2009-07-01

    Full Text Available Background: The Chronic Inflammatory Periodontal Disease is related with multiple risk factors. Those patients with human immunodeficiency virus have higher risk of presenting this disease and it is usually more serious in these cases. Objective: To describe the prevalence of Chronic Inflammatory Periodontal Disease in patients with HIV. Methods: Descriptive, observational, cross-sectional study including patients with HIV in Sancti Spiritus province. The occurrence of the disease was determined after the Periodontics Cuban Standards, and oral hygiene was assessed through the simplified oral hygiene index. Other variables were measured, such as smoking habits, T CD4+ lymphocyte counting and virus load. The independent association of each risk factor with the disease was determined through a logistic regression model. Results: The 56, 5 % of the 154 patients presented Chronic Inflammatory Periodontal Disease; 60 (39.0% gingivitis and 27 (17,5% periodontitis. Gingivitis was associated with poor oral hygiene (OR: 3,71 and periodontitis with smoking habit (OR: 5,20. The severe forms of periodontitis occurred mainly in patients with lymphocyte counting lower than 500 cells/mm3 . Conclusions: The prevalence of Chronic Inflammatory Periodontal Disease in patients with HIV in Sancti Spiritus province is linked to known risk factors such as smoking habits and oral hygiene.

  19. Atypical idiopathic inflammatory demyelinating lesions (IIDL): Conventional and diffusion-weighted MR imaging (DWI) findings in 42 cases

    Energy Technology Data Exchange (ETDEWEB)

    Koelblinger, Claus; Fruehwald-Pallamar, Julia [Department of Biomedical Imaging and Image-Guided Therapy, Medical University Vienna, Vienna (Austria); Kubin, Klaus [CT/MRI Institut Dr. Klaus Kubin, Salzburg (Austria); Wallner-Blazek, Mirja [Department of Neurology, Medical University Graz, Graz (Austria); Hauwe, Luc van den [Department of Radiology, Medical University of Antwerp, Antwerp (Belgium); Macedo, Leonardo [Department of Radiology, CEDIMAGEM, Centro - Juiz de Fora (Brazil); Puchner, Stefan B. [Department of Biomedical Imaging and Image-Guided Therapy, Medical University Vienna, Vienna (Austria); Thurnher, Majda M., E-mail: majda.thurnher@meduniwien.ac.at [Department of Biomedical Imaging and Image-Guided Therapy, Medical University Vienna, Vienna (Austria)

    2013-11-01

    Introduction: The purpose of this study was to evaluate MR imaging characteristics with conventional and advanced MR imaging techniques in patients with IIDL. Methods: MR images of the brain in 42 patients (20 male, 22 female) with suspected or known multiple sclerosis (MS) from four institutions were retrospectively analyzed. Lesions were classified into five different subtypes: (1) ring-like lesions; (2) Balo-like lesions; (3) diffuse infiltrating lesions; (4) megacystic lesions; and (5) unclassified lesions. The location, size, margins, and signal intensities on T1WI, T2WI, and diffusion-weighted images (DWI), and the ADC values/ratios for all lesions, as well as the contrast enhancement pattern, and the presence of edema, were recorded. Results: There were 30 ring-like, 10 Balo-like, 3 megacystic-like and 16 diffuse infiltrating-like lesions were detected. Three lesions were categorized as unclassified lesions. Of the 30 ring-like lesions, 23 were hypointense centrally with a hyperintense rim. The mean ADC, measured centrally, was 1.50 ± 0.41 × 10{sup −3} mm{sup 2}/s. The mean ADC in the non-enhancing layers of the Balo-like lesions was 2.29 ± 0.17 × 10{sup −3} mm{sup 2}/s, and the mean ADC in enhancing layers was 1.03 ± 0.30 × 10{sup −3} mm{sup 2}/s. Megacystic lesions had a mean ADC of 2.14 ± 0.26 × 10{sup −3} mm{sup 2}/s. Peripheral strong enhancement with high signal on DWI was present in all diffuse infiltrating lesions. Unclassified lesions showed a mean ADC of 1.43 ± 0.13 mm{sup 2}/s. Conclusion: Restriction of diffusion will be seen in the outer layers of active inflammation/demyelination in Balo-like lesions, in the enhancing part of ring-like lesions, and at the periphery of infiltrative-type lesions.

  20. [Demyelinating diseases in children with acute neurological symptoms].

    Science.gov (United States)

    Olofsson, Isa Amalie; Skov, Liselotte; Miranda, Maria Jose

    2015-12-01

    Demyelinating diseases in children is a broad group of illnesses, which affect the central nervous system. Demyelinating diseases can be monophasic or chronic and comprise acute disseminated encephalomyelitis, optic neuritis, transverse myelitis, multiple sclerosis and neuromyelitis optica. Demyelinating diseases are rare, but it is important for the physician to recognize these diseases, as well as to understand the differential diagnoses. This review summarizes the current knowledge of demyelinating disorders in children, focusing on an approach to diagnosis and management. PMID:26651911

  1. CXCR2 signaling protects oligodendrocytes and restricts demyelination in a mouse model of viral-induced demyelination.

    Directory of Open Access Journals (Sweden)

    Martin P Hosking

    Full Text Available BACKGROUND: The functional role of ELR-positive CXC chemokines during viral-induced demyelination was assessed. Inoculation of the neuroattenuated JHM strain of mouse hepatitis virus (JHMV into the CNS of susceptible mice results in an acute encephalomyelitis that evolves into a chronic demyelinating disease, modeling white matter pathology observed in the human demyelinating disease Multiple Sclerosis. METHODOLOGY/PRINCIPAL FINDINGS: JHMV infection induced the rapid and sustained expression of transcripts specific for the ELR+ chemokine ligands CXCL1 and CXCL2, as well as their binding receptor CXCR2, which was enriched within the spinal cord during chronic infection. Inhibiting CXCR2 signaling with neutralizing antiserum significantly (p<0.03 delayed clinical recovery. Moreover, CXCR2 neutralization was associated with an increase in the severity of demyelination that was independent of viral recrudescence or modulation of neuroinflammation. Rather, blocking CXCR2 was associated with increased numbers of apoptotic cells primarily within white matter tracts, suggesting that oligodendrocytes were affected. JHMV infection of enriched oligodendrocyte progenitor cell (OPC cultures revealed that apoptosis was associated with elevated expression of cleaved caspase 3 and muted Bcl-2 expression. Inclusion of CXCL1 within JHMV infected cultures restricted caspase 3 cleavage and increased Bcl-2 expression that was associated with a significant (p<0.001 decrease in apoptosis. CXCR2 deficient oligodendrocytes were refractory to CXCL1 mediated protection from JHMV-induced apoptosis, readily activating caspase 3 and down regulating Bcl-2. CONCLUSION/SIGNIFICANCE: These findings highlight a previously unappreciated role for CXCR2 signaling in protecting oligodendrocyte lineage cells from apoptosis during inflammatory demyelination initiated by viral infection of the CNS.

  2. Tumefactive Demyelinating Lesions in Multiple Sclerosis and Associated Disorders.

    Science.gov (United States)

    Frederick, Meredith C; Cameron, Michelle H

    2016-03-01

    Tumefactive demyelinating lesions are rare consequences of central nervous system (CNS) idiopathic inflammatory demyelinating diseases. Tumefactive demyelinating lesions pose a diagnostic challenge because they can mimic tumors and abscesses and because they can be caused by a heterogeneous range of disorders. This article reviews the recent literature on the clinical presentation; radiographic features; prognosis; and management of tumefactive demyelinating lesions in multiple sclerosis, acute demyelinating encephalomyelitis, neuromyelitis optica, and the rare variants of multiple sclerosis including Schilder's disease, Marburg acute multiple sclerosis, and Balo's concentric sclerosis.

  3. A histopathologic analysis of chronic inflammatory infiltrate in patients of h. pylori associated chronic gastritis

    International Nuclear Information System (INIS)

    To determine the relationship between H. pylori density with severity of chronic inflammatory infiltrate. Study Design: A cross-sectional study. Place and Duration of Study: The study was carried out in the Department of Pathology (Histopathology), Army Medical College, National University of Sciences and Technology (NUST) Islamabad, from Nov 2011 to Nov 2012. Methodology: Gastric antral biopsies of H. pylori associated chronic gastritis were included in the study. Demographic characteristics and relevant clinical information were collected. First hundred biopsies of H. pylori associated chronic gastritis were assessed for density of H. pylori and chronic inflammatory infiltrate. istopathological features like lymphoid aggregates, ulcer slough, superficial epithelial damage, dysplasia and nuclear reactive changes were simply assessed in case of their presence or absence. Results: A significant moderate positive correlation was found between grades of H. pylori and chronic inflammatory infiltrate (rs= 0.636). Insignificant correlation was found with lymphoid aggregates, superficial epithelial damage, dysplasia and nuclear reactive changes. Conclusion: In conclusion this study corroborated the determination of histopathological parameters and depicted that, the greater the density of H. pylori infection, the greater the degrees of chronic inflammatory infiltrate. (author)

  4. Chronic Inflammatory Disease and Osteopathy: A Systematic Review

    OpenAIRE

    Luca Cicchitti; Marta Martelli; Francesco Cerritelli

    2015-01-01

    Background Chronic inflammatory diseases (CID) are globally highly prevalent and characterized by severe pathological medical conditions. Several trials were conducted aiming at measuring the effects of manipulative therapies on patients affected by CID. The purpose of this review was to explore the extent to which osteopathic manipulative treatment (OMT) can be benefi-cial in medical conditions also classified as CID. Methods This review included any type of experimental study which enrolled...

  5. A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

    OpenAIRE

    Katsenos Chrysostomos; Androulaki Despoina; Lyra Stavroula; Tsoutsouras Theodoros; Mandragos Costas

    2013-01-01

    Abstract Background Demyelinating diseases cause destruction of the myelin sheath, while axons are relatively spared. Pathologically, demyelination can be the result of an inflammatory process, viral infection, acquired metabolic derangement and ischemic insult. Three diseases that can cause inflammatory demyelination of the CNS are: Multiple sclerosis (MS), Acute disseminated encephalomyelitis (ADEM) and Acute hemorrhagic leucoencephalitis. Differentiation is not always easy and there is con...

  6. Are electrophysiological criteria useful in distinguishing childhood demyelinating neuropathies?

    Science.gov (United States)

    Potulska-Chromik, Anna; Ryniewicz, Barbara; Aragon-Gawinska, Karolina; Kabzinska, Dagmara; Seroka, Andrzej; Lipowska, Marta; Kaminska, Anna M; Kostera-Pruszczyk, Anna

    2016-03-01

    Childhood chronic inflammatory demyelinating polyneuropathy (CIDP) needs to be differentiated from hereditary neuropathy. We aimed to validate existing CIDP nerve conduction study (NCS) criteria in a group of children with demyelinating neuropathies of chronic or subacute onset. Retrospective analysis of clinical and NCS results in 18 children with CIDP, 7 with hereditary neuropathy with pressure palsy (HNPP), and 24 with Charcot-Marie-Tooth 1a (CMT1a). AAN and EFNS electrodiagnostic CIDP criteria were fulfilled in 17 of 18 CIDP, 3 of 7 HNPP, and 23 of 24 CMT1a patients. A distal compound muscle action potential (dCMAP) of >9 ms was observed in 14 of 18 CIDP patients but not in any patients with HNPP. Abnormal median/normal sural SNAP (AMNS) and a 10 m/s difference between conduction velocities (CV) of two corresponding nerves were not observed in any CMT1a patients. NCS in CMT1a, HNPP, and CIDP reflect demyelination. dCMAP duration, sensory AMNS, and a 10 m/s CV difference parameter are most useful in the differential diagnosis of pediatric CIDP. PMID:26663344

  7. Autoimmune antigenic targets at the node of Ranvier in demyelinating disorders.

    Science.gov (United States)

    Stathopoulos, Panos; Alexopoulos, Harry; Dalakas, Marinos C

    2015-03-01

    Mounting evidence suggests that autoantibodies contribute to the pathogenesis of demyelination in the PNS and CNS. Rapid reversal of electrophysiological blockade after plasmapheresis or intravenous immunoglobulin treatment for acute or chronic inflammatory demyelinating polyneuropathy is more likely to result from removal or neutralization of an antibody that impairs saltatory conduction than from remyelination. Although up to 30% of patients with acute or chronic inflammatory demyelinating polyneuropathy harbour autoantibodies, specific antigens have been identified in no more than 13% of cases. To date, autoantigens identified at the node of Ranvier include neurofascin 186, gliomedin and possibly moesin in the nodal domain, and contactin-1, Caspr1 and neurofascin 155 in the paranodal domain. In some patients with multiple sclerosis, paranodal CNPase and juxtaparanodal contactin-2 trigger a humoral response. This Review explores the molecular anatomy of the node of Ranvier, focusing on proteins with extracellular domains that could serve as antigens. The clinical implications of node-specific antibody responses are addressed, and the best approaches to identify antibodies that target nodal proteins are highlighted. Also discussed are the roles of these antibodies as either secondary, disease-exacerbating responses, or as a primary effector mechanism that defines demyelination or axonal degeneration at the node, identifies disease subtypes or determines response to treatments.

  8. Chronic Inflammatory Diseases: Progress and Prospect with Herbal Medicine.

    Science.gov (United States)

    Ghosh, Nilanjan; Ali, Asif; Ghosh, Rituparna; Das, Shaileyee; Mandal, Subhash C; Pal, Mahadeb

    2016-01-01

    Diseases associated with chronic inflammatory pathology claim a major share of worldwide deaths each year. A principal reason behind the huge number of casualties is associated with mild or unnoticed symptoms for long period of time since the outset, and that specific treatment options for these diseases have not yet emerged. Current anti-inflammatory drugs essentially have become ineffective for long term protection from these diseases as they also interfere with essential cellular pathways and associated toxicities. Notably, recent studies with a number of phytochemicals have shown promising results. These compounds isolated from various medicinal plants express their anti-inflammatory activities by down regulating expression of several crucial pro-inflammatory mediators. These are mostly antioxidants; inhibit induction of key transcription factors like nuclear factor kappa B (NF-κB) that are responsible for expression of proinflammatory mediators, and other growth regulators. Definitely, some of these compounds have the potential to be developed into new therapeutic agents to better control inflammation associated diseases in near future. This review summarizes recent findings on the molecular mechanisms through which various inflammatory activities are linked to disease progression and a group of natural products that have shown promise in controlling these processes. PMID:26561064

  9. The effects of acute and chronic exercise on inflammatory markers in children and adults with a chronic inflammatory disease : a systematic review

    NARCIS (Netherlands)

    Ploeger, Hilde E.; Takken, Tim; de Greef, Mathieu H. G.; Timmons, Brian W.

    2009-01-01

    Background: Chronic inflammatory diseases strike millions of people all over the world, and exercise is often prescribed for these patients to improve overall fitness and quality of life. In healthy individuals, acute and chronic exercise is known to alter inflammatory markers; however, less is know

  10. Central and peripheral demyelination

    OpenAIRE

    Man Mohan Mehndiratta; Natasha Singh Gulati

    2014-01-01

    Several conditions cause damage to the inherently normal myelin of central nervous system, perepheral nervous system or both central and perepheral nervous system and hence termed as central demyelinating diseases, perepheral demyelinating diseases and combined central and perepheral demyelinating diseases respectively. Here we analysed and foccused on the etiology, prevalance, incidence and age of these demyelinating disorders. Clinical attention and various diagnostic tests are needed to ad...

  11. Managing Inflammatory Manifestations in Patients with Chronic Granulomatous Disease.

    Science.gov (United States)

    Magnani, Alessandra; Mahlaoui, Nizar

    2016-10-01

    Chronic granulomatous disease (CGD) is a primary immunodeficiency caused by lack of phagocyte nicotinamide adenine dinucleotide phosphate (NADPH) oxidase, which results in inflammatory dysregulation and increased susceptibility to infections. Patients with CGD may develop severe obstructive disorders of the digestive tract as a result of their dysregulated inflammatory response. Despite a growing focus on inflammatory manifestations in CGD, the literature data on obstructive complications are far less extensive than those on infectious complications. Diagnosis and management of patients with concomitant predispositions to infections and hyperinflammation are particularly challenging. Although the inflammatory and granulomatous manifestations of CGD usually respond rapidly to steroid treatment, second-line therapies (immunosuppressants and biologics) may be required in refractory cases. Indeed, immunosuppressants (such as anti-tumor necrosis factor agents, thalidomide, and anakinra) have shown some efficacy, but the value of this approach is controversial, given the questionable risk-to-benefit ratio and the small numbers of patients treated to date. Significant progress in allogeneic hematopoietic stem cell transplantation (the only curative treatment for CGD) has been made through better supportive care and implementation of improved, reduced-intensity conditioning regimens. Gene therapy may eventually be an option for patients lacking a suitable donor; clinical trials with new, safer vectors are ongoing at a few centers. PMID:27299584

  12. Sarah's Knee: A Famous Actress With Chronic, Inflammatory Monoarthritis.

    Science.gov (United States)

    Pinals, Robert S

    2004-02-01

    Sarah Bernhardt had a recurrent and later persistent inflammatory arthritis of her right knee for more than 25 years. She probably had pulmonary tuberculosis, starting a dozen years before the arthritis, and her chronic synovitis may have been tuberculous. Several months in a cast led to deterioration and later amputation of the leg, an outcome that might have been prevented by surgical arthrodesis. Despite the loss of her limb and progressive renal failure, she continued an active theatrical career until her death at age 78. PMID:17043454

  13. The microbiome in chronic inflammatory airway disease: A threatened species.

    Science.gov (United States)

    Green, Robin John; Van Niekerk, Andre; Jeevarathnum, Ashley C; Feldman, Charles; Richards On Behalf Of The South African Allergic Rhinitis Working Group, Guy A

    2016-08-01

    The human body is exposed to a multitude of microbes and infectious organisms throughout life. Many of these organisms colonise the skin, gastrointestinal tract (GIT) and airway. We now recognise that this colonisation includes the lower airway, previously thought to be sterile. These colonising organisms play an important role in disease prevention, including an array of chronic inflammatory conditions that are unrelated to infectious diseases. However, new evidence of immune dysregulation suggests that early colonisation, especially of the GITand airway, by pathogenic micro-organisms, has deleterious effects that may contribute to the potential to induce chronic inflammation in young children, which may only express itself in adult life. PMID:27499401

  14. Prevention of human cancer by modulation of chronic inflammatory processes

    Energy Technology Data Exchange (ETDEWEB)

    Ohshima, Hiroshi [International Agency for Research on Cancer, 150 Cours Albert Thomas, 69372 Lyon Cedex 08 (France)]. E-mail: ohshima@iarc.fr; Tazawa, Hiroshi [International Agency for Research on Cancer, 150 Cours Albert Thomas, 69372 Lyon Cedex 08 (France); Sylla, Bakary S. [International Agency for Research on Cancer, 150 Cours Albert Thomas, 69372 Lyon Cedex 08 (France); Sawa, Tomohiro [International Agency for Research on Cancer, 150 Cours Albert Thomas, 69372 Lyon Cedex 08 (France)

    2005-12-11

    Chronic inflammation induced by biological, chemical and physical factors has been associated with increased risk of human cancer at various sites. Inflammation facilitates the initiation of normal cells and their growth and progression to malignancy through production of pro-inflammatory cytokines and diverse reactive oxygen and nitrogen species. These also activate signaling molecules involved in inflammation and carcinogenesis such as nuclear transcription factor (NF-{kappa}B), inducible nitric oxide synthase (iNOS) and cyclooxygenase-2 (COX-2). Several chemopreventive agents act through inhibition of signaling pathways (e.g. NF-{kappa}B), inhibition of oxidant-generating enzymes (e.g. iNOS) and mediators of inflammation (e.g. COX-2), scavenging reactive oxygen and nitrogen species, and modulation of xenobiotic-metabolizing enzymes (especially phase II enzyme induction). Some anti-inflammatory drugs have been tested in clinical trials to prevent human cancer at several sites. Better understanding of the molecular mechanisms by which chronic inflammation increases cancer risk will lead to further development of new strategies for cancer prevention at many sites.

  15. Treatment Responsiveness in CIDP Patients with Diabetes Is Associated with Higher Degrees of Demyelination

    Science.gov (United States)

    Abraham, Alon; Alabdali, Majed; Qrimli, Mohammad; Albulaihe, Hana; Breiner, Ari; Barnett, Carolina; Katzberg, Hans D.; Lovblom, Leif E.; Perkins, Bruce A.; Bril, Vera

    2015-01-01

    Introduction Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is one of several chronic treatable acquired demyelinating neuropathies. Objectives To explore the association between the degree of demyelination in CIDP, and treatment responsiveness. Methods A retrospective chart review of CIDP subjects assessed between 1997 and 2013 was performed to compare treatment responsiveness using different sets of criteria. Results 99 CIDP patients were included, 34 with diabetes mellitus (DM). Treatment responsiveness was higher in CIDP-DM fulfilling 1 or more EFNS/PNS criteria, (63% vs. 31%, p = 0.03), and in CIDP+DM fulfilling 2 or more criteria (89% vs. 36%, p = 0.01). Nonetheless, treatment responsiveness in CIDP+DM had the highest odds ratio (3.73, p = 0.01). Similar results were also shown in simplified uniform study criteria, with 10% cut off values for CIDP-DM, compared to 30% for CIDP+DM. Conclusion In CIDP+DM, higher degrees of demyelination are associated with treatment responsiveness, implying the need to adjust current criteria in these patients. PMID:26461125

  16. Treatment Responsiveness in CIDP Patients with Diabetes Is Associated with Higher Degrees of Demyelination.

    Directory of Open Access Journals (Sweden)

    Alon Abraham

    Full Text Available Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP is one of several chronic treatable acquired demyelinating neuropathies.To explore the association between the degree of demyelination in CIDP, and treatment responsiveness.A retrospective chart review of CIDP subjects assessed between 1997 and 2013 was performed to compare treatment responsiveness using different sets of criteria.99 CIDP patients were included, 34 with diabetes mellitus (DM. Treatment responsiveness was higher in CIDP-DM fulfilling 1 or more EFNS/PNS criteria, (63% vs. 31%, p = 0.03, and in CIDP+DM fulfilling 2 or more criteria (89% vs. 36%, p = 0.01. Nonetheless, treatment responsiveness in CIDP+DM had the highest odds ratio (3.73, p = 0.01. Similar results were also shown in simplified uniform study criteria, with 10% cut off values for CIDP-DM, compared to 30% for CIDP+DM.In CIDP+DM, higher degrees of demyelination are associated with treatment responsiveness, implying the need to adjust current criteria in these patients.

  17. Probiotics and prebiotics in chronic inflammatory bowel diseases

    Institute of Scientific and Technical Information of China (English)

    Julia B Ewaschuk; Levinus A Dieleman

    2006-01-01

    The prokaryotic and eukaryotic cells of the colon exist in a highly complex, but harmonious relationship.Disturbances in this remarkable symbiosis can result in the development of inflammatory bowel diseases (IBD).Although the etiology of IBD is not entirely understood,it is known that the chronic inflammation of Crohn's disease, ulcerative colitis and chronic pouchitis are a result of an overly aggressive immune response to the commensal intestinal flora in genetically susceptible hosts. Recent studies have enhanced our ability to understand the interaction between the host and its intestinal microflora and the role the microflora plays in maintaining intestinal homeostasis. As we begin to understand the benefits conferred to the intestine by the microflora, the notion of modifying the composition of the bacterial load to improve human health has arisen.A significant body of research now exists investigating the role of probiotics and prebiotics in ameliorating chronic intestinal inflammation. This article will begin with an overview of the role of the commensal microflora in maintaining mucosal immune homeostasis, and how a dysregulated immune response to the intestinal microflora results in IBD. This will be followed by a summary of the use of probiotics and prebiotics in experimental and human IBD.

  18. MRI for chronic inflammatory bowel disease; MRT chronisch entzuendlicher Darmerkrankungen

    Energy Technology Data Exchange (ETDEWEB)

    Hansmann, H.J.; Hess, T.; Hahmann, M.; Erb, G.; Richter, G.M.; Duex, M. [Heidelberg Univ. (Germany). Abt. Roentgendiagnostik; Elsing, C. [Heidelberg Univ. (Germany). Abt. IV - Gastroenterologie

    2001-01-01

    Chronic inflammatory bowel disease is diagnosed and monitored by the combination of colonoscopy and small bowel enteroklysis. Magnetic resonance imaging has become the gold standard for the imaging of perirectal and pelvic fistulas. With the advent of ultrafast MRI small and large bowel imaging has become highly attractive and is being advocated more and more in the diagnostic work up of inflammatory bowel disease. Imaging protocols include fast T{sub 1}-weighted gradient echo and T{sub 2}-weighted TSE sequences and oral or rectal bowel distension. Furthermore, dedicated imaging protocols are based on breath-hold imaging under pharmacological bowel paralysis and gastrointestinal MR contrast agents (Hydro-MRI). High diagnostic accuracy can be achieved in Crohn's disease with special reference to the pattern of disease, depth of inflammation, mesenteric reaction, sinus tract depiction and formation of abscess. In ulcerative colitis, the mucosa-related inflammation causes significantly less bowel wall thickening compared to Crohn's disease. Therefore with MRI, the extent of inflammatory changes is always underestimated compared to colonoscopy. According to our experience in more than 200 patients as well as the results in other centers, Hydro-MRI possesses the potential to replace enteroklysis in the diagnosis of chronic inflammatory bowel disease and most of the follow-up colonoscopies in Crohn's disease. Further technical improvements in 3D imaging will allow interactive postprocessing of the MR data. (orig.) [German] Zusammenfassung: Die Standardverfahren in der Diagnostik und der Verlaufskontrolle chronisch entzuendlicher Darmerkrankungen, speziell des Morbus Crohn und der Colitis ulcerosa, sind die Koloskopie und das Enteroklysma. Die MRT hat sich dazu ihren festen Platz in der Diagnostik perirektaler Fisteln erobert. Mit schnellen, T{sub 1}-gewichteten Gradienten-Echo-Sequenzen und T{sub 2}-gewichteten Turbo-Spin-Echo-Sequenzen koennen auch Duenn

  19. 肌电图对颈髓髓内肿瘤和炎性脱髓鞘病的鉴别诊断研究%The value of electromyography in differentiating intramedullary tumor from inflammatory demyelinating disease of cervical region

    Institute of Scientific and Technical Information of China (English)

    王红芬; 陈朝晖; 凌丽; 尚爱加; 乔广宇; 崔芳; 杨飞; 黄旭升

    2014-01-01

    Objective To investigate the value of needle electromyography (EMG) in differentiating intramedullary tumor from inflammatory demyelinating disease of cervical region.Methods Patients hospitalized in the Chinese PLA General Hospital from March 2008 to June 2013 with abnormalities on MRI of cervical vertebra and preliminary diagnosed as intramedullary tumor or inflammatory demyelinating disease of cervical region were enrolled in the study.Electrophysiological examination was performed before any treatment.Pathological findings were analyzed and prognosis was evaluated in all the subjects.Results A total of fifty-five patients were enrolled in the study with 33 cases of inflammatory demyelinating disease and 22 cases of intramedullary tumor defined by the postoperative pathological findings.In all the 33 cases with demyelinating disease,only one case (3.03%) presented as neurogenic damage by needle EMG.While in all the 22 cases with intramedullary tumor,needle EMG revealed neurogenic damage in 15 cases (68.18%) and the spinal segments of muscles with neurogenic damage were all within the spinal lesions demonstrated by MRI.The diagnostic sensitivity of EMG for intramedullary tumor was 68.18% and the diagnostic specificity was 96.97%,while the diagnostic sensitivity and specificity for intramedullary tumor by the medical history,symptoms and signs were 59.09% and 75.76% respectively.Conclusion Needle EMG might play an important role in distinguishing intramedullary tumor from inflammatory demyelinating disease of cervical spinal cord.%目的 探讨肌电图(EMG)对颈髓髓内肿瘤和炎性脱髓鞘病的鉴别诊断价值.方法 选择2008年3月至2013年6月解放军总医院收治的颈椎MRI呈现异常信号,拟诊髓内肿瘤或炎性脱髓鞘病的住院患者为研究对象,于手术等治疗前行电生理检查,结合手术病理结果进行分析,对预后进行随访观察,并比较EMG诊断以及依据病史、症状和体征对髓内肿

  20. Treg inducing adjuvants for therapeutic vaccination against chronic inflammatory diseases

    Directory of Open Access Journals (Sweden)

    Chantal eKeijzer

    2013-08-01

    Full Text Available Many existing therapies in autoimmune diseases are based on systemic suppression of inflammation, the observed side effects illustrate the need for more specific interventions. Regulatory T cells (Treg are pivotal controllers of (autoaggressive immune responses, and decreased Treg numbers and/or functioning have been associated with autoimmune disease. Especially antigen-specific targeting of Treg would enable tailor made interventions, while obviating negative side effects of general immuno-suppression. Self-antigens that participate in inflammation, irrespective of the etiology of the different autoimmune diseases, are held to be candidate antigens for such interventions. Rather than tolerance induction to disease inciting self-antigens, which are frequently unknown, general self-antigens expressed at sites of inflammation would allow targeting of disease independent, but inflammatory-site specific, regulatory mechanisms. Preferably, such self-antigens should be abundantly expressed and up-regulated at the inflammatory site. Heat shock proteins show several of these characteristics.The development of antigen-specific Treg inducing vaccines is a major novel goal in the field of immunotherapy in autoimmune diseases. Progress is hampered by the lack of effective antigens and by the fact that other factors such as dose, route and the presence or absence of an adjuvant, turned out to be critical unknowns, with respect to effective induction of Treg. The use of a Treg inducing adjuvant might be required to achieve effective regulatory responses, in the case of ongoing inflammation. Future goals will be the optimization of natural Treg expansion (or the induction of adaptive Treg without loss of their suppressive function or the concomitant induction of non-regulatory T cells. Here, we discuss the potential use of protein/peptide-based vaccines combined with Treg inducing adjuvants for the development of therapeutic vaccines against chronic

  1. Clinical Study on Treatment of Acute Inflammatory Demyelinating Polyneu-ropathy%急性炎症性脱髓鞘性多发性神经病临床治疗探讨

    Institute of Scientific and Technical Information of China (English)

    张凤媛

    2015-01-01

    目的:探讨急性炎症性脱髓鞘性多发性神经病的治疗效果。方法收集2009年-2014年辽西医学院附属医院急性炎症性脱髓鞘性多发性神经病患者共62例,进行治疗的回顾性分析。结果62例患者经系统性综合治疗,总有效率为90.32%,其中治愈36例,好转20例,自动出院4例,死亡2例;IVIG组与PE组治疗有效率比较,差异无统计学意义(P>0.05)。结论综合治疗能有效治疗急性炎症性脱髓鞘性多发性神经病;IVIG组与PE组治疗效果无太大差别,各医院可以根据自身硬件条件开展不同的治疗方案。%Objective To explore the treatment effect of acute inflammatory demyelinating polyneuropathy.Methods 62 cases of patients with acute inflammatory demyelinating polyneuropathy in The Affiliated Hospital of Liaoning Medical University from 2009 to 2014 were collected , then they were retrospectively analyzed the treatment. Results 62 cases of patients with comprehen-sive treatment , the total effective rate was 90.32%, 36 cases were cured, 20 cases improved, 4 cases leave hospital automatically, there was 2 case of death;the effective rate of the treatment comparison between IVIG group and PE group is no significant differ-ence (P>0.05). Conclusion The comprehensive treatment is effective in the treatment of acute inflammatory demyelinating polyneu-ropathy;the therapeutic effect is't significant difference between the IVIG group and PE group, the hospital can carry out different treatment according to their own hardware conditions.

  2. Atherosclerotic cardiovascular disease in patients with chronic inflammatory joint disorders.

    Science.gov (United States)

    Agca, R; Heslinga, S C; van Halm, V P; Nurmohamed, M T

    2016-05-15

    Inflammatory joint disorders (IJD), including rheumatoid arthritis (RA), ankylosing spondylitis (ASp) and psoriatic arthritis (PsA), are prevalent conditions worldwide with a considerable burden on healthcare systems. IJD are associated with increased cardiovascular (CV) disease-related morbidity and mortality. In this review, we present an overview of the literature. Standardised mortality ratios are increased in IJD compared with the general population, that is, RA 1.3-2.3, ASp 1.6-1.9 and PsA 0.8-1.6. This premature mortality is mainly caused by atherosclerotic events. In RA, this CV risk is comparable to that in type 2 diabetes. Traditional CV risk factors are more often present and partially a consequence of changes in physical function related to the underlying IJD. Also, chronic systemic inflammation itself is an independent CV risk factor. Optimal control of disease activity with conventional synthetic, targeted synthetic and biological disease-modifying antirheumatic drugs decreases this excess risk. High-grade inflammation as well as anti-inflammatory treatment alter traditional CV risk factors, such as lipids. In view of the above-mentioned CV burden in patients with IJD, CV risk management is necessary. Presently, this CV risk management is still lacking in usual care. Patients, general practitioners, cardiologists, internists and rheumatologists need to be aware of the substantially increased CV risk in IJD and should make a combined effort to timely initiate CV risk management in accordance with prevailing guidelines together with optimal control of rheumatic disease activity. CV screening and treatment strategies need to be implemented in usual care. PMID:26888573

  3. Chronic Inflammatory Disease and Osteopathy: A Systematic Review

    Science.gov (United States)

    Cicchitti, Luca; Martelli, Marta; Cerritelli, Francesco

    2015-01-01

    Background Chronic inflammatory diseases (CID) are globally highly prevalent and characterized by severe pathological medical conditions. Several trials were conducted aiming at measuring the effects of manipulative therapies on patients affected by CID. The purpose of this review was to explore the extent to which osteopathic manipulative treatment (OMT) can be benefi-cial in medical conditions also classified as CID. Methods This review included any type of experimental study which enrolled sub-jects with CID comparing OMT with any type of control procedure. The search was conducted on eight databases in January 2014 using a pragmatic literature search approach. Two independent re-viewers conducted study selection and data extraction for each study. The risk of bias was evaluated according to the Cochrane methods. Heterogeneity was assessed and meta-analysis performed where possible. Results 10 studies met the inclusion criteria for this review enrolling 386 subjects. The search identified six RCTs, one laboratory study, one cross-over pilot studies, one observation-al study and one case control pilot study. Results suggest a potential effect of osteopathic medicine on patients with medical pathologies associated with CID (in particular Chronic Obstructive Pul-monary Disease (COPD), Irritable Bowel Syndrome, Asthma and Peripheral Arterial Disease) com-pared to no treatment or sham therapy although data remain elusive. Moreover one study showed possible effects on arthritis rat model. Meta-analysis was performed for COPD studies only show-ing no effect of any type of OMT applied versus control. No major side effects were reported by those receiving OMT. Conclusion The present systematic review showed inconsistent data on the effect of OMT in the treatment of medical conditions potentially associated with CID, however the OMT appears to be a safe approach. Further more robust trials are needed to determine the direction and magnitude of the effect of OMT and to

  4. Chronic inflammatory disease and osteopathy: a systematic review.

    Directory of Open Access Journals (Sweden)

    Luca Cicchitti

    Full Text Available Chronic inflammatory diseases (CID are globally highly prevalent and characterized by severe pathological medical conditions. Several trials were conducted aiming at measuring the effects of manipulative therapies on patients affected by CID. The purpose of this review was to explore the extent to which osteopathic manipulative treatment (OMT can be benefi-cial in medical conditions also classified as CID.This review included any type of experimental study which enrolled sub-jects with CID comparing OMT with any type of control procedure. The search was conducted on eight databases in January 2014 using a pragmatic literature search approach. Two independent re-viewers conducted study selection and data extraction for each study. The risk of bias was evaluated according to the Cochrane methods. Heterogeneity was assessed and meta-analysis performed where possible.10 studies met the inclusion criteria for this review enrolling 386 subjects. The search identified six RCTs, one laboratory study, one cross-over pilot studies, one observation-al study and one case control pilot study. Results suggest a potential effect of osteopathic medicine on patients with medical pathologies associated with CID (in particular Chronic Obstructive Pul-monary Disease (COPD, Irritable Bowel Syndrome, Asthma and Peripheral Arterial Disease com-pared to no treatment or sham therapy although data remain elusive. Moreover one study showed possible effects on arthritis rat model. Meta-analysis was performed for COPD studies only show-ing no effect of any type of OMT applied versus control. No major side effects were reported by those receiving OMT.The present systematic review showed inconsistent data on the effect of OMT in the treatment of medical conditions potentially associated with CID, however the OMT appears to be a safe approach. Further more robust trials are needed to determine the direction and magnitude of the effect of OMT and to generalize favorable results.

  5. Inflammatory mechanisms in patients with chronic obstructive pulmonary disease.

    Science.gov (United States)

    Barnes, Peter J

    2016-07-01

    Chronic obstructive pulmonary disease (COPD) is associated with chronic inflammation affecting predominantly the lung parenchyma and peripheral airways that results in largely irreversible and progressive airflow limitation. This inflammation is characterized by increased numbers of alveolar macrophages, neutrophils, T lymphocytes (predominantly TC1, TH1, and TH17 cells), and innate lymphoid cells recruited from the circulation. These cells and structural cells, including epithelial and endothelial cells and fibroblasts, secrete a variety of proinflammatory mediators, including cytokines, chemokines, growth factors, and lipid mediators. Although most patients with COPD have a predominantly neutrophilic inflammation, some have an increase in eosinophil counts, which might be orchestrated by TH2 cells and type 2 innate lymphoid cells though release of IL-33 from epithelial cells. These patients might be more responsive to corticosteroids and bronchodilators. Oxidative stress plays a key role in driving COPD-related inflammation, even in ex-smokers, and might result in activation of the proinflammatory transcription factor nuclear factor κB (NF-κB), impaired antiprotease defenses, DNA damage, cellular senescence, autoantibody generation, and corticosteroid resistance though inactivation of histone deacetylase 2. Systemic inflammation is also found in patients with COPD and can worsen comorbidities, such as cardiovascular diseases, diabetes, and osteoporosis. Accelerated aging in the lungs of patients with COPD can also generate inflammatory protein release from senescent cells in the lung. In the future, it will be important to recognize phenotypes of patients with optimal responses to more specific therapies, and development of biomarkers that identify the therapeutic phenotypes will be important. PMID:27373322

  6. Microglia Play a Major Role in Direct Viral-Induced Demyelination

    Directory of Open Access Journals (Sweden)

    Dhriti Chatterjee

    2013-01-01

    Full Text Available Microglia are the resident macrophage-like populations in the central nervous system (CNS. Microglia remain quiescent, unable to perform effector and antigen presentation (APC functions until activated by injury or infection, and have been suggested to represent the first line of defence for the CNS. Previous studies demonstrated that microglia can be persistently infected by neurotropic mouse hepatitis virus (MHV which causes meningoencephalitis, myelitis with subsequent axonal loss, and demyelination and serve as a virus-induced model of human neurological disease multiple sclerosis (MS. Current studies revealed that MHV infection is associated with the pronounced activation of microglia during acute inflammation, as evidenced by characteristic changes in cellular morphology and increased expression of microglia-specific proteins, Iba1 (ionized calcium-binding adaptor molecule 1, which is a macrophage/microglia-specific novel calcium-binding protein and involved in membrane ruffling and phagocytosis. During chronic inflammation (day 30 postinfection, microglia were still present within areas of demyelination. Experiments performed in ex vivo spinal cord slice culture and in vitro neonatal microglial culture confirmed direct microglial infection. Our results suggest that MHV can directly infect and activate microglia during acute inflammation, which in turn during chronic inflammation stage causes phagocytosis of myelin sheath leading to chronic inflammatory demyelination.

  7. MicroRNA in human cancer and chronic inflammatory diseases.

    Science.gov (United States)

    Kanwar, Jagat R; Mahidhara, Ganesh; Kanwar, Rupinder K

    2010-06-01

    MicroRNAs (miRNAs) are the non-coding RNAs that act as post-translational regulators to their complimentary messenger RNAs (mRNA). Due to their specific gene silencing property, miRNAs have been implicated in a number of cellular and developmental processes. Also, it has been proposed that a particular set of miRNA spectrum is expressed only in a particular type of tissue. Many interesting findings related to the differential expression of miRNAs in various human diseases including several types of cancers, neurodegenerative diseases and metabolic diseases have been reported. Deregulation of miRNA expression in different types of human diseases and the roles various miRNAs play as tumour suppressors as well as oncogenes, suggest their contribution to cancer and/or in other disease development. These findings have possible implications in the development of diagnostics and/or therapeutics in human malignancies. In this review, we discuss various miRNAs that are differentially expressed in human chronic inflammatory diseases, neurodegenerative diseases, cancer and the further prospective development of miRNA based diagnostics and therapeutics.

  8. Sonography in the diagnosis of chronic inflammatory bowel disease

    International Nuclear Information System (INIS)

    The accuracy of ultrasonography (US) in diagnosing active inflammatory bowel disease (IBD) is assessed on the basis of a randomized prospective study of 61 patients. Twenty-six of the patients were affected with crohn's disease (CD) and 12 with ulcerative colitis, while the remaining 23 patients were control subjects with no specific chronic IBD. The US signs considered as a significant for active CD and UC were: -visualization of a typical target image, that is a hyperechoic center corresponding to luminal bowel content, surrounded by a hypoechoic ring corresponding to loop walls; -at least 2 of the following: solid abdominal mass, distended loops, luminal narrowing, reduced peristalsis, stiff loops, and accumulation of fluid between the loops. US sensitivity and specificity for CD were 77% and 95.6%, respectively. As for UC, no significant results were obtained. In our experiance, US is a reliable method for detecting alterations and, especially, comlpications typical of CD in its active phase. Considering the young age of patients affected with CD and the number of exams they must undergo, US is considered as a useful tool in disease follow-up

  9. Prevalence of cholelithiasis in patients with chronic inflammatory bowel disease

    Institute of Scientific and Technical Information of China (English)

    Wolfgang Kratzer; Mark M Haenle; Richard A Mason; Christian von Tirpitz; Volker Kaechele

    2005-01-01

    AIM: To investigate the effect of chronic inflammatory bowel disease (CIBD) specific risk factors for cholecystolithiasis,as duration and involvement pattern of the disease and prior surgery in patients with Crohn's disease (CD) and ulcerative colitis (UC).METHODS: A total of 222 patients with CD (135 females,87 males; average age, 35.8±11.8 years; range 17-81 years)and 88 patients with UC (39 females, 49 males; average age, 37.2±13.6 years; range 16-81 years) underwent clinical and ultrasound examinations. Besides age, sex and degree of obesity, patients' CIBD specific parameters, including duration and extent of disease and prior operations were documented and evaluated statistically using logistic regression.RESULTS: The overall prevalence of gallbladder stone disease in patients with CD was 13% (n = 30). Only age could be shown to be an independent risk factor (P = 0.014).Compared to a collective representative for the general population in the same geographic region, the prevalence of cholecystolithiasis was higher in all corresponding age groups. Patients with UC showed an overall prevalence of gallbladder stone disease of only 4.6%.CONCLUSION:Only age but not disease-specific factors such as duration and extent of disease, and prior surgery are independent risk factors for the development of cholecystolithiasis in patients with CIBD.

  10. Immunohistochemical analysis of carbohydrate antigens in chronic inflammatory gastrointestinal diseases.

    Science.gov (United States)

    Kobayashi, Motohiro; Nakayama, Jun

    2010-01-01

    Over the last four decades, immunohistochemistry (IHC) has become an invaluable technique to detect antigens in tissue sections. Compared to Western blotting analysis, IHC is advantageous in determining histological distribution and localization of the antigen. Another advantage, if one can access human formalin-fixed paraffin-embedded (FFPE) blocks of disease tissues, is that IHC makes it possible to analyze diseases retrospectively from archived pathological tissue specimens. In this chapter, we describe protocols used for both conventional and multiple immunostainings using FFPE tissue sections, which have been used for quantitative analysis of high endothelial venule (HEV)-like vessels and lymphocyte subsets attached to HEV-like vessels in our studies of chronic inflammatory gastrointestinal diseases. We also describe in detail a protocol using an L-selectinIgM chimera in situ binding assay on FFPE tissue sections for functional detection of L-selectin ligand carbohydrates expressed on HEV-like vessels. After presenting each protocol, we provide practical examples for its use obtained from our studies.

  11. Induction and Clinical Scoring of Chronic-Relapsing Experimental Autoimmune Encephalomyelitis

    OpenAIRE

    Beeton, Christine; Garcia, Adriana; Chandy, K. George

    2007-01-01

    Multiple sclerosis (MS) is a chronic inflammatory disease of the central nervous system (CNS) that commonly affects young adults. It is characterized by demyelination and glial scaring in areas disseminated in the brain and spinal cord. These lesions alter nerve conduction and induce the disabling neurological deficits that vary with the location of the demyelinated plaques in the CNS (e.g. paraparesis, paralysis, blindness, incontinence).

  12. Recognizing the Risks of Chronic Nonsteroidal Anti-Inflammatory Drug Use in Older Adults

    OpenAIRE

    Marcum, Zachary A.; Hanlon, Joseph T.

    2010-01-01

    Older adults commonly take nonsteroidal anti-inflammatory drugs (NSAIDs) chronically. Studies of older adults show that chronic NSAID use increases the risk of peptic ulcer disease, acute renal failure, and stroke/myocardial infarction. Moreover, chronic NSAID use can exacerbate a number of chronic diseases including heart failure and hypertension, and can interact with a number of drugs (eg, warfarin, corticosteroids). Preferred analgesics in older adults that may have a lower risk of these ...

  13. Contrasting Pattern of Chronic Inflammatory Bowel Disease in Primary and Autoimmune Sclerosing Cholangitis

    Directory of Open Access Journals (Sweden)

    Ingvar Bjarnason

    2015-10-01

    Interpretation: Collectively these findings lend support to the suggestion that the chronic inflammatory bowel disease associated with PSC and in particular AISC may represent a distinct nosologic entity different from classic ulcerative colitis and Crohn's disease.

  14. Solitary osteosclerotic plasmacytoma: association with demyelinating polyneuropathy and amyloid deposition

    Energy Technology Data Exchange (ETDEWEB)

    Voss, S.D.; Hall, F.M. [Dept. of Radiology, Beth Israel Deaconess Medical Center, Boston, MA (United States); Harvard Medical School, Boston, MA (United States); Murphey, M.D. [Dept. of Radiologic Pathology, Armed Forces Institute of Pathology, Washington, DC (United States); Dept. of Radiology and Nuclear Medicine, Uniformed Services University of the Health Sciences, Bethesda, MD (United States); Department of Radiology, University of Maryland School of Medicine, Baltimore, Maryland (United States)

    2001-09-01

    A 51-year-old man presented with a 1-year history of polyneuropathy necessitating the use of a wheelchair. Initial diagnosis was idiopathic chronic inflammatory demyelinating polyneuropathy (CIDP) and associated monoclonal gammopathy. Investigations for multiple myeloma, including bone marrow aspiration and biopsy, were negative. What was initially felt to be an incidental osteosclerotic focus noted on the radiographic bone survey was eventually shown to be a solitary osteosclereotic plasmacytoma with associated amyloid. This dramatically altered treatment. This case emphasizes the importance of including osteosclerotic plasmacytoma in the differential diagnosis of a focal sclerotic bone lesion in the clinical setting of polyneuropathy. These lesions are less likely to progress to multiple myeloma than lytic plasma cell neoplasms, and the presence of polyneuropathy often results in earlier diagnosis and treatment with enhanced prospect of cure. The finding of amyloid deposition within the osteosclerotic lesion may be of prognostic importance. (orig.)

  15. Chronic post-inflammatory fatigue in sarcoidosis : from cytokines to behavior

    NARCIS (Netherlands)

    Korenromp, I.H.E.

    2011-01-01

    Sarcoidosis is a systemic inflammatory disorder that is characterized by granuloma formation in different organs. Sarcoidosis patients frequently report fatigue. Even when the clinical symptoms of the inflammatory disease sarcoidosis have resolved, chronic fatigue may persist. In this study 75 patie

  16. Immunohistological evidence for a chronic intramyocardial inflammatory process in dilated cardiomyopathy.

    OpenAIRE

    Kühl, U.; Noutsias, M; Seeberg, B.; Schultheiss, H P

    1996-01-01

    OBJECTIVE: To determine whether immunohistochemical analysis of cardiac biopsies from patients presenting clinically as dilated cardiomyopathy (DCM) show a chronic inflammatory process. DESIGN: Comparative case control study. SETTING: Tertiary referral centre. PATIENTS: Biopsies from 170 patients with DCM and 85 control patients with other cardiac diseases. RESULTS: Nine patients had sufficient interstitial inflammatory cells to be called borderline myocarditis on conventional histology, leav...

  17. Chronic inflammatory systemic diseases: An evolutionary trade-off between acutely beneficial but chronically harmful programs.

    Science.gov (United States)

    Straub, Rainer H; Schradin, Carsten

    2016-01-01

    It has been recognized that during chronic inflammatory systemic diseases (CIDs) maladaptations of the immune, nervous, endocrine and reproductive system occur. Maladaptation leads to disease sequelae in CIDs. The ultimate reason of disease sequelae in CIDs remained unclear because clinicians do not consider bodily energy trade-offs and evolutionary medicine. We review the evolution of physiological supersystems, fitness consequences of genes involved in CIDs during different life-history stages, environmental factors of CIDs, energy trade-offs during inflammatory episodes and the non-specificity of CIDs. Incorporating bodily energy regulation into evolutionary medicine builds a framework to better understand pathophysiology of CIDs by considering that genes and networks used are positively selected if they serve acute, highly energy-consuming inflammation. It is predicted that genes that protect energy stores are positively selected (as immune memory). This could explain why energy-demanding inflammatory episodes like infectious diseases must be terminated within 3-8 weeks to be adaptive, and otherwise become maladaptive. Considering energy regulation as an evolved adaptive trait explains why many known sequelae of different CIDs must be uniform. These are, e.g. sickness behavior/fatigue/depressive symptoms, sleep disturbance, anorexia, malnutrition, muscle wasting-cachexia, cachectic obesity, insulin resistance with hyperinsulinemia, dyslipidemia, alterations of steroid hormone axes, disturbances of the hypothalamic-pituitary-gonadal (HPG) axis, hypertension, bone loss and hypercoagulability. Considering evolved energy trade-offs helps us to understand how an energy imbalance can lead to the disease sequelae of CIDs. In the future, clinicians must translate this knowledge into early diagnosis and symptomatic treatment in CIDs. PMID:26817483

  18. Canine distemper virus persistence in demyelinating encephalitis by swift intracellular cell-to-cell spread in astrocytes is controlled by the viral attachment protein

    OpenAIRE

    Wyss-Fluehmann, Gaby; Zurbriggen, Andreas; Vandevelde, Marc; Plattet, Philippe

    2010-01-01

    The mechanism of viral persistence, the driving force behind the chronic progression of inflammatory demyelination in canine distemper virus (CDV) infection, is associated with non-cytolytic viral cell-to-cell spread. Here, we studied the molecular mechanisms of viral spread of a recombinant fluorescent protein-expressing virulent CDV in primary canine astrocyte cultures. Time-lapse video microscopy documented that CDV spread was very efficient using cell processes contacting remote target ce...

  19. Paediatric UK demyelinating disease longitudinal study (PUDDLS

    Directory of Open Access Journals (Sweden)

    Likeman Marcus

    2011-07-01

    Full Text Available Abstract Background There is evidence that at least 5% of Multiple sclerosis (MS cases manifest in childhood. Children with MS present with a demyelinating episode involving single or multiple symptoms prior to developing a second event (usually within two years to then meet criteria for diagnosis. There is evidence from adult cohorts that the incidence and sex ratios of MS are changing and that children of immigrants have a higher risk for developing MS. A paediatric population should reflect the vanguard of such changes and may reflect trends yet to be observed in adult cohorts. Studying a paediatric population from the first demyelinating event will allow us to test these hypotheses, and may offer further valuable insights into the genetic and environmental interactions in the pathogenesis of MS. Methods/Design The Paediatric UK Demyelinating Disease Longitudinal Study (PUDDLS is a prospective longitudinal observational study which aims to determine the natural history, predictors and outcomes of childhood CNS inflammatory demyelinating diseases. PUDDLS will involve centres in the UK, and will establish a cohort of children affected with a first CNS inflammatory demyelinating event for long-term follow up by recruiting for approximately 5 years. PUDDLS will also establish a biological sample archive (CSF, serum, and DNA, allowing future hypothesis driven research. For example, the future discovery of a biomarker will allow validation within this dataset for the evaluation of novel biomarkers. Patients will also be requested to consent to be contacted in the future. A secondary aim is to collaborate internationally with the International Paediatric Multiple Sclerosis Study Group when future collaborative studies are proposed, whilst sharing a minimal anonymised dataset. PUDDLS is the second of two jointly funded studies. The first (UCID-SS is an epidemiological surveillance study that already received ethical approvals, and started on the 1st

  20. Prevalence of chronic diseases at the onset of inflammatory arthritis: a population-based study.

    NARCIS (Netherlands)

    Ursum, J.; Korevaar, J.C.; Twisk, J.W.R.; Peters, M.J.L.; Schellevis, F.G.; Nurmohamed, M.T.; Nielen, M.M.J.

    2013-01-01

    Objective. Little is known about the presence of chronic morbidity in inflammatory arthritis (IA) patients at disease onset. Previous studies have been mainly performed in established IA patients or they focus on isolated co-morbid diseases. Our aim was to determine the prevalence of chronic disease

  1. Use of nonsteroidal anti-inflammatory drugs prior to chronic renal replacement therapy initiation

    DEFF Research Database (Denmark)

    Fosbøl, Emil L; Kamper, Anne-Lise; Køber, Lars;

    2012-01-01

    PURPOSE: Nonsteroidal anti-inflammatory drugs (NSAIDs) may be associated with severe renal complications, including acute renal failure, reduced glomerular filtration rate and interstitial nephritis. Caution against NSAIDs is therefore recommended in advanced chronic kidney disease. In this study......, we examined NSAID use, aetiology and comorbidity among a national cohort of patients before the initiation of chronic renal replacement therapy (RRT). METHODS: Patients initiated on chronic RRT in the period 1997-2006 were identified in the Danish National Registry on Regular Dialysis...

  2. Anti-inflammatory and antioxidant effects of umbelliferone in chronic alcohol-fed rats

    OpenAIRE

    Sim, Mi-Ok; Lee, Hae-In; Ham, Ju Ri; Seo, Kwon-Il; Kim, Myung-Joo; Lee, Mi-Kyung

    2015-01-01

    BACKGROUND/OBJECTIVES Inflammation is associated with various types of acute and chronic alcohol liver diseases. In this study, we examined whether umbelliferone (7-hydroxycoumarin, UF) ameliorates chronic alcohol-induced liver damage by modulating inflammatory response and the antioxidant system. METHODS Rats were fed a Liber-Decarli liquid diet containing 5% alcohol with or without UF (0.05 g/L) for 8 weeks, while normal rats received an isocaloric carbohydrate liquid diet. RESULTS Chronic ...

  3. Obesity as a risk and severity factor in rheumatic diseases (Autoimmune Chronic inflammatory diseases-ACIDs

    Directory of Open Access Journals (Sweden)

    Elisa eGremese

    2014-11-01

    Full Text Available The growing body of evidence recognizing the adipose tissue as an active endocrine organ secreting bioactive mediators involved in metabolic and inflammatory disorders, together with the global epidemic of overweight and obesity, rise obesity as a hot topic of current research. The chronic state of low grade inflammation present in the obese condition and the multiple pleiotropic effects of adipokines on the immune system has been implicated in the pathogenesis of several inflammatory conditions including rheumatic autoimmune and inflammatory diseases. We will discuss the main relevant evidences on the role of the adipose tissue on immune and inflammatory networks and the more recent evidences regarding the effects of obesity on the incidence and outcomes of the major autoimmune chronic inflammatory diseases.

  4. Nodes of Ranvier and Paranodes in Chronic Acquired Neuropathies

    OpenAIRE

    Carmen Cifuentes-Diaz; Odile Dubourg; Theano Irinopoulou; Marc Vigny; Sylvie Lachkar; Laurence Decker; Patrick Charnay; Natalia Denisenko; Thierry Maisonobe; Jean-Marc Léger; Karine Viala; Jean-Jacques Hauw; Jean-Antoine Girault

    2011-01-01

    Chronic acquired neuropathies of unknown origin are classified as chronic inflammatory demyelinating polyneuropathies (CIDP) and chronic idiopathic axonal polyneuropathies (CIAP). The diagnosis can be very difficult, although it has important therapeutic implications since CIDP can be improved by immunomodulating treatment. The aim of this study was to examine the possible abnormalities of nodal and paranodal regions in these two types of neuropathies. Longitudinal sections of superficial per...

  5. Inflammatory biomarkers and comorbidities in chronic obstructive pulmonary disease

    DEFF Research Database (Denmark)

    Thomsen, Mette; Dahl, Morten; Lange, Peter;

    2012-01-01

    Patients with chronic obstructive pulmonary disease (COPD) have evidence of systemic inflammation that may be implicated in the development of comorbidities.......Patients with chronic obstructive pulmonary disease (COPD) have evidence of systemic inflammation that may be implicated in the development of comorbidities....

  6. STUDY ON INFLAMMATORY CELLS IN BALF OF SMOKE-INDUCED CHRONIC BRONCHITIS RAT MODEL

    Institute of Scientific and Technical Information of China (English)

    李庆云; 黄绍光; 吴华成; 程齐俭; 项轶; 万欢英

    2004-01-01

    Objective To establish a smoke-induced chronic bronchitis rat model and evaluate the pathological change semi-quantitatively, and study the characteristics of the inflammatory cells in the bronchoalveolar lavage fluid (BALF) in various stages. Methods Chronic bronchitis sequential rat model was established by passively inhaling smoke mixture. Experiments were performed in 30 young male Sprague-Dawley rats, which comprised 5 groups in random, i.e.,4 chronic bronchitis model groups and I control group. After stained with hematoxylin and eosin, the specimens were studied by semi-quantitative method to evaluate the morphologic changes in various stages. Meanwhile, the inflammatory cells of the BALF and the activity of myeloperoxidase ( MPO ) of lung tissue were analysed. Results During the process of the chronic bronchitis, the pathologic score was increasing as time went on, and the typical morphologic changes of chronic bronchitis emerged in the group 7 weeks. The total number of inflammatory cells in BALF was increasing as time went on, correlated with the pathologic scores ( P < 0. 01 ).And the percentage of lymphocyte increased as well as positively correlated with pathologic scores ( P < 0. 05 ),whereas that of macrophage decreased and negatively correlated with pathologic scores (P <0. 05). The MPO lever of lung tissue was correlated with the pathologic scores ( P < 0. 01 ). But the percentage of the neutrophil in the BALF was just in a high level during the first week, then it maintained relatively lower. Conclusion Smoke-induced chronic bronchitis is a slowly progressive inflammation process. The model we established is convenient and simple for the longitudinal study on the inflammatory process of chronic bronchitis and the therapy in the early stage. The semi-quantitative evaluation for the pathological change is with much more value. During the inflammatory sequential process of early stage of chronic bronchitis, the cellular characteristics are

  7. The role of axonopathy in the mechanisms of development of demyelination processes in the central and peripheral nervous system.

    Science.gov (United States)

    Merkulov, Yu A; Zavalishin, I A; Merkulova, D M

    2009-01-01

    The role of axonopathy in the development of demyelinating processes in the CNS and peripheral nervous system was addressed in studies of 43 patients with multiple sclerosis (MS) and 144 patients with chronic inflammatory demyelinating polyneuropathy (CIDPN). Patients with MS were found to have foci of reduced MRI intensity in the T1 regime ("black holes," present in 28%) and regional atrophy of the cerebral cortex (in 46%), which showed a significant association with the degree of invalidity on the EDSS (Kendall tau = 0.38 and 0.43; p = 0.038 and 0.021, respectively). The mean fatigue score on the FSS was 4.9 (3.6; 5.4). A significant increase in the central conduction time on the background of fatigue (p = 0.016), along with an absence of signs of impaired reliability of neuromuscular transmission and an absence of past-activation phenomena, suggested that central mechanisms were predominant in the formation of fatigue phenomena in MS. In addition, 34.9% of patients with MS showed signs of peripheral nervous system involvement, while the clinical-electrophysiological pattern in 12.5% of patients with CIDPN showed signs of CNS involvement. These data widen existing concepts of the mechanisms of formation of axonopathy in the CNS, based on evidence for the development of axon-demyelinating processes in CIDPN, which is the most accessible model of demyelination for study using contemporary neurophysiological methods. PMID:19089637

  8. PPARγ as a Potential Target to Treat Airway Mucus Hypersecretion in Chronic Airway Inflammatory Diseases

    Directory of Open Access Journals (Sweden)

    Yongchun Shen

    2012-01-01

    Full Text Available Airway mucus hypersecretion (AMH is a key pathophysiological feature of chronic airway inflammatory diseases such as bronchial asthma, cystic fibrosis, and chronic obstructive pulmonary disease. AMH contributes to the pathogenesis of chronic airway inflammatory diseases, and it is associated with reduced lung function and high rates of hospitalization and mortality. It has been suggested that AMH should be a target in the treatment of chronic airway inflammatory diseases. Recent evidence suggests that a key regulator of airway inflammation, hyperresponsiveness, and remodeling is peroxisome proliferator-activated receptor gamma (PPARγ, a ligand-activated transcription factor that regulates adipocyte differentiation and lipid metabolism. PPARγ is expressed in structural, immune, and inflammatory cells in the lung. PPARγ is involved in mucin production, and PPARγ agonists can inhibit mucin synthesis both in vitro and in vivo. These findings suggest that PPARγ is a novel target in the treatment of AMH and that further work on this transcription factor may lead to new therapies for chronic airway inflammatory diseases.

  9. IFNγ Influences Type I Interferon Response and Susceptibility to Theiler's Virus-Induced Demyelinating Disease

    OpenAIRE

    Bowen, Jenna L.; Olson, Julie K.

    2013-01-01

    Theiler's murine encephalomyelitis virus (TMEV) induces a demyelinating disease in susceptible SJL mice that has similarities to multiple sclerosis in humans. TMEV infection of susceptible mice leads to a persistent virus infection of the central nervous system (CNS), which promotes the development of demyelinating disease associated with an inflammatory immune response in the CNS. TMEV infection of resistant C57BL6 mice results in viral clearance without development of demyelinating disease....

  10. Demyelinizing neurological disease after treatment with tumor necrosis factor alpha-inhibiting agents in a rheumatological outpatient clinic

    DEFF Research Database (Denmark)

    Theibich, Ali; Dreyer, Lene; Magyari, Melinda;

    2014-01-01

    multiple sclerosis (MS) have been described as a rare AE. During about 10-year use of anti TNF-alpha, the Danish Medicines Agency has recorded eight cases of MS like AEs. The objective of this study was to estimate the incidence of demyelinizing AEs both in the central and peripheral nervous system after......Biological treatment with inhibitors of the pro-inflammatory cytokine TNF-alpha has dramatically improved the disease course of several chronic rheumatologic conditions. Adverse events (AEs) are primarily infections and hypersensitivity reactions. Demyelinizing neurological symptoms resembling...... treatment with anti TNF-alpha in a cohort of patients from a large rheumatologic outpatient clinic in Copenhagen. In a 4-year period from January 2008 to December 2011, approximately 550 patients annually were undergoing treatment with anti TNF-alpha inhibitors in our department. We collected data on all...

  11. 急、慢性炎症性脱髓鞘性多发性神经病神经电生理对比研究%Electrophysiological features of acute inflammatory demyelinating polyneuropathy and chronic inflammatory demyelinating polyneuropathy

    Institute of Scientific and Technical Information of China (English)

    赵东红; 王可人; 朱丹; 赵东辉; 叶玉琴

    2013-01-01

    目的 比较分析急性炎症性脱髓鞘性多发性神经病(AIDP)与慢性炎症性脱髓鞘性多发性神经病(CIDP)的电生理表现.方法 收集2011年1月~2013年1月在吉林大学白求恩第一医院神经内科就诊的19例AIDP患者及15例CIDP患者,分析上下肢周围神经传导检查各项指标.结果 AIDP与CIDP均表现为运动传导速度(MCV)减慢、远端潜伏期延长、波幅降低、传导阻滞、F波及H反射异常,但CIDP组MCV减慢明显,与AIDP组存在显著差异,且CIDP组感觉传导检测异常明显,AIDP组感觉神经传导异常少见.结论 AIDP患者主要以周围神经运动纤维受损为主,存在明显的脱髓鞘及轴索的损伤,但周围神经感觉纤维受损不明显.CIDP患者周围神经运动纤维及感觉纤维受损均非常明显,且脱髓鞘程度明显重于AIDP患者.

  12. Mesenchymal stromal cells and chronic inflammatory bowel disease.

    Science.gov (United States)

    Algeri, M; Conforti, A; Pitisci, A; Starc, N; Tomao, L; Bernardo, M E; Locatelli, F

    2015-12-01

    Recent experimental findings have shown the ability of mesenchymal stromal cells (MSCs) to home to damaged tissues and to produce paracrine factors with anti-inflammatory properties, potentially resulting in reduction of inflammation and functional recovery of the damaged tissues. Prompted by these intriguing properties and on the basis of encouraging preclinical data, MSCs are currently being studied in several immune-mediated disorders. Inflammatory bowel diseases (IBD) represent a setting in which MSCs-based therapy has been extensively investigated. Phase I and II studies have documented the safety and feasibility of MSCs. However, efficacy results have so far been conflicting. In this review, we will discuss the biologic rationale that makes MSCs a promising therapeutic tool for IBD, and analyze recent experimental and clinical findings, highlighting current limitations and future perspectives of MSCs-related immunotherapy for IBD. PMID:26170204

  13. Atherosclerosis: a chronic inflammatory disease mediated by mast cells.

    Science.gov (United States)

    Conti, Pio; Shaik-Dasthagirisaeb, Yazdami

    2015-01-01

    Inflammation is a process that plays an important role in the initiation and progression of atherosclerosis and immune disease, involving multiple cell types, including macrophages, T-lymphocytes, endothelial cells, smooth muscle cells and mast cells. The fundamental damage of atherosclerosis is the atheromatous or fibro-fatty plaque which is a lesion that causes several diseases. In atherosclerosis the innate immune response, which involves macrophages, is initiated by the arterial endothelial cells which respond to modified lipoproteins and lead to Th1 cell subset activation and generation of inflammatory cytokines and chemoattractant chemokines. Other immune cells, such as CD4+ T inflammatory cells, which play a critical role in the development and progression of atherosclerosis, and regulatory T cells [Treg], which have a protective effect on the development of atherosclerosis are involved. Considerable evidence indicates that mast cells and their products play a key role in inflammation and atherosclerosis. Activated mast cells can have detrimental effects, provoking matrix degradation, apoptosis, and enhancement as well as recruitment of inflammatory cells, which actively contributes to atherosclerosis and plaque formation. Here we discuss the relationship between atherosclerosis, inflammation and mast cells. PMID:26648785

  14. Targeted anti-inflammatory therapeutics in asthma and chronic obstructive lung disease

    OpenAIRE

    Durham, Andrew L.; Caramori, Gaetano; Chung, Kian F; Adcock, Ian M.

    2016-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are chronic inflammatory diseases of the airway, although the drivers and site of the inflammation differ between diseases. Asthmatics with a neutrophilic airway inflammation are associated with a poor response to corticosteroids, whereas asthmatics with eosinophilic inflammation respond better to corticosteroids. Biologicals targeting the Th2-eosinophil nexus such as anti–interleukin (IL)-4, anti–IL-5, and anti–IL-13 are ineffective in ...

  15. THE MICROBIOLOGICAL EFFICACY ESTIMATION OF DIFFERENT TYPES OF CHRONIC PERIODONTITIS’ ANTI-INFLAMMATORY THERAPY

    Directory of Open Access Journals (Sweden)

    O.B. Ryba

    2008-09-01

    Full Text Available The article deals with microbiological status of patients with chronic generalized periodontitis of medium severity. On the basis of clinical and microbiological data the analysis of different methods efficacy of anti-inflammatory therapy was carried out. We studied antimicrobial effect of laser therapy, ozonotherapyandcombinations oflaser- ozonotherapyin comparison with influence ofchlorhexidine 0,2%. Combined laser and ozone influence on periodontium provided high antibacterial effect with increased local nonspecific resistance, and it extended remission term of patients with chronic periodontitis.

  16. Cell replacement therapies to promote remyelination in a viral model of demyelination

    OpenAIRE

    Tirotta, Emanuele; Carbajal, Kevin S.; Schaumburg, Chris S; Whitman, Lucia; Lane, Thomas E

    2010-01-01

    Persistent infection of the central nervous system (CNS) of mice with the neuroadapted JHM strain of mouse hepatitis (MHV) is characterized by ongoing demyelination mediated by inflammatory T cells and macrophages that is similar both clinically and histologically with the human demyelinating disease multiple sclerosis (MS).

  17. Enhanced B7 costimulatory molecule expression in inflammatory human sural nerve biopsies

    OpenAIRE

    Kiefer, R.; Dangond, F; M. Mueller; TOYKA, K.; Hafler, D; Hartung, H

    2000-01-01

    OBJECTIVES—To define the role of the costimulatory molecules B7-1 and B7-2 in inflammatory disorders of the peripheral nervous system. B7 molecules are essential for effective antigen presentation and may determine the differentiation of T cells into a Th-1 or Th-2 phenotype, thus modulating immune response and disease course.
METHODS—Forty nine sural nerve biopsies from patients with neuroborreliosis, Guillain-Barré syndrome (GBS), chronic inflammatory demyelinating poly...

  18. Blood Dendritic Cells: Canary in the Coal Mine to Predict Chronic Inflammatory Disease?

    Directory of Open Access Journals (Sweden)

    Brodie eMiles

    2014-01-01

    Full Text Available The majority of risk factors for chronic inflammatory diseases are unknown. This makes personalized medicine for assessment, prognosis, and choice of therapy very difficult. It is becoming increasingly clear, however, that low-grade subclinical infections may be an underlying cause of many chronic inflammatory diseases and thus may contribute to secondary outcomes (e.g. cancer. Many diseases are now categorized as inflammatory-mediated diseases that stem from a dysregulation in host immunity. There is a growing need to study the links between low-grade infections, the immune responses they elicit, and how this impacts overall health. One such link explored in detail here is the extreme sensitivity of myeloid dendritic cells (mDC in peripheral blood to chronic low-grade infections and the role that these mDCs play in arbitrating the resulting immune responses. We find that emerging evidence supports a role for pathogen-induced mDCs in chronic inflammation leading to increased risk of secondary clinical disease. The mDCs that are elevated in the blood as a result of low-grade bacteremia often do not trigger a productive immune response, but can disseminate the pathogen throughout the host. This aberrant trafficking of mDCs can accelerate systemic inflammatory disease progression. Conversely, restoration of DC homeostasis may aid in pathogen elimination and minimize dissemination. Thus it would seem prudent when assessing chronic inflammatory disease risk to consider blood mDC numbers, and the microbial content (microbiome and activation state of these mDCs. These may provide important clues (the canary in the coal mine of high inflammatory disease risk. This will facilitate development of novel immunotherapies to eliminate such smoldering infections in atherosclerosis, cancer, rheumatoid arthritis, and pre-eclampsia.

  19. MRI and MRS diagnosis of single acute inflammatory demyelinating disease of the brain Value Analysis%MRI及MRS诊断单发急性炎性脑脱髓鞘疾病的应用价值探析

    Institute of Scientific and Technical Information of China (English)

    杜碧茵

    2014-01-01

    Objective Study investigated the characteristics and MRI imaging single acute inflammatory demyelinating disease of the brain, MRS applications in disease diagnosis. Methods Admitted to our hospital in recent years, single acute inflammatory demyelinating disease of the brain in patients with nine cases for the study, the basic clinical data and imaging findings were retrospectively analyzed patients and analyzed for signs of central nervous system imaging discuss its clinical characteristic. Results By MRI diagnosis of basal ganglia lesions in one case, the white matter is located eight cases, the lesion edges smooth, round shape rules, hierarchy typical. DWI and FLIAR, ADC figure are low signal lesion center, the surrounding high signal;T1WI center of low signal, T2WI high signal center. Through enhanced scan showed irregular lesions strengthening the open-loop and no significant mass effect. After MRS diagnosis, al patients had lesions in the central region increased Cho and NAA peak lower peak performance. Three patients had a peak increase in mI, 5 patients had lower Cr peak condition. Review of al patients seen by the relevant treatment lesion volume, area shrink, Cho and NAA peak reduce peak recovery. Conclusion Patients with single acute inflammatory demyelinating disease of the brain detected by MRI, the lesion can be clearly observed in the location, number, morphology, signal characteristics, such as MRI performance, but also can accurately display the NAA peak in the MRS diagnosis, Cho peak, changes Cr peak, mI peak, to help doctors accurately diagnose and determine the progress of the disease in patients from the characteristic radiographic signs of.%目的:研究探讨单发急性炎性脑脱髓鞘疾病的影像学特征及MRI、MRS在疾病诊断中的应用价值。方法选取我院近年来收治的单发急性炎性脑脱髓鞘疾病患者9例作为研究对象,回顾性分析患者的基本临床资料和影像学检查结果,并对其

  20. Anti-proline-glycine-proline or antielastin autoantibodies are not evident in chronic inflammatory lung disease.

    LENUS (Irish Health Repository)

    Greene, Catherine M

    2010-01-01

    In patients with chronic inflammatory lung disease, pulmonary proteases can generate neoantigens from elastin and collagen with the potential to fuel autoreactive immune responses. Antielastin peptide antibodies have been implicated in the pathogenesis of tobacco-smoke-induced emphysema. Collagen-derived peptides may also play a role.

  1. Interaction of inflammatory cytokines and erythropoeitin in iron metabolism and erythropoiesis in anaemia of chronic disease

    NARCIS (Netherlands)

    M. Jongen-Lavrencic (Mojca); H.R.M. Peeters (H. R M); G. Vreugdenhil (Gerard); A.J.G. Swaak (Antonius)

    1995-01-01

    textabstractIn chronic inflammatory conditions increased endogenous release of specific cytokines (TNFα, IL-1, IL-6, IFNγ and others) is presumed. It has been shown that those of monocyte lineage play a key role in cytokine expression and synthesis. This may be associated with changes in iron metabo

  2. Early severe inflammatory responses to uropathogenic E. coli predispose to chronic and recurrent urinary tract infection.

    Directory of Open Access Journals (Sweden)

    Thomas J Hannan

    Full Text Available Chronic infections are an increasing problem due to the aging population and the increase in antibiotic resistant organisms. Therefore, understanding the host-pathogen interactions that result in chronic infection is of great importance. Here, we investigate the molecular basis of chronic bacterial cystitis. We establish that introduction of uropathogenic E. coli (UPEC into the bladders of C3H mice results in two distinct disease outcomes: resolution of acute infection or development of chronic cystitis lasting months. The incidence of chronic cystitis is both host strain and infectious dose-dependent. Further, development of chronic cystitis is preceded by biomarkers of local and systemic acute inflammation at 24 hours post-infection, including severe pyuria and bladder inflammation with mucosal injury, and a distinct serum cytokine signature consisting of elevated IL-5, IL-6, G-CSF, and the IL-8 analog KC. Mice deficient in TLR4 signaling or lymphocytes lack these innate responses and are resistant, to varying degrees, to developing chronic cystitis. Treatment of C3H mice with the glucocorticoid anti-inflammatory drug dexamethasone prior to UPEC infection also suppresses the development of chronic cystitis. Finally, individuals with a history of chronic cystitis, lasting at least 14 days, are significantly more susceptible to redeveloping severe, chronic cystitis upon bacterial challenge. Thus, we have discovered that the development of chronic cystitis in C3H mice by UPEC is facilitated by severe acute inflammatory responses early in infection, which subsequently are predisposing to recurrent cystitis, an insidious problem in women. Overall, these results have significant implications for our understanding of how early host-pathogen interactions at the mucosal surface determines the fate of disease.

  3. Dissociated sterol-based liver X receptor agonists as therapeutics for chronic inflammatory diseases.

    Science.gov (United States)

    Yu, Shan; Li, Sijia; Henke, Adam; Muse, Evan D; Cheng, Bo; Welzel, Gustav; Chatterjee, Arnab K; Wang, Danling; Roland, Jason; Glass, Christopher K; Tremblay, Matthew

    2016-07-01

    Liver X receptor (LXR), a nuclear hormone receptor, is an essential regulator of immune responses. Activation of LXR-mediated transcription by synthetic agonists, such as T0901317 and GW3965, attenuates progression of inflammatory disease in animal models. However, the adverse effects of these conventional LXR agonists in elevating liver lipids have impeded exploitation of this intriguing mechanism for chronic therapy. Here, we explore the ability of a series of sterol-based LXR agonists to alleviate inflammatory conditions in mice without hepatotoxicity. We show that oral treatment with sterol-based LXR agonists in mice significantly reduces dextran sulfate sodium colitis-induced body weight loss, which is accompanied by reduced expression of inflammatory markers in the large intestine. The anti-inflammatory property of these agonists is recapitulated in vitro in mouse lamina propria mononuclear cells, human colonic epithelial cells, and human peripheral blood mononuclear cells. In addition, treatment with LXR agonists dramatically suppresses inflammatory cytokine expression in a model of traumatic brain injury. Importantly, in both disease models, the sterol-based agonists do not affect the liver, and the conventional agonist T0901317 results in significant liver lipid accumulation and injury. Overall, these results provide evidence for the development of sterol-based LXR agonists as novel therapeutics for chronic inflammatory diseases.-Yu, S., Li, S., Henke, A., Muse, E. D., Cheng, B., Welzel, G., Chatterjee, A. K., Wang, D., Roland, J., Glass, C. K., Tremblay, M. Dissociated sterol-based liver X receptor agonists as therapeutics for chronic inflammatory diseases. PMID:27025962

  4. [The application of "preventive treatment theory" in chronic airway inflammatory disease].

    Science.gov (United States)

    Dong, Jing-Cheng; Liu, Bao-Jun; Zhang, Hong-Ying

    2013-07-01

    Bronchial asthma and chronic obstructive pulmonary disease (COPD), as chronic airway inflammatory diseases, seriously threaten the health of human beings. Chinese medicine has obvious advantages in prevention and treatment of them. "Preventive treatment theory" is a sort summarization of preventive medicine in Chinese medicine. The theory is not only reflected at the disease prevention levels, also embodied in the active treatment and the rehabilitation process. It was especially deep and colorfully embodied in the prevention and treatment of chronic airway inflammatory diseases such as asthma and COPD. In this paper,clarified were the prevention and treatment targets, ways of thinking and methods in different stages of asthma and COPD from various viewpoints including prevention before disease occurrence, treating disease at disease onset, preventing the aggravation once disease occurs, and consolidation after disease occurs. We hope to improve ways of thinking and prevention and treatment levels of bronchial asthma and COPD by Chinese medicine. PMID:24063226

  5. The Central Role of the Gut Microbiota in Chronic Inflammatory Diseases

    Directory of Open Access Journals (Sweden)

    Caroline Marcantonio Ferreira

    2014-01-01

    Full Text Available The commensal microbiota is in constant interaction with the immune system, teaching immune cells to respond to antigens. Studies in mice have demonstrated that manipulation of the intestinal microbiota alters host immune cell homeostasis. Additionally, metagenomic-sequencing analysis has revealed alterations in intestinal microbiota in patients suffering from inflammatory bowel disease, asthma, and obesity. Perturbations in the microbiota composition result in a deficient immune response and impaired tolerance to commensal microorganisms. Due to altered microbiota composition which is associated to some inflammatory diseases, several strategies, such as the administration of probiotics, diet, and antibiotic usage, have been utilized to prevent or ameliorate chronic inflammatory diseases. The purpose of this review is to present and discuss recent evidence showing that the gut microbiota controls immune system function and onset, development, and resolution of some common inflammatory diseases.

  6. 瘤样炎性脱髓鞘病临床影像特点%The clinical features, neuroimaging findings and pathological characteristics of 26 patients with pathologically proven tumor-like inflammatory demyelinating diseases

    Institute of Scientific and Technical Information of China (English)

    戚晓昆; 刘建国; 钱海蓉; 邱峰; 姚生; 李长青; 王亚明

    2010-01-01

    目的 总结经病理证实的26例瘤样炎性脱髓鞘病(TIDD)临床、影像及病理特点以期提高诊治水平.方法 对24例脑型和2例脊髓型TIDD的临床、影像及病理资料进行回顾性分析.结果 26例(男14例、女12例)患者发病年龄6~69(36.7±13.8)岁.3例失访,2例死亡.TIDD首发以头痛多见,其次为淡漠伴记忆力减退4例.病变以双侧受累及多发病灶最为多见.22例行脑CT示病灶均为低密度.MRI上呈片状长T1、长T2信号,呈开环形或闭合环形强化;病理除炎性脱髓鞘表现外,少数可见核分裂状的Creutzfeuldt细胞.脑脊液寡克隆带(OCB)阳性率(72.2%)及髓鞘碱性蛋白(MBP)异常率(77.8%)较高.结论 TIDD为特殊类型的脱髓鞘病,虽与肿瘤相似,但其病灶以双侧、多发且彼此孤立,CT为低密度,若示高密度基本可除外TIDD;脑脊液OCB及MBP检查对TIDD有价值.%Objective To summarize the clinical features, neuroimaging findings and pathological characteristics of 26 patients with tumor-like inflammatory demyelinating diseases (TIDD) confirmed by histopathology for better diagnosis and differential diagnosis. Methods The clinical features, neuroimaging findings and pathological characteristics of 26 patients (14 male, 12 female) with pathologically proven TIDD(24 brain-type and 2 spinal cord-type ) were retrospectively analysed. Results The mean onset age was 6-69 (36.7±13.8) years. Twenty-one patients had good prognosis with a median followed-up duration of 51.0 months. Two patients were died of post-operative complication and pulmonary infection respectively and the remaining 3 patients were lost to followed up. The TIDD patients almost showed monophasic clinical setting. Headache, indifference accompanied with hypomnesis were the commonest initial symptoms. The positive or abnormol rates of cerebrospinal fluid oligoclonal bands (OCB) and myelin basic protein (MBP)in TIDD patients were high. The involvements of bilateral and multi

  7. Chronic inflammatory disorders of the gastrointestinal tract of companion animals.

    Science.gov (United States)

    Cave, N J

    2003-12-01

    In the inflammatory bowel diseases (IBD) that affect dogs and cats there appears to be dysregulation of normal mucosal immunity, characterised by polyclonal lymphocytic infiltrates which are presumably specific for luminal antigens. There is an absence of a classical polarisation of either T-helper (Th) 1 or Th2 cytokine responses, although increased expression of mRNA for interleukin (IL) 2 and IL-12p40 and a shift towards mucosal immunoglobulin (Ig) G production are consistent findings, whilst variable responses are seen in tumour necrosis factor-alpha (TNF-alpha), IL-1, IL-4, IL-6, and interferon-gamma (IFN-gamma). Increased mucosal permeability and deranged intestinal motility are common sequelae. Despite obvious similarities with Crohn's disease and ulcerative colitis in humans, important differences exist. Of these, the diffuse superficial nature but with no Th1 or Th2 bias, and the prevalence of proximal small intestinal disease are notable. Potential hypotheses for these disparities include specific differences in the types or locations of agonistic gut flora, diffuse abnormalities in microbial-host interactions, a greater importance of diet, or anatomical or cellular differences in mucosal immune responses. Although specific pathogens and genetic susceptibilities may be involved, quantitative or qualitative changes in the normal flora or abnormal responses to a normal flora are more likely to be involved in the immunopathogenesis. Dietary influences include a large source of antigen, promotion of abnormal microbial growth through Maillard compounds within canned diets, and specific macro- and micronutrient deficiencies. Although dependent on a histopathological diagnosis, limitations of biopsies procured endoscopically, lack of histopathological standardisation and difficulty distinguishing inflammation from neoplasia remain significant problems. Clinician-pathologist dialogue, immunohistochemistry, cytokine profiling and lymphocyte clonality assessment

  8. Dietary resistant starch and chronic inflammatory bowel diseases.

    Science.gov (United States)

    Jacobasch, G; Schmiedl, D; Kruschewski, M; Schmehl, K

    1999-11-01

    These studies were performed to test the benefit of resistant starch on ulcerative colitis via prebiotic and butyrate effects. Butyrate, propionate, and acetate are produced in the colon of mammals as a result of microbial fermentation of resistant starch and other dietary fibers. Butyrate plays an important role in the colonic mucosal growth and epithelial proliferation. A reduction in the colonic butyrate level induces chronic mucosal atrophy. Short-chain fatty acid enemas increase mucosal generation, crypt length, and DNA content of the colonocytes. They also ameliorate symptoms of ulcerative colitis in human patients and rats injected with trinitrobenzene sulfonic acid (TNBS). Butyrate, and also to a lesser degree propionate, are substrates for the aerobic energy metabolism, and trophic factors of the colonocytes. Adverse butyrate effects occur in normal and neoplastic colonic cells. In normal cells, butyrate induces proliferation at the crypt base, while inhibiting proliferation at the crypt surface. In neoplastic cells, butyrate inhibits DNA synthesis and arrests cell growth in the G1 phase of the cell cycle. The improvement of the TNBS-induced colonic inflammation occurred earlier in the resistant starch (RS)-fed rats than in the RS-free group. This benefit coincided with activation of colonic epithelial cell proliferation and the subsequent restoration of apoptosis. The noncollagenous basement membrane protein laminin was regenerated initially in the RS-fed group, demonstrating what could be a considered lower damage to the intestinal barrier function. The calculation of intestinal short-chain fatty acid absorption confirmed this conclusion. The uptake of short-chain fatty acids in the colon is strongly inhibited in the RS-free group, but only slightly reduced in the animals fed with RS. Additionally, RS enhanced the growth of intestinal bacteria assumed to promote health. Further studies involving patients suffering from ulcerative colitis are necessary to

  9. Appearance of attenuated intestinal polyposis during chronic non-steroidal anti-inflammatory drugs use

    Institute of Scientific and Technical Information of China (English)

    Hugh; James; Freeman

    2012-01-01

    Aspirin and non-steroidal anti-inflammatory drugs (NSAIDS) may prevent sporadic colonic neoplasia and reduce the polyp burden in familial adenomatous polyposis. A 41-year-old pharmacologist with no family history of intestinal polyps or cancer chronically consumed daily aspirin and other non-steroidal anti-inflammatory drugs for decades despite recurrent and multiple gastric ulcers. A cancerous polyp in the colon was endoscopically resected. Over the next 2 decades, almost 50 adenomatous polyps were removed from the rest of his colon and duodenum, typical of an attenuated form of adenomatous polyposis. Chronic and habitual use of aspirin or NSAIDS may have important significance in delaying the appearance of adenomas. The observations here emphasize the important implications for clinical risk assessment in screening programs designed to detect or prevent colon cancer.

  10. Fulminant Demyelinating Diseases of the Central Nervous System.

    Science.gov (United States)

    Bevan, Carolyn J; Cree, Bruce A

    2015-12-01

    Fulminant demyelinating diseases of the central nervous system include acute disseminated encephalomyelitis, the related acute hemorrhagic leukoencephalitis, multiple sclerosis variants, neuromyelitis optica spectrum disorders, and idiopathic transverse myelitis. These syndromes are often managed with similar acute treatments including high-dose corticosteroids and plasmapheresis; however, long-term management varies. Although the prognosis of fulminant demyelinating disease was historically poor, outcomes today may be improved due to earlier diagnosis, rapid implementation of anti-inflammatory therapies such as high-dose corticosteroids and plasmapheresis, and improved supportive care. PMID:26595866

  11. Vitamin D deficiency in chronic inflammatory rheumatic diseases: results of the cardiovascular in rheumatology [CARMA] study

    OpenAIRE

    Urruticoechea Arana, Ana; Martín Martínez, María A.; Castañeda Sanz, Santos; Sánchez Piedra, Carlos A.; González Juanatey, Carlos; Llorca Díaz, Francisco Javier; Díaz González, Federico; González-Gay Mantecón, Miguel Ángel; CARMA Project Collaborative Group

    2015-01-01

    Introduction The aim was to study the association between 25-hydroxyvitamin D (25(OH)D) levels and the clinical characteristics of patients with chronic inflammatory rheumatic diseases (CIRD). Methods We studied a cross-section from the baseline visit of the CARMA project (CARdiovascular in rheuMAtology), a 10-year prospective study evaluating the risk of cardiovascular events in rheumatoid arthritis (RA), ankylosing spondylitis (AS) and psoriatic arthritis (PsA) patients, and non-CIRD patien...

  12. Systemic Inflammatory Response to Smoking in Chronic Obstructive Pulmonary Disease: Evidence of a Gender Effect

    OpenAIRE

    Faner, Rosa; Gonzalez, Nuria; Cruz, Tamara; Kalko, Susana Graciela; Agustí, Alvar

    2014-01-01

    Background Tobacco smoking is the main risk factor of chronic obstructive pulmonary disease (COPD) but not all smokers develop the disease. An abnormal pulmonary and systemic inflammatory response to smoking is thought to play a major pathogenic role in COPD, but this has never been tested directly. Methods We studied the systemic biomarker and leukocyte transcriptomic response (Affymetrix microarrays) to smoking exposure in 10 smokers with COPD and 10 smokers with normal spirometry. We also ...

  13. Effect of Probiotic Consortium on the Local Inflammatory Process in Chronic Periodontitis

    OpenAIRE

    Zhanagul Khasenbekova; Saule Saduakhasova; Alexandr Gulayev; Almagul Kushugulova; Samat Kozhakhmetov; Gulnara Shakhabayeva; Indira Tynybayeva; Talgat Nurgozhin; Zhaxybay Zhumadilov

    2014-01-01

    Introduction: Inflammatory periodontal disease is one of the major concerns of researchers and clinicians, because it can lead to tooth loss and an increased risk of systemic pathologies, even at the age of 35. The purpose of this study was to determine the effects of gelatin-based probiotic consortium on the local and general factors of inflammation in rats with chronic periodontitis. Methods: The study object was a complex of probiotic bacteria based in an odourless 6% gelatin plate with ne...

  14. Impaired clearance of apoptotic cells in chronic inflammatory diseases: therapeutic implications

    Directory of Open Access Journals (Sweden)

    Zsuzsa eSzondy

    2014-08-01

    Full Text Available In healthy individuals billions of cells die by apoptosis every day. Removal of the dead cells by phagocytosis (a process called efferocytosis must be efficient to prevent secondary necrosis and the consequent release of proinflammatory cell contents that damages the tissue environment and provokes autoimmunity. In addition, detection and removal of apoptotic cells generally induces an anti-inflammatory response. As a consequence improper clearance of apoptotic cells, being the result of either genetic anomalies and /or a persistent disease state, contributes to the establishment and progression of a number of human chronic inflammatory diseases such as autoimmune and neurological disorders, inflammatory lung diseases, obesity, type 2 diabetes or atherosclerosis. During the past decade our knowledge about the mechanism of efferocytosis has significantly increased, providing therapeutic targets through which impaired phagocytosis of apoptotic cells and the consequent inflammation could be influenced in these diseases.

  15. Virus-Specific Antibody, in the Absence of T Cells, Mediates Demyelination in Mice Infected with a Neurotropic Coronavirus

    OpenAIRE

    Kim, Taeg S.; Perlman, Stanley

    2005-01-01

    Mice infected with mouse hepatitis virus strain JHM develop an inflammatory demyelinating disease in the central nervous system with many similarities to human multiple sclerosis. The mouse disease is primarily immune-mediated because demyelination is not detected in JHM-infected mice lacking T or B cells but does occur after transfer of JHM-specific T cells. Although less is known about the ability of antibodies to mediate demyelination, the presence of oligoclonally expanded B cells and hig...

  16. SPECT radiopharmaceuticals for imaging chronic inflammatory diseases in the last decade

    International Nuclear Information System (INIS)

    In the recent years, many radiopharmaceuticals have been described for the diagnosis of inflammatory chronic diseases. Several peptides, receptor ligands and monoclonal antibodies have been radiolabelled, allowing in-vivo visualization of inflammatory processes at a cellular and molecular level. The labelling of cytokines such as interleukin-1, interleukin-2, interleukin-12 and MCP-1 has facilitated the identification of inflamed synovia in patients with rheumatoid arthritis, active Crohn’s disease, vulnerable atherosclerotic plaques and other targets. The possibility of using monoclonal antibodies against TNF-α, CD2, CD3, CD4 and anti-selectin has not only allowed the localization of inflamed sites but had also a significant impact in helping the selection of patients who can benefit from biological therapies. Regarding radiolabelled peptides, it is important to highlight the increasing use of somatostatin analogues targeting somatostatin receptors in inflammatory diseases, particularly for rheumatoid arthritis, Sjögren syndrome and autoimmune thyroid diseases. In the present review we describe the state of the art of SPECT radiopharmaceuticals to image chronic inflammatory diseases.

  17. Targeted anti-inflammatory therapeutics in asthma and chronic obstructive lung disease.

    Science.gov (United States)

    Durham, Andrew L; Caramori, Gaetano; Chung, Kian F; Adcock, Ian M

    2016-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are chronic inflammatory diseases of the airway, although the drivers and site of the inflammation differ between diseases. Asthmatics with a neutrophilic airway inflammation are associated with a poor response to corticosteroids, whereas asthmatics with eosinophilic inflammation respond better to corticosteroids. Biologicals targeting the Th2-eosinophil nexus such as anti-interleukin (IL)-4, anti-IL-5, and anti-IL-13 are ineffective in asthma as a whole but are more effective if patients are selected using cellular (eg, eosinophils) or molecular (eg, periostin) biomarkers. This highlights the key role of individual inflammatory mediators in driving the inflammatory response and for accurate disease phenotyping to allow greater understanding of disease and development of patient-oriented antiasthma therapies. In contrast to asthmatic patients, corticosteroids are relatively ineffective in COPD patients. Despite stratification of COPD patients, the results of targeted therapy have proved disappointing with the exception of recent studies using CXC chemokine receptor (CXCR)2 antagonists. Currently, several other novel mediator-targeted drugs are undergoing clinical trials. As with asthma specifically targeted treatments may be of most benefit in specific COPD patient endotypes. The use of novel inflammatory mediator-targeted therapeutic agents in selected patients with asthma or COPD and the detection of markers of responsiveness or nonresponsiveness will allow a link between clinical phenotypes and pathophysiological mechanisms to be delineated reaching the goal of endotyping patients. PMID:26334389

  18. IL-32: A Novel Pluripotent Inflammatory Interleukin, towards Gastric Inflammation, Gastric Cancer, and Chronic Rhino Sinusitis

    Directory of Open Access Journals (Sweden)

    Muhammad Babar Khawar

    2016-01-01

    Full Text Available A vast variety of nonstructural proteins have been studied for their key roles and involvement in a number of biological phenomenona. Interleukin-32 is a novel cytokine whose presence has been confirmed in most of the mammals except rodents. The IL-32 gene was identified on human chromosome 16 p13.3. The gene has eight exons and nine splice variants, namely, IL-32α, IL-32β, IL-32γ, IL-32δ, IL-32ε, IL-32ζ, IL-32η, IL-32θ, and IL-32s. It was found to induce the expression of various inflammatory cytokines including TNF-α, IL-6, and IL-1β as well as macrophage inflammatory protein-2 (MIP-2 and has been reported previously to be involved in the pathogenesis and progression of a number of inflammatory disorders, namely, inflammatory bowel disease (IBD, gastric inflammation and cancer, rheumatoid arthritis, and chronic obstructive pulmonary disease (COPD. In the current review, we have highlighted the involvement of IL-32 in gastric cancer, gastric inflammation, and chronic rhinosinusitis. We have also tried to explore various mechanisms suspected to induce the expression of this extraordinary cytokine as well as various mechanisms of action employed by IL-32 during the mediation and progression of the above said problems.

  19. Targeted anti-inflammatory therapeutics in asthma and chronic obstructive lung disease

    Science.gov (United States)

    Durham, Andrew L.; Caramori, Gaetano; Chung, Kian F.; Adcock, Ian M.

    2016-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are chronic inflammatory diseases of the airway, although the drivers and site of the inflammation differ between diseases. Asthmatics with a neutrophilic airway inflammation are associated with a poor response to corticosteroids, whereas asthmatics with eosinophilic inflammation respond better to corticosteroids. Biologicals targeting the Th2-eosinophil nexus such as anti–interleukin (IL)-4, anti–IL-5, and anti–IL-13 are ineffective in asthma as a whole but are more effective if patients are selected using cellular (eg, eosinophils) or molecular (eg, periostin) biomarkers. This highlights the key role of individual inflammatory mediators in driving the inflammatory response and for accurate disease phenotyping to allow greater understanding of disease and development of patient-oriented antiasthma therapies. In contrast to asthmatic patients, corticosteroids are relatively ineffective in COPD patients. Despite stratification of COPD patients, the results of targeted therapy have proved disappointing with the exception of recent studies using CXC chemokine receptor (CXCR)2 antagonists. Currently, several other novel mediator-targeted drugs are undergoing clinical trials. As with asthma specifically targeted treatments may be of most benefit in specific COPD patient endotypes. The use of novel inflammatory mediator-targeted therapeutic agents in selected patients with asthma or COPD and the detection of markers of responsiveness or nonresponsiveness will allow a link between clinical phenotypes and pathophysiological mechanisms to be delineated reaching the goal of endotyping patients. PMID:26334389

  20. Targeted anti-inflammatory therapeutics in asthma and chronic obstructive lung disease.

    Science.gov (United States)

    Durham, Andrew L; Caramori, Gaetano; Chung, Kian F; Adcock, Ian M

    2016-01-01

    Asthma and chronic obstructive pulmonary disease (COPD) are chronic inflammatory diseases of the airway, although the drivers and site of the inflammation differ between diseases. Asthmatics with a neutrophilic airway inflammation are associated with a poor response to corticosteroids, whereas asthmatics with eosinophilic inflammation respond better to corticosteroids. Biologicals targeting the Th2-eosinophil nexus such as anti-interleukin (IL)-4, anti-IL-5, and anti-IL-13 are ineffective in asthma as a whole but are more effective if patients are selected using cellular (eg, eosinophils) or molecular (eg, periostin) biomarkers. This highlights the key role of individual inflammatory mediators in driving the inflammatory response and for accurate disease phenotyping to allow greater understanding of disease and development of patient-oriented antiasthma therapies. In contrast to asthmatic patients, corticosteroids are relatively ineffective in COPD patients. Despite stratification of COPD patients, the results of targeted therapy have proved disappointing with the exception of recent studies using CXC chemokine receptor (CXCR)2 antagonists. Currently, several other novel mediator-targeted drugs are undergoing clinical trials. As with asthma specifically targeted treatments may be of most benefit in specific COPD patient endotypes. The use of novel inflammatory mediator-targeted therapeutic agents in selected patients with asthma or COPD and the detection of markers of responsiveness or nonresponsiveness will allow a link between clinical phenotypes and pathophysiological mechanisms to be delineated reaching the goal of endotyping patients.

  1. Chronic Inflammatory Periodontal Disease in Patients Diagnosed with Human Immunodeficiency Virus/AIDS in Cienfuegos

    Directory of Open Access Journals (Sweden)

    Nivia Gontán Quintana

    2013-08-01

    Full Text Available Background: human immunodeficiency virus increases patients´ susceptibility to infections. Consequently, a high incidence of periodontal diseases is observed among them. It is often associated with other lesions of the oral mucous. Objective: to determine the evolution of chronic inflammatory periodontal disease in patients diagnosed with human immunodeficiency virus/AIDS.Methods: a case series study involving HIV-positive patients who attended the Stomatology consultation in Cienfuegos was conducted. The Russell Periodontal Index and the Simplified Oral Hygiene Index were used. Patients were classified taking into account clinical and immunological categories. Statistical processing was performed through SPSS program version 15.0 and Chi-square tests were applied.Results: a high prevalence of chronic inflammatory periodontal disease was observed in patients with human immunodeficiency virus. Correlation with the oral hygiene of the patients studied was found. CD4 count showed no statistical significance in periodontal disease severity. All patients classified as A2 suffer from some stage of periodontal disease, which was the most affected clinical category in spite of presenting mild immunodeficiency.Conclusions: there is a high prevalence of chronic inflammatory periodontal disease in patients diagnosed with Human Immunodeficiency Virus in Cienfuegos and it is correlated with patient’s oral hygiene.

  2. The Role of Inflammatory Pathways in Implantation Failure: Chronic Endometritis and Hydrosalpinges.

    Science.gov (United States)

    Akopians, Alin L; Pisarska, Margareta D; Wang, Erica T

    2015-07-01

    The process of implantation is highly complex and involves a delicate interplay between the embryo and the appropriate maternal environment. The failure to implant is thought to be due to maternal factors or embryonic factors. Inflammation can be a part of the normal physiologic process during implantation; however, there are also pathologic entities that adversely affect uterine receptivity. This review will focus on chronic endometritis and hydrosalpinges as two specific inflammatory processes that contribute to implantation failure. For both chronic endometritis and hydrosalpinges, we will review the diagnosis, pathophysiology, and effect on implantation following treatment. The existing literature conclusively demonstrates that hydrosalpinges should be addressed by either laparoscopic salpingectomy or proximal tubal occlusion prior to in vitro fertilization. The picture for chronic endometritis is less clear since the diagnosis and treatment of chronic endometritis are not standardized, and there are no available randomized controlled trials on this topic. Future studies may target gene expression arrays as a method for further elucidating the role of inflammatory markers in normal and abnormal implantation processes. PMID:26132934

  3. Quantitative analysis of the cellular inflammatory response against biofilm bacteria in chronic wounds

    DEFF Research Database (Denmark)

    Fazli, Mustafa; Bjarnsholt, Thomas; Kirketerp-Møller, Klaus;

    2011-01-01

    counting on the tissue sections from wounds containing either Pseudomonas aeruginosa or Staphylococcus aureus. The P. aeruginosa-containing wounds had significantly higher numbers of neutrophils accumulated compared with the S. aureus-containing wounds. These results are discussed in relation...... to the wound. One such stimulus might be the presence of bacterial biofilms in chronic wounds. In the present study, biopsy specimens from chronic venous leg ulcers were investigated for the detection of bacteria using peptide nucleic acid-based fluorescence in situ hybridization (PNA-FISH) and confocal laser...... to the hypothesis that the presence of P. aeruginosa biofilms in chronic wounds may be one of the main factors leading to a persistent inflammatory response and impaired wound healing....

  4. Nutmeg oil alleviates chronic inflammatory pain through inhibition of COX-2 expression and substance P release in vivo

    OpenAIRE

    Zhang, Wei Kevin; Tang, He-Bin; Cong, Ren-Huai; Ma, Fang-Li; Wan, Chu-Jun; Tao, Shan-Shan; Li, Ting-Ting; Li, Yu-Sang; Li, Xiao-Jun

    2016-01-01

    Background: Chronic pain, or sometimes referred to as persistent pain, reduces the life quality of patients who are suffering from chronic diseases such as inflammatory diseases, cancer and diabetes. Hence, herbal medicines draw many attentions and have been shown effective in the treatment or relief of pain.Methods and Results: Here in this study, we used the CFA-injected rats as a sustainable pain model to test the anti-inflammatory and analgesic effect of nutmeg oil, a spice flavor additiv...

  5. Diffusion-weighted imaging in acute demyelinating myelopathy

    Energy Technology Data Exchange (ETDEWEB)

    Zecca, Chiara; Cereda, Carlo; Tschuor, Silvia; Staedler, Claudio; Nadarajah, Navarajah; Bassetti, Claudio L.; Gobbi, Claudio [Ospedale Regionale di Lugano, Servizio di Neurologia e Neuroradiologia, Neurocenter of Southern Switzerland, Lugano (Switzerland); Wetzel, Stephan [Swiss Neuro Institute (SNI), Abteilung fuer Neuroradiologie, Hirslanden Klinik Zuerich, Zuerich (Switzerland); Santini, Francesco [University of Basel Hospital, Division of Radiological Physics, Basel (Switzerland)

    2012-06-15

    Diffusion-weighted imaging (DWI) has become a reference MRI technique for the evaluation of neurological disorders. Few publications have investigated the application of DWI for inflammatory demyelinating lesions. The purpose of the study was to describe diffusion-weighted imaging characteristics of acute, spinal demyelinating lesions. Six consecutive patients (two males, four females; aged 28-64 years) with acute spinal cord demyelinating lesions were studied in a prospective case series design from June 2009 to October 2010. We performed magnetic resonance imaging studies from 2 to 14 days from symptom onset on the patients with relapsing remitting multiple sclerosis (n = 3) or clinically isolated syndrome (n = 3). Main outcome measures were diffusion-weighted imaging and apparent diffusion coefficient pattern (ADC) of acute spinal cord demyelinating lesions. All spinal lesions showed a restricted diffusion pattern (DWI+/ADC-) with a 24% median ADC signal decrease. A good correlation between clinical presentation and lesion site was observed. Acute demyelinating spinal cord lesions show a uniform restricted diffusion pattern. Clinicians and neuro-radiologists should be aware that this pattern is not necessarily confirmatory for an ischaemic aetiology. (orig.)

  6. Optic neuritis: Experience from a south Indian demyelinating disease registry

    Directory of Open Access Journals (Sweden)

    Lekha Pandit

    2012-01-01

    Full Text Available Background: Natural history of optic neuritis (OPN has not been studied in India. Aim: To study consecutive patients with optic neuritis as the initial manifestation of the neurologic disease and with disease duration of 3 or more years registered in the Mangalore Demyelinating Disease Registry. Materials and Methods: The study included 59 patients with a primary diagnosis of optic neuritis (confirmed by either an ophthalmologist or a neurologist or both. All the patients were investigated and followed-up in the clinic. Results: During the follow-up of the 59 patients, 29 (49% patients developed multiple sclerosis (MS; 3 (5% patients neuromyelitis optica (NMO; and 13 (22% patients chronic relapsing inflammatory optic neuritis (CRION, while the remaining 14 (24% did not either progress or relapse, monophasic OPN. An initial abnormal magnetic resonance imaging predicted conversion to MS in all 7 patients who had imaging at onset. Patients with NMO were left with significant residual visual loss distinguishing NMO from MS. In this large series of patients with CRION, nearly 50% of patients had deterioration in vision while steroids were being tapered. Long-term immunosuppression was essential for maintaining good visual outcome in both NMO and CRION. Conclusions: Optic neuritis in India appears similar to that in the West with nearly 50% developing MS in the long term.

  7. Developmental endothelial locus-1 is a homeostatic factor in the central nervous system limiting neuroinflammation and demyelination.

    Science.gov (United States)

    Choi, E Y; Lim, J-H; Neuwirth, A; Economopoulou, M; Chatzigeorgiou, A; Chung, K-J; Bittner, S; Lee, S-H; Langer, H; Samus, M; Kim, H; Cho, G-S; Ziemssen, T; Bdeir, K; Chavakis, E; Koh, J-Y; Boon, L; Hosur, K; Bornstein, S R; Meuth, S G; Hajishengallis, G; Chavakis, T

    2015-07-01

    Inflammation in the central nervous system (CNS) and disruption of its immune privilege are major contributors to the pathogenesis of multiple sclerosis (MS) and of its rodent counterpart, experimental autoimmune encephalomyelitis (EAE). We have previously identified developmental endothelial locus-1 (Del-1) as an endogenous anti-inflammatory factor, which inhibits integrin-dependent leukocyte adhesion. Here we show that Del-1 contributes to the immune privilege status of the CNS. Intriguingly, Del-1 expression decreased in chronic-active MS lesions and in the inflamed CNS in the course of EAE. Del-1-deficiency was associated with increased EAE severity, accompanied by increased demyelination and axonal loss. As compared with control mice, Del-1(-/-) mice displayed enhanced disruption of the blood-brain barrier and increased infiltration of neutrophil granulocytes in the spinal cord in the course of EAE, accompanied by elevated levels of inflammatory cytokines, including interleukin-17 (IL-17). The augmented levels of IL-17 in Del-1-deficiency derived predominantly from infiltrated CD8(+) T cells. Increased EAE severity and neutrophil infiltration because of Del-1-deficiency was reversed in mice lacking both Del-1 and IL-17 receptor, indicating a crucial role for the IL-17/neutrophil inflammatory axis in EAE pathogenesis in Del-1(-/-) mice. Strikingly, systemic administration of Del-1-Fc ameliorated clinical relapse in relapsing-remitting EAE. Therefore, Del-1 is an endogenous homeostatic factor in the CNS protecting from neuroinflammation and demyelination. Our findings provide mechanistic underpinnings for the previous implication of Del-1 as a candidate MS susceptibility gene and suggest that Del-1-centered therapeutic approaches may be beneficial in neuroinflammatory and demyelinating disorders. PMID:25385367

  8. Developmental endothelial locus-1 (Del-1) is a homeostatic factor in the central nervous system limiting neuroinflammation and demyelination

    Science.gov (United States)

    Neuwirth, Ales; Economopoulou, Matina; Chatzigeorgiou, Antonios; Chung, Kyoung-Jin; Bittner, Stefan; Lee, Seung-Hwan; Langer, Harald; Samus, Maryna; Kim, Hyesoon; Cho, Geum-Sil; Ziemssen, Tjalf; Bdeir, Khalil; Chavakis, Emmanouil; Koh, Jae-Young; Boon, Louis; Hosur, Kavita; Bornstein, Stefan R.; Meuth, Sven G.; Hajishengallis, George; Chavakis, Triantafyllos

    2014-01-01

    Inflammation in the central nervous system (CNS) and disruption of its immune privilege are major contributors to the pathogenesis of multiple sclerosis (MS) and of its rodent counterpart, experimental autoimmune encephalomyelitis (EAE). We have previously identified developmental endothelial locus-1 (Del-1) as an endogenous anti-inflammatory factor, which inhibits integrin-dependent leukocyte adhesion. Here we show that Del-1 contributes to the immune privilege status of the CNS. Intriguingly, Del-1 expression decreased in chronic active MS lesions and in the inflamed CNS in the course of EAE. Del-1-deficiency was associated with increased EAE severity, accompanied by increased demyelination and axonal loss. As compared to control mice, Del-1−/− mice displayed enhanced disruption of the blood brain barrier and increased infiltration of neutrophil granulocytes in the spinal cord in the course of EAE, accompanied by elevated levels of inflammatory cytokines, including IL-17. The augmented levels of IL-17 in Del-1-deficiency derived predominantly from infiltrated CD8+ T cells. Increased EAE severity and neutrophil infiltration due to Del-1-deficiency was reversed in mice lacking both Del-1 and IL-17-receptor, indicating a crucial role for the IL-17/neutrophil inflammatory axis in EAE pathogenesis in Del-1−/− mice. Strikingly, systemic administration of Del-1-Fc ameliorated clinical relapse in relapsing-remitting EAE. Therefore, Del-1 is an endogenous homeostatic factor in the CNS protecting from neuroinflammation and demyelination. Our findings provide mechanistic underpinnings for the previous implication of Del-1 as a candidate MS susceptibility gene and suggest that Del-1-centered therapeutic approaches may be beneficial in neuroinflammatory and demyelinating disorders. PMID:25385367

  9. Depletion of Blood-Borne Macrophages Does Not Reduce Demyelination in Mice Infected with a Neurotropic Coronavirus

    OpenAIRE

    Xue, Shurong; Sun, Ning; van Rooijen, Nico; Perlman, Stanley

    1999-01-01

    Mice infected with the neurotropic coronavirus mouse hepatitis virus strain JHM (MHV-JHM) develop a chronic demyelinating disease with symptoms of hindlimb paralysis. Histological examination of the brains and spinal cords of these animals reveals the presence of large numbers of activated macrophages/microglia. In two other experimental models of demyelination, experimental allergic encephalomyelitis and Theiler’s murine encephalomyelitis virus-induced demyelination, depletion of hematogenou...

  10. Sleep loss and the inflammatory response in mice under chronic environmental circadian disruption.

    Directory of Open Access Journals (Sweden)

    Allison J Brager

    Full Text Available Shift work and trans-time zone travel lead to insufficient sleep and numerous pathologies. Here, we examined sleep/wake dynamics during chronic exposure to environmental circadian disruption (ECD, and if chronic partial sleep loss associated with ECD influences the induction of shift-related inflammatory disorder. Sleep and wakefulness were telemetrically recorded across three months of ECD, in which the dark-phase of a light-dark cycle was advanced weekly by 6 h. A three month regimen of ECD caused a temporary reorganization of sleep (NREM and REM and wake processes across each week, resulting in an approximately 10% net loss of sleep each week relative to baseline levels. A separate group of mice were subjected to ECD or a regimen of imposed wakefulness (IW aimed to mimic sleep amounts under ECD for one month. Fos-immunoreactivity (IR was quantified in sleep-wake regulatory areas: the nucleus accumbens (NAc, basal forebrain (BF, and medial preoptic area (MnPO. To assess the inflammatory response, trunk blood was treated with lipopolysaccharide (LPS and subsequent release of IL-6 was measured. Fos-IR was greatest in the NAc, BF, and MnPO of mice subjected to IW. The inflammatory response to LPS was elevated in mice subjected to ECD, but not mice subjected to IW. Thus, the net sleep loss that occurs under ECD is not associated with a pathological immune response.

  11. from Neuropathology and Experimental Demyelination

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    Graham R. Campbell

    2011-01-01

    Full Text Available Mitochondria are the most efficient producers of energy in the form of ATP. Energy demands of axons, placed at relatively great distances from the neuronal cell body, are met by mitochondria, which when functionally compromised, produce reactive oxygen species (ROS in excess. Axons are made metabolically efficient by myelination, which enables saltatory conduction. The importance of mitochondria for maintaining the structural integrity of myelinated axons is illustrated by neuroaxonal degeneration in primary mitochondrial disorders. When demyelinated, the compartmentalisation of ion channels along axons is disrupted. The redistribution of electrogenic machinery is thought to increase the energy demand of demyelinated axons. We review related studies that focus on mitochondria within unmyelinated, demyelinated and dysmyelinated axons in the central nervous system. Based on neuropathological observations we propose the increase in mitochondrial presence within demyelinated axons as an adaptive process to the increased energy need. An increased presence of mitochondria would also increase the capacity to produce deleterious agents such as ROS when functionally compromised. Given the lack of direct evidence of a beneficial or harmful effect of mitochondrial changes, the precise role of increased mitochondrial presence within axons due to demyelination needs to be further explored in experimental demyelination in-vivo and in-vitro.

  12. Chronic fatigue is associated with increased disease-related worries and concerns in inflammatory bowel disease

    Institute of Scientific and Technical Information of China (English)

    Lars-Petter Jelsness-Jφrgensen; Tomm Bernklev; Magne Henriksen; Roald Torp; Bjφrn Mourn

    2012-01-01

    AIM: To investigate the impact of chronic fatigue on disease-related worries in inflammatory bowel disease (IBD) and the potential multicolinearity between subjective questionnaires. METHODS: Patients in remission or with mild-tomoderate disease activity completed the fatigue questionnaire (FQ), the rating form of IBD patient concerns (RFIPC), the Short-Form 36 (SF-36), and IBD questionnaire (N-IBDQ). In addition, clinical and epidemiological data were obtained. RESULTS: In total, 140 patients were included; of which 92 were diagnosed with ulcerative colitis and 48 with Crohn's disease. the mean age of patients with chronic fatigue was 44.2 years (SD =15.8) and for non-fatigued patients was 44.7 years (SD = 16.0). Chrnnic fatigued patients had clinically significantly increased levels of disease-related worries, as measured by Cohen's d effect size. Worries about having an ostomy bag, loss of bowel control, and energy levels were most prominent in both chronic fatigued and non-chronic fatigued IBD patients. Variance inflation factor (VIF) and tolerance indicated that there were no problematic multicolinearity among the FQ, RFIPC, SF-36 and N-IBDQ responses (VIF 2). CONCLUSION: Chronic fatigue is associated with increased levels of disease-related worries and concerns in IBD. Increased levels of worries were also associated with impaired health-related quality of life.

  13. Pseudomonas aeruginosa biofilm aggravates skin inflammatory response in BALB/c mice in a novel chronic wound model

    DEFF Research Database (Denmark)

    Trøstrup, Hannah; Thomsen, Kim; Christophersen, Lars J;

    2013-01-01

    Chronic wounds are presumed to persist in the inflammatory state, preventing healing. Emerging evidence indicates a clinical impact of bacterial biofilms in soft tissues, including Pseudomonas aeruginosa (PA) biofilms. To further investigate this, we developed a chronic PA biofilm wound infection...

  14. Doxycycline Promotes Carcinogenesis & Metastasis via Chronic Inflammatory Pathway: An In Vivo Approach.

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    Neha Nanda

    Full Text Available Doxycycline (DOX exhibits anti-inflammatory, anti-tumor, and pro-apoptotic activity and is being tested in clinical trials as a chemotherapeutic agent for several cancers, including colon cancer.In the current study, the chemotherapeutic activity of doxycycline was tested in a rat model of colon carcinogenesis, induced by colon specific cancer promoter, 1,2, dimethylhydrazine (DMH as well as study the effect of DOX-alone on a separate group of rats.Doxycycline administration in DMH-treated rats (DMH-DOX unexpectedly increased tumor multiplicity, stimulated progression of colonic tumor growth from adenomas to carcinomas and revealed metastasis in small intestine as determined by macroscopic and histopathological analysis. DOX-alone treatment showed markedly enhanced chronic inflammation and reactive hyperplasia, which was dependent upon the dose of doxycycline administered. Moreover, immunohistochemical analysis revealed evidence of inflammation and anti-apoptotic action of DOX by deregulation of various biomarkers.These results suggest that doxycycline caused chronic inflammation in colon, small intestine injury, enhanced the efficacy of DMH in tumor progression and provided a mechanistic link between doxycycline-induced chronic inflammation and tumorigenesis. Ongoing studies thus may need to focus on the molecular mechanisms of doxycycline action, which lead to its inflammatory and tumorigenic effects.

  15. Acute Inflammatory Bowel Disease Complicating Chronic Alcoholism and Mimicking Carcinoid Syndrome

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    Piercarlo Ballo

    2012-08-01

    Full Text Available We report the case of a woman with a history of chronic alcohol abuse who was hospitalized with diarrhea, severe hypokalemia refractory to potassium infusion, nausea, vomiting, abdominal pain, alternations of high blood pressure with phases of hypotension, irritability and increased urinary 5-hydroxyindoleacetic acid and cortisol. Although carcinoid syndrome was hypothesized, abdominal computed tomography and colonoscopy showed non-specific inflammatory bowel disease with severe colic wall thickening, and multiple colic biopsies confirmed non-specific inflammation with no evidence of carcinoid cells. During the following days diarrhea slowly decreased and the patient’s condition progressively improved. One year after stopping alcohol consumption, the patient was asymptomatic and serum potassium was normal. Chronic alcohol exposure is known to have several deleterious effects on the intestinal mucosa and can favor and sustain local inflammation. Chronic alcohol intake may also be associated with high blood pressure, behavior disorders, abnormalities in blood pressure regulation with episodes of hypotension during hospitalization due to impaired baroreflex sensitivity in the context of an alcohol withdrawal syndrome, increased urinary 5-hydroxyindoleacetic acid as a result of malabsorption syndrome, and increased urinary cortisol as a result of hypothalamic-pituitary-adrenal axis dysregulation. These considerations, together with the regression of symptoms and normalization of potassium levels after stopping alcohol consumption, suggest the intriguing possibility of a alcohol-related acute inflammatory bowel disease mimicking carcinoid syndrome.

  16. Mutual interaction of Basophils and T cells in chronic inflammatory diseases

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    Marika eSarfati

    2015-08-01

    Full Text Available Basophils are, together with mast cells, typical innate effector cells of allergen-induced IgE-dependent allergic diseases. Both cell types express the high affinity receptor for IgE (FcεR1, release histamine, inflammatory mediators and cytokines following FcεR1 cross-linking. Basophils are rare granulocytes in blood, lymphoid and non-lymphoid tissues and the difficulties to detect and isolate these cells has hampered the study of their biology and the understanding of their possible role in pathology. Furthermore, the existence of other FcεR1-expressing cells, including professional Ag-presenting dendritic cells, generated some controversy regarding the ability of basophils to express MHC Class II molecules, present Ag and drive naïve T cell differentiation into Th2 cells. The focus of this review is to present the recent advances on the interactions between basophils and peripheral blood and tissue memory Th1, Th2 and Th17 cells, as well as their potential role in IgE-independent non allergic chronic inflammatory disorders, including human inflammatory bowel diseases. Basophils interactions with the innate players of IgE-dependent allergic inflammation, particularly innate lymphoid cells, will also be considered. The previously unrecognized function for basophils in skewing adaptive immune responses opens novel perspectives for the understanding of their contribution to the pathogenesis of inflammatory diseases.

  17. The current understanding of the treatment chronic non-bacterial prostatitis with inflammatory component

    Directory of Open Access Journals (Sweden)

    Yu. Yu. Vinnik

    2015-01-01

    Full Text Available A literature review is dedicated to one of the most urgent problems of modern andrology – the treatment of chronic non-bacterial prostatitis (CP with inflammatory component. Over the past decades, despite numerous methods of prevention and treatment offered by the Russian and foreign urologists, the incidence of CP has been steadily progressing. Treatment of patients with CP should be comprehensive and be sure to include the effects on all the links in the pathogenesis of the disease. It is different depending on the age of the patient and the presence and nature of CP manifestations, including changes in mental status, especially disease progression, stage and phase inflammatory activity in the prostate, the degree of involvement in the disease process nearby organs, immune status and other factors. The proposed scientific review covers the basic techniques of therapeutic effect on patients with CP. Given in article data allows a more rational approach to the treatment of these patients.

  18. Drosophila as a model for intestinal dysbiosis and chronic inflammatory diseases.

    Science.gov (United States)

    Lee, Kyung-Ah; Lee, Won-Jae

    2014-01-01

    The association between deregulated intestinal microbial consortia and host diseases has been recognized since the birth of microbiology over a century ago. Intestinal dysbiosis refers to a state where living metazoans harbor harmful intestinal microflora. However, there is still an issue of whether causality arises from the host or the microbe because it is unclear whether deregulation of the gut microbiota community is the consequence or cause of the host disease. Recent studies using Drosophila and its simple microbiota have provided a valuable model system for dissecting the molecular mechanisms of intestinal dysbiosis. In this review, we examine recent exciting observations in Drosophila gut-microbiota interactions, particularly the links among the host immune genotype, the microbial community structure, and the host inflammatory phenotype. Future genetic analyses using Drosophila model system will provide a valuable outcome for understanding the evolutionarily conserved mechanisms that underlie intestinal dysbiosis and chronic inflammatory diseases.

  19. Demyelinating disease simulating brain tumours: A histopathologic assessment of seven cases

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    Jain Deepali

    2006-02-01

    Full Text Available Background: Demyelinating diseases can present as space occupying lesions with in the brain. It is clinically and radiologically difficult to differentiate them from primary neoplasms. Histopathologically they mimic astrocytic neoplasms closely and identifying these lesions correctly has a profound impact in treatment and prognosis of these patients. Aims and Objectives: The objective was to determine the histopathologic features of such acute focal demyelinating disease that clinically presented as brain tumors. Material and Methods: Seven cases were included for the study. Detailed histopathological examination including stains for myelin and axon were performed. The histopathological keys in arriving at the right diagnoses included a well demarcated lesion that contains uniform distribution of foamy macrophages in the absence of any associated coagulative necrosis, sheets of gemistocytic astrocytes in the white matter that show well-formed processes, perivascular chronic inflammatory cell infiltration and total absence of myelin with relative preservation of axons within these areas. Conclusion: The degree of suspicion (clinical, radiological and histopathological should be high to diagnose these group of lesions. The above-mentioned diagnostic keys should help in arriving at the correct histopathological diagnoses of such cases.

  20. Wegener’s granulomatosis mimicking inflammatory bowel disease and presenting with chronic enteritis

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    Shahedi K

    2013-10-01

    Full Text Available Kamyar Shahedi,1,2 Ramy Magdy Hanna,1,2 Oleg Melamed,1,2 James Wilson2,31Department of Medicine Olive-View UCLA Medical Center, Sylmar, CA, 2David Geffen School of Medicine at UCLA, Los Angeles, CA, 3UCLA Medical Center-UCLA Stone Center, Los Angeles, CA, USAAbstract: Wegener’s granulomatosis, also known as anti-neutrophil cytoplasmic antibody (ANCA-associated vasculitis, is a small vessel vasculitis with primarily pulmonary, renal, and sinus disease manifestations. The prevalence of Wegener’s granulomatosis is three cases per 100,000 patients. Cardiovascular, neurologic, cutaneous, and joint manifestations have been reported in many case reports and case series. Gastrointestinal manifestations are less noted in Wegener’s granulomatosis, although they have been previously reported in the form of intestinal perforation and intestinal ischemia. Additionally, there are characteristic findings of vasculitis that are noted with active Wegener’s granulomatosis of the small bowel. We report a case of an elderly patient who presented with weight loss, diarrhea, and hematochezia. His symptoms were chronic and had lasted for more than 1 year before diagnosis. Inflammatory bowel disease or chronic enteritis due to Salmonella arizonae because of reptile exposure originally were suspected as etiologies of his presentation. The findings of proteinuria, renal failure, and pauci-immune glomerulonephritis on renal biopsy, in conjunction with an elevated c-ANCA titer, confirmed the diagnosis of Wegener’s granulomatosis with associated intestinal vasculitis. This case demonstrates an atypical presentation of chronic duodenitis and jejunitis secondary to Wegener’s granulomatosis, which mimicked inflammatory bowel disease.Keywords: ANCA-associated vasculitis, Wegener’s syndrome, pauci-immune glomerulonephritis, Salmonella arizonae, inflammatory bowel disease

  1. Olfactory system and demyelination.

    Science.gov (United States)

    Garcia-Gonzalez, D; Murcia-Belmonte, V; Clemente, D; De Castro, F

    2013-09-01

    Within the central nervous system, the olfactory system represents one of the most exciting scenarios since it presents relevant examples of long-life sustained neurogenesis and continuous axonal outgrowth from the olfactory epithelium with the subsequent plasticity phenomena in the olfactory bulb. The olfactory nerve is composed of nonmyelinated axons with interesting ontogenetic interpretations. However, the centripetal projections from the olfactory bulb are myelinated axons which project to more caudal areas along the lateral olfactory tract. In consequence, demyelination has not been considered as a possible cause of the olfactory symptoms in those diseases in which this sense is impaired. One prototypical example of an olfactory disease is Kallmann syndrome, in which different mutations give rise to combined anosmia and hypogonadotropic hypogonadism, together with different satellite symptoms. Anosmin-1 is the extracellular matrix glycoprotein altered in the X-linked form of this disease, which participates in cell adhesion and migration, and axonal outgrowth in the olfactory system and in other regions of the central nervous system. Recently, we have described a new patho-physiological role of this protein in the absence of spontaneous remyelination in multiple sclerosis. In the present review, we hypothesize about how both main and satellite neurological symptoms of Kallmann syndrome may be explained by alterations in the myelination. We revisit the relationship between the olfactory system and myelin highlighting that minor histological changes should not be forgotten as putative causes of olfactory malfunction.

  2. A 17 year-old girl with a demyelinating disease requiring mechanical ventilation: a case report

    Directory of Open Access Journals (Sweden)

    Katsenos Chrysostomos

    2013-01-01

    Full Text Available Abstract Background Demyelinating diseases cause destruction of the myelin sheath, while axons are relatively spared. Pathologically, demyelination can be the result of an inflammatory process, viral infection, acquired metabolic derangement and ischemic insult. Three diseases that can cause inflammatory demyelination of the CNS are: Multiple sclerosis (MS, Acute disseminated encephalomyelitis (ADEM and Acute hemorrhagic leucoencephalitis. Differentiation is not always easy and there is considerable overlaping. Data about adults with acute demyelination requiring ICU admission is limited. Case presentation A 17 year old Greek female was hospitalised in the ICU because of acute respiratory failure requiring mechanical ventilation. She had a history of febrile disease one month before, acute onset of paraplegia, diplopia, progressive arm weakness and dyspnea. Her consciousness was not impaired. A demyelinating central nervous system (CNS disease, possibly post infectious encephalomyelitis (ADEM was the underlying condition. The MRI of the brain disclosed diffused expanded cerebral lesions involving the optic nerve, basal ganglia cerebellum, pons and medulla oblongata. There was also extended involvement of the cervical and thoracic part of the spinal cord. CSF leukocyte count was elevated with lymphocyte predominance. The patient required mechanical ventilation for two months. Then she was transferred to a rehabilitation centre. Three years later she remains paraplegic. Since then she has not suffered any other demyelination attack. Conclusions Demyelinating diseases can cause acute respiratory failure when the spinal cord is affected. Severe forms of these diseases, making necessary ICU admission, is less frequently reported. Intensivists should be aware of the features of these rare diseases.

  3. Quality of Life Considering Patients with Chronic Inflammatory Bowel Diseases - Natural and Parenteral Nutrition

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    Raczkowska Aneta

    2014-12-01

    Full Text Available One of the elements of treatment considering inflammatory bowel diseases is nutritional therapy. The duration of the above-mentioned depends on the prevalence of such symptoms as fever, bowel move-ments, length of the functioning gastrointestinal tract, stoma and intestinal fistula presence. Nutritional therapy is an essential element of successful treatment alongside pharmacological, surgical, and biological therapy, as well as other methods. Crohn's disease and ulcerative colitis considered as chronic diseases, lead towards physical and biopsychosocial disability, being responsible for the reduction in the quality of life.

  4. Review article: the potential role of nitric oxide in chronic inflammatory bowel disorders

    DEFF Research Database (Denmark)

    Perner, Anders; Rask-Madsen, J

    1999-01-01

    The aetiology of the chronic inflammatory bowel diseases-ulcerative colitis and Crohn's disease-as well as 'microscopic colitis'-both collagenous (COC) and lymphocytic colitis (LC)-remains unknown. Autoimmune mechanisms, cytokine polymorphism, commensal bacteria, infectious agents and vascular...... by the generation of superoxide with reduced L-arginine availability. In active ulcerative colitis, and to a lesser extent in Crohn's disease, a greatly increased production of NO has been demonstrated by indirect and direct measurements. Surprisingly, even higher rates of production have been observed in COC...

  5. Current concepts in chronic inflammatory diseases: Interactions between microbes, cellular metabolism, and inflammation.

    Science.gov (United States)

    Garn, Holger; Bahn, Sabine; Baune, Bernhard T; Binder, Elisabeth B; Bisgaard, Hans; Chatila, Talal A; Chavakis, Triantafyllos; Culmsee, Carsten; Dannlowski, Udo; Gay, Steffen; Gern, James; Haahtela, Tari; Kircher, Tilo; Müller-Ladner, Ulf; Neurath, Markus F; Preissner, Klaus T; Reinhardt, Christoph; Rook, Graham; Russell, Shannon; Schmeck, Bernd; Stappenbeck, Thaddeus; Steinhoff, Ulrich; van Os, Jim; Weiss, Scott; Zemlin, Michael; Renz, Harald

    2016-07-01

    Recent research indicates that chronic inflammatory diseases, including allergies and autoimmune and neuropsychiatric diseases, share common pathways of cellular and molecular dysregulation. It was the aim of the International von-Behring-Röntgen Symposium (October 16-18, 2014, in Marburg, Germany) to discuss recent developments in this field. These include a concept of biodiversity; the contribution of urbanization, lifestyle factors, and nutrition (eg, vitamin D); and new mechanisms of metabolic and immune dysregulation, such as extracellular and intracellular RNAs and cellular and mitochondrial stress. Epigenetic mechanisms contribute further to altered gene expression and therefore to the development of chronic inflammation. These novel findings provide the foundation for further development of preventive and therapeutic strategies. PMID:27373325

  6. Marine Invertebrate Natural Products for Anti-Inflammatory and Chronic Diseases

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    Kalimuthu Senthilkumar

    2013-01-01

    Full Text Available The marine environment represents a relatively available source of functional ingredients that can be applied to various aspects of food processing, storage, and fortification. Moreover, numerous marine invertebrates based compounds have biological activities and also interfere with the pathogenesis of diseases. Isolated compounds from marine invertebrates have been shown to pharmacological activities and are helpful for the invention and discovery of bioactive compounds, primarily for deadly diseases like cancer, acquired immunodeficiency syndrome (AIDS, osteoporosis, and so forth. Extensive research within the last decade has revealed that most chronic illnesses such as cancer, neurological diseases, diabetes, and autoimmune diseases exhibit dysregulation of multiple cell signaling pathways that have been linked to inflammation. On the basis of their bioactive properties, this review focuses on the potential use of marine invertebrate derived compounds on anti-inflammatory and some chronic diseases such as cardiovascular disease, osteoporosis, diabetes, HIV, and cancer.

  7. Effect of intravenous immunoglobulin in Guilain-Barre syndrome, myasthenia gravis and chronic idiopathic demyelinative polyneuropathy, A survey in Imam Khomeini Hospital

    Directory of Open Access Journals (Sweden)

    Qaffarpoor M

    1999-09-01

    Full Text Available With retrospective evaluation of 44 patients suffering from Guilan-Barre Syndrome (GBS, Chronic Idiopathic Demtyelinative Polyradiculoneuropathy (CIDP and Myasthenia Gravis (MG treated with intravenous immunoglobulin, we found following results: 1 Initial symptoms of improvement on forth or fifth days. 2 Maximum recovery for CIDP and MG were after 16-24 and 3-11 days, respectively. 3 No major complication, but mild side effects in 32% of patients. 4 In patients with GBS one grade improvement achieved after 8-30 days. 5 Intravenous immunoglobulin (IVIG plus plasmapheresis had no advantages over IVIG alone. 6 No reasonable conclusion about relapsing rate and duration of response due to follow up restrictions.

  8. Microglial cystatin F expression is a sensitive indicator for ongoing demyelination with concurrent remyelination.

    Science.gov (United States)

    Ma, Jianmei; Tanaka, Kenji F; Shimizu, Takahiro; Bernard, Claude C A; Kakita, Akiyoshi; Takahashi, Hitoshi; Pfeiffer, Steven E; Ikenaka, Kazuhiro

    2011-05-01

    Demyelination coincides with numerous changes of gene expression in the central nervous system (CNS). Cystatin F, which is a papain-like lysosomal cysteine proteinase inhibitor that is normally expressed by immune cells and not in the brain, is massively induced in the CNS during acute demyelination. We found that microglia, which are monocyte/macrophage-lineage cells in the CNS, express cystatin F only during demyelination. By using several demyelinating animal models and the spinal cord tissues from multiple sclerosis (MS) patients, we examined spatiotemporal expression pattern of cystatin F by in situ hybridization and immunohistochemistry. We found that the timing of cystatin F induction matches with ongoing demyelination, and the places with cystatin F expression overlapped with the remyelinating area. Most interestingly, cystatin F induction ceased in chronic demyelination, in which remyelinating ability was lost. These findings demonstrate that the expression of cystatin F indicates the occurrence of ongoing demyelination/remyelination and the absence of cystatin F expression indicates the cessation of remyelination in the demyelinating area.

  9. Chronic inflammatory diseases: do immunological patterns drive the choice of biotechnology drugs? A critical review.

    Science.gov (United States)

    Sozzani, Silvano; Abbracchio, Maria P; Annese, Vito; Danese, Silvio; De Pità, Ornella; De Sarro, Giovambattista; Maione, Sabatino; Olivieri, Ignazio; Parodi, Aurora; Sarzi-Puttini, Piercarlo

    2014-08-01

    Chronic inflammatory diseases represent a heterogeneous group of conditions that can affect practically any organ or system. An increasing number of biologic agents have been developed to selectively target the cell populations and signaling pathways involved in chronic inflammation, including cytokines, monoclonal antibodies and engineered receptors. This approach has been remarkably successful in alleviating some of the signs and symptoms of refractory autoimmune diseases. The use of this therapeutic strategy is likely to increase with the introduction of biosimilar agents. The different nature of these biological products makes the comparison of their pharmaceutical and clinical characteristics difficult, including safety and potency and these issues may be particularly relevant in the case of biosimilars. In addition, the heterogeneity of autoimmune diseases and of autoimmune patients, further adds to the complexity of choosing the right drug for each patient and predicting efficacy and safety of the treatment. In this review, we summarize actual knowledge about current biological agents and their use in autoimmune diseases, with a special emphasis for rheumatoid arthritis, inflammatory bowel diseases and psoriasis. The purpose of this analysis is to address the most critical issues raised by the rapid advancements in this field over recent years, and to acknowledge the potentially valuable gains brought about by the increasing availability of these new biologic agents. PMID:24697663

  10. Chronic fluoride exposure-induced testicular toxicity is associated with inflammatory response in mice.

    Science.gov (United States)

    Wei, Ruifen; Luo, Guangying; Sun, Zilong; Wang, Shaolin; Wang, Jundong

    2016-06-01

    Previous studies have indicated that fluoride (F) can affect testicular toxicity in humans and rodents. However, the mechanism underlying F-induced testicular toxicity is not well understood. This study was conducted to evaluate the sperm quality, testicular histomorphology and inflammatory response in mice followed F exposure. Healthy male mice were randomly divided into four groups with sodium fluoride (NaF) at 0, 25, 50, 100 mg/L in the drinking water for 180 days. At the end of the exposure, significantly increased percentage of spermatozoa abnormality was found in mice exposed to 50 and 100 mg/L NaF. Disorganized spermatogenic cells, vacuoles in seminiferous tubules and loss and shedding of sperm cells were also observed in the NaF treated group. In addition, chronic F exposure increased testicular interleukin-17(IL-17), interleukin-17 receptor C (IL-17RC), tumor necrosis factor-α (TNF-α) and interleukin-6 (IL-6) in transcriptional levels, as well as IL-17 and TNF-α levels in translational levels. Interestingly, we observed that F treated group elevated testicular inducible nitric oxide synthase (iNOS) mRNA level and nitric oxide (NO) concentration. Taken together, these results indicated that testicular inflammatory response could contribute to chronic F exposure induced testicular toxicity in mice.

  11. The immunomodulatory properties of Helicobacter pylori confer protection against allergic and chronic inflammatory disorders

    Directory of Open Access Journals (Sweden)

    Anne eMüller

    2012-02-01

    Full Text Available Chronic infection with the gastric bacterial pathogen Helicobacter pylori causes gastritis and predisposes carriers to a high risk of developing gastric and duodenal ulcers, gastric cancer and gastric lymphoma, but has also recently been shown to protect against certain allergic and chronic inflammatory disorders. The immunomodulatory properties that allow the bacteria to persist for decades in infected individuals in the face of a vigorous, yet ultimately non-protective, innate and adaptive immune response may at the same time confer protection against allergies, asthma and inflammatory bowel diseases. Experimental evidence from mouse models suggests that H. pylori has evolved to skew the adaptive immune response towards immune tolerance rather than immunity, which promotes persistent infection on the one hand, and inhibits auto-aggressive and allergic T-cell responses on the other. Regulatory T-cells mediating peripheral immune tolerance have emerged as key cellular players in facilitating persistent infection as well as protection from allergies, in both observational studies in humans and experimental work in mice. Recent data suggest that H. pylori actively targets dendritic cells to promote tolerance induction. The findings discussed in this review raise the possibility of harnessing the immunomodulatory properties of H. pylori for the prevention and treatment of allergic and auto-immune diseases, and also provide new insights relevant for H. pylori-specific vaccine development.

  12. Cerebrospinal fluid analysis in the context of CNS demyelinating diseases

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    Sandro Luiz de Andrade Matas

    2013-09-01

    Full Text Available The central nervous system demyelinating diseases are a group of disorders with different etiologies, characterized by inflammatory lesions that are associated with loss of myelin and eventually axonal damage. In this group the most studied ones are multiple sclerosis (MS, neuromyelitis optic (NMO and acute disseminated encephalomyelitis (ADEM. The cerebrospinal fluid is essential to differentiate between these different syndromes and to define multiple sclerosis, helping to assess the probability of Clinical Isolated Syndrome turn into multiple sclerosis.

  13. Cerebrospinal fluid analysis in the context of CNS demyelinating diseases

    OpenAIRE

    Sandro Luiz de Andrade Matas; Felipe von Glehn; Gustavo Bruniera Peres Fernandes; Carlos Augusto Senne Soares

    2013-01-01

    The central nervous system demyelinating diseases are a group of disorders with different etiologies, characterized by inflammatory lesions that are associated with loss of myelin and eventually axonal damage. In this group the most studied ones are multiple sclerosis (MS), neuromyelitis optic (NMO) and acute disseminated encephalomyelitis (ADEM). The cerebrospinal fluid is essential to differentiate between these different syndromes and to define multiple sclerosis, helping to assess the pro...

  14. Chronic calcium pyrophosphate crystal inflammatory arthritis induced by extreme hypomagnesemia in short bowel syndrome

    Directory of Open Access Journals (Sweden)

    Hahn Markus

    2012-09-01

    Full Text Available Abstract Background Short bowel syndrome (SBS may induce a plethora of clinical symptoms ranging from underweight to nutrient-, vitamin- and electrolyte deficiencies. The objective of this case report is to illustrate how demanding the management of a 60 year old patient with SBS and recurrent joint attacks was for different medical disciplines. Case presentation The patient with SBS presented with a body mass index of 16.5 kg/m2 after partial jejunoileal resection of the small intestine with a six year long history of recurrent pain attacks in multiple peripheral joints, chronic diarrhoea and food intolerances. Pain attacks occurred 4–5 times a week with a median consumption of 15 mg prednisone per day. The interdisciplinary workup after several gastroenterologic, rheumatologic, radiologic, psychiatric and orthopedic consultations is shown including successful treatment steps. Clinical diagnosis revealed no systemic inflammatory disease, but confirmed extreme hypomagnesemia (0.2 mmol/l after reproducible pathological magnesium resorption tests as causative for chronic calcium pyrophosphate crystal inflammatory arthritis (pseudogout, chondrocalcinosis. Multidisciplinary treatment included application of colchicines, parenteral nutrition and magnesium substitution, antiperistaltic agents and avoidance of intolerant foods. Normalization of magnesium levels and a marked remission of joint attacks were achieved after six months with significant reduction of prednisone to 1.5 mg/day. Conclusion Despite the rarity of this condition, it is important to know that hypomagnesaemia may be associated with calcium pyrophosphate crystal inflammatory arthritis (chondrocalcinosis and that SBS patients may be prone to develop extreme hypomagnesaemia causing recurrent joint attacks without systemic inflammation.

  15. Genetic and metabolic signals during acute enteric bacterial infection alter the microbiota and drive progression to chronic inflammatory disease

    Energy Technology Data Exchange (ETDEWEB)

    Kamdar, Karishma; Khakpour, Samira; Chen, Jingyu; Leone, Vanessa; Brulc, Jennifer; Mangatu, Thomas; Antonopoulos, Dionysios A.; Chang, Eugene B; Kahn, Stacy A.; Kirschner, Barbara S; Young, Glenn; DePaolo, R. William

    2016-01-13

    Chronic inflammatory disorders are thought to arise due to an interplay between predisposing host genetics and environmental factors. For example, the onset of inflammatory bowel disease is associated with enteric proteobacterial infection, yet the mechanistic basis for this association is unclear. We have shown previously that genetic defiency in TLR1 promotes acute enteric infection by the proteobacteria Yersinia enterocolitica. Examining that model further, we uncovered an altered cellular immune response that promotes the recruitment of neutrophils which in turn increases metabolism of the respiratory electron acceptor tetrathionate by Yersinia. These events drive permanent alterations in anti-commensal immunity, microbiota composition, and chronic inflammation, which persist long after Yersinia clearence. Deletion of the bacterial genes involved in tetrathionate respiration or treatment using targeted probiotics could prevent microbiota alterations and inflammation. Thus, acute infection can drive long term immune and microbiota alterations leading to chronic inflammatory disease in genetically predisposed individuals.

  16. Anti-inflammatory effects of naringin in chronic pulmonary neutrophilic inflammation in cigarette smoke-exposed rats

    OpenAIRE

    Nie, YC; Wu, H.; Li, PB; Luo, YL; Long, K.; Xie, LM; Shen, JG; Su, WW

    2012-01-01

    Naringin, a well-known flavanone glycoside of grapefruit and citrus fruits, was found to be as an effective anti-inflammatory compound in our previous lipopolysaccharide-induced acute lung injury mouse model via blockading activity of nuclear factor κB. The current study sought to explore the anti-inflammatory effects of naringin on chronic pulmonary neutrophilic inflammation in cigarette smoke (CS)-induced rats. Seventy Sprague-Dawley rats were randomly divided into seven groups to study the...

  17. Clinical analysis on 32 cases of chronic inflammatory demyelinating polyradiculoneuropathy%慢性吉兰-巴雷综合征32例临床分析

    Institute of Scientific and Technical Information of China (English)

    崔芳; 黄旭升; 陈朝晖; 刘淑贤

    2005-01-01

    目的总结慢性吉兰-巴雷综合征(CIDP)的临床表现、电生理、病理学等特点.方法对32例CIDP病例进行回顾性分析.结果多为亚急性或慢性起病,首发症状以肢体麻木或感觉异常、肢体无力最为多见,也有以复视、视物模糊、构音障碍和吞咽困难起病.常见的临床表现为进行性或复发性肌无力和感觉障碍,可合并有自主神经、颅神经受损,多数患者临床表现为对称性,少部分为非对称性.肌电图呈神经性受损改变,以运动及感觉神经传导速度减慢为主,部分伴有波幅下降.腓肠神经活检可见髓鞘脱失、髓鞘及胶原纤维增生.静脉滴注人血免疫球蛋白(IVIG)及糖皮质激素治疗有效.结论CIDP为广泛的周围神经损害,多数合并有自主神经损害,颅神经受损者并非少见.神经电生理表现为以脱髓鞘为主,部分伴有轴索变性.腓肠神经活检对CIDP具有重要的诊断价值.IVIG及糖皮质激素是目前治疗CIDP的有效方法.

  18. Microglial Hv1 proton channel promotes cuprizone-induced demyelination through oxidative damage.

    Science.gov (United States)

    Liu, Junli; Tian, Daishi; Murugan, Madhuvika; Eyo, Ukpong B; Dreyfus, Cheryl F; Wang, Wei; Wu, Long-Jun

    2015-10-01

    NADPH oxidase (NOX)-dependent reactive oxygen species (ROS) production in inflammatory cells including microglia plays an important role in demyelination and free radical-mediated tissue injury in multiple sclerosis (MS). However, the mechanism underlying microglial ROS production and demyelination remains largely unknown. The voltage-gated proton channel, Hv1, is selectively expressed in microglia and is required for NOX-dependent ROS generation in the brain. In the present study, we sought to determine the role of microglial Hv1 proton channels in a mouse model of cuprizone-induced demyelination, a model for MS. Following cuprizone exposure, wild-type mice presented obvious demyelination, decreased myelin basic protein expression, loss of mature oligodendrocytes, and impaired motor coordination in comparison to mice on a normal chow diet. However, mice lacking Hv1 (Hv1(-/-) ) are partially protected from demyelination and motor deficits compared with those in wild-type mice. These rescued phenotypes in Hv1(-/-) mice in cuprizone-induced demyelination is accompanied by reduced ROS production, ameliorated microglial activation, increased oligodendrocyte progenitor cell (NG2) proliferation, and increased number of mature oligodendrocytes. These results demonstrate that the Hv1 proton channel is required for cuprizone-induced microglial oxidative damage and subsequent demyelination. Our study suggests that the microglial Hv1 proton channel is a unique target for controlling NOX-dependent ROS production in the pathogenesis of MS.

  19. The Comparison of Efficacy of Tricyclic Antidepressant with and without Non Steroidal Anti Inflammatory Drugs in Chronic Low Back Pain

    OpenAIRE

    A.R. Yavarikia

    2007-01-01

    Introduction & Objectives: Low back pain (LBP) is one of common medical problems with several accepted medical modalities such as drugs, physiotherapy, surgery, etc. We studied the efficacy of tricyclic antidepressant (TCA), and tricyclic antidepressant plus non steroidal anti inflammatory drugs (TCA + NSAID) in 200 patients with chronic LBP. Materials & Methods: In an experimental clinical trial study on patients with chronic low back pain without organic findings, patients were divided in t...

  20. Effect of Probiotic Consortium on the Local Inflammatory Process in Chronic Periodontitis

    Directory of Open Access Journals (Sweden)

    Zhanagul Khasenbekova

    2014-01-01

    Full Text Available Introduction: Inflammatory periodontal disease is one of the major concerns of researchers and clinicians, because it can lead to tooth loss and an increased risk of systemic pathologies, even at the age of 35. The purpose of this study was to determine the effects of gelatin-based probiotic consortium on the local and general factors of inflammation in rats with chronic periodontitis. Methods: The study object was a complex of probiotic bacteria based in an odourless 6% gelatin plate with neutral flavour. A cellular biomass of the consortium consists of following lactobacilli: Lactobacillus casei subsp. pseudoplantarum, Lactobacillus caseisubsp.casei, L.fermentum, and L. helveticus. The viable cell number was 2.5 x 109 CFU/ml. The model of chronic periodontitis was reproduced in the white random-bred rats that weighed 160-220g, by keeping them on a low-protein diet. After three months, symptoms associated with medium and severe chronic periodontitis were observed in the rats. Application was carried out on the oral mucosa of rats 1 time per day for 14 days. The stickers lacking consortium of microorganisms were used as the placebo. The "Solcoseril" gel was chosen as a comparator. The hematologic, biochemical, and morphological characteristics were investigated. Results: A complete clearance of periodontal pockets was observed during an objective examination of the experimental group rats on the 14th day of the experiment. Moreover, a gingival mucous turned pink, and there were no cyanosis tissues. The local changes were accompanied by improvement in hematological parameters, such as a reduction of blood eosinophilia and neutrophilia, and a recovery of the white blood cells number to the normal degree within the group that received the probiotic complex. A decrease of the acute plethora of microvasculature was observed morphologically as a result of the treatment. There were signs of basal layer activation of the stratified squamous epithelium

  1. Chronic inflammatory and non-inflammatory diseases of the gastrointestinal tract in cats: diagnostic advantages of full-thickness intestinal and extraintestinal biopsies.

    Science.gov (United States)

    Kleinschmidt, Sven; Harder, Jasmine; Nolte, Ingo; Marsilio, Sina; Hewicker-Trautwein, Marion

    2010-02-01

    An evaluation of histological findings in full-thickness biopsies from the gastrointestinal tract (GIT) and extraintestinal samples of 43 cats with chronic GIT disease signs was performed. In the majority of cases (46.5%) inflammatory bowel disease, ie, lymphocytic-plasmacytic enteritis/colitis (32.6%), eosinophilic gastroenterocolitis (11.6%) and mixed inflammatory infiltration (2.3%), was diagnosed. Furthermore, in four animals non-inflammatory mucosal band-shaped fibrosis (9.3%), and in 10 cats (23.3%) a diffuse lymphoma, was found. Six cats displayed only a gastritis (7.0%) or lymphangiectasia (7.0%), respectively. In two cats a mast cell tumour (4.7%) was diagnosed. In one cat no histopathological lesions were found. The availability of transmural biopsies from all segments of the intestine and the collection of extraintestinal samples, especially mesenteric lymph nodes, is especially helpful for diagnosing intestinal tumours such as lymphomas and tumours of mast cell origin.

  2. Rosacea: Molecular Mechanisms and Management of a Chronic Cutaneous Inflammatory Condition

    Directory of Open Access Journals (Sweden)

    Yu Ri Woo

    2016-09-01

    Full Text Available Rosacea is a chronic cutaneous inflammatory disease that affects the facial skin. Clinically, rosacea can be categorized into papulopustular, erythematotelangiectatic, ocular, and phymatous rosacea. However, the phenotypic presentations of rosacea are more heterogeneous. Although the pathophysiology of rosacea remains to be elucidated, immunologic alterations and neurovascular dysregulation are thought to have important roles in initiating and strengthening the clinical manifestations of rosacea. In this article, we present the possible molecular mechanisms of rosacea based on recent laboratory and clinical studies. We describe the genetic predisposition for rosacea along with its associated diseases, triggering factors, and suggested management options in detail based on the underlying molecular biology. Understanding the molecular pathomechanisms of rosacea will likely aid toward better comprehending its complex pathogenesis.

  3. Chronic relapsing inflammatory optic neuropathy: a systematic review of 122 cases reported.

    Science.gov (United States)

    Petzold, Axel; Plant, Gordon T

    2014-01-01

    Chronic relapsing inflammatory optic neuropathy (CRION) is an entity that was described in 2003. Early recognition of patients suffering from CRION is relevant because of the associated risk for blindness if treated inappropriately. It seems timely to have a clinical review on this recently defined entity. A systematic literature review, irrespective of language, retrieved 22 case series and single reports describing 122 patients with CRION between 2003 and 2013. We review the epidemiology, diagnostic workup, differential diagnosis, and treatment (acute, intermediate, and long term) in view of the collective data. These data suggest that CRION is a distinct nosological entity, which is seronegative for anti-aquaporin four auto-antibodies and recognized by and managed through its dependency on immuno-suppression. Revised diagnostic criteria are proposed in light of the data compromising a critical discussion of relevant limitations.

  4. Chronic Intestinal Inflammation: Inflammatory Bowel Disease and Colitis-Associated Colon Cancer

    Directory of Open Access Journals (Sweden)

    Deborah C. Rubin

    2012-05-01

    Full Text Available The inflammatory bowel diseases (IBD, including Crohn’s disease and ulcerative colitis, are chronic inflammatory disorders of the intestine. The prevalence in the United States is greater than 200 cases per 100,000, with the total number of IBD patients between 1 and 1.5 million. Crohn’s disease may affect all parts of the gastrointestinal tract, from mouth to anus, but most commonly involves the distal part of the small intestine or ileum, and colon. Ulcerative colitis results in colonic inflammation that can affect the rectum only, or can progress proximally to involve part of or the entire colon. Clinical symptoms include diarrhea, abdominal pain, gastrointestinal bleeding and weight loss. A serious long-term complication of chronic inflammation is the development of colorectal cancer. A genetic basis for IBD had long been recognized based on the increased familial risk. However, significant discordance for Crohn’s disease in twins, and a much less robust phenotypic concordance for ulcerative colitis, suggested additional factors play a role in disease pathogenesis, including environmental factors. In the past several years, progress in understanding the molecular basis of IBD has accelerated, beginning with the generation of animal models of colitis and progressing to the identification of specific genetic markers from candidate gene, gene linkage and genome wide association analyses . Genetic studies have also resulted in the recognition of the importance of environmental factors, particularly the crucial role of the gut microbiota in Crohn’s disease and ulcerative colitis. Altered immune responses to the normal intestinal flora are key factors in IBD pathogenesis. In this Research Topic, the genetic basis of IBD, the genetic and cellular alterations associated with colitis-associated colon cancer, and the emerging role of the intestinal microbiota and other environmental factors will be reviewed.

  5. [Mast cells, their adenosine receptors and reactive oxygen species in chronic inflammatory pathologies of childhood].

    Science.gov (United States)

    Renke, Joanna; Popadiuk, Stefan; Wozniak, Michał; Szlagatys-Sidorkiewicz, Agnieszka; Hansdorfer-Korzon, Rita

    2006-01-01

    Mast cells were described by Erhlich at the end of XIX-th century. Their role was deeply investigated in asthma and allergy. The massive degranulation of mast cells in allergy can lead to anaphylactic shock. Recently, mast cells have been recognized again as a very interesting topic for investigation, due to their possible role in chronic inflammation. Moreover, through adenosine receptors, mast cells can be activated or inactivated. That is why these cells are regarded as a potential target of new drugs. It has been reported, that mast cells generate intracellular reactive oxygen species (ROS) in response to stimulation with divergent physiologically relevant stimulants. The intensification of ROS production may be measured by the level of carbonyl groups, as a marker of protein peroxidation. However, the role of mast cells in other than asthma diseases with chronic inflammation needs further investigation. It was found out that the information about mast cell distribution in colonic mucosa may serve as help in differentiation between inflammatory bowel disease and collagenous colitis. Moreover, its accumulation in focal active gastritis was confirmed in patients with Crohn's disease. An important role in regulation of inflammatory process seems to be reserved for adenosine receptors present on mastocytes. The activation of mast cells through the adenosine receptor is connected with 11-8 release, which stimulate the migration of leukocytes and oxidation reactions. The detection of mast cells in tissues should not be limited only to the simple histologic examination. It should be completed by the detection of products of degranulation, e.g. tryptase. This is the way to find out their actual function and state of activation. PMID:17203808

  6. The Case for Increased Physical Activity in Chronic Inflammatory Bowel Disease: A Brief Review.

    Science.gov (United States)

    Shephard, R J

    2016-06-01

    Regular physical activity reduces the risk of colon cancer, but there is little information on the merits of such activity in the prevention and management of chronic inflammatory bowel disease (CIBD). The present systematic review thus documents current levels of habitual physical activity and aerobic and muscular function in CIBD, and examines the safety, practicality and efficacy of exercise programmes in countering the disease process, correcting functional deficits and enhancing quality of life. A systematic search of the Ovid/Medline database from January 1996 to May 2015 linked the terms physical activity/motor activity/physical fitness/physical training/physical education/training/exercise/exercise therapy with Crohn's disease/colitis/ulcerative colitis/inflammatory bowel disease, supplementing this information by a scanning of reference lists and personal files.12 of 16 published studies show a low level of habitual physical activity in CIBD, with sub-normal values for aerobic power, lean tissue mass and muscular strength. 3 of 4 studies suggest physical activity may reduce the risk of developing IBD, and 11 interventions all note that exercise programmes are well tolerated with some decreases of disease activity, and functional gains leading to an increased health-related quality of life. Moreover, programme compliance rates compare favourably with those seen in the treatment of other chronic conditions. More information on mechanisms is needed, but regular moderate aerobic and/or resistance exercise improves the health status of patients with CIBD both by modulating immune function and by improving physical function. A regular exercise programme should thus become an important component in the management of CIBD. PMID:27116344

  7. Extracellular matrix assessment of infected chronic venous leg ulcers: role of metalloproteinases and inflammatory cytokines.

    Science.gov (United States)

    Serra, Raffaele; Grande, Raffaele; Buffone, Gianluca; Molinari, Vincenzo; Perri, Paolo; Perri, Aldina; Amato, Bruno; Colosimo, Manuela; de Franciscis, Stefano

    2016-02-01

    Chronic venous ulcer (CVU) represents a dreaded complication of chronic venous disease (CVD). The onset of infection may further delay the already precarious healing process in such lesions. Some evidences have shown that matrix metalloproteinases (MMPs) are involved and play a central role in both CVUs and infectious diseases. Two groups of patients were enrolled to evaluate the expression of MMPs in infected ulcers and the levels of inflammatory cytokines as well as their prevalence. Group I comprised 63 patients (36 females and 27 males with a median age of 68·7 years) with infected CVUs, and group II (control group) comprised 66 patients (38 females and 28 males with a median age of 61·2 years) with non-infected venous ulcers. MMP evaluation and dosage of inflammatory cytokines in plasma and wound fluid was performed by means of enzyme-linked immunosorbent assay test; protein extraction and immunoblot analysis were performed on biopsied wounds. The first three most common agents involved in CVUs were Staphylococcus aureus (38·09%), Corynebacterium striatum (19·05%) and Pseudomonas aeruginosa (12·7%). In this study, we documented overall higher levels of MMP-1 and MMP-8 in patients with infected ulcers compared to those with uninfected ulcers that showed higher levels of MMP-2 and MMP-9. We also documented higher levels of interleukin (IL)-1, IL-6, IL-8, vascular endothelial growth factor and tumour necrosis factor-alpha in patients with infected ulcers with respect to those with uninfected ulcers, documenting a possible association between infection, MMP activation, cytokine secretions and symptoms. The present results could represent the basis for further studies on drug use that mimic the action of tissue inhibitors of metalloproteinases in order to make infected CVU more manageable.

  8. The Anti-Inflammatory Actions of Auricular Point Acupressure for Chronic Low Back Pain.

    Science.gov (United States)

    Lin, Wei-Chun; Yeh, Chao Hsing; Chien, Lung-Chang; Morone, Natalia E; Glick, Ronald M; Albers, Kathryn M

    2015-01-01

    Background. Auricular point acupressure (APA) is a promising treatment for pain management. Few studies have investigated the physiological mechanisms of APA analgesics. Method. In this pilot randomized clinical trial (RCT), a 4-week APA treatment was used to manage chronic low back pain (CLBP). Sixty-one participants were randomized into a real APA group (n = 32) or a sham APA group (n = 29). Blood samples, pain intensity, and physical function were collected at baseline and after 4 weeks of treatment. Results. Subjects in the real APA group reported a 56% reduction of pain intensity and a 26% improvement in physical function. Serum blood samples showed (1) a decrease in IL-1β, IL-2, IL-6, and calcitonin gene-related peptide [CGRP] and (2) an increase in IL-4. In contrast, subjects in the sham APA group (1) reported a 9% reduction in pain and a 2% improvement in physical function and (2) exhibited minimal changes of inflammatory cytokines and neuropeptides. Statistically significant differences in IL-4 and CGRP expression between the real and sham APA groups were verified. Conclusion. These findings suggest that APA treatment affects pain intensity through modulation of the immune system, as reflected by APA-induced changes in serum inflammatory cytokine and neuropeptide levels. PMID:26170869

  9. Systemic inflammatory response to smoking in chronic obstructive pulmonary disease: evidence of a gender effect.

    Directory of Open Access Journals (Sweden)

    Rosa Faner

    Full Text Available BACKGROUND: Tobacco smoking is the main risk factor of chronic obstructive pulmonary disease (COPD but not all smokers develop the disease. An abnormal pulmonary and systemic inflammatory response to smoking is thought to play a major pathogenic role in COPD, but this has never been tested directly. METHODS: We studied the systemic biomarker and leukocyte transcriptomic response (Affymetrix microarrays to smoking exposure in 10 smokers with COPD and 10 smokers with normal spirometry. We also studied 10 healthy never smokers (not exposed to smoking as controls. Because some aspects of COPD may differ in males and females, and the inflammatory response to other stressors (infection might be different in man and women, we stratified participant recruitment by sex. Differentially expressed genes were validated by q-PCR. Ontology enrichment was evaluated and interaction networks inferred. RESULTS: Principal component analysis identified sex differences in the leukocyte transcriptomic response to acute smoking. In both genders, we identified genes that were differentially expressed in response to smoking exclusively in COPD patients (COPD related signature or smokers with normal spirometry (Smoking related signature, their ontologies and interaction networks. CONCLUSIONS: The use of an experimental intervention (smoking exposure to investigate the transcriptomic response of peripheral leukocytes in COPD is a step beyond the standard case-control transcriptomic profiling carried out so far, and has facilitated the identification of novel COPD and Smoking expression related signatures which differ in males and females.

  10. Subcutaneous injection of Mycobacterium ulcerans causes necrosis, chronic inflammatory response and fibrosis in skeletal muscle.

    Science.gov (United States)

    Houngbédji, Mabèrou Germain; Boissinot, Maurice; Bergeron, G Michel; Frenette, Jérôme

    2008-10-01

    Mycobacterium ulcerans (M. ulcerans) causes Buruli ulcer, a very debilitating disease that affects the skin and other tissues. The disease occurs mainly in children in sub-Sahara Africa. While contracture, fibrosis and functional limitation of range of motion are frequent complications of Buruli ulcer, no fundamental or clinical studies have investigated the impact of M. ulcerans infections on skeletal muscle. In the present study, we subcutaneously infected mice in the proximity of the right biceps muscle to evaluate the histological, biochemical and functional impact of M. ulcerans on skeletal muscles. The concentration of mast cells decreased but the number of neutrophils and macrophages increased steadily in proximate-infected biceps muscles. Pro- and anti-inflammatory cytokines as well as fibrogenic growth factor mRNA also increased. Significantly more membrane damage and fibrosis occurred in proximate-infected biceps muscles than in control and sham muscles. Passive biomechanical testing also revealed that the presence of M. ulcerans increased muscle stiffness. These findings show for the first time that M. ulcerans can induce local and chronic inflammatory responses in skeletal muscles that are associated with muscle fiber damage and fibrosis.

  11. Impact of hepcidin, interleukin 6, and other inflammatory markers with respect to erythropoietin on anemia in chronic hemodialysis patients

    Directory of Open Access Journals (Sweden)

    Ihab A. Ibrahim

    2014-01-01

    Conclusion Serum hepcidin levels were associated with iron status and inflammation in maintenance hemodialysis patients, and the high hepcidin serum levels, found in hemodialysis (HD patients, are dependent on the magnitude of the inflammatory process and on recombinant human erythropoietin doses. Hepcidin and its regulatory pathways are potential therapeutic targets, which could lead to effective treatment of anemia in chronic hemodialysis.

  12. Interferon-gamma (IFN-gamma) treatment decreases the inflammatory response in chronic Pseudomonas aeruginosa pneumonia in rats

    DEFF Research Database (Denmark)

    Johansen, H K; Hougen, H P; Rygaard, J;

    1996-01-01

    In a rat model of chronic Pseudomonas aeruginosa lung infection mimicking cystic fibrosis (CF), we studied whether the inflammatory response could be altered by intraperitoneal treatment with recombinant rat interferon-gamma (rrIFN-gamma). Rats were treated either before or after intratracheal ch...

  13. A Pathogenic Role for CD8+ T Cells in a Spontaneous Model of Demyelinating Disease

    DEFF Research Database (Denmark)

    Brisebois, Marcel; Zehntner, Simone P.; Estrada, José;

    2006-01-01

    Transgenic (Tg) mice that overexpress the costimulatory ligand B7.2/CD86 on microglia spontaneously develop a T cell-mediated demyelinating disease. Characterization of the inflammatory infiltrates in the nervous tissue revealed a predominance of CD8+ T cells, suggesting a prominent role of this T...... pathogenesis. Collectively, our data indicate that the spontaneous demyelinating disease in this animal model occurs as a consequence of an inflammatory response initiated through the activation of CNS-specific CD8+ T cells by Tg expression of B7.2 within the target organ. Thus, autoreactive CD8+ T cells can...

  14. Chronic inflammatory state in sickle cell anemia patients is associated with HBB(*)S haplotype.

    Science.gov (United States)

    Bandeira, Izabel C J; Rocha, Lillianne B S; Barbosa, Maritza C; Elias, Darcielle B D; Querioz, José A N; Freitas, Max Vitor Carioca; Gonçalves, Romélia P

    2014-02-01

    The chronic inflammatory state in sickle cell anemia (SCA) is associated with several factors such as the following: endothelial damage; increased production of reactive oxygen species; hemolysis; increased expression of adhesion molecules by leukocytes, erythrocytes, and platelets; and increased production of proinflammatory cytokines. Genetic characteristics affecting the clinical severity of SCA include variations in the hemoglobin F (HbF) level, coexistence of alpha-thalassemia, and the haplotype associated with the HbS gene. The different haplotypes of SCA are Bantu, Benin, Senegal, Cameroon, and Arab-Indian. These haplotypes are associated with ethnic groups and also based on the geographical origin. Studies have shown that the Bantu haplotype is associated with higher incidence of clinical complications than the other haplotypes and is therefore considered to have the worst prognosis. This study aimed to evaluate the profile of the proinflammatory cytokines interleukin-6, tumor necrosis factor-α, and interleukin-17 in patients with SCA and also to assess the haplotypes associated with beta globin cluster S (HBB(*)S). We analyzed a total of 62 patients who had SCA and had been treated with hydroxyurea; they had received a dose ranging between 15 and 25 (20.0±0.6)mg/kg/day for 6-60 (18±3.4)months; their data were compared with those for 30 normal individuals. The presence of HbS was detected and the haplotypes of the beta S gene cluster were analyzed by polymerase chain reaction-restriction fragment length polymorphism (PCR-RFLP). Our study demonstrated that SCA patients have increased inflammatory profile when compared to the healthy individuals. Further, analysis of the association between the haplotypes and inflammatory profile showed that the levels of IL-6 and TNF-α were greater in subjects with the Bantu/Bantu haplotype than in subjects with the Benin/Benin haplotype. The Bantu/Benin haplotype individuals had lower levels of cytokines than those with

  15. Inflammatory response in chronic degenerative endometritis mares treated with platelet-rich plasma.

    Science.gov (United States)

    Reghini, Maria Fernanda S; Ramires Neto, Carlos; Segabinazzi, Lorenzo G; Castro Chaves, Maria Manoela B; Dell'Aqua, Camila de Paula F; Bussiere, Maria Clara C; Dell'Aqua, José Antonio; Papa, Frederico O; Alvarenga, Marco Antonio

    2016-07-15

    Degenerative changes of the endometrium are directly related to age and fertility in mares. Chronic degenerative endometritis (CDE) is correlated with uterine fluid retention and reduced ability to clear uterine inflammation. Recent research in the areas of equine surgery and sports medicine has shown that platelet-rich plasma (PRP) treatment acts as an immunomodulator of the inflammatory response. Therefore, the aim of this study was to determine if the uterine infusion of PRP could modulate the local inflammatory response and modify the intrauterine NO concentrations after artificial insemination (AI) in both normal mares and those with CDE. Thirteen mares with endometrium classified as grade III on the histology (mares with CDE) and eight mares with endometrial histological classification I or II-a normal mares were selected to investigate the effect of PRP therapy. The mares were inseminated with fresh semen in two consecutive cycles in a crossover study design. Thereby, each mare served as its own control and the treatment was performed with intrauterine PRP infusion four hours after AI. The percentage of neutrophils in uterine cytology (CIT, %), uterine fluid accumulation observed on ultrasonography (FLU, mm) and nitric oxide concentration of uterine fluid (NO, μM) were analyzed before and 24 hours after AI. The results reported that mares with CDE (CIT, 68.3 ± 3.27, FLU, 10.7 ± 1.61) have a higher (P  0.05) between categories of mares. In treated cycles with PRP, the intrauterine inflammatory response decrease (P < 0.05) in CDE mares (CDE: CIT, 31.4 ± 6.48, FLU, 5.5 ± 1.28; normal mares: CIT, 13.5 ± 4.31, FLU, 0) when compared with nontreated cycle (CDE: CIT, 68.3 ± 3.27, FLU, 10.7 ± 1.61; NM: CIT, 24.4 ± 3.56, FLU, 0), but did not modify NO concentrations in uterine fluid. Thus, we can conclude that PRP was effective in modulating the exacerbated uterine inflammatory response to semen in mares with CDE but did not reduce NO

  16. Chronic inflammatory diseases are stimulated by current lifestyle: how diet, stress levels and medication prevent our body from recovering

    Directory of Open Access Journals (Sweden)

    Bosma-den Boer Margarethe M

    2012-04-01

    Full Text Available Abstract Serhan and colleagues introduced the term "Resoleomics" in 1996 as the process of inflammation resolution. The major discovery of Serhan's work is that onset to conclusion of an inflammation is a controlled process of the immune system (IS and not simply the consequence of an extinguished or "exhausted" immune reaction. Resoleomics can be considered as the evolutionary mechanism of restoring homeostatic balances after injury, inflammation and infection. Under normal circumstances, Resoleomics should be able to conclude inflammatory responses. Considering the modern pandemic increase of chronic medical and psychiatric illnesses involving chronic inflammation, it has become apparent that Resoleomics is not fulfilling its potential resolving capacity. We suggest that recent drastic changes in lifestyle, including diet and psycho-emotional stress, are responsible for inflammation and for disturbances in Resoleomics. In addition, current interventions, like chronic use of anti-inflammatory medication, suppress Resoleomics. These new lifestyle factors, including the use of medication, should be considered health hazards, as they are capable of long-term or chronic activation of the central stress axes. The IS is designed to produce solutions for fast, intensive hazards, not to cope with long-term, chronic stimulation. The never-ending stress factors of recent lifestyle changes have pushed the IS and the central stress system into a constant state of activity, leading to chronically unresolved inflammation and increased vulnerability for chronic disease. Our hypothesis is that modern diet, increased psycho-emotional stress and chronic use of anti-inflammatory medication disrupt the natural process of inflammation resolution ie Resoleomics.

  17. A STUDY OF ANTI - INFLAMMATORY ACTIVITY OF PLANT “TRIANTHEMA PORTULACASTRUM” IN CHRONIC MODELS OF INFLAMMATION

    Directory of Open Access Journals (Sweden)

    Suresh. S

    2015-06-01

    Full Text Available BACKGROUND: Trianthema portulacastrum is being used in Ayurveda since centuries for its medicinal values , hence this study was done to know if it has got anti - inflammatory activity in chronic models of inflammation, MATERIALS AND METHODS: Wistar albino rats were treated with whole plant ethanolic extract of trianthema portulacastrum 100mg \\ kg orally with 2% gum acacia , as suspending agent and indomethacin 20mg \\ kg as standard. And the effects were observed in chronic model of inflammation namely, rexin pellet induced granuloma model, RESULT: This study demon - strated that trianthema portulacastrum reduced significantly the dry weight of granuloma that was formed after rexin pellet implantation, CONCLUSION: Trianthema po rtulacastrum has got significant anti - inflammatory activity in chronic models of inflammation.

  18. Nutmeg oil alleviates chronic inflammatory pain through inhibition of COX-2 expression and substance P release in vivo

    Directory of Open Access Journals (Sweden)

    Wei Kevin Zhang

    2016-04-01

    Full Text Available Background: Chronic pain, or sometimes referred to as persistent pain, reduces the life quality of patients who are suffering from chronic diseases such as inflammatory diseases, cancer and diabetes. Hence, herbal medicines draw many attentions and have been shown effective in the treatment or relief of pain. Methods and Results: Here in this study, we used the CFA-injected rats as a sustainable pain model to test the anti-inflammatory and analgesic effect of nutmeg oil, a spice flavor additive to beverages and baked goods produced from the seed of Myristica fragrans tree. Conclusions: We have demonstrated that nutmeg oil could potentially alleviate the CFA-injection induced joint swelling, mechanical allodynia and heat hyperanalgesia of rats through inhibition of COX-2 expression and blood substance P level, which made it possible for nutmeg oil to be a potential chronic pain reliever.

  19. Significance of oligoclonal bands,myelin basic protein antibody and myelin oligodendrocyte glycoprotein anti-body in diagnosis of inflammatory central nervous system demyelinating diseases%OCB、抗MBP抗体及抗MOG抗体在中枢神经系统炎性脱髓鞘疾病诊断中的意义

    Institute of Scientific and Technical Information of China (English)

    覃惠洵; 刘琳琳; 郝洪军; 高枫

    2014-01-01

    目的:通过观察中枢神经系统炎性脱髓鞘疾病患者寡克隆区带(OCB)、抗髓鞘碱性蛋白(MBP)抗体及抗髓鞘少突胶质细胞糖蛋白(M OG )抗体的阳性率,为临床诊断提供一定的参考。方法入组91例中枢神经系统炎性脱髓鞘疾病患者为观察组(其中MS 30例,NMO 61例)及50例神经系统非炎性病变患者为对照组。首先分析观察组与对照组间OCB、脑脊液及血清中抗MBP抗体、抗MOG抗体阳性率的差异,再分析观察组中MS亚组及NMO亚组间上述指标阳性率的差异。结果2组比较,OCB、血清抗MBP抗体及血清抗MOG抗体阳性率比较差异有统计学意义(P<0.05),脑脊液抗MBP抗体及脑脊液抗MOG抗体阳性率比较差异无统计学意义(P>0.05)。MS亚组与NMO亚组,OCB、血清抗MBP抗体阳性率比较差异有统计学意义(P<0.05),脑脊液抗MBP抗体、血清抗MOG抗体及脑脊液抗MOG抗体阳性率比较差异无统计学意义(P>0.05)。结论 OCB阳性对诊断MS具有重要意义。血清抗MBP抗体及抗MOG抗体可为中枢神经系统炎性脱髓鞘疾病的诊断提供重要实验室依据,血清抗MBP抗体在MS与NMO的鉴别诊断中具有一定价值。%Objective To observe the positive rate of oligoclonal bands (OCB) ,myelin basic protein (MBP) antibody and myelin oligodendrocyte glycoprotein (MOG) antibody in patients of inflammatory central nervous system demyelinating disea-ses ,in order to give help to clinical diagnosis in these kind of diseases.Methods The observation group consisted of 91 cases of inflammatory central nervous system demyelinating diseases (30 cases of MS and 61 cases of NMO) ,and the control group con-sisted of 50 cases of neurological non-inflammatory diseases. The statistical analysis was developed on data of the positive rate of OCB ,MBP and MOG antibody in cerebrospinal fluid and serum between the observation group and control group. The

  20. Gadolinium enhancement patterns of tumefactive demyelinating lesions: correlations with brain biopsy findings and pathophysiology.

    Science.gov (United States)

    Kobayashi, Masaki; Shimizu, Yuko; Shibata, Noriyuki; Uchiyama, Shinichiro

    2014-10-01

    Tumefactive demyelinating lesions (TDLs) can mimic brain tumors on radiological images. TDLs are often referred to as tumefactive multiple sclerosis (TMS), but the heterogeneous nature and monophasic course of TDLs do not fulfill clinical and magnetic resonance imaging (MRI) criteria for multiple sclerosis. Redefining TDLs, TMS and other inflammatory brain lesions is essential for the accurate clinical diagnosis of extensive demyelinating brain lesions. We retrospectively analyzed MRI from nine TDL cases that underwent brain biopsy. Patterns of gadolinium enhancement on MRI were categorized as homogenous, inhomogeneous, patchy and diffuse, open ring or irregular rim, and were compared with pathological hallmarks including demyelination, central necrosis, macrophage infiltration, angiogenesis and perivascular lymphocytic cuffing. All cases had coexistence of demyelinating features and axonal loss. Open-ring and irregular rim patterns of gadolinium enhancement were associated with macrophage infiltrations and angiogenesis at the inflammatory border. An inhomogeneous pattern of gadolinium enhancement was associated with perivascular lymphocytic cuffing. Central necrosis was seen in cases of severe multiple sclerosis and hemorrhagic leukoencephalopathy. These results suggest that the radiological features of TDLs may be related to different pathological processes, and indicate that MRI may be useful in understanding their pathophysiology. Further investigation is needed to determine the precise disease entity of these inflammatory demyelinating brain lesions.

  1. The role of chronic inflammation in the development of gastrointestinal cancers: reviewing cancer prevention with natural anti-inflammatory intervention.

    Science.gov (United States)

    Lee, Ho-Jae; Park, Jong-Min; Han, Young Min; Gil, Hong Kwon; Kim, Jinhyung; Chang, Ji Young; Jeong, Migyeong; Go, Eun-Jin; Hahm, Ki Baik

    2016-01-01

    Inflammatory mediators alter the local environment of tumors, known as the tumor microenvironment. Mechanistically, chronic inflammation induces DNA damage, but understanding this hazard may help in the search for new chemopreventive agents for gastrointestinal (GI) cancer which attenuate inflammation. In the clinic, GI cancer still remains a major cause of cancer-associated mortality, chemoprevention with anti-inflammatory agents is thought to be a realistic approach to reduce GI cancer. Proton pump inhibitors, monoclonal antibodies targeting tumor necrosis factor-alpha, anti-sense targeted smad7 and non-steroidal anti-inflammatory agents have been investigated for their potential to prevent inflammation-based GI cancer. Besides these, a wide variety of natural products have also shown potential for the prevention of GI cancer. In this review, the authors will provide insights to explain the mechanistic connection between inflammation and GI cancer, as well as describe a feasible cancer prevention strategy based on anti-inflammatory treatments.

  2. [THE CHARACTERISTICS OF MORPHOLOGY OF BIOFILM OF PERIODONTIUM UNDER INFLAMMATORY DISEASES OF GUMS (CHRONIC CATARRHAL GINGIVITIS, CHRONIC PERIODONTITIS, CANDIDA-ASSOCIATED PERIODONTITIS) ACCORDING RESULTS OF ELECTRONIC MICROSCOPY].

    Science.gov (United States)

    Ippolitov, E V; Didenko, L V; Tzarev, V N

    2015-12-01

    The study was carried out to analyze morphology of biofilm of periodontium and to develop electronic microscopic criteria of differentiated diagnostic of inflammatory diseases of gums. The scanning electronic microscopy was applied to analyze samples of bioflm of periodont from 70 patients. Including ten patients with every nosologic form of groups with chronic catarrhal periodontitis. of light, mean and severe degree, chronic catarrhal gingivitis, Candida-associated paroperiodontitis and 20 healthy persons with intact periodontium. The analysis was implemented using dual-beam scanning electronic microscope Quanta 200 3D (FEI company, USA) and walk-through electronic micJEM 100B (JEOL, Japan). To detect marker DNA of periodont pathogenic bacteria in analyzed samples the kit of reagentsfor polymerase chain reaction "MultiDent-5" ("GenLab", Russia). The scanning electronic microscopy in combination with transmission electronic microscopy and polymerase chain reaction permits analyzing structure, composition and degree of development of biofilm of periodontium and to apply differentiated diagnostic of different nosologic forms of inflammatory diseases of periodontium, including light form of chronic periodontitis and gingivitis. The electronic microscopical indications of diseases ofperiodontium of inflammatory character are established: catarrhal gingivitis, (coccal morphological alternate), chronic periodontitis (bacillary morphological alternate), Candida-associated periodontitis (Candida morphological alternate of biofilm ofperiodontium).

  3. [THE CHARACTERISTICS OF MORPHOLOGY OF BIOFILM OF PERIODONTIUM UNDER INFLAMMATORY DISEASES OF GUMS (CHRONIC CATARRHAL GINGIVITIS, CHRONIC PERIODONTITIS, CANDIDA-ASSOCIATED PERIODONTITIS) ACCORDING RESULTS OF ELECTRONIC MICROSCOPY].

    Science.gov (United States)

    Ippolitov, E V; Didenko, L V; Tzarev, V N

    2015-12-01

    The study was carried out to analyze morphology of biofilm of periodontium and to develop electronic microscopic criteria of differentiated diagnostic of inflammatory diseases of gums. The scanning electronic microscopy was applied to analyze samples of bioflm of periodont from 70 patients. Including ten patients with every nosologic form of groups with chronic catarrhal periodontitis. of light, mean and severe degree, chronic catarrhal gingivitis, Candida-associated paroperiodontitis and 20 healthy persons with intact periodontium. The analysis was implemented using dual-beam scanning electronic microscope Quanta 200 3D (FEI company, USA) and walk-through electronic micJEM 100B (JEOL, Japan). To detect marker DNA of periodont pathogenic bacteria in analyzed samples the kit of reagentsfor polymerase chain reaction "MultiDent-5" ("GenLab", Russia). The scanning electronic microscopy in combination with transmission electronic microscopy and polymerase chain reaction permits analyzing structure, composition and degree of development of biofilm of periodontium and to apply differentiated diagnostic of different nosologic forms of inflammatory diseases of periodontium, including light form of chronic periodontitis and gingivitis. The electronic microscopical indications of diseases ofperiodontium of inflammatory character are established: catarrhal gingivitis, (coccal morphological alternate), chronic periodontitis (bacillary morphological alternate), Candida-associated periodontitis (Candida morphological alternate of biofilm ofperiodontium). PMID:27032256

  4. Inflammatory airway features and hypothalamic-pituitary adrenal axis function in asthmatic rats combined with chronic obstructive pulmonary disease

    Institute of Scientific and Technical Information of China (English)

    CAI Cui; CAO Yu-xue; ZHANG Hong-ying; LE Jing-jing; DONG Jing-cheng; CUI Yan; XU Chang-qing; LIU Bao-jun; WU Jin-feng; DUAN Xiao-hong

    2010-01-01

    Background Bronchial asthma (BA) and chronic obstructive pulmonary disease (COPD) are both inflammatory airway diseases with different characteristics. However, there are many patients who suffer from both BA and COPD. This study was to evaluate changes of inflammatory airway features and hypothalamic-pituitary-adrenal (HPA) axis function in asthmatic rats combined with COPD.Methods Brown Norway (BN) rats were used to model the inflammatory airway diseases of BA, COPD and COPD+BA.These three models were compared and evaluated with respect to clinical symptoms, pulmonary histopathology, airway hyperresponsiveness (AHR), inflammatory cytokines and HPA axis function.Results The inflammatory airway features and HPA axis function in rats in the COPD+BA model group were greatly influenced. Rats in this model group showed features of the inflammatory diseases BA and COPD. The expression of inflammatory cytokines in this model group might be up or downregulated when both disease processes are present. The levels of corticotrophin releasing hormone mRNA and corticosterone in this model group were both significantly decreased than those in the control group (P <0.05).Conclusions BN rat can be used as an animal model of COPD+BA. By evaluating this animal model we found that the features of inflammation in rats in this model group seem to be exaggerated. The HPA axis functions in rats in this model group have been disturbed or impaired, which is prominent at the hypothalamic level.

  5. Aerobic exercise improves the inflammatory profile correlated with cardiac remodeling and function in chronic heart failure rats

    Directory of Open Access Journals (Sweden)

    Ramiro B. Nunes

    2013-06-01

    Full Text Available OBJECTIVE: The aim of the present study was to evaluate the effect of 8 weeks of aerobic exercise training on cardiac functioning and remodeling and on the plasma levels of inflammatory cytokines in chronic heart failure rats. METHODS: Wistar rats were subjected to myocardial infarction or sham surgery and assigned to 4 groups: chronic heart failure trained (n = 7, chronic heart failure sedentary (n = 6, sham trained (n = 8 and sham sedentary (n = 8. Four weeks after the surgical procedures, the rats were subjected to aerobic training in the form of treadmill running (50 min/day, 5 times per week, 16 m/min. At the end of 8 weeks, the rats were placed under anesthesia, the hemodynamic variables were recorded and blood samples were collected. Cardiac hypertrophy was evaluated using the left ventricular weight/body weight ratio, and the collagen volume fraction was assessed using histology. RESULTS: The chronic heart failure trained group showed a reduction in left ventricular end-diastolic pressure, a lower left ventricular weight/body weight ratio and a lower collagen volume fraction compared with the chronic heart failure sedentary group. In addition, exercise training reduced the plasma levels of TNF-α and IL-6 and increased the plasma level of IL-10. CONCLUSION: An 8-week aerobic exercise training program improved the inflammatory profile and cardiac function and attenuated cardiac remodeling in chronic heart failure rats.

  6. Aspirin or Nonsteroidal Anti-inflammatory Drug-Exacerbated Chronic Rhinosinusitis.

    Science.gov (United States)

    Ledford, Dennis K; Lockey, Richard F

    2016-01-01

    Aspirin (ASA)-exacerbated respiratory disease (AERD) is characterized by upper airway congestion due to eosinophilic inflammation of the nasal and sinus membranes and nasal polyposis, associated with increased leukotriene production that is further accentuated by ASA or other nonsteroidal anti-inflammatory drug (NSAID) ingestion. It occurs in 5% to 10% of subjects with chronic rhinosinusitis (CRS) and in 15% to 40% of those with nasal polyposis. Although AERD with CRS is usually associated with asthma, this is not always the case. The eosinophilic airway inflammation and symptoms precede clinical reactions to ASA or other NSAIDs, but ultimately affected subjects experience worsening of symptoms with ingestion of ASA/NSAIDs. The endotypic mechanism for this worsening is related to a chronic increase in leukotriene and a decrease in prostaglandin production, particularly prostaglandin E2, that is further aggravated by the inhibition of cycloxgenase I. IgE does not likely play a role in the pathogenesis of the disease although nasal and sinus staphylococcal infection increases local IgE level and may increase total IgE and specific IgE levels. Genetic studies suggest that multiple genes may be involved, but the genetic abnormalities may differ in affected subjects from different ethnicities and candidate genes have not been confirmed in multiple studies. Genome-wide association studies have not been revealing. The phenotype is recognized by the mucosal inflammation and worsening of symptoms acutely with ASA/NSAID. There is clinical improvement with ASA desensitization followed by regular ingestion of ASA or other NSAIDs. Further understanding of this unique phenotype and endotype of CRS will likely improve the understanding of other eosinophilic airway diseases. PMID:27393773

  7. Evaluation of the inflammatory activity in chronic osteomyelitis. Contribution of the scintigraphy with polyclonal antibodies

    International Nuclear Information System (INIS)

    Active chronic osteomyelitis or complicating osteomyelitis (superimposed on diseases that changes the normal bone structure fractures, post-surgery, prosthesis) can be difficult to diagnose by anatomic radiological imaging modalities, like plain radiograph and CT. These diseases frequently cause also increased bone remodeling, leading to nonspecific uptake of Tc-99m-bone scan agents and gallium-67. New radiopharmaceuticals with greater inflammation/infection avidity and specificity are being developed, including the nonspecific polyclonal immunoglobulin (IgG) labeled with technetium-99. Tc-99m-IgG may be available as a ready to use kit, with no reported side effects, low patient absorbed radiation dose and low cost. The mechanism of IgG uptake at the inflammation site has not been fully elucidated yet. Specific (receptor linking, physico-chemical immunoglobulin properties) and nonspecific mechanisms (enhanced vascular permeability and macromolecular exudate) has been suggested. IgG scintigraphy results are affected by the isotope, labeling procedure adopted and characteristics of the inflammatory focus. Nineteen patients with suspected osteomyelitis (active chronic osteomyelitis or violated bone osteomyelitis) were studied by Tc-99m-IgG scintigraphy (directly labeled polyclonal immunoglobulin, Sandoglobuilina - Sandoz). All patients also underwent standard three-phase bone scintigraphy using methylene diphosphonate (Tc-99m-MDP), gallium-67 scintigraphy and plain radiographs. Infection was found in 8 sites. Sensitivity and specificity for Tc-99m-MDP, gallium-67 and Tc 99m-IgG scintigraphy were, respectively, 88 and 36%, 75 and 73%,88 and 82%. All patients with false positive IgG scintigraphies had previous surgery. Other current scintigraphic procedures used in the diagnosis of osteomyelitis are also reviewed. (author)

  8. Effects of chronic inflammatory bowel diseases on left ventricular structure and function: a study protocol

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    Botti Fiorenzo

    2002-09-01

    Full Text Available Abstract Background Experimental evidences suggest an increased collagen deposition in inflammatory bowel diseases (IBD. In particular, large amounts of collagen type I, III and V have been described and correlated to the development of intestinal fibrotic lesions. No information has been available until now about the possible increased collagen deposition far from the main target organ. In the hypothesis that chronic inflammation and increased collagen metabolism are reflected also in the systemic circulation, we aimed this study to evaluate the effects on left ventricular wall structure by assessing splancnic and systemic collagen metabolism (procollagen III assay, deposition (ultrasonic tissue characterization, and cardiac function (echocardiography in patients with different long standing history of IBD, before and after surgery. Methods Thirty patients affected by active IBD, 15 with Crohn and 15 with Ulcerative Colitis, submitted to surgery will be enrolled in the study in a double blind fashion. They will be studied before the surgical operation and 6, 12 months after surgery. A control group of 15 healthy age and gender-matched subjects will also be studied. At each interval blood samples will be collected in order to assess the collagen metabolism; a transthoracic echocardiogram will be recorded for the subsequent determination of cardiac function and collagen deposition. Discussion From this study protocol we expect additional information about the association between IBD and cardiovascular disorders; in particular to address the question if chronic inflammation, through the altered collagen metabolism, could affect left ventricular structure and function in a manner directly related to the estimated duration of the disease.

  9. Systemic inflammatory responses in patients with type 2 diabetes with chronic periodontitis

    Science.gov (United States)

    Mesia, Ruben; Gholami, Fatemeh; Huang, Hong; Clare-Salzler, Michael; Aukhil, Ikramuddin; Wallet, Shannon M; Shaddox, Luciana M

    2016-01-01

    Objective The objective of this case–control study was to quantify the immune responsiveness in individuals with type 2 diabetes (T2D) as compared with patients without diabetes (NT2D) diagnosed with periodontitis. Research Design and Methods Peripheral blood was collected from 20 patients with moderate-to-severe chronic periodontitis (10 T2D, 10 NT2D). Blood samples were stimulated with ultrapure Porphyromonas gingivalis and Escherichia coli lipopolysaccharide (LPS) for 24 hours. 14 cytokines/chemokines were quantified in culture supernatants using multiplex technology. Results T2D individuals demonstrated higher unstimulated levels of interleukin 6 (IL-6), IL-1β, tumor necrosis factor α, interferon γ, IL-10, IL-8, macrophage inflammatory protein 1α (MIP1α), and 1β (MIP1β), and higher stimulated levels of IL-6, IL-8, IL-10, MIP1α and MIP1β, along with lower unstimulated and stimulated levels of granulocyte-macrophage colony-stimulating factor (GM-CSF) when compared with NT2D (pperiodontitis, patients with T2D seem to have an enhanced LPS-induced immune responsiveness than individuals without diabetes, which correlates with periodontal disease severity, concomitant with a less robust GM-CSF response. This data may in part explain the higher predisposition to periodontitis in this population. PMID:27651910

  10. Anemia of Chronic Disease and Iron Deficiency Anemia in Inflammatory Bowel Diseases: Pathophysiology, Diagnosis, and Treatment.

    Science.gov (United States)

    Murawska, Natalia; Fabisiak, Adam; Fichna, Jakub

    2016-05-01

    Anemia coexists with inflammatory bowel disease (IBD) in up to two-thirds of patients, significantly impairing quality of life. The most common types of anemia in patients with IBD are iron deficiency anemia and anemia of chronic disease, which often overlap. In most cases, available laboratory tests allow successful diagnosis of iron deficiency, where difficulties appear, recently established indices such as soluble transferrin-ferritin ratio or percentage of hypochromic red cells are used. In this review, we discuss the management of the most common types of anemia in respect of the latest available data. Thus, we provide the mechanisms underlying pathophysiology of these entities; furthermore, we discuss the role of hepcidin in developing anemia in IBD. Next, we present the treatment options for each type of anemia and highlight the importance of individual choice of action. We also focus on newly developed intravenous iron preparations and novel, promising drug candidates targeting hepcidin. Concurrently, we talk about difficulties in differentiating between the true and functional iron deficiency, and discuss tools facilitating the process. Finally, we emphasize the importance of proper diagnosis and treatment of anemia in IBD. We conclude that management of anemia in patients with IBD is tricky, and appropriate screening of patients regarding anemia is substantial. PMID:26818422

  11. Chronic Stanford type A aortic dissection manifesting as systemic inflammatory disorder

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    Yana Kogan

    2016-05-01

    Full Text Available Typical presentation of type A aortic dissection usually encompasses severe acute chest pain, frequently radiating to the upper back, which is seen in more than 80% of the patients, while isolated back or abdominal pain have been repeatedly reported as the first manifestation of the disease as well. Occasionally, dyspnea due to acute aortic regurgitation, syncope, or stroke, secondary to obstruction of major cerebral vessels, have also been described at presentation of type A aortic dissection. Presentation of aortic dissection as a prolonged systemic illness with a number of nonspecific clinical and laboratory findings, such as low-grade fever, fatigue, malaise, weight loss, anemia, elevated acute phase response laboratory parameters, and absence of any of typical clinical features of the dissection syndrome has been only rarely reported. We describe a patient with type A chronic aortic dissection, manifesting as a systemic inflammatory disorder in the absence of acute chest syndrome. The diagnosis was made accidentally by computed tomography, ordered in the course of the regular work up. The patient underwent emergent surgery with resection and grafting of the dissected aorta. Pathological investigation demonstrated intense acute inflammation with neutrophilic infiltration in the vicinity of the intramural hemorrhage and necrosis, as well as granulation tissue with new vessels formation and collagen deposition in the outer media. The possible pathogenic mechanisms of the phenomenon are discussed. [Int J Res Med Sci 2016; 4(5.000: 1768-1771

  12. Natural Products as Tools for Defining How Cellular Metabolism Influences Cellular Immune and Inflammatory Function during Chronic Infection

    Directory of Open Access Journals (Sweden)

    Erica S. Lovelace

    2015-11-01

    Full Text Available Chronic viral infections like those caused by hepatitis C virus (HCV and human immunodeficiency virus (HIV cause disease that establishes an ongoing state of chronic inflammation. While there have been tremendous improvements towards curing HCV with directly acting antiviral agents (DAA and keeping HIV viral loads below detection with antiretroviral therapy (ART, there is still a need to control inflammation in these diseases. Recent studies indicate that many natural products like curcumin, resveratrol and silymarin alter cellular metabolism and signal transduction pathways via enzymes such as adenosine monophosphate kinase (AMPK and mechanistic target of rapamycin (mTOR, and these pathways directly influence cellular inflammatory status (such as NF-κB and immune function. Natural products represent a vast toolkit to dissect and define how cellular metabolism controls cellular immune and inflammatory function.

  13. The possibility of evaluation on inflammatory change at respiratory tract in chronic bronchial asthma using 67Ga scintigraphy

    International Nuclear Information System (INIS)

    67Ga scintigraphy was performed in 17 patients with chronic bronchial asthma to grasp the inflammatory change of respiratory tract. On 67Ga scintigraphy, abnormal accumulations were detected on lung fields in 6 cases (35.3%) of 17 cases. In 5 cases of these 6 cases, the defect areas which were pointed out on 81mKr ventilation scintigraphy were matched to the abnormal accumulation areas which were pointed out on 67Ga scintigraphy. In dynamics, the abnormal accumulation areas which were pointed out on 67Ga scintigraphy were matched to the defect areas which had been at all times pointed out on 81mKr ventilation scintigraphy. 67Ga scintigraphy was expected to be one of index to grasp the inflammatory change of respiratory tract in patients with chronic bronchial asthma. (author)

  14. Estimation of efficiency eradication therapies at sick of inflammatory periodontal diseases against a chronic Helicobacter pylori gastritis

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    Bulkina N.V.

    2011-03-01

    Full Text Available As a result of the conducted research results of an estimation of efficiency of application of the general eradication are presented therapy and local therapy at sick of inflammatory periodontal diseases against a chronic gastritis. Authors notice a positive effect at application as pathogenetic therapy of balm for gums of "Asepta" that normalisation of level of hygiene of the oral cavity, proof remission of periodontal diseases against a pathology of a gastroenteric path allows to achieve

  15. Differentiation of chronical rhinosinusitis with and without nasal polyposis on basis of symptomatology, course of disease, inflammatory mediators and comorbidity

    OpenAIRE

    Traser, Louisa

    2012-01-01

    Recent cytokine and chemokine research has rapidly expanded our understanding of chronic rhinosinusitis (CRS) and underlined that it is necessary to divide this disease into subgroups. The objective of the present study was to investigate the differentiation of CRS patients with (CRSwNP) and without (CRSsNP) nasal polyposis on basis of symptomatology, course of disease, inflammatory mediators and comorbidity. Nasal Polyps (n=16), tissue samples taken from the paranasal sinuses (n=32) and ...

  16. Distinct Inflammatory Profiles in Atopic and Nonatopic Patients With Chronic Rhinosinustis Accompanied by Nasal Polyps in Western China

    OpenAIRE

    Ba, Luo; Du, Jintao; Liu, Feng; Yang, Fenglin; Han, Miaomiao; Liu, Sixi; Lin, Ping; Li, Huabin

    2015-01-01

    Purpose The role of systemic sensitization in the pathophysiology of chronic rhinosinusitis with nasal polyps (CRSwNP) remains elusive. This study sought to characterize the pattern of cytokines in polyp tissues from atopic and nonatopic patients with CRSwNP. Methods Atopic and nonatopic polyp and normal tissues were collected from 70 CRSwNP patients and 26 control subjects, respectively. The distribution of inflammatory cells (eosinophils, neutrophils, mast cells, etc.) were examined using i...

  17. Polymorphism in the Alpha Cardiac Muscle Actin 1 Gene Is Associated to Susceptibility to Chronic Inflammatory Cardiomyopathy

    OpenAIRE

    Amanda Farage Frade; Priscila Camilo Teixeira; Barbara Maria Ianni; Cristina Wide Pissetti; Bruno Saba; Lin Hui Tzu Wang; Andréia Kuramoto; Luciana Gabriel Nogueira; Paula Buck; Fabrício Dias; Helene Giniaux; Agnes Llored; Sthefanny Alves; Andre Schmidt; Eduardo Donadi

    2013-01-01

    AIMS: Chagas disease, caused by the protozoan Trypanosoma cruzi is endemic in Latin America, and may lead to a life-threatening inflammatory dilated, chronic Chagas cardiomyopathy (CCC). One third of T. cruzi-infected individuals progress to CCC while the others remain asymptomatic (ASY). A possible genetic component to disease progression was suggested by familial aggregation of cases and the association of markers of innate and adaptive immunity genes with CCC development. Since mutations i...

  18. CCR1 and CCR5 expression on inflammatory cells is related to cigarette smoking and chronic obstructive pulmonary disease severity

    Institute of Scientific and Technical Information of China (English)

    WANG Fei; HE Bei

    2012-01-01

    Background Chronic obstructive pulmonary disease (COPD) is a progressive disease associated with a cellular inflammatory response mostly concerned with cigarette smoking.Chemokine receptors CCR1/5 play an important role in the inflammatory cells recruitment in the lung of COPD patients.The aim of this study was to determine the impact of cigarette smoking on the expression of CCR1/5 on inflammatory cells in induced sputum,and the relationship between the receptors expression and COPD severity.Methods Differential cells in induced sputum were counted and the optical densities of CCR1 and CCR5 on inflammatory cells in induced sputum from COPD patients (n=29),healthy smokers (n=11),and nonsmokers (n=6) were measured using immunocytochemistry.Concentrations of CCL3,the ligand of CCR1/5,in supernatant of induced sputum were detected by enzyme-linked immunosorbent assay.Results The expressions of CCR1 and CCR5 on inflammatory cells in healthy smokers were significantly higher than those in nonsmokers,and the expression of CCR1 in patients with COPD was significantly increased when compared with nonsmokers but not healthy smokers.The expressions of CCR1 and CCR5 on inflammatory cells in severe and very severe COPD patients were higher compared with mild and moderate COPD patients.CCL3 level was positively correlated with the total cell counts in induced sputum and smoking history,and negatively correlated with percentage of predicted FEV1.Conclusions Cigarette smoking could increase the expression of CCR1 on the inflammatory cells.Both CCR1 and CCR5 expressions on the inflammatory cells in induced sputum could be associated with COPD severity.

  19. Association between Serum Neopterin and Inflammatory Activation in Chronic Kidney Disease

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    Ashok Kumar Yadav

    2012-01-01

    Full Text Available Background. The serum levels of neopterin, a marker associated with cell-mediated immunity are elevated in chronic kidney disease (CKD. We evaluated serum neopterin levels and investigated its association with markers of inflammation in a cross-section of CKD subjects without known cardiovascular disease. Methods. Serum neopterin levels were measured in 118 patients with stage 3–5 CKD and 41 healthy subjects with normal kidney function (HC. Patients with known cardiovascular disease were excluded. We also estimated highly sensitive CRP (hsCRP and interluekin-6 (IL-6, tumor necrosis factor-α (TNF-α and interferon-γ (IFN-γ in the CKD subjects. All assays were done using commercially available ELISA kits. The correlation between neopterin and markers of inflammation were investigated. Results. Of the CKD population, 82 were in stage 5 (60 stage 5 D, 24 in stage 4, and 12 in stage 3. The mean age was 51.04±1.3 years and 66% were males. The commonest cause of CKD was diabetes (36%. Serum neopterin levels were 5-fold higher in CKD patients as compared to HC (74.8±3.6 versus 15.0±2.8 nmol/L, P<0.0001. There was a graded increase of serum neopterin from stages 3 to 4 and 5. CKD 5 D patients exhibited significantly higher levels compared to nondialysis stage 5 patients (P<0.0001. An inverse correlation was noted between serum neopterin and eGFR (r=−0.359, P<0.0001. Serum neopterin correlated with hsCRP (r=0.285, P=0.002, IL-6 (r=0.212, P=0.034, and IFN-γ (r=0.32, P=0.001 but not with TNF-α. Conclusion. Serum neopterin level is elevated and correlates with the severity of CKD. The elevation correlates with elevation of most, but not all, inflammatory markers. Its role in future development of cardiovascular disease and modulation with anti-inflammatory therapies needs further studies.

  20. Association of terpinolene and diclofenac presents antinociceptive and anti-inflammatory synergistic effects in a model of chronic inflammation

    Directory of Open Access Journals (Sweden)

    E.M.A. Macedo

    2016-01-01

    Full Text Available Pharmacological treatment of inflammatory pain is usually done by administration of non-steroidal anti-inflammatory drugs (NSAIDs. These drugs present high efficacy, although side effects are common, especially gastrointestinal lesions. One of the pharmacological strategies to minimize such effects is the combination of drugs and natural products with synergistic analgesic effect. The monoterpene terpinolene (TPL is a chemical constituent of essential oils present in many plant species, which have pharmacological activities, such as analgesic and anti-inflammatory. The association of ineffective doses of TPL and diclofenac (DCF (3.125 and 1.25 mg/kg po, respectively presented antinociceptive and anti-inflammatory effects in the acute (0, 1, 2, 3, 4, 5 and 6 h, after treatment and chronic (10 days inflammatory hyperalgesia induced by Freund's complete adjuvant (CFA in the right hind paw of female Wistar rats (170-230 g, n=6-8. The mechanical hyperalgesia was assessed by the Randall Selitto paw pressure test, which determines the paw withdrawal thresholds. The development of edema was quantified by measuring the volume of the hind paw by plethismography. The TPL/DCF association reduced neutrophils, macrophages and lymphocytes in the histological analysis of the paw, following a standard staining protocol with hematoxylin and eosin and the counts were performed with the aid of optical microscopy after chronic oral administration of these drugs. Moreover, the TPL/DCF association did not induce macroscopic gastric lesions. A possible mechanism of action of the analgesic effect is the involvement of 5-HT2A serotonin receptors, because ketanserin completely reversed the antinociceptive effect of the TPL/DCF association. These results suggest that the TPL/DCF association had a synergistic anti-inflammatory and analgesic effect without causing apparent gastric injury, and that the serotonergic system may be involved in the antinociceptive effect of this

  1. Therapeutic Approach to the Management of Pediatric Demyelinating Disease: Multiple Sclerosis and Acute Disseminated Encephalomyelitis.

    Science.gov (United States)

    Brenton, J Nicholas; Banwell, Brenda L

    2016-01-01

    Acquired pediatric demyelinating diseases manifest acutely with optic neuritis, transverse myelitis, acute disseminated encephalomyelitis, or with various other acute deficits in focal or polyfocal areas of the central nervous system. Patients may experience a monophasic illness (as in the case of acute disseminated encephalomyelitis) or one that may manifest as a chronic, relapsing disease [e.g., multiple sclerosis (MS)]. The diagnosis of pediatric MS and other demyelinating disorders of childhood has been facilitated by consensus statements regarding diagnostic definitions. Treatment of pediatric MS has been modeled after data obtained from clinical trials in adult-onset MS. There are now an increasing number of new therapeutic agents for MS, and many will be formally studied for use in pediatric patients. There are important efficacy and safety concerns regarding the use of these therapies in children and young adults. This review will discuss acute management as well as chronic immunotherapies in acquired pediatric demyelination. PMID:26496907

  2. Zinc status and its relation to growth retardation in children with chronic inflammatory bowel disease.

    Science.gov (United States)

    Nishi, Y; Lifshitz, F; Bayne, M A; Daum, F; Silverberg, M; Aiges, H

    1980-12-01

    Zinc status was studied in 30 patients with chronic inflammatory bowel disease (CIBD) as well as in 17 normal children, 13 primordial short stature, and 17 anorexia nervosa patients. Basal serum and urinary excretion levels of zinc were measured in all patients. In addition, a zinc loading test was performed in 16 CIBD patients, 21 normal and/or short stature children, and nine patients with anorexia nervosa. Eleven of 30 patients with CIBD had serum zinc values less than 0.7 microgram/ml, whereas none of the other patients had hypozincemia. In addition, the mean urinary zinc excretion of CIBD patients was significantly lower than that of patients with primordial short stature and with anorexia nervosa. An altered response to oral zinc load was the most frequent abnormality in CIBD patients. Those with moderate and severe clinical disease activity had a decreased serum rise of zinc after the oral load of this ion. Urinary excretion of zinc after oral load was also marked by deficiency in all CIBD patients. The abnormalities of zinc metabolism were more frequent among the CIBD patients with growth abnormalities, although they were also found in patients who had normal growth. Among the 14 patients with CIBD and growth abnormalities, seven were hypozincemic and four hypozincuric. Hypozincemia was only found in four patients who had normal height; however, the growth velocity was not known. The zinc tolerance test revealed abnormalities in four of five CIBD patients with short stature and in two of three patients with slow growth. On the other hand, similar alterations in zinc tolerance tests were seen in three of seven CIBD patients with normal height and growth. PMID:7435430

  3. Chronic prehepatic portal hypertension in the rat: is it a type of Metabolic Inflammatory Syndrome?

    Directory of Open Access Journals (Sweden)

    García Cruz

    2008-02-01

    Full Text Available Abstract Background A progressive development of hepatic steatosis with an increase in the lipid hepatocyte content and the formation of megamitochondria have been demonstrated in rats with prehepatic portal hypertension. The aim of this study is to verify the existence of liver and serum lipid metabolism impairments in rats with long-term (2 years portal hypertension. Methods Male Wistar rats: Control (n = 10 and with prehepatic portal hypertension by triple partial portal vein ligation (n = 9 were used. Liver content of Triglycerides (TG, phospholipids (PL and cholesterol and serum cholesterol, lipoproteins (HDL and LDL, TG, glucose and Lipid Binding Protein (LBP were assayed with specific colorimetric commercial kits. Serum levels of insulin and somatostatin were assayed by RIA. Results The liver content of TG (6.30 ± 1.95 vs. 4.17 ± 0.59 μg/ml; p vs. 1.10 ± 0.13 μg/ml; p vs. 114.78 ± 37.72 mg/dl, TG (153.41 ± 80.39 vs. 324.39 ± 134.9 mg/dl; p vs. 55.15 ± 17.47 mg/dl; p vs. 1.59 +0.37 mg/dl decreased, whereas LDL (37.83 ± 15.39 vs. 16.77 ± 6.81 mg/dl; p vs. 60.27 ± 42.96 ng/ml; p Conclusion Portal hypertension in the rat presents changes in the lipid and carbohydrate metabolisms similar to those produced in chronic inflammatory conditions and sepsis in humans. These underlying alterations could be involved in the development of hepatic steatosis and, therefore, in those described in the metabolic syndrome in humans.

  4. Effects of intraventricular methotrexate administration on Cuprizone-induced demyelination in mice

    OpenAIRE

    Andre Michael Mueller; Adam Nassery; Hana Conlon; Esther Jun

    2013-01-01

    We previously showed that intrathecal administration of methotrexate slowed disability progression in multiple sclerosis (MS) patients with progressive disease. In general MS patients with progressive disease respond poorly to anti-inflammatory therapies. In order to better understand the mechanism by which methotrexate is protective in progressive MS, we analyzed its impact on the non-inflammatory cuprizone-induced demyelination model. When low-dose methotrexate was administered intracerebro...

  5. The synovial prostaglandin system in chronic inflammatory arthritis: differential effects of steroidal and nonsteroidal anti-inflammatory drugs

    Science.gov (United States)

    Bombardieri, S.; Cattani, P.; Ciabattoni, G.; Di Munno, O.; Pasero, G.; Patrono, C.; Pinca, E.; Pugliese, F.

    1981-01-01

    1 The present study was undertaken to characterize the spectrum of arachidonic acid metabolites present in synovial effusions of patients with rheumatoid or psoriatic arthritis, and to compare changes in their concentration following a short-term treatment with 6α-methyl-prednisolone (6-MeP: 4-8 mg/day) or indoprofen (1.2 g/day), a nonsteroidal anti-inflammatory agent with proven synovial prostaglandin inhibitory effect. 2 Measurements of prostaglandin E2 (PGE2), thromboxane (TX) B2, 6-keto-PGF1α and PGF2α were performed by radioimmunoassay techniques in synovial effusions obtained from 23 patients, and validated by thin-layer chromatographic analysis of the extracted immunoreactivity. 3 PGE2 and TXB2 accounted for more than 60% of the total immunoreactivity in untreated patients. The absence of any constant ratio between the different arachidonic acid metabolites detected in synovial fluid is consistent with a heterogeneous cellular origin of these compounds. 4 Indoprofen treatment was associated with a consistent reduction of synovial prostaglandin and thromboxane concentrations, ranging from 36% in the case of 6-keto-PGF1α to 90% in the case of PGE2. 5 In contrast, 6-MeP caused opposite changes on different metabolites originating via the cyclo-oxygenase pathway. Thus, 6-keto-PGF1α concentrations were reduced by 35%, PGF2α concentrations were increased by 30%, while PGE2 and TXB2 were unchanged following 6-MeP. 6 Although the mechanism(s) underlying the failure of 6-MeP to reduce synovial PGE2 and TXB2 levels are uncertain, the results of the present study clearly indicate that therapeutic doses of steroidal and nonsteroidal anti-inflammatory drugs cause quite distinct changes in arachidonic acid metabolism, which might be relevant to their specific therapeutic actions and side-effects. PMID:6895043

  6. Chronic Inflammatory Periodontal Disease in Patients with Human Immunodeficiency Virus. Enfermedad periodontal inflamatoria crónica en pacientes infectados con el virus de inmunodeficiencia humana.

    OpenAIRE

    Iralys Benítez Guzmán; Vicente Fardales Macías; Emilio Carpio Muñoz; Vania López Rodríguez

    2009-01-01

    Background: The Chronic Inflammatory Periodontal Disease is related with multiple risk factors. Those patients with human immunodeficiency virus have higher risk of presenting this disease and it is usually more serious in these cases. Objective: To describe the prevalence of Chronic Inflammatory Periodontal Disease in patients with HIV. Methods: Descriptive, observational, cross-sectional study including p...

  7. The Role of Hereditary Risk Factors in Forming Low Amylolytic Activity of Saliva in Children with Chronic Inflammatory Gastric and Duodenal Diseases

    Directory of Open Access Journals (Sweden)

    Shkenyova L.N.

    2011-03-01

    Full Text Available Low activity of saliva α-amylase has been stated to be registered in every second child with chronic inflammatory gastric and duodenal diseases at the age of 3—7 years, more frequently against the background of alimentary allergy. The risk factors of forming low activity of α-amylase saliva have been revealed. The data obtained allows recommending a saliva amylolytic activity test as a noninvasive screening of chronic inflammatory gastric and duodenal diseases in preschool children.

  8. Comparative evaluation of clinical, hematological and systemic inflammatory markers in smokers and non-smokers with chronic periodontitis

    Directory of Open Access Journals (Sweden)

    Vinayak Kanakdande

    2015-01-01

    Full Text Available Context: Systemic conditions, especially chronic infections, have a direct impact on the general health and well-being of an individual. Similarly, the long-standing inflammatory changes seen during periodontitis have been associated with the altered diabetic control, preterm, low birth weight infants, and cardiovascular disease. Being a low-grade infection, the signs may not be as severe as seen in other systemic conditions, but they definitely cannot be ignored. Aims: The present study was designed to compare clinical, hematological, and systemic inflammatory markers in patients with chronic periodontitis. Subjects and Methods: A total of 90 chronic periodontitis patients were selected for the present study from the outpatient department of the Department of Periodontology, and the various clinical and hematological parameters were then assessed. Statistical Analysis Used: Z-test was used to compare the probing depth, clinical attachment loss, hematological parameter, and interleukin-6 values between Group A and Group B. Mann-Whitney U-test was used to compare gingival index, plaque index, and bleeding on probing between Group A and Group B. Results: The results of the study were based on the comparison of the clinical, hematological, and systemic inflammatory markers in smokers and nonsmokers with chronic periodontitis and came out to be statistically highly significant. Conclusions: With the resurgence of emphasis on significance of oral diseases related to systemic health, the medical professionals also need to familiarize themselves with the oral cavity and the oral-systemic inter-relationships to treat or reduce the morbidity of the underlying medical condition. Furthermore, the oral health care professionals must reach out to the medical community and the general public to improve patient care through education and communication about the oral health-systemic health link.

  9. Biologics beyond TNF-α inhibitors and the effect of targeting the homologues TL1A-DR3 pathway in chronic inflammatory disorders

    DEFF Research Database (Denmark)

    Tougaard, Peter; Zervides, Kristoffer Alexander; Skov, Søren;

    2016-01-01

    A number of anti-tumor necrosis factor alpha (TNF-α) biologics have been developed in recent years, such as adalimumab, etanercept, and infliximab for the treatment of chronic inflammatory disorders like rheumatoid arthritis (RA), inflammatory bowel disease (IBD), and psoriasis and several other...... not respond to anti-TNF-α treatment and one possible explanation may be the heterogeneity of chronic inflammatory diseases and a dominance of other significant TNF family members. Indeed, polymorphisms in the TNF family member, TL1A gene, is associated with the development of IBD and increased serum...

  10. Chronic aspiration of gastric and duodenal contents and their effects on inflammatory cytokine production in respiratory system of rats.

    Directory of Open Access Journals (Sweden)

    Mitra Samareh Fekri

    2014-02-01

    Full Text Available Gastroesophageal reflux disease (GERD is defined with clinical symptoms of heart burning and regurgitation. It may be associated with external esophageal symptoms such as chronic cough, asthma, laryngitis, chronic lung disease, sinusitis and pulmonary fibrosis. In the present study, rats with chronic aspiration of gastroduodenal contents were studied for cellular phenotypes and cytokine concentrations in bronchoalveolar lavage and lung tissue. Thirty-six male Albino N-MRI rats were randomly divided into six groups. After anesthesia and tracheal intubation, the animals received either 0.5ml/kg of normal saline (control, gastric juice, pepsin, hydrochloric acid or bile salts by injection into their lungs twice a week for 8 weeks. In sham group nothing was injected. Thereafter, cellular phenotypes and cytokine concentrations of Interleukine (IL-1α, IL-1β, Transforming Growth Factor (TGF-β, Tumor Necrosis Factor (TNF-α, and IL-6 were assessed in bronchoalveolar lavage and lung tissue homogenates. The numbers of epithelial cells, macrophages, neutrophils and lymphocytes in BAL and levels of cytokines IL-1α, IL-6, TNF-α and TGF-β in BAL and lung tissue of test groups were significantly higher than the control group. Aspiration of bile salts caused more cytokine levels and inflammatory cells compared to other reflux components. It can be concluded that GERD with increased cytokines and inflammatory cells in lung could cause or exacerbate asthma and pulmonary fibrosis.

  11. Differential Features between Chronic Skin Inflammatory Diseases Revealed in Skin-Humanized Psoriasis and Atopic Dermatitis Mouse Models.

    Science.gov (United States)

    Carretero, Marta; Guerrero-Aspizua, Sara; Illera, Nuria; Galvez, Victoria; Navarro, Manuel; García-García, Francisco; Dopazo, Joaquin; Jorcano, Jose Luis; Larcher, Fernando; del Rio, Marcela

    2016-01-01

    Psoriasis and atopic dermatitis are chronic and relapsing inflammatory diseases of the skin affecting a large number of patients worldwide. Psoriasis is characterized by a T helper type 1 and/or T helper type 17 immunological response, whereas acute atopic dermatitis lesions exhibit T helper type 2-dominant inflammation. Current single gene and signaling pathways-based models of inflammatory skin diseases are incomplete. Previous work allowed us to model psoriasis in skin-humanized mice through proper combinations of inflammatory cell components and disruption of barrier function. Herein, we describe and characterize an animal model for atopic dermatitis using similar bioengineered-based approaches, by intradermal injection of human T helper type 2 lymphocytes in regenerated human skin after partial removal of stratum corneum. In this work, we have extensively compared this model with the previous and an improved version of the psoriasis model, in which T helper type 1 and/or T helper type 17 lymphocytes replace exogenous cytokines. Comparative expression analyses revealed marked differences in specific epidermal proliferation and differentiation markers and immune-related molecules, including antimicrobial peptides. Likewise, the composition of the dermal inflammatory infiltrate presented important differences. The availability of accurate and reliable animal models for these diseases will contribute to the understanding of the pathogenesis and provide valuable tools for drug development and testing. PMID:26763433

  12. [VASCULAR ENDOTHELIAL GROWTH FACTOR AND SOME INDICATORS OF ENDOTHELIAL DYSFUNCTION OF PATIENTS HAVING CHRONIC INFLAMMATORY DISEASES OF THE GASTRO DUODENAL ZONE].

    Science.gov (United States)

    Zavyalova, O V; Spivakovskiy, Yu M; Tchernenkov, Yu V; Lukina, O A

    2015-01-01

    The aim of the study was to determine the content of vaskuloendotelian growth factor and nitric oxide in children with chronic inflammatory diseases of the stomach and duodenum. The study involved 63 children with chronic inflammatory diseases of the gastroduodenal zone. Substrate study was serum. The data obtained were compared with a group of healthy children. The highest possible content vaskuloendotelian growth factor noted in the group of children with duodenal ulcer in the acute phase and in the group of chronic gastroduodenita associated with Helicobacter pylori. According to the results of the study established the role of nitric oxide and vaskuloendotelian growth factor in the pathogenesis of gastroduodenal diseases.

  13. [VASCULAR ENDOTHELIAL GROWTH FACTOR AND SOME INDICATORS OF ENDOTHELIAL DYSFUNCTION OF PATIENTS HAVING CHRONIC INFLAMMATORY DISEASES OF THE GASTRO DUODENAL ZONE].

    Science.gov (United States)

    Zavyalova, O V; Spivakovskiy, Yu M; Tchernenkov, Yu V; Lukina, O A

    2015-01-01

    The aim of the study was to determine the content of vaskuloendotelian growth factor and nitric oxide in children with chronic inflammatory diseases of the stomach and duodenum. The study involved 63 children with chronic inflammatory diseases of the gastroduodenal zone. Substrate study was serum. The data obtained were compared with a group of healthy children. The highest possible content vaskuloendotelian growth factor noted in the group of children with duodenal ulcer in the acute phase and in the group of chronic gastroduodenita associated with Helicobacter pylori. According to the results of the study established the role of nitric oxide and vaskuloendotelian growth factor in the pathogenesis of gastroduodenal diseases. PMID:26415264

  14. Adipose Tissue Is a Neglected Viral Reservoir and an Inflammatory Site during Chronic HIV and SIV Infection.

    Directory of Open Access Journals (Sweden)

    Abderaouf Damouche

    2015-09-01

    Full Text Available Two of the crucial aspects of human immunodeficiency virus (HIV infection are (i viral persistence in reservoirs (precluding viral eradication and (ii chronic inflammation (directly associated with all-cause morbidities in antiretroviral therapy (ART-controlled HIV-infected patients. The objective of the present study was to assess the potential involvement of adipose tissue in these two aspects. Adipose tissue is composed of adipocytes and the stromal vascular fraction (SVF; the latter comprises immune cells such as CD4+ T cells and macrophages (both of which are important target cells for HIV. The inflammatory potential of adipose tissue has been extensively described in the context of obesity. During HIV infection, the inflammatory profile of adipose tissue has been revealed by the occurrence of lipodystrophies (primarily related to ART. Data on the impact of HIV on the SVF (especially in individuals not receiving ART are scarce. We first analyzed the impact of simian immunodeficiency virus (SIV infection on abdominal subcutaneous and visceral adipose tissues in SIVmac251 infected macaques and found that both adipocytes and adipose tissue immune cells were affected. The adipocyte density was elevated, and adipose tissue immune cells presented enhanced immune activation and/or inflammatory profiles. We detected cell-associated SIV DNA and RNA in the SVF and in sorted CD4+ T cells and macrophages from adipose tissue. We demonstrated that SVF cells (including CD4+ T cells are infected in ART-controlled HIV-infected patients. Importantly, the production of HIV RNA was detected by in situ hybridization, and after the in vitro reactivation of sorted CD4+ T cells from adipose tissue. We thus identified adipose tissue as a crucial cofactor in both viral persistence and chronic immune activation/inflammation during HIV infection. These observations open up new therapeutic strategies for limiting the size of the viral reservoir and decreasing low

  15. Inflammatory and Remodeling Events in Asthma with Chronic Exposure to House Dust Mites: A Murine Model

    OpenAIRE

    Ahn, Joong Hyun; Kim, Chi Hong; Kim, Yong Hyun; Kim, Seung Joon; Lee, Sook-Young; Kim, Young Kyoon; Kim, Kwan Hyoung; Moon, Hwa Sik; Song, Jeong Sup; Park, Sung Hak; Kwon, Soon Seog

    2007-01-01

    Although animal models with ovalbumin have been used to study chronic asthma, there are difficulties in inducing recurrence as well as in maintaining chronic inflammation in this system. Using a murine model of house dust mite (HDM)-induced bronchial asthma, we examined the airway remodeling process in response to the chronic exposure to HDM. During the seventh and twelfth weeks of study, HDM were inhaled through the nose for three consecutive days and airway responsiveness was measured. Twen...

  16. Investigating the Burden of Chronic Pain: An Inflammatory and Metabolic Composite

    Directory of Open Access Journals (Sweden)

    Kimberly T. Sibille

    2016-01-01

    Full Text Available Background. Chronic pain is associated with increased morbidity and mortality, predominated by cardiovascular disease and cancer. Investigating related risk factor measures may elucidate the biological burden of chronic pain. Objectives. We hypothesized that chronic pain severity would be positively associated with the risk factor composite. Methods. Data from 12,982 participants in the 6th Tromsø study were analyzed. Questionnaires included demographics, health behaviors, medical comorbidities, and chronic pain symptoms. The risk factor composite was comprised of body mass index, fibrinogen, C-reactive protein, and triglycerides. Chronic pain severity was characterized by frequency, intensity, time/duration, and total number of pain sites. Results. Individuals with chronic pain had a greater risk factor composite than individuals without chronic pain controlling for covariates and after excluding inflammation-related health conditions (p<0.001. A significant “dose-response” relationship was demonstrated with pain severity (p<0.001. In individuals with chronic pain, the risk factor composite varied by health behavior, exercise, lower levels and smoking, and higher levels. Discussion. The risk factor composite was higher in individuals with chronic pain, greater with increasing pain severity, and influenced by health behaviors. Conclusions. Identification of a biological composite sensitive to pain severity and adaptive/maladaptive behaviors would have significant clinical and research utility.

  17. Investigating the Burden of Chronic Pain: An Inflammatory and Metabolic Composite

    Science.gov (United States)

    Sibille, Kimberly T.; Steingrímsdóttir, Ólöf A.; Fillingim, Roger B.; Stubhaug, Audun; Schirmer, Henrik; Chen, Huaihou; McEwen, Bruce S.; Nielsen, Christopher S.

    2016-01-01

    Background. Chronic pain is associated with increased morbidity and mortality, predominated by cardiovascular disease and cancer. Investigating related risk factor measures may elucidate the biological burden of chronic pain. Objectives. We hypothesized that chronic pain severity would be positively associated with the risk factor composite. Methods. Data from 12,982 participants in the 6th Tromsø study were analyzed. Questionnaires included demographics, health behaviors, medical comorbidities, and chronic pain symptoms. The risk factor composite was comprised of body mass index, fibrinogen, C-reactive protein, and triglycerides. Chronic pain severity was characterized by frequency, intensity, time/duration, and total number of pain sites. Results. Individuals with chronic pain had a greater risk factor composite than individuals without chronic pain controlling for covariates and after excluding inflammation-related health conditions (p < 0.001). A significant “dose-response” relationship was demonstrated with pain severity (p < 0.001). In individuals with chronic pain, the risk factor composite varied by health behavior, exercise, lower levels and smoking, and higher levels. Discussion. The risk factor composite was higher in individuals with chronic pain, greater with increasing pain severity, and influenced by health behaviors. Conclusions. Identification of a biological composite sensitive to pain severity and adaptive/maladaptive behaviors would have significant clinical and research utility. PMID:27445627

  18. Investigating the Burden of Chronic Pain: An Inflammatory and Metabolic Composite.

    Science.gov (United States)

    Sibille, Kimberly T; Steingrímsdóttir, Ólöf A; Fillingim, Roger B; Stubhaug, Audun; Schirmer, Henrik; Chen, Huaihou; McEwen, Bruce S; Nielsen, Christopher S

    2016-01-01

    Background. Chronic pain is associated with increased morbidity and mortality, predominated by cardiovascular disease and cancer. Investigating related risk factor measures may elucidate the biological burden of chronic pain. Objectives. We hypothesized that chronic pain severity would be positively associated with the risk factor composite. Methods. Data from 12,982 participants in the 6th Tromsø study were analyzed. Questionnaires included demographics, health behaviors, medical comorbidities, and chronic pain symptoms. The risk factor composite was comprised of body mass index, fibrinogen, C-reactive protein, and triglycerides. Chronic pain severity was characterized by frequency, intensity, time/duration, and total number of pain sites. Results. Individuals with chronic pain had a greater risk factor composite than individuals without chronic pain controlling for covariates and after excluding inflammation-related health conditions (p < 0.001). A significant "dose-response" relationship was demonstrated with pain severity (p < 0.001). In individuals with chronic pain, the risk factor composite varied by health behavior, exercise, lower levels and smoking, and higher levels. Discussion. The risk factor composite was higher in individuals with chronic pain, greater with increasing pain severity, and influenced by health behaviors. Conclusions. Identification of a biological composite sensitive to pain severity and adaptive/maladaptive behaviors would have significant clinical and research utility. PMID:27445627

  19. A Mechanism of Virus-Induced Demyelination

    Directory of Open Access Journals (Sweden)

    Jayasri Das Sarma

    2010-01-01

    Full Text Available Myelin forms an insulating sheath surrounding axons in the central and peripheral nervous systems and is essential for rapid propagation of neuronal action potentials. Demyelination is an acquired disorder in which normally formed myelin degenerates, exposing axons to the extracellular environment. The result is dysfunction of normal neuron-to-neuron communication and in many cases, varying degrees of axonal degeneration. Numerous central nervous system demyelinating disorders exist, including multiple sclerosis. Although demyelination is the major manifestation of most of the demyelinating diseases, recent studies have clearly documented concomitant axonal loss to varying degrees resulting in long-term disability. Axonal injury may occur secondary to myelin damage (outside-in model or myelin damage may occur secondary to axonal injury (inside-out model. Viral induced demyelination models, has provided unique imminent into the cellular mechanisms of myelin destruction. They illustrate mechanisms of viral persistence, including latent infections, virus reactivation and viral-induced tissue damage. These studies have also provided excellent paradigms to study the interactions between the immune system and the central nervous system (CNS. In this review we will discuss potential cellular and molecular mechanism of central nervous system axonal loss and demyelination in a viral induced mouse model of multiple sclerosis.

  20. [Phenotypes of Charcot-Marie-Tooth Syndrome and Differential Diagnosis Focused in Inflammatory Neuropathies].

    Science.gov (United States)

    Iijima, Masahiro

    2016-01-01

    Charcot-Marie-Tooth disease (CMT), the most frequent form of inherited neuropathy, is a genetically heterogeneous syndrome of the peripheral nervous system with a rather homologous clinical phenotype (slowly progressive distal weakness and muscle atrophy, skeletal deformities, and areflexia in each limb). CMT1 is the autosomal-dominant demyelinating form, and CMT1A (mostly PMP22 duplication) is the most frequent subtype, followed by CMTX1, HNPP (hereditary neuropathy with liability to pressure palsies), CMT1B, or CMT2. As CMT is characterized by slowly progressive motor and sensory disturbances in each limb, it could be misdiagnosed as chronic inflammatory demyelinating polyneuropathy (CIDP) occasionally. Some points can distinguish demyelinating CMT from CIDP. CMT1 patients do not show the conduction block that is frequent in CIDP. In addition, ultrasonographic findings are useful because CMT1 suggests diffuse enlargement of peripheral nerves, whereas CIDP is characterized by asymmetrical or focal enlargement of peripheral nerves. Some CMT1 cases show favorable responses to immunomodulating therapeutics such as corticosteroids, IVIg, and plasma exchange. Such CIDP-like CMT1 (especially CMT1B or CMT2A) shows moderate to high levels of cerebrospinal fluid protein and infiltrated inflammatory macrophages. PMID:26764297

  1. Angiostatin overexpression is associated with an improvement in chronic kidney injury by an anti-inflammatory mechanism.

    Science.gov (United States)

    Mu, Wei; Long, David A; Ouyang, Xiaosen; Agarwal, Anupam; Cruz, Pedro E; Roncal, Carlos A; Nakagawa, Takahiko; Yu, Xueqing; Hauswirth, William W; Johnson, Richard J

    2009-01-01

    Angiostatin, a proteolytic fragment of plasminogen, is a potent anti-angiogenic factor recently shown also to have an inhibitory effect on leukocyte recruitment and macrophage migration. Because both angiogenesis and inflammation play key roles in the progression of chronic kidney disease, we evaluated the effect of angiostatin treatment in the rat remnant kidney model. Rats were pretreated for 4 wk with recombinant adeno-associated viruses expressing either angiostatin or green fluorescence protein. Chronic renal disease was then induced by a subtotal nephrectomy, and rats were killed 8 wk later for analysis. Angiostatin treatment was associated with significantly less proteinuria but no alterations in serum creatinine, creatinine clearance, and blood urea nitrogen levels. Treatment with angiostatin reduced renal peritubular capillary number and decreased urinary nitric oxide levels. Despite reducing capillary density, angiostatin diminished interstitial fibrosis in association with reduced macrophage and T-cell infiltration and renal monocyte chemoattractant protein-1 mRNA levels. In conclusion, angiostatin overexpression was associated with attenuated renal disease progression in a model of chronic kidney injury, likely because of its anti-inflammatory actions. However, its anti-angiogenic actions suggest countering effects that could partially offset its benefit in chronic kidney diseases. PMID:18971211

  2. Progression of benign prostatic hyperplasia is associated with pro-inflammatory mediators and chronic activation of prostate-infiltrating lymphocytes.

    Science.gov (United States)

    Norström, Melissa M; Rådestad, Emelie; Sundberg, Berit; Mattsson, Jonas; Henningsohn, Lars; Levitsky, Victor; Uhlin, Michael

    2016-04-26

    Benign prostatic hyperplasia (BPH) is a common chronic non-malignant condition whose prevalence substantially increases with age. Immune cell infiltration and pro-inflammatory mediators have been implicated in the pathogenesis. Here, we characterized 21 extracellular markers on prostate-infiltrating lymphocytes (PILs) and analyzed expression of 26 soluble proteins in prostate tissue obtained from BPH patients (n = 31). These data were correlated with clinical parameters and compared with peripheral blood mononuclear cells (PBMCs) (n = 10). Increased frequencies of T cells expressing co-inhibitory receptors LAG-3, PD-1, TIM-3 or CTLA-4, and co-stimulatory receptors CD28, OX40 or 4-1BB were observed in BPH tissue compared to PBMCs. These findings are consistent with chronic activation and possible functional exhaustion of PILs that may be further augmented by several identified pro-inflammatory factors, such as IL-8 and MCP-1, promoting inflammation and chemotaxis of immune cells to the prostate. Prostate size and plasma prostate-specific antigen levels positively correlated with IL-8 and MCP-1 concentrations, and frequencies of T cells expressing CTLA-4 and TIM-3. It remains to be established whether the link between inflammation and BPH progression supported by our findings reflects a progressive failure of the immune system leading to decreased immune surveillance and development of prostate cancer. PMID:26993768

  3. Is There a Regulatory Role of Immunoglobulins on Tissue Forming Cells Relevant in Chronic Inflammatory Lung Diseases?

    Directory of Open Access Journals (Sweden)

    Michael Roth

    2011-01-01

    Full Text Available Epithelial cells, fibroblasts and smooth muscle cells together form and give structure to the airway wall. These three tissue forming cell types are structure giving elements and participate in the immune response to inhaled particles including allergens and dust. All three cell types actively contribute to the pathogenesis of chronic inflammatory lung diseases such as asthma and chronic obstructive pulmonary disease (COPD. Tissue forming cells respond directly to allergens through activated immunoglobulins which then bind to their corresponding cell surface receptors. It was only recently reported that allergens and particles traffic through epithelial cells without modification and bind to the immunoglobulin receptors on the surface of sub-epithelial mesenchymal cells. In consequence, these cells secrete pro-inflammatory cytokines, thereby extending the local inflammation. Furthermore, activation of the immunoglobulin receptors can induce proliferation and tissue remodeling of the tissue forming cells. New studies using anti-IgE antibody therapy indicate that the inhibition of immunoglobulins reduces the response of tissue forming cells. The unmeasured questions are: (i why do tissue forming cells express immunoglobulin receptors and (ii do tissue forming cells process immunoglobulin receptor bound particles? The focus of this review is to provide an overview of the expression and function of various immunoglobulin receptors.

  4. Deletion of macrophage migration inhibitory factor inhibits murine oral carcinogenesis: Potential role for chronic pro-inflammatory immune mediators.

    Science.gov (United States)

    Oghumu, Steve; Knobloch, Thomas J; Terrazas, Cesar; Varikuti, Sanjay; Ahn-Jarvis, Jennifer; Bollinger, Claire E; Iwenofu, Hans; Weghorst, Christopher M; Satoskar, Abhay R

    2016-09-15

    Oral cancer kills about 1 person every hour each day in the United States and is the sixth most prevalent cancer worldwide. The pro-inflammatory cytokine 'macrophage migration inhibitory factor' (MIF) has been shown to be expressed in oral cancer patients, yet its precise role in oral carcinogenesis is not clear. In this study, we examined the impact of global Mif deletion on the cellular and molecular process occurring during oral carcinogenesis using a well-established mouse model of oral cancer with the carcinogen 4-nitroquinoline-1-oxide (4NQO). C57BL/6 Wild-type (WT) and Mif knock-out mice were administered with 4NQO in drinking water for 16 weeks, then regular drinking water for 8 weeks. Mif knock-out mice displayed fewer oral tumor incidence and multiplicity, accompanied by a significant reduction in the expression of pro-inflammatory cytokines Il-1β, Tnf-α, chemokines Cxcl1, Cxcl6 and Ccl3 and other molecular biomarkers of oral carcinogenesis Mmp1 and Ptgs2. Further, systemic accumulation of myeloid-derived tumor promoting immune cells was inhibited in Mif knock-out mice. Our results demonstrate that genetic Mif deletion reduces the incidence and severity of oral carcinogenesis, by inhibiting the expression of chronic pro-inflammatory immune mediators. Thus, targeting MIF is a promising strategy for the prevention or therapy of oral cancer. PMID:27164411

  5. Peripheral blood gene expression patterns discriminate among chronic inflammatory diseases and healthy controls and identify novel targets

    Directory of Open Access Journals (Sweden)

    Palatka Karoly

    2010-05-01

    Full Text Available Abstract Background Chronic inflammatory diseases including inflammatory bowel disease (IBD; Crohn's disease and ulcerative colitis, psoriasis and rheumatoid arthritis (RA afflict millions of people worldwide, but their pathogenesis is still not well understood. It is also not well known if distinct changes in gene expression characterize these diseases and if these patterns can discriminate between diseased and control patients and/or stratify the disease. The main focus of our work was the identification of novel markers that overlap among the 3 diseases or discriminate them from each other. Methods Diseased (n = 13, n = 15 and n = 12 in IBD, psoriasis and RA respectively and healthy patients (n = 18 were recruited based on strict inclusion and exclusion criteria; peripheral blood samples were collected by clinicians (30 ml in Venous Blood Vacuum Collection Tubes containing EDTA and peripheral blood mononuclear cells were separated by Ficoll gradient centrifugation. RNA was extracted using Trizol reagent. Gene expression data was obtained using TaqMan Low Density Array (TLDA containing 96 genes that were selected by an algorithm and the statistical analyses were performed in Prism by using non-parametric Mann-Whitney U test (P-values Results Here we show that using a panel of 96 disease associated genes and measuring mRNA expression levels in peripheral blood derived mononuclear cells; we could identify disease-specific gene panels that separate each disease from healthy controls. In addition, a panel of five genes such as ADM, AQP9, CXCL2, IL10 and NAMPT discriminates between all samples from patients with chronic inflammation and healthy controls. We also found genes that stratify the diseases and separate different subtypes or different states of prognosis in each condition. Conclusions These findings and the identification of five universal markers of chronic inflammation suggest that these diseases have a common background in

  6. Serial High-Resolution Computed Tomography Imaging in Patients with Wegener Granulomatosis: Differentiation Between Active Inflammatory and Chronic Fibrotic Lesions

    Energy Technology Data Exchange (ETDEWEB)

    Lohrmann, C.; Uhl, M.; Schaefer, O.; Ghanem, N.; Kotter, E.; Langer, M. [Univ. Hospital of Freiburg (Germany). Dept. of Radiology

    2005-08-01

    PURPOSE: To evaluate pulmonary pathologies in Wegener granulomatosis with sequential computed tomography (CT) in order to differentiate active inflammatory lesions from chronic fibrotic lesions. MATERIAL AND METHODS: Serial CT findings in 38 patients with Wegener granulomatosis were retrospectively analyzed (mean follow-up period, 21 months). The presence, extension, and distribution of the following findings were evaluated with CT: parenchymal nodules, masses, ground-glass attenuation, airspace consolidation, bronchial wall-thickening, bronchiectasis, linear areas of attenuation, pleural irregularities, pleural effusions, hilar and mediastinal lymphadenopathy. RESULTS: Observed in 92% of patients, nodules were the most common CT pathology. Areas of ground-glass attenuation, consolidation, masses of linear attenuation, and tracheal/bronchial wall-thickening were detected in 24%, 26%, 32%, 39%, and 68% of patients. At follow-up, the clearance of lesions was most consistent for areas of ground-glass attenuation (89%), masses (87%), and cavitated nodules (85%). In the follow-up scan, 58% of all nodules, 47% of pulmonary consolidations, and 66% of bronchial wall-thickening were completely resolved. Areas of bronchiectasis and septal/non-septal lines remained stable in 70% and 71% of patients. CONCLUSION: The majority of the lesions decreased or resolved completely with or without areas of linear attenuation. Ground-glass attenuation, cavitated nodules and masses appear to represent active inflammatory lesions. In most probability, areas of bronchiectasis and septal/non-septal lines more often represent chronic fibrotic changes rather than active inflammatory changes. In combination with clinical evaluation and bronchoscopy, CT assists in the assessment of disease activity.

  7. Serial High-Resolution Computed Tomography Imaging in Patients with Wegener Granulomatosis: Differentiation Between Active Inflammatory and Chronic Fibrotic Lesions

    International Nuclear Information System (INIS)

    PURPOSE: To evaluate pulmonary pathologies in Wegener granulomatosis with sequential computed tomography (CT) in order to differentiate active inflammatory lesions from chronic fibrotic lesions. MATERIAL AND METHODS: Serial CT findings in 38 patients with Wegener granulomatosis were retrospectively analyzed (mean follow-up period, 21 months). The presence, extension, and distribution of the following findings were evaluated with CT: parenchymal nodules, masses, ground-glass attenuation, airspace consolidation, bronchial wall-thickening, bronchiectasis, linear areas of attenuation, pleural irregularities, pleural effusions, hilar and mediastinal lymphadenopathy. RESULTS: Observed in 92% of patients, nodules were the most common CT pathology. Areas of ground-glass attenuation, consolidation, masses of linear attenuation, and tracheal/bronchial wall-thickening were detected in 24%, 26%, 32%, 39%, and 68% of patients. At follow-up, the clearance of lesions was most consistent for areas of ground-glass attenuation (89%), masses (87%), and cavitated nodules (85%). In the follow-up scan, 58% of all nodules, 47% of pulmonary consolidations, and 66% of bronchial wall-thickening were completely resolved. Areas of bronchiectasis and septal/non-septal lines remained stable in 70% and 71% of patients. CONCLUSION: The majority of the lesions decreased or resolved completely with or without areas of linear attenuation. Ground-glass attenuation, cavitated nodules and masses appear to represent active inflammatory lesions. In most probability, areas of bronchiectasis and septal/non-septal lines more often represent chronic fibrotic changes rather than active inflammatory changes. In combination with clinical evaluation and bronchoscopy, CT assists in the assessment of disease activity

  8. Comparative effects of dexamethasone and bergenin on chronic bronchitis and their anti-inflammatory mechanisms based on NMR metabolomics.

    Science.gov (United States)

    Ren, Xiaolei; Ma, Shuangshuang; Wang, Juan; Tian, Simin; Fu, Xiaorui; Liu, Xinfeng; Li, Zhongfeng; Zhao, Baosheng; Wang, Xueyong

    2016-05-24

    In order to compare the effect of dexamethasone and bergenin on chronic bronchitis and to reveal their anti-inflammatory mechanisms, (1)H NMR-based metabolomics was performed to explore the potential biomarkers of the disease and study the therapeutic mechanisms of the drugs. In this study, 40 Sprague-Dawley male rats were randomly divided into 4 groups, namely control, model, dexamethasone and bergenin groups, with 10 rats in each group. Except for the control group, rats from the other three groups were exposed to tobacco smoke for 1 h d(-1) for 28 days. During the modeling, dexamethasone (0.2 mg kg(-1)) and bergenin (87 mg kg(-1)) were administered orally to dexamethasone or bergenin rats 3 h after exposure every day. On the other hand, control and model rats were intragastrically administered water. According to the results of morphometric analysis of the airway epithelium and the count of white blood cells in the bronchoalveolar lavage fluid (BALF), dexamethasone and bergenin could suppress the infiltration of inflammatory cells, inhibit the secretion of mucus, and reduce white blood cells in BALF. Serum samples from the rats' orbits were collected every week. The metabolic profiles of sera were analyzed by multivariate statistical analyses, including PCA, PLS-DA and OPLS-DA models, and 18 metabolites were identified. The dynamic fluctuations of these biomarkers in sera from different groups were detected. The results suggested that the anti-inflammatory mechanism of dexamethasone may be associated with BCAA metabolism and glycolysis while bergenin could change BCAA metabolism, glycine, serine and threonine metabolism, and glycolysis to treat chronic bronchitis. PMID:27098339

  9. Chronic exposure to exogenous glucocorticoids primes microglia to pro-inflammatory stimuli and induces NLRP3 mRNA in the hippocampus.

    Science.gov (United States)

    Frank, Matthew G; Hershman, Sarah A; Weber, Michael D; Watkins, Linda R; Maier, Steven F

    2014-02-01

    Chronic stress as well as chronic treatment with glucocorticoids (GCs) primes the neuroinflammatory response to a subsequent pro-inflammatory challenge. However, it remains unclear whether chronic GCs sensitize the response of key CNS immune substrates (i.e. microglia) to pro-inflammatory stimuli. In the present set of studies, male Sprague-Dawley rats underwent sham surgery or were adrenalectomized and then treated with varying concentrations of corticosterone (CORT; 0, 25, 50, and 75 μg/ml) administered in their drinking water. After 10 days of CORT exposure, whole hippocampus was collected and expression of glial activation markers measured or hippocampal microglia were isolated and challenged with LPS to probe for CORT-induced sensitization of pro-inflammatory responses. Chronic CORT exposure increased the gene expression of NLRP3, Iba-1, MHCII, and NF-κBIα in a concentration dependent manner. Chronic CORT (75 μg/ml) exposure potentiated the microglial proinflammatory response (TNFα, IL-1β, IL-6 and NLRP3) to LPS compared to the microglial response of sham surgery animals treated with vehicle. The present set of results demonstrate that chronic exposure to GCs primes microglia to pro-inflammatory stimuli and add to a growing body of evidence suggesting that a permissive function of GCs is that of an endogenous danger signal or alarmin.

  10. The Telomere/Telomerase System in Chronic Inflammatory Diseases. Cause or Effect?

    Science.gov (United States)

    Kordinas, Vasileios; Ioannidis, Anastasios; Chatzipanagiotou, Stylianos

    2016-01-01

    Telomeres are specialized nucleoprotein structures located at the end of linear chromosomes and telomerase is the enzyme responsible for telomere elongation. Telomerase activity is a key component of many cancer cells responsible for rapid cell division but it has also been found by many laboratories around the world that telomere/telomerase biology is dysfunctional in many other chronic conditions as well. These conditions are characterized by chronic inflammation, a situation mostly overlooked by physicians regarding patient treatment. Among others, these conditions include diabetes, renal failure, chronic obstructive pulmonary disease, etc. Since researchers have in many cases identified the association between telomerase and inflammation but there are still many missing links regarding this correlation, the latest findings about this phenomenon will be discussed by reviewing the literature. Our focus will be describing telomere/telomerase status in chronic diseases under the prism of inflammation, reporting molecular findings where available and proposing possible future approaches. PMID:27598205

  11. Fibrin depletion decreases inflammation and delays the onset of demyelination in a tumor necrosis factor transgenic mouse model for multiple sclerosis

    OpenAIRE

    Akassoglou, K.; Adams, R. A.; Bauer, J.; Mercado, P; Tseveleki, V; Lassmann, H.; Probert, L.; Strickland, S

    2004-01-01

    In multiple sclerosis, in which brain tissue becomes permeable to blood proteins, extravascular fibrin deposition correlates with sites of inflammatory demyelination and axonal damage. To examine the role of fibrin in neuroinflammatory demyelination, we depleted fibrin in two tumor necrosis factor transgenic mouse models of multiple sclerosis, transgenic lines TgK21 and Tg6074. In a genetic analysis, we crossed TgK21 mice into a fibrin-deficient background. TgK21fib(-/-) mice had decreased in...

  12. Risk factors and prediction for functional and chronic inflammatory bowel diseases in infants

    Directory of Open Access Journals (Sweden)

    Marushko RV

    2014-03-01

    Full Text Available Objective — to determine mainly significant risk factors and prediction for development of functional bowel disease and chronic non-ulcerative non-specific colitis in infants. Materials and methods. Retrospective studies were conducted using the method of questioning parents and analysis of medical records of 344 infants in the age from 6 months to 3 years of life, including 134 infants with functional constipation (FC , 64 infants with functional diarrhea (FD and 146 children with chronic non-specific not-ulcerative colitis (CNNC. The control group included 50 healthy children of similar age and gender. In groups of children retrospectively determined the frequency of risk factors. To compare the data between different groups was used analysis of Pearson c2 criteria and relative risks (relative risk, RR with 95% confidence intervals. Prediction for the development of FC, FD and CNNC was applied the method of discriminant function analysis based on the analysis of 51 essential marks. Results. Mathematical analysis of risk factors for bowel diseases allowed to identify the most important of them, in particular, family history of diseases of the digestive system, including bowel disease, complications during pregnancy and at birth disorders, women chronic extragenital diseases, chronic diseases of women characterized by prolonged exposure in the body to opportunistic or pathogenic bacteria, nutritional and feeding disorders, high infection index and history of infectious diseases. Revealed by the discriminant function analysis significant risk factors, represent their important role in the development of functional bowel disorders and chronic non-specific non-ulcerative colitis. Conclusions. Identifying the risk factors for the development and application of prediction algorithm for functional bowel diseases and chronic non-specific non-ulcerative colitis is enable to develop the effective treatment and preventive measures to reduce the

  13. Protracted, relapsing and demyelinating experimental autoimmune encephalomyelitis in DA rats immunized with syngeneic spinal cord and incomplete Freund's adjuvant

    DEFF Research Database (Denmark)

    Lorentzen, J C; Issazadeh-Navikas, Shohreh; Storch, M;

    1995-01-01

    , protracted and relapsing EAE (SPR-EAE) after a subcutaneous immunization at the tail base with syngeneic spinal cord and incomplete Freund's adjuvant (IFA). The neurological deficits were accompanied by demyelinating inflammatory lesions in the spinal cord, with infiltrating T lymphocytes and perivascular...

  14. Inflammatory and metabolic alterations of Kager's fat pad in chronic achilles tendinopathy

    OpenAIRE

    Jessica Pingel; M Christine H Petersen; Ulrich Fredberg; Søren G Kjær; Bjørn Quistorff; Henning Langberg; Hansen, Jacob B.

    2015-01-01

    BACKGROUND: Achilles tendinopathy is a painful inflammatory condition characterized by swelling, stiffness and reduced function of the Achilles tendon. Kager's fat pad is an adipose tissue located in the area anterior to the Achilles tendon. Observations reveal a close physical interplay between Kager's fat pad and its surrounding structures during movement of the ankle, suggesting that Kager's fat pad may stabilize and protect the mechanical function of the ankle joint.AIM: The aim of this s...

  15. The Immune Protective Effect of the Mediterranean Diet against Chronic Low-grade Inflammatory Diseases

    OpenAIRE

    Casas, Rosa; Sacanella, Emilio; Estruch, Ramon

    2014-01-01

    Dietary patterns high in refined starches, sugar, and saturated and trans-fatty acids, poor in natural antioxidants and fiber from fruits, vegetables, and whole grains, and poor in omega-3 fatty acids may cause an activation of the innate immune system, most likely by excessive production of proinflammatory cytokines associated with a reduced production of anti-inflammatory cytokines. The Mediterranean Diet (MedDiet) is a nutritional model inspired by the traditional dietary pattern of some o...

  16. Risk of ulcerative colitis and Crohn's disease among offspring of patients with chronic inflammatory bowel disease

    DEFF Research Database (Denmark)

    Orholm, Marianne; Fonager, Kirsten; Sørensen, Henrik Toft

    1999-01-01

    OBJECTIVE: The incidence of inflammatory bowel disease (IBD) varies among and within countries, but several studies have indicated that genetic factors may play an important role in the etiology of IBD. A Danish regional study has observed an almost 10-fold increased risk for ulcerative colitis (UC...... significantly increased. CONCLUSION: The risk for UC and CD among offspring of patients with IBD is 2-13 times higher than the risk within the general population....

  17. Experimental Optic Neuritis Induced by a Demyelinating Strain of Mouse Hepatitis Virus▿

    OpenAIRE

    Shindler, Kenneth S.; Kenyon, Lawrence C; Dutt, Mahasweta; Hingley, Susan T.; Sarma, Jayasri Das

    2008-01-01

    Optic neuritis (ON), an inflammatory demyelinating optic nerve disease, occurs in multiple sclerosis (MS). Pathological mechanisms and potential treatments for ON have been studied via experimental autoimmune MS models. However, evidence suggests that virus-induced inflammation is a likely etiology triggering MS and ON; experimental virus-induced ON models are therefore required. We demonstrate that MHV-A59, a mouse hepatitis virus (MHV) strain that causes brain and spinal cord inflammation a...

  18. Chronic inflammatory disease, lymphoid tissue neogenesis and extranodal marginal zone B-cell lymphomas

    NARCIS (Netherlands)

    R.J. Bende; F. van Maldegem; C.J.M. van Noesel

    2009-01-01

    Chronic autoimmune or pathogen-induced immune reactions resulting in lymphoid neogenesis are associated with development of malignant lymphomas, mostly extranodal marginal zone B-cell lymphomas (MZBCLs). In this review we address (i) chemokines and adhesion molecules involved in lymphoid neogenesis;

  19. The role of inflammatory cytokines and ERK1/2 signaling in chronic prostatitis/chronic pelvic pain syndrome with related mental health disorders.

    Science.gov (United States)

    Hu, Chao; Yang, Hualan; Zhao, Yanfang; Chen, Xiang; Dong, Yinying; Li, Long; Dong, Yehao; Cui, Jiefeng; Zhu, Tongyu; Zheng, Ping; Lin, Ching-Shwun; Dai, Jican

    2016-01-01

    Mental health disorders(MHD) in chronic prostatitis/chronic pelvic pain syndrome (CP/CPPS) have been widely studied. However, the underlying role of inflammatory cytokines and their associated signaling pathways have not been investigated. Here, we report the potential role of cytokines and associated signaling pathways in CP/CPPS patients with MHD and in a CP/CPPS animal model. CP/CPPS patients (n = 810) and control subjects (n = 992) were enrolled in this case-control multicenter study, and serum cytokine levels were measured. Male Sprague-Dawley rats received multiple intracutaneous injections of an immuno-agent along with a pertussis-diphtheria-tetanus triple vaccine for autoimmune CP/CPPS development. The results revealed that, in CP/CPPS patients with significant MHD, elevated IL-1α, IL-1β, IL-4, IL-13, and TNF-α serum levels were observed. The above five cytokines in CP/CPPS rats were significantly elevated in prostate tissue (p MHD. PMID:27334333

  20. Neuro-oncology dilemma: Tumour or tumefactive demyelinating lesion.

    Science.gov (United States)

    Abdoli, Mohammad; Freedman, Mark S

    2015-11-01

    Tumefactive demyelinating lesions (TDLs) are not an uncommon manifestation of demyelinating disease but can pose diagnostic challenges in patients without a pre-existing diagnosis of multiple sclerosis (MS) as well as in known MS patients. Brain tumours can also arise in MS patients and can be seen in chronic MS patients as co-morbidities. Delayed diagnosis or unnecessary intervention or treatment will affect the ultimate prognosis of these patients. In this article, we will review some typical cases illustrating the dilemma and review the information that helps to differentiate the two conditions. The intention is not to present an extensive differential diagnosis of both entities, but to examine some typical examples when the decision arises to decide between the two. We take a somewhat different approach, by presenting the cases in "real time", allowing the readers to consider in their own minds which diagnosis they favour, discussing in detail some of the pertinent literature, then revealing later the actual diagnosis. We would urge readers to consider re-visiting their first thoughts about each case after reading the discussion, before reading the follow-up of each case. The overall objective is to highlight the real possibility of being forced to decide between these two entities in clinical practise, present a reasonable approach to help differentiate them and especially to focus on the possibility of TDLs in order to avoid unnecessary biopsy.

  1. The pro- and anti-inflammatory markers in patients with acute myocardial infarction and chronic stable angina.

    Science.gov (United States)

    Wojakowski, Wojciech; Maslankiewicz, Katarzyna; Ochala, Andrzej; Wyderka, Rafal; Zuk-Popiolek, Izabela; Flak, Zbigniew; Mroz, Iwona; Tendera, Michal

    2004-08-01

    The aim of this study was to assess the plasma levels of VEGF and interleukin-10 in patients with acute myocardial infarction (AMI) and stable chronic angina (SA) and correlate the values with traditional CHD risk factors, left ventricular ejection fraction (LVEF) and established inflammatory marker hsCRP. Fifty patients with AMI and 30 with SA were enrolled. IL-10 levels in AMI patients were lower than in SA patients (9.81 +/- 5.0 versus 22.63 +/- 8.38 pg/ml, p 40% and Killip class I-II (338.8 +/- 51.59 versus 271.8 +/- 50.51 pg/ml; p 6 h versus inflamatory markers and CHD risk factors and the function of the left ventricle on admission.

  2. Comparison of indium-111 scintigraphy and colonoscopy with histologic study in children for evaluation of colonic chronic inflammatory bowel disease

    Energy Technology Data Exchange (ETDEWEB)

    Tolia, V.; Kuhns, L.R.; Chang, C.H.; Slovis, T.L. (Department of Pediatric Gastroenterology, Children' s Hospital of Michigan, Detroit (USA))

    1991-04-01

    Indium-111 leukocyte scanning and colonoscopy were performed in 19 children and adolescents with chronic inflammatory bowel disease to study the correlation of evaluation between these two diagnostic modalities in comparison to histologic study for colonic disease. Seven patients had ulcerative colitis, 10 had Crohn's disease, and two patients had no specific diagnosis after evaluation. The sensitivity of indium-111 scan was 18%, specificity was 62.5%, and accuracy for diagnosing colonic disease was only 37%. In comparison, sensitivity and specificity for colonoscopy were 100 and 57%, respectively. Furthermore, accuracy with colonoscopy was 84%. The authors data suggest that the usefulness of scans is limited to patients in whom standard diagnostic procedures are contraindicated. In addition, it is essential to confirm the visual diagnostic impression on colonoscopy with histologic study.

  3. Inflammatory and metabolic alterations of Kager's fat pad in chronic achilles tendinopathy

    DEFF Research Database (Denmark)

    Pingel, Jessica; Petersen, Marie Christine Helby; Fredberg, Ulrich;

    2015-01-01

    BACKGROUND: Achilles tendinopathy is a painful inflammatory condition characterized by swelling, stiffness and reduced function of the Achilles tendon. Kager's fat pad is an adipose tissue located in the area anterior to the Achilles tendon. Observations reveal a close physical interplay between...... Kager's fat pad and its surrounding structures during movement of the ankle, suggesting that Kager's fat pad may stabilize and protect the mechanical function of the ankle joint. AIM: The aim of this study was to characterize whether Achilles tendinopathy was accompanied by changes in expression...

  4. 99th Dahlem conference on infection, inflammation and chronic inflammatory disorders: lifestyle changes affecting the host-environment interface.

    Science.gov (United States)

    Ehlers, S; Kaufmann, S H E

    2010-04-01

    In industrialized nations and high-income regions of the world, the decline of infectious diseases is paralleled by an increase in allergic, autoimmune and chronic inflammatory diseases (AACID). Changes in lifestyle in westernized societies, which impact individually and collectively on intestinal microbiota, may - at least in part - account for the AACID pandemic. Many disease genes that contribute to AACID encode pattern recognition and signalling molecules in barrier-associated cells. Interactions between gene products and environmental factors depend highly upon the host's state of maturation, the composition of the skin and gut microflora, and exposure to pollutants, antibiotics and nutrients. Inflammatory stress responses, if regulated appropriately, ensure immunity, health and relative longevity; when they are dysregulated, they can no longer be terminated appropriately and thus precipitate AACID. The 99th Dahlem Conference brought together experts of various disciplines (genetics, evolution biology, molecular biology, structural biology, cell biology, immunology, microbiology, nutrition science, epidemiology and clinical medicine) to discuss the multi-faceted relationships between infection, immunity and inflammation in barrier organs and the development of AACID. In Clinical and Experimental Immunology we are presenting a compilation of background papers that formed the basis of discussions. Controversial viewpoints and gaps in current knowledge were examined and new concepts for prevention and treatment of CID were formulated.

  5. Chronic unpredictable stress regulates visceral adipocyte‐mediated glucose metabolism and inflammatory circuits in male rats

    OpenAIRE

    Karagiannides, Iordanes; Golovatscka, Viktoriya; Bakirtzi, Kyriaki; Sideri, Aristea; Salas, Martha; Stavrakis, Dimitris; Polytarchou, Christos; Iliopoulos, Dimitrios; Pothoulakis, Charalabos; Bradesi, Sylvie

    2014-01-01

    Abstract Chronic psychological stress is a prominent risk factor involved in the pathogenesis of many complex diseases, including major depression, obesity, and type II diabetes. Visceral adipose tissue is a key endocrine organ involved in the regulation of insulin action and an important component in the development of insulin resistance. Here, we examined for the first time the changes on visceral adipose tissue physiology and on adipocyte‐associated insulin sensitivity and function after c...

  6. The Anti-Inflammatory Actions of Auricular Point Acupressure for Chronic Low Back Pain

    OpenAIRE

    Wei-Chun Lin; Chao Hsing Yeh; Lung-Chang Chien; Morone, Natalia E.; Glick, Ronald M.; Albers, Kathryn M.

    2015-01-01

    Background. Auricular point acupressure (APA) is a promising treatment for pain management. Few studies have investigated the physiological mechanisms of APA analgesics. Method. In this pilot randomized clinical trial (RCT), a 4-week APA treatment was used to manage chronic low back pain (CLBP). Sixty-one participants were randomized into a real APA group (n = 32) or a sham APA group (n = 29). Blood samples, pain intensity, and physical function were collected at baseline and after 4 weeks of...

  7. Cefditoren versus levofloxacin in patients with exacerbations of chronic bronchitis: serum inflammatory biomarkers, clinical efficacy, and microbiological eradication

    Directory of Open Access Journals (Sweden)

    Blasi F

    2013-02-01

    Full Text Available Francesco Blasi, Paolo Tarsia, Marco Mantero, Letizia C Morlacchi, Federico PifferDepartment of Pathophysiology and Transplantation, University of Milan, IRCCS Fondazione Cà Granda Ospedale Maggiore Policlinico, Milan, ItalyBackground: The aim of this open-label, randomized, parallel-group pilot study was to evaluate the efficacy of cefditoren pivoxil and levofloxacin in terms of speed of reduction in inflammatory parameters, clinical recovery, and microbiological eradication.Methods: Forty eligible patients with acute exacerbation of chronic bronchitis (AECB were randomized to receive cefditoren 200 mg twice a day for 5 days (n = 20 or levofloxacin 500 mg once daily for 7 days (n = 20.Results: The inflammatory parameters which were significantly reduced at test-of-cure with respect to visit 1 were Krebs von den Lundgen-6 (KL-6 and interleukin-6. KL-6 decreased both in the overall study population (from 19 ± 11 UI/mL to 6 ± 8 UI/mL, P = 0.000 and in the cefditoren (from 19 ± 13 UI/mL to 8 ± 10 UI/mL, P = 0.006 and levofloxacin (from 19 ± 10 UI/mL to 5 ± 5 UI/mL, P = 0.000 arms. Similarly, interleukin-6 decreased both in the overall study population (from 13.35 ± 16.41 pg/mL to 3 ± 4.7 pg/mL, P = 0.000 and in the cefditoren (from 15.90 ± 19.54 pg/mL to 4.13 ± 6.42 pg/mL, P = 0.015 and levofloxacin (from 10.80 ± 12.55 pg/mL to 1.87 ± 1.16 pg/mL, P = 0.003 arms. At the end of treatment (test-of-cure, 6–9 days after drug initiation, the clinical success rate in the overall study population was 78%; the clinical cure rate was 80% in the cefditoren arm and 75% in the levofloxacin arm. Globally, bacteriological eradication at test-of-cure was obtained in 85% of the overall study population. Both treatments were well tolerated.Conclusion: Cefditoren represents a valid option in the treatment of mild to moderately severe cases of AECB in the outpatient care setting. Moreover, the use of this cephalosporin is associated with a significant

  8. Anti-nociceptive and anti-inflammatory effects of cyanocobalamin (vitamin B12) against acute and chronic pain and inflammation in mice.

    Science.gov (United States)

    Hosseinzadeh, H; Moallem, S A; Moshiri, M; Sarnavazi, M S; Etemad, L

    2012-07-01

    In this study, the anti-nociceptive and anti-inflammatory effects of cyanocobalamin (Vit B12) against acute and chronic pain and inflammation were evaluated in mice. Vit B12 (0.87, 1 and 1.77 mg/kg) were injected intraperitoneally. The anti-nociceptive effects against acute pain were examined using hot-plate and writhing tests. The chronic pain was examined 14 days after sciatic nerve ligation using the hot-plate test. Morphine (10 mg/kg) was used as a positive control. Anti-inflammatory effects of Vit B12 against acute and chronic inflammation were assessed using xylene-induced edema in ears and granuloma caused by compressed cotton implantation, respectively. In these tests, sodium diclofenac (15 mg/kg) was used as a positive control. Vit B12 showed a dose related effect in acute anti-nociceptive test and increased the anti-nociceptive effect of morphine in chronic treatment. Vit B12 demonstrated an anti-nociceptive effect in chronic studies as single or continues daily treatment and increased significantly the anti-nociceptive effect of morphine. All doses of Vit B12 significantly decreased xylene-induced ear edema. Maximum anti-inflammatory effect (37.5%) was obtained at dose of 1 mg/kg. In chronic inflammation, Vit B12 significantly decreased granuloma formation in mice. In conclusion our work presents some experimental evidence supporting the administration of cyanocobalamin in controlling acute and chronic neuropathic pain. Cyanocobalamin may have anti-inflammatory effect. It may reduce tolerance to anti-nociceptive effect of morphine as well. PMID:22588629

  9. The Comparison of Efficacy of Tricyclic Antidepressant with and without Non Steroidal Anti Inflammatory Drugs in Chronic Low Back Pain

    Directory of Open Access Journals (Sweden)

    A.R. Yavarikia

    2007-07-01

    Full Text Available Introduction & Objectives: Low back pain (LBP is one of common medical problems with several accepted medical modalities such as drugs, physiotherapy, surgery, etc. We studied the efficacy of tricyclic antidepressant (TCA, and tricyclic antidepressant plus non steroidal anti inflammatory drugs (TCA + NSAID in 200 patients with chronic LBP. Materials & Methods: In an experimental clinical trial study on patients with chronic low back pain without organic findings, patients were divided in two groups of 100 cases. At certain times the response to treatment protocols were collected and compared using VAS system. Patient’s data including age, sex, smoking and response to treatment were recorded and analyzed using chi-square, t-tests, ANOVA and SPSS software. Results: 83 (41.5% of patients were males and 117 (58.5% were females. The age range was 21 to 75 (mean age 43.1 14.1y there was no meaning full statistical difference in demographic characteristics such as age, sex (respectively p=0.66, p=0.78 the ultimate pain was less (p0.05.Conclusion: TCA prescription is an efficient method of treatment of low back pain with or with out NSAIDS. But using NSAID+TCA will be almost more powerful and efficient method in the long term period.

  10. Environmental and genetic factors in pediatric inflammatory demyelinating diseases.

    Science.gov (United States)

    Waubant, Emmanuelle; Ponsonby, Anne-Louise; Pugliatti, Maura; Hanwell, Heather; Mowry, Ellen M; Hintzen, Rogier Q

    2016-08-30

    The onset of multiple sclerosis (MS) occurs in childhood in about 5% of all patients with MS. The disease in adults has a complex genetic and environmental inheritability. One of the main risk factors, also confirmed in pediatric MS, is HLA DRB1*1501 In addition to genetic factors, a large part of disease susceptibility in adults is conferred by environmental risk factors such as low vitamin D status, exposure to cigarette smoking, and remote Epstein-Barr virus (EBV) infection. In children, both exposure to cigarette smoking and prior EBV infection have been reported consistently as risk factors for MS. The role of vitamin D remains to be confirmed in this age category. Finally, although very likely critical in disease processes, few gene-environment interactions and epigenetic changes have been reported for adult and pediatric MS susceptibility. Of interest, some of the risk factors for MS have also been associated with disease course modification, such as low 25(OH) vitamin D serum levels in pediatric and adult MS. Age is also a clear disease modifier of clinical, CSF, and MRI phenotype in children with the disease. Finally, although much has yet to be unraveled regarding molecular processes at play in MS, there is a larger gap in our knowledge of genetic and environmental risk factors for pediatric neuromyelitis optica spectrum disorders and acute disseminated encephalomyelitis and only collaborative studies will answer those questions. PMID:27572857

  11. Demyelinating polyneuropathy in Leber hereditary optic neuropathy.

    NARCIS (Netherlands)

    Gilhuis, H.J.; Schelhaas, H.J.; Cruysberg, J.R.M.; Zwarts, M.J.

    2006-01-01

    We report a patient with Leber hereditary optic neuropathy (G11778A mtDNA) and a severe demyelinating neuropathy, for which no other cause except his mitochondrial disorder could be found. The involvement of the peripheral nervous system of patients with LHON, in particular with a 11778 mtDNA, is di

  12. Implications of chronic daily anti-oxidant administration on the inflammatory response to intracortical microelectrodes

    Science.gov (United States)

    Potter-Baker, Kelsey A.; Stewart, Wade G.; Tomaszewski, William H.; Wong, Chun T.; Meador, William D.; Ziats, Nicholas P.; Capadona, Jeffrey R.

    2015-08-01

    Objective. Oxidative stress events have been implicated to occur and facilitate multiple failure modes of intracortical microelectrodes. The goal of the present study was to evaluate the ability of a sustained concentration of an anti-oxidant and to reduce oxidative stress-mediated neurodegeneration for the application of intracortical microelectrodes. Approach. Non-functional microelectrodes were implanted into the cortex of male Sprague Dawley rats for up to sixteen weeks. Half of the animals received a daily intraperitoneal injection of the natural anti-oxidant resveratrol, at 30 mg kg-1. The study was designed to investigate the biodistribution of the resveratrol, and the effects on neuroinflammation/neuroprotection following device implantation. Main results. Daily maintenance of a sustained range of resveratrol throughout the implantation period resulted in fewer degenerating neurons in comparison to control animals at both two and sixteen weeks post implantation. Initial and chronic improvements in neuronal viability in resveratrol-dosed animals were correlated with significant reductions in local superoxide anion accumulation around the implanted device at two weeks after implantation. Controls, receiving only saline injections, were also found to have reduced amounts of accumulated superoxide anion locally and less neurodegeneration than controls at sixteen weeks post-implantation. Despite observed benefits, thread-like adhesions were found between the liver and diaphragm in resveratrol-dosed animals. Significance. Overall, our chronic daily anti-oxidant dosing scheme resulted in improvements in neuronal viability surrounding implanted microelectrodes, which could result in improved device performance. However, due to the discovery of thread-like adhesions, further work is still required to optimize a chronic anti-oxidant dosing regime for the application of intracortical microelectrodes.

  13. Genetic dysbiosis: the role of microbial insults in chronic inflammatory diseases

    Directory of Open Access Journals (Sweden)

    Luigi Nibali

    2014-02-01

    Full Text Available Thousands of bacterial phylotypes colonise the human body and the host response to this bacterial challenge greatly influences our state of health or disease. The concept of infectogenomics highlights the importance of host genetic factors in determining the composition of human microbial biofilms and the response to this microbial challenge. We hereby introduce the term ‘genetic dysbiosis’ to highlight the role of human genetic variants affecting microbial recognition and host response in creating an environment conducive to changes in the normal microbiota. Such changes can, in turn, predispose to, and influence, diseases such as: cancer, inflammatory bowel disease, rheumatoid arthritis, psoriasis, bacterial vaginosis and periodontitis. This review presents the state of the evidence on host genetic factors affecting dysbiosis and microbial misrecognition (i.e. an aberrant response to the normal microbiota and highlights the need for further research in this area.

  14. Chronic inflammatory diseases of the bowel: diagnosis and follow-up

    Energy Technology Data Exchange (ETDEWEB)

    Gorincour, Guillaume; Aschero, Audrey; Desvignes, Catherine; Bourliere-Najean, Brigitte; Ruocco-Angari, Alix; Devred, Philippe; Colavolpe, Nathalie; Petit, Philippe [Hopital Timone Enfant, Department of Pediatric Radiology, Marseille Cedex 5 (France); Portier, Francois [Hopital Ambroise Pare, 13291 Marseille Cedex 6 (France); Roquelaure, Bertrand [Hopital Timone Enfant, Department of Pediatric Gastroenterology, Marseille Cedex 5 (France); Delarue, Arnaud [Hopital Timone Enfant, Department of Pediatric Surgery, Marseille Cedex 5 (France)

    2010-06-15

    Approximately one fourth of cases of inflammatory bowel disease (IBD) occur during childhood and children are more prone than their adult counterparts to have severe disease at presentation. To investigate these diseases MR imaging is no longer an emerging tool. Numerous reviews and articles have been published on this topic underlying the advances of imaging but also the complexity and the financial impact on management of such diseases. In children it seems reasonable to consider US as the first imaging examination to perform, especially when the diagnosis of IBD is unknown. However, we believe that recent and future technical progress, especially the ability of MR to display reproducible data, and the need for gold standard evaluation of new medical therapies will increase the role of MR enterography. (orig.)

  15. Vesicular demyelination induced by raised intracellular calcium.

    Science.gov (United States)

    Smith, K J; Hall, S M; Schauf, C L

    1985-11-01

    Incubation of nerve with high concentrations of the divalent cation ionophore A23187 produces myelin vesiculation (Schlaepfer 1977). This observation has now been extended using segments of rat ventral or dorsal root incubated with high (19 microM, 10 micrograms/ml) or low (1-1.5 microM) concentrations of A23187, or another divalent ionophore, ionomycin. Low concentrations of A23187 induced no vesiculation within a 2-h period. However, subsequent incubation of these roots in fresh, ionophore-free medium for 20 h, resulted in a prominent vesicular demyelination at the Schmidt-Lanterman incisures and paranodes of many fibres. At this time (22 h) the Schwann cells associated with some demyelinating internodes appeared vital upon ultrastructural examination: the cells also excluded the nuclear dye nigrosin. High concentrations of A23187 induced a similar vesicular demyelination in affected fibres within only 15-20 min. While the Schwann cells continued to exclude nigrosin for a further 4 h, their ultrastructural appearance indicated that they were probably in the early stages of necrosis. Incubation of moribund root with the ionophore produced no myelin vesiculation. At all ionophore concentrations, the myelin vesiculation was dependent upon the presence of extracellular Ca2+, and could be modulated in severity by varying this concentration. Other divalent cations (Ba2+, Co2+, Mg2+, Mn2+, Ni2+, Sr2+) could not substitute for Ca2+. The vesiculation induced by A23187 could be entirely prevented by the addition of Zn2+ (greater than or equal to 1 microM), Ni2+ (greater than or equal to 1-10 microM), Co2+ (greater than or equal to 100 microM) or Mn2+ (greater than or equal to 100 microM) to the bathing medium. A23187 applied to only part of an isolated internode resulted in a localization of the myelin disruption to that region. Ionomycin (greater than or equal to 1 microM), an ionophore with a greater selectivity for Ca2+ than A23187, also induced a prompt Ca2+-dependent

  16. Chronic intestinal pseudo-obstruction as an expression of inflammatory enteric neuropathy

    Directory of Open Access Journals (Sweden)

    Rita Pimentel

    2014-11-01

    Full Text Available Chronic intestinal pseudo-obstruction (CIPO is characterised by inadequate digestive tract motility and can lead to severely disordered motility. CIPO manifests as recurrent episodes of intestinal sub-occlusion without an anatomical obstruction. We present the case of a 41-year-old female, with severe chronic constipation and several episodes of intestinal sub-occlusion. Investigation revealed colonic inertia and marked distension of the small bowel and colon with no evidence of stenosis or obstructive lesions, compatible with CIPO. After several treatments were tried (domperidone, erythromycin, cisapride, octreotide, total enteral nutrition, with partial or no response, further work-up was done trying to identify an etiology. Gastrointestinal manometry showed neuropathic type abnormalities, transmural biopsy of the jejunum revealed degenerative enteric neuropathy and anti-HU antineuronal antibody screen was positive, suggesting an autoimmune type neuropathy with diffuse involvement of the digestive tract. Corticosteroids showed partial improvement of short duration and azathioprine was also tried but discontinued due to intolerance. Marked dietary intolerance and malnutrition lead to total parenteral nutrition (TPN at home since October 2011. Since then, symptoms and nutritional status improved, with rare episodes of pseudo-obstruction, not requiring hospitalisation.

  17. Correlation of Chronic Pelvic Inflammatory Disease and Female Infertility%慢性盆腔炎与女性不孕不育的相关性探析

    Institute of Scientific and Technical Information of China (English)

    王家荣

    2015-01-01

    目的:探讨慢性盆腔炎与女性不孕不育的相关性。方法回顾性分析125例慢性盆腔炎患者的临床资料,包括患者的病程、受孕情况,并检测血清六项性激素水平。结果125例慢性盆腔炎患者中不孕发生率为26.4%。随着慢性盆腔炎病程的延长,不孕率逐渐升高(P<0.05)。与受孕组比较,卵泡期PRL和P升高,而FSH、E2、T降低,差异均有统计学意义(P<0.05)。结论慢性盆腔炎与女性不孕不育密切相关。%Objective To investigate the correlation between chronic pelvic inflammatory disease and female infertility. Methods To analysis the data of 125 cases of chronic pelvic inflammatory disease,including the course of disease,pregnancy,and detection of serum sex hormone levels of six. Results 125 cases of infertility and chronic pelvic inflammatory disease patients the incidence rate was 26.4%. Along with the extension of the course of chronic pelvic inflammatory disease,infertility rate increased gradual y(P<0.05).Compared with the control group,while PRL and P increased,while FSH,E2,T decreased,the differences were statistical y significant(P<0.05). Conclusion Chronic pelvic inflammatory disease is closely related with female infertility.

  18. CXCR4 Signaling Regulates Remyelination by Endogenous Oligodendrocyte Progenitor Cells in a Viral Model of Demyelination

    Science.gov (United States)

    CARBAJAL, KEVIN S.; MIRANDA, JUAN L.; TSUKAMOTO, MICHELLE R.; LANE, THOMAS E.

    2016-01-01

    Following intracranial infection with the neurotropic JHM strain of mouse hepatitis virus (JHMV), susceptible mice will develop widespread myelin destruction that results in pathological and clinical outcomes similar to those seen in humans with the demyelinating disease Multiple Sclerosis (MS). Partial remyelination and clinical recovery occurs during the chronic phase following control of viral replication yet the signaling mechanisms regulating these events remain enigmatic. Here we report the kinetics of proliferation and maturation of oligodendrocyte progenitor cells (OPCs) within the spinal cord following JHMV-induced demyelination and that CXCR4 signaling contributes to the maturation state of OPCs. Following treatment with AMD3100, a specific inhibitor of CXCR4, mice recovering from widespread demyelination exhibit a significant (P < 0.01) increase in the number of OPCs and fewer (P < 0.05) mature oligodendrocytes compared with HBSS-treated animals. These results suggest that CXCR4 signaling is required for OPCs to mature and contribute to remyelination in response to JHMV-induced demyelination. To assess if this effect is reversible and has potential therapeutic benefit, we pulsed mice with AMD3100 and then allowed them to recover. This treatment strategy resulted in increased numbers of mature oligodendrocytes, enhanced remyelination, and improved clinical outcome. These findings highlight the possibility to manipulate OPCs in order to increase the pool of remyelination-competent cells that can participate in recovery. PMID:21830237

  19. EFFECTS OF COMBINED PHYSIOTHERAPY IN CHRONIC ENDOMETRITIS: DYNAMICS OF INFLAMMATORY ACTIVITY

    Directory of Open Access Journals (Sweden)

    M. P. Рlyasunova

    2014-01-01

    Full Text Available We have performed immunohistochemical staining of lymphocyte subpopulations (CD16+, CD56+, CD20+, CD138+, and HLA-DR antigen (II class using monoclonal antibodies by “Novocastra” (United Kingdom. The cell were counted in uterine scrapings of patients with verified chronic endometritis (CE. The samples were taken before and after treatment. The endometritis treatment was carried out according to standard procedures. In a group of patients, rehabilitation treatment was made by means of CAPELM-01 “Andro-Gin”, whereas another group was treated by of hormone replacement therapy and combined physiotherapy with CAP-ELM-01 “Andro-Gin”. There was a significant reduction of cytotoxic lymphocytes after treatment applied.

  20. Escherichia coli in chronic inflammatory bowel diseases: An update on adherent invasive Escherichia coli pathogenicity

    Institute of Scientific and Technical Information of China (English)

    Margarita; Martinez-Medina; Librado; Jesus; Garcia-Gil

    2014-01-01

    Escherichia coli(E. coli), and particularly the adherent invasive E. coli(AIEC) pathotype, has been increasingly implicated in the ethiopathogenesis of Crohn’s disease(CD). E. coli strains with similar pathogenic features to AIEC have been associated with other intestinal disorders such as ulcerative colitis, colorectal cancer, and coeliac disease, but AIEC prevalence in these diseases remains largely unexplored. Since AIEC was described one decade ago, substantial progress has been made in deciphering its mechanisms of pathogenicity. However, the molecular bases that characterize the phenotypic properties of this pathotype are still not well resolved. A review of studies focused on E. coli populations in inflammatory bowel disease(IBD) is presented here and we discuss about the putative role of this species on each IBD subtype. Given the relevance of AIEC in CD pathogenesis, we present the latest research findings concerning AIEC host-microbe interactions and pathogenicity. We also review the existing data regarding the prevalence and abundance of AIEC in CD and its association with other intestinal diseases from humans and animals, in order to discuss the AIEC disease- and hostspecificity. Finally, we highlight the fact that dietarycomponents frequently found in industrialized countries may enhance AIEC colonization in the gut, which merits further investigation and the implementation of preventative measures.

  1. Gut microbiome diversity in acute infective and chronic inflammatory gastrointestinal diseases in North India.

    Science.gov (United States)

    Kedia, Saurabh; Rampal, Ritika; Paul, Jaishree; Ahuja, Vineet

    2016-07-01

    The disease profile in the Indian population provides a unique opportunity for studying the host microbiome interaction in both infectious (amebiasis) and autoimmune diseases like inflammatory bowel disease (IBD) from a similar environment and genetic background. Analysis of fecal samples from untreated amebic liver abscess (ALA) patients, Entamoeba histolytica (Eh)-negative and -positive asymptomatic individuals, and pus samples from naive ALA patients revealed a significant reduction in Lactobacillus in asymptomatic individuals (Eh +ve) and ALA patients. Two anaerobic genera, namely Bacteroides and Peptostreptococcus, were detected in naive ALA pus samples. Analysis of fecal samples from amoebic colitis patients showed a significant decline in population of Bacteroides, Clostridium coccoides and leptum subgroup, Lactobacillus, Campylobacter, and Eubacterium, whereas a significant increase in Bifidobacterium was observed. Mucosa-associated bacterial flora analysis from IBD patients and healthy controls revealed a significant difference in concentration of bacteria among predominating and subdominating genera between ulcerative colitis (UC), Crohn's disease (CD) patients, and controls. In contrast to the mucosal studies, we found a significant increase in lactobacilli population in fecal samples of active UC patients. Another study revealed a significant decrease of Clostridium coccoides and leptum clusters in fecal samples of active UC patients along with decreased concentrations of fecal SCFAs, especially of n-butyrate, iso-butyrate, and acetate. We therefore found similar perturbations in gut microbiome in both infectious and autoimmune diseases, indicating inflammation to be the major driver for changes in gut microbiome. PMID:26994772

  2. Galanin transgenic mice with elevated circulating galanin levels alleviate demyelination in a cuprizone-induced MS mouse model.

    Directory of Open Access Journals (Sweden)

    Lin Zhang

    Full Text Available Multiple Sclerosis (MS is a demyelinating autoimmune disease of the central nervous system (CNS with a presumed autoimmune etiology. Approved treatments for MS are immunoregulatory and are able to reduce the inflammatory components of the disease. However, these treatments do not suppress progressive clinical disability. Approaches that directly protect myelin-producing oligodendrocytes and enhance remyelination are likely to improve long-term outcomes and reduce the rate of axonal damage. Galanin (GAL is a bioactive neuropeptide that is widely distributed throughout the nervous system and has diverse neuromodulatory effects. In this study, using the cuprizone (CPZ demyelination model of MS, we demonstrate that GAL has pronounced neuroprotective effects with respect to demyelination and remyelination. Using our GAL transgenic mouse (GAL-Tg, we identified a novel attenuation of OLs against CPZ induced demyelination, which was exerted independently of progenitor cells. Alleviation of myelin breakdown in the GAL-Tg mice was observed to be significant. Furthermore, we observed changes in the expression of the GAL receptor GalR1 during the demyelination and remyelination processes. Our data strongly indicate that GAL has the capacity to influence the outcome of primary insults that directly target OLs, as opposed to cases where immune activation is the primary pathogenic event. Taken together, these results suggest that GAL is a promising next-generation target for the treatment of MS.

  3. Inhibitory Effect of Natural Anti-Inflammatory Compounds on Cytokines Released by Chronic Venous Disease Patient-Derived Endothelial Cells

    Directory of Open Access Journals (Sweden)

    Veronica Tisato

    2013-01-01

    Full Text Available Large vein endothelium plays important roles in clinical diseases such as chronic venous disease (CVD and thrombosis; thus to characterize CVD vein endothelial cells (VEC has a strategic role in identifying specific therapeutic targets. On these bases we evaluated the effect of the natural anti-inflammatory compounds α-Lipoic acid and Ginkgoselect phytosome on cytokines/chemokines released by CVD patient-derived VEC. For this purpose, we characterized the levels of a panel of cytokines/chemokines (n=31 in CVD patients’ plasma compared to healthy controls and their release by VEC purified from the same patients, in unstimulated and TNF-α stimulated conditions. Among the cytokines/chemokines released by VEC, which recapitulated the systemic profile (IL-8, TNF-α, GM-CSF, INF-α2, G-CSF, MIP-1β, VEGF, EGF, Eotaxin, MCP-1, CXCL10, PDGF, and RANTES, we identified those targeted by ex vivo treatment with α-Lipoic acid and/or Ginkgoselect phytosome (GM-CSF, G-CSF, CXCL10, PDGF, and RANTES. Finally, by investigating the intracellular pathways involved in promoting the VEC release of cytokines/chemokines, which are targeted by natural anti-inflammatory compounds, we documented that α-Lipoic acid significantly counteracted TNF-α-induced NF-κB and p38/MAPK activation while the effects of Ginkgo biloba appeared to be predominantly mediated by Akt. Our data provide new insights into the molecular mechanisms of CVD pathogenesis, highlighting new potential therapeutic targets.

  4. Antinociceptive effects of the selective CB2 agonist MT178 in inflammatory and chronic rodent pain models.

    Science.gov (United States)

    Vincenzi, Fabrizio; Targa, Martina; Corciulo, Carmen; Tabrizi, Mojgan Aghazadeh; Merighi, Stefania; Gessi, Stefania; Saponaro, Giulia; Baraldi, Pier Giovanni; Borea, Pier Andrea; Varani, Katia

    2013-06-01

    Cannabinoid CB(2) receptor activation by selective agonists has been shown to produce analgesic effects in preclinical models of inflammatory, neuropathic, and bone cancer pain. In this study the effect of a novel CB(2)agonist (MT178) was evaluated in different animal models of pain. First of all, in vitro competition binding experiments performed on rat, mouse, or human CB receptors revealed a high affinity, selectivity, and potency of MT178. The analgesic properties of the novel CB(2) agonist were evaluated in various in vivo experiments, such as writhing and formalin assays, showing a good efficacy comparable with that produced by the nonselective CB agonist WIN 55,212-2. A dose-dependent antiallodynic effect of the novel CB(2) compound in the streptozotocin-induced diabetic neuropathy was found. In a bone cancer pain model and in the acid-induced muscle pain model, MT178 was able to significantly reduce mechanical hyperalgesia in a dose-related manner. Notably, MT178 failed to provoke locomotor disturbance and catalepsy, which were observed following the administration of WIN 55,212-2. CB(2) receptor mechanism of action was investigated in dorsal root ganglia where MT178 mediated a reduction of [(3)H]-d-aspartate release. MT178 was also able to inhibit capsaicin-induced substance P release and NF-κB activation. These results demonstrate that systemic administration of MT178 produced a robust analgesia in different pain models via CB(2) receptors, providing an interesting approach to analgesic therapy in inflammatory and chronic pain without CB(1)-mediated central side effects.

  5. Fluoroquinolone-macrolide combination therapy for chronic bacterial prostatitis: retrospective analysis of pathogen eradication rates, inflammatory findings and sexual dysfunction

    Institute of Scientific and Technical Information of China (English)

    Vittorio Magri; Emanuele Montanari; Vi(s)nja (S)kerk; Alemka Markoti(c); Emanuela Marras; Antonella Restelli; Kurt G Naber; Gianpaolo Perletti

    2011-01-01

    We previously demonstrated the safety and efficacy of fluoroquinolone-macrolide combination therapy in category Ⅱ chronic bacterial prostatitis (CBP).The aim of this study is to retrospectively compare the microbiological and clinical findings of two treatment schemes for CBP based on the combination of azithromycin (500 mg,thrice-weekly) with a once-daily 500-or 750-mg dose of ciprofloxacin (Cipro-500 or Cipro-750 cohort,respectively).Combined administration of azithromycin (1500 mg week-1) with ciprofloxacin at the rate of 750 mg day-1 for 4 weeks rather than at 500 mg day-1 for 6 weeks increased the eradication rates from 62.35% to 77.32% and the total bacteriological success from 71.76% to 85.57%.A significant decrease in pain and voiding signs/symptoms and a significant reduction in inflammatory leukocyte counts and serum prostate-specific antigen (PSA) were sustained throughout an 18-month follow-up period in both groups.Ejaculatory pain,haemospermia and premature ejaculation were significantly attenuated on microbiological eradication in both groups,but the latter subsided more promptly in the Cipro-750 cohort.In total,59 Cipro-750 patients showed mild-to-severn erectile dysfunction (ED) at baseline,while 22 patients had no ED on microbiological eradication and throughout the follow-up period.In conclusion fluoroquinolone-macrolide therapy resulted in pathogen eradication and CBP symptom attenuation,including pain,voiding disturbances and sexual dysfunction.A once-daily 750-mg dose of ciprofloxacin for 4 weeks showed enhanced eradication rates and lower inflammatory white blood cell counts compared to the 500-mg dose for 6 weeks.Our results are open to further prospective validation.

  6. Inflammatory and Immune Response Genes Polymorphisms are Associated with Susceptibility to Chronic Obstructive Pulmonary Disease in Tatars Population from Russia.

    Science.gov (United States)

    Korytina, Gulnaz Faritovna; Akhmadishina, L Z; Kochetova, O V; Aznabaeva, Y G; Zagidullin, Sh Z; Victorova, T V

    2016-08-01

    Chronic obstructive pulmonary disease (COPD) is a complex chronic inflammatory disease of the respiratory system affecting primarily distal respiratory pathways and lung parenchyma. This work was designed as a case-control study aimed at investigating the association of COPD with polymorphisms in inflammatory and immune response genes (JAK1, JAK3, STAT1, STAT3, NFKB1, IL17A, ADIPOQ, ADIPOR1, etc.) in Tatar population from Russia. Ten SNPs (rs310216, rs3212780, rs12693591, rs2293152, rs28362491, rs4711998, rs1974226, rs1501299, rs266729, and rs12733285) were genotyped by the real-time polymerase chain reaction (TaqMan assays) in a case-control study (425 COPD patients and 457 in the control group, from Ufa, Russia). Logistic regression was used to detect the association of SNPs in different models. Linear regression analyses were performed to estimate the relationship between SNPs and lung function parameters and pack-years. In Tatar population, significant associations of JAK1 (rs310216) (P = 0.0002, OR 1.70 in additive model), JAK3 (rs3212780) (P = 0.001, OR 1.61 in dominant model), and IL17A (rs1974226) (P = 0.0037, OR 2.31 in recessive model) with COPD were revealed. The disease risk was higher in carriers of insertion allele of NFKB1 (rs28362491) (P = 0.045, OR 1.22). We found a significant gene-by-environment interaction of smoking status and IL17A (rs1974226) (P interact = 0.016), JAK3 (rs3212780) (P interact = 0.031), ADIPOQ (rs266729) (P interact = 0.013), and ADIPOR1 (rs12733285) (P interact = 0.018). The relationship between the rs4711998, rs1974226, rs310216, rs3212780, rs28362491, and smoking pack-years was found (P = 0.045, P = 0.004, P = 0.0005, P = 0.021, and P = 0.042). A significant genotype-dependent variation of forced vital capacity was observed for NFKB1 (rs28362491) (P = 0.017), ADIPOR1 (rs12733285) (P = 0.043), and STAT1 (rs12693591) (P = 0.048). The genotypes of STAT1 (rs12693591) (P = 0.013) and JAK1 (rs

  7. Acute and long-term effect of infliximab on humoral and echocardiographic parameters in patients with chronic inflammatory diseases.

    Science.gov (United States)

    Tomáš, L'ubomír; Lazúrová, Ivica; Pundová, Lýdia; Oetterová, Mária; Zakuciová, Mária; Petrášová, Darina; Studenčan, Martin

    2013-01-01

    Tumor necrosis factor alpha (TNF-alpha) plays an important role in the pathogenesis of chronic inflammatory diseases, i.e., rheumatoid arthritis (RA), ankylosing spondylitis (AS), Crohn's disease (CD), and ulcerative colitis (UC). Anti-TNF-alpha strategies are successfully used in their treatment. However, their effect on heart function is still uncertain. The objectives of the study were to examine the acute and long-term effect of infliximab on the heart morphology and function in patients with chronic inflammatory disorders. Thirty-one patients (21 men and 10 women) were included. Ten percent of them were diagnosed with RA, 22.5 % with AS, 22.5 % with CD, and 45 % with UC, respectively. N-terminal fragment of pro-brain natriuretic peptide (NT-proBNP) was measured before and immediately after infliximab administration at the beginning of the study and in the sixth and 12th months. Echocardiography was performed at baseline and in the sixth and 12th months. There was a significant increase in NT-proBNP after the first infliximab infusion (88.40 ± 14.09 vs. 95.24 ± 14.28 pg/ml, p = 0.0046) and similar response was detected after each infusion in the sixth and 12th months. Plasma NT-proBNP slightly but not significantly decreased (88.40 ± 14.09 vs. 81.74 ± 23.14 pg/ml, p = 0.583, and 88.40 ± 14.09 vs. 56.83 ± 17.77 pg/ml, p = 0.0576, in the sixth and 12th months, respectively). There were no significant changes in echocardiographic structural and functional parameters of the left ventricle during follow-up. Plasma NT-proBNP mildly but significantly increases immediately after infliximab infusion. However, long-term infliximab administration does not deteriorate both cardiac morphology and function. PMID:23010850

  8. A comparative study of anti-inflammatory activity of lovastatin, simvastatin, atorvastatin and rosuvastatin on acute and chronic inflammation in animal models

    Institute of Scientific and Technical Information of China (English)

    Santoshkumar R Jeevangi; S Manjunath; Sachidananda G Shetti; Chetan Manjunath; Prashant Dass

    2012-01-01

    Objective: To study the anti-inflammatory activity of Lovastatin, Simvastatin, Atorvastatin, and Rosuvastatin on acute and chronic models of inflammation, to compare with the effect of Diclofenac sodium and amongst themselves in rats. Methods: Carrageenin induce rat paw edema method in which 5 animals of each group (6 groups) received orally 4% gum acacia, Diclofenac and 4 statins respectively 1 h before Carrageenin injection in paw. The paw edema volume measured with plethysmograph after 3 h and percentage inhibition of edema in various groups calculated. Rexin pellet granuloma method in which 4 rexin pellets were implanted into dorsum of skin of each rat of 6 groups (n=5) including control, Diclofenac and 4 statin groups respectively. Rats were orally fed with drugs daily for 7 days and on 8th day rexin pellets were removed after sacrificing the rat and dried in incubator 60oC overnight. Pellets were then weighed and percentage inhibition of granulation tissue was calculated and sent for histopathological examination.Results:All the 4 statins showed significant anti-inflammatory activity in the present study in both acute as well as chronic models of inflammation. The anti-inflammatory activity of the 4 statins was significant on comparison with Diclofenac. Lovastatin and Simvastatin demonstrated 10-20% more anti-inflammatory activity than Atorvastatin and Rosuvastatin. Conclusions: The present study revealed the anti-inflammatory effect of statins and thus suggests that the statins have a potential anti-atherosclerotic activity along with its lipid lowering property.

  9. Polymorphism in the alpha cardiac muscle actin 1 gene is associated to susceptibility to chronic inflammatory cardiomyopathy.

    Directory of Open Access Journals (Sweden)

    Amanda Farage Frade

    Full Text Available AIMS: Chagas disease, caused by the protozoan Trypanosoma cruzi is endemic in Latin America, and may lead to a life-threatening inflammatory dilated, chronic Chagas cardiomyopathy (CCC. One third of T. cruzi-infected individuals progress to CCC while the others remain asymptomatic (ASY. A possible genetic component to disease progression was suggested by familial aggregation of cases and the association of markers of innate and adaptive immunity genes with CCC development. Since mutations in multiple sarcomeric genes, including alpha-cardiac actin (ACTC1 have been involved in hereditary dilated cardiomyopathy, we investigated the involvement of the ACTC1 gene in CCC pathogenesis. METHODS AND RESULTS: We conducted a proteomic and genetic study on a Brazilian study population. The genetic study was done on a main cohort including 118 seropositive asymptomatic subjects and 315 cases and the replication was done on 36 asymptomatic and 102 CCC cases. ACTC1 protein and mRNA levels were lower in myocardial tissue from patients with end-stage CCC than those found in hearts from organ donors. Genotyping a case-control cohort of CCC and ASY subjects for all informative single nucleotide polymorphism (SNP in the ACTC1 gene identified rs640249 SNP, located at the 5' region, as associated to CCC. Associations are borderline after correction for multiple testing. Correlation and haplotype analysis led to the identification of a susceptibility haplotype. Functional assays have shown that the rs640249A/C polymorphism affects the binding of transcriptional factors in the promoter regions of the ACTC1 gene. Confirmation of the detected association on a larger independent replication cohort will be useful. CONCLUSIONS: Genetic variations at the ACTC1 gene may contribute to progression to chronic Chagas Cardiomyopathy among T. cruzi-infected patients, possibly by modulating transcription factor binding to ACTC1 promoter regions.

  10. Selective Vitamin D Receptor Activation as Anti-Inflammatory Target in Chronic Kidney Disease

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    J. Donate-Correa

    2014-01-01

    Full Text Available Paricalcitol, a selective vitamin D receptor (VDR activator used for treatment of secondary hyperparathyroidism in chronic kidney disease (CKD, has been associated with survival advantages, suggesting that this drug, beyond its ability to suppress parathyroid hormone, may have additional beneficial actions. In this prospective, nonrandomised, open-label, proof-of-concept study, we evaluated the hypothesis that selective vitamin D receptor activation with paricalcitol is an effective target to modulate inflammation in CKD patients. Eight patients with an estimated glomerular filtration rate between 15 and 44 mL/min/1.73 m2 and an intact parathyroid hormone (PTH level higher than 110 pg/mL received oral paricalcitol (1 μg/48 hours as therapy for secondary hyperparathyroidism. Nine patients matched by age, sex, and stage of CKD, but a PTH level <110 pg/mL, were enrolled as a control group. Our results show that five months of paricalcitol administration were associated with a reduction in serum concentrations of hs-CRP (13.9%, P<0.01, TNF-α (11.9%, P=0.01, and IL-6 (7%, P<0.05, with a nonsignificant increase of IL-10 by 16%. In addition, mRNA expression levels of the TNFα and IL-6 genes in peripheral blood mononuclear cells decreased significantly by 30.8% (P=0.01 and 35.4% (P=0.01, respectively. In conclusion, selective VDR activation is an effective target to modulate inflammation in CKD.

  11. Anti-Inflammatory and Antinociceptive Effects of Salbutamol on Acute and Chronic Models of Inflammation in Rats: Involvement of an Antioxidant Mechanism

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    Hulya Uzkeser

    2012-01-01

    Full Text Available The possible role of β-2 adrenergic receptors in modulation of inflammatory and nociceptive conditions suggests that the β-2 adrenergic receptor agonist, salbutamol, may have beneficial anti-inflammatory and analgesic effects. Therefore, in this study, we induced inflammatory and nociceptive responses with carrageenan-induced paw edema or cotton-pellet-induced granuloma models, both of which result in oxidative stress. We hypothesized that salbutamol would prevent inflammatory and nociceptive responses by stimulating β-2 adrenergic receptors and the prevention of generation of ROS during the acute inflammation process in rats. Both doses of salbutamol used in the study (1 and 2 mg/kg effectively blocked the acute inflammation and inflammatory nociception induced by carrageenan. In the cotton-pellet-induced granuloma test, both doses of salbutamol also significantly decreased the weight of granuloma tissue on the cotton pellets when compared to the control. Anti-inflammatory and analgesic effects of salbutamol were found to be comparable with those of indomethacin. Salbutamol decreased myeloperoxidase (MPO activity and lipid peroxidation (LPO level and increased the activity of superoxide dismutase (SOD and level of glutathione (GSH during the acute phase of inflammation. In conclusion, salbutamol can decrease acute and chronic inflammation, possibly through the stimulation of β-2 adrenergic receptors. This anti-inflammatory effect may be of significance in asthma treatment, where inflammation also takes part in the etiopathology. This study reveals that salbutamol has significant antioxidative effects, which at least partially explain its anti-inflammatory capabilities. These findings presented here may also shed light on the roles of β-2 adrenergic receptors in inflammatory and hyperalgesic conditions.

  12. B Cell, Th17, and Neutrophil Related Cerebrospinal Fluid Cytokine/Chemokines Are Elevated in MOG Antibody Associated Demyelination.

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    Kavitha Kothur

    Full Text Available Myelin oligodendrocyte glycoprotein antibody (MOG Ab associated demyelination represents a subgroup of autoimmune demyelination that is separate from multiple sclerosis and aquaporin 4 IgG-positive NMO, and can have a relapsing course. Unlike NMO and MS, there is a paucity of literature on immunopathology and CSF cytokine/chemokines in MOG Ab associated demyelination.To study the differences in immunopathogenesis based on cytokine/chemokine profile in MOG Ab-positive (POS and -negative (NEG groups.We measured 34 cytokines/chemokines using multiplex immunoassay in CSF collected from paediatric patients with serum MOG Ab POS [acute disseminated encephalomyelitis (ADEM = 8, transverse myelitis (TM = 2 n = 10] and serum MOG Ab NEG (ADEM = 5, TM = 4, n = 9 demyelination. We generated normative data using CSF from 20 non-inflammatory neurological controls.The CSF cytokine and chemokine levels were higher in both MOG Ab POS and MOG Ab NEG demyelination groups compared to controls. The CSF in MOG Ab POS patients showed predominant elevation of B cell related cytokines/chemokines (CXCL13, APRIL, BAFF and CCL19 as well as some of Th17 related cytokines (IL-6 AND G-CSF compared to MOG Ab NEG group (all p<0.01. In addition, patients with elevated CSF MOG antibodies had higher CSF CXCL13, CXCL12, CCL19, IL-17A and G-CSF than patients without CSF MOG antibodies.Our findings suggest that MOG Ab POS patients have a more pronounced CNS inflammatory response with elevation of predominant humoral associated cytokines/chemokines, as well as some Th 17 and neutrophil related cytokines/chemokines suggesting a differential inflammatory pathogenesis associated with MOG antibody seropositivity. This cytokine/chemokine profiling provides new insight into disease pathogenesis, and improves our ability to monitor inflammation and response to treatment. In addition, some of these molecules may represent potential immunomodulatory targets.

  13. Chronic Ethanol Feeding Modulates Inflammatory Mediators, Activation of Nuclear Factor-κB, and Responsiveness to Endotoxin in Murine Kupffer Cells and Circulating Leukocytes

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    Miriam Maraslioglu

    2014-01-01

    Full Text Available Chronic ethanol abuse is known to increase susceptibility to infections after injury, in part, by modification of macrophage function. Several intracellular signalling mechanisms are involved in the initiation of inflammatory responses, including the nuclear factor-κB (NF-κB pathway. In this study, we investigated the systemic and hepatic effect of chronic ethanol feeding on in vivo activation of NF-κB in NF-κBEGFP reporter gene mice. Specifically, the study focused on Kupffer cell proinflammatory cytokines IL-6 and TNF-α and activation of NF-κB after chronic ethanol feeding followed by in vitro stimulation with lipopolysaccharide (LPS. We found that chronic ethanol upregulated NF-κB activation and increased hepatic and systemic proinflammatory cytokine levels. Similarly, LPS-stimulated IL-1β release from whole blood was significantly enhanced in ethanol-fed mice. However, LPS significantly increased IL-6 and TNF-α levels. These results demonstrate that chronic ethanol feeding can improve the responsiveness of macrophage LPS-stimulated IL-6 and TNF-α production and indicate that this effect may result from ethanol-induced alterations in intracellular signalling through NF-κB. Furthermore, LPS and TNF-α stimulated the gene expression of different inflammatory mediators, in part, in a NF-κB-dependent manner.

  14. Relationship between proinflammatory cytokines and anti-inflammatory cytokines in patients with chronic pelvic inflammatory disease%慢性盆腔炎患者促炎因子与抗炎因子的关系

    Institute of Scientific and Technical Information of China (English)

    王莉; 吕耀凤; 姚丽娟

    2012-01-01

    目的:探讨慢性盆腔炎患者血清促炎因子与抗炎因子的表达与相关性.方法:选择87例慢性盆腔炎患者作为病例组,选择同期健康体检妇女69例作为对照组,ELISA法检测血清TNF-α、IL-1 β、IL-6和抗炎细胞因子IL-4、IL-10的表达.结果:病例组患者血清TNF-α、IL-1β和IL-6表达高于对照组(P<0.05),而血清IL-4和IL-10表达低于对照组(P<0.05);病例组患者血清促炎因子的表达与抗炎因子的表达呈负相关(P<0.05).结论:慢性盆腔炎患者促炎因子过度激活,而抗炎因子被抑制,并且二者表达具有一定的相关性,共同促进慢性盆腔炎的发生发展.%Objective: To explore the expressions of serum proinflammatory cytokines and anti — inflammatory cytokines in patients with chronic pelvic inflammatory disease and their correlation. Methods; A total of 87 patients with chronic pelvic inflammatory disease were selected as case group, and 69 women who received physical examination during the same period were selected as control group, ELISA was used to detect the expression levels of serum inflammatory cytokines ( TNF — α, IL - 1β, IL — 6 ) and anti — inflammatory cytokines (IL — 4, IL — 10) . Results; The expression levels of serum TNF — α, IL — 1 β, and IL - 6 in case group were statistically significantly higher than those in control group (P < 0. 05 ) . The expression levels of serum TL —4 and IL — 10 in case group were statistically significantly lower than those in control group (P<0. 05) . There was a negative correlation between expressions of serum proinflammatory cytokines and expressions of serum anti - inflammatory cytokines in case group (P <0. 05 ) . Conclusion; Among the patients with chronic pelvic inflammatory disease, proinflammatory cytokines were activated and anti — inflammatory cytokines were inhibited, and there was a certain correlation between them, both proinflammatory cytokines and anti — inflammatory

  15. Directional diffusivity as a magnetic resonance (MR) biomarker in demyelinating disease

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    Benzinger, Tammie L. S.; Cross, Anne H.; Xu, Junqian; Naismith, Robert; Sun, Shu-Wei; Song, Sheng-Kwei

    2007-09-01

    Directional diffusivities derived from diffusion tensor magnetic resonance imaging (DTI) measurements describe water movement parallel to (λ ||, axial diffusivity) and perpendicular to (λ⊥radial diffusivity) axonal tracts. λ || and λ⊥ have been shown to differentially detect axon and myelin abnormalities in several mouse models of central nervous system white matter pathology in our laboratory. These models include experimental autoimmune encephalomyelitis (EAE), (1) myelin basic protein mutant mice with dysmyelination and intact axons, (2) cuprizone-induced demyelination, and remyelination, with reversible axon injury (2, 3) and a model of retinal ischemia in which retinal ganglion cell death is followed by Wallerian degeneration of optic nerve, with axonal injury preceding demyelination. (4) Decreased λ|| correlates with acute axonal injury and increased λ⊥ indicates myelin damage. (4) More recently, we have translated this approach to human MR, investigating acute and chronic optic neuritis in adults with multiple sclerosis, brain lesions in adults with multiple sclerosis, and acute disseminated encephalomyelitis (ADEM) in children. We are also investigating the use of this technique to probe the underlying structural change of the cervical spinal cord in acute and chronic T2- hyperintense lesions in spinal stenosis, trauma, and transverse myelitis. In each of these demyelinating diseases, the discrimination between axonal and myelin injury which we can achieve has important prognostic and therapeutic implications. For those patients with myelin injury but intact axons, early, directed drug therapy has the potential to prevent progression to axonal loss and permanent disability.

  16. A Neural Model of Demyelination of the Mouse Spinal Cord

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    Petreska, Biljana; Yovel, Yossi

    2008-01-01

    This paper presents a neural network model of demyelination of the mouse motor pathways, coupled to a central pattern generation (CPG) model for quadruped walking. Demyelination is the degradation of the myelin layer covering the axons which can be caused by several neurodegenerative autoimmune diseases such as multiple sclerosis. We use this model - to our knowledge first of its kind - to investigate the locomotion deficits that appear following demyelination of axons in the spinal cord. Our...

  17. Pro-inflammatory interleukins in middle ear effusions from atopic and non-atopic children with chronic otitis media with effusion.

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    Zielnik-Jurkiewicz, Beata; Stankiewicz-Szymczak, Wanda

    2016-06-01

    Chronic otitis media with effusion (OME) is associated with irreversible changes in the middle ear, sometimes leading to hearing loss and abnormal language development in children. While the pathogenesis of OME is not fully understood, inflammatory and allergic factors are thought to be involved. The study aimed to investigate the role of cytokines in the local development of chronic OME, and assess differences in the cytokine profiles between atopic and non-atopic children. 84 atopic and non-atopic children with chronic OME (mean age of 6 years 7 months) were studied. Age-matched children with hypertrophy of the adenoids and Eustachian tube dysfunction served as the control group. The number of past acute otitis media (AOM) episodes, their age, and the type of effusion were recorded for all children. Pro-inflammatory cytokine concentrations (TNF-α, IL-1β, IL-6 and IL-8) were determined and the presence of pathogenic bacteria in the patients' effusions was examined. High concentrations of TNF-α, IL-1β, IL-6 and IL-8 were found in the effusions in all children with chronic OME, with the highest levels observed in the non-atopic group. The atopic group showed persistently high IL-1β levels, while in the non-atopic children, IL-1β and TNF-α levels positively correlated with the patient's age and the number of past AOM episodes. Pathogenic bacteria were more frequently isolated from effusions in non-atopic children. In both atopic and non-atopic children, pro-inflammatory cytokines are found at high concentrations. This argues in favor of instituting anti-inflammatory management for treating OME, regardless of atopy. PMID:26078091

  18. Chronic Pancreatitis and Systemic Inflammatory Response Syndrome Prevent Impact of Chemotherapy with Gemcitabine in a Genetically Engineered Mouse Model of Pancreatic Cancer

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    Richard F. Knoop

    2014-06-01

    CONCLUSION: We could demonstrate for the first time that an improvement in median overall survival with gemcitabine is significantly abolished by a persistent mild chronic pancreatitis and a systemic inflammatory response syndrome. In particular, the inflammation biomarkers C-reactive protein, IL-6, and IL-1α could indicate the prognostic benefit of gemcitabine chemotherapy and should now be tested in prospective patient-controlled trials.

  19. Research on Trypanosoma cruzi and Analysis of Inflammatory Infiltrate in Esophagus and Colon from Chronic Chagasic Patients with and without Mega

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    Eliângela de Castro Côbo

    2012-01-01

    Full Text Available To compare parasitism and inflammatory process in esophagus and colon from chronic chagasic patients, immunohistochemistry was carried out to research for T. cruzi and to evaluate the inflammatory infiltrate in the muscular and myenteric plexus in 39 esophagi (20 with and 19 without megaesophagus and 50 colons (25 with and 25 without megacolon. The frequency of T. cruzi in megaesophagus was 20%, and in megacolon it was 4%. No amastigotes were found in organs without mega; considering the total of esophagi (with and without mega, the frequency of T. cruzi would be 10% and 2% in the colon. Myositis and ganglionitis were more frequent and intense in organs with mega compared to those without mega, and in esophagus compared to colon. Qualitatively, inflammatory infiltration in esophagus and colon, with or without mega, was similar, consisting predominantly of T lymphocytes (CD3+, scarce macrophages (CD68+, and rare B lymphocytes (CD20+.

  20. Osmotic demyelination syndrome with recent chemotherapy in normonatremic patient: A case report

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    Lee, Sungjae; Baek, Hye Jin; Jung, Hyun Kyung; Kim, Seon Jeong; Lee, Yedaun; Lee, Kwaghwi; Ryu, Ji Hwa; Kim, Hong Dae [Dept. of Radiology, Haeundae Paik Hospital, Inje University College of Medicine, Busan (Korea, Republic of)

    2014-11-15

    Osmotic demyelination syndrome (ODS), an acquired demyelinating condition of the central pons and/or other regions of the brain, is frequently associated with rapid correction of hyponatremia. There are several reports of ODS in other clinical setting such as malnutrition, alcoholism, transplantation, malignancy, and chronic debilitating illness. However, cases of ODS associated with chemotherapy have not been frequently reported. Here, we describe a case of ODS in a normonatremic patient recently underwent chemotherapy for colon cancer. The diagnosis was confirmed by MRI showing a typical T2 hyperintensity in the central pons. This case suggests that ODS is not always associated with hyponatremia and that ODS can have a favorable clinical and radiologic prognosis.