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1.
J Neuroimmunol ; 385: 578238, 2023 12 15.
Artigo em Inglês | MEDLINE | ID: mdl-37925900

RESUMO

To evaluate B-cell involvement in chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), 11 patients with CIDP, 8 patients with Guillain-Barré syndrome and 13 patients with idiopathic normal pressure hydrocephalus (iNPH) were studied. CSF cytokine and chemokine (IL-10, IL-15, TNF-α, TGF-ß1, GM-CSF, BAFF, CXCL10, and CXCL13) levels were measured by ELISA. The CSF CXCL13 level was significantly higher in patients with CIDP than in those with iNPH. The CSF CXCL13 level was significantly higher in CIDP patients with higher annualized relapse rates and higher modified Rankin scale scores. The CSF CXCL13 level is elevated in CIDP, especially in those with higher disease activity.


Assuntos
Quimiocina CXCL13 , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Humanos , Quimiocina CXCL13/líquido cefalorraquidiano , Fator Estimulador de Colônias de Granulócitos e Macrófagos , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Síndrome de Guillain-Barré/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Masculino , Feminino , Pessoa de Meia-Idade , Idoso
2.
J Peripher Nerv Syst ; 25(2): 162-170, 2020 06.
Artigo em Inglês | MEDLINE | ID: mdl-32364302

RESUMO

Motor chronic inflammatory demyelinating polyneuropathy (CIDP) is a rare and poorly described subtype of CIDP. We aimed to study their clinical and electrophysiological characteristics and response to treatment. From a prospective database of CIDP patients, we included patients with definite or probable CIDP with motor signs and without sensory signs/symptoms at diagnosis. Patients were considered to have pure motor CIDP (PM-CIDP) if sensory conductions were normal or to have motor predominant CIDP (MPred-CIDP) if ≥2 sensory nerve action potential amplitudes were abnormal. Among the 700 patients with CIDP, 17 (2%) were included (PM-CIDP n = 7, MPred-CIDP n = 10); 71% were male, median age at onset was 48 years (range: 13-76 years), 47% had an associated inflammatory or infectious disease or neoplasia. At the more severe disease stage, 94% of patients had upper and lower limb weakness, with distal and proximal weakness in 4 limbs for 56% of them. Three-quarters (75%) responded to intravenous immunoglobulins (IVIg) and four of five patients to corticosteroids including three of three patients with MPred-CIDP. The most frequent conduction abnormalities were conduction blocks (CB, 82%) and F-wave abnormalities (88%). During follow up, 4 of 10 MPred-CIDP patients developed mild sensory symptoms; none with PM-CIDP did so. Patients with PM-CIDP had poorer outcome (median ONLS: 4; range: 22-5) compared to MPred-CIDP (2, range: 0-4; P = .03) at last follow up. This study found a progressive clinical course in the majority of patients with motor CIDP as well as frequent associated diseases, CB, and F-wave abnormalities. Corticosteroids might be considered as a therapeutic option in resistant IVIg patients with MPred-CIDP.


Assuntos
Progressão da Doença , Condução Nervosa/fisiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Criança , Eletromiografia , Feminino , Humanos , Itália , Masculino , Pessoa de Meia-Idade , Neuroimagem , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Recidiva , Estudos Retrospectivos , Resultado do Tratamento , Adulto Jovem
3.
Front Immunol ; 10: 515, 2019.
Artigo em Inglês | MEDLINE | ID: mdl-30984164

RESUMO

Objective: Utilize immune cell profiles in the cerebrospinal fluid (CSF) to advance the understanding and potentially support the diagnosis of inflammatory neuropathies. Methods: We analyzed CSF cell flow cytometry data of patients with definite Guillain-Barré syndrome (GBS, n = 26) and chronic inflammatory demyelinating polyneuropathy (CIDP, n = 32) based on established diagnostic criteria in comparison to controls with relapsing-remitting multiple sclerosis (RRMS, n = 49) and idiopathic intracranial hypertension (IIH, n = 63). Results: Flow cytometry revealed disease-specific changes of CSF cell composition with a significant increase of NKT cells and CD8+ T cells in CIDP, NK cells in GBS, and B cells and plasma cells in MS in comparison to IIH controls. Principal component analysis demonstrated distinct CSF immune cells pattern in inflammatory neuropathies vs. RRMS. Systematic receiver operator curve (ROC) analysis identified NKT cells as the best parameter to distinguish GBS from CIDP. Composite scores combing several of the CSF parameters differentiated inflammatory neuropathies from IIH and GBS from CIDP with high confidence. Applying a novel dimension reduction technique, we observed an intra-disease heterogeneity of inflammatory neuropathies. Conclusion: Inflammatory neuropathies display disease- and subtype-specific alterations of CSF cell composition. The increase of NKT cells and CD8+ T cells in CIDP and NK cells in GBS, suggests a central role of cytotoxic cell types in inflammatory neuropathies varying between acute and chronic subtypes. Composite scores constructed from multi-dimensional CSF parameters establish potential novel diagnostic tools. Intra-disease heterogeneity suggests distinct disease mechanisms in subgroups of inflammatory neuropathies.


Assuntos
Síndrome de Guillain-Barré/líquido cefalorraquidiano , Células Matadoras Naturais/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Linfócitos T/imunologia , Adolescente , Adulto , Idoso , Idoso de 80 Anos ou mais , Feminino , Citometria de Fluxo , Síndrome de Guillain-Barré/imunologia , Humanos , Inflamação/líquido cefalorraquidiano , Inflamação/imunologia , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla Recidivante-Remitente/líquido cefalorraquidiano , Esclerose Múltipla Recidivante-Remitente/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Pseudotumor Cerebral/líquido cefalorraquidiano , Pseudotumor Cerebral/imunologia , Adulto Jovem
4.
Muscle Nerve ; 60(2): 180-183, 2019 08.
Artigo em Inglês | MEDLINE | ID: mdl-30989684

RESUMO

INTRODUCTION: Recent literature has concluded that cerebrospinal fluid total protein (CSF-TP) upper reference limits (URL) should be higher than 45 mg/dl and stratified by age. METHODS: Data-driven URLs were applied to the analysis of a cohort of patients with correctly and incorrectly diagnosed chronic inflammatory demyelinating polyneuropathy (CIDP). Descriptive statistics were calculated, and exploratory analyses were used to test the impact of different CSF-TP URLs on sensitivity and specificity of CIDP diagnosis. RESULTS: The adoption of higher and age-dependent CSF-TP URLs reduced the sensitivity of CSF analysis slightly (from 95% to 84%-86%); however, the overall CIDP detection rate was unchanged. Twelve of 36 (33%) false-positive diagnoses occurred with CSF-TP elevation as the sole supportive criteria. By applying updated CSF-TP URLs, the specificity of CSF analysis increased from 39% to 57%-64%. DISCUSSION: Implementation of data-driven CSF-TP URLs improves CIDP diagnostic specificity without compromising sensitivity, thereby lessening CIDP misdiagnosis. Muscle Nerve 60: 180-183, 2019.


Assuntos
Proteínas do Líquido Cefalorraquidiano/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Fatores Etários , Biópsia , Erros de Diagnóstico , Eletrodiagnóstico , Feminino , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Nervos Periféricos/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Valores de Referência , Sensibilidade e Especificidade
5.
Eur Cytokine Netw ; 30(4): 130-134, 2019 Dec 01.
Artigo em Inglês | MEDLINE | ID: mdl-32096474

RESUMO

This pilot study was designed to compare the levels of interleukin-8 (IL-8), a pro-inflammatory chemokine, in the cerebrospinal fluid (CSF) of patients with Guillain-Barre syndrome (GBS), chronic inflammatory demyelinating polyneuropathy (CIDP), non-inflammatory polyneuropathy (PNP), and other non-inflammatory neurological diseases (functional syndrome or migraine). The results show elevated CSF IL-8 levels in GBS compared to the other groups (p < 0.05). IL-8 could be considered a potential biomarker to differentiate GBS from CIDP. This distinction could be relevant in terms of therapeutic decisions and functional prognosis.


Assuntos
Síndrome de Guillain-Barré/diagnóstico , Interleucina-8/líquido cefalorraquidiano , Transtornos de Enxaqueca/diagnóstico , Polineuropatias/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Adulto , Idoso , Biomarcadores/líquido cefalorraquidiano , Diagnóstico Diferencial , Feminino , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Síndrome de Guillain-Barré/imunologia , Humanos , Interleucina-8/imunologia , Masculino , Pessoa de Meia-Idade , Transtornos de Enxaqueca/líquido cefalorraquidiano , Transtornos de Enxaqueca/imunologia , Projetos Piloto , Polineuropatias/líquido cefalorraquidiano , Polineuropatias/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Estudos Retrospectivos , Fator de Necrose Tumoral alfa/líquido cefalorraquidiano , Fator de Necrose Tumoral alfa/imunologia
6.
Medicina (B.Aires) ; Medicina (B.Aires);73(3): 259-262, jun. 2013. ilus, tab
Artigo em Espanhol | LILACS | ID: lil-694775

RESUMO

La polirradiculopatía inflamatoria crónica sensitiva es una entidad definida, frecuentemente subdiagnosticada y potencialmente tratable. Debe ser sospechada en pacientes con ataxia sensitiva, estudios de conducción nerviosa normales y una resonancia magnética que muestre engrosamiento y realce con gadolinio de las raíces lumbosacras. Presentamos el caso de un hombre de 57 años de edad con marcada ataxia sensitiva en pierna izquierda. Al examen físico presentaba fuerza conservada, reflejos osteotendinosos disminuidos, tacto fino y superficial reducidos por debajo de las rodillas; abatiestesia y apalestesia en ambos pies. Los estudios de conducción nerviosa eran normales, los potenciales evocados somatosensitivos tibiales con ausencia de respuesta bilateral. El líquido cefalorraquídeo presentaba hiperproteinorraquia sin células. La resonancia magnética mostró engrosamiento y realce con gadolinio de las raíces lumbosacras. El paciente fue tratado con inmunoglobulina endovenosa (IgEV) a 2 g/kg durante 5 días, con buena respuesta. La evolución clínica, la hiperproteinorraquia, el realce de raíces en la resonancia magnética, la buena respuesta a la inmunoterapia y la exclusión de otras causas de ataxia sensitiva fueron compatibles con el diagnóstico de polirradiculopatía inflamatoria crónica sensitiva. Para el diagnóstico de esta enfermedad se requiere la identificación del compromiso aislado de las raíces sensitivas.


Chronic inflammatory sensory polyradiculopathy is a defined entity, frequently underdiagnosed, and potentially treatable. It must be suspected in patients with sensory ataxia, normal nerve conduction studies, and MRI with thickened lumbosacral nerve roots and gadolinium enhancement. We present the case of a 57-year-old man with marked sensory ataxia on his left leg. Examination showed normal strength, decreased knee and ankle jerks. Light touch and pinprick sensations were reduced below the knees. Vibration and joint position sense were absent at the feet. Nerve conduction studies were normal. Tibial sensory evoked potentials disclosed absent responses bilaterally. CSF was acellular with elevated protein. Lumbosacral magnetic resonance showed thickening of roots, with gadolinium enhancement. The patient was treated with IV-Ig, 2 g/kg, for 5 days with improvement of symptoms. The clinical course, elevated CSF protein, the evidence of root enhancement on the MRI, good response to immunotherapy, and the exclusion of other causes of sensory ataxia, were compatible with the diagnosis of chronic inflammatory sensory polyradiculopathy. To diagnose this disease the identification of isolated involvement of the sensory roots is required.


Assuntos
Humanos , Masculino , Pessoa de Meia-Idade , Gadolínio , Condução Nervosa/fisiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Eletromiografia , Imageamento por Ressonância Magnética , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Raízes Nervosas Espinhais/patologia
7.
Semin Neurol ; 32(3): 187-95, 2012 Jul.
Artigo em Inglês | MEDLINE | ID: mdl-23117943

RESUMO

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.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Corticosteroides/uso terapêutico , Albuminas/metabolismo , Anti-Inflamatórios/uso terapêutico , Eletrodiagnóstico , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Imunossupressores/uso terapêutico , Nervos Periféricos/patologia , Troca Plasmática , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Transplante de Células-Tronco
8.
Nat Rev Neurol ; 7(9): 507-17, 2011 Aug 16.
Artigo em Inglês | MEDLINE | ID: mdl-21844897

RESUMO

Chronic inflammatory demyelinating polyneuropathy (CIDP) is the most common chronic autoimmune neuropathy. Despite clinical challenges in diagnosis-owing in part to the existence of disease variants, and different views on how many electrophysiological abnormalities are needed to document demyelination-consensus criteria seem to have been reached for research or clinical practice. Current standard of care involves corticosteroids, intravenous immunoglobulin (IVIg) and/or plasmapheresis, which provide short-term benefits. Maintenance therapy with IVIg can induce sustained remission, increase quality of life and prevent further axonal loss, but caution is needed to avoid overtreatment. Commonly used immunosuppressive drugs offer minimal benefit, necessitating the development of new therapies for treatment-refractory patients. Advances in our understanding of the underlying immunopathology in CIDP have identified new targets for future therapeutic efforts, including T cells, B cells, and transmigration and transduction molecules. New biomarkers and scoring systems represent emerging tools with the potential to predict therapeutic responses and identify patients with active disease for enrollment into clinical trials. This Review highlights the recent advances in diagnosing CIDP, provides an update on the immunopathology including new target antigens, and discusses current treatments, ongoing challenges and future therapeutic directions.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Corticosteroides/uso terapêutico , Adulto , Idade de Início , Biópsia , Diagnóstico Diferencial , Progressão da Doença , Eletrodiagnóstico , Transplante de Células-Tronco Hematopoéticas , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Pessoa de Meia-Idade , Plasmaferese , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/complicações , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia
9.
Brain Res ; 1361: 140-5, 2010 Nov 18.
Artigo em Inglês | MEDLINE | ID: mdl-20849835

RESUMO

Cystatin C (CC) is a cysteine protease inhibitor abundantly expressed in the central nervous system. Bunina bodies, small eosinophilic intraneuronal inclusions, are stain positive for CC and are the most specific histological hallmark of amyotrophic lateral sclerosis (ALS). In this study, employing a latex turbidimetric immunoassay, levels of CC in cerebrospinal fluid (CSF) were quantified in 130 age-matched individuals with either a neurological disorder [ALS, Alzheimer's disease (AD), Parkinson's disease (PD), tauopathy (TP), multiple system atrophy (MSA), chronic inflammatory demyelinating polyneuropathy (CIDP)] or no known neurological condition (normal control, NC). The CC level in CSF was found to be correlated with the age during the investigation but not the protein concentration. There was no difference in CC levels between NC and ALS or CIDP cases, whereas CC levels were significantly lower in MSA compared with NC. Of the 130 cases, 96 were genotyped, and G/A or A/A polymorphism at +73 within the CST3 gene was found in 28 individuals. The CC level was significantly lower in the combined group of G/A and A/A genotypes compared with G/G. The present data demonstrate that the level of CC in CSF should not be considered as a biomarker of ALS, but there is a correlation between CC levels and the CST3 genotype.


Assuntos
Cistatina C/líquido cefalorraquidiano , Doenças do Sistema Nervoso/líquido cefalorraquidiano , Doenças do Sistema Nervoso/genética , Polimorfismo de Nucleotídeo Único , Idoso , Idoso de 80 Anos ou mais , Alanina , Doença de Alzheimer/líquido cefalorraquidiano , Doença de Alzheimer/genética , Esclerose Lateral Amiotrófica/líquido cefalorraquidiano , Esclerose Lateral Amiotrófica/genética , Biomarcadores , Estudos de Casos e Controles , Cistatina C/sangue , Cistatina C/genética , Feminino , Glicina , Humanos , Imunoensaio/métodos , Látex , Masculino , Pessoa de Meia-Idade , Atrofia de Múltiplos Sistemas/líquido cefalorraquidiano , Atrofia de Múltiplos Sistemas/genética , Nefelometria e Turbidimetria/métodos , Doenças do Sistema Nervoso/sangue , Doença de Parkinson/líquido cefalorraquidiano , Doença de Parkinson/genética , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/genética , Tauopatias/líquido cefalorraquidiano , Tauopatias/genética
10.
J Neuroimmunol ; 225(1-2): 149-52, 2010 Aug 25.
Artigo em Inglês | MEDLINE | ID: mdl-20510468

RESUMO

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.


Assuntos
Esclerose Lateral Amiotrófica , Herpesvirus Humano 4/imunologia , Esclerose Múltipla , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Adulto , Idoso , Esclerose Lateral Amiotrófica/sangue , Esclerose Lateral Amiotrófica/líquido cefalorraquidiano , Esclerose Lateral Amiotrófica/virologia , Anticorpos/sangue , Anticorpos/líquido cefalorraquidiano , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Esclerose Múltipla/sangue , Esclerose Múltipla/líquido cefalorraquidiano , Esclerose Múltipla/virologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/virologia
11.
Cytokine ; 51(2): 138-43, 2010 Aug.
Artigo em Inglês | MEDLINE | ID: mdl-20538476

RESUMO

INTRODUCTION: Guillain-Barré syndrome (GBS) and chronic inflammatory demyelinating polyneuropathy (CIDP) share histopathological features but display different disease courses; we measured the concentration of 50 inflammatory mediators in the cerebrospinal fluid (CSF) of patients with either of these diseases. PATIENTS AND METHODS: CSF samples were collected during a diagnostic lumbar puncture and stored at -30 degrees C. We analyzed the CSF of nine subjects with GBS; eight with CIDP; eight with diabetic polyneuropathy (DP) and seven with headache (controls). Fifty inflammatory mediators were simultaneously measured with a multiplex bead-based ELISA on a Suspension Array System. After Bonferroni's correction for repeated measures, non-parametric variance and post hoc test were calculated. RESULTS: Thirty-two inflammatory mediators were expressed. The median concentration of IL-6, IL-9, IL-15, IL-18, CCL4, CXCL1, LIF, MIF, PDGFbb, IFN-gamma2, IL-2ra, IL-12(p40), IL-16, SCGF-b, TRAIL, FGF, G-CSF, GM-CSF, and M-CSF was not different among groups (variance: n.s.). The median concentration of CCL2, CCL7, CCL27, CXCL9, CXCL10, CXCL12, ICAM-1, VCAM1 and VEGF was higher in CIDP and GBS compared with controls (p<0.002). The median concentration of IL-8 and IL-1ra was higher in GBS than CIDP or DP or controls, whereas stem cell factor (SCF) and hepatocyte growth factor (HGF) were higher in CIDP than GBS or DP or controls (p<0.002). DISCUSSION: Mediators of the recruitment and activation of lymphocytes and monocytes are expressed in the CSF of CIDP and GBS. IL-8 and IL-1ra are characteristic of GBS, whereas growth factors (SCF, HGF) of CIDP are possibly related to chronicity or to the survival/repair processes of neurons.


Assuntos
Síndrome de Guillain-Barré/líquido cefalorraquidiano , Mediadores da Inflamação/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Adulto , Fatores de Crescimento de Células Hematopoéticas/líquido cefalorraquidiano , Fator de Crescimento de Hepatócito/líquido cefalorraquidiano , Humanos , Proteína Antagonista do Receptor de Interleucina 1/líquido cefalorraquidiano , Interleucina-8/líquido cefalorraquidiano
12.
J Neurol Sci ; 269(1-2): 138-42, 2008 Jun 15.
Artigo em Inglês | MEDLINE | ID: mdl-18279894

RESUMO

INTRODUCTION: Gas6 enhances survival of Schwann cells and neurons in vitro and participates in autoimmunity in animal models. Since its concentration in human cerebrospinal fluid (CSF) is unknown, we measured it in samples from patients with non-inflammatory/non-autoimmune neurological diseases (NINAD) and autoimmune polyneuropathies. MATERIALS AND METHODS: Samples collected after informed consent during diagnostic lumbar puncture in the period 1999-2006 were stored at -30 degrees C. We considered subjects with NINAD (stroke, ALS, headache, psychiatric conditions simulating neurological diseases, otologic dizziness) or with Guillain-Barré syndrome (GBS) or CIDP. CSF and plasma total protein and age were obtained from clinical records. Gas6 was measured with an ELISA developed and validated in our laboratory (inter-, intra-assay CVs <10%, recovery 96%). Variance, Tukey's post-hoc test, regression were calculated with a statistical software (Statsoft). RESULTS: Mean Gas6 concentration in patients with NINAD was 6.5+/-2.4 ng/ml, 7.2+/-2.6 ng/ml in GBS and significantly higher (11.5+/-1.7 ng/ml) in CIDP than in the other conditions (post-hoc, p<0.005). It was not related to age, CSF total proteins or to CSF/plasma ratio of total proteins (regression, p>0.1). CONCLUSIONS: Gas6 is detectable in CSF and may be involved in chronic autoimmune demyelination or myelin repair.


Assuntos
Regulação da Expressão Gênica/fisiologia , Peptídeos e Proteínas de Sinalização Intercelular/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Adulto , Idoso , Idoso de 80 Anos ou mais , Ensaio de Imunoadsorção Enzimática/métodos , Feminino , Síndrome de Guillain-Barré/sangue , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Humanos , Peptídeos e Proteínas de Sinalização Intercelular/sangue , Masculino , Pessoa de Meia-Idade , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Análise de Regressão , Reprodutibilidade dos Testes , Estudos Retrospectivos
13.
J Neurol Neurosurg Psychiatry ; 78(12): 1349-53, 2007 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-17494979

RESUMO

AIM: The clinical and epidemiological characteristics of chronic inflammatory demyelinating polyneuropathy (CIDP) in an Italian population were assessed. SUBJECTS AND METHODS: All subjects with a diagnosis of demyelinating neuropathy after 1990 in Piemonte and Valle d'Aosta (4,334,225 inhabitants) were considered. The diagnosis of CIDP was based on the research criteria of the American Academy of Neurology. 165 of 294 patients met the diagnostic criteria. RESULTS: The crude prevalence rate was 3.58/100,000 population (95% CI 3.02 to 4.20). At the prevalence day, 76 (49.0%) cases had definite, 67 (43.2%) probable and 12 (7.7%) possible CIDP; disability was mild in 105 (67.7%) cases, moderate in 32 (20.6%) and severe in 18 (11.6%). The course was remitting-relapsing in 40 cases (25.8%), chronic progressive in 96 (61.9%) and monophasic in 19 (12.3%). Considering the 95 patients whose disorder presented in the period 1995-2001, the mean annual crude incidence rate was 0.36/100,000 population (95% CI 0.29 to 0.44), with a male to female ratio of 2.3:1. 14 cases were affected by diabetes mellitus. In multivariate analysis, factors related to severe disability at the prevalence day were: age >60 years; failure of immunomodulating therapies at the time of diagnosis; worse disability at nadir; and chronic course. CONCLUSION: Incidence and prevalence rates of CIDP in Italy were higher than those observed in most previous studies. At the prevalence day, more than 80% of cases had a mild or moderate disability, indicating either a good response to immunomodulating therapy or a tendency of CIDP to have a mild course in most cases.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/epidemiologia , Distribuição por Idade , Idoso , Biópsia , Área Programática de Saúde , Avaliação da Deficiência , Progressão da Doença , Feminino , Humanos , Incidência , Itália/epidemiologia , Masculino , Pessoa de Meia-Idade , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Prevalência , Índice de Gravidade de Doença , Distribuição por Sexo , Nervo Sural/patologia
14.
Rev Neurol (Paris) ; 162(4): 533-8, 2006 Apr.
Artigo em Francês | MEDLINE | ID: mdl-16585917

RESUMO

Although there are no specific biological markers of chronic inflammatory demyelinating polyneuropathies (CIDP), biological investigations have played an important role in determining the limits of the concept of CIDP. This is best exemplified by the individualisation of demyelinating neuropathies associated with IgM monoclonal proteins reacting with glycolipids. The signification of CIDP associated with diabetes mellitus, monoclonal IgG or IgA, or mutations of myelin proteins has recently been discussed as they may have implications in our understanding of the pathophysiology of CIDP and raise the question of knowing whether they confer to CIDP a particular clinical presentation or evolution.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/metabolismo , Adulto , Autoanticorpos/imunologia , Autoantígenos/imunologia , Biópsia , Proteínas do Líquido Cefalorraquidiano/análise , Doença de Charcot-Marie-Tooth/genética , Criança , Neuropatias Diabéticas/metabolismo , Glicolipídeos/imunologia , Humanos , Imunoglobulina M/análise , Imunoglobulina M/imunologia , Mutação , Proteína P0 da Mielina/genética , Proteínas da Mielina/genética , Paraproteinemias/complicações , Paraproteinemias/imunologia , Nervos Periféricos/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/etiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/genética , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia
15.
J Neuroimmunol ; 159(1-2): 165-76, 2005 Feb.
Artigo em Inglês | MEDLINE | ID: mdl-15652416

RESUMO

The role of antigen-presenting cells (APC) involved in induction of T and B cell mediated autoaggressive immunity in Guillain-Barre syndrome (GBS) and chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is poorly understood. We studied the numbers and phenotype of dendritic cells (DC) in blood and cerebrospinal fluid (CSF) over the course of GBS and CIDP before and after immunomodulatory treatment. Four out of seven GBS patients examined prior to treatment with high-dose intravenous immunoglobulins (IvIg) had elevated numbers of CD123(+) plasmacytoid DC in the CSF, while both GBS and CIDP patients examined prior to treatment had elevated numbers of CD11c(+) myeloid DC in the CSF, as compared to patients with noninflammatory neurological diseases (OND). The percentages of blood DC expressing the cell surface marker CD1a, co-stimulatory molecules CD80 and CD86, adhesion molecule CD54, and chemokine receptors CCR1, CCR2, CCR5, and CXCR4 were not affected in GBS or CIDP. The immunohistochemistry of sural nerve biopsies revealed CD11c(+)CD83(-)CD14(-)CD16(-) immature myeloid DC at low numbers, mostly in the perineurium, without difference between CIDP patients and controls. In contrast, the numbers of CD11c(+)CD14(+)/CD16(+) macrophages were higher within the endoneurium in CIDP patients compared with the controls. The recruitment of DC to CSF in GBS and CIDP may be important in capturing antigens released from inflamed spinal nerve roots into CSF and in transferring these antigens from CSF to local lymph nodes, where naive T and B cells may be activated.


Assuntos
Células Dendríticas/imunologia , Células Dendríticas/patologia , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Síndrome de Guillain-Barré/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Nervo Sural/imunologia , Nervo Sural/patologia , Antígenos CD/biossíntese , Antígeno B7-1/biossíntese , Antígeno B7-2 , Antígeno CD11c/biossíntese , Diferenciação Celular/imunologia , Células Dendríticas/metabolismo , Avaliação da Deficiência , Síndrome de Guillain-Barré/sangue , Imuno-Histoquímica , Imunofenotipagem , Molécula 1 de Adesão Intercelular/biossíntese , Subunidade alfa de Receptor de Interleucina-3 , Contagem de Leucócitos , Macrófagos/imunologia , Macrófagos/patologia , Glicoproteínas de Membrana/biossíntese , Células Mieloides/imunologia , Células Mieloides/metabolismo , Células Mieloides/patologia , Doenças do Sistema Nervoso/líquido cefalorraquidiano , Doenças do Sistema Nervoso/imunologia , Doenças do Sistema Nervoso/patologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/sangue , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Receptores de Quimiocinas/biossíntese , Receptores de Interleucina-3/biossíntese , Nervo Sural/metabolismo
16.
J Neurol Sci ; 228(1): 75-85, 2005 Jan 15.
Artigo em Inglês | MEDLINE | ID: mdl-15607214

RESUMO

To investigate the intra- and extracellular levels of various cytokines and chemokines in CSF in chronic inflammatory demyelinating polyneuropathy (CIDP) and vasculitic neuropathy (VN), 16 cytokines, IL-1beta, IL-2, IL-4, IL-5, IL-6, IL-7, IL-8, IL-10, IL-12 (p70), IL-13, IL-17, IFN-gamma, TNF-alpha, G-CSF, MCP-1 and MIP-1beta, were measured in CSF supernatant by a multiplexed fluorescent bead-based immunoassay and intracellular production of IFN-gamma and IL-4 in CSF CD4+ T cells were simultaneously measured by flow cytometry in 14 patients with CIDP, 8 patients with VN and 25 patients with other noninflammatory neurologic diseases (OND). In the CSF supernatant, a significant increase of IL-17, IL-8 and IL-6, and a significant decrease of IL-4, IL-5 and IL-7 levels were detected in pretreated CIDP as compared with OND. A significant increase of IL-6, IL-8 and IL-10 levels was found in pretreated VN. Both IL-17 and IL-8 levels correlated strongly with CSF protein levels in CIDP, although the correlation of IL-6 levels was weak. In CSF CD4+ T cells, IFN-gamma+ IL-4- cell percentages were markedly elevated in CIDP compared with OND, but not in VN, resulting in a significant increase of intracellular IFN-gamma/IL-4 ratio in CIDP, even in the absence of CSF pleocytosis. The nonresponders to intravenous immunoglobulins (IVIGs) showed a significantly lower IFN-gamma- IL-4+ CD4+ T cell percentage, and tended to have a higher intracellular IFN-gamma/IL-4 ratio than the responders in CSF. Marked upregulation of Th1 cytokine, IL-17, and downregulation of Th2 cytokines, together with infiltration of IFN-gamma-producing CD4+ T cells are useful markers for CIDP, while several Th2 cytokines are upregulated in VN in CSF.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Células Th1/metabolismo , Células Th2/metabolismo , Vasculite do Sistema Nervoso Central/líquido cefalorraquidiano , Adulto , Idoso , Idoso de 80 Anos ou mais , Linfócitos T CD4-Positivos/metabolismo , Quimiocinas/líquido cefalorraquidiano , Citocinas/líquido cefalorraquidiano , Espaço Extracelular/metabolismo , Feminino , Citometria de Fluxo/métodos , Humanos , Imunoensaio/métodos , Modelos Lineares , Masculino , Pessoa de Meia-Idade , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/patologia , Vasculite do Sistema Nervoso Central/patologia
18.
Neurology ; 60(8 Suppl 3): S8-15, 2003 Apr 01.
Artigo em Inglês | MEDLINE | ID: mdl-12707417

RESUMO

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is one of several chronic acquired demyelinating neuropathies that are considered to be autoimmune diseases. The prevalence of these illnesses may be underestimated because of limitations in clinical, serologic, and electrophysiologic diagnostic criteria. An Ad Hoc Subcommittee of the American Academy of Neurology (AAN) proposed a set of diagnostic criteria for CIDP to be used for research purposes, and several other criteria followed. Of these, the AAN electrophysiologic criteria are the most restrictive and fit only a subset of patients with CIDP. Other criteria, including the Inflammatory Neuropathy Cause and Treatment (INCAT) clinical and electrophysiologic criteria and the Nicolas or Thaisetthawatkul electrophysiologic criteria, are more sensitive and can therefore identify a broader range of patients with CIDP for clinical trials. Regardless of which criteria are chosen for use in clinical trials, patients who fall outside of these criteria may also have CIDP and may benefit from treatment. Unfortunately, because of the lack of clarity with regard to diagnostic criteria for CIDP, many patients remain untreated. In addition, certain CIDP variants may also respond to treatment. These include sensory CIDP, multifocal motor neuropathy (MMN) with or without conduction block, multifocal acquired demyelinating sensory and motor (MADSAM) neuropathy, distal acquired demyelinating sensory (DADS) neuropathy, and multifocal acquired sensory and motor (MASAM) neuropathy. Therefore, although patients may not meet the diagnostic criteria for inclusion in clinical trials of CIDP, they may still benefit from current and future treatments used in CIDP.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico , Biópsia , Proteínas do Líquido Cefalorraquidiano/análise , Ensaios Clínicos como Assunto/normas , Doenças Desmielinizantes/diagnóstico , Neuropatias Diabéticas/diagnóstico , Diagnóstico Diferencial , Eletrodiagnóstico/normas , Humanos , Condução Nervosa , Seleção de Pacientes , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/classificação , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Pesquisa/normas , Transtornos de Sensação/diagnóstico , Nervo Sural/patologia
19.
J Leukoc Biol ; 73(5): 584-90, 2003 May.
Artigo em Inglês | MEDLINE | ID: mdl-12714572

RESUMO

The recruitment of lymphocytes across the blood brain barrier (BBB) is mediated by adhesion molecules and chemokines. The expression of activation markers and of chemokine receptors on T cells homing to the nervous system (NS) may help define their functional state. In the cerebrospinal fluid (CSF) of subjects with inflammatory neurological diseases (IND), including multiple sclerosis, we observed an increased number of T cells coexpressing CXCR3 and CCR5 as well as T cells with a CD45RO+ CCR7+ CD27+ memory phenotype. A subset of CCR7+ T cells coexpressed CXCR3 and CCR5. We also detected an increased number of interferon-gamma-producing T cells in the CSF compared with peripheral blood, mostly but not exclusively in the CD45RO+ CCR7- CD27- compartment. T helper 1 (Th1) clones, established from the CSF of individuals with IND and from a healthy subject, similarly migrated to CXCL10, CXCL12, and CCL5. CXCL10, CXCL12, and CCL19 were increased in the CSF of individuals with neuroinflammation. These findings suggest that CSF is enriched in Th1-polarized memory T cells capable of differentiating into effector cells upon antigen encounter. These cells are recruited into the CSF by inducible chemokines. Thus, CSF represents a transitional station for T cells trafficking to and from the NS.


Assuntos
Esclerose Múltipla/líquido cefalorraquidiano , Subpopulações de Linfócitos T/imunologia , Adulto , Idoso , Idoso de 80 Anos ou mais , Diferenciação Celular , Movimento Celular , Quimiocina CCL19 , Quimiocina CXCL10 , Quimiocina CXCL12 , Quimiocinas CC/biossíntese , Quimiocinas CC/genética , Quimiocinas CXC/biossíntese , Quimiocinas CXC/genética , Quimiocinas CXC/farmacologia , Quimiotaxia de Leucócito/efeitos dos fármacos , Encefalite/líquido cefalorraquidiano , Encefalite/imunologia , Feminino , Humanos , Memória Imunológica/imunologia , Imunofenotipagem , Interferon gama/metabolismo , Antígenos Comuns de Leucócito/análise , Neuroborreliose de Lyme/líquido cefalorraquidiano , Neuroborreliose de Lyme/imunologia , Ativação Linfocitária , Masculino , Meningite/líquido cefalorraquidiano , Meningite/imunologia , Pessoa de Meia-Idade , Esclerose Múltipla/imunologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/imunologia , Receptores CCR5/análise , Receptores CCR7 , Receptores CXCR3 , Receptores de Quimiocinas/análise , Células Th1/imunologia , Membro 7 da Superfamília de Receptores de Fatores de Necrose Tumoral/análise
20.
Neurology ; 55(12): 1828-32, 2000 Dec 26.
Artigo em Inglês | MEDLINE | ID: mdl-11134381

RESUMO

BACKGROUND: In CSF, proteolytic enzymes are believed to have crucial roles in the initiation and progression of inflammatory neurologic diseases (IND). Cystatin C, a major cysteine protease inhibitor in CSF, is tightly bound to cathepsin B and H. OBJECTIVE: To determine if cystatin C is involved in the disease process of IND, the authors measured the cystatin C concentration by ELISA method and cathepsin B and H activities in the CSF of patients with acute IND. METHODS: Cystatin C concentration and cathepsin B and H activities were measured in CSF samples taken from patients during the acute phase of their disease. Subjects studied were 8 patients with Guillain-Barré syndrome (GBS), 5 with chronic inflammatory demyelinating polyneuropathy (CIDP), 12 with MS, 16 with aseptic meningitis, 15 with neurodegenerative diseases as disease controls, and 35 healthy controls. RESULTS: A significant decrease in CSF cystatin C level was seen in the patients with GBS, CIDP, and MS compared to the control subjects. High cathepsin B activity, but not cathepsin H activity, was also observed in the patients with GBS, CIDP, and MS. CONCLUSION: Cystatin C levels in CSF measured by ELISA may help the physician recognize GBS, CIDP, and MS. Decreased levels of cystatin C may be related to the high levels of cathepsin B activity seen in the CSF of patients with GBS, CIDP, and MS.


Assuntos
Catepsina B/líquido cefalorraquidiano , Cistatinas/líquido cefalorraquidiano , Síndrome de Guillain-Barré/líquido cefalorraquidiano , Distrofias Musculares/líquido cefalorraquidiano , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/líquido cefalorraquidiano , Adulto , Idoso , Idoso de 80 Anos ou mais , Cistatina C , Ensaio de Imunoadsorção Enzimática , Feminino , Humanos , Interleucina-6/líquido cefalorraquidiano , Contagem de Leucócitos , Masculino , Pessoa de Meia-Idade
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