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1.
Eur J Neurol ; 28(7): 2327-2338, 2021 07.
Artigo em Inglês | MEDLINE | ID: mdl-33909329

RESUMO

BACKGROUND AND OBJECTIVE: Nerve ultrasound is a promising new tool in chronic inflammatory neuropathies. The aim of this study was to determine its prognostic value in a prospective multicenter cohort study including incident and prevalent patients with CIDP and MMN. METHODS: We enrolled 126 patients with CIDP, and 72 with MMN; 71 were treatment-naive. Patients with chronic idiopathic axonal polyneuropathy (CIAP; n = 35) were considered as disease controls. Standardized neurological examination, questionnaires, and nerve ultrasonography were obtained at time of inclusion and 1-year follow-up. Nerve size development over time and correlation between nerve size and clinical outcome measures were determined using linear mixed effects models. RESULTS: Nerve size development over time was heterogeneous. Only in MMN was there a correlation between C5 nerve root size and deterioration of grip strength (-1.3 kPa/mm2 (95% confidence interval [CI] -2.3 to -0.2). No other significant correlations between nerve size and clinical outcome measures were found. In MMN, presence of nerve enlargement at inclusion predicted deterioration of grip strength, and MMN patients with enlargement confined to the brachial plexus seemed to have more favorable outcomes. No other predictive effects of sonographic nerve size were found. CONCLUSIONS: The present study indicates that the natural course of nerve size development in CIDP and MMN is heterogeneous, and that the prognostic value of sonographic nerve enlargement is limited. It had some predictive effect in patients with MMN. Further research in specific subgroups of chronic inflammatory neuropathy is necessary to determine the usefulness of nerve ultrasonography after the diagnostic phase.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica , Estudos de Coortes , Humanos , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico por imagem , Prognóstico , Estudos Prospectivos , Ultrassonografia
2.
Neurobiol Aging ; 101: 79-84, 2021 05.
Artigo em Inglês | MEDLINE | ID: mdl-33582569

RESUMO

OBJECTIVE: To gain further insight in the immunopathology underlying multifocal motor neuropathy (MMN) by exploring the association between MMN and the human leukocyte antigen (HLA) class II DRB1, DQB1, and DQA loci in depth and by correlating associated haplotypes to detailed clinical and anti-ganglioside antibody data. METHODS: We performed high-resolution HLA-class II typing for the DRB1, DQB1, and DQA1 loci in 126 well-characterized MMN patients and assessed disease associations with haplotypes. We used a cohort of 1305 random individuals as a reference for haplotype distribution in the Dutch population. RESULTS: The DRB1*15:01-DQB1*06:02 haplotype (OR 1.6 [95% CI 1.1-2.2], p < 0.05) and the DRB1*12:01-DQB1*03:01 haplotype (OR 2.7 [95% CI 1.2-5.5], p < 0.05) were more frequent in patients with MMN than in controls. These haplotypes were not associated with disease course, response to treatment or anti-ganglioside antibodies. CONCLUSIONS: MMN is associated with the DRB1*15:01-DQB1*06:02 and DRB1*12:01-DQB1*03:01 haplotypes. These HLA molecules or gene variants in their immediate vicinity may promote the specific inflammatory processes underlying MMN.


Assuntos
Estudos de Associação Genética/métodos , Predisposição Genética para Doença/genética , Cadeias alfa de HLA-DQ/genética , Cadeias beta de HLA-DQ/genética , Cadeias beta de HLA-DR/genética , Teste de Histocompatibilidade/métodos , Polineuropatias/genética , Adulto , Estudos de Coortes , Feminino , Gangliosídeos/imunologia , Loci Gênicos , Haplótipos , Humanos , Masculino , Pessoa de Meia-Idade , Países Baixos
3.
Neurology ; 95(12): e1745-e1753, 2020 09 22.
Artigo em Inglês | MEDLINE | ID: mdl-32675082

RESUMO

OBJECTIVE: To validate the diagnostic accuracy of a previously described short sonographic protocol to identify chronic inflammatory neuropathy (CIN), including chronic inflammatory demyelinating polyneuropathy (CIDP), Lewis Sumner syndrome, and multifocal motor neuropathy (MMN), and to determine the added value of nerve ultrasound to detect treatment-responsive patients compared to nerve conduction studies (NCS) in a prospective multicenter study. METHODS: We included 100 consecutive patients clinically suspected of CIN in 3 centers. The study protocol consisted of neurologic examination, laboratory tests, NCS, and nerve ultrasound. We validated a short sonographic protocol (median nerve at forearm, upper arm, and C5 nerve root) and determined its diagnostic accuracy using the European Federation of Neurological Societies/Peripheral Nerve Society criteria of CIDP/MMN (reference standard). In addition, to determine the added value of nerve ultrasound in detecting treatment-responsive patients, we used previously published diagnostic criteria based on clinical, NCS, and sonographic findings and treatment response (alternative reference standard). RESULTS: Sensitivity and specificity of the sonographic protocol for CIN according to the reference standard were 87.4% and 67.3%, respectively. Sensitivity and specificity of this protocol according to the alternative reference standard were 84.6% and 72.8%, respectively, and of NCS 76.1% and 93.4%. With addition of nerve ultrasound, 44 diagnoses of CIN were established compared to 33 diagnoses with NCS alone. CONCLUSIONS: A short sonographic protocol shows high diagnostic accuracy for detecting CIN. Nerve ultrasound is able to detect up to 25% more patients who respond to treatment. CLASSIFICATION OF EVIDENCE: This multicenter study provides Class IV evidence that nerve ultrasound improves diagnosis of CIN.


Assuntos
Polirradiculoneuropatia/diagnóstico por imagem , Ultrassonografia/métodos , Adulto , Idoso , Estudos de Coortes , Eletromiografia/métodos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Condução Nervosa/fisiologia , Estudos Prospectivos , Sensibilidade e Especificidade
4.
Neurology ; 94(14): e1470-e1479, 2020 04 07.
Artigo em Inglês | MEDLINE | ID: mdl-31959710

RESUMO

OBJECTIVE: To examine the diagnostic accuracy of nerve ultrasound in a prospective cohort of consecutive patients with a clinical suspicion of chronic inflammatory neuropathies, including chronic inflammatory demyelinating polyneuropathy, Lewis-Sumner syndrome, and multifocal motor neuropathy, and to determine the added value in the detection of treatment-responsive patients. METHODS: Between February 2015 and July 2018, we included 100 consecutive incident patients with a clinical suspicion of chronic inflammatory neuropathy. All patients underwent nerve ultrasound, extensive standardized nerve conduction studies (NCS), and other relevant diagnostic investigations. We evaluated treatment response using predefined criteria. A diagnosis of chronic inflammatory neuropathy was established when NCS were abnormal (fulfilling criteria of demyelination of the European Federation of Neurological Societies/Peripheral Nerve Society) or when the degree of nerve enlargement detected by sonography was compatible with chronic inflammatory neuropathy and there was response to treatment. RESULTS: A diagnosis of chronic inflammatory neuropathy was established in 38 patients. Sensitivity and specificity of nerve ultrasound and NCS were 97.4% and 69.4% and 78.9% and 93.5%, respectively. The added value of nerve ultrasound in detection of treatment-responsive chronic inflammatory neuropathy was 21.1% compared to NCS alone. CONCLUSIONS: Nerve ultrasound and NCS are complementary techniques with superior sensitivity in the former and specificity in the latter. Addition of nerve ultrasound significantly improves the detection of chronic inflammatory neuropathies. Therefore, it deserves a prominent place in the diagnostic workup of chronic inflammatory neuropathies. CLASSIFICATION OF EVIDENCE: This study provides Class IV evidence that nerve ultrasound is an accurate diagnostic tool to detect chronic inflammatory neuropathies.


Assuntos
Neurite (Inflamação)/diagnóstico por imagem , Neurite (Inflamação)/tratamento farmacológico , Ultrassonografia/métodos , Adulto , Idoso , Idoso de 80 Anos ou mais , Doença Crônica , Estudos de Coortes , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Doença dos Neurônios Motores/diagnóstico por imagem , Doença dos Neurônios Motores/tratamento farmacológico , Condução Nervosa , Exame Neurológico , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico por imagem , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/tratamento farmacológico , Estudos Prospectivos , Sensibilidade e Especificidade , Resultado do Tratamento
5.
J Neurol ; 266(11): 2734-2742, 2019 Nov.
Artigo em Inglês | MEDLINE | ID: mdl-31325017

RESUMO

OBJECTIVE: The primary aim was to determine the safety of treatment with human immune globulin 10% with recombinant human hyaluronidase (fSCIg) compared to intravenous immunoglobulin (IVIg) in a prospective open-label study in patients with multifocal motor neuropathy (MMN). METHODS: Our study consisted of two phases: the IVIg phase (visits 1-3; 12 weeks), in which patients remained on IVIg treatment, and the fSCIg phase (visits 4-7; 36 weeks), in which patients received fSCIg treatment. After visit 3, IVIg was switched to an equivalent dose and frequency of fSCIg. Outcome measures were safety, muscle strength, disability and treatment satisfaction. RESULTS: Eighteen patients were enrolled in this study. Switching to fSCIg reduced the number of systemic adverse events (IVIg 11.6 vs. fSCIg 5.0 adverse events/per person-year, p < 0.02), and increased the number of local reactions at the injection site (IVIg 0 vs. fSCIg 3.3 local reactions/per person-year, p < 0.01). Overall, no significant differences in muscle strength and disability between fSCIg and IVIg were found. Treatment with fSCIg was perceived as optimal treatment option by 8 of the 17 patients (47.1%) and they continued with fSCIg after study closure because of improved independence and flexibility to administer treatment. CONCLUSION: Treatment with fSCIg can be considered a safe alternative for patients with MMN on IVIg treatment. fSCIg could be a favorable option in patients who prefer self-treatment and more independency, and in patients who experience systemic adverse events on IVIg or have difficult intravenous access.


Assuntos
Hialuronoglucosaminidase/uso terapêutico , Imunoglobulinas Intravenosas/uso terapêutico , Polirradiculoneuropatia/tratamento farmacológico , Adulto , Idoso , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Força Muscular/efeitos dos fármacos , Polineuropatias/tratamento farmacológico , Proteínas Recombinantes/uso terapêutico , Resultado do Tratamento
6.
Muscle Nerve ; 60(4): 415-419, 2019 10.
Artigo em Inglês | MEDLINE | ID: mdl-31294858

RESUMO

INTRODUCTION: We present a case series of six treatment-naive patients with clinical phenotypes compatible with chronic inflammatory demyelinating polyneuropathy and multifocal motor neuropathy without electrodiagnostic features of demyelination but with abnormal peripheral ultrasound findings who responded to treatment. METHODS: All six patients underwent a complete set of ancillary investigations, including extensive nerve conduction studies. We also performed standardized nerve ultrasound of median nerves and brachial plexus as part of a larger effort to evaluate diagnostic value of sonography. RESULTS: Nerve conduction studies did not show conduction block or other signs of demyelination in any of the six patients. Sonographic nerve enlargement was present in all patients and was most prominent in proximal segments of the median nerve and brachial plexus. Treatment with intravenous immunoglobulin resulted in objective clinical improvement. DISCUSSION: Our study provides evidence that nerve ultrasound represents a useful complementary diagnostic tool for the identification of treatment-responsive inflammatory neuropathies.


Assuntos
Plexo Braquial/diagnóstico por imagem , Nervo Mediano/diagnóstico por imagem , Condução Nervosa/fisiologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/diagnóstico por imagem , Idoso , Plexo Braquial/patologia , Plexo Braquial/fisiopatologia , Eletrodiagnóstico , Feminino , Humanos , Imunoglobulinas Intravenosas/uso terapêutico , Fatores Imunológicos/uso terapêutico , Masculino , Nervo Mediano/patologia , Nervo Mediano/fisiopatologia , Pessoa de Meia-Idade , Tamanho do Órgão , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Ultrassonografia
7.
Neurology ; 2018 Dec 28.
Artigo em Inglês | MEDLINE | ID: mdl-30593519

RESUMO

OBJECTIVE: To determine interobserver variability of nerve ultrasound in peripheral neuropathy in a prospective, systematic, multicenter study. METHODS: We enrolled 20 patients with an acquired chronic demyelinating or axonal polyneuropathy and 10 healthy controls in 3 different centers. All participants underwent an extensive nerve ultrasound protocol, including cross-sectional area measurements of median, ulnar, fibular, tibial, and sural nerves, and brachial plexus. Real-time image acquisition was performed blind by a local and a visiting investigator (reference). Five patients were investigated using different types of sonographic devices. Intraclass correlation coefficients were calculated, and a random-effects model was fitted to identify factors with significant effect on interobserver variability. RESULTS: Systematic differences between measurements made by different investigators were small (mean difference 0.11 mm2 [95% confidence interval 0.00-0.23 mm2]). Intraclass correlation coefficients were generally higher in arm nerves (0.48-0.96) than leg nerves (0.46-0.61). The hospital site and sonographic device did not contribute significantly to interobserver variability in the random-effects model. CONCLUSIONS: Interobserver variability of nerve ultrasound in peripheral neuropathy is generally limited, especially in arm nerves. Different devices and a multicenter setting have no effect on interobserver variability. Therefore, nerve ultrasound is a reproducible tool for diagnostics in routine clinical practice and (multicenter) research.

8.
Clin Neurophysiol ; 129(1): 232-237, 2018 01.
Artigo em Inglês | MEDLINE | ID: mdl-29202391

RESUMO

OBJECTIVE: Wartenberg's migrant sensory neuritis (WMSN) is a rare, patchy, pure sensory neuropathy of unknown etiology. High-resolution ultrasonography (HRUS) is an emerging diagnostic technique for neuropathies, but it has not been applied in WMSN. In this study we aimed to determine HRUS abnormalities in WMSN. METHODS: We performed a case-control study of 8 newly diagnosed patients with WMSN and 22 treatment-naive disease controls (16 patients with pure sensory axonal neuropathy and 6 with pure sensory chronic inflammatory demyelinating polyneuropathy (CIDP) or Lewis-Sumner syndrome (LSS)). All patients underwent routine diagnostic evaluations and a predefined HRUS protocol. RESULTS: We found multifocal nerve enlargement in all 8 WMSN patients. The median nerve in the upper arm and the sural nerve were significantly larger in WMSN than in axonal controls (p = 0.01 and p = 0.04). In CIDP/LSS, sonographic enlargement was more extensive. Furthermore we found brachial plexus involvement in 3 of 8 (38%) WMSN patients. CONCLUSION: HRUS showed enlargement of multiple nerves in all WMSN patients even if clinical testing and NCS were normal. SIGNIFICANCE: The feature of multifocal nerve enlargement may be of additional value in establishing the diagnosis of WMSN and may support the suggestion of an auto-immune etiology.


Assuntos
Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/diagnóstico por imagem , Neurite (Inflamação)/diagnóstico por imagem , Ultrassonografia/métodos , Adulto , Axônios/patologia , Plexo Braquial/diagnóstico por imagem , Plexo Braquial/patologia , Estudos de Casos e Controles , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/etiologia , Doenças Autoimunes Desmielinizantes do Sistema Nervoso Central/patologia , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Neurite (Inflamação)/etiologia , Neurite (Inflamação)/patologia , Valor Preditivo dos Testes , Ultrassonografia/normas
9.
Seizure ; 23(6): 468-74, 2014 Jun.
Artigo em Inglês | MEDLINE | ID: mdl-24768269

RESUMO

PURPOSE: We examined whether early EEG changes in a 24-h EEG at 6 weeks of treatment were related to the later clinical response to the ketogenic diet (KD) in a 6-month period of treatment. METHODS: We examined 34 patients with heterogeneous epilepsy syndromes (21 children, 13 adults) and found 9 clinical responders (≥50% seizure reduction); this is a responder rate of 26%. We visually counted the interictal epileptic discharge index (IED index) in % during 2h of wakefulness and in the first hour of sleep (method 1), and also globally reviewed EEG changes (method 2), while blinded to the effect of the KD. RESULTS: At group level we saw a correlation between nocturnal reduction of IED-index at 6 weeks and seizure reduction in the follow-up period. A proportional reduction in IED index of 30% from baseline in the sleep EEG, was associated with being a responder to the diet (Pearson Chi-square p=0.04). EEG scoring method 2 observed a significantly larger proportion of patients with EEG-improvement in sleep in KD responders than in non-responders (p=0.03). At individual level, however, EEG changes did not correlate very strongly to the response to the diet, as IED reduction in sleep was also seen in 15% (method 1) to 26% (method 2) of the non-responders. CONCLUSION: Nocturnal reduction of IEDs is related to the response to the KD, however in daily clinical practice, an early EEG to predict seizure reduction should not be advised for individual patients.


Assuntos
Encéfalo/fisiopatologia , Dieta Cetogênica , Eletroencefalografia/métodos , Epilepsia/dietoterapia , Epilepsia/fisiopatologia , Adolescente , Adulto , Anticonvulsivantes/uso terapêutico , Criança , Pré-Escolar , Epilepsia/diagnóstico , Epilepsia/tratamento farmacológico , Feminino , Seguimentos , Humanos , Lactente , Masculino , Pessoa de Meia-Idade , Prognóstico , Estudos Prospectivos , Convulsões/diagnóstico , Convulsões/dietoterapia , Convulsões/tratamento farmacológico , Convulsões/fisiopatologia , Sono/fisiologia , Resultado do Tratamento , Vigília/fisiologia , Adulto Jovem
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