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1.
Eur J Neurol ; 27(3): 522-528, 2020 03.
Artigo em Inglês | MEDLINE | ID: mdl-31574194

RESUMO

BACKGROUND AND PURPOSE: Whether the Lewis-Sumner syndrome (L-SS) is a distinct entity from other types of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP-ot) remains controversial. METHOD: The clinical/electrophysiological characteristics and long-term outcomes of 45 L-SS and 35 CIDP-ot patients were retrospectively compared. RESULTS: The CIDP-ot group was composed of 11 patients with a typical CIDP, 17 with a pure sensory form, four with a distal form and three with a pure motor form. In the L-SS group, asymmetric (P < 0.001) and monomelic involvement (P = 0.04) of the upper limbs (P < 0.001) was significantly more frequent; paucisymptomatic forms (Overall Neuropathy Limitations Scale ≤ 1) were less frequent (P < 0.001); electroneuromyography showed that conduction block in intermediate nerve segments was the main demyelinating feature, with frequent F-wave abnormalities on nerves without conduction block (44%). Long-term prognosis was globally poorer in the L-SS group with more frequent aggravation during treatment (P = 0.02), less frequent treatment withdrawal (P = 0.03) and longer time to achieve successful withdrawal (39 vs. 15 months). CONCLUSIONS: Our study suggests that L-SS patients have a less favourable therapeutic response rate and long-term outcomes. Rapid differentiation of L-SS from other forms of CIDP is important in order to anticipate a more complicated disease course management, with from one side the inefficacy or even harmfulness of corticosteroids and from the other side a difficult weaning procedure. A prospective study is necessary to confirm these results.


Assuntos
Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/fisiopatologia , Polirradiculoneuropatia Desmielinizante Inflamatória Crônica/terapia , Adulto , Idoso , Doenças Desmielinizantes/patologia , Avaliação da Deficiência , Eletrodiagnóstico , Fenômenos Eletrofisiológicos , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Condução Nervosa , Prognóstico , Estudos Retrospectivos , Síndrome , Centros de Atenção Terciária , Resultado do Tratamento
2.
Rev Neurol (Paris) ; 176(10): 870-873, 2020 Dec.
Artigo em Inglês | MEDLINE | ID: mdl-32653215

RESUMO

Fifteen ALS patients, with troublesome symptoms linked to masseter spasticity, benefited from BoNT-A injections in each masseter. Based on the medical records of patients, the effect of the first injection was assessed one month later. We retrospectively collected information for 12 patients. Eight of them reported a beneficial effect after the injection for the following symptoms: trismus, tongue, lip and cheek biting, and jaw clonus. Five patients indicated that dental care was easier after injection. Our study showed that injections of BoNT-A unequivocally reduced masseter spasticity in ALS patients who subsequently enjoyed greater comfort in their daily living.


Assuntos
Esclerose Lateral Amiotrófica , Toxinas Botulínicas Tipo A/uso terapêutico , Humanos , Injeções Intramusculares , Espasticidade Muscular , Estudos Retrospectivos
3.
Acta Oncol ; 57(3): 403-411, 2018 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-29243538

RESUMO

BACKGROUND: Charcot Marie Tooth (CMT) disease is the most common form of hereditary neuropathy. Due to the high prevalence of mild and undiagnosed forms, patients with CMT disease may be exposed to severe neurotoxicity following the administration of neurotoxic chemotherapies. The aim of this report is to alert oncologists to the potential to precipitate severe irreversible peripheral neuropathies when administering neurotoxic compounds to undiagnosed CMT patients. MATERIAL AND METHODS: A retrospective research in the OncoNeuroTox database was performed (2010-2016), searching for patients with the diagnosis of chemotherapy-induced peripheral neuropathy (CIPN) and CMT disease. A comprehensive literature review for previously published cases was performed using the Pubmed and Cochrane databases (1972-2017). RESULTS: Among 428 patients with CIPN, we identified eight patients with concomitant CMT disease. Seven patients out of the eight had no previous diagnosis of CMT disease, although accurate familial history disclosed mild signs of peripheral neuropathy in five cases. Patients themselves had minor stigmata of long-standing peripheral damage. Patients received chemotherapy regimens based on vinca alkaloids, taxanes or a combination of vinca alkaloids and platinum compounds. In two cases, cumulative doses were below or equal to the expected neurotoxic threshold. Following chemotherapy administration, patients developed severe length-dependent sensory-motor deficits. Despite early drug discontinuation, most patients remained severely disabled. CONCLUSION: A brief checklist to disclose long-standing signs of peripheral neuropathy could be helpful to detect patients with undiagnosed hereditary neuropathies who could be at risk of developing severe irreversible neurotoxicity following the administration of neurotoxic agents.


Assuntos
Antineoplásicos/efeitos adversos , Doença de Charcot-Marie-Tooth/complicações , Neoplasias/complicações , Neoplasias/tratamento farmacológico , Adulto , Idoso de 80 Anos ou mais , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Estudos Retrospectivos , Adulto Jovem
4.
Eur J Neurol ; 24(8): 1040-1046, 2017 08.
Artigo em Inglês | MEDLINE | ID: mdl-28586096

RESUMO

BACKGROUND AND PURPOSE: Assessing survival is a critical issue in patients with amyotrophic lateral sclerosis (ALS). Neuroimaging seems to be promising in the assessment of disease severity and several studies also suggest a strong relationship between spinal cord (SC) atrophy described by magnetic resonance imaging (MRI) and disease progression. The aim of the study was to determine the predictive added value of multimodal SC MRI on survival. METHODS: Forty-nine ALS patients were recruited and clinical data were collected. Patients were scored on the Revised ALS Functional Rating Scale and manual muscle testing. They were followed longitudinally to assess survival. The cervical SC was imaged using the 3 T MRI system. Cord volume and cross-sectional area (CSA) at each vertebral level were computed. Diffusion tensor imaging metrics were measured. Imaging metrics and clinical variables were used as inputs for a multivariate Cox regression survival model. RESULTS: On building a multivariate Cox regression model with clinical and MRI parameters, fractional anisotropy, magnetization transfer ratio and CSA at C2-C3, C4-C5, C5-C6 and C6-C7 vertebral levels were significant. Moreover, the hazard ratio calculated for CSA at the C3-C4 and C5-C6 levels indicated an increased risk for patients with SC atrophy (respectively 0.66 and 0.68). In our cohort, MRI parameters seem to be more predictive than clinical variables, which had a hazard ratio very close to 1. CONCLUSIONS: It is suggested that multimodal SC MRI could be a useful tool in survival prediction especially if used at the beginning of the disease and when combined with clinical variables. To validate it as a biomarker, confirmation of the results in bigger independent cohorts of patients is warranted.


Assuntos
Esclerose Lateral Amiotrófica/diagnóstico por imagem , Imageamento por Ressonância Magnética/métodos , Medula Espinal/diagnóstico por imagem , Adulto , Idoso , Esclerose Lateral Amiotrófica/mortalidade , Esclerose Lateral Amiotrófica/patologia , Anisotropia , Estudos Transversais , Imagem de Tensor de Difusão/métodos , Progressão da Doença , Feminino , Humanos , Masculino , Pessoa de Meia-Idade , Imagem Multimodal , Prognóstico , Medula Espinal/patologia , Taxa de Sobrevida
5.
Rev Neurol (Paris) ; 173(5): 280-287, 2017 May.
Artigo em Inglês | MEDLINE | ID: mdl-28461025

RESUMO

Amyotrophic lateral sclerosis (ALS) is a degenerative motor neuron disease (MND) which prognosis is poor. Early diagnosis permit to set up immediately adapted treatment and cares. Available diagnostic criteria are based on the detection of both central and peripheral motor neuron injury in bulbar, cervical, thoracic and lumbar regions. Electrodiagnostic (EDX) tests are the key tools to identify peripheral motor neuron involvement. Needle examination records abnormal activities at rest, and looks for neurogenic pattern during muscle contraction. Motor unit potentials morphology is modified primary to recruitment. Motor evoked potentials remain the test of choice to identify impairment of central motor neurons. In the absence of diagnostic biomarker of ALS and among essential investigations of suspected MND, a careful clinical and neurophysiological work-up is essential to rule out the differential diagnosis.


Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Doença dos Neurônios Motores/diagnóstico , Diagnóstico Diferencial , Eletrodiagnóstico , Potencial Evocado Motor , Humanos , Prognóstico
6.
Rev Neurol (Paris) ; 172(6-7): 384-8, 2016.
Artigo em Inglês | MEDLINE | ID: mdl-27371132

RESUMO

Posterior reversible encephalopathy syndrome (PRES) is a serious neurological condition encountered in various medical fields. Pathophysiological factor(s) common to PRES cases of apparently unrelated etiologies are yet to be found. Based on the hypothesis that hypomagnesemia might participate in the cascade leading to PRES, our study sought to verify whether hypomagnesemia is frequently associated with PRES regardless of etiology. From a retrospective study of a cohort of 57 patients presenting with PRES of different etiologies, presented here are the findings of 19 patients with available serum magnesium levels (SMLs) during PRES. In the acute phase of PRES, hypomagnesemia was present in all 19 patients in spite of differences in etiology (including immunosuppressive drugs, hypertensive encephalopathy, eclampsia, systemic lupus erythematosus, iatrogenic etiology and unknown). SMLs were within normal ranges prior to PRES and below normal ranges during the first 48h of PRES, with a significant decrease in SMLs during the acute phase. In this retrospective study, constant hypomagnesemia was observed during the acute phase of PRES regardless of its etiology. These results now require larger studies to assess the particular importance of acute hypomagnesemia in PRES and especially the possible need to treat PRES with magnesium sulfate.


Assuntos
Hipercalciúria/epidemiologia , Magnésio/sangue , Nefrocalcinose/epidemiologia , Síndrome da Leucoencefalopatia Posterior/sangue , Síndrome da Leucoencefalopatia Posterior/epidemiologia , Erros Inatos do Transporte Tubular Renal/epidemiologia , Adulto , Criança , Comorbidade , Feminino , Humanos , Hipercalciúria/sangue , Hipercalciúria/complicações , Masculino , Pessoa de Meia-Idade , Nefrocalcinose/sangue , Nefrocalcinose/complicações , Síndrome da Leucoencefalopatia Posterior/complicações , Prevalência , Erros Inatos do Transporte Tubular Renal/sangue , Erros Inatos do Transporte Tubular Renal/complicações , Estudos Retrospectivos , Adulto Jovem
7.
Eur J Neurol ; 21(9): 1233-41, 2014 Sep.
Artigo em Inglês | MEDLINE | ID: mdl-24847978

RESUMO

BACKGROUND AND PURPOSE: The 'snake eyes' sign refers to bilateral hyperintensities of the anterior horns on axial spinal cord imaging. Based on sporadic reports, it has been associated with a range of lower motor neuron (LMN) syndromes, such as spondylotic amyotrophy and Hirayama disease, as well as spinal cord infarction. The objective of our study was to comprehensively characterize the full diagnostic spectrum of LMN syndromes with this radiological clue and discuss potential aetiological factors. METHODS: A large patient cohort with snake eyes sign and upper limb LMN degeneration was recruited from three French neuromuscular units. Patients underwent detailed electrophysiological, radiological, clinical and anamnestic profiling. RESULTS: Twenty-nine patients were ascertained and followed up for 9.5 ± 8.6 years. The majority of the patients were male (86.2%) with a mean age of 37.3 ± 14.4 years. Symptoms were bilateral in most cases (86.2%). Patients with predominantly proximal and distal deficits were equally represented (44.8% and 55.2%, respectively). A history of preceding trauma or intense physical activity was confirmed in 58.6% of the cases; 27.6% of the patients were given an initial clinical diagnosis of amyotrophic lateral sclerosis (ALS), and 51.7% were originally suspected to have multifocal motor neuropathy. None of the patients developed ALS on longitudinal follow-up. CONCLUSION: The snake eyes sign on magnetic resonance imaging is associated with a wide spectrum of neurological conditions and is more common in young men with a history of strenuous activity or antecedent trauma. The recognition of this syndrome is crucial as many of these patients are initially misdiagnosed with ALS.


Assuntos
Células do Corno Anterior/patologia , Doença dos Neurônios Motores/patologia , Medula Espinal/patologia , Adolescente , Adulto , Idoso , Estudos de Coortes , Eletromiografia , Feminino , França , Humanos , Imageamento por Ressonância Magnética , Masculino , Pessoa de Meia-Idade , Fatores de Risco , Estatísticas não Paramétricas , Adulto Jovem
8.
Eur J Neurol ; 21(3): 529-36, 2014 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-24447620

RESUMO

BACKGROUND AND PURPOSE: To assess the efficacy and safety of olesoxime, a molecule with neuroprotective properties, in patients with amyotrophic lateral sclerosis (ALS) treated with riluzole. METHODS: A double-blind, randomized, placebo-controlled, multicenter trial of 18 months' duration was conducted in 512 subjects, with probable or definite ALS and a slow vital capacity (SVC) ≥70%, receiving 330 mg olesoxime daily or matching placebo and 50 mg riluzole twice a day in all. The primary intention-to-treat (ITT) outcome analysis was 18 months' survival. Secondary outcomes were rates of deterioration of the revised ALS functional rating scale (ALSFRS-R), focusing on the 9-month assessment, SVC and manual muscle testing. Blood levels, safety and tolerability of olesoxime were also assessed. RESULTS: At 18 months, 154 of the 512 ITT patients had died (79 of 253 placebo, 75 of 259 olesoxime). Estimated overall survival according to Kaplan-Meier analysis was 67.5% (95% CI 61.0%-73.1%) in the placebo group and 69.4% (95% CI 63.0%-74.9%) in the olesoxime group; hence survival was not significantly different between treatment arms (P = 0.71, stratified bulbar/spinal log-rank). The other efficacy end-points evaluated were also negative, with the exception of a small difference in ALSFRS-R global score at 9 months in favor of olesoxime but not sustained after 18 months' treatment nor evident in either the stratified bulbar or spinal subpopulations. Treatment did not raise any safety concerns. CONCLUSIONS: Olesoxime, although well tolerated, did not show a significant beneficial effect in ALS patients treated with riluzole.


Assuntos
Esclerose Lateral Amiotrófica/tratamento farmacológico , Colestenonas/uso terapêutico , Fármacos Neuroprotetores/uso terapêutico , Adulto , Idoso , Estudos de Casos e Controles , Método Duplo-Cego , Europa (Continente) , Feminino , Humanos , Cooperação Internacional , Estimativa de Kaplan-Meier , Masculino , Pessoa de Meia-Idade , Resultado do Tratamento
9.
J Neurol ; 271(3): 1235-1246, 2024 Mar.
Artigo em Inglês | MEDLINE | ID: mdl-37910250

RESUMO

BACKGROUND: Respiratory complications resulting from motor neurons degeneration are the primary cause of death in amyotrophic lateral sclerosis (ALS). Predicting the need for non-invasive ventilation (NIV) in ALS is important for advance care planning and clinical trial design. The aim of this study was to assess the potential of quantitative MRI at the brainstem and spinal cord levels to predict the need for NIV during the first six months after diagnosis. METHODS: Forty-one ALS patients underwent MRI and spirometry shortly after diagnosis. The need for NIV was monitored according to French health guidelines for 6 months. The performance of four regression models based on: clinical variables, brainstem structures volumes, cervical spinal measurements, and combined variables were compared to predict the need for NIV within this period. RESULTS: Both the clinical model (R2 = 0.28, AUC = 0.85, AICc = 42.67, BIC = 49.8) and the brainstem structures' volumes model (R2 = 0.30, AUC = 0.85, AICc = 40.13, BIC = 46.99) demonstrated good predictive performance. In addition, cervical spinal cord measurements model similar performance (R2 = 0.338, AUC = 0.87, AICc = 37.99, BIC = 44.49). Notably, the combined model incorporating predictors from all three models yielded the best performance (R2 = 0.60, AUC = 0.959, AICc = 36.38, BIC = 44.8). These findings are supported by observed positive correlations between brainstem volumes, cervical (C4/C7) cross-sectional area, and spirometry-measured lung volumes. CONCLUSIONS: Our study shows that brainstem volumes and spinal cord area are promising measures to predict respiratory intervention needs in ALS.


Assuntos
Esclerose Lateral Amiotrófica , Ventilação não Invasiva , Humanos , Esclerose Lateral Amiotrófica/diagnóstico por imagem , Esclerose Lateral Amiotrófica/terapia , Esclerose Lateral Amiotrófica/complicações , Ventilação não Invasiva/métodos , Progressão da Doença , Imageamento por Ressonância Magnética/métodos , Tronco Encefálico/diagnóstico por imagem
10.
Rev Neurol (Paris) ; 168(12): 939-50, 2012 Dec.
Artigo em Francês | MEDLINE | ID: mdl-22742890

RESUMO

INTRODUCTION: Because of the improvement of cancer prognosis, long-term damages of treatments become a medical and public health problem. Among the iatrogenic complications, neurological impairment is crucial to consider since motor disability and pain have a considerable impact on quality of life of long cancer survivors. However, radiation-induced neuropathies have not been the focus of great attention. The objective of this paper is to provide an updated review about the radiation-induced lesions of the peripheral nerve system. STATE OF THE ART: Radiation-induced neuropathies are characterized by their heterogeneity in both symptoms and disease course. Signs and symptoms depend on the affected structures of the peripheral nerve system (nerve roots, nerve plexus or nerve trunks). Early-onset complications are often transient and late complications are usually progressive and associated with a poor prognosis. The most frequent and well known is delayed radiation-induced brachial plexopathy, which may follow breast cancer irradiation. Radiation-induced lumbosacral radiculoplexopathy is characterized by pure or predominant lower motor neuron signs. They can be misdiagnosed, confused with amyotrophic lateral sclerosis (ALS) or with leptomeningeal metastases since nodular MRI enhancement of the nerve roots of the cauda equina and increased cerebrospinal fluid protein content can be observed. In the absence of specific markers of the link with radiotherapy, the diagnosis of post-radiation neuropathy may be difficult. Recently, a posteriori conformal radiotherapy with 3D dosimetric reconstitution has been developed to link a precise anatomical site to unexpected excess irradiation. PERSPECTIVES AND CONCLUSION: The importance of early diagnosis of radiation-induced neuropathies is underscored by the emergence of new disease-modifying treatments. Although the pathophysiology is not fully understood, it is already possible to target radiation-induced fibrosis but also associated factors such as ischemia, oxidative stress and inflammation. A phase III trial evaluating the association of pentoxifylline, tocopherol and clodronate (PENTOCLO, NCT01291433) in radiation-induced neuropathies is now recruiting.


Assuntos
Neoplasias/radioterapia , Doenças do Sistema Nervoso/etiologia , Lesões por Radiação/etiologia , Radioterapia/efeitos adversos , Humanos , Neoplasias Induzidas por Radiação/epidemiologia , Neoplasias Induzidas por Radiação/terapia , Doenças do Sistema Nervoso/diagnóstico , Doenças do Sistema Nervoso/patologia , Doenças do Sistema Nervoso/fisiopatologia , Doenças do Sistema Nervoso/terapia , Prognóstico , Lesões por Radiação/diagnóstico , Lesões por Radiação/patologia , Lesões por Radiação/fisiopatologia , Lesões por Radiação/terapia , Fatores de Risco
13.
Sci Rep ; 9(1): 2918, 2019 02 27.
Artigo em Inglês | MEDLINE | ID: mdl-30814647

RESUMO

Accurate patient stratification into prognostic categories and targeting Amyotrophic Lateral Sclerosis (ALS)-associated pathways may pave the way for promising trials. We evaluated blood-based prognostic indicators using an array of pathological markers. Plasma samples were collected as part of a large, phase III clinical trial (Mitotarget/TRO19622) at months 1, 6, 12 and 18. The ALSFRS-r score was used as a proxy of disease progression to assess the predictive value of candidate biological indicators. First, established clinical predictors were evaluated in all 512 patients. Subsequently, pathologic markers, such as proxies of neuronal integrity (Neurofilament light chain and phosphorylated heavy chain), DNA oxidation (8-oxo-2'-desoxyguanosine), lipid peroxidation (4-hydroxy-2-nonenal, isoprostane), inflammation (interleukin-6) and iron status (ferritin, hepcidin, transferrin) were assessed in a subset of 109 patients that represented the whole cohort. Markers of neuronal integrity, DNA and lipid oxidation, as well as iron status at baseline are accurate predictors of disability at 18-month follow-up. The composite scores of these markers in association with established clinical predictors enable the accurate forecasting of functional decline. The identified four biomarkers are all closely associated with 'ferroptosis', a recently discovered form of programmed cell death with promising therapeutic targets. The predictive potential of these pathophysiology-based indicators may offer superior patient stratification for future trials, individualised patient care and resource allocation.


Assuntos
Esclerose Lateral Amiotrófica/diagnóstico , Biomarcadores/sangue , Neurônios/patologia , 8-Hidroxi-2'-Desoxiguanosina/sangue , Adulto , Aldeídos/sangue , Progressão da Doença , Feminino , Ferritinas/sangue , Ferroptose , Seguimentos , Humanos , Ferro/metabolismo , Isoprostanos/sangue , Peroxidação de Lipídeos , Masculino , Pessoa de Meia-Idade , Proteínas de Neurofilamentos/sangue , Neurônios/metabolismo , Valor Preditivo dos Testes , Prognóstico
14.
Rev Med Interne ; 23(5): 465-8, 2002 May.
Artigo em Francês | MEDLINE | ID: mdl-12064219

RESUMO

INTRODUCTION: Enterocolic phlebitis is an entity characterized by ischemic injury of the gastrointestinal tract caused by thrombophlebitis of the mesenteric veins without arterial involvement or systemic disease. EXEGESIS: We report a case of enterocolic phlebitis in a 57-year-old female treated by rutoside, revealed by intestinal obstruction related to a pseudotumoral lesion of the caecum. CONCLUSION: This case adds to the four cases of enterocolic phlebitis under rutoside already reported in the literature, suggesting a possible involvement of this drug in this rare disease.


Assuntos
Doenças do Ceco/induzido quimicamente , Enterocolite/induzido quimicamente , Granuloma de Células Plasmáticas/induzido quimicamente , Obstrução Intestinal/induzido quimicamente , Rutina/análogos & derivados , Rutina/efeitos adversos , Doenças do Ceco/diagnóstico por imagem , Doenças do Ceco/patologia , Enterocolite/diagnóstico por imagem , Enterocolite/patologia , Feminino , Granuloma de Células Plasmáticas/diagnóstico por imagem , Granuloma de Células Plasmáticas/patologia , Humanos , Hiperplasia , Obstrução Intestinal/diagnóstico por imagem , Obstrução Intestinal/patologia , Pessoa de Meia-Idade , Pomadas/efeitos adversos , Tomografia Computadorizada por Raios X
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