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INTRODUCTION/AIMS: The single simple question (SSQ), "What percentage of normal (0%-100%) do you feel regarding your disease?" has proven feasible and valid in assessing myasthenia gravis and a heterogeneous spectrum of neuropathies. This study explores the utility of the SSQ in axonal polyneuropathies (PNPs), encompassing diabetic neuropathy, and evaluates its responsiveness to scale changes. METHODS: A retrospective chart review of 150 patients with axonal PNP responding to the SSQ was performed. Patients underwent clinical and electrophysiological evaluations, and were evaluated by clinical and disability scales, including the Medical Research Council sum score, modified Toronto Clinical Neuropathy score (mTCNS), Overall Neuropathy Limitation Scale, and Rasch-built Overall Disability Scale (RODS). RESULTS: The SSQ total scores correlated moderately with both the RODS score (r = .59, p < .001) and the mTCNS symptom score (r = -.43, p < .001), maintaining significance after adjustment for multiple comparisons. Longitudinally, after adjusting for multiple comparisons, the change in mTCNS symptom score retained statistical significance (adjusted p = .048). The SSQ did not show any association with electrophysiological parameters or sensory symptoms, other than a lower score in those with pain (100% with SSQ <40%, 85% with SSQ 40%-70%, and 34% with SSQ >70%). DISCUSSION: The SSQ is a feasible, valid scale that may be utilized to assess and follow patients with length-dependent axonal PNPs. Given that the SSQ is not strongly associated with clinical and disability scales or electrophysiological findings, additional investigations are required for a comprehensive assessment of PNP.
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BACKGROUND: Several studies of patients with polyneuropathy failed to show differences between patients with and without pain. In the current study, we aimed to explore the association between neuropathic symptoms, mainly pain, and polyneuropathy characteristics. METHODS: A prospective cross-sectional study recruiting 151 patients with non-diabetic polyneuropathy was performed between November 2016 and May 2017. A total of 38 patients with chronic inflammatory demyelinating neuropathy were excluded. Patients underwent clinical, electrophysiological and functional assessments of their polyneuropathy. Polyneuropathy characteristics were compared depending on the presence and severity of neuropathic symptoms. RESULTS: The presence and the severity of weakness were associated with a more severe neuropathy as measured by clinical, electrophysiological and functional assessments, while the presence of pain was associated with higher sural amplitudes, and the severity of pain showed a curvilinear association with neuropathy severity. CONCLUSIONS: Our study shows a novel finding of a curvilinear association between pain and polyneuropathy severity.
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Neuropatías Diabéticas/fisiopatología , Dolor/fisiopatología , Polineuropatías/complicaciones , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/fisiopatología , Adulto , Anciano , Estudios Transversales , Neuropatías Diabéticas/complicaciones , Femenino , Humanos , Masculino , Persona de Mediana Edad , Conducción Nerviosa/fisiología , Dolor/complicaciones , Polineuropatías/diagnóstico , Polineuropatías/fisiopatología , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/complicaciones , Estudios ProspectivosRESUMEN
INTRODUCTION: A single and simple question, namely "What percentage of normal (PoNL) do you feel regarding your disease?" is feasible and valid in myasthenia gravis. In this study, we aimed to determine the validity of this question in patients with nondiabetic polyneuropathy. METHODS: Clinical, electrophysiological, and functional and disability assessments were performed in 151 patients with nondiabetic polyneuropathy. One hundred forty patient answers were recorded for the PoNL question, and these were included in the current study. RESULTS: The PoNL correlated moderately with functional and disability scales. DISCUSSION: "What PoNL do you feel?" is a simple, quick, and valid question, which correlates moderately with functional and disability scales in nondiabetic polyneuropathy, and it may be incorporated in polyneuropathy assessment.
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Autoevaluación Diagnóstica , Evaluación de la Discapacidad , Polineuropatías/diagnóstico , Adulto , Anciano , Femenino , Humanos , Masculino , Persona de Mediana Edad , Polineuropatías/fisiopatología , Calidad de VidaRESUMEN
BACKGROUND: In the current study, we aimed to determine normative values for muscle thickness and fasciculation prevalence in healthy subjects. METHODS: We performed a prospective study from October to December 2018 in 65 healthy subjects. All subjects underwent quantitative sonographic evaluation of muscle thickness and fasciculation prevalence in the following 8 muscles: Biceps brachii, abductor pollicis brevis, first dorsal interosseous, abductor digiti minimi, quadriceps, tibialis anterior, extensor digitorum brevis, and abductor hallucis brevis. RESULTS: Subject ages ranged from 21 to 82 years, with 63% women. Normative values for muscle thickness were determined using the fifth percentile. Multivariate regression analysis showed that sex, age, body mass index, and hand dominance affected muscle thickness. Fasciculations were observed frequently only in distal muscles. CONCLUSIONS: Normal values for muscle thickness were determined, and may enhance neuromuscular ultrasound sensitivity and serve as a basis for future studies. Larger series are needed to confirm these values.
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Fasciculación/diagnóstico por imagen , Fasciculación/epidemiología , Músculo Esquelético/diagnóstico por imagen , Adulto , Anciano , Anciano de 80 o más Años , Electromiografía , Femenino , Músculos Isquiosurales/diagnóstico por imagen , Voluntarios Sanos , Humanos , Masculino , Persona de Mediana Edad , Prevalencia , Estudios Prospectivos , Valores de Referencia , Ultrasonografía , Adulto JovenRESUMEN
RATIONALE & OBJECTIVE: The renin-angiotensin-aldosterone system (RAAS) is associated with renal and cardiovascular disease in diabetes. Unfortunately, early RAAS blockade in patients with type 1 diabetes mellitus (T1DM) does not prevent the development of complications. We sought to examine the role of hyperfiltration and RAAS activation across a wide range of T1DM duration to better understand renal hemodynamic status in patients with T1DM. STUDY DESIGN: Post hoc analysis of blood samples. SETTING & PARTICIPANTS: 148 Canadian patients with T1DM: 28 adolescents (aged 16.2±2.0 years), 54 young adults (25.4±5.6 years), and 66 older adults (65.7±7.5 years) studied in a clinical investigation unit. EXPOSURE: Angiotensin II infusion (1ng/kg/min; a measure of RAAS activation) during a euglycemic clamp. OUTCOMES: Glomerular filtration rate measured using inulin clearance, effective renal plasma flow measured using para-aminohippurate, afferent (RA) and efferent (RE) arteriolar resistances, and glomerular hydrostatic pressure estimated using the Gomez equations. RESULTS: In a stepwise fashion, glomerular filtration rate, effective renal plasma flow, and glomerular hydrostatic pressure were higher, while renal vascular resistance and RA were lower in adolescents versus young adults versus older adults. RE was similar in adolescents versus young adults but was higher in older adults. Angiotensin II resulted in blunted renal hemodynamic responses in older adults (renal vascular resistance increase of 3.3% ± 1.6% vs 4.9% ± 1.9% in adolescents; P<0.001), suggesting a state of enhanced RAAS activation. LIMITATIONS: Homogeneous study participants limit the generalizability of findings to other populations. Studying older adult participants with T1DM may be associated with a survivorship bias. CONCLUSIONS: A state of relatively low RAAS activity and predominant afferent dilation rather than efferent constriction characterize early adolescents and young adults with T1DM. This state of endogenous RAAS inactivity in early T1DM may explain why pharmacologic blockade of this neurohormonal system is often ineffective in reducing kidney disease progression in this setting. Older adults with long-standing T1DM who have predominant afferent constriction and RAAS activation may experience renoprotection from therapies that target the afferent arteriole. Further work is required to understand the potential role of non-RAAS pharmacologic agents that target RA in patients with early and long-standing T1DM.
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Angiotensina II/administración & dosificación , Diabetes Mellitus Tipo 1/fisiopatología , Hemodinámica/fisiología , Sistema Renina-Angiotensina/efectos de los fármacos , Sistema Renina-Angiotensina/fisiología , Adolescente , Adulto , Factores de Edad , Anciano , Canadá , Estudios de Cohortes , Diabetes Mellitus Tipo 1/sangre , Femenino , Estudios de Seguimiento , Tasa de Filtración Glomerular/efectos de los fármacos , Tasa de Filtración Glomerular/fisiología , Hemodinámica/efectos de los fármacos , Humanos , Masculino , Persona de Mediana Edad , Estudios Prospectivos , Medición de Riesgo , Resistencia Vascular/efectos de los fármacos , Resistencia Vascular/fisiología , Adulto JovenRESUMEN
INTRODUCTION: Elevated creatine kinase (CK) level was redefined by the European Federation of Neurological Societies)EFNS(as 1.5 times the upper limit of normal. In the current study we sought to determine the sensitivity and specificity of CK testing for the diagnosis of neuromuscular disorders. METHODS: Demographics and CK levels were retrospectively extracted from an electronic database for 234 patients with neuromuscular disorders. Sensitivity, specificity, and likelihood ratios and the area under curve were determined for each diagnosis and different cutoff CK values. RESULTS: Using the EFNS cutoff values significantly reduced CK test sensitivity. Creatine kinase values >1000 IU/L showed a high likelihood (11.04) for myopathies and a low likelihood for polyneuropathies (0). DISCUSSION: European Federation of Neurological Societies cutoff values significantly reduce CK sensitivity for diagnosing neuromuscular disorders. While low CK values cannot exclude a neuromuscular disease, values >1000 IU/L are associated with a high likelihood of myopathy.
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Creatina Quinasa/sangre , Enfermedad de la Neurona Motora/diagnóstico , Enfermedades Musculares/diagnóstico , Polineuropatías/diagnóstico , Adulto , Anciano , Área Bajo la Curva , Europa (Continente) , Femenino , Humanos , Masculino , Persona de Mediana Edad , Enfermedad de la Neurona Motora/sangre , Enfermedades Musculares/sangre , Neurología , Enfermedades Neuromusculares/sangre , Enfermedades Neuromusculares/diagnóstico , Polineuropatías/sangre , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/sangre , Polirradiculoneuropatía Crónica Inflamatoria Desmielinizante/diagnóstico , Valores de Referencia , Sociedades MédicasRESUMEN
INTRODUCTION: Nerve imaging has a limited role in axonal and muscle fiber loss. In this study, we sought to explore the utility of standardized muscle ultrasound (US) assessment in these clinical scenarios. METHODS: We performed a prospective study from March to August 2018 of patients attending the neuromuscular clinic. All patients underwent clinical evaluation and standardized muscle thickness measurement by US in seven muscles. RESULTS: The study cohort consisted of 114 participants, including patients with polyneuropathy, motor neuron disease, and myopathy. The smallest distal muscle thickness was found in patients with polyneuropathy, while the smallest proximal muscle thickness was found in patients with myopathy. Muscle thickness was strongly correlated with muscle strength (r 2 = 0.62), electrophysiological findings (r 2 : 0.44-0.55), and disability score (r 2 = 0.53). DISCUSSION: Standardized muscle thickness measured by US shows diagnostic usefulness in a spectrum of neuromuscular disorders and correlates with clinical and electrophysiological findings.
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Músculo Esquelético/diagnóstico por imagen , Enfermedades Neuromusculares/diagnóstico por imagen , Potenciales de Acción/fisiología , Adulto , Anciano , Esclerosis Amiotrófica Lateral/diagnóstico por imagen , Esclerosis Amiotrófica Lateral/patología , Esclerosis Amiotrófica Lateral/fisiopatología , Electromiografía , Femenino , Humanos , Masculino , Síntomas sin Explicación Médica , Persona de Mediana Edad , Fuerza Muscular , Músculo Esquelético/patología , Músculo Esquelético/fisiopatología , Atrofia Muscular Espinal/diagnóstico por imagen , Atrofia Muscular Espinal/patología , Atrofia Muscular Espinal/fisiopatología , Enfermedades Musculares/diagnóstico por imagen , Enfermedades Musculares/patología , Enfermedades Musculares/fisiopatología , Conducción Nerviosa/fisiología , Enfermedades Neuromusculares/patología , Enfermedades Neuromusculares/fisiopatología , Tamaño de los Órganos , Polineuropatías/diagnóstico por imagen , Polineuropatías/patología , Polineuropatías/fisiopatología , Estudios Prospectivos , Radiculopatía/diagnóstico por imagen , Radiculopatía/patología , Radiculopatía/fisiopatología , UltrasonografíaRESUMEN
AIMS: To examine the relationship between normal plasma uric acid (PUA) levels, renal haemodynamic function, arterial stiffness and plasma renin and aldosterone over a wide range of type 1 diabetes (T1D) durations in adolescents, young adults and older adults. MATERIALS AND METHODS: PUA, glomerular filtration rate (GFR), effective renal plasma flow (ERPF), vascular stiffness parameters (aortic augmentation index [AIx], carotid AIx, carotid femoral pulse wave velocity [cfPWV]), and plasma renin and aldosterone were measured during a euglycaemic clamp in people with T1D: 27 adolescents (mean ± SD age 16.8 ± 1.9 years), 52 young adults (mean ± SD age 25.6 ± 5.5 years) and 66 older adults (mean ± SD age 65.7 ± 7.5 years). RESULTS: PUA was highest in patients with the longest T1D duration: 197 ± 44 µmol/L in adolescents versus 264 ± 82 µmol/L in older adults (P < 0.001). Higher PUA correlated with lower GFR only in older adults, even after correcting for age, glycated haemoglobin and sex (ß = -2.12 ± 0.56; P = 0.0003), but not in adolescents or young adults. Higher PUA correlated with lower carotid AIx (ß = -1.90, P = 0.02) in adolescents. In contrast, PUA correlated with higher cfPWV (P = 0.02) and higher plasma renin (P = 0.01) in older adults with T1D. CONCLUSIONS: The relationship between higher PUA with lower GFR, increased arterial stiffness and renin angiotensin aldosterone system (RAAS) activation was observed only in older adults with longstanding T1D. T1D duration may modify the association between PUA, renal haemodynamic function and RAAS activation, leading to renal vasoconstriction and ischaemia. Further work must determine whether pharmacological PUA-lowering prevents or reverses injurious haemodynamic and neurohormonal sequelae of longstanding T1D, thereby improving clinical outcomes.
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Diabetes Mellitus Tipo 1 , Riñón , Ácido Úrico/sangre , Rigidez Vascular/fisiología , Adolescente , Adulto , Factores de Edad , Anciano , Diabetes Mellitus Tipo 1/sangre , Diabetes Mellitus Tipo 1/epidemiología , Diabetes Mellitus Tipo 1/fisiopatología , Femenino , Tasa de Filtración Glomerular/fisiología , Hemodinámica/fisiología , Humanos , Riñón/irrigación sanguínea , Riñón/fisiología , Masculino , Persona de Mediana Edad , Análisis de la Onda del Pulso , Estudios Retrospectivos , Adulto JovenRESUMEN
OBJECTIVE: Our aim was to define the relationships between plasma biomarkers of kidney injury and intrarenal haemodynamic function (glomerular filtration rate [GFR], effective renal plasma flow [ERPF], renal vascular resistance [RVR]) in adults with type 1 diabetes (T1D). METHODS: The study sample comprised patients with longstanding T1D (duration ≥50 years), among whom 44 were diabetic kidney disease (DKD) resistors (eGFR >60 mL/min/1.73 m2 and <30 mg/d urine albumin excretion) and 22 had DKD, in addition to 73 control participants. GFRINULIN and ERPFPAH were measured, RVR was calculated, and afferent (RA )/efferent (RE ) areteriolar resistances were derived from Gomez equations. Plasma neutrophil gelatinase-associated lipocalin (NGAL), ß2 microglobulin (B2M), osteopontin (OPN) and uromodulin (UMOD) were measured using immunoassay kits from Meso Scale Discovery. RESULTS: Plasma NGAL, B2M and OPN were higher and UMOD was lower in DKD patients vs DKD resistors and non-diabetic controls. In participants with T1D, plasma NGAL inversely correlated with GFR (r = -0.33; P = 0.006) and ERPF (r = -0.34; P = 0.006), and correlated positively with RA (r = 0.26; P = 0.03) and RVR (r = 0.31; P = 0.01). In participants without T1D, NGAL and B2M inversely correlated with GFR (NGAL r = -0.18; P = 0.13 and B2M r = -0.49; P < 0.0001) and with ERPF (NGAL r = -0.19; P = 0.1 and B2M r = -0.42; P = 0.0003), and correlated positively with RA (NGAL r = 0.19; P = 0.10 and B2M r = 0.3; P = 0.01) and with RVR (NGAL r = 0.20; P = 0.09 and B2M r = 0.34; P = 0.003). Differences were significant after adjusting for age, sex, HbA1c, SBP and LDL. There were statistical interactions between T1D status, B2M and intrarenal haemodynamic function (P < 0.05). CONCLUSIONS: Elevated NGAL relates to intrarenal haemodynamic dysfunction in T1D, whereas elevated NGAL and B2M relate to intrarenal haemodynamic dysfunction in adults without T1D. These data may define a diabetes-specific interplay between tubular injury and intrarenal haemodynamic dysfunction.
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Biomarcadores/sangre , Diabetes Mellitus Tipo 1/diagnóstico , Nefropatías Diabéticas/diagnóstico , Hemodinámica/fisiología , Riñón/irrigación sanguínea , Riñón/fisiopatología , Anciano , Anciano de 80 o más Años , Biomarcadores/análisis , Canadá , Estudios de Casos y Controles , Estudios Transversales , Diabetes Mellitus Tipo 1/sangre , Diabetes Mellitus Tipo 1/complicaciones , Diabetes Mellitus Tipo 1/fisiopatología , Nefropatías Diabéticas/sangre , Nefropatías Diabéticas/fisiopatología , Progresión de la Enfermedad , Femenino , Tasa de Filtración Glomerular , Humanos , Lipocalina 2/análisis , Lipocalina 2/sangre , Longevidad , Masculino , Persona de Mediana Edad , Valor Predictivo de las Pruebas , Pronóstico , Flujo Sanguíneo Regional/fisiología , Resistencia Vascular/fisiología , Microglobulina beta-2/análisis , Microglobulina beta-2/sangreRESUMEN
To explore whether higher degrees of electrophysiological abnormalities are associated with a more frequent exposure to a more aggressive treatment regimen, we performed a retrospective chart review of patients attending the neuromuscular clinic from June 2012 to December 2015 and included 87 patients. We compared treatment regimens during the follow-up period between patients with high and low jitter and decrement. Myasthenia gravis patients with high jitter or decrement at baseline were more frequently treated with intravenous immunoglobulins (IVIG) and/or plasma exchange (PLEX) during the follow-up period. In patients with mild disease, IVIG or PLEX treatment was associated with high decrement.
La diminution initiale du potentiel moteur de patients atteints de myasthénie grave peut permettre de prédire le type d'immuno-modulation thérapeutique prodiguée. Afin d'explorer dans quelle mesure des niveaux plus élevés d'anomalies électro-physiologiques peuvent être associés à une exposition davantage fréquente à des régimes de traitement plus vigoureux, nous avons effectué un examen rétrospectif des dossiers de patients, 87 au total, s'étant présentés à une clinique neuromusculaire de juin 2012 à décembre 2015. Nous avons alors comparé les régimes de traitement des patients montrant de basses mesures de gigue (jitter) et une faible diminution d'amplitude du potentiel d'action au cours de leur période de suivi avec les régimes de traitement d'autres patients pour qui ces mesures étaient élevées. Les patients atteints de myasthénie grave (MG) dont les mesures de gigue et la diminution d'amplitude du potentiel d'action étaient initialement élevées ont été plus fréquemment traités, lors d'un suivi, avec des immunoglobulines intraveineuses et/ou des échanges plasmatiques. Chez les patients atteints de la forme bénigne de cette maladie, ces deux traitements ont été associés à une diminution d'amplitude du potentiel d'action plus élevée.
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Inmunoglobulinas Intravenosas/uso terapéutico , Miastenia Gravis/terapia , Intercambio Plasmático , Adulto , Anciano , Azatioprina/uso terapéutico , Electromiografía , Femenino , Humanos , Inmunosupresores/uso terapéutico , Masculino , Persona de Mediana Edad , Miastenia Gravis/diagnóstico , Miastenia Gravis/tratamiento farmacológico , Miastenia Gravis/fisiopatología , Prednisona/uso terapéutico , Estudios Retrospectivos , Índice de Severidad de la EnfermedadRESUMEN
BACKGROUND: High levels of uric acid (UA) are associated with various peripheral neuropathies. Furthermore, uric acid levels have been found to correlate with both the clinical and electrophysiological severity of diabetic sensorimotor polyneuropathy, mainly with sensory functions. OBJECTIVES: To determine whether higher UA levels are associated negatively with nerve function in healthy subjects. METHODS: A total of 126 healthy subjects recruited prospectively for another study were included. We extracted demographic data, body mass index (BMI), blood pressure, Toronto Clinical Neuropathy Score (TCNS), electrophysiological findings, vibration perception thresholds (VPT), and laboratory test results including UA, hemoglobin A1c (HbA1c), estimated glomerular filtration rate (eGFR), and lipid levels. RESULTS: The mean age of the cohort was 56 ± 17 years with 56% females. Males had higher UA values compared with females. Univariate beta regression coefficient analysis between UA levels and demographic, clinical, electrophysiological, and laboratory findings showed significant positive correlations with male gender, components of the metabolic syndrome, and with VPT, while an inverse correlation was found with electrophysiological sensory parameters. A multivariate regression model showed positive correlations only with BMI, finger VPT, and triglycerides. CONCLUSION: Higher UA levels correlate with lower sensory nerve function in healthy subjects, expanding the evidence of possible negative influence of UA on peripheral nerves, although a causative role has not yet established.
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Nervios Periféricos/fisiología , Sensación/fisiología , Umbral Sensorial/fisiología , Ácido Úrico/sangre , Adulto , Anciano , Femenino , Voluntarios Sanos , Humanos , Masculino , Persona de Mediana EdadRESUMEN
BACKGROUND: Lumbar puncture (LP) performed with the assistance of ultrasound (US) may improve success rate, with fewer puncture attempts and less pain. OBJECTIVES: To explore the utility of US-assisted LP in a neuromuscular clinic. METHODS: We performed a prospective, randomized, open-label study between May 2016 and January 2017. The primary outcome measure was LP success rate, and the secondary outcome measures included procedure time, number of attempts, and the levels of pain, anxiety, and satisfaction. RESULTS: Lumbar puncture was performed in 40 consecutive patients. Ultrasound-assisted LP had a 100% success rate, compared with 85% (95% confidence interval: 58%-96%) without US assistance, although this difference failed to reach statistical significance. Ultrasound-assisted LP was associated with less pain and, also in patients >60 years of age, with fewer needle insertions. Both groups reported high satisfaction rates, regardless of the use of US. CONCLUSION: Ultrasound-assisted LP has a high success rate and less pain than unassisted LP, and can be accomplished easily in the outpatient neuromuscular clinic setting equipped with US.
CONTEXTE: En plus de diminuer le nombre de tentatives de ponction alors nécessaires et la douleur qui en résulte, il est possible qu'un examen de ponction lombaire effectué avec l'aide d'appareils à ultrasons obtienne un plus haut taux de réussite. OBJECTIFS: Dans le cadre d'une clinique neuromusculaire, se pencher sur l'utilité des examens de ponction lombaire assistés par des appareils à ultrasons. MÉTHODES: De mai 2016 à janvier 2017, nous avons mené une étude prospective sans insu à répartition aléatoire. La principale mesure de nos résultats a porté sur le taux de succès des examens de ponction lombaire. D'autres mesures de résultats ont inclus les aspects suivants: la durée des examens, le nombre de tentatives de ponction ainsi que les niveaux de douleur, d'anxiété et de satisfaction des patients. RÉSULTATS: Des examens de ponction lombaire ont été effectués chez 40 patients consécutifs. Ceux menés à l'aide d'appareils à ultrasons ont donné à voir un taux de succès de 100 % comparativement à 85 % (IC 95 % : 58 % - 96 %) dans le cas de ceux effectués sans ces appareils. Rappelons toutefois que cette différence ne s'est pas révélée significative sur le plan statistique. Les examens de ponction lombaire au moyen d'appareils à ultrasons ont aussi été associés à moins de douleurs ressenties et, chez des patients âgés de plus de 60 ans, à moins d'insertions d'aiguilles. Enfin, ces deux groupes ont rapporté des taux de satisfaction élevés, et ce, sans égard à l'utilisation d'appareils à ultrasons. CONCLUSIONS: Les examens de ponction lombaire assistés par des appareils à ultrasons possèdent un haut taux de succès et entraînent moins de douleurs que ceux menés sans ces appareils. Ils peuvent aussi être effectués facilement dans le cadre de cliniques neuromusculaires externes qui en sont munies.
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Enfermedades Neuromusculares/diagnóstico por imagen , Dolor/etiología , Punción Espinal/efectos adversos , Ultrasonografía/métodos , Anciano , Ansiedad/etiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Evaluación de Resultado en la Atención de Salud , Dolor/diagnóstico por imagen , Dolor/prevención & control , Estudios Prospectivos , Estadísticas no Paramétricas , Escala Visual AnalógicaRESUMEN
AIMS/HYPOTHESIS: Small cohort studies raise the hypothesis that corneal nerve abnormalities (including corneal nerve fibre length [CNFL]) are valid non-invasive imaging endpoints for diabetic sensorimotor polyneuropathy (DSP). We aimed to establish concurrent validity and diagnostic thresholds in a large cohort of participants with and without DSP. METHODS: Nine hundred and ninety-eight participants from five centres (516 with type 1 diabetes and 482 with type 2 diabetes) underwent CNFL quantification and clinical and electrophysiological examination. AUC and diagnostic thresholds were derived and validated in randomly selected samples using receiver operating characteristic analysis. Sensitivity analyses included latent class models to address the issue of imperfect reference standard. RESULTS: Type 1 and type 2 diabetes subcohorts had mean age of 42 ± 19 and 62 ± 10 years, diabetes duration 21 ± 15 and 12 ± 9 years and DSP prevalence of 31% and 53%, respectively. Derivation AUC for CNFL was 0.77 in type 1 diabetes (p < 0.001) and 0.68 in type 2 diabetes (p < 0.001) and was approximately reproduced in validation sets. The optimal threshold for automated CNFL was 12.5 mm/mm2 in type 1 diabetes and 12.3 mm/mm2 in type 2 diabetes. In the total cohort, a lower threshold value below 8.6 mm/mm2 to rule in DSP and an upper value of 15.3 mm/mm2 to rule out DSP were associated with 88% specificity and 88% sensitivity. CONCLUSIONS/INTERPRETATION: We established the diagnostic validity and common diagnostic thresholds for CNFL in type 1 and type 2 diabetes. Further research must determine to what extent CNFL can be deployed in clinical practice and in clinical trials assessing the efficacy of disease-modifying therapies for DSP.
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Córnea/diagnóstico por imagen , Neuropatías Diabéticas/diagnóstico por imagen , Microscopía Confocal , Adolescente , Adulto , Anciano , Área Bajo la Curva , Estudios de Cohortes , Estudios Transversales , Diabetes Mellitus Tipo 1/diagnóstico por imagen , Diabetes Mellitus Tipo 2/diagnóstico por imagen , Femenino , Humanos , Cooperación Internacional , Masculino , Persona de Mediana Edad , Sensibilidad y EspecificidadRESUMEN
INTRODUCTION: Monoclonal gammopathy has been reported in several polyneuropathies. In this study, we sought to explore the frequency and characteristics of monoclonal gammopathy in patients with diabetic sensorimotor polyneuropathy (DSP). METHODS: Patients with type 1 and type 2 diabetes mellitus (DM 1, DM 2) and controls without diabetes were evaluated between November 2008 and December 2013. RESULTS: Fifty controls, 66 patients with DM 1, and 106 patients with DM 2 were included, with average ages of 43 ± 18, 45 ± 17, and 65 ± 10 years, respectively; the frequency of monoclonal gammopathy was 0%, 8%, and 15%, respectively. In patients with DSP, the frequency of monoclonal gammopathy increased to 14% in DM 1 and 21% in DM 2; the most common monoclonal proteins were immunoglobulin (Ig) M and IgG, respectively. DISCUSSION: DSP might be associated with a high frequency of monoclonal gammopathy, with different characteristics in DM 1 and in DM 2. Muscle Nerve 57: 1018-1021, 2018.
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Diabetes Mellitus/epidemiología , Neuropatías Diabéticas/epidemiología , Gammopatía Monoclonal de Relevancia Indeterminada/epidemiología , Polineuropatías/epidemiología , Adulto , Anciano , Comorbilidad , Femenino , Humanos , Masculino , Persona de Mediana Edad , PrevalenciaRESUMEN
To study the frequency of laboratory test abnormalities, and electrophysiological correlations, we performed a retrospective chart review of 226 patients with polyneuropathy. The frequency of laboratory test abnormalities, and correlations with electrophysiological findings were explored. Abnormal glucose handling tests were the most common findings (54%), followed by paraproteinemia (21%) and anemia (21%). The frequencies of paraproteinemia and anemia in our cohort were significantly higher than previously reported. In addition, several laboratory abnormalities correlated with electrophysiological findings of median neuropathy at the wrist, expanding current knowledge about the deleterious effects of various metabolic and hematologic derangements at this site.
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Anemia/etiología , Electrofisiología/métodos , Paraproteinemias/etiología , Polineuropatías/complicaciones , Anciano , Estudios de Cohortes , Femenino , Intolerancia a la Glucosa/etiología , Humanos , Masculino , Persona de Mediana Edad , Muñeca/inervaciónRESUMEN
INTRODUCTION: Repetitive nerve stimulation (RNS) showing ≥ 10% decrement is considered the cutoff for myasthenia gravis (MG), but this has never been validated. The objective of this study was to find an optimal validated cutoff value for decrement on RNS. METHODS: We performed retrospective chart review of patients who had electrophysiological assessment for possible MG from 2013 to 2015. RESULTS: A total of 122 patients with MG and 182 controls were identified. RNS sensitivities for generalized and ocular MG using the traditional ≥10% cutoff value were 46% and 15%, respectively, for frontalis recordings, and 35% and 19%, respectively, for nasalis recordings. Using a decrement cutoff value of 7% for frontalis and 8% for nasalis increased the sensitivities by 6-11%, with specificities of 95-96%. CONCLUSIONS: For RNS in facial muscles, we suggest a cutoff value of 7-8%, which increases test sensitivity by 6-11%, while preserving high specificity for the diagnosis of MG. Muscle Nerve, 2016 Muscle Nerve 55: 166-170, 2017.
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Estimulación Eléctrica/métodos , Potenciales Evocados Motores/fisiología , Miastenia Gravis/diagnóstico , Adulto , Anciano , Biofisica , Electromiografía , Femenino , Humanos , Masculino , Persona de Mediana Edad , Músculo Esquelético/fisiopatología , Miastenia Gravis/fisiopatología , Estudios RetrospectivosRESUMEN
INTRODUCTION: Electrophysiological studies play an important role in the diagnosis of myasthenia gravis (MG). The objectives of this study was to explore the correlation of jitter and decrement with various clinical symptoms and signs and disease severity. METHODS: We performed a retrospective chart review of 75 MG patients who attended the neuromuscular clinic from April 2013 to May 2014. We compared clinical characteristics between patients with high jitter (>100 µs) and decrement (>10%), and patients with lower values to explore the correlations and optimal thresholds of jitter and decrement for different clinical features. RESULTS: High jitter and decrement values were associated with more severe disease, manifested by more frequent symptomatic bulbar and limb muscle weakness, more frequent ocular and limb muscle weakness on examination, higher quantitative MG score, and generalized disease. CONCLUSIONS: The yield of the electrophysiological assessment in MG extends beyond disease diagnosis and correlates with disease severity and the presence of generalized disease. Muscle Nerve 56: 445-448, 2017.
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Electromiografía/métodos , Miastenia Gravis/diagnóstico , Miastenia Gravis/fisiopatología , Índice de Severidad de la Enfermedad , Adulto , Anciano , Anciano de 80 o más Años , Fenómenos Electrofisiológicos/fisiología , Femenino , Humanos , Masculino , Persona de Mediana Edad , Estudios RetrospectivosRESUMEN
INTRODUCTION: High-resolution ultrasound (HRU) is used in the diagnosis of peripheral neuropathies. There are conflicting data regarding HRU findings in patients with diabetic sensorimotor polyneuropathy (DSP). Our purpose in this study was to measure nerve cross-sectional areas (CSAs) in patients with diabetes, with and without DSP. METHODS: We performed a prospective peripheral nerve HRU study of 100 diabetic subjects, assessed the CSA at predefined sites, and compared the results with those of 100 normal subjects. We evaluated the use of individual CSA values and various summary scores for diagnosis of DSP. RESULTS: Diabetic subjects had higher CSA values than healthy volunteers, and those with DSP had higher CSA values. Three or more enlarged CSA sites predicted DSP with 64% sensitivity and 77% specificity. CONCLUSIONS: Peripheral nerves are enlarged diffusely in diabetic patients, including sites not susceptible to bony compression. The number of enlarged CSA values can help predict the presence of DSP. Muscle Nerve, 2016 Muscle Nerve 55: 171-178, 2017.
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Diabetes Mellitus/diagnóstico por imagen , Diabetes Mellitus/patología , Neuropatías Diabéticas/diagnóstico por imagen , Nervios Periféricos/diagnóstico por imagen , Nervios Periféricos/fisiopatología , Ultrasonografía , Adulto , Anciano , Estudios de Cohortes , Femenino , Humanos , Masculino , Persona de Mediana Edad , Curva ROC , Estadísticas no Paramétricas , Adulto JovenRESUMEN
BACKGROUND: Older patients with longstanding type 1 diabetes have high cardiovascular disease (CVD) risk such that statin therapy is recommended independent of prior CVD events. We aimed to determine self-reported CVD prevention guideline adherence in patients with longstanding diabetes. RESEARCH DESIGN AND METHODS: 309 Canadians with over 50 years of type 1 diabetes completed a medical questionnaire for presence of lifestyle and pharmacological interventions, stratified into primary or secondary CVD prevention subgroups based on absence or presence of self-reported CVD events, respectively. Associations with statin use were analyzed using multivariable logistic regression. RESULTS: The 309 participants had mean ± SD age 65.7 ± 8.5 years, median diabetes duration 54.0 [IQR 51.0, 59.0] years, and HbA1c of 7.5 ± 1.1 % (58 mmol/mol). 159 (52.7 %) participants reported diet adherence, 296 (95.8 %) smoking avoidance, 217 (70.5 %) physical activity, 218 (71.5 %) renin-angiotensin-system inhibitor use, and 220 (72.1 %) statin use. Physical activity was reported as less common in the secondary prevention subgroup, and current statin use was significantly lower in the primary prevention subgroup (65.5 % vs. 84.8 %, p = 0.0004). In multivariable logistic regression, the odds of statin use was 0.38 [95 % CI 0.15-0.95] in members of the primary compared to the secondary prevention subgroup, adjusting for age, sex, hypertension history, body mass, HbA1c, cholesterol, microvascular complications, acetylsalicylic acid use, and renin-angiotensin system inhibitor use. CONCLUSION: Despite good self-reported adherence to general CVD prevention guidelines, against the principles of these guidelines we found that statin use was substantially lower in those without CVD history. Interventions are needed to improve statin use in older type 1 diabetes patients without a history of CVD.
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Enfermedades Cardiovasculares/prevención & control , Diabetes Mellitus Tipo 1/terapia , Dislipidemias/tratamiento farmacológico , Adhesión a Directriz , Inhibidores de Hidroximetilglutaril-CoA Reductasas/uso terapéutico , Longevidad , Cumplimiento de la Medicación , Prevención Primaria/métodos , Conducta de Reducción del Riesgo , Prevención Secundaria/métodos , Anciano , Bloqueadores del Receptor Tipo 1 de Angiotensina II/uso terapéutico , Inhibidores de la Enzima Convertidora de Angiotensina/uso terapéutico , Canadá , Enfermedades Cardiovasculares/diagnóstico , Enfermedades Cardiovasculares/epidemiología , Distribución de Chi-Cuadrado , Estudios Transversales , Diabetes Mellitus Tipo 1/diagnóstico , Diabetes Mellitus Tipo 1/epidemiología , Dieta/efectos adversos , Dislipidemias/diagnóstico , Dislipidemias/epidemiología , Ejercicio Físico , Femenino , Encuestas de Atención de la Salud , Humanos , Modelos Logísticos , Masculino , Persona de Mediana Edad , Actividad Motora , Análisis Multivariante , Oportunidad Relativa , Guías de Práctica Clínica como Asunto , Factores de Riesgo , Autoinforme , Fumar/efectos adversos , Cese del Hábito de Fumar , Prevención del Hábito de Fumar , Factores de Tiempo , Resultado del TratamientoRESUMEN
INTRODUCTION: High-resolution ultrasonography (HRU) is a novel method that provides morphological information about peripheral nerves. We aimed to determine reference values for nerve cross-sectional area (CSA) on HRU. METHODS: One hundred healthy volunteers had HRU of median, radial, ulnar, fibular, tibial, sural, and superficial fibular nerves at defined sites. The CSA was measured and the effects of age, gender, and body mass index (BMI) were evaluated. RESULTS: CSA values in healthy subjects are described. CSA is larger in lower limb motor nerves than in sensory nerves at similar sites, and the CSA tends to be symmetrical. The strongest effect on CSA was for age, although gender and BMI had some effects. CONCLUSIONS: This study provides normative values for HRU, and it suggests that further research with age- and gender-specific distributions must be a key priority in the development of HRU for use as a diagnostic test for peripheral nerve diseases.