Critical illness polyneuromyopathy in septic patients: Is it possible to diagnose it in a bedside clinical examination? / Polineuromiopatia do doente crítico em pacientes sépticos: É possível diagnosticar em um exame clínico à beira do leito?

ABSTRACT Objective: To determine the sensitivity and specificity of peripheral and respiratory muscle strength tests in diagnosing critical illness polyneuromyopathy (CIPNM), compared with an electrophysiological examination. Methods: Fifty septic patients who required mechanical ventilation for at least five days, and without a previous history of muscle weakness, were included. Peripheral muscle strength was assessed using the Medical Research Council (MRC) score, handgrip strength by dynamometry, and respiratory muscle strength with maximum respiratory pressures. Diagnosis of CIPNM was either confirmed or rejected by an electrophysiological examination. Receiver operating characteristic curve analysis was performed to determine the cut-off values with the best sensitivity (SN) and specificity (SP) of the studied variables in the presence or absence of CIPNM. Results: Patients with CIPNM were older, more critical (APACHE IV/SAPS 3), had a longer hospitalization, required mechanical ventilation for longer, and had a higher rate of intensive care unit readmission. Cutoff values identified CIPNM patients using MRC scores, dynamometry according to sex, maximal expiratory and inspiratory pressures, as well as being confirmed by the electrophysiological examination, with good sensitivity and specificity: < 40 (SN: 0.893; SP: 0.955); < 7 kg (SN: 1; SP: 0.909) for men, < 4 kg (SN: 0.882; SP: 1) for women; < 34 cmH2O (SN: 0.808; SP: 0.909) and > −40 cmH2O (SN: 0.846; SP: 0.909), respectively. Conclusion: The MRC score, dynamometry or maximum respiratory pressures can be used to identify patients with CIPNM at the intensive care bedside assessment. The healthcare professional can choose any of the methods studied to evaluate the patient, based on his experience and the resource available.

RESUMO Objetivo: Determinar a sensibilidade (SN) e especificidade (SP) dos testes de força muscular periférica e respiratória no diagnóstico da Polineuromiopatia do Doente Crítico (PNDC) em comparação com o estudo eletrofisiológico. Métodos: Foram incluídos 50 pacientes sépticos, em ventilação mecânica (VM) durante pelo menos cinco dias e sem história prévia de fraqueza muscular. A força muscular foi avaliada utilizando o escore Medical Research Council (MRC), a força de preensão palmar e as pressões respiratórias máximas. O diagnóstico de PNDC foi confirmado ou excluído pelo estudo eletrofisiológico. A análise da curva ROC foi realizada para determinar os valores de corte com a melhor SN e SP. Resultados: Os pacientes com PNDC eram mais velhos, mais graves, tiveram hospitalização mais longa, necessitaram de VM por mais tempo e apresentaram maior taxa de readmissão na Unidade de Terapia Intensiva. Os valores de corte identificaram os pacientes com PNDC usando o MRC, a dinamometria de acordo com o sexo, as pressões expiratórias e inspiratórias máximas, também confirmado pelo estudo eletrofisiológico, com boa sensibilidade e especificidade: < 40 (SN: 0.893; SP: 0.955), < 7 kg (SN: 1; SP: 0,909) para homens, < 4 kg (SN: 0,882; SP: 1) para mulheres, <34 cmH2O (SN: 0,808; SP: 0,909) e > −40 cmH2O (SN: 0,846; SP: 0,909), respectivamente. Conclusão: Tanto o MRC, a dinamometria quanto as pressões respiratórias máximas podem ser usadas para identificar pacientes com PNDC na avaliação à beira do leito, podendo o profissional de saúde escolher qualquer um dos métodos baseado em sua experiência e no recurso disponível.

Peripheral polyneuropathy in severely obese patients with metabolic syndrome but without diabetes: Association with low HDL-cholesterol / Polineuropatia periférica em pacientes obesos graves com síndrome metabólica sem diabetes: associação com baixo HDL-colesterol

Summary Introduction: The purpose of this study was to evaluate the prevalence of peripheral polyneuropathy (PPN) in subjects with grade II and III obesity (Ob-II,III) and metabolic syndrome (MetS) but without diabetes and to investigate possible associated factors. Method: A cross-sectional study was performed in non-diabetic Ob-II,III,MetS patients using the Michigan Neuropathy Screening Instrument (MNSI) to assess the presence of PPN. Results: A total of 24 of 218 non-diabetic Ob-II,III,MetS patients had PPN. Based on univariate analysis, serum levels of LDL-cholesterol (p=0.046) were significantly associated with PPN, while serum triglycerides (p=0.118) and low HDL-cholesterol (p=0.057) showed a tendency toward this association. On a Poisson regression analysis, when the three possible associations were included, low HDL-cholesterol (p=0.047) remained independently associated. Conclusion: In non-diabetic Ob-II,III,MetS patients, PPN defined by the MNSI showed a high prevalence and was associated with low levels of HDL-cholesterol. In order to diagnose that complication, neurological evaluation should be performed in these patients.

Resumo Objetivo: Avaliar a prevalência da polineuropatia periférica (PNP) em indivíduos obesos graus II e III com síndrome metabólica (Ob-II,III,SM) sem diabetes e buscar possíveis fatores associados. Método: Em um estudo transversal, realizado em indivíduos Ob-II,III,SM e sem diagnóstico de diabetes, o Instrumento de Screening de Michigan (MNSI) foi utilizado para avaliar a presença de PNP. Resultados: Um total de 24 de 218 pacientes Ob-II,III,SM e sem diabetes tinham PNP. Quando observamos as associações com PNP em uma análise univariada, níveis séricos de LDL-colesterol (p=0.046) estiveram significativamente associados e houve também uma tendência à associação com níveis séricos de triglicerídeos (p=0.118) e baixo HDL-colesterol (p=0.057). Em uma análise de regressão de Poisson, quando as três possíveis associações foram incluídas, baixo HDL-colesterol (p=0.047) manteve-se independentemente associado. Conclusão: Em pacientes Ob-II,III,SM, mas sem diabetes, a PNP definida pelo MNSI tem uma prevalência elevada e está associada a baixos níveis de HDL-colesterol. Para diagnóstico dessa complicação, recomenda-se realizar o exame neurológico desses pacientes.

Sensory disturbance and polyneuropathy in rheumatoid arthritis patients with foot deformity / Perturbações sensitivas e polineuropatia em pacientes com artrite reumatoide com deformidade do pé

Abstract Introduction Our aim in this study was to present the results of sensory evaluation tests and electrophysiological evaluations in rheumatoid arthritis (RA) patients with foot deformity and to determine their relation with general health status and lower extremity functionality. Materials and methods Fifty-one patients with RA diagnosis and foot deformity were included in the study. Demographic and disease characteristics of the patients were recorded, and a detailed neurological examination was performed. Superficial sensation, pain, heat, vibration, and two-point discrimination sensation were evaluated in each foot, and their sum was used to determine the sensory deficits index (SDI) of 0–10. The presence of polyneuropathy was evaluated with electrophysiological methods. The Health Assessment Questionnaire and mobility and walking subscales of the Arthritis Impact Measurement Scales-2 were used to assess general health status and lower extremity functionality, respectively. According to the sensory examination and electromyography results, patients were compared in terms of their general health status and lower extremity functional status. Results Sensory disturbance was detected in 39 patients (74%) during the examination; however, 27 patients (52.9%) had polyneuropathy determined electrophysiologically. In patients with sensory deficits, statistically significant deterioration was detected in general health and foot functionality, including mobility and walking, when compared to patients with a normal sensory evaluation. Conclusions Even in the presence of normal electrophysiological tests, sensory dysfunction alone seems to be associated with severe disability in general health status and foot functionality when compared to patients with a normal sensory examination.

Resumo Introdução O objetivo deste estudo foi apresentar os resultados dos testes de avaliação sensitiva e do exame eletrofisiológico em pacientes com artrite reumatoide (AR) com deformidade do pé e determinar a sua relação com o estado geral de saúde e o aspecto funcional dos membros inferiores. Materiais e métodos Foram incluídos no estudo 51 pacientes com diagnóstico de AR e deformidade do pé. Foram registradas as características demográficas e da doença de cada indivíduo e foi feito um exame neurológico detalhado. Foi avaliada a sensibilidade superficial, dolorosa, térmica e vibratória e aplicado o teste de discriminação de dois pontos em cada um dos pés. A soma dos escores foi usada para determinar o índice de déficits sensitivos (IDS) de 0‐10. A presença de polineuropatia foi avaliada com métodos eletrofisiológicos. Foram usados o Health Assessment Questionnaire e as subescalas mobilidade e deambulação da Arthritis Impact Measurement Scales‐2 para avaliar o estado geral de saúde e o aspecto funcional de membros inferiores, respectivamente. De acordo com os resultados dos exames de eletromiografia e de sensibilidade, os pacientes foram comparados em relação ao seu estado geral de saúde e estado funcional de membros inferiores. Resultados Foram detectados distúrbios sensitivos em 39 pacientes (74%) durante o exame; contudo, 27 (52,9%) tinham polineuropatia determinada eletrofisiologicamente. Em pacientes com déficits sensitivos, foi detectada deterioração estatisticamente significativa no estado geral de saúde e no aspecto funcional do pé, inclusive na mobilidade e deambulação, quando comparados com os pacientes com uma avaliação sensitiva normal. Conclusão Mesmo na presença de testes eletrofisiológicos normais, a disfunção sensitiva isolada parece estar associada a incapacidade grave no estado geral de saúde e ao aspecto funcional do pé em comparação com pacientes com um exame sensitivo normal.

Síndrome de POEMS / Poems syndrome

El síndrome de POEMS es un trastorno paraneoplásico asociado a discrasia de células plasmáticas que reúne, entre sus criterios diagnósticos mayores, la presencia de polineuropatía y de gammapatía monoclonal, entre otras como esclerosis ósea y/o cutánea. Se reportó un caso que reúne las características dominantes del síndrome

POEMS syndrome is a paraneoplastic disorder associated with plasma cell dyscrasia which joins among itsmajor diagnostic criteria, the presence of polyneuropathy and monoclonal gammopathy, such as bone and / or skin sclerosis. a case that meets the key features of the syndrome is reported

Actividad espontánea de nociceptores cutáneos en pacientes con neuropatía de fibras delgadas / Spontaneous activity of cutaneous nociceptors in patients with painful polyneuropathy. Report of three patients

Background: Painful polyneuropathy may result from selective impairment of small diameter nerve fibers, while tactile and motor functions are preserved. In these patients clinical and electrophysiological assessment is usually unrevealing. We report three patients with a pure painful polyneuropathy. One of them had neurogenic pruritus additionally. Quantitative sensory analysis disclosed a slight warm hypoesthesia (3/3) and paradoxical hot sensation (2/3) in the feet. Intraneural recordings from the peroneal nerve demonstrated abnormal spontaneous activity in 8 of 17 nociceptive afferents. One of them displayed double firing reflecting impulse multiplication. These results support the notion that patients with pain or pruritus with a distal distribution similar to a polyneuropathy, could have small diameter afferent fiber damage, despite normal function of large diameter fibers.

Thyrotoxic myo-neuropathy.

Myopathy is an important complication of thyrotoxicosis. Neuropathy is a less commonly reported complication, and is often subclinical. Here we report a patient of throtoxic myopathy with sub-clinical entrapment neuropathy. This case is reported to emphasise the importance of NCV to look for neuropathy.

Focal and multifocal diabetic neuropathies

Diabetic neuropathy is the most common neuropathy in industrialized countries, with a remarkable range of clinical manifestations. The vast majority of the patients with clinical diabetic neuropathy have a distal symmetrical form that progress following a fiber-length dependent pattern, with predominant sensory and autonomic manifestations. This pattern of neuropathy is associated with a progressive distal axonopathy. Patients are exposed to trophic changes in the feet, pains and autonomic disturbances. Less often, diabetic patients may develop focal and multifocal neuropathy that includes cranial nerve involvement, limb and truncal neuropathies. This neuropathic pattern tends to occur after 50 years of age, mostly in patients with longstanding diabetes mellitus. The LDDP does not show any trend to improvement and either relentlessly progresses or remain relatively stable over years. Conversely the focal diabetic neuropathies, which are often associated with inflammatory vasculopathy on nerve biopsies, remain self limited, sometimes after a relapsing course.

A neuropatia diabética é a mais predominante das neuropatias nos países industrializados apresentando uma gama variável de manifestações clinicas. A maioria dos pacientes com neuropatia diabética apresenta uma forma simétrica distal que progride para um padrão fibra comprimento dependente com manifestações sensitivas e autonomicas. Este tipo de neuropatia é associado com uma axonopatia distal progressiva. Os pacientes apresentam modificações tróficas nos pés, dores e distúrbios autonômicos. Menos freqüentemente os pacientes diabéticos podem desenvolver neuropatia focal e multifocal que incluem envolvimento de nervos cranianos, tronco e membros inferiores. Este padrão de neuropatia é mais freqüente em pacientes com mais de 50 anos e com longa historia de diabetes. Este tipo de neuropatia fibra-comprimento dependente não apresenta melhora, progride lentamente ou permanece estável por vários anos. As neuropatias focais que são associadas freqüentemente com vasculopatias inflamatórias nas biópsias de nervo, permanecem auto limitadas por vezes com surtos de remissão.

[Comparison of nerve conduction abnormalities in lumbosacral radiculopathy and axonal polyneuropathy]

The clinical differentiation between lumbosacral radiculopathy and generalized peripheral neuropathies of the distal-axonal type is often difficult. In such cases, the electromyographer relies on the presence of paraspinal or proximal muscle denervation and normal sensory conduction studies to indicate radicular disease. These criteria are often is inadequate, such as in elderly patients, when amplitudes of sensory nerve action potentials may be reduced as an age-related phenomenon. So if motor conduction changes in lumbosacral radiculopathy and generalized peripheral neuropathies are adequately described, a useful electrodiagnostic criterion for differentiation of radiculopathy and neuropathy will be available. Patients were entered in this study that met strict clinical and electrophysiological criteria for L5/S1 lumbosacral radiculopathy or generalized peripheral neuropathies of the distal-axonal type. The distal axonopathy group consisted of 19 men and 17 women, and lumbosacral radiculopathy group consisted of 28 men and 22 women. To minimize classification errors resulting from age-related changes, we excluded patients older than 60 years. Lower limbs of all patients were examined, recorded parameters included compound muscle action potential [CMAP] amplitude, distal latencies and conduction velocities; minimal, mean and maximal latencies of F-waves, and maximum-minimum latency ranges, and persistence of F waves of tibial and deep peroneal nerves. The mean values of minimal, mean and maximal latencies of F-waves of tibial and deep peroneal nerves in neuropathy group were significantly more prolonged than lumbosacral radiculopathy group [p<0.001]. The mean CMAPs amplitudes and persistence of tibial and deep peroneal nerves F-waves in neuropathy group were lower than lumbosacral radiculopathy group [p<0.001], but mean latency range of F-waves for both nerves in lumbosacral radiculopathy group were significantly more prolonged, [p<0.001], that this prolongation is more significant in deep peroneal nerves [p=0.004]. In young persons that significant decrease of SNAPs amplitude can reliably help to decision, mere dependence to motor conduction studies is not necessary. Also when there is denervation in proximal muscles that innervated by L5-S1 roots, the radiculopathy is more probable than neuropathy. When a patient is presented with numbness and paresthesia in lower limbs, and sensory nerve conduction or electromyographic studies can not help to decision, in these cases motor nerve conduction studies especially various parameters of F-wave such as; minimal latency, latency range and persistence could be useful

Síndrome Poems: relato de um caso brasileiro com evoluçäo de curso prolongado / Poems Syndrome: a brazilian case record with prolongued course evolution

Os autores relatam o caso de paciente do sexo masculino, de 58 anos, com polineuropatia, organomegalia, alterações endócrinas e dermatológicas e diversas lesões osteoescleróticas ao exame radiológico. Alterações no mielograma e na eletroforese de proteínas foram compatíveis com discrasia plasmocitária. Foi realizado o diagnóstico de Síndrome POEMS. A revisão do assunto mostra que o paciente apresentava vários dos critérios clínicos e laboratoriais utilizados para o diagnóstico desta síndrome. A suspeita diagnóstica de síndrome POEMS deve ser lembrada em todos os casos de polineuropatia inexplicada

Asymptomatic electrophysiologic carpal tunnel syndrome in diabetics: entrapment or polyneuropathy

Electrophysiologic carpal tunnel syndrome (CTS) is common and is frequently asymptomatic in diabetics. In order to evaluate the clinical significance of asymptomatic electrophysiologic CTS, the nerve conduction studies (NCS) of 48 diabetics with asymptomatic electrophysiologic CTS were compared with those of 56 age and gender-matched controls, as well as 50 patients with symptomatic CTS without diabetes. Nerve conduction velocities of the ulnar, peroneal, and posterior tibial nerves were significantly slower in diabetics with asymptomatic electrophysiologic CTS than in normal controls. Compared to symptomatic non-diabetic CTS, there was also significant slowing of the median and ulnar nerve conduction velocities in asymptomatic diabetic CTS. However, in diabetics with asymptomatic CTS, abnormalities of the distal segment of the median NCS were more prominent compared with those of all the other tested nerves. These findings suggested that asymptomatic electrophysiologic CTS in diabetics is a manifestation of increased vulnerability to the entrapment of the peripheral nerve.

The usefulness of minimal F-wave latency and sural/radial amplitude ratio in diabetic polyneuropathy

The possibility of whether minimal F-wave latency and a simple ratio between the sural and superficial radial sensory response amplitudes may provide a useful electrodiagnostic test in diabetic patients was investigated in this report. To evaluate the diagnostic sensitivity of minimal F-wave latency, the Z-scores of the minimal F-wave latency, motor nerve conduction velocity (MCV), amplitude of compound muscle action potentials (CMAP), and distal latency (DL) of the median, ulnar, tibial, and peroneal nerve were compared in 37 diabetic patients. For the median, ulnar, and tibial nerves, the Z scores of the minimal F-wave latency were significantly larger than those of the MCV. In addition for all four motor nerves, the Z scores of the minimal F-wave latency were significantly larger than those for the CMAP amplitude. Furthermore, 19 subjects showing abnormal results in the standard sensory nerve conduction study had a significantly lower sural/radial amplitude ratio (SRAR), and 84% of them had an SRAR of less than 0.5. In conclusion, minimal F-wave latency and the ratio between the amplitudes of the sural and superficial radial sensory nerve action potential are sensitive measures for the detection of nerve pathology and should be considered in electrophysiologic studies of diabetic polyneuropathy.