Electrophysiological evaluation of the neuromuscular junction: a brief review / Avaliação eletrofisiológica da junção neuromuscular: breve revisão

Abstract The nerve terminal and muscle membrane compose the neuromuscular junction. After opening the voltage-gated calcium channels, action potentials from the motor axons provoke a cascade for the acetylcholine release from synaptic vesicles to the synaptic cleft, where it binds to its receptor at the muscle membrane for depolarization. Low amplitude compound muscle action potential typically presents in presynaptic disorders, increasing by more than 100% after a 10-second effort in the Lambert-Eaton myasthenic syndrome and less in botulism. Needle electromyography may show myopathic motor unit action potentials and morphological instability ("jiggle") due to impulse blocking. Low-frequency repetitive nerve stimulation (RNS) is helpful in postsynaptic disorders, such as myasthenia gravis and most congenital myasthenic syndromes, where the number of functioning acetylcholine receptors is reduced. Low-frequency RNS with a decrement >10% is abnormal when comparing the 4th to the first compound muscle action potential amplitude. High-frequency RNS is helpful in presynaptic disorders like Lambert-Eaton myasthenic syndrome, botulism, and some rare congenital myasthenic syndromes. The high-frequency RNS releases more calcium, increasing the acetylcholine with a compound muscle action potential increment. Concentric needle records apparent single-fiber action potentials (spikes). A voluntary activation measures the jitter between spikes from two endplates. An electrical activation measures the jitter of one spike (one endplate). The jitter is the most sensitive test for detecting a neuromuscular junction dysfunction. Most neuromuscular junction disorders are responsive to treatment.

Resumo O nervo terminal e a membrana muscular compõem a junção neuromuscular. Após a abertura dos canais de cálcio dependentes de voltagem, os potenciais de ação do axônio motor provocam uma cascata de eventos que libera acetilcolina das vesículas para a fenda sináptica, ligando-se ao receptor na membrana muscular para despolarização. O potencial de ação muscular composto de baixa amplitude ocorre nas desordens pré-sinápticas, aumentando em mais de 100% após esforço de 10 segundos na síndrome miastênica de Lambert-Eaton e menos no botulismo. A eletromiografia pode mostrar potenciais de ação da unidade motora miopáticos e instabilidade morfológica ("jiggle") devido ao bloqueio do impulso. Estimulação nervosa repetitiva (ENR) de baixa frequência é útil nos distúrbios pós-sinápticos, como miastenia gravis e a maioria das síndromes miastênicas congênitas, quando há número reduzido de receptores de acetilcolina funcionantes. ENR de baixa frequência com decremento >10% é anormal comparando-se à amplitude do quarto com o primeiro potencial de ação muscular composto. ENR de alta frequência é útil nas doenças pré-sinápticas, como síndrome miastênica de Lambert-Eaton, botulismo e algumas síndromes miastênicas congênitas raras. ENR de alta frequência libera mais cálcio, aumenta acetilcolina, resultando em incremento do potencial de ação muscular composto. O eletrodo de agulha concêntrico registra potenciais de ação aparente de fibra única (PAAFU). Ativação voluntária mede jitter entre dois PAAFUs (duas junções neuromusculares). Ativação elétrica mede jitter de um PAAFU (uma junção neuromuscular). Jitter é o teste mais sensível para detectar disfunção de junção neuromuscular. A maioria dos distúrbios juncionais é responsiva ao tratamento.

Electrophysiological evaluation of the neuromuscular junction: a brief review.

The nerve terminal and muscle membrane compose the neuromuscular junction. After opening the voltage-gated calcium channels, action potentials from the motor axons provoke a cascade for the acetylcholine release from synaptic vesicles to the synaptic cleft, where it binds to its receptor at the muscle membrane for depolarization. Low amplitude compound muscle action potential typically presents in presynaptic disorders, increasing by more than 100% after a 10-second effort in the Lambert-Eaton myasthenic syndrome and less in botulism. Needle electromyography may show myopathic motor unit action potentials and morphological instability ("jiggle") due to impulse blocking. Low-frequency repetitive nerve stimulation (RNS) is helpful in postsynaptic disorders, such as myasthenia gravis and most congenital myasthenic syndromes, where the number of functioning acetylcholine receptors is reduced. Low-frequency RNS with a decrement >10% is abnormal when comparing the 4th to the first compound muscle action potential amplitude. High-frequency RNS is helpful in presynaptic disorders like Lambert-Eaton myasthenic syndrome, botulism, and some rare congenital myasthenic syndromes. The high-frequency RNS releases more calcium, increasing the acetylcholine with a compound muscle action potential increment. Concentric needle records apparent single-fiber action potentials (spikes). A voluntary activation measures the jitter between spikes from two endplates. An electrical activation measures the jitter of one spike (one endplate). The jitter is the most sensitive test for detecting a neuromuscular junction dysfunction. Most neuromuscular junction disorders are responsive to treatment.

O nervo terminal e a membrana muscular compõem a junção neuromuscular. Após a abertura dos canais de cálcio dependentes de voltagem, os potenciais de ação do axônio motor provocam uma cascata de eventos que libera acetilcolina das vesículas para a fenda sináptica, ligando-se ao receptor na membrana muscular para despolarização. O potencial de ação muscular composto de baixa amplitude ocorre nas desordens pré-sinápticas, aumentando em mais de 100% após esforço de 10 segundos na síndrome miastênica de Lambert-Eaton e menos no botulismo. A eletromiografia pode mostrar potenciais de ação da unidade motora miopáticos e instabilidade morfológica ("jiggle") devido ao bloqueio do impulso. Estimulação nervosa repetitiva (ENR) de baixa frequência é útil nos distúrbios pós-sinápticos, como miastenia gravis e a maioria das síndromes miastênicas congênitas, quando há número reduzido de receptores de acetilcolina funcionantes. ENR de baixa frequência com decremento >10% é anormal comparando-se à amplitude do quarto com o primeiro potencial de ação muscular composto. ENR de alta frequência é útil nas doenças pré-sinápticas, como síndrome miastênica de Lambert-Eaton, botulismo e algumas síndromes miastênicas congênitas raras. ENR de alta frequência libera mais cálcio, aumenta acetilcolina, resultando em incremento do potencial de ação muscular composto. O eletrodo de agulha concêntrico registra potenciais de ação aparente de fibra única (PAAFU). Ativação voluntária mede jitter entre dois PAAFUs (duas junções neuromusculares). Ativação elétrica mede jitter de um PAAFU (uma junção neuromuscular). Jitter é o teste mais sensível para detectar disfunção de junção neuromuscular. A maioria dos distúrbios juncionais é responsiva ao tratamento.

A case of mitochondrial DNA depletion syndrome type 11 - expanding the genotype and phenotype.

Mitochondrial DNA depletion syndrome type 11 (MTDPS11) is caused by pathogenic variants in MGME1 gene. We report a woman, 40-year-old, who presented slow progressive drop eyelid at 11-year-old with, learning difficulty and frequent falls. Phisical examination revealed: mild scoliosis, elbow hyperextensibility, flat feet, chronic progressive external ophthalmoplegia with upper eyelid ptosis, diffuse hypotonia, and weakness of arm abduction and neck flexion. Investigation evidenced mild serum creatine kinase increase and glucose intolerance; second-degree atrioventricular block; mild mixed-type respiratory disorder and atrophy and granular appearance of the retinal pigment epithelium. Brain magnetic resonance showed cerebellar atrophy. Muscle biopsy was compatible with mitochondrial myopathy. Genetic panel revealed a homozygous pathogenic variant in the MGME1 gene, consistent with MTDPS11 (c.862C>T; p.Gln288*). This case of MTDPS11 can contribute to the phenotypic characterization of this ultra-rare mitochondrial disorder, presenting milder respiratory and nutritional involvement than the previously reported cases, with possible additional features.

Muscle fiber conduction velocity in situ revisited: A new approach to an ancient technique.

The aim of this study was to measure the muscle fiber conduction velocity (MFCV) in situ in the tibialis anterior muscle in healthy subjects. A total of 36 subjects matched for age and sex were studied. The MFCV was measured with a concentric needle by intramuscular monopolar needle electrical activation at a distance of 50 mm. The mean consecutive difference (MCD) of <5 µs was obtained after a median of 62 muscle fiber action potentials (MFAPs), confirming a direct muscle fiber activation. The measuring latency was at the median point of ascending depolarizing line of the MFAP. The calculated MFCV from 784 MFAPs was 4.10 ± 0.66 m/s, 3.99 ± 0.57 for female subjects (95%, 2.85 to 5.13), and 4.20 ± 0.73 for male subjects (95%, 2.74 to 5.67). The MFCV was 5.22% faster in male subjects. The calculated fast-to-slow MFCV ratio (F/S ratio) was 1.47 for female subjects (95%, 1.27 to 2.54) and 1.67 for male subjects (95%, 1.31 to 3.74). Aging significantly increased the F/S ratio. As the MFCVs mainly depend on the muscle diameter, their assessment is a quick and helpful tool for estimating it. Its variability by the F/S ratio is also a powerful tool in the follow-up of some neuromuscular disorders.

Concentric needle jitter: Reference values in stimulated Tibialis Anterior muscle.

Calculating the reference values for jitter parameters utilizing a disposable concentric needle have been already done for the most often tested muscles. Jitter, expressed as the mean consecutive difference (MCD), was measured in the Tibialis Anterior (TA), not routinely tested muscle. Jitter measurement was taken using the intramuscular microaxonal stimulation technique in 32 healthy subjects. The mean MCD and the mean MCD of the 27th value from the 32 subjects had a normal distribution and were 19.79 ± 2.72 µs and 26.88 ± 3.56 µs, respectively. The suggested limit for the mean MCD is ≥ 26 µs and for the individual values is > 34 µs.

Concentric Needle Jitter in 97 Myasthenia Gravis Patients.

Objectives: To estimate the jitter parameters (single-fiber electromyography) in myasthenia gravis patients mostly by electrical activation in Frontalis, Orbicularis Oculi, and Extensor Digitorum muscles using a concentric needle electrode. Methods: Between 2009 and 2019, a total of 97 myasthenia gravis patients, 52 male, and mean age 54 years were included. Results: Any abnormal jitter parameter in individual muscles was 90.5% (Frontalis), 88.5% (Orbicularis Oculi), and 86.6% (Extensor Digitorum). Any jitter parameter combining Orbicularis Oculi and Frontalis muscle was abnormal in 100% for the ocular, and in 92.9% for the generalized myasthenia gravis. The most abnormal muscle was Orbicularis Oculi for the generalized, and Frontalis for the ocular myasthenia gravis. The decrement was abnormal in 78.4%, 85.9% for the generalized, and 25% for the ocular myasthenia gravis. The mean jitter ranged from 14.2 to 86 µs (mean 33.3 µs) for the ocular myasthenia gravis and from 14.4 to 220.4 µs (mean 66.3 µs) for the generalized myasthenia gravis. The antibody titers tested positive in 86.6%, 91.8% for the generalized, and 50% for the ocular myasthenia gravis. Thymectomy was done in 48.5%, thymoma was found in 19.6%, and myasthenic crisis occurred by 21.6%. Conclusion: The jitter parameters achieved a 100% abnormality in ocular myasthenia gravis if both the Orbicularis Oculi and Frontalis muscles were tested. There was a high jitter abnormality in generalized myasthenia gravis cases with one muscle tested, with about a 2% increase in sensitivity when a second is added. Concentric needle electrode jitter had high sensitivity similar to the single fiber electrode (93.8%), followed by antibody titers (86.6%), and abnormal decrement (78.4%).

Electrophysiological study of neuromuscular junction in congenital myasthenic syndromes, congenital myopathies, and chronic progressive external ophthalmoplegia.

This study was designed to analyze the sensitivity, specificity, and accuracy of jitter parameters combined with repetitive nerve stimulation (RNS) in congenital myasthenic syndrome (CMS), chronic progressive external ophthalmoplegia (CPEO), and congenital myopathies (CM). Jitter was obtained with a concentric needle electrode during voluntary activation of the Orbicularis Oculi muscle in CMS (n = 21), CPEO (n = 20), and CM (n = 18) patients and in controls (n = 14). RNS (3 Hz) was performed in six different muscles for all patients (Abductor Digiti Minimi, Tibialis Anterior, upper Trapezius, Deltoideus, Orbicularis Oculi, and Nasalis). RNS was abnormal in 90.5% of CMS patients and in only one CM patient. Jitter was abnormal in 95.2% of CMS, 20% of CPEO, and 11.1% of CM patients. No patient with CPEO or CM presented a mean jitter higher than 53.6 µs or more than 30% abnormal individual jitter (> 45 µs). No patient with CPEO or CM and mild abnormal jitter values presented an abnormal decrement. Jitter and RNS assessment are valuable tools for diagnosing neuromuscular transmission abnormalities in CMS patients. A mean jitter value above 53.6 µs or the presence of more than 30% abnormal individual jitter (> 45 µs) strongly suggests CMS compared with CPEO and CM.

Jitter evaluation in denervation and reinnervation in 32 cases of chronic radiculopathy.

OBJECTIVE: To measure the jitter parameters in muscles with denervation/reinnervation in 32 chronic radiculopathy cases. METHODS: Measurements were done in chronic denervated muscles by voluntary and electrical activation using a concentric needle electrode. RESULTS: Mean jitter was abnormal in 87.5% (mean 49.2 µs) and 81.25% (mean 36.8 µs), for voluntary and electrical activation. In muscles with fibrillation potentials (FPs), the mean jitter was abnormal in all cases, and impulse blocking was frequent (53.4-92.3%). In muscles without FPs, the mean jitter was abnormal in 78.9% for voluntary activation and 68.4% for electrical activation. No correlation was found between jitter and motor unit action potential amplitude. CONCLUSION: The muscles with FPs were associated with the immature spread of acetylcholine receptors (AChRs) throughout the muscle membrane. Conversely, the neuromuscular junctions (NMJs) assemble may be repressed by the already reinnervated muscles. For those, higher jitter may be due to the persistence of atrophic fibers expressing neonatal myosin heavy chain (MHC) and immaturity of NMJ composting instead of the overspread of immature AChRs. SIGNIFICANCE: Jitter measurement must be avoided in chronic denervated muscles, regardless of FPs' presence. The activity of reinnervated muscle could maintain neonatal MHC and repress new NMJs development.

Jitter Evaluation in Distant and Adjacent Muscles after Botulinum Neurotoxin Type A Injection in 78 Cases.

To study the jitter parameters in the distant (DM) and the adjacent muscle (AM) after botulinum neurotoxin type A (BoNT/A) injection in 78 patients, jitter was measured by voluntary activation in DM (n = 43), and in AM (n = 35). Patients were receiving BoNT/A injections as a treatment for movement disorders. Mean age 65.1 years (DM) and 61.9 years (AM). The mean jitter was abnormal in 13.9% (maximum 41.4 µs) of DM, and 40% (maximum 43.7 µs) of AM. Impulse blocking was sparse. We found no correlation of the mean jitter to age, BoNT/A most recent injection (days/units), number of muscles injected, total BoNT/A units summated, number of total BoNT/A sessions, beta-blockers/calcium channel blockers use, and cases with local spread symptoms such as eyelid drop/difficulty swallowing. Maximum mean jitter (41.4/43.7 µs) for DM/AM occurred 61 and 131 days since the most recent BoNT/A, respectively. The far abnormal mean jitter (32.6/36.9 µs) occurred 229 and 313 days since the most recent BoNT/A. We suggested that jitter measurement can be done after BoNT/A in a given muscle other than the injected one, after 8 (DM) and 11 (AM) months, with reference >33 µs and >37 µs, respectively.

Guidelines for single fiber EMG.

This document is the consensus of international experts on the current status of Single Fiber EMG (SFEMG) and the measurement of neuromuscular jitter with concentric needle electrodes (CNE - CN-jitter). The panel of authors was chosen based on their particular interests and previous publications within a specific area of SFEMG or CN-jitter. Each member of the panel was asked to submit a section on their particular area of interest and these submissions were circulated among the panel members for edits and comments. This process continued until a consensus was reached. Donald Sanders and Erik Stålberg then edited the final document.

Problems in Comparing Jitter Values Obtained with Voluntary Activation and Electrical Stimulation.

Comparing results from jitter studies performed with voluntary and electrical activation is difficult to perform quantitatively, particularly in complex signals as seen in reinnervation. High jitter values in individual spikes in these multispike signals can be missed with both activation methods, which introduces a bias towards more normal values.With voluntary activation, triggering on a spike from an abnormal end-plate in multispike potentials will overestimate individual jitter values and the number of abnormal jitter values.With electrical stimulation, artefactually-increased jitter may be caused by subliminal stimulation, which causes uncertainty at the stimulation point. Electrical stimulation also may activate many axons, causing signal summation, with erroneous or impossible jitter estimation.Awareness of such pitfalls can improve the correct performance and interpretation of jitter recordings. Quantitative comparisons of results can be made between studies in reinnervated muscle performed with the same activation method, but not between results obtained with different activation methods.

Pitfalls and errors in measuring jitter.

The safety factor of neuromuscular transmission can be assessed by measuring the neuromuscular jitter, which reflects the time variability of processes in the motor end-plate. Jitter is increased in any condition with disturbed end-plate function, such as myasthenic conditions and ongoing reinnervation. Jitter is increasingly being measured with concentric needle (CN) electrodes, which are more prone to artefacts than single fiber EMG recordings. The objective of this review is to identify and demonstrate pitfalls that can be seen with CN jitter measurements, made with both voluntary activation and electrical stimulation. With voluntary activation, errors are caused by poor signal quality; inappropriate time reference points on the signal; an irregular firing rate; and signals with dual latencies, i.e., "flip-flop." With electrical stimulation, additional errors result from insufficient stimulation intensity; from abrupt change in firing rate; and from axon reflexes. Many pitfalls cannot be avoided during recording and can only be detected during post-processing. It is critical to be aware of these artefacts when measuring jitter with CN electrodes.

Time perception and age.

UNLABELLED: Our internal clock system is predominantly dopaminergic, but memory is predominantly cholinergic. Here, we examined the common sensibility encapsulated in the statement: "time goes faster as we get older". OBJECTIVE: To measure a 2 min time interval, counted mentally in subjects of different age groups. METHOD: 233 healthy subjects (129 women) were divided into three age groups: G1, 15-29 years; G2, 30-49 years; and G3, 50-89 years. Subjects were asked to close their eyes and mentally count the passing of 120 s. RESULTS: The elapsed times were: G1, mean = 114.9 ± 35 s; G2, mean = 96.0 ± 34.3 s; G3, mean = 86.6 ± 34.9 s. The ANOVA-Bonferroni multiple comparison test showed that G3 and G1 results were significantly different (P < 0.001). CONCLUSION: Mental calculations of 120 s were shortened by an average of 24.6% (28.3 s) in individuals over age 50 years compared to individuals under age 30 years.

Time perception and age / Percepção do tempo e idade

ABSTRACT Our internal clock system is predominantly dopaminergic, but memory is predominantly cholinergic. Here, we examined the common sensibility encapsulated in the statement: “time goes faster as we get older”. Objective To measure a 2 min time interval, counted mentally in subjects of different age groups. Method 233 healthy subjects (129 women) were divided into three age groups: G1, 15-29 years; G2, 30-49 years; and G3, 50-89 years. Subjects were asked to close their eyes and mentally count the passing of 120 s. Results The elapsed times were: G1, mean = 114.9 ± 35 s; G2, mean = 96.0 ± 34.3 s; G3, mean = 86.6 ± 34.9 s. The ANOVA-Bonferroni multiple comparison test showed that G3 and G1 results were significantly different (P < 0.001). Conclusion Mental calculations of 120 s were shortened by an average of 24.6% (28.3 s) in individuals over age 50 years compared to individuals under age 30 years.

RESUMO Nosso sistema de relógio interno é predominantemente dopaminérgico, mas a memória é predominantemente colinérgica. Neste estudo, examinamos a assertiva comum que “o tempo passa mais rápido para pessoas mais velhas”. Objetivo Medir o intervalo de tempo 2 min contados mentalmente em pessoas de diferentes faixas etárias. Método 233 pessoas saudáveis (129 mulheres) foram divididos em três grupos: G1, 15-29 anos; G2, 30-49 anos; e G3, 50-89 anos. Foi solicitado que contassem mentalmente, com os olhos fechados, a passagem de 120 s. Resultados Os tempos aferidos foram: G1, média = 114,9 ± 35 s; G2, média = 96,0 ± 34,3 s; G3, média = 86,6 ± 34,9 s. A comparação entre os tempos de G3 e G1 (teste de comparação múltipla ANOVA-Bonferroni) foi muito significante (P < 0,001). Conclusão Cálculo mental de 120 s foi encurtado em média 24,6% (28,3 s) em pessoas maiores que 50 anos quando comparado com pessoas menores que 30 anos.

Expressão de antígenos MHC classe I e de células CD4 e CD8 na polimiosite e dermatomiosite / MHC class I antigens, CD4 and CD8 expressions in polymyositis and dermatomyositis

Objetivo: Analisar as frequências de expressão dos antígenos de complexo principal de histocompatibilidade classe I (MHC-I) e células CD4 e CD8 no músculo esquelético na polimiosite (PM) e dermatomiosite (DM). Métodos: Estudo retrospectivo de 34 casos de PM, oito casos de DM e 29 controles com miopatias não inflamatórias. Resultados: Os antígenos MHC-I expressaram-se no sarcolema e/ou sarcoplasma em 79,4% dos casos de PM, 62,5% dos casos de DM e 27,6% dos controles (a expressão de CD4 foi observada em 76,5%, 75% e 13,8%, respectivamente). Quando os antígenos de MHC-I foram coexpressados com CD4, houve elevada suspeita de PM/DM (principalmente PM). Em 14,3% dos casos de PM/DM, observou-se a expressão isolada dos antígenos MHC-I, sem células inflamatórias. Conclusão: A expressão dos antígenos MHC-I e a positividade do CD4 podem aumentar a suspeita diagnóstica de PM/DM. Não foi observado infiltrado celular em 14,3% dos casos. .

Objective: To analyze the frequencies of the expression of major histocompatibility complex class I (MHC-I) antigens, and CD4 and CD8 cells in skeletal muscle in polymyositis (PM) and dermatomyositis (DM). Methods: This was a retrospective study of 34 PM cases, 8 DM cases, and 29 control patients with non-inflammatory myopathies. Results: MHC-I antigens were expressed in the sarcolemma and/or sarcoplasm in 79.4% of PM cases, 62.5% of DM cases, and 27.6% of controls (CD4 expression was observed in 76.5%, 75%, and 13.8%, respectively). There was a high suspicion of PM/DM (mainly PM) in participants in whom MHC-I antigens and CD4 were co-expressed. In 14.3% of PM/DM cases, we observed MHC-I antigens expression alone, without inflammatory cells. Conclusion: MHC-I antigens expression and CD4 positivity might add to strong diagnostic suspicion of PM/DM. No cellular infiltration was observed in approximately 14.3% of such cases. .

[MHC class I antigens, CD4 and CD8 expressions in polymyositis and dermatomyositis]. / Expressão de antígenos MHC classe I e de células CD4 e CD8 na polimiosite e dermatomiosite.

OBJECTIVE: To analyze the frequencies of the expression of major histocompatibility complex class I (MHC-I) antigens, and CD4 and CD8 cells in skeletal muscle in polymyositis (PM) and dermatomyositis (DM). METHODS: This was a retrospective study of 34 PM cases, 8 DM cases, and 29 control patients with non-inflammatory myopathies. RESULTS: MHC-I antigens were expressed in the sarcolemma and/or sarcoplasm in 79.4% of PM cases, 62.5% of DM cases, and 27.6% of controls (CD4 expression was observed in 76.5%, 75%, and 13.8%, respectively). There was a high suspicion of PM/DM (mainly PM) in patients in whom MHC-I antigens and CD4 were co-expressed. In 14.3% of PM/DM cases, we observed MHC-I antigens expression alone, without inflammatory cells. CONCLUSION: MCH-I antigens expression and CD4 positivity might add to strong diagnostic suspicion of PM/DM. No cellular infiltration was observed in 14.3% of such cases.

Stimulated jitter with concentric needle in 42 myasthenia gravis patients.

OBJECTIVE: To estimate jitter parameters in myasthenia gravis in stimulated frontalis and extensor digitorum muscles using the concentric needle electrode. METHODS: Forty-two confirmed myasthenia gravis patients, being 22 males (aged 45.6±17.2 years-old) were studied. Jitter was expressed as the mean consecutive difference (MCD). RESULTS: MCD in extensor digitorum was 61.6 µs (abnormal in 85.7%) and in frontalis 57.3 µs (abnormal in 88.1%). Outliers represented 90.5% for extensor digitorum and 88.1% for frontalis. At least one jitter parameter was abnormal in 90.5% of the combined studies. Acetylcholine receptor antibody was abnormal in 85.7% of the cases. CONCLUSIONS: Stimulated jitter recordings measured from muscles using concentric needle electrode can be used for myasthenia gravis diagnosis with high sensitivity. Extensive normative studies are still lacking and, therefore, borderline findings should be judged with great caution.

Stimulated jitter with concentric needle in 42 myasthenia gravis patients / Jitter estimulado obtido com agulha concêntrica em 42 pacientes com miastenia gravis

Objective: To estimate jitter parameters in myasthenia gravis in stimulated frontalis and extensor digitorum muscles using the concentric needle electrode. Methods: Forty-two confirmed myasthenia gravis patients, being 22 males (aged 45.6±17.2 years-old) were studied. Jitter was expressed as the mean consecutive difference (MCD). Results: MCD in extensor digitorum was 61.6 µs (abnormal in 85.7%) and in frontalis 57.3 µs (abnormal in 88.1%). Outliers represented 90.5% for extensor digitorum and 88.1% for frontalis. At least one jitter parameter was abnormal in 90.5% of the combined studies. Acetylcholine receptor antibody was abnormal in 85.7% of the cases. Conclusions: Stimulated jitter recordings measured from muscles using concentric needle electrode can be used for myasthenia gravis diagnosis with high sensitivity. Extensive normative studies are still lacking and, therefore, borderline findings should be judged with great caution. .

Objetivo: Mensurar os valores do jitter em pacientes com miastenia gravis nos músculos frontalis e extensor digitorum pela técnica estimulada, utilizando-se eletrodo de agulha concêntrica. Métodos: Foram estudados 42 pacientes, sendo 22 homens (idade 45,6±17,2 anos), com miastenia gravis confirmada. O jitter foi expresso como a média das diferenças consecutivas (MDC). Resultados: A MDC para o extensor digitorum foi 61,6 µs (anormal em 85,7%) e para o frontalis 57,3 µs (anormal em 88,1%). Outliers representaram 90,5% para o extensor digitorum e 88,1% para o frontalis. Pelo menos um parâmetro do jitter foi anormal em 90,5% dos estudos combinados. Anticorpo receptor de acetilcolina estava anormal em 85,7% dos casos. Conclusões: Jitter estimulado mensurado por meio de eletrodo de agulha concêntrica pode ser utilizado para diagnóstico de miastenia gravis com elevada sensibilidade. Estudos normativos mais amplos ainda são necessários e, portanto, valores limítrofes devem ser avaliados com cautela. .

Concentric needle jitter on stimulated frontalis and extensor digitorum in 20 myasthenia gravis patients.

INTRODUCTION: Our objective was to study jitter parameters using a concentric needle electrode (CNE) in the extensor digitorum (ED) and frontalis (FR) muscles. METHODS: Twenty myasthenia gravis (MG) patients, mean age 44.5 years, were studied. Percutaneous (FR) and intramuscular needle (ED) stimulation approaches were used. Jitter was expressed as the mean consecutive difference (MCD). The filter settings were from 1000 HZ to 10 kHZ. RESULTS: Abnormal MCD was found in 85% for both ED and FR and in 90% when combining the two muscles. An abnormal percentage of outliers was found in 90% for ED and 85% for FR. The mean MCD did not show a difference for ED and FR, but the percentage of outliers and blocking were higher in FR. Abnormality was found in 93.7% (generalized) and in 75% (ocular) of MG cases. For ED outliers abnormality was greater than the MCD. CONCLUSION: CNE jitter is reliable for investigation of MG, although borderline findings should be judged with caution.

Lactato basal sanguíneo em indivíduos não diabéticos e diabéticos: mensuração por meio de tiras / Basal blood lactate in non-diabetics and diabetics’ patients: measurementby means of test-strip method

Lactato resulta da glicólise anaeróbica e sua dosagem é usualmente realizada após coleta de sangue venoso. Não há relatos de valores de referência para mensuração por meio de fitas após uso de lancetas na extremidade do dedo. O objetivo principal do estudo é mensurar o lactato sanguíneo por meio de fitas em voluntários normais e em diabéticos. Foram constituídos 2 grupos: normal, composto por 69 voluntários sadios, com média de idade de 41,3 ± 17,5 anos (15 a 86), 27 masculino e 42 feminino, e diabéticos, composto por 31 doentes, com média de idade de 52,1 ± 14,8 anos (13 a 76), 10 masculino e 21 feminino. Gota de sangue após jejum de 12 horas foi colocada em fita e os valores do lactato e glicose mensurados em aparelhos portáteis, Accutrend® Lactate e Accutrend® GCT . No grupo normal o lactato sanguíneo foi 2,7 ± 0,95 mmol/L (1,0 a 5,2), com limite superior de normalidade de 4,6 mmol/L (95%). No grupo de diabéticos foi 3,94 ± 1,27 mmol/L (1,7 a 7,0). Houve diferença estatisticamente significativa entre os grupos (P<0,0001) e não foi encontrada correlação entre os níveis de glicose e lactato em ambos os grupos. Os valores de referência de lactato sanguíneo mensurado por meio de fitas foram mais elevados e tiveram maior variação em relação à dosagem usual; indivíduos diabéticos tem nível sanguíneo mais elevado em relação aos normais, mas sem correlação com o nível glicêmico.

Lactate results from anaerobic glycolysis, and it is usually measured after a vein puncture. There are noknown reports on reference values for lactate measure through strips after a blood drop from digit. The main purpose of the study is to get reference values for blood lactate using strips in normal subjects and patients with diabetes. Two groups were constituted: control with 69 health subjects with mean age of41.3 ± 17.5 years (15 - 86), 42 females and 27 males, and 32 patients with diabetes melito with mean age of52.1 ± 14.8 years (13 - 76), 21 females and 10 males. Blood lactate and glucose were measured after 12 hour-fasting through a blood drop on a strip for immediate reading on portable Accutrend® Lactate and Accutrend® GCT. Control group: lactate 2.7 ± 0.95 mmol/L (1.0 - 5.2) and upper limit of normality 4.6mmol/L (95%). Diabetic group: lactate 3.94 ± 1.27 mmol/L (1.7 - 7.0). There was a significant difference between groups (P<0.0001) and there was no correlation between lactate and glucose values in bothgroups. The reference values of blood lactate measured by the test-strip method were higher and had a greater variation from the usual dosage; diabetics’ patients have higher blood levels compared to normal ones, but no correlation with the glucose level.