Anti-3-Hydroxy-3-Methylglutaryl-Coenzyme A Reductase Immune-Mediated Necrotizing Myopathy following mRNA SARS-CoV-2 Vaccination.

The new coronavirus (COVID-19) pandemic has resulted in the unprecedented production of vaccines. In this context, the possible adverse effects remain to be identified and reported. In this article, we report the case of a young female patient who developed anti-3-hydroxy-3-methylglutaryl-coenzyme A reductase (anti-HMG-CoA) immune-mediated necrotizing myositis (IMNM) after receiving the Pfizer-BioNTech (BNT162b2) COVID-19 vaccine. The diagnosis of probable post-vaccination IMNM was made due to the absence of other factors that may have led to the development of autoantibodies (medicines; e.g., statins, drugs) and the temporal relationship between exposure and event. This case report is the first to suggest that a COVID-19 vaccine may trigger anti-HMG-CoA reductase necrotizing myopathy.

Frequency of exposure to arboviruses and characterization of Guillain Barré syndrome in a clinical cohort of patients treated at a tertiary referral center in Brasília, Federal District.

BACKGROUND: Guillian Barré syndrome (GBS) is an acute autoimmune polyradiculoneuropathy often associated with previous exposure to infectious agents. METHODS: A clinical cohort of 41 patients with GBS admitted to the Base Hospital Institute of the Federal District between May 2017 and April 2019 was followed up for 1 year. Serological tests for arbovirus detection and amplification of nucleic acids using polymerase chain reaction for zika virus (ZIKV), dengue virus (DENV), and chikungunya virus (CHIKV) were performed. RESULTS: The cohort consisted of 61% men with a median age of 40 years, and 83% had GBS-triggering events. A total of 54% had Grade 4 disability, 17% had Grade 3, 12% had Grade 2, 10% had Grade 5, and 7% had Grade 1. The classic form occurred in 83% of patients. Nerve conduction evaluations revealed acute demyelinating inflammatory polyneuropathy (51%), acute motor axonal neuropathy (17%), acute sensory-motor neuropathy (15%), and indeterminate forms (17%). Four patients were seropositive for DENV. There was no laboratory detection of ZIKV or CHIKV infection. Ninety percent of patients received human immunoglobulin. Intensive care unit admission occurred in 17.1% of the patients, and mechanical ventilation was used in 14.6%. One patient died of Bickerstaff's encephalitis. Most patients showed an improvement in disability at 10 weeks of follow-up. CONCLUSIONS: GBS in the Federal District showed a variable clinical spectrum, and it was possible to detect recent exposure to DENV.

Epidemiological and clinical aspects of Guillain-Barré syndrome and its variants.

BACKGROUND: Guillain-Barré syndrome (GBS), an acute polyradiculoneuropathy that occurs because of an abnormal inflammatory response in the peripheral nervous system, is clinically characterized by acute flaccid paresis and areflexia with or without sensory symptoms. This syndrome can lead to disabling or even life-threatening sequelae. OBJECTIVE: This study aimed to present the clinical and epidemiological aspects of GBS in patients admitted to a tertiary-level hospital in the Federal District between January 2013 and June 2019. METHODS: In this observational, cross-sectional and retrospective study, medical records of patients diagnosed with acute inflammatory demyelinating polyradiculoneuropathy, acute motor axonal neuropathy or acute axonal motor-sensitive neuropathy based on electromyographic findings were included, and clinical data were collected retrospectively. RESULTS: A total of 100 patients (63 males and 37 females; ratio, 1.7:1) aged 2-86 years (mean, 36.4 years) were included. The mean annual incidence rate of GBS was 0.54 cases/100,000 inhabitants, with 52 and 49% of the cases occurring between October and March (rainy season) and between April and September (dry season), respectively. The proportions of patients showing each GBS variant were as follows: demyelinating forms, 57%; axonal forms, 39%; and undetermined, 4%. The mean duration of hospitalization was 8-15 days for most patients (38%). During hospitalization, 14% of the patients required mechanical ventilation and 20% experienced infectious complications. CONCLUSION: The findings indicate that there was an increase in the incidence of GBS during the rainy season. Moreover, we did not observe the typical bimodal distribution regarding age at onset.

How can neurophysiological studies help with movement disorders characterization in clinical practice? A review.

BACKGROUND: Neurophysiological studies are ancillary tools to better understand the features and nature of movement disorders. Electromyography (EMG), together with electroencephalography (EEG) and accelerometer, can be used to evaluate a hypo and hyperkinetic spectrum of movements. Specific techniques can be applied to better characterize the phenomenology, help distinguish functional from organic origin and assess the most probable site of the movement generator in the nervous system. OBJECTIVE: We intend to provide an update for clinicians on helpful neurophysiological tools to assess movement disorders in clinical practice. METHODS: Non-systematic review of the literature published up to June 2019. RESULTS: A diversity of protocols was found and described. These include EMG analyses to define dystonia, myoclonus, myokymia, myorhythmia, and painful legs moving toes pattern; EMG in combination with accelerometer to study tremor; and EEG-EMG to study myoclonus. Also, indirect measures of cortical and brainstem excitability help to describe and diagnose abnormal physiology in Parkinson's disease, atypical parkinsonism, dystonia, and myoclonus. CONCLUSION: These studies can be helpful for the diagnosis and are usually underutilized in neurological practice.

How can neurophysiological studies help with movement disorders characterization in clinical practice? A review / Como os exames neurofisiológicos podem ajudar na caracterização dos transtornos do movimento na prática clínica? Uma revisão

ABSTRACT Background: Neurophysiological studies are ancillary tools to better understand the features and nature of movement disorders. Electromyography (EMG), together with electroencephalography (EEG) and accelerometer, can be used to evaluate a hypo and hyperkinetic spectrum of movements. Specific techniques can be applied to better characterize the phenomenology, help distinguish functional from organic origin and assess the most probable site of the movement generator in the nervous system. Objective: We intend to provide an update for clinicians on helpful neurophysiological tools to assess movement disorders in clinical practice. Methods: Non-systematic review of the literature published up to June 2019. Results: A diversity of protocols was found and described. These include EMG analyses to define dystonia, myoclonus, myokymia, myorhythmia, and painful legs moving toes pattern; EMG in combination with accelerometer to study tremor; and EEG-EMG to study myoclonus. Also, indirect measures of cortical and brainstem excitability help to describe and diagnose abnormal physiology in Parkinson's disease, atypical parkinsonism, dystonia, and myoclonus. Conclusion: These studies can be helpful for the diagnosis and are usually underutilized in neurological practice.

RESUMO Introdução: Os estudos neurofisiológicos são métodos auxiliares para compreender melhor as características e a natureza dos distúrbios do movimento. A eletromiografia (EMG), em associação com o eletroencefalograma (EEG) e o acelerômetro, podem ser utilizados para avaliar um espectro de movimentos hipo e hipercinéticos. Técnicas específicas podem ser aplicadas para melhor caracterizar a fenomenologia, ajudar a distinguir a origem psicogênica da orgânica e avaliar o local mais provável de geração do movimento no sistema nervoso. Objetivo: Pretendemos fornecer ao clínico uma atualização sobre ferramentas neurofisiológicas úteis para avaliar distúrbios do movimento na prática clínica. Métodos: Revisão não sistemática da literatura publicada até junho de 2019. Resultados: Uma diversidade de protocolos foi encontrada e descrita. Dentre eles, inclui-se o uso de EMG para a definição do padrão de distonia, mioclonia, mioquimia, miorritmia e painfull legs moving toes, além do uso de EMG em associação ao acelerômetro para avaliar tremor e, em associação ao EEG para avaliar mioclonia. Ademais, técnicas para medida indireta de excitabilidade cortical e do tronco encefálico ajudam a descrever e diagnosticar a fisiologia anormal da doença de Parkinson, parkinsonismo atípico, distonia e mioclonia. Conclusão: Esses estudos podem ser úteis para o diagnóstico e geralmente são subutilizados na prática neurológica.

Is it possible to substitute the monofilament test for the Ipswich Touch Test in screening for peripheral diabetic neuropathy?

BACKGROUND: This study aimed to assess the agreement and efficacy of the Ipswich Touch Test compared to the monofilament test in individuals with type 2 diabetes. MATERIALS AND METHODS: A cross-sectional and analytical study was conducted. The inclusion criteria were patients with type II diabetes (n = 250) who did not present ulcers or amputation in either foot. The exclusion criteria were as follows: patients who presented sequelae of cerebrovascular disease or other neurological pathologies, as well as diagnoses of malignancy, alcohol abuse, liver cirrhosis, hepatitis B, AIDS, hypothyroidism, chronic kidney disease or lupus erythaematosus, as these clinical conditions could influence or bias the results (Won and Park in Endocrinol Metab 31:230-238, 2016). Sensitivity, specificity, predictive values, likelihood ratios, and Kappa index were calculated. Other factors assessed were glycated haemoglobin and body mass index. RESULTS: Most of the participants were female (71.2%), and glycated haemoglobin (HbA1c) was greater than 7% in 54.4% of the patients. The mean age was 59.43 years, and the mean time since diagnosis was 12.38 years. The Kappa index was 0.819 (p < 0.001), and the Ipswich Touch Test had a sensitivity of 83.33%, a specificity of 97.66%, a positive predictive value of 85.71%, a negative predictive value of 97.21%, a positive likelihood ratio of 30.19%, and a negative likelihood ratio of 0.17%. The level of significance was 5% in this study. CONCLUSION: The Ipswich Touch Test resented good agreement and efficacy compared to the gold standard-the 10 g monofilament test.