Research progress of diabetes mellitus complicated with chronic inflammatory demyelinating polyneuropathy / 天津医药

In recent years,the results of epidemiological research findings on chronic inflammatory demyelinating polyneuropathy(CIDP)in patients with diabetes mellitus(DM)are quite different,and its correlation is still controversial.Nevertheless,due to the high incidence and treatability of CIDP in DM patients,accurate diagnosis and differentiation of DM-CIDP are of great value for guiding clinical drug use.In terms of clinical manifestations,it can be distinguished by age of onset,disease duration,blood glucose control level and main symptom characteristics.Supplementary diagnostic methods frequently involve evaluating cerebrospinal fluid protein levels,demyelination patterns in electrophysiological tests and neuroimaging studies.Additionally,neuropathological examination and specific serum antibodies can provide more diagnostic support for distinguishing DM-CIDP.Furthermore,a positive response to immunomodulatory and/or immunosuppressive therapies supports the diagnosis of DM-CIDP.By comprehensively evaluating clinical manifestations,electrophysiological characteristics,laboratory tests,neuropathology,imaging,and treatment responses,the accuracy of the differential diagnosis for DM-CIDP can be improved.

Recomendaciones para la inmunización con vacuna recombinante para herpes zóster en pacientes con enfermedades reumáticas inflamatorias crónicas autoinmunes / Recommendations for immunization with recombinant herpes zoster vaccine in patients with autoimmune chronic inflammatory rheumatic diseases

En enero-febrero de 2022 se publicó el último consenso de recomendaciones de vacunas en adultos con enfermedades reumáticas inflamatorias crónicas autoinmunes (ERICA), realizado en conjunto con expertos de la Sociedad Argentina de Reumatología y la Sociedad Argentina de Infectología (SAR/SADI). A modo de addendum del mismo, desarrollamos las recomendaciones para la inmunización con la vacuna recombinante para herpes zóster (VRHZ) en la población de pacientes con ERICA. En el último año, en Argentina, se aprobó la vacuna para el virus varicela zóster, vacuna inactivada de subunidades, recombinante, que contiene la glicoproteína E del virus de la varicela zóster junto con el sistema adyuvante AS0I. La misma demostró una alta eficacia y seguridad en la población inmunocompetente, así como en poblaciones de pacientes con inmunocompromiso. Por este motivo, en conjunto con un grupo de infectólogos expertos, desarrollamos las recomendaciones para el uso de esta vacuna en la población de pacientes con ERICA. Este documento se divide en dos partes. En la primera, figuran las recomendaciones de vacunación en distintos escenarios de presentación habitual en la práctica diaria. Se realizó una revisión bibliográfica y un consenso de expertos para los puntos que contaban con menor evidencia. La segunda parte consiste en seis recomendaciones desarrolladas a través de la metodología GRADE. Se analizó la evidencia en la población de pacientes con ERICA, con o sin tratamiento inmunosupresor, observándose una respuesta de la vacuna VRHZ aceptable, tanto humoral como celular, y demostrando ser segura en esta población de pacientes. Por otro lado, se comprobó una alta eficacia/efectividad en prevenir episodios de herpes zóster y neuralgia posherpética, así como una mayor eficacia/efectividad que la vacuna a virus vivo atenuado. En conclusión, en base a la evidencia disponible, este panel de expertos recomienda la vacuna VRHZ en pacientes con ERICA, independientemente de que hayan recibido previamente la vacuna a virus vivo atenuado contra el herpes zóster, ya que es segura, efectiva y, por el momento, tampoco se asoció a reactivación/brote de la enfermedad de base(AU)

In January-February 2022, the latest consensus on vaccine recommendations in adults with autoimmune chronic inflammatory rheumatic diseases (ACIRD) was published, carried out jointly with experts from the Argentine Society of Rheumatology and the Argentine Society of Infectious Diseases (SAR/SADI). As an addendum to the same, we make recommendations for immunization with the recombinant herpes zoster vaccine (RHZV) in the population of patients with ERICA. In the last year in Argentina, the vaccine for the varicella zoster virus was approved, an inactivated subunit vaccine, a recombinant that contains the glycoprotein E of the varicella zoster virus together with the AS0I adjuvant system. It demonstrated high efficacy and safety in the immunocompetent population, as well as in immunocompromised patient populations. For this reason, together with a group of expert infectologists, we developed recommendations for the use of this vaccine in the population of patients with ACIRD. This document is divided into two parts. The first contains vaccination recommendations in different scenarios of common presentation in daily practice. A bibliographic review and a consensus of experts was carried out for the points that had less evidence. The second part consists of six recommendations developed through the GRADE methodology. The evidence was analyzed in the population of patients with ACIRD, with or without immunosuppressive treatment, observing an acceptable response to the RHZV vaccine, both humoral and cellular, and proving to be safe in this patient population. On the other hand, high efficacy/effectiveness was proven in preventing episodes of herpes zoster and postherpetic neuralgia, as well as greater efficacy/effectiveness than the attenuated virus vaccine. In conclusion, based on the available evidence, this panel of experts recommends the RHZV vaccine in patients with ACIRD, regardless of whether they have previously received the live attenuated virus vaccine, since it is safe, effective and, for the moment, was also not associated with reactivation/outbreak of the underlying disease(AU)

Secondary amyloidosis with concomitant gastric GIST and gastric adenocarcinoma - A case report

Secondary amyloidosis is a well-established entity and has been described in association with chronic inflammatory conditions such as rheumatoid arthritis, ankylosing spondylitis, bronchiectasis, tuberculosis, etc., It has also been reported in association with neoplasms such as Hodgkin's lymphoma, Waldenstrom's macroglobulinemia, renal cell carcinoma, lung carcinoma, etc. However, only a few case reports documenting the association of amyloidosis with gastrointestinal tumor (GIST) and gastric adenocarcinoma are available in the literature. Hereby, we report a case of a 74-year-old male who presented with colicky abdominal pain and vomiting. Ultrasonography revealed a common bile duct (CBD) stone and a small extra-luminal gastric mass. Endoscopic retrograde cholangiopancreatography (ERCP) was performed to remove the CBD stone which revealed an incidental finding of gastric ulcer. A biopsy was taken from the gastric ulcer which on histopathological examination was confirmed as adenocarcinoma leading onto total gastrectomy. During total gastrectomy, an inadvertent injury to the spleen led to simultaneous splenectomy. Multiple samples from the gastric ulcer, the extra-luminal gastric mass, and the spleen were subjected to histopathological examination. Gastric ulcer was confirmed as adenocarcinoma, gastric extra-luminal mass was confirmed as GIST, and splenic examination revealed widespread deposition of amyloid which on Congo-red stain imparted an apple-green birefringence on polarizing microscopy. To the best of our knowledge, this is the first-ever case of such an association where gastric adenocarcinoma occurred with concomitant gastric GIST and secondary amyloidosis of the spleen.

Hidradenitis suppurativa: a review

Hidradenitis suppurativa (HS) is a chronic inflammatory disorder that is characterized by recurrence, as well as the characteristic location of skin lesions. Patients usually develop very painful inflammatory nodules that generally end in the formation of multiple abscesses and fistulas that typically occur in the skin of the axillary, inguinal, buttock, and perianal folds. It significantly affects the quality of life of patients, leaving physical, economic and psychological sequelae. There is a wide therapeutic arsenal available, but each patient must be individualized and the best possible treatment determined. Early assessment and intensive treatment of the disease can prevent and even avoid significant sequelae and permanent deformities.

Diagnostic value of F wave changes in patients with Charcot-Marie-Tooth1A and chronic inflammatory demyelinating polyneuropathy / 北京大学学报(医学版)

OBJECTIVE@#To analyze and compare the characteristics and causes of F wave changes in patients with Charcot-Marie-Tooth1A (CMT1A) and chronic inflammatory demyelinating polyneuropathy (CIDP).@*METHODS@#Thirty patients with CMT1A and 30 patients with CIDP were enrolled in Peking University Third Hospital from January 2012 to December 2018. Their clinical data, electrophysiological data(nerve conduction velocity, F wave and H reflex) and neurological function scores were recorded. Some patients underwent magnetic resonance imaging of brachial plexus and lumbar plexus, and the results were analyzed and compared.@*RESULTS@#The average motor conduction velocity (MCV) of median nerve was (21.10±10.60) m/s in CMT1A and (31.52±12.46) m/s in CIDP. There was a significant difference between the two groups (t=-6.75, P < 0.001). About 43.3% (13/30) of the patients with CMT1A did not elicit F wave in ulnar nerve, which was significantly higher than that of the patients with CIDP (4/30, 13.3%), χ2=6.65, P=0.010. Among the patients who could elicit F wave, the latency of F wave in CMT1A group was (52.40±17.56) ms and that in CIDP group was (42.20±12.73) ms. There was a significant difference between the two groups (t=2.96, P=0.006). The occurrence rate of F wave in CMT1A group was 34.6%±39%, and that in CIDP group was 70.7%±15.2%. There was a significant difference between the two groups (t=-5.13, P < 0.001). The MCV of median nerve in a patient with anti neurofascin 155 (NF155) was 23.22 m/s, the latency of F wave was 62.9-70.7 ms, and the occurrence rate was 85%-95%. The proportion of brachial plexus and lumbar plexus thickening in CMT1A was 83.3% (5/6) and 85.7% (6/7), respectively. The proportion of brachial plexus and lumbar plexus thickening in the CIDP patients was only 25.0% (1/4, 2/8). The nerve roots of brachial plexus and lumbar plexus were significantly thickened in a patient with anti NF155 antibody.@*CONCLUSION@#The prolonged latency of F wave in patients with CMT1A reflects the homogenous changes in both proximal and distal peripheral nerves, which can be used as a method to differentiate the CIDP patients characterized by focal demyelinating pathology. Moreover, attention should be paid to differentiate it from the peripheral neuropathy caused by anti NF155 CIDP. Although F wave is often used as an indicator of proximal nerve injury, motor neuron excitability, anterior horn cells, and motor nerve myelin sheath lesions can affect its latency and occurrence rate. F wave abnormalities need to be comprehensively analyzed in combination with the etiology, other electrophysiological results, and MRI imaging.

Mechanism of Traditional Chinese Medicine in Prevention and Treatment of Ulcerative Colitis： Based on Intestinal Microenvironment / 中国实验方剂学杂志

Ulcerative colitis （UC）， a chronic inflammatory bowel disease affecting the colorectum with high morbidity and prevalence， has become a global burden. However， the causes and pathogenesis are still unclear. Available studies have verified that the imbalance of intestinal microenvironment is crucial in the occurrence and development of UC. Intestinal microenvironment is mainly composed of intestinal microbiota and intestinal mucosal cells， which are involved in the physiological and pathological activities of the body through the intestinal microbial barrier， chemical barrier， mechanical barrier， and immune barrier. Thus， probiotic agents， 5-aminosalicylic acid preparations， corticosteroids， immunosuppressants， biological preparations and other drugs are commonly used in western medicine for the treatment of UC， which， however， have limitations. Therefore， it is the key task for the prevention and treatment of UC to find new therapies. In recent years， it has been found that traditional Chinese medicine （TCM） has unique advantages in the prevention and treatment of UC. Chinese medicine compounds and Chinese medicine monomers can regulate the intestinal microenvironment through multiple pathways and targets， thereby intervening the occurrence and development of UC. It has gradually become a hot spot in the prevention and treatment of UC and attracted extensive attention. Therefore， this study first discussed the correlation between intestinal microenvironment imbalance and UC and then summarized the mechanisms of TCM against UC from the aspects of regulating intestinal flora， improving chemical barrier， protecting mechanical barrier， and inhibiting immune inflammatory response， in order to provide new ideas for the research on TCM in the treatment of UC.

The function and drug development progress of free fatty acid receptor 1 in chronic inflammatory diseases / 药学学报

As a member of G protein coupled-receptors superfamily, free fatty acid receptor 1 (FFAR1), is also known as GPR40, has been shown to regulate numerous pathophysiological processes in a variety of tissues and organs. The activated FFAR1 has a variety of biological functions. For instance, it can not only regulate metabolism of fatty acids and glucose, but also play an important role in immune inflammatory response, it may be a potential drug target for the treatment of various chronic inflammatory diseases. In this review, we focus on the recent researches of FFAR1's action in the regulation of pathophysiological processes, its molecular mechanism and new agonists development. At the same time, this review will take the discovery of series FFAR1 agonists as examples, and display the applied prospects of FFAR1.

Neuronal intranuclear inclusion disease misdiagnosed as CIDP： a case report / 中风与神经疾病杂志

@#A middle-aged male patient，had the chief complaint of recurrent lower limb weakness，and he was misdiagnosed with chronic inflammatory demyelinating polyradiculoneuropathy. Later the patient attended the hospital again due to lower limb weakness，recurrent headache，and nausea and vomiting. The cerebral magnetic resonance imaging showed diffuse restricted high-signal lesions at the bilateral cerebral corticomedullary junctions； immunohistochemical staining showed that inclusion bodies strongly stained with P62 and ubiquitin antibodies were observed in the nuclei of some sweat gland cells，adipocytes，and fibroblasts； genetic testing showed 142 times of the abnormal amplification of GGC in the NOTCH2NLC gene. Therefore，a confirmed diagnosis of neuronal intranuclear inclusion disease （NIID） was made. This case suggests that we should pay attention to whether the central nervous system is involved when peripheral neuropathy is observed.

Research advances in chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a chronic autoimmune disease affecting the peripheral nervous system mediated by cellular and humoral immunity, characterized by limb weakness and sensory impairment. The main feature of CIDP by electrophysiological and pathological examinations is the demyelination of peripheral nerves. First-line treatment for CIDP includes glucocorticoids, intravenous immunoglobulins, and plasmapheresis. Some patients respond to current treatment not well and have a poor prognosis. Progress in the pathogenesis, diagnosis, and treatment of CIDP worldwide was reviewed in this article, aiming to provide references for the clinical diagnosis and treatment of CIDP.

The key points in diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

The diagnosis of chronic inflammatory demyelinating polyradiculoneuropathy relies on clinical features, demyelinating changes on motor and sensory nerve conduction studies, elevated cerebrospinal fluid protein, peripheral nerve image, nerve pathology, and response to immune therapy. Each diagnostic proof should be interpreted in light of clinical background and other findings, to avoid misdiagnosis or overdiagnosis, treatment delay or unnecessary treatment with immunotherapy.

Is cutaneous microbiota a player in disease pathogenesis? Comparison of cutaneous microbiota in psoriasis and seborrheic dermatitis with scalp involvement

Background: Knowledge about cutaneous microbiota in psoriasis vulgaris and seborrheic dermatitis is limited, and a comparison of microbiota in the two diseases was not yet previously undertaken. Aims/Objectives: This study aimed to compare the scalp lesional and non-lesional microbiota in psoriasis vulgaris and seborrheic dermatitis with that in a healthy control group. Methods: Fifty samples were taken with sterile swabs from patients’ and controls’ scalps, and 16S rRNA gene sequencing analyses were performed. Results: Alpha and beta diversity analyses showed that bacterial load and diversity were significantly increased in psoriasis vulgaris and seborrheic dermatitis lesions compared to the controls. As phyla, Actinobacteria decreased and Firmicutes increased, while as genera, Propionibacterium decreased; Staphylococcus, Streptococcus, Aquabacterium, Neisseria and Azospirillum increased in lesions of both diseases. Specifically, Mycobacterium, Finegoldia, Haemophilus and Ezakiella increased in psoriasis vulgaris and Enhydrobacter, Micromonospora and Leptotrichia increased in seborrheic dermatitis lesions. Mycobacterium, Ezakiella and Peptoniphilus density were higher in psoriasis vulgaris compared to seborrheic dermatitis lesions. The bacterial diversity and load values of non-lesional scalp in psoriasis vulgaris and seborrheic dermatitis lay between those of lesional areas and controls. Limitations: The small sample size is the main limitation of this study. Conclusion: Higher bacterial diversity was detected in lesions of both psoriasis and seborrheic dermatitis compared to the controls, but similar alterations were observed when the two diseases were compared. Although these differences could be a result rather than a cause of the two diseases, there is a need to analyze all members of the microbiota and microbiota-host interactions

Chronic inflammatory demyelinating polyneurophaty: assessment of the cognitive function and quality of life / Polineuropatia desmielinizante inflamatória crônica: avaliação da função cognitiva e qualidade de vida

Abstract Background Studies were carried out with the objective of evaluating the quality of life (QoL) of patients affected by chronic inflammatory demyelinating polyradiculopathy (CIDP). However, the cognitive issue is still little addressed. Objectives To assess the QoL and cognitive impairment of patients with CIDP and to analyze whether there is a correlation between these parameters. Methods Seven patients with CIDP and seven paired controls were subjected to: mini-mental state examination (MMSE); Montreal cognitive assessment (MoCA); digit symbol replacement/symbol copy test (DSST); fatigue severity scale (FSS); Beck depressive inventory-I (BDI-I), and a short-form of health survey (SF-36). Results The mean age of the participants was 50 years (71.4% male). The MMSE and MoCA had no statistical difference between the groups. Patients showed superior results in the memory domain in the MoCA (5 vs. 2, p = 0.013). In the DSST, we observed a tendency for patients to be slower. There was a strong negative correlation between fatigue levels and vitality domain (SF-36). There was no significant correlation between depression levels and QoL, and there was no correlation between depression and the results obtained in the cognitive tests. The patients presented higher levels of depression (15.28 vs. 3.42, p < 0.001). A total of 57% had severe fatigue, 28.8% self-reported pain, and 57.1% complained of cramps. Conclusion There was no cognitive impairment in these patients. However, there was a tendency of slower processing speed. To better evaluate the alterations found, a study with a larger number of individuals would be necessary. Chronic inflammatory demyelinating polyradiculopathy affects the QoL of patients in different ways.

Resumo Antecedentes Estudos foram realizados com o objetivo de avaliar a qualidade de vida (QV) de pacientes acometidos pela polirradiculopatia desmielinizante inflamatória crônica (PDIC). No entanto, a questão cognitiva ainda é pouco abordada. Objetivos Avaliar a QV e o comprometimento cognitivo em pacientes com PDIC bem como se existe correlação entre esses parâmetros. Métodos Sete pacientes com PDIC e sete controles pareados foram submetidos a: miniexame do estado mental (MEEM); avaliação cognitiva de Montreal (MoCA); teste de substituição de símbolo de dígito/cópia de símbolo (DSST); escala de gravidade da fadiga (FSS);Beck depressive inventory-I (BDI-I) e um short-form of health survey (SF-36). Resultados A média de idade dos pacientes foi de 50 anos (71,4% do sexo masculino). O MMSE e o MoCA não apresentaram diferença estatística entre os grupos. Os pacientes apresentaram resultados superiores no domínio memória do MoCA (5 vs. 2, p = 0,013). No DSST, observamos uma tendência de os pacientes serem mais lentos. Houve forte correlação negativa entre os níveis de fadiga e o domínio vitalidade (SF-36). Não houve correlação significativa entre níveis de depressão e QV. Não houve correlação entre depressão e os resultados obtidos nos testes cognitivos. Níveis elevados de depressão foram observados nos pacientes (15,28 vs. 3,42, p < 0,001). Um total de 57% apresentou fadiga intensa, 28,8% dor autorreferida, e 57,1% queixam-se de câimbras. Conclusão Não há comprometimento cognitivo nos pacientes estudados. Observamos somente uma tendência de lentificação na velocidade de processamento. Para melhor avaliar as alterações encontradas, será necessário estudo com um número maior de indivíduos. A PDIC afeta de diferentes formas o nível de QV de seus portadores.

Processing speed impairment in chronic inflammatory demyelinating polyneuropathy patients: a cross-sectional study / Prejuízo na velocidade de processamento em pacientes com polineuropatia inflamatória desmielinizante crônica: um estudo transversal

Abstract Background There is a lack of evidence of cognitive involvement in chronic inflammatory demyelinating polyneuropathy (CIDP) and, the reports about the involvement of the brain and central nervous system (CNS) are few and controversial. The Five Digit Test (FDT) evaluates processing speed (PS) and executive functions orally. Objective To evaluate the performance on the FDT of CIDP patients with and without CNS (brain/cerebellum) alterations observed on brain Magnetic Resonance Imaging (MRI) scans. Methods The Hospital Anxiety and Depression Scale (HADS, to assess neuropsychiatry symptoms), the Rasch-built Overall Disability Scale (R-ODS; to assess disability), and the FDT (to assess cognition) were applied to 14 CIDP patients and 24 age-matched healthy control subjects. The patients were submitted to routine brain MRI and, according to the results, they were divided into two groups: those with abnormalities on the MRI (CIDPabnl) and those with normal parameters on the MRI (CIDPnl). The FDT data of five CIDPnl patients and nine CIDPabnl subjects were analyzed. Comparisons between the groups were performed for each task of the FDT. Results We found statistical differences for both groups of CIDP patients in terms of PS, for the patients spent more time performing the PS tasks than the controls. The PS measures were negatively associated with disability scores (reading: r = −0.47; p = 0.003; counting: r = −0.53; p = 0.001). Conclusions Our data suggested the presence of PS impairment in CIDP patients. Disability was associated with slow PS.

Resumo Antecedentes Faltam evidências de envolvimento cognitivo na polineuropatia inflamatória desmielinizante crônica (PIDC), e há poucos e controversos estudos que tratam do envolvimento cerebral e do sistema nervoso central (SNC). O Teste dos Cinco Dígitos (Five Digit Test, FDT, em inglês) avalia a velocidade de processamento (VP) e as funções executivas oralmente. Objetivo Avaliar o desempenho no FDT de pacientes com PIDC com e sem alterações no SNC (cérebro/cerebelo) de acordo com o exame de imagem cerebral por ressonância magnética (RM). Métodos Ao todo, 14 pacientes e 24 controles saudáveis pareados por idade responderam a Escala Hospitalar de Ansiedade e Depressão (que avalia sintomas neuropsiquiátricos), a Escala de Incapacidade Geral elaborada pelo método Rasch (que avalia a incapacidade) e o FDT (que avalia a cognição). Os pacientes foram submetidos a RM cerebral e, de acordo com os resultados, divididos em dois grupos: aqueles com anormalidades (PIDCabnl) e aqueles sem alterações (PIDCnl) na RM. Cinco pacientes PIDCnl e nove PIDCabnl tiveram os dados analisados. Comparações entre os grupos foram realizadas para cada parte do FDT. Resultados Os dois grupos de pacientes foram estatisticamente mais lentos nas tarefas de VP comparados ao grupo controle. As medidas de VP foram negativamente associadas às pontuações de incapacidade (leitura: r = −0,47; p = 0,003; contagem: r = −0,53; p = 0,001). Conclusões Os dados indicaram a presença de prejuízo na VP em pacientes com PIDC. A incapacidade foi associada à lentidão na VP.

Controversias en neuroinmunología: esclerosis múltiple, vacunación, SARS-CoV-2 y otros dilemas / Controversies in neuroimmunology: multiple sclerosis, vaccination, SARS-CoV-2 and other dilemas

La neuroinmunología es una disciplina que cada vez amplía más sus horizontes en la comprensión de las enfermedades neurológicas. Contemporáneamente, y a la luz de los nexos fisiopatológicos de las enfermedades neurológicas y la inmunología, se han planteado enfoques diagnósticos y terapéuticos específicos. A pesar de los importantes avances de esta disciplina, existen múltiples dilemas que le conciernen y se filtran en la práctica clínica. En esta revisión, se presentan y discuten 15 controversias, las cuales se construyen con la información clínica disponible más actualizada. Los temas incluidos son: disminución de esteroides en recaídas de esclerosis múltiple; recomendaciones terapéuticas en esclerosis múltiple a la luz de la pandemia por el SARS-CoV-2; evidencia de vacunación en esclerosis múltiple y en otras enfermedades desmielinizantes; panorama actual del síndrome clínico y radiológico aislado; y fallas terapéuticas en esclerosis múltiple; además, criterios para suspender las terapias modificadoras de la enfermedad; evidencia del manejo en recaídas leves; recomendaciones para la profilaxis contra Strongyloides stercolaris; utilidad de un segundo ciclo de inmunoglobulina en el síndrome de Guillain-Barré; criterios para diferenciar una polineuropatía crónica desmielinizante inflamatoria de inicio agudo de un síndrome de Guillain-Barré y, utilidad de la enzima convertidora de angiotensina en neurosarcoidosis. En cada una de las controversias, se presenta la problemática general y se ofrecen recomendaciones específicas que pueden adoptarse en la práctica clínica diaria.

Neuroimmunology is a discipline that increasingly broadens its horizons in the understanding of neurological diseases. At the same time, and in front of the pathophysiological links of neurological diseases and immunology, specific diagnostic and therapeutic approaches have been proposed. Despite the important advances in this discipline, there are multiple dilemmas that concern and filter into clinical practice. This article presents 15 controversies and a discussion about them, which are built with the most up-to-date evidence available. The topics included in this review are: steroid decline in relapses of multiple sclerosis; therapeutic recommendations in MS in light of the SARS-CoV-2 pandemic; evidence of vaccination in multiple sclerosis and other demyelinating diseases; overview current situation of isolated clinical and radiological syndrome; therapeutic failure in multiple sclerosis, as well as criteria for suspension of disease-modifying therapies; evidence of the management of mild relapses in multiple sclerosis; recommendations for prophylaxis against Strongyloides stercolaris; usefulness of a second course of immunoglobulin in the Guillain-Barré syndrome; criteria to differentiate an acute-onset inflammatory demyelinating chronic polyneuropathy versus Guillain-Barré syndrome; and, the utility of angiotensin-converting enzyme in neurosarcoidosis. In each of the controversies, the general problem is presented, and specific recommendations are offered that can be adopted in daily clinical practice.

The changes in European Academy of Neurology/Peripheral Nerve Society guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

European Academy of Neurology/Peripheral Nerve Society revised the guideline on diagnosis and treatment of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). Typical CIDP and CIDP variants were distinguished. The levels of diagnostic certainty of CIDP and possible CIDP can be defined according to the changes in motor and sensory nerve conduction studies. The utility of cerebrospinal fluid, peripheral nerve image, and nerve biopsy in diagnosis of CIDP was suggested. Serum auto-antibodies, including anti-nodal and paranodal antibodies, anti-myelin-associated glycoprotein antibodies were discussed. Monoclonal protein should be tested routinely in CIDP. Principles for treatment of CIDP were recommended. This guideline updated the development in CIDP and is more suitable for clinical practice.

The electrophysiological features of patients with anti-neurofascin 155 IgG4 antibodies positive chronic inflammatory demyelinating polyradiculoneuropathy / 中华神经科杂志

Objective:To investigate the electrophysiological features of patients with anti-neurofascin 155 (NF155) IgG4 antibodies positive chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).Methods:The electrophysiological data of 6 anti-NF155 IgG4 antibodies positive CIDP patients in Beijing Tiantan Hospital, Capital Medical University from September 2018 to May 2021 were retrospectively analyzed. Parameters studies included standard motor and sensory nerve conduction studies, electromyography (EMG) and F waves studies. Correlation analysis was made to explore the relationships between clinical indicators and electrophysiological data.Results:There was statistically significant difference in the motor nerve conduction study on abnormal rate of tibial nerve (χ 2=11.08, P=0.011). Motor nerve conduction abnormalities were presented in a majority of patients with decreased motor conduction velocity (MCV) and prolonged distal motor latency (DML). There was no statistically significant difference in the overall abnormal rate between lower limbs and upper limbs (30/32, 93.8% vs 22/22, 100.0%; χ 2=1.43, P=0.508), sensory nerve conduction and motor nerve conduction (52/54, 96.3% vs 42/42, 100.0%; χ 2=1.59, P=0.503). There was statistically significant difference in the overall abnormal rate in median nerve, ulnar nerve and sural nerve (χ 2=14.96, P=0.001;χ 2=10.00, P=0.007; χ 2=9.95, P=0.008),and absent sensory nerve action potential was the most common abnormality in sensory nerve conduction abnormalities. The abnormal rate of EMG was 9/14, which was constituted by upper limbs (4/8) and lower limbs (5/6), and the spontaneous activity accompanied with prolonged duration and increased amplitude of motor unit action potential was common. Significant negative correlations were established between disease duration and compound muscle action potential (CMAP) amplitude and negative area of ulnar nerve ( r=-0.84, P=0.036; r =-0.76, P=0.011), and the correlations between age and MCV of median nerve and ulnar nerve were also found ( r=0.89, P=0.019; r=0.95, P=0.003). The DML of median nerve was negatively correlated with CMAP amplitude ( r=-0.63, P=0.049). Moreover, the correlation analysis revealed associations of the F wave latency with the DML, CMAP amplitude and MCV of tibial nerve ( r= 0.90, P=0.039; r=-0.96, P=0.012; r=-0.96, P=0.010). Conclusions:The motor nerve and sensory nerve, the myelin sheath and axon of peripheral nerves in anti-NF155 IgG4 CIDP patients were largely affected. The CMAP amplitude and negative area of ulnar nerve might be of certain clinical value in reflecting the disease duration.The younger the patients, the more severe the demyelination degree of the upper limbs. The F wave latency of tibial nerve not only reflected the abnormal proximal conduction, but also the distal damage degree of myelin sheath and axon.

Antibody detection and clinical characteristics of chronic inflammatory demyelinating polyradiculopathy with anti-nodal/paranodal antibodies / 中华检验医学杂志

Objective:To investigate the detecting method and clinical characteristics of anti-nodal/paranodal antibodies in chronic inflammatory demyelinating polyradiculopathy.Methods:Serum samples were collected from 212 patients with chronic inflammatory demyelinating polyradiculopathy who were admitted to Huashan Hospital of Fudan University or from other clinical centers from January 2018 to July 2021. Autoantibodies (anti-NF155, anti-NF186, anti-CNTN1) and IgG subtypes were detected with cell-based assay. According to the test results, patients were divided into anti-NF155 positive group, anti-NF186 positive group and anti-CNTN1 positive group, clinical characteristics of patients in each group, including limb weakness, superficial sensation and proprioception, tremor, cerebrospinal fluid protein level, brachial plexus magnetic resonance (MRI) were retrospectively analyzed and compared.Results:A total of 23 patients (10.8%,23/212) were positive for anti-NF155 antibody, 12 (5.7%,12/212) for anti-NF186 antibody, and 4 (1.9%,4/212) for anti-CNTN1 antibody. IgG 4 was the predominant subtype in anti-NF155 and anti-CNTN1 groups. In the anti-NF186 group, all cases were IgG positive and antibody subtypes could be detected in 4 cases (4/12). In anti-NF155 group, 23 patients (100%,23/23) had limb weakness and deep sensory disturbance, 19 patients (82.6%,19/23) had superficial sensory disturbance, 22 patients (95.7%,22/23) were symmetrically involved, 18 patients (78.3%,18/23) showed tremor, 19 patients (19/19) showed abnormal in brachial plexus MRI. In anti-NF186 group, 12 patients had limb weakness (12/12), 9 patients (9/12) and 6 patients (6/12) had superficial sensory disturbance and deep sensory disturbance respectively, 8 patients (8/12) were asymmetrically involved, and only 1 patient (1/12) showed tremor, 1 (1/7) showed abnormal brachial plexus MRI. In anti-CNTN1 group, 4 cases showed symmetrical limb weakness and sensory disturbance, 3 patients had tremor, and four patients showed brachial plexus MRI abnormality. There were statistically significant differences in onset age, proprioception, tremor and MRI abnormalities of brachial plexus among the 3 groups ( P<0.01). Conclusions:The clinical characteristics of CIDP patients with anti-NF155, anti-NF186 and anti-CNTN1 antibodies are different. Screening anti-nodal/paranodal antibodies is of great significance for accurate diagnosis and treatment of patients with peripheral neuropathy.

Chronic inflammatory diseases, subclinical atherosclerosis, and cardiovascular diseases: Design, objectives, and baseline characteristics of a prospective case-cohort study ‒ ELSA-Brasil

Abstract Objectives This analysis describes the protocol of a study with a case-cohort to design to prospectively evaluate the incidence of subclinical atherosclerosis and Cardiovascular Disease (CVD) in Chronic Inflammatory Disease (CID) participants compared to non-diseased ones. Methods A high-risk group for CID was defined based on data collected in all visits on self-reported medical diagnosis, use of medicines, and levels of high-sensitivity C-Reactive Protein >10 mg/L. The comparison group is the Aleatory Cohort Sample (ACS): a group with 10% of participants selected at baseline who represent the entire cohort. In both groups, specific biomarkers for DIC, markers of subclinical atherosclerosis, and CVD morbimortality will be tested using weighted Cox. Results The high-risk group (n = 2,949; aged 53.6 ± 9.2; 65.5% women) and the ACS (n=1543; 52.2±8.8; 54.1% women) were identified. Beyond being older and mostly women, participants in the high-risk group present low average income (29.1% vs. 24.8%, p < 0.0001), higher BMI (Kg/m2) (28.1 vs. 26.9, p < 0.0001), higher waist circumference (cm) (93.3 vs. 91, p < 0.0001), higher frequencies of hypertension (40.2% vs. 34.5%, p < 0.0001), diabetes (20.7% vs. 17%, p = 0.003) depression (5.8% vs. 3.9%, p = 0.007) and higher levels of GlycA a new inflammatory marker (p < 0.0001) compared to the ACS. Conclusions The high-risk group selected mostly women, older, lower-income/education, higher BMI, waist circumference, and of hypertension, diabetes, depression, and higher levels of GlycA when compared to the ACS. The strategy chosen to define the high-risk group seems adequate given that multiple sociodemographic and clinical characteristics are compatible with CID.

Colitis ulcerosa: a propósito de un caso pediátrico / Ulcerative colitis: about a pediatric case / Colite ulcerativa: sobre um caso pediátrico

RESUMEN Se presentó el caso de un paciente masculino de 8 años de edad con antecedentes de salud anterior, referido a la consulta externa de Gastroenterología del Hospital Nacional Guido Valadares, de Timor Oriental, por episodios recurrentes de diarreas mucosanguinolentas acompañadas de dolor abdominal en hipogastrio de cuatro meses de evolución. Se realizaron exámenes de laboratorio, ultrasonido abdominal y videocolonoscopia con citología y biopsia de la mucosa del colon. El estudio endoscópico informó una colitis ulcerativa extensa y la histología arrojó el diagnóstico de una enfermedad inflamatoria crónica intestinal del tipo colitis ulcerosa. La colitis ulcerosa es un proceso inflamatorio intestinal de origen desconocido que causa inflamación crónica, difusa y continua, en la mucosa y submucosa. Su incidencia en los niños está aumentando y afecta, incluso, a los lactantes. Se indicó tratamiento dietético y medicamentoso. Actualmente se encuentra asintomático y lleva seguimiento mensual en la consulta de Digestivo.

ABSTRACT The case of an 8-year-old male patient with a previous health history was presented, referred to the Gastroenterology outpatient clinic of the Guido Valadares National Hospital, East Timor, for recurrent episodes of mucosanguineous diarrhea accompanied by abdominal pain in the hypogastrium with four months of evolution. Laboratory tests, abdominal ultrasound and video colonoscopy with cytology and biopsy of the colon mucosa were performed. The endoscopic study reported extensive ulcerative colitis and the histology gave the diagnosis of a chronic inflammatory bowel disease of the ulcerative colitis type. Ulcerative colitis is an inflammatory intestinal process of unknown origin that causes chronic, diffuse and continuous inflammation in the mucosa and submucosa. Its incidence in children is increasing and affects even infants. Dietary and drug treatment was indicated. He is currently asymptomatic and undergoes monthly follow-up in the Digestive Clinic.

RESUMO Foi apresentado o caso de um paciente do sexo masculino, 8 anos, com antecedentes de saúde, encaminhado ao ambulatório de Gastroenterologia do Hospital Nacional Guido Valadares, Timor Leste, por episódios recorrentes de diarreia mucosanguínea acompanhada de dor abdominal em hipogástrio de quatro meses de evolução. Foram realizados exames laboratoriais, ultrassonografia abdominal e videocolonoscopia com citologia e biópsia da mucosa do cólon. O estudo endoscópico relatou colite ulcerativa extensa e a histologia deu o diagnóstico de doença inflamatória intestinal crônica do tipo colite ulcerativa. A colite ulcerosa é um processo inflamatório intestinal de origem desconhecida que causa inflamação crônica, difusa e contínua na mucosa e na submucosa. Sua incidência em crianças está aumentando e afeta até mesmo bebês. Foi indicado tratamento dietético e medicamentoso. Ele atualmente é assintomático e tem acompanhamento mensal na consulta Digestiva.

Eficacia de la movilización de células madre en pacientes con Polineuropatía Desmielinizante Inflamatoria Crónica refractaria / Efficacy of mobilization of stem cells in patients with Refractory Chronic Inflammatory Demyelinating Polyneuropathy (CIDP)

Introducción: La polineuropatía desmielinizante inflamatoria crónica (CIDP) es una enfermedad desmielinizante e inflamatoria de mediación autoinmune. El tratamiento convencional es basado en la inmunomodulación e inmunosupresión. El uso de células madre es una terapia novedosa en los trastornos autoinmune, siendo incluida como terapia. Objetivo: Determinar la eficacia de la movilización de células madre mediante la aplicación del factor estimulador de colonias granulocíticas (F-ECG) en pacientes con CIDP que han recibido otras líneas de tratamiento. Material y Métodos: Se realizó un estudio aleatorizado, doble ciego sobre una cohorte de 45 pacientes con CIDP, donde se administró el (F-ECG) en 25 pacientes y 20 continuaron con el tratamiento habitual, tratados anteriormente con otras variantes terapéuticas por más de tres años, sin respuesta satisfactoria. Resultados: Predominio de los hombres para 64,4 por ciento, la Diabetes Mellitus tuvo mayor asociación y la medicación más usada fueron los esteroides. Los síntomas y signos clínicos mejoraron significativamente tras el tratamiento. Los valores de la puntuación del TCSS al mes y 3 meses después del tratamiento disminuyeron significativamente; pero este decremento no se mantuvo al final del estudio. La velocidad de conducción y el potencial de acción de los nervios sensoriales y motores mejoraron considerablemente después del tratamiento. Conclusiones: La efectividad de la aplicación del (F-ECG) para la mejoría de los síntomas clínicos y resultados de estudios neurofisiológicos evolutivamente son mayores que otras variantes terapéuticas en los primeros meses, con buena seguridad y tolerabilidad, por lo que se puede incluir en la terapéutica convencional para la CIDP(AU)

Introduction: Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune demyelinating disease. Conventional treatment is based on immunomodulation and immunosuppression. The use of stem cells is a novel therapy in autoimmune disorders, so it is included as therapy. Objective: To determine the efficacy of mobilization of stem cells by applying granulocyte colony-stimulating factor (G-CSF) in patients with CIDP who have followed other lines of treatment. Material and Methods: A randomized, double-blind study was carried out on a cohort of 45 patients with CIDP. G-CSF was administered to 25 patients and 20 of them continued with the usual treatment. These patients were previously treated with other therapeutic variants for more than three years without satisfactory response. Results: There was a prevalence of men (64.4 percent), Diabetes Mellitus had a greater association, and the most used medications were steroids. Clinical symptoms and signs improved significantly after treatment. TCSS scores significantly decreased at one and three months after treatment, but this decrease was not maintained at the end of the study. The conduction velocity and action potential of sensory and motor nerves improved considerably after treatment. Conclusions: The effectiveness of the use of G-CSF shows an improvement of clinical symptoms. The results of neurophysiological studies have a better course than other therapeutic variants during the first months, with good safety and tolerability, so it can be included in the conventional therapy for the CIDP(AU)