Abstract Background Tafamidis is a kinetic stabilizer that binds to the transthyretin (TTR) gene, inhibiting its dissociation. It is the only disease-modifying treatment for hereditary TTR amyloidosis with peripheral neuropathy (ATTRv-PN) available in the National Therapeutic Form (Formulário Terapêutico Nacional, FTN, in Portuguese) of the Brazilian Unified Health System (Sistema Único de Saúde, SUS, in Portuguese). Objective To assess if the efficacy and safety of tafamidis in the Brazilian real-world experience are comparable to the results of clinical trials. Methods We retrospectively studied all patients with ATTRv-PN evaluated at our center from September 2011 to March 2022 (data cut-off) who were initiated on tafamidis and had at least 1 follow up visit 6 months after the initiation of the drug treatment. Neurologic and functional outcomes were compared from day 1 (D1) of the tafamidis treatment (baseline) to the last follow-up. Results In total, 33 patients were included, 18 (55%) of whom were female. All patients were carriers of the V30M mutation of ATTRv-PN, and 20 (61%) presented early onset (EO) ATTRv-PN. At baseline, the median age of the sample was of 40 (interquartile range [IQR]: 36–68) years, the median Neuropathy Impairment Score (NIS) was of 10 (6–24) points, and the median body mass index (BMI) was of 26 (23–28) kg/m2. The mean follow-up time was of 3.4 years. At the last follow-up, the BMI, the neurological impairment, and the level of disability slightly worsened compared with baseline, while the findings of the nerve conduction studies remained stable. These same results were observed across EO and late-onset (LO) ATTRv-PN patients. A total of 25 (75.8%) patients were considered responders, and 8 (24.2%), non-responders. Conclusion The efficacy and safety of tafamidis reported in clinical trials is expandable to the Brazilian real-world scenario in EO and late-onset (LO) ATTRv-PN.

ABSTRACT Introduction/framework/objectives Multiple Sclerosis has a reasonable prevalence and the diagnosis is generally made at working age, at a stage that is still demanding on a professional level, a time when there are also generally personal/family particularities that require some dedication. Furthermore, given the incidence, diversity and severity of the symptoms presented, limitations often arise that can seriously affect the ability to work or even lead to unemployment. It is then desirable that the members working in Occupational Health and Safety teams have some knowledge on the subject, in order to better guide their performance, safeguarding the interests of everyone involved. Methodology This is a Bibliographic Review, initiated through a search carried out in April 2023, in the databases “CINALH plus with full text, Medline with full text, Database of Abstracts of Reviews of Effects, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Cochrane Methodology Register, Nursing and Allied Health Collection: comprehensive, MedicLatina and RCAAP”. Content It is a demyelinating and autoimmune disease of the Central Nervous System; it is characterized by recurrent and intermittent episodes of inflammation, with generally progressive destruction of axonal myelin. In most cases there is a cycle of relapses and remissions and symptoms of varying intensity; the evolution is unpredictable. Causes autonomic, visual, motor and sensory symptoms; which makes independent daily living and the ability to work difficult; sometimes becoming debilitating. It is incurable and neurodegenerative. The severity varies depending on the neurological areas most affected. The function of myelin is to facilitate conduction at the level of axons; with changes in this, the electrical impulses that coordinate mental and physical functions become less effective. As myelin deteriorates, it is replaced by scars of abnormal tissue, with different consistency (stiffness, plaques), which further disrupts conduction. Injuries to the frontal and parietal lobes often result in cognitive and emotional changes; in the spinal cord, problems arise more easily in the lower limbs. Changes in the optic nerve and occipital lobe can cause visual problems. This is the neurological desease that causes most limitations in the young adult active population. Given the reasonably young age at which symptoms begin, there will be a decrease in quality of life, more significant than in other chronic diseases. Discussion and Conclusions Although the bibliography is not scarce, the emphasis in the vast majority of articles is not on Occupational Health or Safety. It would be relevant for some teams working in large companies with some prevalence, to evaluate the cases in detail, describing the main problems detected, proposed measures and their subsequent evaluation, in order to publish not only the path, but also the conclusions obtained, for greater dissemination of the subject.

RESUMO Introdução/enquadramento/objetivos A Esclerose Múltipla tem uma prevalência razoável e o diagnóstico faz-se geralmente em idade ativa, numa fase ainda exigente a nível profissional, altura essa onde geralmente também existem particularidades pessoais/familiares que necessitam de alguma dedicação. Para além disso, dada a incidência, diversidade e gravidade dos sintomas apresentados, com frequência surgem limitações que podem condicionar seriamente a capacidade de trabalho ou até levar ao desemprego. Será então desejável que os elementos a exercer em equipas de Saúde e Segurança Ocupacionais tenham alguns conhecimentos sobre o tema, de forma a melhor orientar o seu desempenho, salvaguardando os interesses de todos os envolvidos. Metodologia Trata-se de uma Revisão Bibliográfica, iniciada através de uma pesquisa realizada em abril de 2023, nas bases de dados “CINALH plus with full text, Medline with full text, Database of Abstracts of Reviews of Effects, Cochrane Central Register of Controlled Trials, Cochrane Database of Systematic Reviews, Cochrane Methodology Register, Nursing and Allied Health Collection: comprehensive, MedicLatina e RCAAP”. Conteúdo É uma doença desmielinizante e auto-imune do Sistema Nervoso Central; carateriza-se por episódios recorrentes e intermitentes de inflamação, com destruição de mielina axonal, geralmente progressiva. Na maioria dos casos existe um ciclo de relapsos e remissões e sintomas de intensidade oscilante; a evolução é imprevisível. Causa sintomas autonómicos, visuais, motores e sensoriais; o que dificulta a vida quotidiana independente e o trabalho, tornando-se por vezes debilitante. É incurável e neurodegenerativa. A gravidade varia com as áreas neurológicas mais atingidas. À medida que a mielina se detiora, ela é substituída por cicatrizes de tecido anormal, com diferente consistência (rigidez, placas), o que perturba ainda mais a condução. Lesões nos lóbulos frontal e parietal geralmente resultam em alterações cognitivas e emocionais; na espinal medula, mais facilmente surgem problemas nos membros inferiores. Alterações no nervo ótico e lobo occipital podem originar alterações visuais. Trata-se da doença neurológica que mais limitações causa na população ativa jovem adulta. Dada a idade razoavelmente jovem do início de sintomas, irá ocorrer diminuição da qualidade de vida, mais significativa que noutras doenças crónicas. Discussão e Conclusões Ainda que a bibliografia não seja escassa, a enfase da grande maioria dos artigos não é dada à Saúde ou Segurança Ocupacionais. Seria relevante que algumas equipas a exercer em empresas grandes e com alguma incidência, avaliassem com detalhe os casos, descrevendo os principais problemas detetados, medidas propostas e avaliação posterior das mesmas, de forma a publicar não só o caminho, mas também as conclusões obtidas, para maior divulgação do assunto.

Abstract Introduction Facial trauma can cause damage to the facial nerve, which can have negative effects on function, aesthetics, and quality of life if left untreated. Objective To evaluate the effectiveness of peripheral facial nerve direct end-to-end anastomosis and/or nerve grafting surgery for patients with facial nerve injury after facial trauma. Methods Fifty-nine patients with peripheral facial nerve paralysis after facial injuries underwent facial nerve rehabilitation surgery from November 2017 to December 2021 at Ho Chi Minh City National Hospital of Odontology. Results All 59 cases of facial trauma with damage to the peripheral facial nerve underwent facial nerve reconstruction surgery within 8 weeks of the injury. Of these cases, 25/59 (42.3%) had end-to-end anastomosis, 22/59 (37.3%) had nerve grafting, and 12/59 (20.4%) had a combination of nerve grafting and end-to-end anastomosis. After surgery, the rates of moderate and good recovery were 78.4% and 11.8%, respectively. All facial paralysis measurements showed statistically significant improvement after surgery, including the Facial Nerve Grading Scale 2.0 (FNGS 2.0) score, the Facial Clinimetric Evaluation (FaCE) scale, and electroneurography. The rate of synkinesis after surgery was 34%. Patient follow-up postoperatively ranged from 6 to > 36 months; 51 out of 59 patients (86.4%) were followed-up for at least 12 months or longer. Conclusion Nerve rehabilitation surgery including direct end-to-end anastomosis and nerve grafting is effective in cases of peripheral facial nerve injury following facial trauma. The surgery helps restore nerve conduction and improve facial paralysis. function aesthetics untreated endtoend end andor Fiftynine Fifty nine 201 202 Odontology 5 2559 25 25/5 42.3% 423 42 3 (42.3% 2259 22 22/5 37.3% 373 37 (37.3% 1259 12/5 20.4% 204 20 4 (20.4% 784 78 78.4 118 11 11.8% respectively 2 0 2. FNGS score FaCE (FaCE scale electroneurography 34 34% followup follow up 86.4% 864 86 (86.4% followedup followed 1 longer 255 25/ 42.3 (42.3 225 22/ 37.3 (37.3 125 12/ 20.4 (20.4 7 78. 11.8 86.4 (86.4 42. (42. 37. (37. 20. (20. 11. 86. (86. (42 (37 (20 (86 (4 (3 (2 (8 (

RESUMEN Determinar los valores normales de los parámetros de velocidad de conducción nerviosa (VCN) en residentes de una ciudad localizada a gran altitud. Estudio observacional analítico aplicado a personas residentes en Cusco (ubicado a 3399 m s. n. m.) sin antecedentes comórbidos conocidos. Se evaluó la VCN motora de los nervios mediano, cubital, peroneo común y tibial, así como la VCN sensitiva, latencias y amplitudes de los nervios mediano, cubital y sural. El estudio incluyó 56 participantes, con edad mediana de 24 (22-26) años. Las latencias de los nervios medianos, cubital y tibial anterior fueron menores de 5 ms; y las de los nervios peroneos, mayores de 5 ms. Las amplitudes de los nervios motores fueron superiores a 5 mV y las de los sensitivos estuvieron por encima de los 20 μV. Las velocidades motoras estuvieron por encima de los 50 m/s y las sensitivas fueron mayores a 45 m/s. Se exponen cifras que pueden representar valores de referencia local de VCN obtenidos en población adulta joven residente en localidades de gran altura.

ABSTRACT To determine the normal values of nerve conduction speed (VCN, acronym in Spanish) parameters from people residing in a city localized at high-altitude. Analytical observational study of people without a comorbid history, residing in Cusco, a city located at 3399 meters above sea level. The motor VCN of the median, ulnar, common peroneal, tibial, and the sensory VCN of the median, ulnar, and sural nerves, their latencies and amplitudes, were evaluated. The study included 56 participants, with a median age of 24 (22-26) years. The latencies of the median, ulnar, and anterior tibial nerves were <5 m/s, and of the peroneal nerves were >5 m/s. The amplitudes of the motor nerves were greater than 5mV and the sensory nerves were above 20μV; the motor velocities were greater than 50m/s, and the sensory nerves were greater than 45m/s. Figures are presented reflecting local VCN reference values, obtained from a young adult population residing in high altitude localities.

Abstract Background As a common disabling disease, irreversible neuronal death due to spinal cord injury (SCI) is the root cause of functional impairment; however, the capacity for neuronal regeneration in the developing spinal cord tissue is limited. Therefore, there is an urgent need to investigate how defective neurons can be replenished and functionally integrated by neural regeneration; the reprogramming of intrinsic cells into functional neurons may represent an ideal solution. Methods A mouse model of transection SCI was prepared by forceps clamping, and an adeno-associated virus (AAV) carrying the transcription factors NeuroD1 and Neurogenin-2(Ngn2) was injected in situ into the spinal cord to specifically overexpress these transcription factors in astrocytes close to the injury site. 5-bromo-2´-deoxyuridine (BrdU) was subsequently injected intraperitoneally to continuously track cell regeneration, neuroblasts and immature neurons marker expression, neuronal regeneration, and glial scar regeneration. In addition, immunoprotein blotting was used to measure the levels of transforming growth factor-β (TGF-β) pathway-related protein expression. We also evaluated motor function, sensory function, and the integrity of the blood-spinal cord barrier(BSCB). Results The in situ overexpression of NeuroD1 and Ngn2 in the spinal cord was achieved by specific AAV vectors. This intervention led to a significant increase in cell regeneration and the proportion of cells with neuroblasts and immature neurons cell properties at the injury site(p < 0.0001). Immunofluorescence staining identified astrocytes with neuroblasts and immature neurons cell properties at the site of injury while neuronal marker-specific staining revealed an increased number of mature astrocytes at the injury site. Behavioral assessments showed that the intervention did not improve The BMS (Basso mouse scale) score (p = 0.0726) and gait (p > 0.05), although the treated mice had more sensory sensitivity and greater voluntary motor ability in open field than the non-intervention mice. We observed significant repair of the BSCB at the center of the injury site (p < 0.0001) and a significant improvement in glial scar proliferation. Electrophysiological assessments revealed a significant improvement in spinal nerve conduction (p < 0.0001) while immunostaining revealed that the levels of TGF-β protein at the site of injury in the intervention group were lower than control group (p = 0.0034); in addition, P70 s6 and PP2A related to the TGF-β pathway showed ascending trend (p = 0.0036, p = 0.0152 respectively). Conclusions The in situ overexpression of NeuroD1 and Ngn2 in the spinal cord after spinal cord injury can reprogram astrocytes into neurons and significantly enhance cell regeneration at the injury site. The reprogramming of astrocytes can lead to tissue repair, thus improving the reduced threshold and increasing voluntary movements. This strategy can also improve the integrity of the blood-spinal cord barrier and enhance nerve conduction function. However, the simple reprogramming of astrocytes cannot lead to significant improvements in the striding function of the lower limbs.

Abstract Objectives This study evaluated pain intensity in elderly subjects with hip fractures admitted to the emergency sector and undergoing preoperative pericapsular nerve group (PENG) block. Additionally, the degree of tolerable hip flexion was assessed. Methods A prospective, randomized, and controlled clinical trial with parallel groups. The control group consisted of elderly subjects with hip fractures undergoing standardized intravenous systemic analgesia. The intervention group consisted of elderly patients with hip fractures undergoing PENG block and standardized systemic analgesia. The groups were evaluated at rest and during movement using the Pain Assessment in Advanced Dementia (PAINAD) scale. We determined pain intensity and reduction, in addition to the degree of tolerable flexion of the fractured hip. All patient assessments occurred before the medication or block administration and at 45 minutes, 12, 24, and 36 hours postmedication or block. Results Preoperatively and 24 hours after PENG block, elderly subjects with hip fracture showed a significant reduction in pain at rest or movement compared to control patients (p< 0.05), with 60% of patients assessed at rest demonstrating desirable pain reduction (≥50%) and only 13.3% of the control group achieving the desired pain reduction. During movement, after undergoing PENG block, 40% of subjects demonstrated the desired pain reduction and no patient from the control group. The intervention group also showed a significant improvement in the tolerable hip flexion group (p< 0.05). Conclusion Preoperative PENG block in elderly subjects with hip fractures admitted to the emergency sector provided a significant reduction in pain compared with the control group. (PENG Additionally prospective randomized analgesia PAINAD (PAINAD scale 4 minutes 12 3 2 p< p (p 0.05, 005 0.05 , 0 05 0.05) 60 ≥50% 50 (≥50% 133 13 13.3 40 0.05. . 1 00 0.0 6 ≥50 5 (≥50 13. 0. ≥5 (≥5 ≥ (≥ (

Resumo Objetivos Este estudo avaliou a intensidade da dor em idosos acometidos por fratura do quadril internados no setor de emergência e submetidos ao Pericapsular Nerve Group (PENG) block no pré-operatório. Ademais, o grau de flexão tolerável do quadril foi avaliado. Métodos Ensaio clínico, prospectivo, aleatorizado e controlado em grupos paralelos. O grupo controle consiste em idosos com fratura do quadril, submetidos à analgesia sistêmica endovenosa padronizada. O grupo intervenção consiste em idosos com fratura do quadril submetidos ao PENG block e analgesia sistêmica padronizada. Os grupos foram avaliados em repouso e durante o movimento pela escala de dor Pain Assessment in Advance Dementia (PAINAD). Aferiram-se intensidade da dor e redução álgica, assim como o grau de flexão tolerável do quadril fraturado. Todos os pacientes foram avaliados previamente à administração de medicação ou bloqueio e aos 45 minutos, 12, 24 e 36 horas pós-medicação ou bloqueio. Resultados No pré-operatório e 24 horas após o PENG block, idosos com fratura do quadril apresentaram redução significativa da dor em repouso ou movimento em comparação com o controle (p< 0,05), com 60% dos pacientes avaliados em repouso, demonstrando a redução álgica desejável de ≥ 50% e apenas 13,3% do grupo controle com redução álgica desejável. Durante o movimento, após o PENG block, 40% demonstraram redução álgica desejada e nenhum paciente do grupo controle apresentou a redução desejada. Verificou-se, também, no grupo intervenção a melhora significativa da flexão tolerável do quadril (p < 0,05). Conclusão O PENG block no pré-operatório de idosos com fratura do quadril, internados no setor de emergência, proporcionou redução significativa da dor em comparação ao grupo controle. (PENG préoperatório. préoperatório pré operatório. operatório Ademais avaliado clínico prospectivo paralelos padronizada PAINAD. PAINAD . (PAINAD) Aferiramse Aferiram se fraturado 4 minutos 12 2 3 pósmedicação pós p< p 0,05, 005 0,05 , 0 05 0,05) 60 50 133 13 13,3 40 Verificouse, Verificouse Verificou se, Verificou-se também 0,05. (PAINAD 1 00 0,0 6 5 13, 0,

Abstract Amyloidosis results from deposition of insoluble fibrillar protein compounds in various organs, including the heart. Cardiac involvement occurs in both atria and ventricles, leading to restrictive cardiomyopathy, heart failure (HF), and arrhythmias. Five amyloid proteins affect cardiac tissue: light and heavy chain forms of immunoglobulin, transthyretin (TTR), apolipoprotein 1, and amyloid A. Approximately 95% of cardiac involvement stems from light chain of immunoglobulin and TTR deposition in heart. Chamber stiffness and rhythm disturbances occur in cardiac amyloidosis (CA), encompassing from HF to atrial and ventricular arrhythmias (VA), and both can compromise each other. It is known that conduction system disorders and VA increase sudden death risk, as amyloid infiltration, inflammation, and fibrosis alter myocardial electrophysiology. Beyond the heart, autonomous nerve systems suffer amyloid deposition, contributing to syncope manifestation and increasing the risk of death and poor prognosis. In this context, our review sheds light on the importance of recognizing syncope and rhythm disturbances as crucial markers in the early identification and management of CA, potentially offering avenues for prompt intervention and improved patient outcomes. organs ventricles cardiomyopathy HF, , (HF) tissue TTR, (TTR) 1 A 95 CA (CA) VA, (VA) other infiltration inflammation electrophysiology prognosis context outcomes (HF (TTR 9 (CA (VA

Abstract Background: This study explored the correlation between pancreatic islet α cell function, as reflected by the plasma glucagon levels, and Diabetic Peripheral Neuropathy (DPN) in patients with Type 2 Diabetes Mellitus (T2DM). Methods: A total of 358 patients with T2DM were retrospectively enrolled in this study and divided into the Non-DPN (NDPN) group (n = 220) and the DPN group (n = 138). All patients underwent an oral glucose tolerance test to detect levels of blood glucose, insulin and glucagon, and the Area Under the Curve (AUC) for Glucagon (AUCglu) was used to estimate the overall glucagon level. The Peripheral Nerve Conduction Velocity (PNCV), Amplitude (PNCA) and Latency (PNCL) were obtained with electromyography, and their Z scores were calculated. Results: There were significant differences regarding the age, disease duration, serum levels of alanine aminotransferase, aspartate aminotransferase, urea nitrogen, high-density lipoprotein, and 2h-C peptide between these two groups (p < 0.05). The NDPN group had higher glucagon levels at 30, 60 and 120 min and AUCglu (p < 0.05). The Z-scores of PNCV and PNCA showed an increasing trend (p < 0.05), while the Z-score of PNCL showed a decreasing trend (p < 0.05). The glucagon levels were positively correlated with PNCV and PNCA, but negatively correlated with PNCL, with Gluca30min having the strongest correlation (p < 0.05). Gluca30min was independently related to PNCV, PNCL, PNCA and DPN, respectively (p < 0.05). The function of pancreatic α islet cells, as reflected by the plasma glucagon level, is closely related to the occurrence of DPN in T2DM patients. Conclusion: Gluca30min may be a potentially valuable independent predictor for the occurrence of DPN. Background (DPN T2DM. TDM . T DM (T2DM) Methods 35 NonDPN Non (NDPN n 220 138. 138 138) AUC (AUC (AUCglu level , (PNCV) (PNCA (PNCL electromyography calculated Results age duration aminotransferase nitrogen highdensity high density lipoprotein 2hC hC 2h C h p 0.05. 005 0.05 0 05 0.05) 30 6 12 Zscores 0.05, Zscore score Glucamin Gluca cells Conclusion (T2DM 3 22 13 (PNCV 00 0.0 1 0.

Resumo Antecedentes A síndrome de Guillain-Barré (GBS) é a causa mais comum de paralisia flácida aguda em todo o mundo e pode ser classificada em subtipos eletrofisiológicos e variantes clínicas. Objetivo Este estudo teve como objetivo comparar a frequência do padrão de preservação do sural (SSP) em subtipos e variantes de GBS. Métodos É um estudo de coorte retrospectivo que analisou dados clínicos e eletrofisiológicos de 171 pacientes com GBS internados em hospitais públicos e privados de Natal, Rio Grande do Norte, Brasil, entre 1994 e 2018. Todos os casos foram acompanhados pelo mesmo neurologista em centro de referência em neurologia. Os pacientes foram classificados de acordo com os subtipos eletrofisiológicos e variantes clínicas e a frequência do SSP foi comparada em ambas as categorias. O teste exato de Fisher e a correção de Bonferroni foram utilizados para análise estatística. Resultados O SSP esteve presente em 53% (57 de 107) dos pacientes com polirradiculoneuropatia desmielinizante inflamatória aguda (PDIA), em 8% (4 de 48) dos pacientes com subtipos axonais e em 31% (5 de 16) dos casos não definidos. A frequência do SSP no AIDP foi significativamente maior do que nos subtipos axonais (p < 0,0001); o valor manteve-se elevado após exames eletrofisiológicos seriados. Apenas o subtipo paraparético não apresentou SSP. Conclusão O SSP pode estar presente na PDIA e nos subtipos axonais, incluindo a neuropatia axonal motora aguda, mas está significativamente mais presente na PDIA. Além disso, as variantes clínicas refletem um processo patológico específico e estão correlacionadas ao seu subtipo eletrofisiológico típico, afetando a frequência do SSP. GuillainBarré Guillain Barré (GBS (SSP 17 Natal Norte Brasil 199 2018 neurologia categorias estatística 53 57 107 PDIA, , (PDIA) 8 4 ( 48 31 5 16 definidos p 0,0001 00001 0 0001 0,0001) mantevese manteve se seriados disso típico 1 19 201 10 (PDIA 3 0,000 0000 000 20 0,00 00 2 0,0 0,

Abstract Background Guillain-Barré syndrome (GBS) is the most common cause of acute flaccid paralysis worldwide and can be classified into electrophysiological subtypes and clinical variants. Objective This study aimed to compare the frequency of the sural-sparing pattern (SSP) in subtypes and variants of GBS. Methods This retrospective cohort study analyzed clinical and electrophysiological data of 171 patients with GBS hospitalized in public and private hospitals of Natal, Rio Grande do Norte, Brazil, between 1994 and 2018; all cases were followed up by the same neurologist in a reference neurology center. Patients were classified according to electrophysiological subtypes and clinical variants, and the SSP frequency was compared in both categories. The exact Fisher test and Bonferroni correction were used for statistical analysis. Results The SSP was present in 53% (57 of 107) of the patients with acute inflammatory demyelinating polyradiculoneuropathy (AIDP), 8% (4 of 48) of the patients with axonal subtypes, and 31% (5 of 16) of the equivocal cases. The SSP frequency in the AIDP was significantly higher than in the axonal subtypes (p < 0.0001); the value was kept high after serial electrophysiological examinations. Only the paraparetic subtype did not present SSP. Conclusion The SSP may be present in AIDP and axonal subtypes, including acute motor axonal neuropathy, but it is significantly more present in AIDP. Moreover, the clinical variants reflect a specific pathological process and are correlated to its typical electrophysiological subtype, affecting the SSP frequency. GuillainBarré Guillain Barré (GBS suralsparing sural sparing (SSP 17 Natal Norte Brazil 199 2018 center categories analysis 53 57 107 AIDP, , (AIDP) 8 4 ( 48 31 5 16 p 0.0001 00001 0 0001 0.0001) examinations neuropathy Moreover 1 19 201 10 (AIDP 3 0.000 0000 000 20 0.00 00 2 0.0 0.

ABSTRACT A 34-year-old man developed severe envenomation after being bitten by a Crotalus durissus (rattlesnake), which was treated with anticrotalic serum. Three weeks later, the patient reported paresthesia and neuropathic pain in the left hand, which had progressed to all four limbs. Electroneuromyography revealed asymmetric axonal sensorimotor multiple mononeuropathy. The patient was treated with prednisone, and six months later there was significant improvement in sensorimotor conduction. This is the first reported case of multiple mononeuropathy associated with C. durissus envenomation. Post-snake envenomation peripheral neuropathy is a rare complication requiring prompt recognition and treatment to optimize nerve function and enhance patient outcomes. 34yearold yearold 34 year old rattlesnake, rattlesnake , (rattlesnake) serum hand limbs prednisone conduction C Postsnake Post snake outcomes 3 (rattlesnake

Resumo O schwannoma do nervo facial é uma neoplasia benigna com origem nas células de Schwann do nervo facial. Corresponde a cerca de 5% das paralisias faciais e deve ser incluído no diagnóstico diferencial de uma paralisia facial periférica. Esta neoplasia atinge mais frequentemente os segmentos timpânico e mastoideu do nervo facial. As opções terapêuticas incluem cirurgia, radiocirurgia estereotáxica ou vigilância clínico-imagiológica. Descrevemos o caso de um doente com um quadro agudo de paralisia facial periférica, otalgia e hipoacusia de condução esquerdas. O quadro clínico foi interpretado como uma otite média aguda complicada com paralisia facial com base na história, exame objetivo e achados imagiológicos de urgência. Por persistência das queixas, após uma semana, foi submetido a uma timpanomastoidectomia exploradora que confirmou o diagnóstico de schwannoma do nervo facial. Durante o procedimento foi efetuada uma ressecção subtotal da lesão, que se traduziu numa melhoria clínica e audiológica pós-operatória. Após resseção subtotal, optou-se por vigilância clínica e avaliação imagiológica regulares do resíduo tumoral.

Abstract Facial nerve schwannoma is a benign neoplasm originating from the Schwann cells of the facial nerve. It represents approximately 5% of facial paralysis cases and should be included in the differential diagnosis of peripheral facial paralysis. This neoplasm most frequently affects the tympanic and mastoid segments of the facial nerve. Therapeutic options include surgery, stereotactic radiosurgery or ’wait and scan’. We describe the case of a patient with an acute peripheral facial palsy, otalgia and left conduction hearing loss, that was interpreted as an acute otitis media complicated with facial paralysis based on the history, physical examination and imaging findings. Due to persistence the patient was submitted to an exploratory tympanomastoidectomy which confirmed the diagnosis of facial nerve schwannoma. During the procedure, a subtotal resection of the lesion was performed, which resulted in postoperative clinical and audiological improvement. After subtotal resection, we opted for regular observation and imaging evaluation of the tumor residue.

Abstract Carpal Tunnel Syndrome is a mononeuropathy associated with the entrapment of the median nerve at the level of the carpal joint, in the tunnel that gives it its name, and it is a common and underdiagnosed condition. Currently, a group of techniques are used for its diagnosis, which include electrophysiological tests of nerve conduction velocity, sensory and motor. The objective of this study was to identify the method or combination of electrophysiological methods with the best accuracy in the diagnosis of Carpal Tunnel Syndrome. An analytical observational study of the validity of diagnostic tests was carried out, based on the comparison of distal sensory and motor conduction velocities, in the palm-wrist segment and the electromyography of the patients. It was observed that the best was the combination of latency with sensory conduction velocity and motor conduction velocity in the palm-wrist segment with a sensitivity of 98.62%, specificity of 90%, the positive predictive value was 99.08%. the positive likelihood ratio was 9.8624 and the negative likelihood ratio was 0.0153. For this reason, it is considered that the ideal sequence for diagnosing the condition is to first study the sensory and motor latencies, and if the result is negative, then determine the palm-wrist motor conduction speed.

Resumen El Síndrome del Túnel Carpiano es una mononeuropatía asociada al atrapamiento del nervio mediano a nivel de la articulación carpiana, en el túnel que le da su nombre, y se trata de un padecimiento frecuente y subdiagnosticada. Actualmente se emplean un grupo de técnicas para su diagnóstico, que incluyen pruebas electrofisiológicas de velocidad de conducción nerviosa, sensitiva y motora. El presente estudio tuvo como objetivo identificar el método o combinación de métodos electrofisiológicos con mayor precisión en el diagnóstico del Síndrome del Túnel Carpiano. Se realizó un estudio observacional analítico de validez de pruebas diagnósticas, basado en la comparación de velocidades de conducción sensitiva y motora distal, en el segmento palma muñeca y la electromiografía de los pacientes. Se observó que la mejor fue la combinación de la latencia con la velocidad de conducción sensitiva y la velocidad de conducción motora en el segmento palma-muñeca con una sensibilidad del 98.62%, especificidad del 90%, el valor predictivo positivo fue de 99.08%, la razón de verosimilitud positiva fue de 9.8624 y la razón de verosimilitud negativa 0.0153. Por esta razón se considera que la secuencia ideal para el diagnóstico del padecimiento es el estudio de las latencias sensoriales y motoras en primer lugar, y si el resultado es negativo, entonces determinar la velocidad de conducción motora palma-muñeca.

RESUMEN Desde 2019, se han venido publicando casos de pacientes con COVID-19 que desarrollaron el Síndrome de Guillain-Barré (GBS). Esta revisión explora mecanismos que expliquen fisiopatología, características clínicas, hallazgos de laboratorio y características imagenológicas en estos pacientes. Metodología : Búsqueda bibliográfica de estudios publicados en NCBI y Scielo, entre diciembre de 2019 y abril de 2022. Resultados : Se encontraron noventa artículos, 53 de los cuales se incluyen esta síntesis. No se encontraron estudios que expliquen una asociación entre GBS y COVID-19. Clínicamente, se encontró arreflexia (56.95%), hiporreflexia (19.44%), debilidad muscular (65.28%), alteración de la marcha (12.5%), hipoestesia (26.39%), parestesia (30.55%) y alteración de la micción (6.94%). Los hallazgos en el líquido cefalorraquídeo incluyeron disociación albumino-citológica (66.67%) y un nivel promedio de proteínas de 140.23 mg/dL (DE: 106.71). Algunos casos mostraron realce de las leptomeninges cervicales, tronco encefálico y nervios craneales en tests de resonancia magnética. La variante predominante de GBS fue polineuropatía desmielinizante inflamatoria aguda (56.94%). Los hallazgos en los estudios de conducción nerviosa incluyeron ausencia de ondas F (61.54%), aumento de la latencia motora distal (80%), disminución de la amplitud motora (93.1%) y disminución de la velocidad de conducción motora (75%). Los nervios principalmente involucrados fueron el tibial (20.21%), peroneal (24.47%), mediano (20.21%) y cubital (18.09%). La alteración más frecuente de los nervios craneales fue parálisis facial bilateral (25%) y unilateral (13.89%). Conclusión : La variante primaria del Síndrome de Guillain-Barré (GBS) fue la Polineuropatía Dismielinizante Inflamatoria Aguda. El análisis del líquido cefalorraquídeo reveló una disociación albumino-citológica como el hallazgo más común, y las imágenes en tests de resonancia magnética mostraron incremento de los nervios craneales. Otro hallazgo diferencial fue el menor compromiso del sistema autónomo.

ABSRACT Since 2019, cases of patients with COVID-19 who developed Guillain-Barré Syndrome (GBS) have been reported. This review explores mechanisms that explain pathophysiology, clinical features, laboratory findings, and imaging characteristics in these patients. Methodology : A bibliographic search was made of studies on the topic published in NCBI and Scielo, between December 2019 and April 2022. Results : Ninety articles were found, 53 of which were included in this article. No studies were found that explain an association between GBS and COVID19. Specific clinical manifestations found were areflexia (56.95%), hyporeflexia (19.44%), muscle weakness (65.28%), gait disturbance (12.5%), hypoesthesia (26.39%), paresthesia (30.55%), and micturition disturbance (6.94%). The CSF findings included albumin-cytological dissociation (66.67%), and an average protein level of 140.23 mg/dL (SD: 106.71). Some cases reported enhancement of the cervical leptomeningeal, brainstem and cranial nerves on magnetic resonance imaging tests. The predominant variant of GBS was acute inflammatory demyelinating polyneuropathy (56.94%). The findings in the nerve conduction studies were the absence of F waves (61.54%), increased distal motor latency (80%), decreased motor amplitude (93.1%), and decreased motor conduction velocity (75%). In addition, the nerves mainly involved were the tibial (20.21%), peroneal (24.47%), median (20.21%), and ulnar (18.09%). The most frequent alteration of cranial nerves was bilateral (25%) and unilateral (13.89%) facial palsy. Conclusion : The primary GBS variant was Acute Inflammatory Demyelinating Polyneuropathy. Cerebrospinal fluid analysis revealed albumin-cytological dissociation as the most common finding, and MRI tests showed cranial nerves enhancements. An additional differential feature was the lower commitment of the autonomous system.

Abstract Background The distinction between sensory neuronopathies (SN), which is by definition purely sensory, and sensory polyneuropathies (SP) and sensory multineuropathies (SM) is important for etiologic investigation and prognosis estimation. However, this task is often challenging in clinical practice. We hypothesize that F-wave assessment might be helpful, since it is able to detect subtle signs of motor involvement, which are found in SP and SM, but not in SN. Objective The aim of the present study was to determine whether F-waves are useful to distinguish SN from SP and SM. Methods We selected 21 patients with SP (12 diabetes mellitus, 4 transthyretin familial amyloid polyneuropathy, 4 others), 22 with SM (22 leprosy), and 26 with SN (13 immune-mediated, 10 idiopathic, 3 others) according to clinical-electrophysiological-etiological criteria. For every subject, we collected data on height and performed 20 supramaximal distal stimuli in median, ulnar, peroneal, and tibial nerves, bilaterally, to record F-waves. Latencies (minimum and mean) and persistences were compared across groups using the Kruskal-Wallis and Bonferroni tests. P-values < 0.05 were considered significant. Results All groups were age, gender, and height-matched. Overall, there were no significant between-group differences regarding F-wave latencies. In contrast, F-wave persistence was able to stratify the groups. Peroneal F-wave persistence was higher, bilaterally, in the SN group compared to SM and SP (p < 0.05). In addition, F-waves persistence of the ulnar and tibial nerves was also helpful to separate SN from SP (p < 0.05). Conclusion F-wave persistence of the peroneal nerves might be an additional and useful diagnostic tool to differentiate peripheral sensory syndromes.

Resumo Antecedentes A distinção entre neuronopatias sensitivas (SN) e polineuropatias sensitivas (SP) e multineuropatias sensitivas (SM) é importante para a investigação etiológica e para o prognóstico. Contudo, esta tarefa é desafiadora na prática clínica. Hipotetizou-se que a avaliação das ondas-F pode ser útil, por ser capaz de detectar envolvimento motor nas SP e SM, mas não nas SN. Objetivo Determinar se as ondas-F podem ajudar a distinguir entre SN, SP e SM. Métodos Selecionou-se 21 pacientes com SP (12 diabetes mellitus, 4 ATTR-FAP e 4 com outras neuropatias), 22 com SM (22 hanseníases) e 26 com SN (13 imunomediadas, 10 idiopáticas e 3 com outras neuronopatias), de acordo com critérios clínicos, etiológicos e eletrofisiológicos. Para cada indivíduo, foi aferida a altura e foram aplicados 20 estímulos distais supramáximos nos nervos mediano, ulnar, fibular e tibial, bilateralmente, para registrar as ondas-F. Uma comparação foi feita, por grupo, das latências (mínimas e médias) e persistências pelos testes Kruskal-Wallis e Bonferroni. Valores de p < 0.05 foram considerados estatisticamente significativos. Resultados Todos os grupos foram pareados por idade, sexo e altura. Não houve diferença estatística significativa entre os grupos quanto às latências das ondas-F. A persistência da onda-F foi capaz de estratificar os grupos, sendo as dos nervos fibulares bilateralmente maiores no grupo SN que nos grupos SM e SP (p < 0.05). Adicionalmente, a persistência das ondas-F dos nervos ulnares e tibiais também foi útil para distinguir SN de SP (p < 0.05). Conclusão A persistência das ondas-F dos nervos fibulares pode ser uma ferramenta adicional e útil para diferenciar síndromes sensitivas periféricas.

Resumen Introducción: La hiponatremia se presenta en el 21,5-48% de los pacientes con síndrome de Guillaín-Barré (SGB), sin embargo, existe poca información sobre sus implicaciones clínicas en el SGB. Material y métodos: Se realizó un estudio observacional, transversal, de pacientes con diagnóstico de SGB. Recabamos información de variables clínicas, resultado del LCR y conducción nerviosa. Los pacientes con niveles de sodio < 135 meq/L en estudios de laboratorio al ingreso y antes del inicio de tratamiento fueron considerados con hiponatremia. Definimos buen pronóstico funcional a la presencia de marcha independiente (Hughes ≤ 2 puntos) a los 3 meses de seguimiento. Resultados: De 212 pacientes, 31 (14.6%) presentaron hiponatremia, sexo masculino 72%, edad promedio 45.9 ± 16 años, MRC score 30.1 ± 17 puntos, requerimiento de VMI 32%, presencia de disautonomía 32%. Recabamos ciento ochenta y dos estudios de conducción nerviosa y 165 resultados citoquímicos de punción lumbar. En el análisis comparativo entre los pacientes con hiponatremia vs sin hiponatremia, observamos diferencias significativas: edad (50.4 ± 14,9 vs. 43.8 ± 16.3, p = 0.03), días de hospitalización (mediana) (16 [IQR = 7-51] vs. 7 [IQR = 5-12] p = 0.03), disautonomía (54.8% vs. 28.2%, p = 0.006), niveles de proteínas (mgs/dL) en LCR (mediana) (105 [IQR = 59-165] vs. 39 [IQR = 28-57], p ≤ 0.001) y variante AIDP (70.4% vs. 42%, p = 0.011); En la recuperación de la marcha a los 3 meses, no hubo diferencia significativa (34,6% vs. 42,6%, p = 0.51). Conclusión: 14% de los pacientes con SGB presentan hiponatremia, con formas clínicas más severas, presencia de disautonomía, variante de AIDP más frecuente, niveles elevados de proteínas en LCR y mayor número de días de estancia hospitalaria, pero no afecta el pronóstico funcional.

Abstract Introduction: Hyponatremia occurs in 21-48% of patients with Guillain-Barre syndrome (GBS). However, little is known about the clinical implications in GBS. Materials and methods: In this observational, cross-sectional study, of patients diagnosed with GBS was conducted. Information used included: demographic clinical variables, cerebrospinal fluid (CSF) results, and nerve conduction studies. Patients with sodium levels < 135 meq/L in laboratory studies on admission and before treatment were considered to have hyponatremia. We defined good functional prognosis as the presence of independent gait (Hughes ≤ 2 points) at 3 months of follow-up. Results: Two hundred and twelve patients were initially screen and 31 (14.6%) presented hyponatremia; male gender was predominant with 72%, mean age was 45.9 ± 16 years, mean Modified Medical Research Council (MRC) score was 30.1 ± 17 points, invasive mechanical ventilation requirement was 32%, and the presence of dysautonomia 32%. One hundred and eighty-two nerve conduction studies, and 165 CSF cytochemical samples were collected. In the comparative analyses between the patients with hyponatremia versus without hyponatremia, we observed significant differences in mean age (50.4 ± 14.9 vs. 43.8 ± 16.3, p = 0.03), days of hospitalization (median) (16 [IQR = 7-51] vs. 7 [IQR = 5-12] p = 0.03), dysautonomia (54.8% vs. 28.2%, p = 0.006), protein levels (mgs/dL) in CSF (median) (105 [IQR = 59-165] vs. 39 [IQR = 28-57], p ≤ 0.001), and acute inflammatory demyelinating polyneuropathy (AIDP) variant (70.4% vs. 42%, p = 0.011). There was no significant finding in recovery of independent gait at 3 months (34.6% vs. 42.6%, p = 0.51). Conclusion: About 14% of patients with GBS presented with hyponatremia in our series and is associated with presence of dysautonomia, AIDP variant, elevated CSF protein levels and a greater number of days of hospital stay but did not impact functional prognosis.