Olfactory groove monophasic sinovial sarcoma and von Recklinghausen's disease: A case report and literature review.

Background: Soft-tissue sarcomas are a rare and diverse group of neoplastic lesions. They represent only 1% of malignant tumors in adults and 15% in children. Synovial sarcoma (SS) is a type of soft-tissue sarcoma, accounting for 5-10% of cases, and commonly affecting extremities. Diagnosis, treatment, and prognosis remain challenging especially when localized in uncommon areas, such as intracranial lesions. Case Description: A 13-year-old male patient with a clinical history of neurofibromatosis Type I (NF1) presenting holocranial headache with jet vomiting and apathy 2 days before admission, without neurological deficits and/or focal findings. On magnetic resonance imaging: an extra-axial infiltrative lesion with contrast uptake at the base of the skull in the olfactory groove topography. After total tumor resection, the anatomopathological examination showed monophasic SS. The patient returned after 6 months with similar symptoms, and the lesion recurred and was reoperated. Unfortunately, 7 months after the second surgery, the patient died. Conclusion: SS can occur extraarticulously and with a variable clinical presentation and poor prognosis despite adjuvant therapies with radiotherapy and chemotherapy. In individuals with clinical history of NF1, there is still no direct correlation between the two manifestations, although current descriptions are suggestive of a possible interaction.

Outcomes in Guillain-Barré Syndrome following a second therapeutic cycle - A single-centre retrospective observational study.

INTRODUCTION: The treatment of Guillain-Barré Syndrome (GBS) with intravenous immunoglobulin (IVIg) or plasma exchange (PE) reduces time to clinical recovery. Although sometimes used in clinical practice, the benefit of a second treatment cycle is of unproven benefit. AIMS: Our aim was to compare GBS prognosis in patients treated with one or two cycles of IVIg or PE. METHODS: We selected patients with electrophysiological studies compatible with acute inflammatory demyelinating polyneuropathy or acute motor-sensory axonal neuropathy, from January 2018 to December 2020 in our hospital. Our primary outcome was any improvement in the Guillain-Barré Syndrome Disability Score (GBS-DS) at a mean of twelve weeks. We compared patients treated with one or two treatment cycles with a binary regression. RESULTS: We included twenty-six patients, 65.4% with the classical presentation and 30.8% were treated with two cycles. Patients treated with two cycles presented a higher basal GBS-DS (median 4; IQR 1-5) compared with the group of patients treated with one cycle (median 3; IQR 1-5), p = 0.01. The remaining basal characteristics were similar between groups. The two-cycle treatment regimen did not associate with an improvement in GBS-DS (OR 0.28, 95% CI 0.03-2.35, p = 0.24). Likewise there was no benefit in the need for intensive care unit (OR 2.0, 95% CI 0.37-10.92, p = 0.42) or mechanical invasive ventilation (OR 10.2, 95% CI 0.86-120.96, p = 0.66). DISCUSSION: Our analysis reinforces the recent literature data regarding the absence of benefit of two treatment cycles in patients with GBS.

Quality of life in subjects with upper- and lower-limb spasticity treated with incobotulinumtoxinA.

BACKGROUND: We evaluated quality of life among subjects with upper- and lower-limb spasticity who received escalating doses of incobotulinumtoxinA (total body doses up to 800 U) in the prospective, single-arm, dose-titration TOWER study. METHODS: In this exploratory trial, subjects (N = 155; 18-80 years of age) with upper- and lower-limb spasticity due to cerebral causes who were deemed to require total body doses of up to 800 U incobotulinumtoxinA received three consecutive injection cycles of incobotulinumtoxinA (400, 600, and up to 800 U), each with 12 to 16 weeks' follow-up. QoL was assessed using the EuroQol 5-dimensions questionnaire, three-level (EQ-5D), before and 4 weeks post-injection in each injection cycle and at the end of injection cycle 3. RESULTS: The mean EQ-5D visual analog scale scores of 155 participants continuously improved from study baseline to 4 weeks post-injection in all injection cycles (mean [standard deviation] change 6.7 [14.1], 9.6 [16.3], and 8.6 [17.0] for injection cycles 1, 2, and 3, respectively; p < 0.0001 for all, paired sample t-test). In general, among those with a change in the EQ-5D rating of their condition, the proportion of subjects with 'improvement' was greater than that with 'worsening' for individual EQ-5D dimensions across all injection cycles. At the end of injection cycle 3, the proportion of subjects rating their condition as 'normal' increased from study baseline for all dimensions, and there was a ≥ 46% reduction in the proportion of subjects with a rating of 'severe impairment'. CONCLUSION: These preliminary results suggest that escalating incobotulinumtoxinA doses up to 800 U are associated with improvement in quality of life ratings in subjects with multifocal upper- and lower-limb spasticity, and form a basis for future comparator studies. TRIAL REGISTRATION: ClinicalTrials.gov, NCT01603459. Date of registration: May 22, 2012.

Correction to the article "Contralateral Upper Limb Weakness Following Botulinum Toxin A Injection for Poststroke Spasticity", published on Acta Med Port 2020 Nov;33(11):761-764.

On page 761, in the first sentence of the Introduction, where it reads: "Cardiovascular Botulinum toxin A (BoNT-A) has beenapproved for spasticity management."It should read: "Botulinum toxin A (BoNT-A) has been approved for spasticity management."Article published with errors: https://www.actamedicaportuguesa.com/revista/index.php/amp/article/view/11503.

Contralateral Upper Limb Weakness Following Botulinum Toxin A Injection for Poststroke Spasticity.

Botulinum toxin type A has been approved for spasticity management in poststroke patients. The adverse effects are generally of two types: those related to local injection; and those related to the systemic effects from spread of the toxin. Contralateral weakness after botulinum toxin A treatment is a rarely reported adverse effect. We report the case of a 33-year-old female who had been receiving regular injections of incobotulinum toxin A due to spasticity of the right limbs after a hemorrhagic stroke. A switch was made to abobotulinum toxin A with an overall conversion ratio of 1:3.83. The patient presented contralateral upper limb paresis, especially of the deltoid muscle, in the second week post-injection. The electroneuromyography showed neuromuscular block due to botulinum toxin A. She recovered completely after eight months. A switch between different formulations of botulinum toxin type A should prompt caution when carrying out unit conversions. Distant side effects may appear, including paresis in the contralateral limbs.

A toxina botulínica A foi aprovada para o tratamento da espasticidade em doentes pós-AVC. Os efeitos adversos são geralmente de dois tipos: efeitos adversos relacionados com a administração local de toxina botulínica A; e efeitos adversos sistémicos relacionados com a difusão à distância da toxina. A paresia muscular dos membros contralaterais após tratamento com toxina botulínica A é um efeito adverso raro. Descrevemos o caso de uma mulher de 33 anos de idade que recebia infiltrações regulares de toxina incobotulínica A por espasticidade dos membros direitos pós-AVC hemorrágico. Foi feita uma troca para toxina abobotulínica A com um factor de conversão global de 1:3,83. A doente apresentou parésia do membro superior contralateral, especialmente do músculo deltóide. A electroneuromiografia foi compatível com bloqueio neuromuscular devido a toxina botulínica A. Recuperou totalmente após oito meses. A troca entre diferentes formulações de toxina botulínica A deve exigir precaução na conversão das unidades. Efeitos adversos à distância podem surgir, incluindo parésia dos membros contralaterais.

Screening for Pompe disease in a Portuguese high risk population.

Pompe disease is a rare metabolic disorder with available enzymatic replacement therapy. Contrasting with the classic infantile form, the others subtypes have a heterogeneous presentation that makes an early and accurate diagnosis difficult. We conducted a prospective, multicenter, observational study to identify undiagnosed patients. During a one-year period, patients followed in Portuguese neuromuscular outpatient clinics with proximal muscle weakness affecting upper and/or lower limbs, hyperCKemia in two or more determinations or hypotonia and hyperCKemia, were screened for acid α-glucosidase deficiency by dried blood spots. Lysosomal acid-alpha-1,4-glucosidase activity was determined by tandem mass spectrometry and positive results were confirmed by molecular study. From the 99 patients screened, Pompe disease was confirmed in 4, with age of onset ranging from 2.5 to 48 years, all with limb girdle muscle weakness, corresponding to a frequency of 4% in our cohort and 4.9% of limb girdle muscle weakness. Screening for Pompe disease in high risk populations, using dried blood spots, was already performed in some European populations. Apart from two negative Scandinavian studies, positive cases were confirmed in 2.8-7.9% of patients presenting with limb girdle muscle weakness and in 0-2.5% with isolated hyperCKemia.

Tinnitus and cell phones: the role of electromagnetic radiofrequency radiation / Zumbido e telefones celulares: o papel da radiação eletromagnética de radiofrequência

ABSTRACT INTRODUCTION: Tinnitus is a multifactorial condition and its prevalence has increased on the past decades. The worldwide progressive increase of the use of cell phones has exposed the peripheral auditory pathways to a higher dose of electromagnetic radiofrequency radiation (EMRFR). Some tinnitus patients report that the abusive use of mobiles, especially when repeated in the same ear, might worsen ipsilateral tinnitus. OBJECTIVE: The aim of this study was to evaluate the available evidence about the possible causal association between tinnitus and exposure to electromagnetic waves. METHODS: A literature review was performed searching for the following keywords: tinnitus, electromagnetic field, mobile phones, radio frequency, and electromagnetic hypersensitivity. We selected 165 articles that were considered clinically relevant in at least one of the subjects. RESULTS: EMRFR can penetrate exposed tissues and safety exposure levels have been established. These waves provoke proved thermogenic effects and potential biological and genotoxic effects. Some individuals are more sensitive to electromagnetic exposure (electrosensitivity), and thus, present earlier symptoms. There may be a common pathophysiology between this electrosensitivity and tinnitus. CONCLUSION: There are already reasonable evidences to suggest caution for using mobile phones to prevent auditory damage and the onset or worsening of tinnitus.

RESUMO INTRODUÇÃO: Zumbido é uma condição multifatorial cuja prevalência vem aumentando nas últimas décadas. Em todo o mundo, o aumento progressivo do uso de telefones celulares tem exposto as orelhas a uma maior carga de radiação eletromagnética de radiofrequência (REM-RF). Alguns pacientes com zumbido referem que o uso excessivo do telefone celular, especialmente quando sempre na mesma orelha, é um fator de piora do zumbido ipsilateral. OBJETIVO: O objetivo deste trabalho foi avaliar as evidências disponíveis sobre a possível associação causal entre zumbido e exposição a ondas eletromagnéticas. MÉTODO: Foi realizada uma revisão de literatura com palavras-chave como: tinnitus, electromagnectic field, cellular phone, radiofrequency, electromagnectic hypersensitivity. Foram selecionados 165 artigos com maior relevância clínica em pelo menos um dos assuntos. RESULTADOS: As REM-RF podem penetrar tecidos expostos e existem níveis seguros de exposição. Apresentam efeitos termogênicos comprovados e potenciais efeitos biológicos e genotóxicos. Alguns indivíduos são mais sensíveis à exposição eletromagnética (eletrossensibilidade) e, portanto, adquirem sintomas precocemente. A fisiopatologia da eletrossensibilidade pode ser semelhante à do zumbido. CONCLUSÃO: Consideramos que há evidências suficientes que sugerem maior cautela para o uso desses aparelhos e assim prevenir lesões na via auditiva e o aparecimento/agravamento do zumbido.

Tinnitus and cell phones: the role of electromagnetic radiofrequency radiation.

INTRODUCTION: Tinnitus is a multifactorial condition and its prevalence has increased on the past decades. The worldwide progressive increase of the use of cell phones has exposed the peripheral auditory pathways to a higher dose of electromagnetic radiofrequency radiation (EMRFR). Some tinnitus patients report that the abusive use of mobiles, especially when repeated in the same ear, might worsen ipsilateral tinnitus. OBJECTIVE: The aim of this study was to evaluate the available evidence about the possible causal association between tinnitus and exposure to electromagnetic waves. METHODS: A literature review was performed searching for the following keywords: tinnitus, electromagnetic field, mobile phones, radio frequency, and electromagnetic hypersensitivity. We selected 165 articles that were considered clinically relevant in at least one of the subjects. RESULTS: EMRFR can penetrate exposed tissues and safety exposure levels have been established. These waves provoke proved thermogenic effects and potential biological and genotoxic effects. Some individuals are more sensitive to electromagnetic exposure (electrosensitivity), and thus, present earlier symptoms. There may be a common pathophysiology between this electrosensitivity and tinnitus. CONCLUSION: There are already reasonable evidences to suggest caution for using mobile phones to prevent auditory damage and the onset or worsening of tinnitus.

The molluscicidal activity of niclosamide (Bayluscide WP70(R)) on Melanoides tuberculata (Thiaridae), a snail associated with habitats of Biomphalaria glabrata (Planorbidae).

The aim of this study was to determine the toxicity of niclosamide (Bayluscide (R)) on Melanoides tuberculata and Biomphalaria glabrata under laboratory conditions. The latter species is the intermediate host of Schistosoma mansoni (Sambon 1917). M. tuberculata was successfully used as competitor of B. glabrata in biological control programs in French West Indies. Both molluscicide and biological control using M. tuberculata have proved to be successful in reducing the population density of B. glabrata. The associated use of molluscicide in this area would be an effective measure if M. tuberculata were less susceptibility to the molluscicide than B. glabrata. Three hundreds individuals each of B. glabrata and of M. tuberculata, collected in Sumidouro, State of Rio de Janeiro, were used in the experiment. The molluscs were exposed to 14 different concentrations of niclosamide as recommended by the World Health Organization. Probit analysis was used to determine the LC 50 and LC 90. The LC 50 and LC 90 values for B. glabrata were 0.077 mg/l and 0.175 mg/l, respectively and the LC 50 and LC 90 values for M. tuberculata were 0.082 mg/l and 0.221 mg/l respectively. As the lethal concentrations of niclosamide were approximately the same to both species, this could be a disadvantage when controlling B. glabrata with niclosamide in an area of M. tuberculata occurrence. It might therefore be preferable to utilize the latex extracted from the Euphorbia splendens, which presented a much higher efficiency for B. glabrata than to M. tuberculata.

The molluscicidal activity of niclosamide (Bayluscide WP70®) on Melanoides tuberculata (Thiaridae), a snail associated with habitats of Biomphalaria glabrata (Planorbidae)

The aim of this study was to determine the toxicity of niclosamide (Bayluscide ®) on Melanoides tuberculata and Biomphalaria glabrata under laboratory conditions. The latter species is the intermediate host of Schistosoma mansoni (Sambon 1917). M. tuberculata was successfully used as competitor of B. glabrata in biological control programs in French West Indies. Both molluscicide and biological control using M. tuberculata have proved to be successful in reducing the population density of B. glabrata. The associated use of molluscicide in this area would be an effective measure if M. tuberculata were less susceptibility to the molluscicide than B. glabrata. Three hundreds individuals each of B. glabrata and of M. tuberculata, collected in Sumidouro, State of Rio de Janeiro, were used in the experiment. The molluscs were exposed to 14 different concentrations of niclosamide as recommended by the World Health Organization. Probit analysis was used to determine the LC 50 and LC 90. The LC 50 and LC 90 values for B. glabrata were 0.077 mg/l and 0.175 mg/l, respectively and the LC 50 and LC 90 values for M. tuberculata were 0.082 mg/l and 0.221 mg/l respectively. As the lethal concentrations of niclosamide were approximately the same to both species, this could be a disadvantage when controlling B. glabrata with niclosamide in an area of M. tuberculata occurrence. It migth therefore be preferable to utilize the latex extracted from the Euphorbia splendens, which presented a much higher efficiency for B. glabrata than to M. tuberculata

The molluscicidal activity of the latex of Euphorbia splendens var. hislopii on Melanoides tuberculata (Thiaridae), a snail associated with habitats of Biomphalaria glabrata (Planorbidae)

The use of the latex of Euphorbia splendens var. hislopii was considered as an effective control method for Biomphalaria glabrata in Sumidouro, Rio de Janeiro. However, the appearance and expansion of the snail Melanoides tuberculata since August 1997, with the concomitant reduction of the population of B. glabrata suggest that competitive exclusion might be taking place. Depending on the susceptibility of the thiarid to the E. splendens toxin, the natural control that is occurring could be interrupted by the employment of the latex if the planorbid were less susceptible to the toxin. The aim of this study is to investigate the molluscicidal activity of the latex on M. tuberculata. We used 420 M. tuberculata, from Sumidouro. Fourteen different latex concentrations were tested using World Health Organization general methodology. Probit analysis was used for LD90 and LD50 determination. The LD50 was 3.57 mg/l and LD90 was 6.22 mg/l. At the highest concentration (10 mg/l) there was no survival. No significant differences among replicas (chi2 = 8.31; gl = 13; p > 0.05) were found. The LD90 dose for M. tuberculata was 13.8 times greater than that for B. glabrata, so that the molluscicide in the presence of the thiarid may have a synergic effect on reduction of Biomphalaria populations