Clinical characteristics and management of paroxysmal nocturnal haemoglobinuria in Latin America: a narrative review.

Paroxysmal nocturnal haemoglobinuria (PNH) is a rare, complement-associated, haematological disorder. The level of knowledge about the disease and its management varies around the world. This narrative review provides an overview of available clinical data on PNH in Latin America (LATAM). A search of the PubMed, EMBASE and LILACS/IBECS databases to February 2023, and addition of author-known articles, yielded 24 relevant published articles, the majority (n = 15) from Brazil. Fourteen articles were full papers; 10 were conference abstracts. The prevalence of PNH in Brazil is estimated at 1:237,000 inhabitants. Among blood samples sent for flow cytometry screening for suspected PNH in Brazil and Colombia, 14 - 30% were positive. There is suggestion that disease subtypes may differ among LATAM populations, with classical PNH more common in Brazilian patients and PNH with aplastic anaemia more common in Mexican patients. Median age at diagnosis of PNH ranged from 24 to 41 years. Common symptoms included fatigue, haemoglobinuria, and abdominal pain, although the symptom profile varied by subtype. Three available studies indicated that eculizumab was effective at reducing haemolysis, improving anaemia, and reducing the risk of thrombosis in patients with PNH with intravascular haemolysis. A consensus document from the Brazilian Association of Hematology, Hemotherapy and Cell Therapy RBC and Iron Committee provides guidance on identifying and managing PNH patients, including appropriate selection of patients for eculizumab. Additional data on the epidemiology, natural history and outcomes of patients with PNH in LATAM countries are needed to better understand the disease and its management throughout the region.

[Contribution of the Ibero-American Academy of Neuropediatrics to the knowledge of self-limited epilepsy in infants]. / Aporte de la Academia Iberoamericana de Neuropediatría al conocimiento de la epilepsia autolimitada del lactante.

Since the first presentation at the IV Iberoamerican Academy of Neuropediatrics Congress in 1995, our group has studied self-limited infantile epilepsy (SeLIE), both familial and non-familial, corroborating that they belong to the same entity due to their clinical and electroencephalographic characteristics and excellent prognosis. Associations were found with paroxysmal dyskinesias and migraine, as well as with hemiplegic migraine, episodic ataxia and intellectual disability in atypical cases. Mutations in PRRT2 are the main cause of SeLIE, however, other genes, such as SCN2A, KCNQ2-3 and SCN8A, have been recognized. Drugs for focal seizures that act on sodium channels are indicated. In emergencies, during cluster seizures, the use of benzodiazepines is important. In this publication, we review our contribution in SeLIE from our first report to the present and review the existing literature on the subject.

Desde la primera presentación en el IV Congreso de la Academia Iberoamericana de Neurología Pediátrica en 1995, nuestro grupo ha estudiado las epilepsias autolimitadas del lactante (EAL), tanto familiares y no familiares, corroborando que pertenecen a una misma entidad por sus características clínicas, electroencefalográficas y excelente pronóstico. Se encontraron asociaciones con discinesias paroxísticas y migraña, como también con la migraña hemipléjica, la ataxia episódica y la discapacidad intelectual en casos atípicas. Las mutaciones en PRRT2 son la principal causa de EAL. Sin embargo, otros genes, como SCN2A, KCNQ2-3 y SCN8A, han sido reconocidos. Los fármacos para las crisis focales que actúan sobre los canales de sodio son los indicados. En emergencias, durante las convulsiones agrupadas, es importante el uso de benzodiacepinas. En esta publicación, hacemos un recorrido de nuestro aporte en EAL desde nuestra primera contribución hasta la actualidad y además realizamos una revisión de la literatura existente sobre el tema.

[Epilepsy or functional neurological disorder. Diagnostic strategies]. / Epilepsia o trastorno neurológico funcional. Estrategias para el diagnóstico.

A seizure is the manifestation of symptoms or signs produced by excessive or synchronous neuronal activity in the brain. It usually presents as brief, self-limited episodes of involuntary movements that can affect a part or the entire body and that are sometimes accompanied by loss of consciousness and sphincter control. Epilepsy may be considered after a single unprovoked seizure in a patient with a high risk of recurrence. Paroxysmal non-epileptic disorders are defined as episodes of sudden onset and short duration that imitate an epileptic seizure, caused by a brain dysfunction of diverse origin that, unlike epilepsy, is not due to excessive neuronal discharge. Its incidence is much higher than epilepsy and it can appear at any age. It is important for diagnosis to analyze the triggering factors, the details of each episode, physical examination and only proceed to basic complementary tests such as video-electroencephalogram in case of doubt or for diagnostic confirmation. There is a tendency to overdiagnose epilepsy and excessive use of anticonvulsant drugs. Those that can most frequently be confused are syncope, "daydreams" and pseudoseizures.

Una convulsión es la manifestación de signos o síntomas producidos por una actividad neuronal excesiva o sincrónica en el cerebro. Suele presentarse como episodios breves, autolimitados, de movimientos involuntarios que pueden afectar a una parte del cuerpo o su totalidad y que, en ocasiones, se acompañan de pérdida de la conciencia y control de esfínteres. Puede considerarse epilepsia una sola crisis no provocada en un paciente con un elevado riesgo de recurrencia. Los trastornos paroxísticos no epilépticos se definen como episodios de aparición brusca y de breve duración que imitan a una crisis epiléptica, originados por una disfunción cerebral de origen diverso que a diferencia de la epilepsia no obedecen a una descarga neuronal excesiva. Su incidencia es mucho más elevada que la epilepsia y pueden aparecer a cualquier edad. Es importante para el diagnóstico analizar los factores desencadenantes, los pormenores de cada episodio, examen físico y solamente proceder a los exámenes complementarios básicos como video-electroencefalograma en caso de duda o para confirmación diagnóstica. Existe la tendencia a sobrediagnosticar epilepsia y al uso excesivo de fármacos anticonvulsivos. Los que con mayor frecuencia se pueden confundir son los síncopes, ensoñaciones y las pseudocrisis.

Association between Benign Paroxysmal Positional Vertigo and Thyroid Diseases: Systematic Review and Meta-Analysis.

Introduction Benign paroxysmal positional vertigo (BPPV) is the peripheral vestibular dysfunction that most affects people worldwide, but its etiopathogenesis is still not fully understood. Considering the etiological diversity, some studies highlight the association between BPPV and thyroid diseases. Objective To investigate the association between thyroid diseases and BPPV. Data Synthesis Systematic review and meta-analysis of epidemiological studies searched in the PubMed, Web of Science, Embase, Cochrane Library, and Scopus databases. Studies that were fully available and investigated the association between BPPV and thyroid diseases were selected. The articles that composed the meta-analysis were analyzed using the dichotomous model, the Mantel-Haenszel statistical test, odds ratio (OR), and a 95% confidence interval (CI). Of the 67 articles retrieved from the databases, 7 met the eligibility criteria of the systematic review, and 4 had data necessary to perform the meta-analysis. Qualitative analysis revealed that the studies were conducted in the European and Asian continents. The predominant methodological design was the case-control type, and thyroid dysfunctions, hypothyroidism, and Hashimoto thyroiditis occurred more frequently. The meta-analysis showed no association between hypothyroidism and BPPV; however, there was a statistically significant relationship between Hashimoto thyroiditis and BPPV. Conclusion The meta-analysis results suggest a possible association between BPPV and Hashimoto thyroiditis. Nevertheless, we emphasize the need for further studies to elucidate the evidence obtained.

Pulsed-field ablation versus thermal ablation for atrial fibrillation: A meta-analysis.

Background: Pulsed-field ablation (PFA) is an alternative to thermal ablation (TA) in patients with atrial fibrillation (AF) receiving catheter-based therapy for pulmonary vein isolation (PVI). However, its efficacy and safety have yet to be fully elucidated. Objective: The purpose of this study was to compare the acute and long-term efficacies and safety of PFA and TA. Methods: We performed a systematic review and meta-analysis of randomized and nonrandomized controlled trials comparing PFA and TA in patients with AF undergoing their first PVI ablation. The TA group was divided into cryoballoon (CB) and radiofrequency subgroups. AF patients were divided into paroxysmal atrial fibrillation (PAF) and persistent atrial fibrillation (PersAF) subgroups for further analysis. Results: Eighteen studies involving 4998 patients (35.2% PFA) were included. Overall, PFA was associated with a shorter procedure time (mean difference [MD] -21.68; 95% confidence interval [CI] -32.81 to -10.54) but longer fluoroscopy time (MD 4.53; 95% CI 2.18-6.88) than TA. Regarding safety, lower (peri-)esophageal injury rates (odds ratio [OR] 0.17; 95% CI 0.06-0.46) and higher tamponade rates (OR 2.98; 95% CI 1.27-7.00) were observed after PFA. In efficacy assessment, PFA was associated with a better first-pass isolation rate (OR 6.82; 95% CI 1.37-34.01) and a lower treatment failure rate (OR 0.83; 95% CI 0.70-0.98). Subgroup analysis showed no differences in PersAF and PAF. CB was related to higher (peri)esophageal injury, and lower PVI acute success and procedural time. Conclusion: Compared to TA, PFA showed better results with regard to acute and long-term efficacy but significant differences in safety, with lower (peri)esophageal injury rates but higher tamponade rates in procedural data.

Dopa-responsive dystonia and paroxysmal dystonic attacks associated with ATP1A3 gene variant.

An 18-year-old man had episodes of severe generalised dystonia, from aged 7 months and becoming progressively more frequent. He also had gradually developed interictal limb dystonia. He was initially diagnosed with paroxysmal kinesigenic dyskinesia but he did not improve with several medications. A levodopa trial led to levodopa-induced dyskinetic movements. However, a lower titration of 25 mg of levodopa two times per day substantially improved his motor features and quality of life. Laboratory investigations and MR scans of the brain were unremarkable. Whole-exome sequencing identified a pathogenic variant in the ATP1A3 gene. The ATP1A3-spectrum disorders include non-classical phenotypes such as paroxysmal dystonic attacks. A response to dopamine response is unusual in these disorders. This case highlights the importance of levodopa trials in early-onset dystonia cases.

Role of PIGM and PIGX in glycosylphosphatidylinositol biosynthesis and human health (Review).

Glycosylphosphatidylinositol-glycan (GPI) is an anchor to specific cell surface proteins known as GPI-anchored proteins (APs) that are localized in lipid rafts and may act as cell co-receptors, enzymes and adhesion molecules. The present review investigated the significance of GPI biosynthesis class phosphatidylinositol-glycan (PIG)M and PIGX in GPI synthesis and their implications in human health conditions. PIGM encodes GPI-mannosyltransferase I (MT-I) enzyme that adds the first mannose to the GPI core structure. PIGX encodes the regulatory subunit of GPI-MT-I. The present review summarizes characteristics of the coding sequences of PIGM and PIGX, and their expression in humans, as well as the relevance of GPI-MT-I and the regulatory subunit in maintaining the presence of GPI-APs on the cell surface and their secretion. In addition, the association of PIGM mutations with paroxysmal nocturnal hemoglobinuria and certain types of GPI-deficiency disease and the altered expression of PIGM and PIGX in cancer were also reviewed. In addition, their interaction with other proteins was described, suggesting a complex role in cell biology. PIGM and PIGX are critical genes for GPI synthesis. Understanding gene and protein regulation may provide valuable insights into the role of GPI-APs in cellular processes.

Facial representation of paroxysmal hemicrania and associated temporomandibular disorder: a case report / Hemicrania paroxística com representação na face e associação com disfunção temporomandibular: relato de caso

ABSTRACT Paroxysmal Hemicrania is a trigeminal autonomic cephalalgia described as a severe and strictly unilateral pain, which occurs in paroxysms at orbital, supraorbital and/ or temporal region. A 45-year-old woman presented to an orofacial pain specialist reporting severe, burning, throbbing, strictly right-sided headache associated to ipsilateral autonomic symptoms and orofacial pain. The pain was perceived on the maxillary region followed by pain spread to the head. Interdisciplinary evaluation, along with absolute responsiveness to indomethacin and normal Brain Magnetic Resonance imaging, led to the diagnosis of primary Episodic paroxysmal hemicrania with facial representation and myofascial pain of masticatory muscles. Dentists should be aware of paroxysmal hemicrania with facial representation and the possibility of temporomandibular disorder coexistence, in order to avoid misdiagnosis and inadequate management. Paroxysmal hemicrania may be first perceived on the face and may be associated with interparoxysmal pain. In these cases, efficient anamnesis and clinical evaluation followed by interdisciplinary approach is needed.

RESUMO A Hemicrania Paroxística é uma cefalalgia autonômica trigeminal descrita como uma dor severa e estritamente unilateral, que ocorre em paroxismos na região orbital, supraorbital e/ou temporal. Uma mulher de 45 anos de idade apresentou-se a um especialista em dor orofacial, referindo uma cefaleia intensa, ardente, latejante, estritamente do lado direito, associada a sintomas autonómicos ipsilaterais e dor orofacial. A dor era sentida na região maxilar, seguida de dor que se estendia à cabeça. A avaliação interdisciplinar, aliada à resposta absoluta à indometacina e à normalidade em ressonância magnética cerebral, levou ao diagnóstico de hemicrania paroxística episódica primária com representação facial e dor miofascial dos músculos mastigatórios. Os médicos dentistas devem estar atentos à hemicrania paroxística com representação facial e à possibilidade de coexistência de Disfunção Temporomandibular, de modo a evitar diagnósticos incorretos e um tratamento inadequado. A hemicrania paroxística pode ser percebida inicialmente na face e pode estar associada à dor interparoxística. Nesses casos, é necessária uma anamnese e avaliação clínica eficientes, seguidas de abordagem interdisciplinar.

Doble tormenta tiroidea y arrítmica en una gestante con múltiples taquicardias refractarias mediadas por un haz de kent intermitente ablacionado exitosamente

La tormenta tiroidea es un estado crítico y poco frecuente que condiciona la disfunción de múltiples órganos por el efecto del exceso de las hormonas tiroideas, esta disfunción endócrina tiene una elevada mortalidad y genera manifestaciones típicas como la taquicardia, fiebre, alteraciones gastrointestinales, cardiovasculares y del sistema nervioso central. El embarazo se ha asociado con un incremento en la incidencia de arritmias. Necesitan un tratamiento inmediato con drogas antiarrítmicas, cardioversión eléctrica o cesárea de urgencia. El WPW es una anormalidad cardiaca congénita que consiste en la presencia de un haz anómalo (Haz de Kent) que evita el sistema normal de conducción uniendo directamente aurículas y ventrículos. Veremos el caso de una gestante de 32 semanas que presenta un cuadro de tormenta tiroidea y múltiples episodios de taquicardia paroxística supraventricular (TPS), de tórpida y sombría evolución clínica mediada por un haz anómalo de Kent intermitente. Es evidente que la tormenta tiroidea en el contexto de la gestación produjo cambios en las propiedades electrofisiológicas del haz anómalo de Kent intermitente lo cual propició el desarrollo de múltiples taquicardias paroxísticas supraventriculares refractarias a la cardioversión eléctrica y farmacológica. Tampoco mejoró con la tiroidectomía total, solamente cedió por completo con la ablación por catéter de radiofrecuencia del haz anómalo de Kent.

Thyroid storm is a critical and infrequent state that conditions the dysfunction of multiple organs due to the effect of excess thyroid hormones. This endocrine dysfunction has a high mortality and generates typical manifestations such as tachycardia, fever, gastrointestinal, cardiovascular and heart disorders, and the central nervous system. Pregnancy has been associated with an increased incidence of arrhythmias. They need immediate treatment with antiarrhythmic drugs, electrical cardioversion, or emergency caesarean section. WPW is a congenital cardiac abnormality that consists of the presence of an abnormal bundle (Kent bundle) that prevents the normal conduction system, directly joining the atria and ventricles. We will see the case of a 32-week pregnant woman who presented symptoms of thyroid storm and multiple episodes of paroxysmal supraventricular tachycardia (PST), with a torpid clinical course mediated by an abnormal intermittent Kent bundle. It is evident that the thyroid storm in the context of pregnancy produced changes in the electrophysiological properties of the intermittent Kent bundle, which led to the development of multiple PST refractory to electrical and pharmacological cardioversion. Moreover, it also did not improve with total thyroidectomy, only resolved completely with radiofrequency catheter ablation of the Kent bundle.

Prevalencia de hipofunción vestibular en el vértigo postural paroxístico benigno post traumatismo craneoencefálico / Prevalence of vestibular hypofunction in benign paroxysmal postural vertigo after head trauma

Introducción: El traumatismo craneoencefálico (TCE) puede generar vértigo, mareo e inestabilidad. Posibles causas otorrinolaringológicas son el vértigo postural paroxístico benigno (VPPB) que constituye el diagnóstico más frecuente, y la hipofunción vestibular. Objetivo: Describir la prevalencia de hipofunción vestibular en un grupo de pacientes con VPPB asociado a TCE. Material y Método: Estudio retrospectivo de pacientes con VPPB asociado a TCE que requirieron maniobra de reposición (MRP) entre los años 2017 y 2021. La información clínica, características clínico-demográficas, hallazgos en pruebas de función vestibular y número de MRP fueron evaluados. Resultados: Se incluyeron 48 pacientes con una edad promedio de 60,8 ± 16,5 años, siendo un 52% mujeres. La prevalencia de pacientes con paresia vestibular concomitante correspondió al 35,4%. Al comparar al grupo con y sin paresia se observó: (1) en el grupo con paresia fue, significativamente, más frecuente presentar contusión cerebral asociada, 47,1% vs 12,9%; (2) el sexo masculino fue, significativamente, más frecuente en el grupo con paresia, 70,59% vs 35,5%; (3) en ambos grupos, la mediana de MRP fue 1. Conclusión: La presencia de paresia vestibular en pacientes con VPPB secundario a TCE, no es un hallazgo infrecuente, en nuestro estudio, correspondió a un 35,4%, siendo este más frecuente en hombres. Adicionalmente, la contusión cerebral asociada es más frecuente en el grupo con paresia.

Introduction: Head trauma can generate vertigo, dizziness and instability. Possible otorhinolaryngologic causes are benign paroxysmal postural vertigo (BPPV), which is the most frequent diagnosis, and vestibular hypofunction. Aim: To describe the prevalence of vestibular hypofunction in a group of patients with BPPV associated with head trauma. We studied the clinical characteristics, vestibular function test findings and the number of (PRM). Material and Method: Retrospective study of patients with BPPV associated with head trauma who underwent particle repositioning maneuvers (PRM) during the years 2017 to 2021. Clinical characteristics, vestibular function test findings and the number of PRM were evaluated. Results: 48 patents were included. The mean age was 60.8 ± 16.5 years old, 52% were women. The prevalence of patients with concomitant vestibular paresis was 35.4%. When comparing the groups with and without paresis the following was observed: (1) associated brain contusions were significatively more frequent in the paresis group, 47.1% vs 12.9%; (2) male sex was significatively more frequent in the paresis group, 70.59% vs 35.5%; (3) in both groups, the median of needed PRM was 1. Conclusion: The presence of vestibular paresis in patients with BPPV secondary to head trauma is not an infrequent finding. In our study, its prevalence was 35.4%, being significatively more frequent in men. Also, associated brain contusions were significatively more frequent in the paresis group.

Canalith jam del horizontal: Revisión y reporte de dos casos / Horizontal canalith jam: Review and two cases report

El vértigo posicional paroxístico benigno (VPPB) es un síndrome vestibular episódico (SVE) que es reconocido por ser el trastorno más frecuente observado en la clínica, siendo de buena y pronta resolución en la gran mayoría de los casos. Sin embargo, pueden presentarse variantes muy poco habituales o atípicas, donde el canalith jam es una de las formas más resistentes al tratamiento mediante maniobras de reposición, y por lo mismo, el reconocimiento adecuado de este cuadro es esencial para su correcto abordaje. Se presentan dos casos de VPPB con canalith jam en el CSC horizontal y se proponen cinco criterios diagnósticos para su identificación.

Benign paroxysmal positional vertigo (BPPV) is an episodic vestibular syndrome (EVS) that is recognized for being the most frequent disorder observed in the clinic, with good and prompt resolution in the vast majority of cases. However, very unusual or atypical variants can occur, where the canalith jam is one of the forms most resistant to treatment by means of repositioning maneuvers, and for the same reason, the adequate recognition of this condition is essential for its correct approach. Two cases of BPPV with canalith jam in the horizontal semicircular canal and five diagnostic criteria for its identification are presented.

Geriatric benign paroxysmal positional vertigo: a single-center study.

OBJECTIVE: To compare the clinical features, risk factors, distribution of Benign Paroxysmal Positional Vertigo (BPPV) subtypes, and effectiveness of canalith repositioning between geriatric and non-geriatric patients with BPPV. METHODS: A total of 400 patients with BPPV were enrolled. Canalith repositioning was performed according to the semicircular canals involved. Patients were divided by age into a geriatric group (≥60 years) and a non-geriatric group (20-59 years). Clinical characteristics, potential age-related risk factors, distribution of subtypes, and effectiveness of canalith repositioning were compared between the groups. RESULTS: Female sex was significantly more common in all age groups, with a peak female-to-male ratio of 5.1:1 in the group aged 50-59 years. There was a higher proportion of men in the geriatric group. A history of disease associated with atherosclerosis was significantly more common in the geriatric group (p < 0.05). Migraine was significantly more common in the non-geriatric group (p = 0.018), as was posterior canal BPPV. The horizontal canal BPPV (especially horizontal canal BPPV-cupulolithiasis), and multicanal BPPV subtypes were more common in the geriatric group, whereas anterior canal BPPV was more common in the non-geriatric group. Two canalith repositioning sessions were effective in 58.0% of the geriatric cases and in 72.6% of the non-geriatric cases (p = 0.002). There was a tendency for the effectiveness of canalith repositioning to decrease with increasing age. CONCLUSION: BPPV was more common in women. However, the proportion of men with BPPV increased with age. Elderly patients often had a history of diseases associated with atherosclerosis (i.e., hypertension, diabetes, and hyperlipidemia). The horizontal canal BPPV (particularly horizontal canal BPPV-cupulolithiasis) and multicanal BPPV subtypes were more common and the anterior canal BPPV subtype was less common in elderly patients. The effectiveness of canalith repositioning may decrease with age. Therefore, older patients should receive more comprehensive medical treatment.

Assessment of short forms of recurrent atrial extra systoles by echocardiography with left atrial strain in ambulatory patients without organic cardiopathy / Evaluación de formas cortas de extrasístoles auriculares recurrentes mediante ecocardiografía con deformación auricular izquierda en pacientes ambulatorios sin cardiopatía orgánica

Abstract Aim: To analyse the potential usefulness and clinical relevance of the assessment by echocardiography with left atrial strain, based on the myocardial atrial deformation curves with speckle-tracking velocity vector imaging (VVI), in the analysis of short-form recurrent atrial extra systoles in ambulatory patients not suffering from organic cardiopathy. Methods: We designed a descriptive, prospective, and observational study including 270 patients between the ages of 18 and 75 assessed during an outpatient cardiology consultation attended due to palpitations over a period of two years. Using ambulatory electrocardiographic monitoring, we selected cases with short forms of repetitive atrial extrasystole, isolated or recurrentatrial fibrillation and a control group formed by those patients without repetitive ectopia. All patients underwent a thorough echocardiographic study during their first cardiological visit. Results: The analysis of the dynamic curves segmental deformation generated after an atrial extrasystole can reveal different points of origin of the extrasystole and detect specific anatomical alterations in the interatrial conduction at the level of the Bachmann's fascicle showing different models of electro anatomical activation possibly involved in the appearance of repetitive forms. Higher values of dyssynchrony between the septal and lateral wall and elongation in the time of interatrial electromechanical conduction could also be related to the existence of repetitive ectopic beats. Conclusions: Our ambulatory study employing the left atrial longitudinal strain, particularly in its segmental analysis, provides new insights into its the usefulness and potential clinical relevance.

Resumen Objetivo: Analizar la utilidad y relevancia clínica de la evaluación mediante ecocardiografía basada en las curvas de deformación auricular miocárdica con imágenes vectoriales de velocidad (VVI) de speckle-tracking, en el análisis de las extrasístoles auriculares recurrentes de corta duración en pacientes ambulatorios sin cardiopatía orgánica. Métodos: Se diseñó un estudio descriptivo, prospectivo y observacional que incluyó a 270 pacientes de entre 18 y 75 años evaluados durante una consulta externa de cardiología a la que acudieron por palpitaciones durante un periodo de dos años. Mediante el uso de monitorización electrocardiográfica ambulatoria, se seleccionaron casos con formas cortas de extrasistolia auricular repetitiva, fibrilación auricular aislada o repetitiva y un grupo control formado por aquellos pacientes sin ectopia repetitiva. Todos los pacientes se sometieron a un estudio ecocardiográfico exhaustivo durante su primera visita cardiológica. Resultados: El análisis de las curvas dinámicas de deformación segmentaria generadas tras un extrasístole auricular diferentes modelos de activación electroanatómica posiblemente implicados en la aparición de formas repetitivas. Valores mayores de disincronía entre la pared septal y lateral y el alargamiento en el tiempo de conducción electromecánica intraauricular pudieran también relacionarse con la existencia de latidos ectópicos repetitivos. Conclusiones: Nuestro estudio ambulatorio empleando la deformación longitudinal auricular izquierda, particularmente en su análisis segmentario, proporciona nuevas perspectivas sobre su utilidad y potencial relevancia clínica.

Assessment of Saudi Physicians and Physical Therapists' Adherence to the Updated Clinical Practice Guideline for Benign Paroxysmal Positional Vertigo: A Cross-Sectional Study

Abstract Introduction Variations in clinical practice regarding the management of benign paroxysmal positional vertigo (BPPV) among clinicians have been noted in previous studies. Such variations might be related to the different adherence to clinical practice guidelines. Objective To evaluate clinicians' adherence to BPPV guidelines and investigate the variations in the adherence between different specialties and qualifications. Methods This is a cross-sectional study with a vignettes-based survey conducted between June and August, 2020. We included clinicians engaged in managing BPPV that had at least one year of clinical experience. We excluded students, and clinicians who were not involved in the management of individuals with BPPV. Participants were asked to make their management choices based on four hypothetical patient vignettes. The sample ranged from 77 participants for the first vignette to 45 participants for the last vignette. Results We included 77 clinicians in the study, with the majority being Otolaryngologists (31.2%). The respondents' mean adherence to the guideline was of 63.3%. Result showed that Otolaryngologists' adherence was higher than that of clinicians from different specialties (p = 0.006, d = 0.72). Furthermore, clinicians with a postgraduate degree were more likely to adhere than those with a bachelor's degree only (p = 0.014, d = 0.58) and participants who were aware of the guideline were more likely to adhere to it (p < 0.001, d = 1.05). Lastly, regression analysis exhibited that adherence was affected by postgraduate degree and guideline awareness. Conclusion Otolaryngologists were more likely to adhere to the guideline than other specialties. Among all specialties, higher adherence was associated with guideline awareness and postgraduate degrees.

Assessment of Saudi Physicians and Physical Therapists' Adherence to the Updated Clinical Practice Guideline for Benign Paroxysmal Positional Vertigo: A Cross-Sectional Study.

Introduction Variations in clinical practice regarding the management of benign paroxysmal positional vertigo (BPPV) among clinicians have been noted in previous studies. Such variations might be related to the different adherence to clinical practice guidelines. Objective To evaluate clinicians' adherence to BPPV guidelines and investigate the variations in the adherence between different specialties and qualifications. Methods This is a cross-sectional study with a vignettes-based survey conducted between June and August, 2020. We included clinicians engaged in managing BPPV that had at least one year of clinical experience. We excluded students, and clinicians who were not involved in the management of individuals with BPPV. Participants were asked to make their management choices based on four hypothetical patient vignettes. The sample ranged from 77 participants for the first vignette to 45 participants for the last vignette. Results We included 77 clinicians in the study, with the majority being Otolaryngologists (31.2%). The respondents' mean adherence to the guideline was of 63.3%. Result showed that Otolaryngologists' adherence was higher than that of clinicians from different specialties ( p = 0.006, d = 0.72). Furthermore, clinicians with a postgraduate degree were more likely to adhere than those with a bachelor's degree only ( p = 0.014, d = 0.58) and participants who were aware of the guideline were more likely to adhere to it ( p < 0.001, d = 1.05). Lastly, regression analysis exhibited that adherence was affected by postgraduate degree and guideline awareness. Conclusion Otolaryngologists were more likely to adhere to the guideline than other specialties. Among all specialties, higher adherence was associated with guideline awareness and postgraduate degrees.

Assessment of short forms of recurrent atrial extra systoles by echocardiography with left atrial strain in ambulatory patients without organic cardiopathy.

Aim: To analyse the potential usefulness and clinical relevance of the assessment by echocardiography with left atrial strain, based on the myocardial atrial deformation curves with speckle-tracking velocity vector imaging (VVI), in the analysis of short-form recurrent atrial extra systoles in ambulatory patients not suffering from organic cardiopathy. Methods: We designed a descriptive, prospective, and observational study including 270 patients between the ages of 18 and 75 assessed during an outpatient cardiology consultation attended due to palpitations over a period of two years. Using ambulatory electrocardiographic monitoring, we selected cases with short forms of repetitive atrial extrasystole, isolated or recurrentatrial fibrillation and a control group formed by those patients without repetitive ectopia. All patients underwent a thorough echocardiographic study during their first cardiological visit. Results: The analysis of the dynamic curves segmental deformation generated after an atrial extrasystole can reveal different points of origin of the extrasystole and detect specific anatomical alterations in the interatrial conduction at the level of the Bachmann's fascicle showing different models of electro anatomical activation possibly involved in the appearance of repetitive forms. Higher values of dyssynchrony between the septal and lateral wall and elongation in the time of interatrial electromechanical conduction could also be related to the existence of repetitive ectopic beats. Conclusions: Our ambulatory study employing the left atrial longitudinal strain, particularly in its segmental analysis, provides new insights into its the usefulness and potential clinical relevance.

Objetivo: Analizar la utilidad y relevancia clínica de la evaluación mediante ecocardiografía basada en las curvas de deformación auricular miocárdica con imágenes vectoriales de velocidad (VVI) de speckle-tracking, en el análisis de las extrasístoles auriculares recurrentes de corta duración en pacientes ambulatorios sin cardiopatía orgánica. Métodos: Se diseñó un estudio descriptivo, prospectivo y observacional que incluyó a 270 pacientes de entre 18 y 75 años evaluados durante una consulta externa de cardiología a la que acudieron por palpitaciones durante un periodo de dos años. Mediante el uso de monitorización electrocardiográfica ambulatoria, se seleccionaron casos con formas cortas de extrasistolia auricular repetitiva, fibrilación auricular aislada o repetitiva y un grupo control formado por aquellos pacientes sin ectopia repetitiva. Todos los pacientes se sometieron a un estudio ecocardiográfico exhaustivo durante su primera visita cardiológica. Resultados: El análisis de las curvas dinámicas de deformación segmentaria generadas tras un extrasístole auricular diferentes modelos de activación electroanatómica posiblemente implicados en la aparición de formas repetitivas. Valores mayores de disincronía entre la pared septal y lateral y el alargamiento en el tiempo de conducción electromecánica intraauricular pudieran también relacionarse con la existencia de latidos ectópicos repetitivos. Conclusiones: Nuestro estudio ambulatorio empleando la deformación longitudinal auricular izquierda, particularmente en su análisis segmentario, proporciona nuevas perspectivas sobre su utilidad y potencial relevancia clínica.

Geriatric benign paroxysmal positional vertigo: a single-center study

Abstract Objective To compare the clinical features, risk factors, distribution of Benign Paroxysmal Positional Vertigo (BPPV) subtypes, and effectiveness of canalith repositioning between geriatric and non-geriatric patients with BPPV. Methods A total of 400 patients with BPPV were enrolled. Canalith repositioning was performed according to the semicircular canals involved. Patients were divided by age into a geriatric group (≥60 years) and a non-geriatric group (20-59 years). Clinical characteristics, potential age-related risk factors, distribution of subtypes, and effectiveness of canalith repositioning were compared between the groups. Results Female sex was significantly more common in all age groups, with a peak female-to-male ratio of 5.1:1 in the group aged 50-59 years. There was a higher proportion of men in the geriatric group. A history of disease associated with atherosclerosis was significantly more common in the geriatric group (p< 0.05). Migraine was significantly more common in the non-geriatric group (p= 0.018), as was posterior canal BPPV. The horizontal canal BPPV (especially horizontal canal BPPV-cupulolithiasis), and multicanal BPPV subtypes were more common in the geriatric group, whereas anterior canal BPPV was more common in the non-geriatric group. Two canalith repositioning sessions were effective in 58.0% of the geriatric cases and in 72.6% of the non-geriatric cases (p= 0.002). There was a tendency for the effectiveness of canalith repositioning to decrease with increasing age. Conclusion BPPV was more common in women. However, the proportion of men with BPPV increased with age. Elderly patients often had a history of diseases associated with atherosclerosis (i.e., hypertension, diabetes, and hyperlipidemia). The horizontal canal BPPV (particularly horizontal canal BPPV-cupulolithiasis) and multicanal BPPV subtypes were more common and the anterior canal BPPV subtype was less common in elderly patients. The effectiveness of canalith repositioning may decrease with age. Therefore, older patients should receive more comprehensive medical treatment. Level of evidence: 4.

Paroxysmal Hemicrania-Trigeminal Neuralgia (PH-Tic) syndrome with no structural lesion in a Brazilian woman: the first case-report / Síndrome de Neuralgia Paroxística Hemicrânia-Trigêmea (PH-Tic) sem lesão estrutural em mulher brasileira: primeiro relato de caso

Introduction: In Brazil there is only one case report of a patient diagnosed with Paroxysmal Hemicrania-Trigeminal (PH-Tic) syndrome reported, however it was observed in a patient with Chiari I malformation. Objective: Here, we describe the first case of primary PH-Tic syndrome in the country. Method: Case report. CARE guideline was used to guide the structuring of this article. This case report was approved by the ethics committee and has been registered under the protocol number 70705623.7.0000.5440 on "Plataforma Brasil". Results:A 72-year-old woman with a five-month history of headaches was admitted at our headache outpatient clinic. The pain was sharp, intense, localized in the periorbital and left temporal regions. Blood counts, liver, renal and thyroid function were normal, as well as brain magnetic resonance imaging. Despite using carbamazepine, the patient had pain in only the left side of the face. Indomethacin was added until the dose of 100 mg a day and resulted in improvement of headache frequency. Conclusion: PH-Tic should be hypothesized in patients with short-lasting headaches associated with facial pain that partially improve with carbamazepine or indomethacin.

Introdução: No Brasil há apenas um relato de caso de paciente com diagnóstico de síndrome Paroxística Hemicrania-Trigeminal (PH-Tic), porém foi observado em um paciente com malformação de Chiari I. Objetivo: Descrevemos aqui o primeiro caso de síndrome PH-Tic primária no país. Método: Relato de caso. A diretriz CARE foi utilizada para orientar a estruturação deste artigo. Este relato de caso foi aprovado pelo comitê de ética e registrado sob o número de protocolo 70705623.7.0000.5440 na "Plataforma Brasil". Resultados: Uma mulher de 72 anos com história de cefaleias há cinco meses foi internada em nosso ambulatório de cefaleias. A dor era aguda, intensa, localizada nas regiões periorbital e temporal esquerda. Os hemogramas, as funções hepática, renal e tireoidiana estavam normais, assim como a ressonância magnética cerebral. Apesar do uso de carbamazepina, o paciente apresentava dor apenas no lado esquerdo da face. A indometacina foi adicionada até a dose de 100 mg ao dia e resultou em melhora da frequência da cefaleia. Conclusão: O PH-Tic deve ser hipotetizado em pacientes com cefaleias de curta duração associadas a dores faciais que melhoram parcialmente com carbamazepina ou indometacina.