Saccades, pupil response and blink abnormalities in Huntington's disease patients during free viewing.

OBJECTIVE: Video-based eye tracking was used to investigate saccade, pupil, and blink abnormalities among patients with Huntington's disease (HD) who watched sequences of short videos. HD, an autosomal dominant neurodegenerative disorder resulting from a CAG mutation on chromosome 4, produces motor and cognitive impairments including slow or irregular eye movements, which have been studied using structured tasks. METHODS: To explore how HD affects eye movements under instruction free conditions, we assessed 22 HD patients and their age matched controls in a 10-minute video-based free viewing task. RESULTS: Patients with HD experienced a significant reduction in saccade exploration rate following video clip transitions, an increase in pupil reactions to luminance changes after clip transitions, and a significant higher blink rate throughout the task compared to the control group. CONCLUSIONS: These results show that HD has a significant impact on how patients visually explore and respond to their environment under unconstrained and ecologically natural conditions. SIGNIFICANCE: Eye tracking in HD patients revealed saccadic, pupil, and blink abnormalities in early HD patients, suggestive of brain circuitry abnormalities that probably involve brain stem deficits. Further research should explore the impact of these changes on the quality of life of the patients affected by the disease.

Thyrotropin-Releasing Hormone and Food Intake in Mammals: An Update.

Thyrotropin-releasing hormone (TRH; pGlu-His-Pro-NH2) is an intercellular signal produced mainly by neurons. Among the multiple pharmacological effects of TRH, that on food intake is not well understood. We review studies demonstrating that peripheral injection of TRH generally produces a transient anorexic effect, discuss the pathways that might initiate this effect, and explain its short half-life. In addition, central administration of TRH can produce anorexic or orexigenic effects, depending on the site of injection, that are likely due to interaction with TRH receptor 1. Anorexic effects are most notable when TRH is injected into the hypothalamus and the nucleus accumbens, while the orexigenic effect has only been detected by injection into the brain stem. Functional evidence points to TRH neurons that are prime candidate vectors for TRH action on food intake. These include the caudal raphe nuclei projecting to the dorsal motor nucleus of the vagus, and possibly TRH neurons from the tuberal lateral hypothalamus projecting to the tuberomammillary nuclei. For other TRH neurons, the anatomical or physiological context and impact of TRH in each synaptic domain are still poorly understood. The manipulation of TRH expression in well-defined neuron types will facilitate the discovery of its role in food intake control in each anatomical scene.

The Relationship between Speech Sound Disorder and Cortical Auditory Evoked Potential.

INTRODUCTION: Speech sound disorder (SSD) is a speech and language disorder associated with difficulties in motor production, perception, and phonological representation of sounds and speech segments. Since auditory perception has a fundamental role in forming and organizing sound representation for its recognition, studies that evaluate the cortical processing of sounds are required. Thus, the present study aimed to verify the relation between SSD severity measured by the percentage of correct consonants (PCCs) with the cortical auditory evoked potentials (CAEPs) using speech stimulus. METHODS: Twenty-nine children with normal hearing participated in this research and were grouped into three groups by SSD level measured by the PCC index. In addition, the groups were subdivided according to the children's age group: between 60-71 months, 72-83 months, and 83-94 months. The CAEP with speech stimulus was carried out in all children. RESULTS: Older children had longer P1 and N1 latencies. In P2 latency, there was an interference of age only in the severe group. The N2 latency was affected by age, where older children had longer latency. CONCLUSION: The amplitude of CAEP has not suffered any interference with the age, or severity of SSD. For the latency, older children generally presented longer averages than younger ones.

Anestesia del tronco encefálico posterior a bloqueo retrobulbar en cirugía oftalmológica. Reporte de caso / Brainstem anesthesia after retrobulbar block in ophthalmic surgery. Case report

Resumen: La morbimortalidad perioperatoria en cirugía oftalmológica es baja, considerando que la mayor parte de la población que requiere estos procedimientos son pacientes de edad avanzada con múltiples comorbilidades. Embriológicamente el nervio óptico y las capas meníngeas que lo recubren se derivan del ectodermo y están estrechamente relacionadas con las capas meníngeas que recubren al sistema nervioso central, de esta forma la punción inadvertida de las mismas durante los bloqueos orbitarios provocan diseminación del fármaco por medio del líquido cefalorraquídeo a través del espacio subaracnoideo hasta el tronco encefálico. Se reporta el caso de paciente femenino de 61 años de edad, programada para lavado de cámara vítrea en ojo izquierdo. La técnica anestésica fue sedación más bloqueo retrobulbar; posterior a dicho procedimiento, se observa deterioro neurológico, alteraciones cardiovasculares, disminución de la saturación de oxígeno manteniendo ventilación espontánea, pero con obstrucción de la vía aérea; se da apoyo ventilatorio y se administra antihipertensivo, respondiendo favorablemente 40 minutos después del bloqueo retrobulbar. La relación entre un bloqueo orbitario y la aparición de sintomatología neurológica, cardiovascular y/o respiratoria debe suscitar sospechas de anestesia del tronco encefálico. Lo más importante es el reconocimiento y atención temprana de esta complicación para evitar secuelas neurológicas, cardiovasculares o incluso la muerte.

Abstract: Perioperative morbidity and mortality in ophthalmic surgery is low, considering that most of the population that requires these procedures are elderly patients with multiple comorbidities. Embryologically, the optic nerve and the meningeal sheaths that cover it originate from the ectoderm, are closely related to the meningeal layers that cover the central nervous system, whereby their inadvertent puncture during orbital blocks causes drug spread by the cerebrospinal fluid through the subarachnoid space to the brainstem. The case of a 61-year-old female patient is reported, programmed for lavage of the left eye vitreous chamber. The anesthetic technique was sedation and retrobulbar block; after this procedure, neurological impairment, cardiovascular alterations, decreased oxygen saturation were observed, maintaining spontaneous ventilation but with airway obstruction; ventilatory support is given and antihypertensive is administered, responding favorably 40 minutes after retrobulbar block. The relationship between an orbital block and the appearance of neurological, cardiovascular or respiratory symptoms should raise suspicion of brainstem anesthesia. The most important thing is early recognition and attention to this complication, to avoid neurological and cardiovascular sequelae or even death.

Evaluation of the maturational auditory process in children with the infection by Zika congenital syndrome / Avaliação do processo maturacional auditivo em crianças portadoras da síndrome da infecção congênita pelo Zika vírus

ABSTRACT Purpose: to analyze the absolute latencies of waves I. III and V and the interpeak intervals I-III. III-V and I-V of the ABR recorded from different age groups of children with congenital zika virus infection and their peers without risk indicators for hearing impairment. Methods: 84 newborns and infants (N=51 study group and N=33 control group) divided into groups with different post-conceptual ages. with the results of their hearing exams analyzed by age group and compared with their peers without other risk indicators for hearing impairment. The assessment of the auditory pathway was conducted through tympanometry. otoacoustic emissions and auditory brain stem responses. Results: only the latency of wave I and the interpeak III-V showed no significant difference between the study and control groups. The absolute latency and interpeak values found in the study group were significantly lower than those found in the control group. Conclusion: the maturation of the brain stem in children with ZIKV infection occurred within normal limits. with no retrocochlear disorders until the age of 5 years.

RESUMO Objetivo: analisar as latências absolutas das ondas I. III e V e os intervalos interpicos I-III. III-V e I-V do Potencial Evocado Auditivo de Tronco Encefálico obtidos em diversos grupos etários de crianças portadoras de infecção congênita por zika vírus e nos seus pares sem indicadores de risco para deficiência auditiva. Métodos: foram recrutados 84 recém-nascidos e lactentes (N=51 grupo de estudo e N=33 grupo controle) separados por grupos com diferentes idades pós-conceptuais. sendo os resultados dos seus exames auditivos analisados por faixa etária e comparados com seus pares sem outros indicadores de risco para deficiência auditiva. A avaliação da via auditiva foi realizada por meio da timpanometria. emissões otoacústicas transientes e potencial evocado de tronco encefálico. Resultados: somente a latência da onda I e o intervalo interpico III-V não apresentaram diferença significante entre grupo de estudo e controle. Os valores de latências absolutas e interpicos encontrados no grupo de estudo foram menores do que os obtidos no grupo controle de forma significativa. Conclusão: a maturação do tronco encefálico nas crianças portadoras de infecção por Zika Vírus ocorreu dentro da normalidade. não apresentando alterações retrococleares até a idade de 5 anos.

Estimated thresholds (est dBHL) for auditory evoked potentials with narrowband CE-Chirp® stimuli / Umbrales estimados (esdB HL) para potenciales evocados auditivos con estímulo Narrow Band CE-Chirp®

ABSTRACT Purpose: to correlate pure-tone audiometry thresholds with electrophysiological thresholds, using NB CE-Chirp® stimuli in listeners aged 18 to 30 years from Santiago, Chile, and determine the estimated threshold at 500, 1000, 2000, and 4000 Hz. This can contribute to the battery of objective and subjective tests applicable to children, aiding the timely detection and implementation before 3 months old, as recommended by the Joint Committee on Infant Hearing. Methods: a quantitative, nonexperimental study with a sample of 30 hearing subjects, aged 18 to 30 years. The Pearson´s correlation test was applied, with a significance p-value of 0.01. Results: higher frequencies (2000 and 4000 Hz) have lower correction factors than medium and lower frequencies (1000 and 500 Hz), which tend to be higher as the frequency is lower. Conclusions: the study obtained the following estimated thresholds: 15 dB at 500 Hz, 10 dB at 1000 and 2000 Hz, and 5 dB at 4000 Hz.

RESUMEN Objetivo: correlacionar umbrales tonales a través de audiometría tonal y umbrales electrofisiológicos usando estímulos NB CE-Chirp® en oyentes de entre 18 a 30 años de la ciudad de Santiago de Chile, de esta forma determinar el umbral estimado para las frecuencias 500, 1000, 2000 y 4000 Hz. De esta forma, es posible aportar con la batería de test objetivos y subjetivos aplicables en la población infantil, colaborando con la detección e implementación oportuna antes de los 3 meses de edad, según lo recomendado por el Joint Committee on Infant Hearing. Métodos: corresponde a un estudio de enfoque cuantitativo, de tipo no experimental, transversal. Utilizando 30 sujetos oyentes, entre 18 y 30 años. Se aplicó la prueba de correlación de Pearson, con un valor p de significancia de 0,01. Resultados: las frecuencias 2000 y 4000 Hz, poseen factores de corrección menores en comparación con las frecuencias medias y graves (1000 y 500 Hz), las cuales tienden a ser mayores a medida que la frecuencia es más baja. Conclusiones: los umbrales estimados obtenidos son de 15dB para 500Hz, 10dB para 1000 y 2000Hz y 5dB para 4000Hz.

Alucinosis peduncular en esclerosis múltiple, síntoma poco común en la expresión de enfermedad desmielinizante / Peduncular hallucinosis in multiple sclerosis, a rare symptom in demyelinating disease

Introducción: La alucinosis peduncular (AP) hace referencia a alucinaciones autodiscriminadas, cuyo origen son lesiones en el mesencéfalo y en el puente. Presentación del caso: Paciente 27 años, femenina, con alucinaciones visuales, auditivas autodiscriminadas por ella misma, sin antecedentes previos de importancia y con lesiones en resonancia magnética cerebral y cervical en el pedúnculo cerebeloso superior, tegmento pontino, y en columna cervical con bandas oligoclonales patrón 2, que cumplían criterios de Mc Donalds para esclerosis múltiple. Discusión: La alucinosis peduncular hace referencia a la presencia de alucinaciones visuales, criticadas por el paciente, con la consecuencia de lesiones de las vías inhibitorias por deaferentación y desinhibición mesencéfalotalámicas, y retinogenículo calcarina, descritas como manifestación de múltiples patologías neurológicas como trauma, afectación vascular, tumores y pocos casos de enfermedad desmielinizante, entre otras. Conclusión: La alucinosis peduncular es una forma atípica de presentación de lesiones pontomesencefálicas descritas en varias patologías; se debe tener en cuenta en la localización de la lesión neurológica; se han reportado pocos casos como síntoma de la enfermedad desmielinizante.

Introduction: Peduncular hallucinosis (PA) refers to self-discriminating hallucinations, these are caused by lesions in the midbrain and pons. Presentation of the case: 27-year-old right handed female patient with visual and auditory hallucinations self-discriminated by the patient, with no prior history of importance and with lesions in cerebral and cervical Magnetic Resonance in the superior cerebellar peduncle, pontine tegmentum, and in the cervical spine with pattern 2 oligo clonal bands, which met Mc Donald's criteria for multiple sclerosis. Discussion: Peduncular hallucinosis refers to the presence of visual hallucinations criticized by the patient, consequence of lesions in the inhibitory pathways with deafferentation and disinhibition of the midbrain-thalamic and retinogeniculus-calcarine pathways. Described as a manifestation of multiple neurological pathologies such as trauma, vascular, tumor and few cases of demyelinating among others. Conclusion: Peduncular hallucinosis is an atypical form of presentation of pontomesencephalic lesions described in several pathologies, it must be taken into account when locating the neurological lesion, few cases have been reported as symptom of the demyelinating disease.

Auditory Pathway Maturation in Full-term Small for Gestational Age Children: A Systematic Review with Meta-analysis.

Introduction Factors of intrauterine growth restriction have been responsible for the births of full-term babies small for their gestational age (SGA). Scientific evidence points that this restriction can cause changes in the neural maturation process. Objectives To analyze the absolute latencies and interpeak intervals of brainstem auditory evoked potential waves in full-term and SGA children to investigate whether there are changes of neural maturation in this population. Data Synthesis The search for articles that reported the assessment of brainstem auditory evoked potential in SGA newborns compared with a control, appropriate for their gestational age, both born full-term, for the entire period available in the database research until October 31, 2021 was performed based on the MEDLINE/PubMed Central and on the Latin America and the Caribbean Health Sciences Literature and Virtual Health Library electronic databases. A total of 311 studies were found in the database research. Out of this total, 10 studies were included in the review, 5 of which were eligible for the meta-analysis, involving a total of 473 participants of both genders, with 193 participants belonging to the study group and 280 to the control group. Differences between the groups were only observed in the absolute latency of wave V (95% confidence interval [CI]: 0.02-0.15; p < 0.01). Conclusion The SGA condition is responsible for the appearance of brainstem neural conduction dysfunction measured by the brainstem auditory evoked potentials, probably by the maturation process of the auditory pathway of this population.

Brazilian Society of Otology task force - Vestibular Schwannoma ‒ evaluation and treatment.

OBJECTIVE: To review the literature on the diagnosis and treatment of vestibular schwannoma. METHODS: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. RESULTS: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. CONCLUSIONS: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Insulinoma: reporte de un caso con resección quirúrgica exitosa

El insulinoma es un tumor endocrino del páncreas, el cual produce hiperinsulinismo y provoca disminución severa de la glucemia, con síntomas crónicos como desvanecimiento. Es más frecuente en varones y puede presentarse a cualquier edad, ya sea como neoplasias solitarias o múltiples, estas últimas conocidas por formar parte del grupo de neoplasias endocrinas múltiples tipo 1 (MEN 1). Los insulinomas son de rara presentación y requieren tratamiento quirúrgico resectivo; la resolución de los síntomas es el marcador de curación. En la presente publicación, se describe un caso de insulinoma en una mujer de 51 años, de presentación típica desde el punto de vista clínico, con síntomas y signos de hiperinsulinismo. Los exámenes de laboratorio indican un aumento de la secreción de insulina, mientras que las imágenes por resonancia magnética muestran características del insulinoma, donde se puede apreciar la tumoración pancreática. Este caso se trató mediante resección quirúrgica por medio de laparotomía exploratoria, utilizando la técnica de enucleación del tumor pancreático, la cual se describe al detalle en la publicación; no se presentaron complicaciones intraoperatorias ni posoperatorias, es decir, desde el punto de vista curativo, el resultado fue exitoso. Esta es la técnica quirúrgica que se emplea en el Hospital Nacional Arzobispo Loayza en Lima, Perú, ante casos como el descrito. Este tipo de neoplasia es muy poco común, por lo que se le debe considerar de alta sospecha y, en este caso, las imágenes fueron fundamentales para optar por la resección quirúrgica. En el hospital Arzobispo Loayza se han tratado otros casos de insulinoma mediante cirugía; asimismo, se ha presentado, al menos, un caso de nesidioblastosis, en donde no se pudo administrar un tratamiento adecuado al paciente.

Insulinoma is a pancreatic neuroendocrine tumor that produces hyperinsulinism and causes a severe lowering of blood glucose levels with chronic symptoms such as fainting. It is more common in males and can occur at any age, either as solitary or multiple neoplasms, the latter known to be part of the multiple endocrine neoplasia, type 1 (MEN 1) group. Insulinomas are rare and require resective surgery, with symptoms resolution being the marker of recovery. This article describes a case of insulinoma in a 51-year-old woman with a clinically typical presentation and signs and symptoms of hyperinsulinism. Laboratory tests revealed increased insulin secretion, while magnetic resonance imaging showed features of insulinoma, where the pancreatic tumor could be seen. This case was treated by surgical resection of the pancreatic tumor during an exploratory laparotomy, using the enucleation technique, which is described in detail herein. There were no intraoperative or postoperative complications, i.e., the result was successful in terms of health recovery. This surgical technique is used at Hospital Nacional Arzobispo Loayza in Lima, Peru, when facing cases such as the one described. This type of neoplasm is very uncommon, so it should be considered highly suspicious and, in this case, the images were essential for choosing the surgical resection. Other cases of insulinoma have been treated surgically at the Hospital; moreover at least one case of nesidioblastosis could not receive an adequate treatment.

Síndrome alterno mesencefálico: síndrome de Weber en dos pacientes varones VIH positivos con toxoplasmosis cerebral

En este reporte se describen dos casos de pacientes varones con VIH diagnosticados por serología, que no estaban recibiendo tratamiento. Ambos pacientes desarrollaron el síndrome alterno mesencefálico y la lesión estructural, causada por la infección del sistema nervioso central por toxoplasmosis cerebral, confirmada mediante una resonancia magnética cerebral. Esta condición se constató por serología en líquido cefalorraquídeo. El síndrome de Weber es un tipo de síndrome alterno mesencefálico poco frecuente y existe poca información de su clínica y evolución. Fue descrito por sir Herman David Weber en 1863, y se caracteriza por la lesión ipsilateral del fascículo del III par craneal, con presencia de hemiparesia contralateral debida a la lesión de la vía corticoespinal y corticobulbar del pedúnculo cerebral. Las causas que lo originan incluyen tanto procesos isquémicos o hemorrágicos, que lesionan el fascículo del III par craneal y pedúnculo cerebral, como neoplasias intraencefálicas, aneurismas, hematomas extradurales y procesos infecciosos que se comportan como procesos expansivos. Los pacientes evaluados presentaron clínica de ptosis palpebral, visión doble vertical, dificultad para bipedestación, hemiparesia, hiporreflexia, sensibilidad superficial y profunda disminuidas, equilibrio y coordinación alterados, III par parético, habla incoherente, desorientación en tiempo, espacio y persona de manera intermitente y asimetría facial. Para la toxoplasmosis se aplicó un tratamiento específico con trimetropim-sulfametoxasol, clindamicina y prednisona por vía oral, lo cual permitió una mejoría del cuadro clínico. En el presente caso clínico se presenta la secuencia de los hechos, manejo y breve revisión de la literatura para su consideración como diagnóstico y su relevancia en el paciente con VIH-toxoplasmosis del sistema nervioso central.

This report describes the case of two male HIV-positive patients who were not receiving treatment and whose diagnosis was confirmed by serology testing. Both patients developed midbrain stroke syndrome and the structural injury, which was caused by a central nervous system infection due to cerebral toxoplasmosis, was verified by brain magnetic resonance imaging. This condition was confirmed by cerebrospinal fluid serology testing. Weber's syndrome is a very rare type of midbrain stroke syndrome with little information available on its symptoms and evolution. It was first described by Sir Herman David Weber in 1863 and is characterized by ipsilateral injury of the third cranial nerve fascicle with contralateral hemiparesis due to injury of the corticospinal and corticobulbar tracts of the cerebral peduncle. Its causes range from ischemic or hemorrhagic processes, which damage the third cranial nerve fascicle and cerebral peduncle, to brain tumors, aneurysms, extradural hematomas and infectious diseases that behave like spreading processes. The assessed patients showed clinical signs and symptoms such as ptosis; vertical double vision; difficulty standing up; hemiparesis; hyporeflexia; decreased superficial and deep sensation; poor balance and coordination; third cranial nerve palsy; slurred speech; intermittent disorientation in time, place and person; and facial asymmetry. Oral trimethoprim-sulfamethoxazole, clindamycin and prednisone were administered as specific treatment for toxoplasmosis, which enabled the improvement of the clinical picture. This case report presents the sequence of events, treatment and a brief review of the literature to be considered in the diagnosis and determine its importance in patients with HIV-toxoplasmosis of the central nervous system.

Brainstem auditory-evoked responses among children afflicted by severely hypoxic CHD.

MAIN AIM: To electrophysiologically determine the impact of moderate to severe chronic hypoxia (H) resulting from a wide array of CHD (HCHD) conditions on the integrity of brainstem function. MATERIALS AND METHODS: Applying brainstem auditory-evoked response methodology, 30 chronically afflicted HCHD patients, who already had undergone heart surgery, were compared to 28 healthy control children (1-15 yo) matched by age, gender and socioeconomic condition. Blood oxygen saturation was clinically determined and again immediately before brainstem auditory-evoked response testing. RESULTS: Among HCHD children, auditory wave latencies (I, III and V) were significantly longer (medians: I, 2.02 ms; III, 4.12 ms, and; V, 6.30 ms) compared to control (medians: I, 1.67ms; III, 3.72 ms, and; V, 5.65 ms), as well as interpeak intervals (HCHD medians: I-V, 4.25 ms, and; III-V, 2.25ms; control medians: I-V, 3.90 ms and, III-V, 1.80 ms) without significant differences in wave amplitudes between groups. A statistically significant and inverse correlation between average blood oxygen saturation of each group (control, 94%; HCHD, 78%) and their respective wave latencies and interpeak intervals was found. CONCLUSIONS: As determined by brainstem auditory-evoked responses, young HCHD patients manifestly show severely altered neuronal conductivity in the auditory pathway strongly correlated with their hypoxic condition. These observations are strongly supported by different brainstem neurological and image studies showing that alterations, either in microstructure or function, result from the condition of chronic hypoxia in CHD. The non-altered wave amplitudes are indicative of relatively well-preserved neuronal relay nuclei.

Brazilian Society of Otology task force - Vestibular Schwannoma - evaluation and treatment

Abstract Objective: To review the literature on the diagnosis and treatment of vestibular schwannoma. Methods: Task force members were educated on knowledge synthesis methods, including electronic database search, review and selection of relevant citations, and critical appraisal of selected studies. Articles written in English or Portuguese on vestibular schwannoma were eligible for inclusion. The American College of Physicians' guideline grading system and the American Thyroid Association's guideline criteria were used for critical appraisal of evidence and recommendations for therapeutic interventions. Results: The topics were divided into 2 parts: (1) Diagnosis - audiologic, electrophysiologic tests, and imaging; (2) Treatment - wait and scan protocols, surgery, radiosurgery/radiotherapy, and systemic therapy. Conclusions: Decision making in VS treatment has become more challenging. MRI can diagnose increasingly smaller tumors, which has disastrous consequences for the patients and their families. It is important to develop an individualized approach for each case, which highly depends on the experience of each surgical team.

Auditory Pathway Maturation in Full-term Small for Gestational Age Children: A Systematic Review with Meta-analysis

Abstract Introduction Factors of intrauterine growth restriction have been responsible for the births of full-term babies small for their gestational age (SCA). Scientific evidence points that this restriction can cause changes in the neural maturation process. Objectives To analyze the absolute latencies and interpeak intervals of brainstem auditory evoked potential waves in full-term and SCA children to investigate whether there are changes of neural maturation in this population. Data Synthesis The search for articles that reported the assessment of brainstem auditory evoked potential in SCA newborns compared with a control, appropriate for their gestational age, both born full-term, for the entire period available in the database research until October31, 2021 was performed based on the MEDLINE/PubMed Central and on the Latin America and the Caribbean Health Sciences Literature and Virtual Health Library electronic databases. A total of 311 studies were found in the database research. Out of this total, 10 studies were included in the review, 5 of which were eligible for the meta-analysis, involving a total of 473 participants of both genders, with 193 participants belonging to the study group and 280 to the control group. Differences between the groups were only observed in the absolute latency of wave V (95% confidence interval [CI]: 0.02-0.15; p<0.01). Conclusion The SCA condition is responsible for the appearance of brainstem neural conduction dysfunction measured by the brainstem auditory evoked potentials, probably by the maturation process of the auditory pathway of this population.

Study of auditory pathways in type 1 diabetes mellitus through brainstem auditory evoked potentials and contralateral acoustic reflex / Estudo das vias auditivas em diabetes mellitus tipo 1 através dos potenciais auditivos evocados de tronco encefálico e reflexo acústico contralateral

ABSTRACT Purpose To investigate the functionalities of the neural pathways through the auditory evoked potentials of the brainstem and the contralateral stapedial acoustic reflexes in normal-hearing individuals with type 1 diabetes mellitus, in order to detect possible alterations in the central auditory pathways. Methods This is a cross-sectional study with a comparison group and a convenience sample, consisting of 32 individuals with type 1 diabetes mellitus and 20 controls without the disease. All subjects had hearing thresholds within normal limits and type A tympanometric curves. The acoustic reflex arc and brainstem auditory potentials were investigated. Statistical analyses were performed using the SPSS 17.0. The Chi-square test, Student´s t-test, and Multiple linear regression were used. Results The auditory thresholds of the acoustic reflex were statistically lower in the group with the disease at frequencies of 0.5 kHz and 1.0 kHz in the left ear (p=0.01 and p=0.01, respectively). The absolute latencies III and V of the auditory potentials of the brainstem in the right ear and V in the left ear were increased in subjects with type 1 diabetes mellitus (p=0.03, p=0.02 and p=0.03, respectively). Conclusion The findings suggest that subjects with type 1 diabetes mellitus are more likely to present alterations in the central auditory pathways, even with auditory thresholds within normal limits.

RESUMO Objetivo Investigar a funcionalidade das vias neurais por meio dos potenciais evocados auditivos de tronco encefálico e os reflexos acústicos estapedianos contralaterais em sujeitos com diabetes mellitus tipo 1 normo-ouvintes, a fim de detectar possíveis alterações nas vias auditivas centrais. Método Trata-se de um estudo transversal com grupo de comparação, e amostra de conveniência, composta por 32 sujeitos com diabetes mellitus tipo 1 e 20 controles sem a doença. Todos os sujeitos apresentavam limiares auditivos dentro dos padrões de normalidade e curva timpanométrica tipo A. Foram investigados o arco-reflexo acústico e os potenciais auditivos de tronco encefálico. As análises dos resultados foram realizadas no SPSS 17.0. Utilizou-se o Teste Qui Quadrado, Teste T de Studant e Regressão linear múltipla. Resultados Os limiares auditivos do reflexo acústico foram estatisticamente menores no grupo com a doença nas frequências de 0,5 kHz e 1,0 kHz na orelha esquerda (p=0,01 e p=0,01, respectivamente). As latências absolutas III e V dos potenciais auditivos de tronco encefálico da orelha direita e V da orelha esquerda estavam aumentadas em sujeitos com diabetes mellitus tipo 1 (p=0,03, p=0.02 e p=0,03, respectivamente). Conclusão Os achados sugerem que sujeitos com diabetes mellitus tipo 1 estão mais propensos a apresentar alterações nas vias auditivas centrais, mesmo com limiares auditivos dentro dos padrões de normalidade.

Foville syndrome: a neurological emergency of clinical relevance. case report

Introduction: Foville syndrome (inferior medial pontine syndrome) is a clinical entity that makes part of a subclassification of a broader category of posterior circulation stroke. It is characterized by a blockage of the basilar artery caused by lesions in the pontine tegmentum. This syndrome has a very low incidence worldwide, but its clinical relevance is considerable. Case report: A 41-year-old patient without any medical history was admitted to the emergency department due to signs of focal neurologic deficits. Imaging findings were compatible with an ischemic lesion in the brainstem, and cerebral angiography showed involvement of the basilar artery territory. The patient was diagnosed and followed up in the intensive care unit (ICU) until his recovery. Conclusion: Foville syndrome is a rare disease with high morbidity and mortality. Early recognition and a multidisciplinary approach are decisive to improve the patient's prognosis.

Introducción. El síndrome de Foville (síndrome pontino medial inferior) es una entidad clínica que hace parte de una subclasificación de la categoría más amplia de eventos cerebrovasculares de la circulación posterior, se presenta como resultado de la oclusión de la arteria basilar debido a lesiones en el tegmento pontino. Este síndrome tiene una muy baja incidencia a nivel mundial, pero es de gran relevancia clínica. Presentación del caso. Hombre de 41 años sin antecedentes patológicos, que ingresó al servicio de urgencias por signos de focalización neurológica. Sus hallazgos imagenológicos fueron compatibles con una lesión isquémica en el tronco encefálico y la angiografía cerebral evidenció un compromiso en el territorio de la arteria basilar. El paciente fue diagnosticado y se le dio seguimiento en la unidad de cuidados intensivos (UCI) hasta su rehabilitación. Conclusión. El síndrome de Foville es una enfermedad infrecuente y de alta mortalidad. El reconocimiento temprano de esta afección y un abordaje multidisciplinario son determinantes para mejorar el pronóstico del paciente.

A Shift in SARS-CoV-2 Omicron Variant's Entry Pathway Might Explain Different Clinical Outcomes.

Globally, SARS CoV-2 omicron variant has led to a notable increase of COVID-19 diagnoses, although with less severe clinical manifestations and decreased hospitalizations. The omicron wave swelled faster than previous waves, completely displacing the delta variant within weeks, and creating worldwide concern about final, successful pandemic control. Some authors contend that symptoms associated to omicron differ from 'traditional' symptoms and more closely resemble those of the common cold. One major COVID-19 symptom frequent with other variants-loss of taste and smell-is rarely present with omicron. This may be of interest, since it has also been suggested that direct SARS-CoV-2 invasion into the brainstem through the olfactory nerves by transsynaptic pathways could provide one explanation for the acute respiratory distress syndrome refractory to treatment. Brainstem infection by SARS-CoV-2 can severely damage the respiratory center, triggering functional deviations that affect involuntary respiration, leading to acute respiratory distress syndrome refractory to treatment, the main cause of death in COVID-19 patients. A shift in the omicron SARS-CoV-2 entry pathway from cell-surface fusion, triggered by TMPRSS2, to cathepsin-dependent fusion within the endosome, may affect transmission, cellular tropism and pathogenesis. Therefore, we can hypothesize that this entrance modification may impact transmission from the olfactory nerve to the brainstem through transsynaptic pathways. A decrement of the virus's direct invasion into the brainstem could diminish respiratory center dysfunction, reducing acute respiratory distress syndrome and the need for mechanical ventilation.

Children with developmental language disorder: a frequency following response in the noise study / Crianças com transtorno de desenvolvimento da linguagem: uma resposta de seguimento de frequência (FFR) no estudo com ruído

Abstract Introduction: Children with developmental language disorder have been reported to have poor temporal auditory processing. This study aimed to examine the frequency following response. Objective: This work aimed to investigate speech processing in quiet and in noise. Methods: Two groups of children were included in this work: the control group (15 children with normal language development) and the study group (25 children diagnosed with developmental language disorder). All children were submitted to intelligence scale, language assessment, full audiological evaluation, and frequency following response in quiet and noise (+5QNR and +10QNR). Results: Results showed no statically significant difference between both groups as regards IQor PTA. In the study group, the advanced analysis of frequency following response showed reduced F0 and F2 amplitudes. Results also showed that noise has an impact on both the transient and sustained components of the frequency following response in the same group. Conclusion: Children with developmental language disorder have difficulty in speech processing especially in the presence of background noise. Frequency following response is an efficient procedure that can be used to address speech processing problems in children with developmental language disorder.

Resumo Introdução: Tem sido relatado que crianças com transtorno do desenvolvimento da linguagem têm processamento auditivo temporal deficiente. Objetivo: Examinar a resposta de seguimento de frequência eo processamento da fala no silêncio e no ruído. Método: Dois grupos de crianças foram incluídos neste trabalho: o grupo controle (15 crianças com desenvolvimento normal de linguagem) e o grupo de estudo (25 crianças com diagnóstico de transtorno do desenvolvimento da linguagem). Todas as crianças foram submetidas à escala de inteligência, avaliação da linguagem, avaliação audiológica completa e resposta de seguimento de frequência em silêncio (Q) e no ruído (N): +5QNR e +10QNR. Resultados: Os resultados não mostraram diferença estatisticamente significante entre os dois grupos no que diz respeito ao QI ou PTA. No grupo de estudo, a análise avançada da resposta de seguimento de frequência mostrou amplitudes reduzidas de F0 e F2. Os resultados também mostraram que o ruído tem um impacto nos componentes transitório e sustentado da resposta de seguimento de frequência no mesmo grupo. Conclusão: Crianças com transtorno do desenvolvimento da linguagem apresentam dificuldade no processamento da fala principalmente na presença de ruído de fundo. A resposta de seguimento de frequência é um procedimento eficiente que pode ser usado para avaliar problemas de processamento de fala em crianças com transtorno do desenvolvimento da linguagem.

Frequency-Following Response and Auditory Behavior in Children with Prenatal Exposure to the Zika Virus

Abstract Introduction Prenatal exposure to the Zika virus can impair neurodevelopment and cause auditory damage. Objective To analyze the frequency-following response (FFR) and the auditory behavior (with the LittlEars® questionnaire) of children with and without prenatal exposure to Zika virus infection. Methods A total of 30 children participated in the present study, divided into 3 groups: 10 children with microcephaly and prenatal exposure to the Zika virus; 10 normocephalic children with prenatal exposure to the Zika virus; and 10 children with no evidence of prenatal exposure to the virus. The FFR test was performed with the /da/ syllable. The LittlEars® questionnaire was used with parents/guardians. Results For the FFR measurements, there was no difference between the groups. The children with exposure to the Zika virus presented a final score in the questionnaire below what is expected from children with normal hearing. A significant difference was observed for the final, semantic, and expressive scores between the group with microcephaly and the other groups. A strong negative correlation was seen between the LittlEars® questionnaire final score and the FFR measurements for the group with microcephaly when compared with the other groups. Conclusion Children exposed to the Zika virus, with and without microcephaly, presented FFR patterns similar to what was seen in children with no evidence of virus exposure. However, they showed signs of immature auditory behavior, suggesting auditory development delay.

Treatment Limitations for Pediatric Diffuse Intrinsic Pontine Gliomas in a Middle-Income Country.

Objectives To evaluate the surgical management outcomes in pediatric patients with diffuse intrinsic pontine gliomas (DIPGs) who underwent intended biopsies and partial resections in a middle-income country, highlighting the barriers and challenges of these procedures for further investigation. Methods A retrospective review of a prospective acquired series of patients who underwent biopsy or resection for DIPG between January 2012 and June 2018 at our institution was performed. Results A total of 43 patients with posterior fossa tumors were identified. From these, seven pediatric DIPG cases were enrolled. Five were males. The median age was 5 years (range: 1-12 years). Only one patient (14.3%) had a ganglioglioma, while the others presented pilocytic and diffuse astrocytomas. Two (28.6%) patients had an intentional biopsy, and the other five (71.4%) had a partial resection. In the three (28.6%) patients who presented with associated hydrocephalus, the endoscopic third ventriculostomy was performed in the same surgical time. The median preoperative Lansky play-performance scale (LPPS) was 80 (range: 60-100), while the median postoperative LPPS was 23 (range: 7-52). Conclusion A decrease in overall survival was noted compared with data reported in other series. Multifactorial barriers were discussed including the social, geographic, and economic features that may influence on final outcomes.