Alucinosis peduncular en esclerosis múltiple, síntoma poco común en la expresión de enfermedad desmielinizante

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.

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.

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.

Effects of transcranial magnetic stimulation on the swallowing and brain-stem auditory evoked potentials of dysphagic stroke survivors / 中华物理医学与康复杂志

Objective:To observe any effects of contralateral repeated transcranial magnetic stimulation (rTMS) of the swallowing motor cortex on the swallowing and brainstem auditory evoked potentials (BAEPs) of stroke survivors with dysphagia.Methods:A total of 83 stroke survivors with dysphagia were randomly divided into an ipsilesional stimulation group ( n=22), a contralesional stimulation group ( n=21), a bilateral stimulation group ( n=20), and a control group ( n=20). In addition to their conventional dysphagia training, those in the three stimulation groups received 3Hz rTMS while the control group was given fake stimulation. The treatment was administered daily for 20 minutes, 6 days a week, for 5 consecutive weeks. Before and after the treatment, swallowing function was assessed videofluoroscopically and using the Dysphagia Outcome and Severity Scale (DOSS). The oral and pharyngeal stages of swallowing were evaluated using the videofluoroscopic dysphagia scale (VDS). Brain stem conduction was assessed using BAEPs. Results:After treatment the average DOSS scores of all 4 groups were significantly better than before the treatment. The average DOSS scores of the contralesional and bilateral sti-mulation groups were then significantly better than those of the other two groups. The sub-item and total VDS scores of all 4 groups had decreased significantly, but the average score of the bilateral stimulation group was significantly lower than the control group′s average. Ipsilesional stimulation significantly improved the VDS sub-item scores for the triggering of pharyngeal swallowing, laryngeal elevation, and pharyngeal transit time compared with the control group. In the contralesional stimulation group the average total score and the VDS sub-item scores for apraxia, premature bolus loss, oral transit times, the triggering of pharyngeal swallowing, vallecular residue, laryngeal elevation, coating on the pharyngeal wall, and pharyngeal transit time were significantly lower than those of the control group, on average. After the treatment the latencies of BAEP waves I, III and V and the I-III, III-V and I-V interpeak intervals had decreased significantly in all four groups, but the average latencies and intervals of the bilateral and contralesional groups were significantly shorter than those of the control group. The latencies and intervals of the bilateral stimulation group were then significantly shorter than those in the ipsilesional stimulation group on average. The average latency of wave V in the bilateral stimulation group (6.53±0.73ms) was significantly shorter than that in the contralesional stimulation group after the treatment.Conclusion:Bilateral rTMS over the swallowing motor cortex combined with conventional dysphagia training can significantly improve the swallowing of dysphagic stroke survivors.

Combining vibroacoustic therapy with audio-visual feedback can improve the swallowing and the emotional state of survivors of a brainstem stroke / 中华物理医学与康复杂志

Objective:To observe any effect of combining vibroacoustic therapy with audio-visual feedback on the swallowing and the emotional state of persons with a swallowing disorder after a brainstem stroke.Methods:Fifty brainstem stroke survivors with dysphagia were randomly divided into a control group and a combination group, each of 25. In addition to routine swallowing function training, the control group received additional direct feeding training, while the combination group was provided with vibroacoustic feeding training and swallowing stimulation from an animated audio-visual presentation. Before and after the treatment, the subjects′ swallowing function was quantified using the Standard Swallowing Assessment (SSA) scale and the Gugging swallowing screen (GUSS). The Positive and Negative Emotions Scale (PNAS) was used to quantify their emotional state.Results:After the treatment the average SSA scores of both groups had decreased significantly, while the average GUSS and PNAS scores had increased significantly. The average improvements in all three measures were significantly greater in the combination group than in the control group.Conclusion:In addition to routine swallowing function training, feeding training applying vibroacoustics and audio-visual stimulation can significantly improve the feeding function and emotional state of brainstem stroke survivors with dysphagia.

Adult-onset X-linked adrenoleukodystrophy characterized by brainstem symptoms: a case report / 中华神经科杂志

X-linked adrenoleukodystrophy (X-ALD) is the most common peroxisomal disorder. Adult-onset X-ALD characterized by brainstem symptoms is a rare phenotype of the disease. A male youth of adult-onset X-ALD characterized by brainstem symptoms in Tianjin Huanhu Hospital at April 12, 2021 is reported. The patient mainly presented with progressively gait disturbance, dysarthria and ataxia, which were consistent with the clinical diagnosis of X-ALD in combination with other ancillary tests. Genetic testing suggested the presence of a hemizygous mutation site in the ABCD1 gene. The clinical, imaging and genetic characteristics of X-ALD patients in the literature with brain stem lesion are also summarized, thereby improving the understanding of the rare clinical phenotype of X-ALD.

Evaluation of brainstem function using vestibular evoked myogenic potentials in patients with early-stage Parkinson′s disease / 中华神经科杂志

Objective:To investigate whether vestibular-evoked myogenic potentials (VEMP) can be used to assess brainstem and its supplementary diagnostic value in patients with early-stage Parkinson′s disease (PD).Methods:A total of 123 patients with early-stage PD (PD group) diagnosed in the Department of Neurology of the Second Affiliated Hospital of Soochow University from January 2019 to January 2022 were consecutively enrolled, and 122 healthy controls (healthy control group) were included. Cervical VEMP (cVEMP) and ocular VEMP (oVEMP) examinations were performed on all subjects. VEMP parameters between the 2 groups were compared, and receiver operating characteristic curve was used to evaluate the auxiliary diagnostic efficacy of VEMP for early-stage PD. Correlations between VEMP parameters and motor and non-motor symptoms such as autonomic dysfunction were analyzed in the PD group using Spearman correlation analysis.Results:Bilateral latencies of cVEMP [left P1 latency (Lp13): 19.0 (16.4, 20.9) ms vs 13.1(12.0, 14.2) ms, Z=-11.18, left N1 latency (Ln23): 27.4 (24.6, 29.9) ms vs 21.2 (19.8, 23.0) ms, Z=-10.14; right P1 latency (Rp13): 18.8 (16.2, 20.9) ms vs 13.0 (11.7, 14.1) ms, Z=-10.84, right N1 latency (Rn23): 27.7 (24.3, 29.7) ms vs 21.1 (19.6, 22.9) ms, Z=-10.50] and bilateral latencies of oVEMP [left N1 latency (Ln10): 12.7 (10.7, 14.4) ms vs 10.4 (9.7, 11.4) ms, Z=-8.02, left P1 latency (Lp15): 16.5 (15.1, 18.3) ms vs 14.5 (13.4, 15.3) ms, Z=-7.96; right N1 latency (Rn10): 12.8 (11.4, 14.0) ms vs 10.5 (9.7, 11.5) ms, Z=-8.85, right P1 latency (Rp15): 16.7 (15.3, 18.3) ms vs 14.4 (13.3, 15.1) ms, Z=-9.39] of the PD group significantly prolonged compared to the healthy control group (all P<0.001). Compared to the healthy control group, the area under the curve (AUC) values of Lp13, Ln23, Rp13 and Rn23 of cVEMP in the PD group were all greater than 0.7, and the AUC values of Lp13 and Rp13 in the PD group were greater than 0.9 (all P<0.001); the AUC values of Ln10, Lp15, Rn10, and Rp15 of oVEMP in the PD group were all greater than 0.7 (all P<0.001). The Rn10-p15 corrected amplitude in PD patients was positively correlated with levodopa equivalent dose ( r=0.21, P=0.020). The Rn10 in PD patients was positively correlated with the Non-Motor Symptoms Questionnaire scores ( r=0.21, P=0.023). The Lp13-n23 corrected amplitude was negatively correlated with the Scale for Outcomes in Parkinson′s Disease-Autonomic scores ( r=-0.20, P=0.023). There was no significant correlation between VEMP parameters and Unified Parkinson′s Disease Rating Scale part Ⅲ score ( P>0.05). Conclusion:VEMP, especially cVEMP, as a non-invasive neuroelectrophysiological index, is an objective marker for brainstem damage and could be used for screening early-stage PD patients.

Clinical study of stereotactic subtentorial approach for brainstem puncture and drainage in the treatment of brain stem hemorrhage in elderly patients / 中华老年医学杂志

Objective:To observe the curative effect of stereotactic subtentorial approach for brainstem puncture and drainage on brain stem hemorrhage in elderly patients.Methods:The clinical data of elderly patients with brain stem hemorrhage admitted to the Department of Neurosurgery, Shangqiu First People 's Hospital from April 2018 to April 2022 were retrospectively analyzed.According to the inclusion and exclusion criteria, a total of 65 elderly patients with brainstem hemorrhage who were treated with stereotactic subtentorial approach for brain stem puncture and drainage, and had complete follow-up data within 90 days after operation were selected.The patient's data were reviewed.The operation time, hematoma clearance rate on the first day after operation, the drainage tube extraction of hematoma cavity, and the postoperative complications were recorded.The follow-up results at 30 days and 90 days after the operation were also recorded.The 30-day Glasgow outcome scale(GOS)was used to evaluate the short-term clinical outcomes of surviving patients, while the modified Rankin score was used to evaluate the neurological function recovery of surviving patients at 90 days.Results:The operation time of the 65 patients was 1.1-2.8 h, with an average of(1.9±0.4)h.On the first day after operation, CT scan showed that the hematoma clearance rate was(84.6±13.6)%.The drainage tube in hematoma cavity was removed within 3-5 days, and there was no puncture-related intracerebral hemorrhage or drainage tube-related intracranial infection after operation.During 30 days of follow-up, 9 patients died and 56 patients survived, with a survival rate of 86.2%.Among the surviving patients, 5 were temporarily in vegetative state and 51 were awake, with varoius degrees of disability.The preoperative hematoma volume of the surviving patients was significantly less than that of the dead patients, and the preoperative GCS score was significantly lower than that of the dead patients( Z=2.386, 2.009, P=0.017, 0.045). After 90 days of follow-up, 3 patients died and 53 survived, with a survival rate of 81.54%.Among the surviving patients, the neurological function of 22 patients recovered well, and the effective rate of clinical treatment was 41.51%. Conclusions:Stereotactic subtentorial approach for brain stem puncture and drainage is an effective and relatively safe surgical method for the treatment of brain stem hemorrhage in elderly patients.

Abnormal changes in gray matter volume and structural covariate network in patients with chronic pontine infarction / 国际脑血管病杂志

Objective:To investigate the changes in gray matter volume (GMV) and abnormalities in structural covariant network (SCN) patterns in patients with chronic pontine infarction (PI).Methods:Patients with unilateral chronic PI (case group) with the first onset admitted to the First Affiliated Hospital of Zhengzhou University and Tianjin Medical University General Hospital from October 2014 to June 2021 were prospectively included. At the same time, healthy subjects matched with age, gender and education years (normal control group) were included. High-resolution three-dimensional T 1 structural MRI images and behavioral scores of the subjects were collected. The voxel-based morphometry and two-sample t test were used to explore the differences in GMV between the groups. Using GMV differential brain regions as seed points, SCN was constructed to explore the abnormality of structural covariant patterns in patients with PI. Spearman rank correlation analysis was used to analyze the correlation between GMV in differential brain regions and behavioral scores. Results:A total of 60 patients with PI were enrolled, including 33 left PI and 27 right PI, while 34 healthy controls were also enrolled. Compared with the normal control group, the GMV in bilateral posterior cerebellar lobe decreased significantly in the left PI group, and the GMV in left anterior and posterior cerebellar lobes and the right posterior cerebellar lobe decreased significantly in the right PI group (Gaussian random field correction with voxel level P<0.001 and cluster level P<0.05, cluster voxel >20), and there was a significant correlation between GMV values in the left anterior and posterior cerebellar lobes and the right posterior cerebellar lobe and the motor function score ( P<0.05). In addition, compared with the normal control group, the right PI group had broader covariate brain regions and a significant increase in the number of structural connections between covariate brain regions (family-wise error correction with voxel level P<0.05, cluster voxel >20). Conclusions:The GMV in bilateral posterior cerebellar lobe decreases significantly in patients with chronic PI, and were secondary to broader covariate brain regions and structural connections. This may be the neural mechanism of impaired behavioral function in patients with PI.

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.

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.

Síndrome de Claude: III par incompleto y ataxia contralateral / Claude syndrome: Incomplete third cranial nerve palsy and contralateral ataxia

Resumen Los accidentes cerebrovasculares mesencefálicos son poco frecuentes y por lo general están acompañados de otras lesiones concomitantes. La presencia simultánea de signos ipsi y contrala terales obliga a pensar en un síndrome alterno por compromiso del tronco encefálico. La resonancia magnética nuclear es el estudio de elección para caracterizar y localizar la lesión. Presentamos el caso de un hombre de 71 años que sufrió parálisis del tercer par derecho y hemiataxia izquierda, cuadro infrecuente, conocido como síndrome de Claude.

Abstract Midbrain strokes are rare and are usually accompanied by other concomitant injuries. The simultaneous presence of ipsi and contralateral signs makes it necessary to think of a brainstem syndrome due to involvement of the brainstem. Mag netic nuclear resonance is the study of choice to characterize and locate the lesion. We report the case of a 71-year-old man who presented right third cranial nerve palsy and hemiataxia, a rare condition known as Claude's syndrome.

Nimotuzumab combinado con radioterapia en el tratamiento de tumores infiltrantes y difusos del tallo cerebral en niños y adolescentes

RESUMEN Introducción: Los gliomas infiltrantes y difusos del tallo cerebral en los niños y adolescentes comportan un mal pronóstico y un tiempo corto de supervivencia. Objetivo: Presentar los resultados obtenidos con la combinación de nimotuzumab y radioterapia en tumores del tallo cerebral en niños y adolescentes. Métodos: El anticuerpo monoclonal nimotuzumab combinado con la radioterapia fue aplicado en una serie de 69 pacientes con edades entre 2 y 18 años, incluidos desde 2008 hasta 2020, y seguidos hasta agosto de 2021. Fueron irradiados en el Instituto Nacional de Oncología y Radiobiología de La Habana, con una dosis en un rango de 54 a 59 Gy. El nimotuzumab se aplicó a la dosis de 150 mg/m2 de superficie corporal, una vez por semana mientras duró el tratamiento radiante, luego cada 2 semanas con igual dosis, y finalmente, una vez por mes durante 1, 2 o más años. Resultados: Al terminar el tratamiento combinado de la radioterapia y el nimotuzumab existió respuesta en 89,9 % de los pacientes. La mediana del tiempo de supervivencia global fue de 18,8 meses y las tasas de supervivencia de la serie fueron de 76,4; 45,4; 38,9; 32,8 y 31,5 % a uno, dos, tres, cuatro, cinco y más años, respectivamente, estabilizada a partir de este tiempo hasta diez años y más. Tres pacientes en recaída fueron reirradiados. Conclusiones: La combinación de radioterapia y nimotuzumab fue bien tolerada y es una opción de tratamiento en tumores del tallo cerebral en niños y adolescentes.

ABSTRACT Introduction: Infiltrating and diffuse gliomas of the brain stem in children and adolescents lead to a poor prognosis and a short survival time. Objective: Present the results obtained with the combination of nimotuzumab and radiotherapy in brain stem tumors in children and adolescents. Methods: The monoclonal antibody called nimotuzumab combined with radiotherapy was applied in a series of 69 patients aged between 2 and 18 years, from 2008 to 2020, and followed until August 2021. They were irradiated at the National Institute of Oncology and Radiobiology in Havana, with a dose in a range of 54 to 59 Gy. Nimotuzumab was applied at the dose of 150 mg/m2 of body surface area, once a week while the radiation treatment was active, then every 2 weeks with the same dose, and finally once a month for 1, 2 or more years. Results: At the end of the combined treatment of radiotherapy and nimotuzumab, there was a response in 89.9% of the patients. The median overall survival time was 18.8 months and series survival rates were 76.4; 45.4; 38.9; 32.8; and 31.5% at one, two, three, four, five and more years, respectively, and it was stabilized from this time to 10 years and more. Three relapsed patients were re-radiated. Conclusions: The combination of radiotherapy and nimotuzumab was well tolerated and is an option for the treatment in brain stem tumors in children and adolescents.

Hipoacusia sensorineural asimétrica en el diagnóstico de schwannoma vestibular / Asymmetric sensorineural hearing loss in the diagnosis of vestibular schwannoma

Introducción: se ha descrito que los pacientes con hipoacusia asimétrica cursan en un 2% con lesiones retrococleares. La conducta de escrutinio se ha basado en la audiometría. Existen varias definiciones de asimetría audiométrica descritas en la literatura, pero sin llegar a un consenso. Aunque la prueba de oro para el diagnóstico de schwannoma vestibular es la resonancia magnética con gadolíneo, la sospecha clínica se hace en base a la asimetría audiométrica. Objetivo: hacemos una revisión de los trabajos publicados al respecto en la literatu-ra y comentamos nuestra experiencia. Reflexión: queremos enfatizar en la importancia de estudiar a los pacientes con hipoacusia asimétrica con el fin de descartar patología retrococlear. Conclusión: a pesar de que no existe un consenso claro en la definición de hi-poacusia asimétrica, la sospecha clínica de un schwannoma vestibular se basa en la audiometría

Background: It is described that 2% of patients with asymmetric hearing loss have retrocochlear lesions. The scrutiny behavior has been based on audiometry. There are several definitions of audiometric asymmetry described in the literature, but without reaching a consensus. Although the gold standard for the diagnosis of vestibular schwannoma is gadolinium magnetic resonance imaging, the clinical sus-picion is based on audiometric asymmetry. Objective: we review the results published in this regard in the literature and com-ment on our own experience. Reflection: we want to emphasize the importance of studying asymmetric hearing loss patients in order to rule out retrocochlear etiology. Conclusions: although there is no clear consensus on the definition of asym-metric hearing loss, the clinical suspicion of a vestibular schwannoma is based on the audiometry.

The value of MR amide proton transfer weighted imaging technique in predicting the pathological grade of brainstem glioma / 中华放射学杂志

Objective:To evaluate the clinical application value of MR amide proton transfer weighted imaging (APTWI) in predicting the pathological grade of brainstem glioma (BSG).Methods:The data of 41 BSG patients in Beijing Tiantan Hospital, Capital Medical University from August 2019 to June 2020 who underwent both MRI and APTWI 2 weeks before surgery and had pathological grading results were retrospectively analyzed. According to the pathological results, 41 patients were classified into high-grade BSG (20 patients) and low-grade BSG (21 patients). Combined with conventional MR images, the signal intensity (%) of amide proton transfer (APT) in the parenchymal area of the tumor was obtained on APTWI images. χ 2 test or independent sample t-test was used to analyze the differences in gender distribution, age and APT signal intensity between patients with high and low grade BSG. Receiver operating characteristic (ROC) curve was drawn to predict the efficacy of APT signal intensity in the differential diagnosis of high and low grade BSG, and Youden index was calculated to obtain the optimal diagnostic threshold; the predictive ability of APT signal intensity was analyzed in combination with Hosmer-Lemeshow goodness of fit test. Results:There was no significant difference in age [(23±18) years, (20±17) years, t=0.97, P=0.340] and gender distribution (9/11, 9/12 for males/females, χ 2=0.02, P=0.890) between high-grade and low-grade BSG patients. The APT signal intensity of high-grade BSG [(3.9±0.9)%] was significantly higher than that of low-grade BSG [(2.8±0.9)%], and the difference had statistical significance ( t=4.16, P<0.001). The area under the ROC curve of APT signal intensity to distinguish high-grade and low grade BSG was 0.836, and with 2.85% as the optimal diagnostic threshold of APT signal intensity, its sensitivity for the diagnosis of high-grade BSG was 90.0% and specificity was 71.4%. The Hosmer-Lemeshow test showed that APTWI had a good predictive ability for BSG grade (χ 2=13.33, P=0.101). Conclusion:APTWI can be applied in distinguishing high grade BSG from low grade BSG, and has clinical value in predicting glioma grading.

Sequential treatment with tirofiban and argatroban in acute isolated pontine infarction caused by branch atheromatous disease / 国际脑血管病杂志

Objective:To investigate the efficacy and safety of sequential treatment with tirofiban and argatroban in acute isolated pontine infarction (AIPI) caused by branch atheromatous disease (BAD).Methods:Consecutive patients with AIPI caused by BAD within 48 h of onset and admitted to Zhengzhou Central Hospital from April 2021 to April 2022 were enrolled retrospectively. The patients were divided into sequential treatment group and tirofiban group according to their therapeutic modalities. In the tirofiban group, tirofiban was pumped intravenously within 48 h after admission, and dual antiplatelet therapy with aspirin and clopidogrel was added 4 h before tirofiban was discontinued. On the basis of tirofiban treatment, the sequential treatment group was followed by argatroban for 5 days when tirofiban is discontinued. The main outcome measure was the modified Rankin Scale (mRS) score at 3 months after the onset . A score of <2 was defined as a good outcome. The secondary outcome measure was all the adverse events during the treatment and follow-up. Multivariate logistic regression analysis was used to determine the independent factors of the outcomes. Results:A total of 64 patients with AIPI caused by BAD were enrolled, including 32 in the sequential treatment group and 32 in the tirofiban group. There was no statistical difference in baseline data between the two groups, but the rate of good outcomes at 3 months after onset in the sequential treatment group was significantly higher than that in the tirofiban group (78.1% vs. 50.0%; χ2=5.497, P=0.019). Multivariate logistic regression analysis showed that after adjusting for low-density lipoprotein cholesterol, the higher baseline National Institutes of Health Stroke Scale score was independently associated with the poor outcomes (odds ratio 2.067, 95% confidence interval 1.343-3.182; P=0.001), while the sequential treatment was independently associated with the good outcomes (odds ratio 0.248, 95% confidence interval 0.064-0.957; P=0.043). Conclusion:Early application of sequential treatment with tirofiban and argatroban in AIPI caused by BAD may effectively improve the outcomes of patients, and the safety is good.