ChOP-CT: quantitative morphometrical analysis of the Hindbrain Choroid Plexus by X-ray micro-computed tomography.

The Hindbrain Choroid Plexus is a complex, cerebrospinal fluid-secreting tissue that projects into the 4th vertebrate brain ventricle. Despite its irreplaceability in the development and homeostasis of the entire central nervous system, the research of Hindbrain Choroid Plexus and other Choroid Plexuses has been neglected by neuroscientists for decades. One of the obstacles is the lack of tools that describe the complex shape of the Hindbrain Choroid Plexus in the context of brain ventricles. Here we introduce an effective tool, termed ChOP-CT, for the noninvasive, X-ray micro-computed tomography-based, three-dimensional visualization and subsequent quantitative spatial morphological analysis of developing mouse Hindbrain Choroid Plexus. ChOP-CT can reliably quantify Hindbrain Choroid Plexus volume, surface area, length, outgrowth angle, the proportion of the ventricular space occupied, asymmetries and general shape alterations in mouse embryos from embryonic day 13.5 onwards. We provide evidence that ChOP-CT is suitable for the unbiased evaluation and detection of the Hindbrain Choroid Plexus alterations within various mutant embryos. We believe, that thanks to its versatility, quantitative nature and the possibility of automation, ChOP-CT will facilitate the analysis of the Hindbrain Choroid Plexus in the mouse models. This will ultimately accelerate the screening of the candidate genes and mechanisms involved in the onset of various Hindbrain Choroid Plexus-related diseases.

Epidemiology, clinical and imaging features of rhombencephalitis caused by L. monocytogenes. A retrospective observational study. / Epidemiología, clínica y resultados de imagen de rombencefalitis causada por L. monocytogenes. Un estudio observacional.

INTRODUCTION: To date, few studies have explored the specific risk factors of patients with listeriosis who develop rhombencephalitis, and there is insufficient information regarding imaging findings and clinical symptoms in patients with this disease. This work aimed to analyze the imaging findings associated with L. monocytogenes rhombencephalitis in a cohort of patients with listeriosis. MATERIALS AND METHODS: We conducted a retrospective observational study of all declared cases of listeriosis in a tertiary hospital from Granada, Spain, from 2008 to 2021. Risk factors, comorbidities, and clinical outcomes were collected for all patients. In addition, clinical symptoms and magnetic resonance imaging (MRI) findings were included for those patients who developed rhombencephalitis. Descriptive and bivariate analyses were performed using SPSS statistical software (IBM SPSS, version 21). RESULTS: Our cohort comprised 120 patients with listeriosis (41.7% women, mean age: 58.6 ± 23.8 years), of which 10 (8.3%) had rhombencephalitis. The most frequent MRI findings in patients with confirmed rhombencephalitis were T2-FLAIR hyperintensity (100%), T1 hypointensity (80%), scattered parenchymal enhancement (80%), and cranial nerve enhancement (70%), while the most frequent anatomical involvement were pons, medulla oblongata, and cerebellum. Complications occurred in 6 patients (abscess in 4, hemorrhage in 2, hydrocephalus in 1). CONCLUSIONS: Rhombencephalitis is associated with an increased in-hospital mortality in patients with listeriosis. The anatomical distribution and imaging characteristics of neurolisteriosis could be useful to suggest the diagnosis. Future studies with greater sample size should explore the association between anatomical location, imaging patterns, and associated complications (e.g., hydrocephalus, hemorrhage), and clinical outcomes.

TITLE: Epidemiología, clínica y resultados de imagen de rombencefalitis causada por L. monocytogenes. Un estudio observacional.Introducción. Hasta la fecha, pocos estudios han explorado los factores de riesgo específicos de los pacientes con listeriosis que desarrollan rombencefalitis, y no hay suficiente información sobre los hallazgos de imagen y los síntomas clínicos en pacientes con esta enfermedad. El objetivo de este trabajo fue analizar los hallazgos de imagen asociados a la rombencefalitis por L. monocytogenes en una cohorte de pacientes con listeriosis. Materiales y métodos. Se realizó un estudio observacional retrospectivo de todos los casos declarados de listeriosis en un hospital terciario de Granada, España, desde 2008 hasta 2021. Se recogieron los factores de riesgo, las comorbilidades y los resultados clínicos de todos los pacientes. Además, se incluyeron los síntomas clínicos y los hallazgos de resonancia magnética (RM) de los pacientes que desarrollaron rombencefalitis. Se realizaron análisis descriptivos y bivariados utilizando el software estadístico SPSS (IBM SPSS, versión 21). Resultados. Nuestra cohorte incluyó a 120 pacientes con listeriosis (41,7%, mujeres; edad media: 58,6 ± 23,8 años), de los cuales 10 (8,3%) tenían rombencefalitis. Los hallazgos más frecuentes en la RM de los pacientes con rombencefalitis confirmada fueron hiperintensidad en T2-FLAIR (100%), hipointensidad en T1 (80%), realce parenquimatoso disperso (80%) y realce de los nervios craneales (70%), mientras que la afectación anatómica más frecuente fue en la protuberancia, la médula oblongada y el cerebelo. Se produjeron complicaciones en seis pacientes (absceso en cuatro, hemorragia en dos e hidrocefalia en uno). Conclusiones. La rombencefalitis se asocia a un aumento de la mortalidad intrahospitalaria en pacientes con listeriosis. La distribución anatómica y las características de imagen de la neurolisteriosis podrían ser útiles para sugerir el diagnóstico. Futuros estudios con mayor tamaño muestral deberían explorar la asociación entre la localización anatómica, los patrones de imagen y las complicaciones asociadas (por ejemplo, hidrocefalia y hemorragia), y los resultados clínicos.

Epidemiología, clínica y resultados de imagen de rombencefalitis causada por L. monocytogenes. Un estudio observacional / Epidemiology, clinical and imaging features of rhombencephalitis caused by L. monocytogenes. A retrospective observational study

Introducción: Hasta la fecha, pocos estudios han explorado los factores de riesgo específicos de los pacientes con listeriosis que desarrollan rombencefalitis, y no hay suficiente información sobre los hallazgos de imagen y los síntomas clínicos en pacientes con esta enfermedad. El objetivo de este trabajo fue analizar los hallazgos de imagen asociados a la rombencefalitis por L. monocytogenes en una cohorte de pacientes con listeriosis. Materiales y métodos: Se realizó un estudio observacional retrospectivo de todos los casos declarados de listeriosis en un hospital terciario de Granada, España, desde 2008 hasta 2021. Se recogieron los factores de riesgo, las comorbilidades y los resultados clínicos de todos los pacientes. Además, se incluyeron los síntomas clínicos y los hallazgos de resonancia magnética (RM) de los pacientes que desarrollaron rombencefalitis. Se realizaron análisis descriptivos y bivariados utilizando el software estadístico SPSS (IBM SPSS, versión 21). Resultados: Nuestra cohorte incluyó a 120 pacientes con listeriosis (41,7%, mujeres; edad media: 58,6 ± 23,8 años), de los cuales 10 (8,3%) tenían rombencefalitis. Los hallazgos más frecuentes en la RM de los pacientes con rombencefalitis confirmada fueron hiperintensidad en T2-FLAIR (100%), hipointensidad en T1 (80%), realce parenquimatoso disperso (80%) y realce de los nervios craneales (70%), mientras que la afectación anatómica más frecuente fue en la protuberancia, la médula oblongada y el cerebelo. Se produjeron complicaciones en seis pacientes (absceso en cuatro, hemorragia en dos e hidrocefalia en uno). Conclusiones: La rombencefalitis se asocia a un aumento de la mortalidad intrahospitalaria en pacientes con listeriosis. La distribución anatómica y las características de imagen de la neurolisteriosis podrían ser útiles para sugerir el diagnóstico. Futuros estudios con mayor tamaño muestral deberían explorar la asociación entre la...

Introduction: To date, few studies have explored the specific risk factors of patients with listeriosis who develop rhombencephalitis, and there is insufficient information regarding imaging findings and clinical symptoms in patients with this disease. This work aimed to analyze the imaging findings associated with L. monocytogenes rhombencephalitis in a cohort of patients with listeriosis. Materials and methods: We conducted a retrospective observational study of all declared cases of listeriosis in a tertiary hospital from Granada, Spain, from 2008 to 2021. Risk factors, comorbidities, and clinical outcomes were collected for all patients. In addition, clinical symptoms and magnetic resonance imaging (MRI) findings were included for those patients who developed rhombencephalitis. Descriptive and bivariate analyses were performed using SPSS statistical software (IBM SPSS, version 21). Results: Our cohort comprised 120 patients with listeriosis (41.7% women, mean age: 58.6 ± 23.8 years), of which 10 (8.3%) had rhombencephalitis. The most frequent MRI findings in patients with confirmed rhombencephalitis were T2-FLAIR hyperintensity (100%), T1 hypointensity (80%), scattered parenchymal enhancement (80%), and cranial nerve enhancement (70%), while the most frequent anatomical involvement were pons, medulla oblongata, and cerebellum. Complications occurred in 6 patients (abscess in 4, hemorrhage in 2, hydrocephalus in 1). Conclusions: Rhombencephalitis is associated with an increased in-hospital mortality in patients with listeriosis. The anatomical distribution and imaging characteristics of neurolisteriosis could be useful to suggest the diagnosis. Future studies with greater sample size should explore the association between anatomical location, imaging patterns, and associated complications (e.g., hydrocephalus, hemorrhage), and clinical outcomes.(AU)

The significance of hindbrain herniation reversal following prenatal repair of neural tube defects.

OBJECTIVE: The aim of this study was to determine whether reversal of hindbrain herniation (HBH) on MRI following prenatal repair of neural tube defects (NTDs) is associated with reduced rates of ventriculoperitoneal (VP) shunt placement or endoscopic third ventriculostomy (ETV) within the 1st year of life. METHODS: This is a secondary analysis of prospectively collected data from all patients who had prenatal open repair of a fetal NTD at a single tertiary care center between 2012 and 2020. Patients were offered surgery according to inclusion criteria from the Management of Myelomeningocele Study (MOMS). Patients were excluded if they were lost to follow-up, did not undergo postnatal MRI, or underwent postnatal MRI without a report assessing hindbrain status. Patients with HBH reversal were compared with those without HBH reversal. The primary outcome assessed was surgical CSF diversion (i.e., VP shunt or ETV) within the first 12 months of life. Secondary outcomes included CSF leakage, repair dehiscence, CSF diversion prior to discharge from the neonatal intensive care unit (NICU), and composite neonatal morbidity. Demographic, prenatal sonographic, and operative characteristics as well as outcomes were assessed using standard univariate statistical methods. Multivariate logistic regression models were fit to assess for independent contributions to the primary and secondary outcomes. RESULTS: Following exclusions, 78 patients were available for analysis. Of these patients, 38 (48.7%) had HBH reversal and 40 (51.3%) had persistent HBH on postnatal MRI. Baseline demographic and preoperative ultrasound characteristics were similar between groups. The primary outcome of CSF diversion within the 1st year of life was similar between the two groups (42.1% vs 57.5%, p = 0.17). All secondary outcomes were also similar between groups. Patients who had occurrence of the primary outcome had greater presurgical lateral ventricle width than those who did not (16.1 vs 12.1 mm, p = 0.02) when HBH was reversed, but not when HBH was persistent (12.5 vs 10.7 mm, p = 0.49). In multivariate analysis, presurgical lateral ventricle width was associated with increased rates of CSF diversion before 12 months of life (adjusted OR 1.18, 95% CI 1.03-1.35) and CSF diversion prior to NICU discharge (adjusted OR 1.18, 95% CI 1.02-1.37). CONCLUSIONS: HBH reversal was not associated with decreased rates of CSF diversion in this cohort. Predictive accuracy of the anticipated benefits of prenatal NTD repair may not be augmented by the observation of HBH reversal on MRI.

Listeria Rhombencephalitis and Infratentorial Brain Abcesses in a Young Adult with Stroke Like Presentation.

Rhombencephalitis refers to the inflammation of rhombencephalon, and Listeria monocytogenes is one of the causes of infectious rhombencephalitis. Listeria rhombencephalitis is a rare and severe infection with high mortality and morbidity. As the disease can present with a variety of neurological symptoms and nonspecific laboratory tests, it can easily be misdiagnosed. Sudden onset of neurological signs without fever can resemble stroke. Magnetic resonance imaging can be useful in patients for confirmation of the diagnosis and during the follow-up. Early diagnosis and treatment are especially important for improvement of the outcomes. Here we report a case with stroke-like presentation that was diagnosed as Listeria rhombencephalitis in follow-up and present the serial brain magnetic resonance imaging features.

Statistical shape models of the posterior cranial fossa and hindbrain volumes may provide a more robust clinical metric for Chiari malformation.

Chiari malformation is characterised by the herniation of the cerebellar tonsils through the foramen magnum. However, tonsillar herniation and other 2D morphometric measurements of the posterior cranial fossa (PCF) have a weak association with patients' symptoms and clinical outcomes. This study aimed to contrast current 2D metrics with a novel 3D shape analysis of the PCF and the hindbrain, to determine if 3D measurements provides further insight into the pathophysiology of Chiari. The cranium of 12 controls and 21 Chiari malformation patients with (N = 9) and without (N = 12) a syrinx were scanned. The morphology of the PCF was quantified with typical 2D measurements. Additionally, a correspondence-based shape model that normalised the PCF volume, was used to find 3D differences in the shape of the PCF, and the distance of the hindbrain from the inner surfaces of the PCF. Shape analysis showed that, compared to controls, the caudal (p = 0.007; 2.3 mm, IQR: 1.6-3.3 mm) and anterior (p = 0.027; 1.3 mm,IQR: 1.1-1.6 mm) surfaces of the hindbrain were closer to the PCF in patients with and without a syrinx, respectively. However, there were negligible differences in the shape of the PCF between patient groups (p > 0.39). Current morphometric measures should be normalised for variation in PCF volume, so that shape measures are not biased. The reduced CSF space between the hindbrain and PCF will alter CSF dynamics, which may compress cerebellar vasculature and contribute to patient symptomatology.

Vascular supply of the hindbrain: Basic longitudinal and axial angioarchitecture.

The basic pattern of arterial vascularization is highly conserved across vertebrates and develops under neuromeric rules. The hindbrain has an angioarchitecture that is homologous to that of the spinal cord, and the hindbrain vascular system can be analyzed at the longitudinal and axial structures. During development, there are two main longitudinal arteries: the longitudinal neural artery and primitive lateral basilovertebral anastomosis. This review discusses the basic pattern of the blood supply of the hindbrain, the development of vascularization, and the anatomical variations, with a special reference to the embryological point of view of two main longitudinal anastomoses (longitudinal neural artery and primitive lateral basilovertebral anastomosis). The formation of commonly observed variations, such as fenestration and duplication of the vertebrobasilar artery, or primitive trigeminal artery variant, can be explained by the partial persistence of the primitive lateral basilovertebral anastomosis. Understanding the pattern and the development of the blood supply of the hindbrain provides useful information of the various anomalies of the vertebrobasilar junction and cerebellar arteries.

Early second-trimester three-dimensional transvaginal neurosonography of fetal midbrain and hindbrain: normative data and technical aspects.

OBJECTIVES: To provide a detailed description of the sonographic appearance and development of various fetal structures of the midbrain and hindbrain (MBHB) during the early second trimester, and to evaluate the impact of the frequency of the transvaginal sonography (TVS) transducer on the early recognition of these structures. METHODS: This was a retrospective analysis of three-dimensional volumetric datasets of the MBHB from apparently normal fetuses at 14-19 gestational weeks, acquired by TVS in the midsagittal view through the posterior fontanelle. Using a multiplanar approach, we measured the tectal thickness and length, aqueductal thickness, tegmental thickness and width and height of the Blake's pouch (BP) neck. In addition, we assessed the existence of early vermian fissures, the linear shape of the brainstem and the components of the fastigium. The correlation between gestational age according to last menstrual period and sonographic measurements of MBHB structures was evaluated using Pearson's correlation (r). A subanalysis was performed to assess the performance of a 5-9-MHz vs a 6-12-MHz TVS transducer in visualizing the MBHB structures in the early second trimester. RESULTS: Sixty brain volumes were included in the study, obtained at a mean gestational age of 16.2 weeks (range, 14.1-19.0 weeks), with a transverse cerebellar diameter range of 13.0-19.8 mm. We found a strong correlation between gestational age and all MBHB measurements, with the exception of the tectal, tegmental and aqueductal thicknesses, for which the correlation was moderate. There was good-to-excellent intraobserver and moderate-to-good interobserver correlation for most MBHB measurements. We observed that the BP neck was patent in all fetuses between 14 and 18 weeks with decreasing diameter, and that the aqueductal thickness was significantly smaller at ≥ 18 weeks compared with at < 16 weeks. The early vermian fissures and the linear shape of the brainstem were present in all fetuses from 14 weeks. We found that, in the early second trimester, the horizontal arm of the presumed 'fastigium' evolves from the fourth ventricular choroid plexus and not the posterior vermis, indicating that this is not the fastigium. Standard- and high-resolution TVS transducers performed similarly in the assessment of MBHB anatomy. CONCLUSION: Detailed early second-trimester assessment of the MBHB is feasible by transvaginal neurosonography and provides reference data which may help in the early detection of brain pathology involving the MBHB. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Post-infectious rhombencephalitis after coronavirus-19 infection: A case report and literature review.

A wide number of neurological manifestations have been described in association with coronavirus disease 19 (COVID-19). We describe an unusual case of a young man who developed severe rhombencephalitis after COVID-19. He demonstrated clinical and radiological improvement with high dose corticosteroids, plasma exchange and intravenous immune globulin. Our findings, along with previously reported cases that we review here, support an autoimmune para- or post-infectious mechanism and highlight a possible role for immunotherapy in patients with rhombencephalitis after COVID-19.

Prenatal diagnosis of rhombencephalosynapsis: neuroimaging features and severity of vermian anomaly.

OBJECTIVES: To describe the prenatal neuroimaging spectrum of rhombencephalosynapsis (RES) and criteria for its classification according to the severity of vermian anomaly. METHODS: In this multicenter retrospective study of fetuses with RES between 2002 and 2020, the medical records and brain ultrasound and magnetic resonance images were evaluated comprehensively to determine the severity of the vermian anomaly and the presence of associated brain findings. RES was classified, according to the pattern of vermian agenesis and the extent of the fusion of the hemispheres, as complete RES (complete absence of the vermis) or partial RES (further classified according to the part of the vermis that was missing and, consequently, the region of hemispheric fusion, as anterior, posterior, severe or mixed RES). Findings were compared between cases with complete and those with partial RES. RESULTS: Included in the study were 62 fetuses with a gestational age ranging between 12 and 37 weeks. Most had complete absence of the vermis (complete RES, 77.4% of cases), a 'round-shaped' cerebellum on axial views (72.6%) and a transverse cerebellar diameter (TCD) < 3rd centile (87.1%). Among the 22.6% of cases with partial RES, 6.5% were classified as severe partial, 6.5% as partial anterior, 8.1% as partial mixed and 1.6% as partial posterior. Half of these cases presented with normal or nearly normal cerebellar morphology and 28.5% had a TCD within the normal limits. Infratentorially, the fourth ventricle was abnormal in 88.7% of cases overall, and anomalies of the midbrain and pons were frequent (93.5% and 77.4%, respectively). Ventriculomegaly was observed in 80.6% of all cases, being more severe in cases with complete RES than in those with partial RES, with high rates of parenchymal and septal disruption. CONCLUSIONS: This study provides prenatal neuroimaging criteria for the diagnosis and classification of RES, and identification of related features, using ultrasound and magnetic resonance imaging. According to our findings, a diagnosis of RES should be considered in fetuses with a small TCD (severe cerebellar hypoplasia) and/or a round-shaped cerebellum on axial views, during the second or third trimester, especially when associated with ventriculomegaly. Partial RES is more common than previously thought, but presents an extreme diagnostic challenge, especially in cases with normal or nearly-normal cerebellar morphobiometric features. © 2021 International Society of Ultrasound in Obstetrics and Gynecology.

Rhombencephalitis due to Listeria monocytogenes infection with GQ1b antibody positivity and multiple intracranial hemorrhage: a case report and literature review.

Listeria monocytogenes is a Gram-positive facultative intracellular bacterium that causes central nervous system infection. We report a case of rhombencephalitis caused by L. monocytogenes infection, which mimicked Bickerstaff's brainstem encephalitis, and GQ1b antibody positivity and multiple intracranial foci were observed. A 68-year-old male patient presented with a nonspecific prodrome of faintness, forehead tightness, and walking instability. This was followed by progressive cranial nerve palsies, limb weakness, cerebellar signs, hyperpyrexia, and impaired consciousness. Brain imaging showed multiple abnormal brainstem and cerebellar signals that were accompanied by blood infiltration without any lesion enhancement. Serum GQ1b antibody positivity led to an initial diagnosis of Bickerstaff's brainstem encephalitis, which was treated with immunosuppressive therapy with limited efficacy. A pathogen examination helped confirm L. monocytogenes infection. A combination of meropenem and trimethoprim-sulfamethoxazole therapy was applied and the patient recovered without sequelae. The symptoms and imaging of Listeria rhombencephalitis are nonspecific. Accurate diagnosis and prompt treatment of this condition are essential. Whether Listeria infection triggers an autoimmune response remains unclear.

Hindbrain Herniation and Banana and Lemon Sign After Open Fetal Myelomeningocele Repair - When Do These Signs Disappear and is Shunting Predictable?

PURPOSE: The aim was to describe the sonographic follow-up of hindbrain herniation (HH), the banana and lemon sign after fetal myelomeningocele (fMMC) repair, and the time of disappearance of these signs after the intervention, and to investigate any predictive value for the necessity of shunting during the infant's first year of life. Additionally, the sonographic evolution of the transcerebellar diameter (TCD) before and after fetal intervention was assessed. PATIENTS AND METHODS: The first 50 patients that underwent fMMC repair at Zurich Center for Fetal Diagnosis and Therapy (www.swissfetus.ch) were included in this study. Sonographic scans performed weekly after fMMC repair focusing on HH and banana and lemon signs were analyzed and compared between the shunted and the non-shunted group. ROC curves were generated for the time intervals of resolution of the signs in order to show their predictive accuracy for the need for shunting until 1 year of age. RESULTS: HH resolved in 48 fetuses (96 %) before delivery. The sonographic disappearance of HH within the first two weeks after fMMC repair was associated with a significantly lower incidence of shunt placement (OR 0.19; 95 % CI 0.4-0.9) during the first year of life (p = 0.03). All fetuses with persistent HH before delivery received a shunt. TCD growth was observed in all fetuses. CONCLUSION: The reversibility of HH within two weeks after fMMC repair is associated with an 80 % lower incidence of shunt placement during the infant's first year of life. Moreover, it allows the cerebellum to grow and to normalize its configuration.

Clinical and molecular evaluation of 13 Brazilian patients with Gomez-López-Hernández syndrome.

We aim to characterize patients with Gomez-López-Hernández syndrome (GLHS) clinically and to investigate them molecularly. A clinical protocol, including a morphological and neuropsychological assessment, was applied to 13 patients with GLHS. Single-nucleotide polymorphism (SNP) array and whole-exome sequencing were undertaken; magnetic resonance imaging was performed in 12 patients, including high-resolution, heavily T2-weighted sequences (HRT2) in 6 patients to analyze the trigeminal nerves. All patients presented alopecia; two did not present rhombencephalosynapsis (RES); trigeminal anesthesia was present in 5 of the 11 patients (45.4%); brachycephaly/brachyturricephaly and mid-face retrusion were found in 84.6 and 92.3% of the patients, respectively. One patient had intellectual disability. HRT2 sequences showed trigeminal nerve hypoplasia in four of the six patients; all four had clinical signs of trigeminal anesthesia. No common candidate gene was found to explain GLHS phenotype. RES does not seem to be an obligatory finding in respect of GLHS diagnosis. We propose that a diagnosis of GLHS should be considered in patients with at least two of the following criteria: focal non-scarring alopecia, rhombencephalosynapsis, craniofacial anomalies (brachyturrycephaly, brachycephaly or mid-face retrusion), trigeminal anesthesia or anatomic abnormalities of the trigeminal nerve. Studies focusing on germline whole genome sequencing or DNA and/or RNA sequencing of the alopecia tissue may be the next step for the better understanding of GLHS etiology.

Fetal Intraventricular Hemorrhage in Open Neural Tube Defects: Prenatal Imaging Evaluation and Perinatal Outcomes.

BACKGROUND AND PURPOSE: Fetal imaging is crucial in the evaluation of open neural tube defects. The identification of intraventricular hemorrhage prenatally has unclear clinical implications. We aimed to explore fetal imaging findings in open neural tube defects and evaluate associations between intraventricular hemorrhage with prenatal and postnatal hindbrain herniation, postnatal intraventricular hemorrhage, and ventricular shunt placement. MATERIALS AND METHODS: After institutional review board approval, open neural tube defect cases evaluated by prenatal sonography between January 1, 2013 and April 24, 2018 were enrolled (n = 504). The presence of intraventricular hemorrhage and gray matter heterotopia by both prenatal sonography and MR imaging studies was used for classification. Cases of intraventricular hemorrhage had intraventricular hemorrhage without gray matter heterotopia (n = 33) and controls had neither intraventricular hemorrhage nor gray matter heterotopia (n = 229). A total of 135 subjects with findings of gray matter heterotopia were excluded. Outcomes were compared with regression analyses. RESULTS: Prenatal and postnatal hindbrain herniation and postnatal intraventricular hemorrhage were more frequent in cases of prenatal intraventricular hemorrhage compared with controls (97% versus 79%, 50% versus 25%, and 63% versus 12%, respectively). Increased third ventricular diameter, specifically >1 mm, predicted hindbrain herniation (OR = 3.7 [95% CI, 1.5-11]) independent of lateral ventricular size and prenatal intraventricular hemorrhage. Fetal closure (n = 86) was independently protective against postnatal hindbrain herniation (OR = 0.04 [95% CI, 0.01-0.15]) and postnatal intraventricular hemorrhage (OR = 0.2 [95% CI, 0.02-0.98]). Prenatal intraventricular hemorrhage was not associated with ventricular shunt placement. CONCLUSIONS: Intraventricular hemorrhage is relatively common in the prenatal evaluation of open neural tube defects. Hindbrain herniation is more common in cases of intraventricular hemorrhage, but in association with increased third ventricular size. Fetal closure reverses hindbrain herniation and decreases the rate of intraventricular hemorrhage postnatally, regardless of the presence of prenatal intraventricular hemorrhage.

Hindbrain Double-Negative Feedback Mediates Palatability-Guided Food and Water Consumption.

Hunger and thirst have distinct goals but control similar ingestive behaviors, and little is known about neural processes that are shared between these behavioral states. We identify glutamatergic neurons in the peri-locus coeruleus (periLCVGLUT2 neurons) as a polysynaptic convergence node from separate energy-sensitive and hydration-sensitive cell populations. We develop methods for stable hindbrain calcium imaging in free-moving mice, which show that periLCVGLUT2 neurons are tuned to ingestive behaviors and respond similarly to food or water consumption. PeriLCVGLUT2 neurons are scalably inhibited by palatability and homeostatic need during consumption. Inhibition of periLCVGLUT2 neurons is rewarding and increases consumption by enhancing palatability and prolonging ingestion duration. These properties comprise a double-negative feedback relationship that sustains food or water consumption without affecting food- or water-seeking. PeriLCVGLUT2 neurons are a hub between hunger and thirst that specifically controls motivation for food and water ingestion, which is a factor that contributes to hedonic overeating and obesity.

Listeria rhombencephalitis mimicking acute disseminated encephalomyelitis in a patient without predisposing medical conditions.

Listeria rhombencephalitis (L. rhombencephalitis) is an uncommon form of central nervous system infection caused by Listeria monocytogenes (LM). It often occurs to immunocompetent individuals. Here, we described the case of a 45-year-old female patient without medical histories, who presented for high-grade fever, headache, and focal neurological manifestations. She was initially empirically diagnosed with acute disseminated encephalomyelitis (ADEM) because of clinical symptoms, acute clinical course, and neuroimaging. However, the biochemical analysis of cerebral spinal fluid (CSF) questioned the diagnosis of ADEM. The final diagnosis of L. rhombencephalitis was based on CSF culture for LM. Thus, L. rhombencephalitis should be preferentially and empirically considered for a patient with significantly elevated lactic acid and moderately increased red cells in CSF at early time, accompanied with rapidly progressive neurological dysfunctions involved in the brain stem.

Gomez-López-Hernández syndrome: A case report with clinical and molecular evaluation and literature review.

Gomez-López-Hernández syndrome (GLHS) is characterized by rhombencephalosynapsis (RES), alopecia, trigeminal anesthesia and a distinctive phenotype, including brachyturricephaly. It has been suggested that GLHS should be considered as part of the spectrum of RES-associated conditions that include alopecia, trigeminal anesthesia, and craniofacial anomalies, rather than a distinct entity. To the best of our knowledge, 57 patients with GLHS have been described. Despite its first description in 1979, the etiology of this syndrome remains unknown. Here, we describe, to our knowledge, the first case of a patient with GLHS who was molecularly evaluated and had been prenatally exposed to misoprostol. We also reviewed the clinical and morphological features of the patients described to date to better delineate the phenotype and focus on any evidence for adverse pregnancy outcomes or exposure, including teratogens.