Variante rara de RNF213 en adolescente con enfermedad de moyamoya / Rare RNF213 variant in adolescent with moyamoya disease

Introducción: La enfermedad de moyamoya es una enfermedad estenooclusiva progresiva de las principales arterias intracraneales. Los individuos afectados corren el riesgo de sufrir un accidente cerebrovascular hemorrágico o isquémico intracraneal, deterioro cognitivo y retrasos en el desarrollo. Se han identificado varios genes de susceptibilidad. La variante p.R4810K en el gen RNF213 se ha identificado en el 95% de los pacientes con enfermedad de moyamoya familiar. Caso clínico: Presentamos el caso de una adolescente de 15 años que se presentó con quejas principales de disgrafía y falta de coordinación en la mano derecha con dos meses de evolución. La resonancia magnética cerebral reveló varias lesiones isquémicas con diferentes ritmos de evolución y la angiorresonancia magnética mostró múltiples estenosis suboclusivas. En el estudio de las secuencias de las regiones codificantes y de las regiones intrónicas flanqueantes (±8 pb) del gen RNF213, se detectó la variante c.12185G>A, p.(Arg4062Gln) en heterocigosidad en el gen RNF213. Este resultado indica que la paciente es heterocigota para la variante c.12185G>A, p.(Arg4062Gln) en el gen RNF213. La variante detectada ya ha sido descrita en la bibliografía como una variante fundadora en la población asiática, asociada a síndrome de moyamoya. Esta variante está descrita en ClinVar como una variante de significado clínico desconocido. Además, no está descrita en las bases de datos poblacionales (dbSNP, ESP y gnomAD). Conclusión: Hasta donde sabemos, la variante p.(Arg4062Gln) se ha notificado en tres pacientes japoneses con enfermedad de moyamoya y en uno europeo. Por lo tanto, nuestro paciente fue el segundo europeo con enfermedad de moyamoya con esta variante identificada.(AU)

Introduction: Moyamoya disease is a progressive steno-occlusive disease of the major intracranial arteries. Affected individuals are at risk for intracranial hemorrhagic or ischemic stroke, cognitive impairment, and developmental delays. Several susceptibility genes have been identified. The p.R4810K variant in the RNF213 gene has been identified in 95% of patients with familial moyamoya disease. Case report: We present the case of a 15-year-old adolescent girl who presented with chief complaints of dysgraphia, lack of coordination in the right hand, with two months of evolution. Cerebral magnetic resonance imaging revealed several ischemic lesions with different rates of evolution and magnetic resonance angiography showed multiple subocclusive stenoses. In the study of the sequences of the coding regions and intronic flanking regions (±8 bp) of the RNF213 gene, the variant c.12185G>A, p.(Arg4062Gln) was detected in heterozygosity in the RNF213 gene. This result indicates that the patient is heterozygous for the c.12185G>A, p.(Arg4062Gln) variant in the RNF213 gene. The detected variant has already been reported in the literature as a founder variant in the Asian population, associated with moyamoya syndrome. This variant is described in ClinVar as a variant of unknown clinical significance? Furthermore, it is not described in population databases (dbSNP, ESP, gnomAD). Conclusion: To our knowledge, the p.(Arg406262Gln) variant has been reported in three Japanese moyamoya disease patients and one European. Therefore, our patient was the second European moyamoya disease patient with this variant identified.(AU)

Cuidados paliativos para personas con enfermedad de Parkinson avanzada. Revisión sistemática / Palliative care for people with advanced Parkinson's disease. A systematic review

Introducción: Los cuidados paliativos hacen referencia al tratamiento dirigido a la identificación precoz e impecable del dolor y otros problemas físicos, psicosociales y espirituales que limitan la calidad de vida de la persona, la familia y sus cuidadores. El objetivo de este artículo es identificar las intervenciones paliativas utilizadas para la valoración y el control de síntomas en personas con enfermedad de Parkinson (EP) avanzada. Materiales y métodos: Se desarrolló una revisión sistémica de la bibliografía aplicando los pasos propuestos por Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). La búsqueda se orientó a partir de una pregunta de revisión estructurada y se incluyeron estudios originales de pacientes con EP avanzada publicados en bases de datos como Medline y Google Scholar entre 2010 a 2021. Resultados: Se revisaron 31 estudios en texto completo y se excluyeron 12 estudios por no alcanzar los criterios de calidad. En total, se incluyeron 19 trabajos en esta revisión sistemática, identificando 10 herramientas clínicas para valorar las necesidades paliativas en EP avanzada, cuatro intervenciones farmacológicas, y tres intervenciones no farmacológicas centradas en disminuir síntomas motores, mejorar la calidad de vida y evitar el estado on/off. Un estudio notificó la derivación del paciente a terapias complementarias y asistidas por dispositivos. Conclusión: Como parte del abordaje paliativo se han descrito un conjunto de herramientas para identificar síntomas y valorar necesidades de atención paliativa. Las intervenciones en la EP avanzada se enfocan en el control de los síntomas motores y no motores para disminuir el impacto de la enfermedad sobre la calidad de vida.(AU)

Introduction: Palliative care refers to treatment aimed at the early and comprehensive identification of pain and other physical, psychosocial and spiritual problems that limit the quality of life of the person, their family and their caregivers. The aim of this article is to identify palliative interventions used for the assessment and control of symptoms in people with advanced Parkinson's disease (PD). Materials and methods: A systematic review of the literature was conducted following the steps proposed by Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA). The search was guided by a structured review question and included original studies of patients with advanced PD published in databases such as Medline and Google Scholar between 2010 and 2021. Results: Thirty-one full-text studies were reviewed and 12 were excluded due to not meeting quality criteria. A total of 19 papers were included in this systematic review, which identified 10 clinical tools to assess palliative needs in advanced PD, four pharmacological interventions, and three non-pharmacological interventions focused on reducing motor symptoms, improving quality of life and avoiding the on/off state. One study reported the referral of patients to complementary and device-assisted therapies. Conclusion: As part of the palliative approach, a set of tools for identifying symptoms and assessing palliative care needs have been described. Interventions in advanced PD focus on the control of motor and non-motor symptoms so as to reduce the impact of the disease on quality of life.(AU)

Comorbilidades neurocognitivas y psicológicas en pacientes con epilepsia de la infancia con puntas centrotemporales. Un estudio de casos y controles / Neurocognitive and psychological comorbidities in patients with self-limited centrotemporal spike epilepsy. A case-control study

Introducción: La epilepsia de la infancia con puntas centrotemporales (EIPCT) es la epilepsia focal autolimitada más frecuente. Este estudio tenía como objetivo evaluar los aspectos cognitivos, conductuales y otros aspectos neuropsicológicos de niños con EIPCT, y compararlos con un grupo de control. Sujetos y métodos: Se realizó un estudio de casos y controles entre enero y mayo de 2022. Se seleccionó para su inclusión en el estudio a pacientes con EIPCT, con edades comprendidas entre 6 y 18 años, en seguimiento en nuestro hospital. Por cada caso, se reclutó, de forma aleatoria, a dos controles de la misma edad. Todos los participantes realizaron la prueba EpiTrack Junior®, y sus padres rellenaron la lista de verificación del comportamiento infantil (CBCL). Resultados: Se incluyó a 18 pacientes (edad media: 8,7 ± 1,7 años). En el momento del diagnóstico de EIPCT, el 83% de los casos presentaba un desarrollo psicomotor adecuado, y el 17%, un trastorno del neurodesarrollo. Las puntuaciones del EpiTrack-Junior® y de la escala total de problemas de la CBCL no se vieron influidas por la lateralidad del foco epiléptico ni por el número de crisis. El 61% de los casos mostró un trastorno leve o significativo en la prueba EpiTrack-Junior® frente al 44% de los controles (p = 0,712), y el 39% de los casos frente al 14% de los controles tuvieron puntuaciones ‘clínicamente significativas’ en la escala total de problemas de la CBCL. Conclusiones: Aunque este estudio no encontró diferencias estadísticamente significativas entre casos y controles, cabe señalar que la mayor parte de los pacientes con EIPCT presentaba un deterioro leve o significativo en las funciones ejecutivas. Un porcentaje considerable de casos se encontraba en el rango patológico en cuanto a problemas emocionales/conductuales. Este estudio destaca la importancia de examinar los problemas cognitivos, conductuales y emocionales de los pacientes con EIPCT.(AU)

Introduction: Self-limited epilepsy with centrotemporal spikes (SeLECTS) is the most frequent self-limited focal epilepsy. This study aimed to assess the cognitive, behavioral, and other neuropsychological aspects of children with SeLECTS, and compare them with a control group. Subjects and methods: A case-control study was carried out between January and May 2022. Patients with SeLECTS, aged between 6 and 18 years, and followed-up at our hospital were selected for inclusion in the study. For each case, two age-matched controls were opportunistically recruited. All the participants performed the EpiTrack Junior® test, and their parents filled out the Child Behavior Checklist (CBCL). Results: Eighteen patients were included (mean age: 8.7 ± 1.7 years). At SeLECTS’ diagnosis, 83% of cases had adequate psychomotor development, and 17% had a neurodevelopmental disorder. The EpiTrack-Junior® and the Total Problems CBCL scores were not influenced by the laterality of the epileptic focus nor by the number of seizures. 61% of cases showed mild or significant impairment in the EpiTrack-Junior® test versus 44% of controls (p = 0.712), and 39% of cases vs. 14% of controls had ‘clinically significant’ scores on the Total Problems CBCL scale (p = 0.087). Conclusions: Although this study did not find statistically significant differences between cases and controls, it should be noted that most patients with SeLECTS had a mild or significant disability in executive functions. A considerable percentage of cases were in the pathological range regarding emotional/behavioral problems. This study highlights the importance of screening the cognitive, behavioral, and emotional problems in all patients with SeLECTS.(AU)

Estudio descriptivo de las alteraciones en pruebas de neuroimagen en pacientes con cefalea en un ámbito ambulatorio / Descriptive study of the alterations in neuroimaging tests in patients with headache in an outpatient setting

Introducción: Los estudios disponibles ofrecen una guía limitada acerca del uso de la neuroimagen en pacientes con cefalea en el ámbito ambulatorio. El objetivo de este estudio fue describir y analizar la frecuencia y el porcentaje de alteraciones en neuroimagen en pacientes valorados por cefalea en una consulta de neurología, así como determinar qué datos de alarma clínicos (red flags) se asocian con mayor frecuencia a estas alteraciones. Pacientes y métodos: Realizamos un estudio observacional, descriptivo y transversal de una serie de pacientes con cefalea de la consulta de neurología del Hospital Universitario de Móstoles a los que se les realizó una prueba de neuroimagen. ResultadosSe incluyó a 279 pacientes en el estudio (190 mujeres y 89 hombres). Ningún paciente sin datos de alarma clínicos presentó alteraciones graves en la neuroimagen y, de los que presentaban datos de alarma clínicos (219 pacientes), sólo el 2,3% (cinco pacientes) presentó alteraciones graves. Los dos datos de alarma clínicos que se asociaron de forma significativa a una mayor probabilidad de alteraciones graves en la neuroimagen fueron la cefalea que se desencadena o empeora significativamente con cambios posturales o maniobras de Valsalva y la cefalea crónica diaria desde su inicio. Conclusiones: La prevalencia de alteraciones significativas en neuroimagen en pacientes con cefalea en un ámbito ambulatorio fue baja, incluso en presencia de datos de alarma clínicos, por lo que la utilidad de estos datos de alarma parece ser menor en un contexto ambulatorio. Podría ser razonable intentar reducir el uso de la neuroimagen en pacientes con cefalea en el ámbito ambulatorio.(AU)

Introduction: Available studies provide limited guidance on the use of neuroimaging in patients with headache in the outpatient setting. The aim of this study was to describe and analyse the frequency and the percentage of neuroimaging abnormalities in patients evaluated for headache in a neurology clinic, as well as to determine which red flags are most commonly associated with these abnormalities. Patients and methods: We conducted an observational, descriptive and cross-sectional study of a series of patients with headache from the neurology department of the Hospital Universitario de Móstoles who underwent a neuroimaging test. Results: A total of 279 patients (190 women and 89 men) were included in the study. No patient without any red flags had severe abnormalities in the neuroimaging study and, of those with red flags (219 patients), only 2.3% (five patients) displayed severe abnormalities. The two red flags that were significantly associated with an increased likelihood of severe neuroimaging abnormalities were headache that is triggered or significantly worsened by postural changes or Valsalva manoeuvres and chronic daily headache since onset. Conclusions: The prevalence of significant neuroimaging abnormalities in headache patients in an outpatient setting was low, even in the presence of red flags, and so their utility appears to be lower in an outpatient setting. It may be reasonable to try to reduce the use of neuroimaging with headache patients in the outpatient setting.(AU)

Teleneurology via tablet technology: an effective low-cost method to increase healthcare accessibility.

INTRODUCTION: Telemedicine is a valuable tool to increase access to health care, especially for patients in rural areas who need to visit a specialist. In place of telemedicine robots, which are costly and complicated, hospitals have implemented successful telemedicine programs using lower-cost tablet technology; opting for tablet technology increases the organizational feasibility of a large-scale telemedicine program. METHODS: Vanderbilt University Medical Center (VUMC), in Nashville, Tennessee, USA, launched its teleneurology network program in 2014 to serve patients in surrounding community hospitals who needed a neurology consult. Consults are conducted using an iPad, including examinations and the secure sharing of images and patient information. This article reports on teleneurology consult data and the results of patient and physician satisfaction surveys. RESULTS: Between February 2014 and November 2021, the VUMC teleneurology network program provided consultations for 14 241 patients with a wide variety of neurological diagnoses presenting to 12 community-based hospitals. Patient and community physician satisfaction surveys showed that 96% of physicians were satisfied with the overall care provided, and 89% of patients reported that the telehealth visits met their medical needs. CONCLUSION: One of the goals of telemedicine programs is to increase access to care. Therefore, it is important that the technology used to implement the program also be accessible in terms of cost and complexity. Tablets are low-cost technology, and their use in telemedicine has been shown to satisfy both physicians and patients with a wide variety of diagnoses.

There is no shortage, but inequality: demographic evolution of neurologists in Brazil (2010-2020).

BACKGROUND: Neurology is a medical specialty that deals with prevalent diseases such as stroke, headache, epilepsy, and neurodegenerative diseases. Many countries, such as Brazil, struggle to provide neurological care for their populations, but the inadequacy and unequal distribution of the neurologist workforce are real challenges. OBJECTIVE: To analyze the demographic evolution of neurologists and the first-year Neurology residency positions in Brazil during the last decade (2010-2020) and the distribution imbalance between regions. METHODS: The demographic and geographic distribution of neurologists was calculated based on data extracted from the Brazilian Federal Medical Council reports, and the number of Neurology residency positions was based on the Brazilian National Commission of Medical Residency reports. Indicators of wealth were associated with demographic data. RESULTS: The number of neurologists per 100,000 population has increased since 2011, with a similar increase in the geographic distribution of neurologists. However, there was a marked inequality of distribution of neurologists through regions, with a gap between the Northern (lowest) and Southeastern (highest) regions. Furthermore, the imbalance of distribution of neurologists strongly correlated with social inequality. The number of Neurology residency positions increased, but with an imbalance between North and Southeast regions. CONCLUSIONS: Brazil has advanced in providing neurologists. However, instead of a shortage, inequality between regions is the greatest challenge regarding the neurological workforce. The training of new neurologists is unequal between regions and occurs at a slower rate than needed. Neurologists, public health authorities, and patients should discuss solutions for these issues.

ANTECEDENTES: A Neurologia é uma especialidade médica que lida com doenças prevalentes, como acidente vascular cerebral, cefaleia, epilepsia e doenças neurodegenerativas. Muitos países, como o Brasil, se esforçam para oferecer assistência neurológica à população, mas a distribuição insuficiente e desigual da força de trabalho de neurologistas são desafios. OBJETIVO: Analisar a evolução demográfica dos médicos neurologistas e das vagas de Programas de Residência Médica em Neurologia no Brasil durante a última década (2010-2020) e o desequilíbrio de distribuição entre as regiões. MéTODOS: A distribuição demográfica e geográfica de neurologistas foi calculada com base nos dados extraídos de relatórios do Conselho Federal do Medicina do Brasil, e o número de vagas em Programas de Residência Médica em Neurologia foi extraído de dados da Comissão Nacional de Residência Médica. Os indicadores de riqueza foram associados aos dados demográficos. RESULTADOS: O número de neurologistas por 100.000 habitantes aumentou desde 2011, com um aumento similar na distribuição geográfica de neurologistas. Entretanto, houve uma nítida desigualdade na distribuição de neurologistas entre as regiões, com um hiato entre as regiões Norte e a Sudeste. Além disso, a desigualdade da distribuição de neurologistas se correlacionou fortemente com a desigualdade social. O número de vagas em Programas de Residência Médica aumentou, porém com desigualdade entre as regiões Norte e Sudeste. CONCLUSõES: O Brasil tem avançado na geração de neurologistas. Porém, ao invés de uma escassez, a desigualdade entre regiões é o maior desafio em relação à força de trabalho neurológica. O treino de novos neurologistas é desigual entre regiões e ocorre em um ritmo mais lento do que o necessário. Neurologistas, autoridades em saúde pública e pacientes devem discutir soluções para estes problemas.

Dosis ultraaltas de inmunoglobulina endovenosa mejoran la fuerza y la funcionalidad motora en una paciente con neuropatía motora multifocal refractaria / Ultra-high dose of intravenous immunoglobulin restores strength and motor function in a patient with refractory multifocal motor neuropathy

Introducción: La neuropatía motora multifocal (NMM) es una enfermedad crónica, progresiva e inmunomediada que afecta predominantemente a los miembros superiores de forma asimétrica. En los estudios electrofisiológicos se evidencian bloqueos en la conducción motora, y el tratamiento de elección es la inmunoglobulina humana (Ig); sin embargo, algunos pacientes pueden desarrollar refractariedad a este tratamiento. Describimos el caso de una paciente con diagnóstico de NMM que desarrolló refractariedad a la gammaglobulina y que mejoró marcadamente con dosis ultraaltas de esta misma medicación. Caso clínico: Mujer de 36 años, con diagnóstico de NMM, que, después de cinco años de estabilidad clínica bajo tratamiento con Ig subcutánea en dosis de 2 g/kg/mes, evolucionó con grave debilidad en ambas manos, por lo que se decidió cambiar el tratamiento a Ig endovenosa. No obstante, progresó hasta quedar incapacitada para realizar actividades básicas de la vida diaria. Iniciamos tratamiento con Ig endovenosa en dosis ultraaltas (5 g/kg/mes) con buena respuesta, logrando independencia funcional en las actividades de la vida diaria y que regresara al trabajo. El único evento adverso relacionado con la Ig endovenosa en dosis ultraaltas fue la presencia de cefalea durante la infusión. Conclusión: La Ig endovenosa en dosis ultraaltas parece ser un tratamiento efectivo para pacientes con NMM y grave discapacidad no respondedores a dosis convencionales. Un bajo índice de riesgo cardiovascular (QRISK2 menor que 10%) y una dosis diaria de Ig endovenosa menor de 35 g reducen el riesgo de complicaciones graves relacionadas con el uso de esta medicación.(AU)

Introduction: Multifocal motor neuropathy (MMN) is a chronic progressive immune-mediated neuropathy, predominantly involving upper limbs asymmetrically with electrophysiologic evidence of motor conduction block. The treatment of choice is immunoglobulin (Ig). Nevertheless, some patients may become resistant to treatment. We describe a patient with history of MMN who became resistant to gammaglobulin treatment but markedly improved using ultra-high doses of intravenous immunoglobulin. Case report: A 36-year-old woman with diagnosis of MMN. After 5 years of clinical stability under subcutaneous Ig (2g/kg/month) the patient developed bilateral weakness involving both hands. Treatment was switched to intravenous Ig 2g/kg/month, nevertheless, she progressed and became totally dependent for activities of daily living. We started ultra-high dose intravenous immunoglobulin 5 g/kg/month, with good response. She became independent for activities of daily living and returned to work. The only treatment related adverse event was headache during infusion. Conclusion: Ultra-high dose intravenous Ig seems to be a useful therapy in aggressive MMN with severe disability despite conventional treatment. A low cardiovascular risk score (QRISK2 less than 10%) and a daily intravenous Ig lower than 35 g reduce the risk of severe complications related to intravenous Ig.(AU)

Herramientas disponibles en español para la valoración clínica del delirium. Revisión sistemática / Tools available in Spanish for clinical assessment of delirium. Systematic review

Introducción: La evaluación del delirium es difícil, porque implica la alteración de diferentes funciones mentales en pacientes de cualquier edad con patologías medicoquirúrgicas graves o demencia. Por tanto, se requieren herramientas de evaluación concretas para necesidades específicas. Esta revisión sistemática sintetiza la información sobre las herramientas disponibles en español para la evaluación clínica del delirium. Materiales y métodos: Ésta es una revisión sistemática que sigue la guía PRISMA-Diagnostic Test Accuracy. Usando un algoritmo de búsqueda, dos investigadores evaluaron cinco sistemas de indexación científica y buscaron artículos adicionales y bibliografía gris. Otros dos investigadores registraron de forma estandarizada los datos de los trabajos incluidos. Se evaluó la calidad de las traducciones, adaptaciones culturales y validaciones. Resultados: Identificamos 21 trabajos sobre traducción, adaptación cultural o validación de 14 herramientas para: a) evaluación detallada, diagnóstico y cuantificación de la gravedad por parte de expertos; b) evaluación de aspectos concretos del delirium; c) cribado en adultos; d) cribado pediátrico; y e) diagnóstico y ponderación de gravedad por técnicos tratantes. El 78,6% de las traducciones y todas las adaptaciones son de buena calidad. El 52,9% de las validaciones tiene riesgo bajo de sesgo; el 41,2%, riesgo moderado; y sólo el 5,9%, riesgo alto. Conclusiones: Dado que el primer paso para tratar correctamente a los pacientes es un diagnóstico preciso y que la selección de los instrumentos adecuados es fundamental para la validez de cualquier estudio, las herramientas disponibles deben elegirse de acuerdo con las necesidades clínicas y de investigación específicas.(AU)

Introduction: Delirium assessment is difficult because it involves the alteration of different mental functions in patients of any age who have serious medical-surgical conditions or dementia. Therefore, concrete assessment tools for specific needs are required. This systematic review synthetizes the information about the tools available in Spanish for delirium clinical assessment. Materials and methods: This is a systematic review that follows the PRISMA Diagnostic Test Accuracy guideline. Using a search algorithm, two researchers assessed five scientific indexing systems and searched for additional papers and grey literature. Other two researchers recorded in a standardized way the data of the papers included. The quality of translations, cultural adaptations, and validations were assessed. Results: We identified 21 reports on translation, cultural adaptation, or validation of 14 tools for 1) detailed evaluation, diagnosis, and quantification of severity by experts, 2) assessment of concrete aspects of delirium, 3) screening in adults, 4) pediatric screening, and 5) diagnosis and weighting of severity by treating staff. 78.6% of translations and all adaptations are of good quality. 52.9% of validations have a low risk of bias, 41.2% have a moderate risk, and only 5.9% have a high risk. Conclusions: As the first step to correctly treat patients is an accurate diagnosis, and the selection of the appropriate instruments is essential for the validity of any study, the available.(AU)

Mitochondrial disease in neurology-Past, present, and future.

This chapter provides a overview of this volume of the Handbook of Clinical Neurology, placing recent advances in our understanding of mitochondrial disorders in a historical context, and speculates about the future.

Diagnostic Ultrasonography in Neurology.

OBJECTIVE: Ultrasonography allows neurologists to complement clinical information with additional useful, easily acquired, real-time data. This article highlights its clinical applications in neurology. LATEST DEVELOPMENTS: Diagnostic ultrasonography is expanding its applications with smaller and better devices. Most indications in neurology relate to cerebrovascular evaluations. Ultrasonography contributes to the etiologic evaluation and is helpful for hemodynamic diagnosis of brain or eye ischemia. It can accurately characterize cervical vascular atherosclerosis, dissection, vasculitis, or other rarer disorders. Ultrasonography can aid in the diagnosis of intracranial large vessel stenosis or occlusion and evaluation of collateral pathways and indirect hemodynamic signs of more proximal and distal pathology. Transcranial Doppler (TCD) is the most sensitive method for detecting paradoxical emboli from a systemic right-left shunt such as a patent foramen ovale. TCD is mandatory for sickle cell disease surveillance, guiding the timing for preventive transfusion. In subarachnoid hemorrhage, TCD is useful in monitoring vasospasm and adapting treatment. Some arteriovenous shunts can be detected by ultrasonography. Cerebral vasoregulation studies are another developing field of interest. TCD enables monitoring of hemodynamic changes related to intracranial hypertension and can diagnose cerebral circulatory arrest. Optic nerve sheath measurement and brain midline deviation are ultrasonography-detectable signs of intracranial hypertension. Most importantly, ultrasonography allows for easily repeated monitoring of evolving clinical conditions or during and after interventions. ESSENTIAL POINTS: Diagnostic ultrasonography is an invaluable tool in neurology, used as an extension of the clinical examination. It helps diagnose and monitor many conditions, allowing for more data-driven and rapid treatment interventions.

Translational veterinary epilepsy: A win-win situation for human and veterinary neurology.

Epilepsy is a challenging multifactorial disorder with a complex genetic background. Our current understanding of the pathophysiology and treatment of epilepsy has substantially increased due to animal model studies, including canine studies, but additional basic and clinical research is required. Drug-resistant epilepsy is an important problem in both dogs and humans, since seizure freedom is not achieved with the available antiseizure medications. The evaluation and exploration of pharmacological and particularly non-pharmacological therapeutic options need to remain a priority in epilepsy research. Combined efforts and sharing knowledge and expertise between human medical and veterinary neurologists are important for improving the treatment outcomes or even curing epilepsy in dogs. Such interactions could offer an exciting approach to translate the knowledge gained from people and rodents to dogs and vice versa. In this article, a panel of experts discusses the similarities and knowledge gaps in human and animal epileptology, with the aim of establishing a common framework and the basis for future translational epilepsy research.

Imaging of Central Nervous System Autoimmune, Paraneoplastic, and Neuro-rheumatologic Disorders.

OBJECTIVE: This article provides an overview of the imaging modalities used in the evaluation of central nervous system (CNS) autoimmune, paraneoplastic, and neuro-rheumatologic disorders. An approach is outlined for interpreting imaging findings in this context, synthesizing a differential diagnosis based on certain imaging patterns, and choosing further imaging for specific diseases. LATEST DEVELOPMENTS: The rapid discovery of new neuronal and glial autoantibodies has revolutionized the autoimmune neurology field and has elucidated imaging patterns characteristic of certain antibody-associated diseases. Many CNS inflammatory diseases, however, lack a definitive biomarker. Clinicians should recognize neuroimaging patterns suggestive of inflammatory disorders, as well as the limitations of imaging. CT, MRI, and positron emission tomography (PET) modalities all play a role in diagnosing autoimmune, paraneoplastic, and neuro-rheumatologic disorders. Additional imaging modalities such as conventional angiography and ultrasonography can be helpful for further evaluation in select situations. ESSENTIAL POINTS: Knowledge of imaging modalities, both structural and functional, is critical in identifying CNS inflammatory diseases quickly and can help avoid invasive testing such as brain biopsy in certain clinical scenarios. Recognizing imaging patterns suggestive of CNS inflammatory diseases can also facilitate the early initiation of appropriate treatments to diminish morbidity and future disability.