The Role of Neurophysiology in Managing Patients with Chiari Malformations

Chiari type 1 malformation; Electromyography; SyringomyeliaMalformación de Chiari tipo 1; Electromiografía; SiringomieliaMalformació de Chiari tipus 1; Electromiografia; SiringomieliaChiari malformation type 1 (CM1) includes various congenital anomalies that share ectopia of the cerebellar tonsils lower than the foramen magnum, in some cases associated with syringomyelia or hydrocephalus. CM1 can cause dysfunction of the brainstem, spinal cord, and cranial nerves. This functional alteration of the nervous system can be detected by various modalities of neurophysiological tests, such as brainstem auditory evoked potentials, somatosensory evoked potentials, motor evoked potentials, electromyography and nerve conduction studies of the cranial nerves and spinal roots, as well as brainstem reflexes. The main goal of this study is to review the findings of multimodal neurophysiological examinations in published studies of patients with CM1 and their indication in the diagnosis, treatment, and follow-up of these patients, as well as their utility in intraoperative monitoring.This study was partially supported by grants FIS PI22/01082, which was co-financed by the European Regional Development Fund (ERDF), awarded to M.A. Poca and Agència de Gestió d’Ajuts Universitaris i de Recerca (AGAUR), Spain, grant 2021SGR/00810. The following nongovernmental associations have generously donated funding to support this research: (1) Asociación Nacional de Amigos de Arnold-Chiari (ANAC, http://www.arnoldchiari.es (accessed on 5 October 2023)), (2) Asociación Chiari y Siringomielia del Principado de Asturias (Ch.y.S.P.A., https://chyspa.org (accessed on 5 October 2023)), (3) Federación Española de Malformación de Chiari y Patologías Asociadas (FEMACPA), and (4) Mariana Dañobeitia https://vhir.vallhebron.com/es/sociedad/noticias/la-hija-de-una-paciente-con-malformacion-de-chiari-dona-21840-euros-para-impulsar-la-investigacion (accessed on 5 October 2023))

A Critical Update of the Classification of Chiari and Chiari-like Malformations

Malformació d'Arnold-Chiari; Classificació; Malalties raresMalformación de Arnold-Chiari; Clasificación; Enfermedades rarasArnold-Chiari malformation; Classification; Rare diseasesChiari malformations are a group of craniovertebral junction anomalies characterized by the herniation of cerebellar tonsils below the foramen magnum, often accompanied by brainstem descent. The existing classification systems for Chiari malformations have expanded from the original four categories to nine, leading to debates about the need for a more descriptive and etiopathogenic terminology. This review aims to examine the various classification approaches employed and proposes a simplified scheme to differentiate between different types of tonsillar herniations. Furthermore, it explores the most appropriate terminology for acquired herniation of cerebellar tonsils and other secondary Chiari-like malformations. Recent advances in magnetic resonance imaging (MRI) have revealed a higher prevalence and incidence of Chiari malformation Type 1 (CM1) and identified similar cerebellar herniations in individuals unrelated to the classic phenotypes described by Chiari. As we reassess the existing classifications, it becomes crucial to establish a terminology that accurately reflects the diverse presentations and underlying causes of these conditions. This paper contributes to the ongoing discussion by offering insights into the evolving understanding of Chiari malformations and proposing a simplified classification and terminology system to enhance diagnosis and management.This research was partially supported by grant FIS PI22/01082, which was co-financed by the European Regional Development Fund (ERDF), awarded to M.A. Poca and by grant 2021SGR/00810 from the Agència de Gestió d’Ajuts Universitaris i de Recerca (AGAUR), Departament de Recerca i Universitats de la Generalitat de Catalunya, Spain. ASM is the recipient of a predoctoral fellowship from grant 2021SGR/00810 from the Agència de Gestió d’Ajuts Universitaris i de Recerca (AGAUR). The following nongovernmental associations have generously donated funding to support this research: 1. Asociación Nacional de Amigos de Arnold-Chiari (ANAC, http://www.arnoldchiari.es (accessed on 7 June 2023)); 2. Asociación Chiari y Siringomielia del Principado de Asturias (CHySPA, https://chyspa.org (accessed on 7 June 2023)); 3. Federación Española de Malformación de Chiari y Patologías Asociadas (FEMACPA); and 4. Mariana Dañobeitia (https://references.neurotrauma.com/chiari (accessed on 7 June 2023))

Intraoperative Neurophysiological Monitoring in Syringomyelia Surgery: A Multimodal Approach

Intraoperative neurophysiological monitoring; Spinal cord; SyringomyeliaMonitorización neurofisiológica intraoperatoria; Médula espinal; SiringomieliaMonitorització neurofisiològica intraoperatoria; Medul·la espinal; SiringomieliaSyringomyelia can be associated with multiple etiologies. The treatment of the underlying causes is first-line therapy; however, a direct approach to the syrinx is accepted as rescue treatment. Any direct intervention on the syrinx requires a myelotomy, posing a significant risk of iatrogenic spinal cord (SC) injury. Intraoperative neurophysiological monitoring (IONM) is crucial to detect and prevent surgically induced damage in neural SC pathways. We retrospectively reviewed the perioperative and intraoperative neurophysiological data and perioperative neurological examinations in ten cases of syringomyelia surgery. All the monitored modalities remained stable throughout the surgery in six cases, correlating with no new postoperative neurological deficits. In two patients, significant transitory attenuation, or loss of motor evoked potentials (MEPs), were observed and recovered after a corrective surgical maneuver, with no new postoperative deficits. In two cases, a significant MEP decrement was noted, which lasted until the end of the surgery and was associated with postoperative weakness. A transitory train of neurotonic electromyography (EMG) discharges was reported in one case. The surgical plan was adjusted, and the patient showed no postoperative deficits. The dorsal nerve roots were stimulated and identified in the seven cases where the myelotomy was performed via the dorsal root entry zone. Dorsal column mapping guided the myelotomy entry zone in four of the cases. In conclusion, multimodal IONM is feasible and reliable and may help prevent iatrogenic SC injury during syringomyelia surgery.This research was partially supported by grant FIS PI22/01082, which was co-financed by the European Regional Development Fund (ERDF), awarded to M.A. Poca and by grant 2021SGR/00810 from the Agència de Gestió d’Ajuts Universitaris i de Recerca (AGAUR), Departament de Recerca i Universitats de la Generalitat de Catalunya, Spain. ASM is the recipient of a predoctoral fellowship from grant 2021SGR/00810 from the Agència de Gestió d’Ajuts Universitaris i de Recerca (AGAUR). The following nongovernmental associations have generously donated funding to support this research: 1. Asociación Nacional de Amigos de Arnold-Chiari (ANAC, http://www.arnoldchiari.es (accessed on 7 June 2023)); 2. Asociación Chiari y Siringomielia del Principado de Asturias (CHySPA, https://chyspa.org (accessed on 7 June 2023)); 3. Federación Española de Malformación de Chiari y Patologías Asociadas (FEMACPA); and 4. Mariana Dañobeitia (https://references. neurotrauma.com/chiari (accessed on 7 June 2023))

Reduced hippocampal subfield volumes and memory performance in preterm children with and without germinal matrix intraventricular hemorrhage.

Preterm newborns with germinal matrix-intraventricular hemorrhage (GM-IVH) are at a higher risk of evidencing neurodevelopmental alterations. Present study aimed to explore the long-term efects that GM-IVH have on hippocampal subfelds, and their correlates with memory. The sample consisted of 58 participants, including 36 preterm-born (16 with GM-IVH and 20 without neonatal brain injury), and 22 full-term children aged between 6 and 15 years old. All participants underwent a cognitive assessment and magnetic resonance imaging study. GM-IVH children evidenced lower scores in Full Intelligence Quotient and memory measures compared to their low-risk preterm and full-term peers. High-risk preterm children with GM-IVH evidenced signifcantly lower total hippocampal volumes bilaterally and hippocampal subfeld volumes compared to both low-risk preterm and full-term groups. Finally, signifcant positive correlations between memory and hippocampal subfeld volumes were only found in preterm participants together; memory and the right CA-feld correlation remained signifcant after Bonferroni correction was applied (p= .002). In conclusion, memory alterations and both global and regional volumetric reductions in the hippocampus were found to be specifcally related to a preterm sample with GM-IVH. Nevertheless, results also suggest that prematurity per se has a long-lasting impact on the association between the right CA-feld volume and memory during childhood

Consensus statement from the 2014 International Microdialysis Forum.

Microdialysis enables the chemistry of the extracellular interstitial space to be monitored. Use of this technique in patients with acute brain injury has increased our understanding of the pathophysiology of several acute neurological disorders. In 2004, a consensus document on the clinical application of cerebral microdialysis was published. Since then, there have been significant advances in the clinical use of microdialysis in neurocritical care. The objective of this review is to report on the International Microdialysis Forum held in Cambridge, UK, in April 2014 and to produce a revised and updated consensus statement about its clinical use including technique, data interpretation, relationship with outcome, role in guiding therapy in neurocritical care and research applications.We gratefully acknowledge financial support for participants as follows: P.J.H. - National Institute for Health Research (NIHR) Professorship and the NIHR Biomedical Research Centre, Cambridge; I.J. – Medical Research Council (G1002277 ID 98489); A. H. - Medical Research Council, Royal College of Surgeons of England; K.L.H.C. - NIHR Biomedical Research Centre, Cambridge (Neuroscience Theme; Brain Injury and Repair Theme); M.G.B. - Wellcome Trust Dept Health Healthcare Innovation Challenge Fund (HICF-0510-080); L. H. - The Swedish Research Council, VINNOVA and Uppsala Berzelii Technology Centre for Neurodiagnostics; S. M. - Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico; D.K.M. - NIHR Senior Investigator Award to D.K.M., NIHR Cambridge Biomedical Research Centre (Neuroscience Theme), FP7 Program of the European Union; M. O. - Swiss National Science Foundation and the Novartis Foundation for Biomedical Research; J.S. - Fondo de Investigación Sanitaria (Instituto de Salud Carlos III) (PI11/00700) co-financed by the European Regional Development; M.S. – NIHR University College London Hospitals Biomedical Research Centre; N. S. - Fondazione IRCCS Cà Granda Ospedale Maggiore Policlinico.This is the final version of the article. It first appeared from Springer via http://dx.doi.org/10.1007/s00134-015-3930-

Consensus statement from the 2014 International Microdialysis Forum

This is the final version of the article. It first appeared from Springer via http://dx.doi.org/10.1007/s00134-015-3930-yMicrodialysis enables the chemistry of the extracellular interstitial space to be measured. Use of this technique in patients with acute brain injury has increased our understanding of the pathophysiology of several acute neurological disorders. In 2004 a consensus document on the clinical application of cerebral microdialysis was published. Since then there have been significant advances in the clinical use of microdialysis in neurocritical care. The objective of this review is to report on the International Microdialysis Forum held in Cambridge, UK, in April 2014 and to produce a revised and updated consensus statement about its clinical use including technique, data interpretation, relationship with outcome, role in guiding therapy in neurocritical care and research applications.We gratefully acknowledge financial support for participants as follows: P.J.H. - National Institute for Health Research (NIHR) Professorship and the NIHR Biomedical Research Centre, Cambridge; I.J. ? Medical Research Council (G1002277 ID 98489); A. H. - Medical Research Council, Royal College of Surgeons of England; K.L.H.C. - NIHR Biomedical Research Centre, Cambridge (Neuroscience Theme; Brain Injury and Repair Theme); M.G.B. - Wellcome Trust Dept Health Healthcare Innovation Challenge Fund (HICF-0510-080); L. H. - The Swedish Research Council, VINNOVA and Uppsala Berzelii Technology Centre for Neurodiagnostics; S. M. - Fondazione IRCCS C? Granda Ospedale Maggiore Policlinico; D.K.M. - NIHR Senior Investigator Award to D.K.M., NIHR Cambridge Biomedical Research Centre (Neuroscience Theme), FP7 Program of the European Union; M. O. - Swiss National Science Foundation and the Novartis Foundation for Biomedical Research; J.S. - Fondo de Investigaci?n Sanitaria (Instituto de Salud Carlos III) (PI11/00700) co-financed by the European Regional Development; M.S. ? NIHR University College London Hospitals Biomedical Research Centre; N. S. - Fondazione IRCCS C? Granda Ospedale Maggiore Policlinico

Variation in neurosurgical management of traumatic brain injury

Background: Neurosurgical management of traumatic brain injury (TBI) is challenging, with only low-quality evidence. We aimed to explore differences in neurosurgical strategies for TBI across Europe. Methods: A survey was sent to 68 centers participating in the Collaborative European Neurotrauma Effectiveness Research in Traumatic Brain Injury (CENTER-TBI) study. The questionnaire contained 21 questions, including the decision when to operate (or not) on traumatic acute subdural hematoma (ASDH) and intracerebral hematoma (ICH), and when to perform a decompressive craniectomy (DC) in raised intracranial pressure (ICP). Results: The survey was completed by 68 centers (100%). On average, 10 neurosurgeons work in each trauma center. In all centers, a neurosurgeon was available within 30 min. Forty percent of responders reported a thickness or volume threshold for evacuation of an ASDH. Most responders (78%) decide on a primary DC in evacuating an ASDH during the operation, when swelling is present. For ICH, 3% would perform an evacuation directly to prevent secondary deterioration and 66% only in case of clinical deterioration. Most respondents (91%) reported to consider a DC for refractory high ICP. The reported cut-off ICP for DC in refractory high ICP, however, differed: 60% uses 25 mmHg, 18% 30 mmHg, and 17% 20 mmHg. Treatment strategies varied substantially between regions, specifically for the threshold for ASDH surgery and DC for refractory raised ICP. Also within center variation was present: 31% reported variation within the hospital for inserting an ICP monitor and 43% for evacuating mass lesions. Conclusion: Despite a homogeneous organization, considerable practice variation exists of neurosurgical strategies for TBI in Europe. These results provide an incentive for comparative effectiveness research to determine elements of effective neurosurgical care

Métodos globales de monitorización de la hemodinámica cerebral en el paciente neurocrítico: fundamentos, controversias y actualizaciones en las técnicas de oximetría yugular

El papel relevante que la hipoxia tisular cerebral juega en la fisiopatología de los pacientes con un traumatismo craneoencefálico (TCE) justifica la necesidad de complementar la monitorización de estos pacientes con sistemas que aporten información sobre el flujo sanguíneo y el metabolismo cerebral. En la búsqueda de sistemas útiles en la cabecera del paciente, se han utilizado extrapolaciones del principio de Fick al encéfalo, utilizando métodos que estiman el flujo sanguíneo cerebral (FSC) a partir de la obtención de muestras de sangre del bulbo de la yugular. En los últimos años, las técnicas de oximetría yugular se han convertido en elementos de uso frecuente en las unidades que tratan pacientes con un TCE u otros pacientes neurocríticos, como los pacientes con una hemorragia subaracnoidea o con infartos masivos de la arteria cerebral media. El uso frecuente de estas técnicas en las últimas dos décadas, junto a la incorporación de otros sistemas de neuromonitorización, permiten en la actualidad matizar la información que estos métodos globales proporcionan y definir mejor tanto sus indicaciones como sus limitaciones. El objetivo de esta revisión es presentar los fundamentos y los conceptos básicos relacionados con la utilización clínica de las técnicas de oximetría yugular en el paciente neurocrítico. También presentamos y discutimos la evidencia más reciente que indica que determinadas variables, obtenidas de muestras de sangre del bulbo de la yugular, tales como las diferencias arterio-yugulares de lactatos (AVDL) y el índice lactato-oxígeno (LOI), a pesar de su amplia utilización en la práctica clínica diaria, no ofrecen una información fiable sobre el metabolismo cerebral que permita la toma de decisiones terapéuticas

Actualizaciones en los métodos de monitorización cerebral regional en los pacientes neurocríticos: presión tisular de oxígeno, microdiálisis cerebral y técnicas de espectroscopía por infrarrojos

El resultado final de los pacientes que han presentado un traumatismo craneoencefálico (TCE) depende de las lesiones primarias, pero también, y en gran medida, de las lesiones secundarias. El diagnóstico de un gran número de lesiones secundarias, y en especial de la isquemia cerebral, se centra en la monitorización simultánea de diversas variables encefálicas y sistémicas. En el momento actual, la monitorización continua de la presión intracraneal (PIC) se considera una medida indispensable en el manejo de los pacientes con un TCE grave que presentan cualquier tipo de lesión intracraneal. Sin embargo, la información que ofrece esta variable es insuficiente para diagnosticar los complejos procesos fisiopatológicos que caracterizan a las lesiones neurotraumáticas. Por ello, cada vez es más frecuente complementar la neuromonitorización de los pacientes con un TCE con métodos de estimación del flujo sanguíneo cerebral (FSC) como el Doppler transcraneal o las técnicas de oximetría yugular. Sin embargo, en el momento actual y en la cabecera del paciente, el conocimiento de la repercusión de las lesiones tisulares y de las medidas terapéuticas sobre el metabolismo cerebral requiere un acceso directo al parénquima encefálico. En esta revisión nos centraremos en tres métodos de monitorización cerebral ¿regional¿: la presión tisular de oxígeno, la microdiálisis cerebral y las técnicas transcutáneas de espectroscopía por infrarrojos. En cada caso se expondrán los fundamentos del método en cuestión, los valores de referencia de los parámetros monitorizados y una serie de recomendaciones sobre cómo pueden interpretarse sus resultados a la luz de los conocimientos actuales.El resultado final de los pacientes que han presentado un traumatismo craneoencefálico (TCE) depende de las lesiones primarias, pero también, y en gran medida, de las lesiones secundarias. El diagnóstico de un gran número de lesiones secundarias, y en especial de la isquemia cerebral, se centra en la monitorización simultánea de diversas variables encefálicas y sistémicas. En el momento actual, la monitorización continua de la presión intracraneal (PIC) se considera una medida indispensable en el manejo de los pacientes con un TCE grave que presentan cualquier tipo de lesión intracraneal. Sin embargo, la información que ofrece esta variable es insuficiente para diagnosticar los complejos procesos fisiopatológicos que caracterizan a las lesiones neurotraumáticas. Por ello, cada vez es más frecuente complementar la neuromonitorización de los pacientes con un TCE con métodos de estimación del flujo sanguíneo cerebral (FSC) como el Doppler transcraneal o las técnicas de oximetría yugular. Sin embargo, en el momento actual y en la cabecera del paciente, el conocimiento de la repercusión de las lesiones tisulares y de las medidas terapéuticas sobre el metabolismo cerebral requiere un acceso directo al parénquima encefálico. En esta revisión nos centraremos en tres métodos de monitorización cerebral ¿regional¿: la presión tisular de oxígeno, la microdiálisis cerebral y las técnicas transcutáneas de espectroscopía por infrarrojos. En cada caso se expondrán los fundamentos del método en cuestión, los valores de referencia de los parámetros monitorizados y una serie de recomendaciones sobre cómo pueden interpretarse sus resultados a la luz de los conocimientos actuales