Intraoperative neuromonitoring in Chiari I malformation surgery: a systematic review and meta-analysis.

Chiari Malformation Type I (CMI) is a prevalent neurosurgical condition characterized by the descent of cerebellar tonsils below the foramen magnum. Surgery, aimed at reducing symptomatology and syrinx size, presents risks, making intraoperative neuromonitoring (IONM) a potentially vital tool. Despite its widespread use in cervical spine surgery, the utility of IONM in CMI surgery remains controversial, with concerns over increased operative time, cost, restricted anesthetic techniques and tongue lacerations. This systematic review and meta-analysis followed the Cochrane Group standards and PRISMA framework. It encompassed an extensive search through PubMed, Embase, and Web of Science up to December 2023, focusing on clinical and surgical outcomes of IONM in CMI surgery. Primary outcomes included the use of various IONM techniques, complication rates, clinical improvement, reoperation, and mortality. The review, registered at PROSPERO (CRD42024498996), included both prospective and retrospective studies, with rigorous selection and data extraction processes. Statistical analysis was conducted using R software. The review included 16 studies, comprising 1358 patients. It revealed that IONM techniques predominantly involved somatosensory evoked potentials (SSEPs), followed by motor evoked potentials (MEPs) and Brainstem auditory evoked potentials (BAEPs). The estimated risk of complications with IONM was 6% (95% CI: 2-11%; I2 = 89%), lower than previously reported rates without IONM. Notably, the clinical improvement rate post-surgery was high at 99% (95% CI: 98-100%; I2 = 56%). The analysis also showed lower reoperation rates in surgeries with IONM compared to those without. Interestingly, no mortality was observed in the included studies. This systematic review and meta-analysis indicate that intraoperative neuromonitoring in Chiari I malformation surgery is associated with favorable clinical outcomes, including lower complication and reoperation rates, and high rates of clinical improvement.

Complex neural tube and skeletal malformations, resembling Chiari malformations, in two calves.

Two 1-day-old full-term female calves from different farms located in the Brazilian state of Rio Grande do Sul were unable to stand due to paresis of the pelvic limbs. Both calves had spina bifida on the spinal lumbar segment and were submitted to euthanasia due to poor prognosis. Postmortem examination revealed cerebellar herniation, caudal displacement of the brainstem, rostral deviation of the cranial nerves, caudal extension of occipital lobes, absence of dorsal lamina of lumbar vertebrae with exposed spinal cord, myelodysplasia, kyphosis, segmental spinal agenesis, renal fusion, muscular atrophy, and arthrogryposis. Histology highlighted myelodysplasia (syringomyelia and diplomyelia) and muscular atrophy. The reverse transcription-polymerase chain reactions for ruminant pestivirus were negative. Based on these lesions, the diagnosis of complex neural tube and skeletal malformations was made. A review of previous publications on calves diagnosed with these malformations, originally called Chiari or Arnold-Chiari malformations, revealed a wide range of nervous system and skeletal lesions. These variations amplified the uncertainty regarding whether all cases represent the same disorder and reinforced the importance of reconfiguring the terminology.

Disfagia orofaríngea na malformação de Chiari tipo I: série de casos clínicos / Oropharyngeal dysphagia in type I Chiari malformation: a clinical case series

RESUMO A malformação de Chiari tipo I é uma condição geralmente congênita de herniação das tonsilas cerebelares abaixo do forame magno, que pode levar à compressão das estruturas próximas da fossa craniana posterior ou à obstrução do fluxo do líquido cefalorraquidiano ventril. O tipo I, apesar de raro, é o mais comumente encontrado na população. Este estudo teve como objetivo relatar os achados da videoendoscopia da deglutição, avaliação fonoaudiológica, nível de ingestão oral, risco nutricional e o planejamento terapêutico em três casos de malformação de Chiari tipo I na fase adulta. Foram coletados dados com relação à avaliação fonoaudiológica de mobilidade e força de língua, Tempo Máximo de Fonação, eficiência da tosse e o Índice de Eichner. Foram analisados o nível de ingestão oral e os sinais faríngeos de disfagia em quatro consistências alimentares, de acordo com a classificação da International Dysphagia Diet Standartisation Initiative), por meio da videoendoscopia da deglutição. Para análise e classificação dos resíduos faríngeos, foi utilizado o Yale Pharyngeal Residue Severity Rating Scale, enquanto que, para rastrear o risco nutricional, foi utilizado o Malnutrition Screening Tool. Observou-se redução mobilidade e força de língua e no Tempo Máximo de Fonação, enquanto que os sinais faríngeos variaram entre os casos, com presença de fechamento glótico incompleto, escape oral posterior, deglutições múltiplas, resíduos faríngeos e penetração laríngea.

ABSTRACT Type I Chiari malformation (MCI) is a generally congenital condition of herniation of the cerebellar tonsils below the foramen magnum, which can lead to compression of structures close to the posterior cranial fossa or obstruction of the flow of ventral cerebrospinal fluid. Type I, although rare, is the most commonly found in the population. This study aimed to report the findings of video endoscopy of swallowing, speech assessment, level of oral intake, nutritional risk and therapeutic planning in three cases of Type I Chiari malformation. Data were collected regarding the assessment of mobility and strength of tongue, Maximum Phonation Time (MPT), cough efficiency and the Eichner Index. The level of oral intake and pharyngeal signs of dysphagia were analyzed in four food consistencies, according to the International Dysphagia Diet Standardization Initiative (IDDSI) classification, using swallowing video endoscopy. For analysis and classification of pharyngeal residues, the Yale Pharyngeal Residue Severity Rating Scale (YPRSRS) was used, while the Malnutrition Screening Tool (MST) was used to track nutritional risk. Reduced tongue mobility and strength and reduced MPT were observed, while pharyngeal signs varied between cases, with the presence of incomplete glottic closure, posterior oral escape, multiple swallows, pharyngeal residues and laryngeal penetration.

Malformación de Arnold-Chiari tipo I con siringomielia / Type I Arnold-Chiari malformation with syringomyelia

Introducción: Las malformaciones de Arnold-Chiari, son un grupo heterogéneo de desórdenes neurológicos caracterizados por alteraciones dentro de las regiones del cerebelo, tallo cerebral y unión cráneo-cervical; todas resultan en un desplazamiento inferior del cerebelo hacia el canal espinal por el foramen magno, sea en conjunto con la médula inferior o no. Objetivo: Describir los hallazgos clínicos, estudios complementarios, criterios diagnósticos, conducta terapéutica y evolución, en un caso con malformación de Chiari tipo I con siringomielia. Presentación de caso: Se presenta a una paciente con cefalea de curso insidioso a la que se le diagnostica malformación de Chiari tipo I con siringomielia, que requirió tratamiento neuroquirúrgico. La evolución posoperatoria fue favorable. Conclusiones: La malformación de Arnold-Chiari es una entidad rara; la aparición de la resonancia magnética nuclear ha permitido que su diagnóstico aumente y por tanto, su incidencia. El caso presentado tuvo un curso clínico clásico(AU)

Introduction: Arnold-Chiari malformations are a heterogeneous group of neurological disorders characterized by alterations within the regions of the cerebellum, brain stem and craniocervical junction; all result in inferior displacement of the cerebellum into the spinal canal through the foramen magnum, whether in conjunction with the inferior cord or not. Objective: To describe the clinical findings, complementary studies, diagnostic criteria, therapeutic approach and evolution, in a case with type I Chiari malformation with syringomyelia. Case report: A patient with an insidious course of headache is reported. She was diagnosed with type I Chiari malformation with syringomyelia, which required neurosurgical treatment. The postoperative evolution was favorable. Conclusions: Conclusions: Arnold-Chiari malformation is a rare entity. the appearance of nuclear magnetic resonance has allowed it to be diagnosed and therefore its incidence has increased. The reported case had a classic clinical course(AU)

Experiencia en patología cervical alta en el Hospital Guillermo Almenara Irigoyen 2016 - 2021

Introducción : El complejo C0-C1-C2 es responsable de la transición de la carga axial, con función biomecánica única, siendo afectada por múltiples patologías, que por lo general la literatura no las considera como un solo ítem, sino que lo desarrolla según su etiología, pero en nuestro estudio se ha considerado en 5 grupos: traumática, congénita, inflamatoria reumática, neoplásica y degenerativa. Objetivo : Determinar las características epidemiológicas, clínicas y del tratamiento en la patología cervical alta. Materiales y métodos : Se incluyeron a todos los pacientes con diagnóstico clínico radiológico de alguna patología cervical alta que hayan sido sometidos a tratamiento quirúrgico entre 2016 y 2021 en el Hospital Almenara. Se usó el test "t" de student y de chi cuadrado. Se dividió a los pacientes en alguno de los 5 grupos antes mencionados. Resultados : Se consideraron 31 pacientes, con una edad media de 51.16 años. La patología cervical alta más frecuente fue la traumática con el 35.48%. El déficit motor se presentó en el 51.61% y el déficit sensitivo se presentó en el 54.84%. La cirugía más frecuente fue la fijación cervical alta con el 43.89%. La tasa de complicaciones fue del 16.13% con una mortalidad del 0%. Conclusiones : La patología cervical alta es rara, siendo la del tipo traumática la más frecuente, pero un manejo oportuno y adecuado permite un mejor pronóstico funcional del paciente.

Introduction : The C0-C1-C2 complex is responsible of axial load transition, and its biomechanical function is unique, it is affected by multiple pathological conditions; and generally speaking, the literature does not consider these conditions as a single item, it describes them according to etiology. For our study we considered five groups: trauma-related, congenital, rheumatic-inflammatory, neoplastic, and degenerative. Objective : To determine epidemiological, clinical, and therapy-related characteristics in upper cervical pathological conditions. Materials and methods : All patients with a clinical-radiological diagnosis of any upper cervical pathological condition that had undergone surgery between 2016 and 2021 in Guillermo Almenara Hospital were included. Student's t test and chi square methods were used. patients were divided into one of the five aforementioned groups. Results : Thirty-one patients were included in the study; their mean age was 51.16 years. The most frequent upper cervical pathological condition was trauma-related, with 35.48%. Motor deficit occurred in 51.61% of all patients, and sensitive deficit occurred in 54.84%. The most frequently surgical procedure performed was upper cervical fixation, in 43.89% of all patients. Complication rate was 16.13%, and mortality was 0%. Conclusions : Upper cervical pathological conditions are rare, trauma-related conditions are most frequent, but timely and adequate management allow us to achieve better functional prognosis for these patients.

Auriculotherapy in a patient with Arnold Chiari malformation type II: Case report.

AIMS: Auriculotherapy is a therapeutic method of traditional Chinese Medicine in which a stimulus is exerted on the ear and activates energy channels throughout the body, helping to control anxiety, stress, pain, inflammation, illnesses chronic, and possibly can be used in patient with Arnold Chiari Malformation type II who have painful symptoms that are difficult to resolve. Thus, the aim of this article is to present a case in a patient with Chiari Malformation II where auriculotherapy and effect of laser therapy were performed, to relieve symptoms of agitation, anxiety and healing of self-mutilation injuries. CASE REPORT: Data from medical records were used to collect medical and dental history, complaints and treatments performed. Eight sessions of auriculotherapy and one of laser therapy were carried out in order to heal the lesions. The patient presented lower levels of anxiety, agitation and ceased the habit of self-mutilation and, consequently, the pain. CONCLUSIONS: The use of auriculotherapy led to a considerable improvement in the patient's painful condition and reduced anxiety, also benefiting the interaction with her family members and laser therapy promoted wound healing.

Malformación de Chiari tipo I en una embarazada. Reporte de caso / Chiari malformation type I in pregnant women, case report

Resumen ANTECEDENTES: La malformación de Chiari es un padecimiento neurológico, congénito o adquirido, infrecuente. Se caracteriza por el desplazamiento de las estructuras rombencefálicas hacia el canal espinal, por debajo del foramen magno. Se desconoce la frecuencia y evolución que puede tener durante el embarazo porque existen cambios durante el trabajo de parto que pueden predisponer a la herniación del tronco encefálico con compresión medular. Esta situación plantea un desafío terapéutico porque no se dispone de recomendaciones unificadas acerca del momento del parto, vía del nacimiento y mejor técnica anestésica en estas pacientes. CASO CLÍNICO: Paciente primípara, de 24 años, con 34 semanas de embarazo. Acudió a Urgencias del Hospital Universitario de la Samaritana debido a un cuadro clínico de cefalea con signos de alarma. En la resonancia magnética cerebral se encontró una malformación de Chiari tipo I. Con el objetivo de limitar las maniobras de Vasalva durante el trabajo de parto se decidió la cesárea, con anestesia neuroaxial, sin complicaciones y con recién nacido sano. CONCLUSIÓN: La malformación de Chiari tipo I durante el embarazo es infrecuente. Las pacientes con diagnóstico previo al embarazo condicionan un seguimiento en el que se valora la indicación de tratamiento neuroquirúrgico antes de la concepción. Durante el embarazo no puede recomendarse un único método de finalización o anestesia y la atención médica debe estar a cargo un equipo multidisciplinario que formule un plan de atención individualizado para mejorar el desenlace materno y fetal.

Abstract BACKGROUND: Chiari malformation is a rare congenital or acquired neurological disorder, characterized by the displacement of the rhombencephalic structures towards the spinal canal below the level of the foramen magnum; The frequency and progression that can occur during pregnancy are unknown, since there are changes during labor that can predispose to brainstem herniation with spinal cord compression; which poses a therapeutic challenge since there are no unified recommendations about the moment of delivery, delivery route and the best anesthetic technique in these patients CLINICAL CASE: A 24-year-old patient, G1P0 with a 34-week pregnancy, consulted the emergency room of the Hospital Universitario de la Samaritana, due to a clinical profile of headache with warning signs; to the study of cerebral magnetic resonance with a finding of Chiari type I malformation. In order to limit the Valsalva maneuvers during labor, a caesarean section was performed at term under neuraxial anesthesia without complications with a healthy newborn. CONCLUSION: Chiari malformation type I during pregnancy is rare. Patients diagnosed prior to pregnancy condition a follow-up in which the indication for neurosurgical treatment prior to conception is assessed. During pregnancy, no single method of termination or anaesthesia can be recommended and medical care should be provided by a multidisciplinary team formulating an individualized care plan to improve maternal and fetal outcome.

Sectioning of the Filum Terminale in Patients with Chiari Malformation Type 1 Associated with Occult Tethered Cord Syndrome: Literature Review / Secção do filum terminale em pacientes com malformação de chiari tipo 1 associado a síndrome oculta da medula presa: revisão de literatura

Approximately 125 years ago, a group of pathologies now known as Chiari malformations was described for the first time. However, some mechanisms of its formation still remain unknown. A bibliographic survey was performed through a search in PubMed. In 1938, it was already theorized that an increase in spinal cord tension could be the cause of Chiari malformation type 1 (CM1) tonsillar herniation. In 1953, a condition known for the anchoring of the filum terminale to the vertebral canal was described for the first time and would later be known as tethered cord syndrome (TCS). Some studies have shown that it is associated with increased tension in the spinal cord, and this formed the basis for a possible pathophysiological explanation of tonsillar herniation. Case series emerged reporting that treatment for TCS with the sectioning of the filum terminale (SFT) could provide clinical improvement of patients with CM1. A new pathological entity emerged when it was realized that patients with the clinical picture of TCS could have the medullary cone in its normal position, differing from the caudal migration expected for the TCS. This condition became known as occult tethered cord syndrome (OTCS). Case series attempted to demonstrate its association with the origin of CM1, a non-intuitive association, since the cone in the normal position contradicts traction as a source of tonsillar herniation. To this day, the absence of randomized control trials limits any conclusions regarding the effectiveness of SFT for the treatment of patients with CM1.

Há cerca de 125 anos, era descrita pela primeira vez um grupo de patologias hoje conhecidas como malformações de Chiari. No entanto, alguns mecanismos de sua formação ainda permanecem desconhecidos. Um levantamento bibliográfico foi feito através do PubMed. Em 1938, já se teorizava que um aumento da tensão medular poderia ser a causa da herniação tonsilar da malformação de Chiari tipo 1 (MC1). Em 1953, foi descrita pela primeira vez uma condição conhecida pelo ancoramento do filum terminale ao canal vertebral e que mais tarde viria a ser conhecida como síndrome da medula presa (SMP). Alguns estudos demonstraram que ela estava associada à tensão aumentada na medula espinhal, e a partir disso estava formada a base para uma possível explicação fisiopatológica da herniação tonsilar. Séries de casos surgiram relatando que o tratamento para a SMP com a secção do filum terminale poderia proporcionar melhora clínica aos pacientes com MC1. Uma nova entidade patológica surgiu quando se percebeu que pacientes com o quadro clínico de SMP poderiam ter o cone medular em sua posição normal, diferente da migração caudal esperada para a SMP. Essa condição ficou conhecida como SMP oculta. Séries de casos tentaram demonstrar sua associação com a origem da MC1, uma associação nada intuitiva, visto que o cone na posição normal contradiz a tração como fonte da herniação tonsilar. A ausência de ensaios randomizados controlados até o dia de hoje não permite concluir a eficácia do método de secção do filum no tratamento de pacientes com MC1.

Lesão medular por traumatismo raquimedular e malformação de Chiari tipo I: uma comparação de características clínicas / Spinal cord injury and Chiari type I malformation: a comparison of clinical features

Foram comparadas características clínicas e epidemiológicas de pacientes com tetraplegia traumática e Malformação de Chiari tipo I, atendidos em centro de neurorreabilitação através de estudo observacional analítico e retrospectivo, realizado por meio de análise de prontuário eletrônico. No grupo de pacientes com Malformação de Chiari tipo I predominaram mulheres, com marcha e que apresentavam transtornos de ansiedade e a presença de siringomielia. No grupo com tetraplegia traumática predominaram os homens, com locomoção em cadeira de rodas e maior presença de espasticidade e disfunções neurogênicas do intestino e do trato urinário inferior. Os pacientes com Malformação de Chiari tipo I representam um grupo com diferentes características clínicas e epidemiológicas em relação aos pacientes com sequelas de traumatismo raquimedular, tradicionalmente o principal grupo atendido dentro dos programas de neurorreabilitação. Desta forma, os pacientes com Malformação de Chiari tipo I necessitam de uma abordagem diferenciada que contemplem suas necessidades nos programas de neurorreabilitação.

Clinical and epidemiological characteristics of patients with traumatic tetraplegia and Chiari malformation type I treated at a neurorehabilitation center were compared through an analytical and retrospective observational study, carried out through electronic medical record analysis. In the group of patients with Chiari malformation type I predominated female gender, with gait, the presence of syringomyelia and anxiety disorders. In the group with traumatic tetraplegia predominated male gender, use of wheelchair and presence of spasticity and neurogenic dysfunctions of the bowel and lower urinary tract. Patients with Chiari malformation type I represent a group with different clinical and epidemiological characteristics in relation to patients with sequelae of spinal cord injury, traditionally the main group treated within neurorehabilitation. Thus, patients with Chiari malformation type I need a differentiated approach that addresses their needs in neurorehabilitation.

Increase of the clivus-canal angle in patients with basilar invagination, without atlantoaxial displacement, treated with a simple maneuver of indirect decompression of the odontoid with the head clamp, during posterior occipitocervical arthrodesis.

BACKGROUND: Basilar invagination (BI) can be defined as the insinuation of the content of the craniovertebral junction through the foramen magnum toward the posterior fossa. BI is a prevalent condition in Northeast Brazil. The present study describes the changes in the clivus-canal angle (CCA) in the postoperative period in patients with symptomatic BI operated by a posterior approach, using a simple technique of indirect reduction of the odontoid associated with occipitocervical fixation. METHODS: Patients underwent radiological evaluations by magnetic resonance imaging in the pre and postoperative periods, where the height of the odontoid tip was measured in relation to the Chamberlain line and the ACC. All patients underwent posterior occipitocervical fixation with specific maneuvers of distraction and extension of the cephalic segment with the aid of a head clamp with three fixation points for anterior reduction of the odontoid. RESULTS: Among the 8 patients evaluated in the series, all had increased ACC in the postoperative period, with a mean of 14.81 ± 1.54°, and statistically significant difference between the pre and postoperative periods (P < 0.05). CONCLUSION: The indirect surgical reduction of the odontoid process by a posterior approach through the manipulation (distraction-extension) of a "Mayfield" type of head clamp followed by occipitocervical fixation proved to be effective in improving the ACC, being easily reproducible.

Siringomielia asociada a malformación de Chiari tipo I. Informe de caso / Syringomyelia associated with Chiari type I malformation. Case report

RESUMEN La siringomielia es una cavidad quística de la medula espinal, y se considera un padecimiento progresivo y degenerativo. Existen múltiples y variadas maneras de clasificarla, basadas principalmente en su origen, o en los mecanismos conocidos de producción. Se presenta el caso de un paciente de 27 años, con cuadro de deficiencia motriz de cuatro meses de evolución, que comenzó en miembros superiores y continuó con torpeza a la marcha. Al examen físico presentó marcha paretoespástica, cuadriparesia con predominio de debilidad en miembros superiores con respecto a los inferiores, además de espasticidad crural y braquial izquierda, con hipotonía braquial derecha, asociado con atrofia distal de ambos miembros superiores e hipoestesia térmica y dolorosa suspendida del miembro superior derecho. La resonancia magnética nuclear mostró cavidad siringomiélica amplia en columna cervical, con descenso de las amígdalas cerebelosas, compatible con malformación de Chiari tipo I. Aunque la asociación de ambas entidades es común, el déficit motriz progresivo en un paciente joven es motivo de ingreso o consulta poco frecuente en el Servicio de Neurología. En estos casos, el tratamiento descompresivo puede disminuir el tamaño de la cavidad siringomiélica.

ABSTRACT Syringomyelia is a cystic cavity of the spinal cord, and is considered a progressive and degenerative condition. There are multiple and varied ways to classify it, based mainly on its origin, or on known production mechanisms. A 27-year-old patient with a four-month history of motor impairment, which began in the upper limbs and continued with clumsy gait, was presented. On physical examination, he presented paretospastic gait, quadriparesis with a predominance of weakness in the upper limbs with respect to the lower ones, as well as crural and left brachial spasticity, with right brachial hypotonia, associated with distal atrophy of both upper limbs and suspended thermal and painful hypoesthesia of the limb upper right. Nuclear magnetic resonance imaging showed a wide syringomyelic cavity in the cervical spine, with descent of the cerebellar tonsils, compatible with type I Chiari malformation. Although the association of both entities is common, progressive motor deficit in a young patient is a reason for admission or consultation rare in the Neurology Service. In these cases, decompressive treatment can decrease the size of the syringomyelic cavity.

Severe Cerebellar Degeneration and Chiari I Malformation - Speculative pathophysiology based on a systematic review

SUMMARY BACKGROUND Symptomatic Chiari Type I Malformation (CM) is treated with posterior fossa decompression with or without duroplasty. We have noticed some cases with concomitant severe cerebellar ataxia due to cerebellar atrophy. The aim of this study is to review the literature of CM associated with severe cerebellar atrophy and discuss its potential physiopathology. METHODS A systematic literature review in the Pubmed Database was performed using the following key-terms: "cerebellar atrophy Chiari", and "cerebellar degeneration Chiari". Articles reporting the presence of cerebellar degeneration/atrophy associated with CM were included. RESULTS We found only six studies directly discussing the association of cerebellar atrophy with CM, with a total of seven cases. We added one case of our own practice for additional discussion. Only speculative causes were described to justify cerebellar atrophy. The potential causes of cerebellar atrophy were diffuse cerebellar ischemia from chronic compression of small vessels (the most mentioned speculative cause), chronic raised intracranial pressure due to CSF block, chronic venous hypertension, and association with platybasia with ventral compression of the brainstem resulting in injury of the inferior olivary nuclei leading to mutual trophic effects in the cerebellum. Additionally, it is not impossible to rule out a degenerative cause for cerebellar atrophy without a causative reason. CONCLUSIONS Severe cerebellar atrophy is found in some patients with CM. Although chronic ischemia due to compression is the most presumed cause, other etiologies were proposed. The real reasons for cerebellar degeneration are not known. Further studies are necessary.

RESUMO OBJETIVO A Malformação de Chiari (MC) tipo I sintomática é tratada através da descompressão da fossa posterior com ou sem duroplastia. Observamos alguns casos com ataxia cerebelar grave concomitante devido à atrofia cerebelar. O objetivo deste estudo é revisar a literatura sobre MC associada à atrofia cerebelar grave e discutir sua possível fisiopatologia. METODOLOGIA Conduzimos uma revisão sistemática da literatura no banco de dados Pubmed utilizando as seguintes palavras-chave: "cerebellar atrophy Chiari", e "cerebellar degeneration Chiari". Artigos sobre a presença de degeneração/atrofia cerebelar associada à MC foram incluídos. RESULTADOS Encontramos apenas seis estudos que discutiam diretamente a associação entre atrofia cerebelar e MC, com um total de sete casos. Nós adicionamos um caso da nossa própria prática para ampliar a discussão. Apenas causas especulativas foram descritas para justificar a atrofia cerebelar, entre elas: isquemia cerebelar difusa devido à compressão crônica de pequenos vasos (a causa especulativa mais citada), pressão intracraniana elevada crônica devido ao bloqueio de LCR, hipertensão venosa crônica e associação com platibasia com compressão ventral do tronco cerebral, resultando em lesão do núcleo olivar inferior e levando a efeitos tróficos mútuos no cerebelo. Além disso, não é possível descartar uma causa degenerativa para atrofia cerebelar sem motivos claros. CONCLUSÃO A atrofia cerebelar grave é encontrada em alguns pacientes com MC. A isquemia crônica causada por compressão é a causa mais apontada como suspeita, porém outras etiologias foram propostas. As reais causas da degeneração cerebelar não são conhecidas. Mais estudos são necessários.

Chiari malformation and types of basilar invagination with/without syringomyelia.

BACKGROUND: Craniometric studies document different subtypes of craniocervical junction malformations (CCJM). Here, we identified the different types and global signs and symptoms (SS) that correlated with these malformations while further evaluating the impact of syringomyelia. METHODS: Prospective data concerning SS and types of CCJM were evaluated in 89 patients between September 2002 and April 2014 using Bindal's scale. RESULTS: The mean Bindal's scores of each type of CCJM were Chiari malformation (CM) = 74.6, basilar invagination Type 1 (BI1) = 78.5, and BI Type 2 (BI2) = 78. Swallowing impairment and nystagmus were more frequently present in the BI patients. Symptomatic burdens were higher in patients with syringomyelia and included weakness, extremity numbness, neck pain, dissociated sensory loss, and atrophy. CONCLUSION: There were no statistically significant differences in SS between the different CCJM types. BI patients had more swallowing and nystagmus complaints versus CM patients, but there were no significant differences in clinical SS between BI1 and BI2 patients. Notably, those with attendant syringomyelia had a higher SS burden.

Determination of Normal Values of the Basal Angle in the Era of Magnetic Resonance Imaging.

BACKGROUND: The flexion of the skull base (basal angle [BA]) is the inclination between the anterior fossae and the basilar ramp of the occipital and sphenoid bones. An increased BA, termed platybasia, is usually associated with basilar invagination. BA reference values in the magnetic resonance imaging (MRI) era and the diagnosis of platybasia are of clinical importance. The transnasal approach has been the surgical technique of choice to remove the odontoid process in cases of ventral brainstem compression in patients with platybasia. The knowledge of normal BA values has been influenced by technological image acquisitions. The aim of this study was to determine the normal BA values in normal subjects in the MRI era. METHODS: For the determination of normal BA values, the literature reporting these values in normal individuals was reviewed and a meta-analysis of pertinent studies was performed. RESULTS: A total of 8 articles were included in this study, evaluating a total of 667 individuals. A summary measure of the results was obtained. The data obtained in this analysis provided a final basal angle value of 116.5 degrees (95% confidence interval, 104.39-128.7). The normal basal angle ranges between 104 and 129 degrees. CONCLUSION: Platybasia can be defined as a value >129 from the basal angle.

Dyke-Davidoff-Masson syndrome and chiari type ii malformation / Síndrome de Dyke-Davidoff-Masson e malformação de chiari tipo II

Dyke-Davidoff-Masson Syndrome is a syndrome associated with refractory epilepsy. The Chiari II malformation is a complex congenital malformation of the brain. The authors report a case of a 15 years-old adolescent presenting Dyke-Davidoff-Masson syndrome and Chiari type II malformation association. This case demonstrates an unusual association in neuroimaging tests that indicates the need to evaluate associated diseases, such as myelomeningocele, corpus callosum dysgenesis and syringohydromyelia.

A Síndrome de Dyke-Davidoff-Masson é uma síndrome associada à epilepsia refratária. A malformação de Chiari II é uma malformação congênita complexa do cérebro. Os autores relatam um caso de uma adolescente de 15 anos apresentando a síndrome de Dyke-Davidoff-Masson associada à malformação de Chiari tipo II. Este caso demonstra uma associação incomum nos exames de neuroimagem que indica a necessidade de avaliar doenças associadas, como mielomeningocele, disgenesia do corpo caloso e a siringohidromielia.

Pyramidal and Proprioceptive Dysfunctions as the Main Neurologic Features in Symptomatic Type I Chiari Malformation Patients

Objective Broader access to magnetic resonance imaging (MRI) has increased the diagnosis of tonsillar ectopia, with most of these patients being asymptomatic. The early diagnosis and treatment of type I Chiari malformation (CM I) patients has impact on the prognosis. This study supplements information about the neurologic exam of symptomatic patients with CM I. Methods The sample was composed of 32 symptomatic patients with CM I diagnosed by a combination of tonsil herniation of more than 5 mm below the magnum foramen (observed in the sagittal T2 MRI) and at least one of the following alterations: intractable occipital headache, ataxia, upper or lower motor neuron impairment, sensitivity deficits (superficial and deep) or lower cranial nerves disorders. Results Occipital headache was the most frequent symptom (53.12%). During the physical exam, the most common dysfunctions were those from the pyramidal system (96.87%), followed by posterior cord syndrome (87.5%). Discussion In this study, patients became symptomatic around the fifth decade of life, which is compatible with previous descriptions. Patients withmore than 2 years of evolution have worse responses to treatment. Occipital headache, symptoms in the upper limbs, gait and proprioceptive disorders are common findings in patients with CM I. Conclusion Deep tendinous reflexes and proprioception disorders were the main neurologic features found in symptomatic CM I patients.

Posterior fossa decompression with duraplasty in Chiari surgery: A technical note / Descompressão de fossa posterior com duroplastia no tratamento cirúrgico do Chiari: nota técnica

Summary Chiari malformation (CM) is the most common and prevalent symptomatic congenital craniocervical malformation. Radiological diagnosis is established when the cerebellar tonsils are located 5 mm or more below the level of the foramen magnum on magnetic resonance imaging (MRI). Surgical treatment is indicated whenever there is symptomatic tonsillar herniation or syringomyelia/hydrocephalus. The main surgical treatment for CM without craniocervical instability (such as atlantoaxial luxation) is posterior fossa decompression, with or without duraplasty. The authors describe in details and in a stepwise fashion the surgical approach of patients with CM as performed at the State University of Campinas, emphasizing technical nuances for minimizing the risks of the procedure and potentially improving patient outcome.

Resumo A malformação de Chiari (MC) é a malformação craniocervical congênita sintomática mais comum e prevalente. O diagnóstico radiológico é definido quando as tonsilas cerebelares estão localizadas pelo menos 5 mm abaixo do nível do forame magno na ressonância magnética (RM). Quando há hérnia tonsilar sintomática, siringomielia ou hidrocefalia, o tratamento cirúrgico é indicado. O principal tratamento cirúrgico para MC sem instabilidade craniocervical (como a luxação atlantoaxial) é a descompressão da fossa posterior com ou sem duroplastia. Os autores descrevem detalhadamente a abordagem cirúrgica de pacientes com MC realizada na Universidade Estadual de Campinas, enfatizando nuances técnicas para minimizar os riscos relacionados ao procedimento e melhorar os resultados pós-operatórios.

Evolución Natural De La Siringomielia: Presentación De Un Caso / Natural Evolution Of Syringomyelia: Case Report

RESUMEN La siringomielia es una enfermedad crónica degenerativa y progresiva de la médula espinal que se caracteriza por una pérdida disociada de la sensibilidad y amiotrofia braquial, con evidencia de cavitación centromedular. Presentamos el caso de una paciente de 62 años de edad, con siringomielia asociada a malformación de Chiari tipo 1 que rechazó espontáneamente cualquier intervención quirúrgica a los cinco años de su diagnóstico por lo cual se ha realizado un seguimiento de 21 años de evolución. Las imágenes de resonancia magnética demuestran la evolución natural de la siringomielia desde 1998 hasta la actualidad.

SUMMARY Syringomyelia is a chronic degenerative and progressive disease of the spinal cord characterized by a dissociated loss of brachial sensitivity and amyotrophy, with evidence of centromedullary cavitation. We report the case of a 62-year-old female patient with syringomyelia associated with Arnold Chiari malformation (type 1) who spontaneously rejected any surgical intervention five years after her diagnosis, which has been followed up for 21 years. Magnetic resonance imaging demonstrates the natural evolution of syringomyelia from 1998 to the present.

[Type I Chiari malformation associated with cerebellar atrophy. Case report]. / Malformación de Chiari Tipo I asociado a atrofia cerebelosa. Reporte de caso.

Chiari malformation is characterized by caudal displacement of the cerebellar tonsils that penetrate into the spinal canal through the foramen magnum, achieving reach the atlas or axis. trunk and any drop of the fourth ventricle is observed. Typically is seen in young adults. In some cases scoliosis and Syringomyelic cavities may occur. The authors present (as far as they know) the first case in the literature with long term follow-up, of a caucasian woman with an unusual form of cerebellar atrophy and Chiari Type I malformation, suffering from weakness in his upper and lower extremities with rapidly progression. The patient was successfully treated with suboccipital decompression and C1 laminectomy.

La malformación de Chiari se caracteriza por un desplazamiento caudal de las amígdalas cerebelosas que penetran hacia el canal raquídeo por el foramen mágnum, logrando llegar hasta el atlas o el axis. No hay descenso del tronco y tampoco del cuarto ventrículo. Típicamente se observa en adultos jóvenes. En algunos casos hay escoliosis y cavidades siringomiélicas. Los autores presentan lo que a su conocimiento es el primer caso de la literatura con seguimiento a largo plazo de una mujer de raza blanca con una forma inusual de atrofia cerebelosa y malformación de Chiari tipo I, que sufre de debilidad en sus extremidades superiores e inferiores rápida y progresivamente. La paciente fue tratada con éxito mediante descompresión suboccipital y laminectomia C1.

Congenital hemangioma in spondylocostal dysostosis: a novel association

Abstract Congenital hemangioma is a benign tumor caused by dysfunction in embryogenesis and vasculogenesis, which progresses during fetal life to manifest as fully developed at birth. Although hemangiomas are the most common tumor of infancy, rapidly involuting congenital hemangioma has not been described in spondylocostal dysostosis. I report the novel association of congenital hemangioma and spondylocostal dysostosis in a Mexican newborn female patient with neural tube defects. Given the embryological relationship between skin and nervous system, I surmise that this association is not coincidental. I also propose that these morphologic alterations be incorporated to the spondylocostal dysostosis phenotype and specifically looked for in other affected children, in order to provide appropriate medical management and genetic counseling.