Malformación de Arnold-Chiari tipo I con siringomielia

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.

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.

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.

Research Progress on the Application of Goel Technique in Craniovertebral Junction Anomalies / 中国医学科学院学报

Craniovertebral junction anomalies are a group of diseases characterized by the pathological changes of occipital bone,atlantoaxial bone,cerebellar tonsil,surrounding soft tissue,and nervous system,which are caused by a variety of factors.Chiari malformation is a common type of craniovertebral junction anomalies,the conventional surgical therapy of which is posterior fossa decompression.Currently,scholars represented by Goel have proposed a new theory on the classification,pathogenesis,and treatment of Chiari malformation based on posterior atlantoaxial fixation (Goel technique).This article introduces the progress in Goel technique,aiming to provide reference for the clinical work.

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.

Juvenile scoliosis as the first manifestation of syringomyelia associated with Chiari malformation type I / Escoliosis juvenil como primera manifestación de siringomielia asociada con malformación de Chiari tipo I

Abstract Background: Scoliosis is a spinal deformity that usually manifests as a structural curve determining a variable deformity of the trunk. According to some published series, 4 to 26% of scoliosis initially classified as idiopathic show neurological alterations when studied with nuclear magnetic resonance, such as syringomyelia and Chiari malformation, among the most frequent. Chiari malformations are characterized by descending herniation of the cerebellar tonsils, brainstem, and IV ventricle into the spinal canal. Case report: We present the case of a patient whose first symptomatic manifestation was early-onset scoliosis. The overlapping of some physical examination signs, such as postural lateralization and scoliosis, reinforced the active suspicion of neuroaxis alterations. Conclusions: Early childhood-onset scoliosis should raise a high degree of suspicion for association with neuro-spinal diseases. Although the frequency of Chiari malformation is low, its early approach could reduce the progression of associated comorbidities. An early detection could change the prognosis of the disease.

Resumen Introducción: La escoliosis es una deformidad de la columna que usualmente se manifiesta como una curva estructural que determina una deformidad variable del tronco. Las malformaciones de Chiari se caracterizan por la herniación descendente de las amígdalas cerebelosas, tronco encefálico y IV ventrículo hacia el canal espinal. Según algunas series publicadas, del 4 al 26% de las escoliosis inicialmente catalogadas como idiopáticas muestran alteraciones neurológicas al ser estudiadas con resonancia magnética nuclear, como la siringomielia y malformación de Chiari, dentro de las más frecuentes. Caso clínico: Se presenta el caso de un paciente cuya primera manifestación sintomática fue escoliosis de comienzo temprano. El solapamiento de algunos signos del examen físico, como la lateralización de la postura y la escoliosis, reforzaron la sospecha activa de alteraciones del neuroeje. Conclusiones: La escoliosis de inicio temprano en la infancia debe despertar un alto grado de sospecha de asociación con enfermedades neuro-espinales. Dentro de este contexto, aunque malformación Chiari es de baja frecuencia, su abordaje precoz permitiría disminuir la progresión de comorbilidades asociadas. La detección temprana podría cambiar el pronóstico de la enfermedad.

Research progress of Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Chiari malformation (CM) is a group of congenital cerebellar tonsillar hernia malformations involving the craniocervical junction. Chiari malformation type I (CMI) is the most common in clinic, however its pathogenesis is still unclear, and there is no consensus on the surgical treatment standard of CMI. At present, the most widely accepted is the theory of posterior fossa incompatibility, so doctors at home and abroad use posterior fossa decompression (PFD) and posterior fossa compression with duraplasty (PFDD) as the gold standard for surgical treatment, and have their own experience and technical improvement. However, the volume of the posterior cranial fossa in some patients is no different from that in healthy people, and about 30% of the patients with CMI have poor results after posterior cranial fossa decompression. As a result, this operation cannot treat all patients with CMI. In recent years, with the development of imaging, the progress of diagnostic technology and the deepening of understanding of CM, some studies have shown that CMI may be related to atlantoaxial instability, and proposed that CMI is the secondary factor of atlantoaxial instability, and atlantoaxial fusion is the standard of surgical treatment, which has caused great controversy in academic circles. Different clinical research results of scholars support or oppose this theory: some studies have shown that the clinical symptom relief rate of patients with CMI treated with atlantoaxial fusion is 96.9%; another study showed that 70% of patients with CMI underwent atlantoaxial fusion had improved neurological function, but the overall postoperative effect was not satisfactory. In short, CMI is related to many diseases and its clinical manifestations are complex. Therefore, individualized management and treatment should be carried out in combination with the clinical manifestations and auxiliary examination results of patients.

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.

Imaging study of simple Chiari malformation type I and atlantoaxial instability / 中华骨科杂志

Objective:To investigate the relationship between simple Chiari malformation type I (CMI) and atlantoaxial instability from the imaging point of view.Methods:A retrospective analysis were performed on 46 patients diagnosed with simple CMI from January 2014 to December 2020. Forty-six normal people matched for age and sex were selected as the normal control group, while 30 patients with atlantoaxial dislocation were selected as the dislocation group. The degree of atlantoaxial joint degeneration in each group was assessed according to Weishaupt degeneration grading; the atlantoaxial joint angulation angle was measured in the control group of patients with simple CMI; and the sagittal imaging parameters of cervical spine X-ray were measured, including C 0-C 1 Cobb angle, C 0-C 2 Cobb angle, C 1-C 2 Cobb angle, C 1-C 7 Cobb angle, C 2-C 7 Cobb angle, C 7 Slope, C 2 Tilt, spino cranial angle (SCA), and C 2-C 7 sagittal vertebral axis (SVA). All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. Results:ICC ranged between 0.842 and 0.974 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. There were significant differences in the distribution of Weishaupt degeneration grading of atlantoaxial joints between simple CMI, normal and dislocation group ( H=53.68, P<0.001 on the left side; H=43.39, P<0.001 on the right side). There were significant differences in the degree of atlantoaxial joint degeneration between the normal group and dislocation group (left, Z=6.60, P<0.001; right, Z=6.29, P<0.001); There were significant differences in the degree of atlantoaxial joint degeneration between the normal group and simple CMI patients (left, Z=5.31, P<0.001; right, Z=4.13, P<0.001); There were significant differences in the degree of atlantoaxial joint degeneration between simple CMI and dislocation group (left, Z=3.20, P=0.001; right, Z=3.15, P=0.002). There were significant difference in the angulation angle of the atlantoaxial articular surface between the normal group and simple CMI patients (left, Z=3.32, P<0.001; right, Z=5.74, P<0.001). There were significant differences in C 0-C 1 Cobb angle ( t=2.41, P=0.018), C 1-C 7 Cobb angle ( t=2.88, P=0.005), C 2-C 7 Cobb angle ( t=3.29, P=0.001), and C 2-C 7 SVA ( t=2.87, P=0.005) between the normal group and simple CMI patients, but there was no significant difference in other parameters. Conclusion:The degree of atlantoaxial joint degeneration in patients with simple CMI is higher than that in normal people, the angulation angle is larger, and the cervical lordosis is larger, suggesting that there may be atlantoaxial joint instability. This study provides further evidence that Chiari malformation type I is associated with atlantoaxial instability.

The clinical effect of posterior fossa decompression combined with dural reconstruction in the treatment of Chiari malformation-Ⅰ complicated with syringomyelia / 中国医师进修杂志

Objective:To investigate the clinical effect of posterior fossa decompression combined with dural reconstruction in the treatment of Chiari malformation-Ⅰ(CM-Ⅰ) complicated with syringomyelia (SM).Methods:The clinical data of 50 patients with CM-Ⅰ complicated with SM who were treated in Yan′an University Xianyang Hospital from June 2019 to January 2021 were analyzed. They were divided into the study group (27 cases) and the control group (23 cases) according to the surgical methods. The former received posterior fossa decompression combined with dural reconstruction, while the latter received posterior fossa decompression alone. The clinical symptom improvement, neurological function, cerebrospinal fluid dynamics and syringomyelia changes were compared between the two groups before and after the surgery, and postoperative complications were compared.Results:The overall clinical symptom improvement rate between the two groups had no significant difference ( P> 0.05). After the surgery, the scores of pain, sensory disturbance, dyskinesia and ataxia in the study group were higher than those in the control group: (4.56 ± 0.35) points vs. (4.28 ± 0.43) points, (3.61 ± 0.82) points vs. (3.15 ± 0.73) points, (3.81 ± 0.44) points vs. (3.59 ± 0.50) points, (4.43 ± 0.41) points vs. (4.09 ± 0.53) points, there were statistical significant ( P<0.05). After the surgery, the cerebrospinal fluid stroke volume (SV) and mean flow (MF) in the study group were higher than those in the control group: (0.05 ± 0.02) ml vs. (0.04 ± 0.01) ml, (0.05 ± 0.01) ml/s vs. (0.04 ± 0.01) ml/s; the maximum peak flow velocity (V max) of the head and tail in the study group were lower than those in the control group: (3.14 ± 1.05) mm/s vs. (3.87 ± 1.13) mm/s, (5.56 ± 1.38) mm/s vs. (6.43 ± 1.22) mm/s, there were statistical significant ( P<0.05). There were no significant differences in the rate of reduction or disappearance of syringomyelia, the rate of no change and the rate of increase of syringomyelia after the surgery between the two groups ( P>0.05). There was no significant difference in the incidence of postoperative complications between the two groups ( P>0.05). Conclusions:Posterior fossa decompression combined with dural reconstruction in CM-Ⅰ complicated with SM can better improve cerebrospinal fluid dynamics, and promote the reduction of syringomyelia without increasing postoperative complications.

Siringomielia por malformación de Chiari: resolución espontánea. Revisión bibliográfica / Syringomyelia due to Chiari malformation: spontaneous resolution. Bibliographic review

Objetivo: describir y analizar los casos de siringomielia por malformación de Chiari tipo 1 con resolución espontánea. Método: se buscó en Pubmed bajo las palabras clave malformación de Chiari tipo 1, siringomielia y resolución espontánea todos los casos relacionados. Se registraron las características poblacionales de cada caso, evolución y mecanismos propuestos. Resultados: En la población adulta se encontraron 22 casos (edad media: 35,81) y en la población pediátrica se encontraron 37 casos (edad media: 9,82). Las hipótesis sobre los mecanismos que produjeron la resolución espontánea fueron: cambios en la posición de las amígdalas relacionadas con la edad, atrofia del cerebelo, fisura medular, reducción del esfuerzo físico, variaciones de la presión intraespinal, crecimiento del cráneo y disminución de la presión intracraneana. Conclusión: La resolución espontánea es posible en pocos casos. Si bien este hecho invita a realizar controles periódicos y evitar la cirugía, la espera conlleva riesgos ante la posibilidad de un agravamiento agudo o de que surjan secuelas permanentes. La información disponible hoy en día no permite predecir con seguridad la evolución de cada caso

Objective: describe and analyze the cases of syringomyelia caused by Chiari type 1 malformation with spontaneous resolution. Method: we searched in Pubmed with the key words Chiari malformation type 1, syringomyelia and spontaneous resolution all related cases. The population characteristics of each case were recorded along with its outcome and proposed mechanisms. Results: in the adult population there were 22 cases (medium age: 35.81) and in the pediatric population there were 37 cases (medium age: 9.82). The hypothesis about the mechanisms that produce the spontaneous resolution were: changes in tonsils position related to age, cerebellar atrophy, cord fissures, reduction of physical effort, variations in intraspinal pressure, skull growth and decrease in intracranial pressure. Conclusion: the spontaneous resolution of syringomyelia invites to carry out regular check-ups and avoid surgery. However, waiting carries risks with the possibility of acute worsening or permanent sequelae. The information available today does not allow to predict with certainty the evolution of each case.

Spinal cord atrophy-like changes in adolescent Chiari malformation type I patients: a neglected imaging change / 中华骨科杂志

Abstract:To compare the morphology of spinal cord between healthy adolescents with adolescent Chiari malformation type I (CMI) patients and investigate the impact of syringomyelia on the morphology of spinal cord in CMI patients.Methods:The clinical and radiological data of 292 CMI patients diagnosed by our center between June 2012 and March 2019 were retrospectively reviewed. Among them 15 CMI patients without syringomyelia were recruited in the CM group. Among the remaining 277 CMI patients, 274 patients had syringomyelia below the C 3-4 intervertebral disc. According to the principle of best matching, CMI patients with syringomyelia were selected with the closest age to the CM group (±18 months), and 30 CMI patients with syringomyelia were included in the CMS group according to a ratio of 1∶2. Thirty healthy adolescents were enrolled as the control group (NC group) in the same way. The anteroposterior diameters of spinal cord at C 2 (DSCO-C 2), spinal canal at C 2 (DSCA-C 2), midbrain-pontine junction (DPJ), the distance between the tip of cerebellar tonsils and the foramen magnum (AB) and the maximal diameter of the syrinx (D-syrinx) were measured on MRI. All radiographic parameters were measured twice independently by two spine surgeons, and intraclass correlation coefficient (ICC) were determined to demonstrate intra- and inter-observer reliability. One-way ANOVA and SNK- q test were used to compare the above radiographic parameters and age between CM, CMS and NC group. The distribution of genders was compared between the three groups using Chi-square tests. Pearson correlation analysis were conducted to demonstrate the relationship between radiographic parameters in CM and CMS group. Results:ICC ranged between 0.91 and 0.95 in the current study, demonstrating "excellent" reliability of radiographic measurements. No significant difference was noted regarding age and the distribution of genders among the three groups. Patients in CM and CMS groups showed similar DSCO-C 2 values ( P=0.254), both of which were significantly lower than that in NC group ( P<0.001). DSCA-C 2 in CMS group was significantly larger than that in CM ( P=0.003) and NC ( P<0.001) groups, while no significant difference was found between the CM and NC groups ( P=0.216). Moreover, DPJ in CMS group was significantly lower than that in CM group ( P<0.001) and NC group ( P<0.001). There was no significant difference in AB between CM and CMS groups ( P=0.948). DSCO-C 2 was significantly positively correlated with DSCA-C 2 in CMS group ( r=0.906, P<0.001), while AB, D-syrinx, DSCO-syrinx, DSCA-syrinx and DPJ were not significantly correlated with DSCA-C 2. There were significant correlations observed between DPJ and other radiographic parameters in the CMS group (all P>0.05). Significant positive correlation between DSCO-C 2 and DPJ was observed in CM group ( r=0.703, P=0.005). There was no significant correlation between DSCO-C 2 and DSCA-C 2 and DPJ in NC group (all P>0.05). Conclusion:CMI adolescents have significant atrophic change of cervical spinal cord and midbrain-pontine junction compared with healthy adolescents, regardless of the existence of syrinx. Moreover, syrinx in CMI patients indicated more obvious atrophic change of midbrain-pontine junction and dilated spinal canal compared with isolated CMI patients.

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.

Observational outcome in surgery for Chiari malformation patients

Chiari malformation is the commonest anomaly of the craniovertebral junction involving both the skeletal as well as the neural structures. It is congenital anomaly of the hindbrain characterised by downward elongation of the brain stem and cerebellum into the cervical portion of spinal cord. Most common presenting symptoms was pain in the nape of neck with sub-occipital headache and weakness. If not intervened early in these cases they may progress to quadriparesis and respiratory failure. This study includes authors experience of 30 surgical corrections of Chiari malformation performed at civil hospital Ahmedabad from 2017 to 2019. The age and sex of the patient, the presence of syrinx, the type of surgical procedure and the clinical outcome were determined post-operatively and on follow up. Cerebro spinal fluid leak and collection were observed in patient who undergone duroplasty only with no leakage in patient undergone syringo-subarachnoid shunt. Overall, tingling/numbness had best improvement showed improvement in 13 out of 16 patients. Power showed improvement in 20 out of 27 patients and pain showed improvement in 18 patients. Wasting, clawing and cerebellar signs and bony deformity showed no improvement in any of the above procedures. Authors can conclude for Chiari malformation decompression with or without duroplasty with additional procedure with post-operative physiotherapy and analgesia is the suitable treatment.

Hemifacial Spasm Associated with Chiari Type I Malformation: Surgical Considerations and Case Report

Hemifacial spasm (HS) is a movement disorder characterized by paroxysmal and irregular contractions of the muscles innervated by the facial nerve. Chiari malformation type I (CM I) is a congenital disease characterized by caudal migration of the cerebellar tonsils, and surgical decompression of foramen magnum structures has been used for treatment. The association of HS with CM I is rare, and its pathophysiology and therapeutics are speculative. There are only a few cases reported in the literature concerning this association. The decompression of the posterior fossa for the treatment of CM I has been reported to relieve the symptoms of HS, suggesting a relation between these diseases. However, the possible complications of posterior fossa surgery cannot be underrated. We report the case of a 66-year-old patient, in ambulatory follow-up due to right HS, no longer responding to botulinum toxin treatment. Magnetic resonance imaging (MRI) of the skull revealed compression of the facial nerve and CM I. The patient underwent surgery for HS by neurovascular microdecompression of the facial nerve via right lateral suboccipital craniectomy, but presented significant clinical worsening in the postoperative period even though the cerebellum edema related to surgical manipulation was mild. Due to the clinical worsening, the patient underwent a median suboccipital craniectomy with decompression of the foramenmagnum structures. After this second surgery, the patient had progressive improvement and was discharged from the hospital for ambulatory care.

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.

The use of magnetic resonance phase-contrast cine in Chiari malformation with syringomyelia

@#mesodermal origin and is commonly associated withsyringomyelia. Foramen magnum decompression is thefirst-line of standard treatment in symptomatic patients witha confirmed radiographic diagnosis. Magnetic resonance(MR) cine allows accurate evaluation of cerebrospinal fluid(CSF) physiology at the craniovertebral junction but oftenthis is under-utilised in Malaysia. Methods: In this series, we looked into nine cases of CM withsyringomyelia from clinical and radiological perspectivebefore and after surgery. The radiological parameters wereherniated tonsillar length, syrinx: cord ratio, syrinx lengthand diameter. Flow velocity and morphologic changes inChiari were illustrated. Results: Seven patients showed either reduction in syrinxlength, syrinx: cord ratio or both postoperatively. Clinicalrecovery somewhat varied in motor and sensory symptoms.Four patients gained better functional grade in modifiedRankin scale (MRS) while the rest remained similar. Thestudy highlighted the advantage of CSF flow dynamicsinformation over MR anatomical radiographic improvementin addressing the neurologic and functional recovery. Wealso discussed the practicality of cine sequence inpreoperative patient selection, syrinx analysis andpostoperative flow evaluation in anticipation of clinicaloutcome. Conclusion: Phase-contrast cine MRI is a useful tooldictated by resource availability. We recommend its routineuse in preoperative analysis and subsequent observationalfollow-up after surgery.

Presyrinx Associated with Post-Traumatic Hydrocephalus Successfully Treated by Ventriculoperitoneal Shunt / 대한신경손상학회지

Presyrinx consists of reversible spinal cord swelling without frank cavitation, as observed on T2 weighted magnetic resonance imaging (MRI). The condition may evolve into syringomyelia, but timely surgical interventions have achieved meaningful results. Here, we report the case of a 27-year-old woman who presented with headache, dizziness, and diplopia 2 months after suffering a mild head trauma. On MRI, hydrocephalus, downward herniation of the cerebellar tonsil, and a diffuse high signal change in the cervical spinal cord were detected. After insertion of a ventriculoperitoneal shunt, her neurological symptoms resolved, and she has had no signs of presyrinx recurrence for >4 years.

Arnold-Chiari Type 1 Malformation Mimicking Benign Paroxysmal Positional Vertigo / 대한평형의학회지

Arnold-Chiari malformation type 1 is a congenital disease characterized by herniation of the cerebellar tonsils through the foramen magnum. Most common clinical symptom is pain, including occipital headache and neck pain, upper limb pain exacerbated by physical activity or valsalva maneuvers. Various otoneurological manifestations also occur in patients with the disease, which has usually associated with dizziness, vomiting, dysphagia, poor hand coordination, unsteady gait, numbness. Patients with Arnold-Chiari malformation may develop vertigo after spending some time with their head inclined on their trunk. Positional and down-beating nystagmus are common forms of nystagmus in them. We experienced a 12-year-old female who presented complaining of vertigo related to changes in head position which was initially misdiagnosed as a benign paroxysmal positional vertigo.

Comparison of the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari I malformation associated with syringomyelia and atlantoaxial instability / 中国基层医药

Objective@#To compare the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari I malformation associated with syringomyelia and atlantoaxial instability.@*Methods@#From January 2016 to October 2017, 36 cases of Chiari I malformation associated with syringomyelia and atlantoaxial instability in Linfen People's Hospital were selected in the research.The patients were divided into two groups according to random number table method, with 18 cases in each group.The observation group was given decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment.The control group was given traditional decompression of posterior fossa + posterior cervical fusion and internal fixation treatment.The efficacy, complications occurred within 1 year after operation, JOA score and spinal cord cavity size at different time points before and after operation of the two groups were compared.@*Results@#The total effective rate of the observation group [94.44%(17/18)] was slightly higher than that of the control group [88.89%(16/18)] (χ2=1.957, P>0.05). The total effective rate of the observation group at 1 year after operation was 88.89%(16/18), which was significantly higher than that of the control group [72.22%(13/18)] (χ2=3.498, P<0.05). The incidence rate of complications of the observation group at 1 year after operation was 11.11%(2/18), which was significantly lower than that of the control group [38.89%(7/18)](χ2=11.685, P<0.05). The JOA scores at discharge[(15.97±1.25)point] and 1 year after operation[(15.53±1.19)point] of the observation group were significantly higher than those of the control group[(14.21±1.18)point, (14.06±1.15)point](t=3.815, 3.706, all P<0.05). The spinal cord cavity size at discharge [(2.13±0.64)mm] and 1 year after operation[(2.28±0.69)mm] of the observation group were significantly higher than those of the control group [(3.41±0.76)mm, (3.45±0.78)mm](t=5.743, 5.511, all P<0.05).@*Conclusion@#The long-term efficacy of decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment on Chiari I malformation associated with syringomyelia and atlantoaxial instability is remarkable, the recovery of spinal cord function and syringomyelia is well and the postoperative complications incidence is relatively low, which is worthy of popularization and application.

Comparison of the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability / 中国基层医药

Objective To compare the efficacy of different decompressions treatment combined with posterior cervical fusion and internal fixation treatment on Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability.Methods From January 2016 to October 2017,36 cases of Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability in Linfen People's Hospital were selected in the research.The patients were divided into two groups according to random number table method,with 18 cases in each group.The observation group was given decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment.The control group was given traditional decompression of posterior fossa + posterior cervical fusion and internal fixation treatment.The efficacy,complications occurred within 1 year after operation,JOA score and spinal cord cavity size at different time points before and after operation of the two groups were compared.Results The total effective rate of the observation group [94.44％ (17/18)] was slightly higher than that of the control group [88.89％ (16/18)] (x2 =1.957,P ＞ 0.05).The total effective rate of the observation group at 1 year after operation was 88.89％ (16/18),which was significantly higher than that of the control group [72.22％ (13/18)] (x2 =3.498,P ＜ 0.05).The incidence rate of complications of the observation group at 1 year after operation was 11.11％ (2/18),which was significantly lower than that of the control group [38.89％ (7/18)] (x2 =11.685,P ＜ 0.05).The JOA scores at discharge[(15.97 ± 1.25) point] and 1 year after operation[(15.53 ± 1.19) point] of the observation group were significantly higher than those of the control group [(14.21 ± 1.18) point,(14.06 ±1.15) point] (t =3.815,3.706,all P ＜ 0.05).The spinal cord cavity size at discharge [(2.13 ± 0.64) mm] and 1 year after operation [(2.28 ± 0.69) mm] of the observation group were significantly higher than those of the control group [(3.41 ±0.76)mm,(3.45 ±0.78)mm] (t =5.743,5.511,all P＜0.05).Conclusion The long-term efficacy of decompression of posterior fossa of small bone window + cerebellar tonsillectomy + posterior cervical fusion and internal fixation treatment on Chiari Ⅰ malformation associated with syringomyelia and atlantoaxial instability is remarkable,the recovery of spinal cord function and syringomyelia is well and the postoperative complications incidence is relatively low,which is worthy of popularization and application.